The 47th Bubble Wrap

Cite this article as:
Currie, V. The 47th Bubble Wrap, Don't Forget the Bubbles, 2021. Available at:
https://dontforgetthebubbles.com/the-47th-bubble-wrap/

With millions upon millions of journal articles being published every year it is impossible to keep up.  Every month we ask some of our friends from PERUKI (Paediatric Emergency Research in UK and Ireland) to point out something that has caught their eye.

Article 1: The safety profile of ceftriaxone

Zeng, L., Wang, C., et al., (2020) Safety of ceftriaxone in paediatrics: a systematic review. Archives of Disease in Childhood. Oct;105(10):981-985. doi: 10.1136/archdischild-2019-317950

What’s it about? 

Ceftriaxone is one of the most commonly prescribed antibiotics for children. It is a broad spectrum third generation cephalosporin, used as a first line empirical agent for meningitis, sepsis and useful against many bacterial infections. It has an elimination half-life of 8 hours and can be given once every 24 hours IV or IM, giving us options when that cannula is particularly tricky! Whilst it is well known that using ceftriaxone in the newborn is contraindicated due to biliary sludging, the authors of this paper delved into the literature to identify other adverse reactions (ADRs) to ceftriaxone.

What did they do?

The authors performed systematic searches across several databases looking for studies to evaluate the type of ADR, the incidence of ADRs in patients aged 0 – 18 years old and to identify any potential risk factors for serious ADRs. A total of 112 studies were identified (22 RCTs, 61 case reports, 19 prospective studies, 7 retrospective studies, 2 case series and 1 case control study) which reported on ADRs of ceftriaxone use (although it was not a primary outcome measurement in any of the studies).

Looking at the RCTs, prospective and retrospective studies, gastrointestinal side effects were the most common ADR (specifically, diarrhoea). The second most common ADR identified amongst these studies was hepatobiliary (biliary sludging and cholelithiasis). These ADRs were all transient, and usually self-resolved after cessation of ceftriaxone. The case reports and case studies identified the more serious ADR of immune haemolytic anaemia, which carries a risk of death, especially for patients with an underlying diagnosis of sickle cell disease.

Clinically Relevant Bottom Line:

Transient gastrointestinal side effects are generally tolerable, and we should closely monitor patients for evolving symptoms of gallstones. Most importantly, we should be mindful and cautious when prescribing ceftriaxone in patients with underlying haematological conditions such as sickle cell anaemia, due to the uncommon but significant risk of immune haemolytic anaemia. Ceftriaxone is really a great antibiotic, and as long as we remember the clinical spectrum of ADRs, we will not cause significant patient harm.

Reviewed by: Tina Abi Abdallah

Article 2: Kawasaki Disease vs Septic Shock: Early Differentiating Features Despite Overlapping Clinical Profiles

Power A, Runeckles K, Manlhiot C, Dragulescu A, Guerguerian AM, McCrindle BW. Kawasaki Disease Shock Syndrome Versus Septic Shock: Early Differentiating Features Despite Overlapping Clinical Profiles. J Pediatr. 2020 Dec 5:S0022-3476(20)31482-7. doi: 10.1016/j.jpeds.2020.12.002. Epub ahead of print. PMID: 33290811.

What’s it about? 

According to literature around 6-7% *of patients with Kawasaki disease present with shock and this can provide a challenge in differentiating Kawasaki disease from septic shock. This paper looks to compare clinical features, resuscitative measures and haemodynamic response to treatment between those presenting with Kawasaki disease shock syndrome and children with septic shock.

*Kanegaye JT, Wilder MS, Molkara D, Frazer JR, Pancheri J, Tremoulet AH, et al. Recognition of a Kawasaki Disease Shock Syndrome. Pediatrics 2009;123:e783-9.

What did they do?

This was a retrospective chart review of patients under the age of 18 over a 10-year period admitted to a tertiary centre in the USA. The charts of children who met the criteria for Kawasaki disease shock syndrome (as defined by the American Heart Association) were analysed and children meeting the criteria for septic shock were used as controls. Over the 10-year period >1000 children were admitted to the centre with Kawasaki disease. Of these 9 met the criteria for Kawasaki disease shock syndrome. They were case matched with 18 controls who were admitted with septic shock.

The study found that children with Kawasaki disease shock syndrome were less likely (1 in 9) to have an underlying significant medical illness than the septic shock group (11 in 18). All the patients in the Kawasaki group had at least one of the five classic features of Kawasaki disease at presentation (rash, conjunctivitis, mucous membrane changes, cervical lymphadenopathy and extremity changes). With rash found in 7 of 9 of the patients either at presentation or during the admission. 5 in 9 of the Kawasaki disease cohort had cardiac involvement with zero of the control group with any cardiac involvement.

The length of stay for children in the Kawasaki disease shock syndrome group was a median of 9 days vs 28 days in the septic shock group, with no difference found in ICU length of stay. Biochemical markers were compared, and this study found a lower platelet count (median 140 vs 258) in the Kawasaki group. Interestingly in children with Kawasaki disease shock syndrome the duration of illness prior to admission was much longer (9 days vs 3 days) than the control group.

There have been no studies that directly compare children with Kawasaki disease shock syndrome and septic shock, so this acts as a starting point. However, it is a very small cohort (only 9 patients out of >1000 presentations of Kawasaki disease with Kawasaki shock syndrome); perhaps a multi- centre trial within a network could be done to increase the numbers.

Clinically Relevant Bottom Line:

This study has found that when compared to children with septic shock children with Kawasaki disease shock syndrome are more likely to have a lower platelet count on admission, a longer duration of illness prior to admission, cardiac involvement if an echo is performed and have a longer stay in hospital. All the patients in this study had at least one of the classic features of Kawasaki disease with rash being the most common here. As clinicians who review these children at the front door perhaps a child with a rash and low platelets fever >5 days will continue to make us think about Kawasaki disease.

Reviewed by: Vicki Currie

Article 3: Is it necessary to evaluate urinary tract infection in children with lower respiratory tract infection?

Kim JM, Koo JW, Kim H-B. Is it necessary to evaluate urinary tract infection in children with lower respiratory tract infection? Journal of Paediatrics and Child Health. 2020 Dec;56(12):1924-1928

What’s it all about?

Lower respiratory tract infections (LRTIs) and urinary tract infections (UTIs) are common childhood infections that previous literature has reported to have a concomitance rate of 3 to 10 per 100 children. While LRTIs are often self-limiting viral infections, UTIs are often caused by a bacterial source that can have long term implications if not adequately treated.

What did they do?

This was a retrospective review of 1574 patients’ medical records under 36 months of age who were hospitalised for a LRTI over a 2 year period in a South Korean hospital. 278 of patients had a fever and underwent a subsequent urine evaluation performed either by catheterisation (<24mo) or voided urine (24-36mo).

Patients with a congenital airway or kidney disease, absence of fever at presentation or whose parents refused or failed to undergo a urinalysis were excluded from the analysis.

The overall prevalence rate of a concomitant UTI with LRTI in this population was 1 in 10 in children <36mo and 13 in 100 in children <24mo. Mean age was significantly younger in the UTI group 7 months vs 12 months in the non UTI group. There was a greater prevalence rate of UTIs in boys (n=23) compared to girls (n=7). The most common organism cultured in the UTI group was Escherichia coli which were all treated with a third-generation cephalosporin. The positive rate of virus detection was 93.3% in the UTI group, and 89.9% in the non-UTI group. Most frequently detected co-infections were adenovirus, rhinovirus, and RSV.

The Bottom Line:

LRTIs and UTIs are common childhood infections that have up to a 1 in 10 concomitance rate.  A child presenting with a LRTI and concomitant UTI may present to ED with early respiratory and non-specific symptoms of a UTI (fever, lethargy and irritability), which may lead clinicians to presume a respiratory source of infection and not perform or delay a urinalysis. Hence a diagnosis of an underlying UTI may be missed. Failure to diagnose and promptly treat an underlying UTI can lead to renal morbidity including renal scars, hypertension and chronic kidney disease. Considering the ease of diagnosing and treating a UTI, this study further reiterates the importance of excluding a UTI in children with LRTIs under 36 months of age, especially of male gender. However, given the nature of this single centre study in South Korea, these findings cannot be generalised to a global population and must be taken in context to the population you encounter in clinical practice.

Reviewed by: Emma Chan

Article 4: Why don’t kids get sick with COVID-19?

Zimmermann P, Curtis N., Why is COVID-19 less severe in children? A review of the proposed mechanisms underlying the age-related difference in severity of SARS-CoV-2 infections Arch Dis Child 2020;0 1-11

What’s it about?

A review article analyzing the possible mechanisms for reduced severity of COVID-19 in paediatric patients. The debate about if children have a lower rate of COVID-19 infection continues but it is known that children are less severely affected (in contrast to other respiratory viruses). This appears to be true even in paediatric patients with immune suppression or preexisting conditions e.g. IDDM. What we don’t know is why. The authors look at the evidence for multiple hypotheses but the two they favor are:

1)     Age related endothelial damage and increased coagulability. This fits the clinical profile of COVID-19 which features endotheliitis, micro thrombi, thrombotic complications and vasculitic skin manifestations. It could also explain COVID-19 being more severe in conditions which damage the endothelium e.g. hypertension and diabetes.

2)     Age related changes to the immune system. There is a decline in innate and adaptive immunity in the elderly compared with children who have not gone through this decline. The chronic proinflammatory state (which predisposes to the cytokine storm seen in severe COVID-19) increases with age. Additionally the authors hypothesize that the effect of chronic CMV infection on T-cells may explain the worsening of COVID-19 with age.

The authors concluded that these were the only two hypotheses which fit with the age-gradient in COVID-19 with mortality and morbidity rising steeply after 60-70.

The bottom line

If we could figure the ‘magic formula’ protecting children against severe COVID-19 we could use this to target treatment in adults. However, this paper is very much exploring theories and cannot yet be extended to clinical treatments.

The interplay between a lack of endothelial damage, lack of propensity to hyper-coagulation and their not yet declined immune system are most likely to protect children from severe COVID-19 infection.

Reviewed by: Sarah Reynolds

Article 5: A Gut Feeling: Abdominal Symptoms as an Initial Presentation of EVALI

Christel Wekon-Kemeni, MD, Prathipa Santhanam, MD, Pallav Halani, MD, Lauren Bradford, MD, Ceila E. Loughlin, MD.A Gut Feeling: Abdominal Symptoms as an initial presentation of EVALI, Paediatrics Volume 147, number 1, January 2021.

What’s it about?

 

Vaping or electronic cigarette use associated lung injury (EVALI) is a syndrome resulting from electronic cigarette use which causes predominantly respiratory symptoms, such as shortness of breath.

This case report describes an American 13-year-old male presenting, on two occasions primarily with abdominal symptoms of pain, nausea and vomiting. Initially, he was managed as a case of gastroenteritis, and had been noted to have borderline saturations. Initial abdominal CT report described bilateral lung pathology (lower lobe consolidation and atelectasis) in addition to mild jejunal loop thickening. However, after a second admission with similar symptoms plus raised inflammatory markers and fever, further workup was commenced. 

Repeat abdominal CT excluded appendicitis and evidence of inflammatory bowel disease. CXR revealed bilateral changes and a Thoracic CT identified multifocal ground-glass changes and infiltrates bilaterally with scattered septal thickening and dependent bibasal opacities.

Following a review of the patient by the respiratory team, a year long history of e -cigarette use preceding this patient’s symptoms was discovered, identifying EVALI as the potential diagnosis.

The patient was started on intravenous methylprednisolone which, following an improvement in all symptoms, was converted to a  course of oral corticosteroids. Repeat thoracic CT one month following discharge showed almost complete resolution of the initial changes. 

Why does it matter?

EVALI is a relatively new syndrome, mostly documented in North America, with the potential to increase in prevalence as we see the popularity of e-cigarette use continuing to rise.

Given this patient’s initial symptoms of nausea and vomiting, detailed smoking history to include e-cigarette use may not have been taken. Thus, a workup for abdominal pathology was justifiably completed. However, considering published case reports of EVALI describing nausea and vomiting as common symptoms, this diagnosis should still be considered in patients presenting without respiratory involvement initially. The data available describing EVALI in the paediatric population is sparse, nevertheless in adult’s progression to respiratory failure requiring invasive ventilatory support is reported.

Clinically Relevant Bottom Line:

 Although challenging, obtaining an accurate smoking history to include e-cigarette use in young people is important for the consideration of EVALI as a diagnosis. We still don’t completely understand the pathophysiology of e-cigarettes, or how much damage they are causing to the young people we see who smoke them, but remembering to ask about this as part of your history is a step we can take to improve knowledge and understanding.

Reviewed by: Joshua Tulley

If we have missed out on something useful or you think other articles are absolutely worth sharing, please add them in the comments!

That’s it for this month. Many thanks to all of our reviewers who have taken the time to scour the literature so you don’t have to.

High flow therapy – when and how?

Cite this article as:
Padmanabhan Ramnarayan. High flow therapy – when and how?, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.31730

Isn’t nasal high flow just a fancy name for plain old high flow oxygen? Or is it CPAP-lite? For a therapy that has become so popular in less than a decade, amazingly, there is more opinion sloshing around than proper scientific evidence…

OK, back up, what is Nasal High Flow therapy?

Nasal high flow therapy (NHF), aka high flow nasal cannula therapy (HFNC), is a non-invasive mode of respiratory support, involving the delivery of heated (to 37° C) and humidified (to nearly 100% relative humidity) gas (oxygen and/or medical air) through nasal cannulae at high gas flow rates. What is a “high” gas flow rate is still not uniformly defined (some studies say >2 L/min and others >4 L/min). In physiological terms, to provide the true benefits of “high flow”, the gas flow rate should exceed the patient’s maximal peak inspiratory flow rate (roughly 8-10 x normal minute ventilation).

Makes sense, but what is a child’s peak inspiratory flow rate?

Short answer – it changes according to the age and the extent of respiratory distress. For example, a 4 kg baby breathing at 40 breaths/min and inhaling a tidal volume of 5 ml/kg (=20 ml) would have a minute ventilation of 0.8 L/min and a peak inspiratory flow (PIF) rate of nearly 3 L/min. However, the same infant would have a much higher PIF in respiratory distress. Matching the maximal PIF rate by aiming for roughly 8-10 x normal minute ventilation (in this case, 8-10 x 0.8 L/min = 7-8 L/min) is the key principle of NHF therapy. NB: Magically, the 8 L/min flow rate in this baby is also 2 L/kg/min (more on that later!)

Got it. But when should I start NHF in the ED?

Case 1. A previously healthy 4-month old infant is seen in the emergency department with a two-day history of coryza and poor feeding. On examination, he has mild/moderate subcostal recession and a respiratory rate of 60 bpm. His oxygen saturation in room air is 89%. Would you start nasal high flow?

This baby most likely has mild viral bronchiolitis and the main question is whether to start standard oxygen therapy (SOT) via nasal cannulae or NHF. The most useful clinical outcomes we are interested in are transfer to paediatric intensive care (PICU) and the need for endotracheal intubation.

What does the evidence say?

A recent systematic review (Lin J et al. Arch Dis Child 2019) is an obvious starting point. For the outcome of transfer to PICU, only two RCTs were included (Franklin et al. NEJM, 2018; Kepreotes et al. Lancet 2017). The overall risk ratio was 1.30 (95% CI 0.98, 1.72) indicating no significant difference between NHF and SOT, although there was a tendency to favour SOT.

Similarly, only two RCTs were included for the outcome of intubation (Franklin et al. NEJM, 2018; Yang et al. Chinese Pediatric Emergency Medicine, 2017). The overall risk ratio was 1.98 (95% CI 0.60, 6.56), again with no significant difference between NHF and SOT. So, not much joy from the systematic review…

Considering that Franklin et al dominated the systematic review in terms of sample size, it is useful to look at this RCT in a bit more detail, from a PICO point of view as well as the flow of patients through the RCT.

Population: Infants <12 months of age with bronchiolitis and needing supplemental oxygen

Intervention: NHF at 2 L/kg/min

Control: Standard oxygen therapy

Outcome: Escalation of care due to treatment failure (composite outcome)

A few reflections on the outcomes of infants in this RCT: although nearly double the number of infants randomised to SOT “failed treatment” compared to NHF, it is notable that over 75% of infants randomised to SOT did not “fail”; the majority of those who did “fail” SOT were rescued by NHF; and since NHF “failure” automatically led to PICU transfer, in effect, more infants were transferred to PICU in the NHF group than in the SOT group (12% vs 9%). Essentially, this RCT could be considered a trial of ‘immediate’ NHF versus ‘rescue’ NHF, as covered by us here previously.

Bottom line: There is no advantage to starting NHF as first-line therapy in an infant with mild bronchiolitis. A more clinically and cost-effective strategy would be to use NHF as ‘rescue’ therapy when standard oxygen therapy has failed.

Case 2. An ex-prem born at 24 weeks gestation, now 4 months old, is seen in the emergency department with a 24-hour history of coryza and cough. On examination, he has moderate/severe subcostal recession and a respiratory rate of 80 bpm. His oxygen saturation in room air is 85%. Would you start nasal high flow?

This baby is much sicker, with significant past medical history, and most likely has moderate/severe bronchiolitis. Would NHF be more useful as first-line therapy in this infant, where previously nasal CPAP would have been an option – can NHF be used as ‘CPAP-lite’? A really useful clinical outcome to focus on is endotracheal intubation.

What does the evidence say?

Lin et al summarise the evidence in their recent systematic review. For the outcome of intubation, 4 RCTs were included, but the total number of patients included were low (n=264). Intubation rates were identical in the NHF and CPAP groups (5.3%), with a risk ratio of 0.96 (95% CI 0.35, 2.61). So, there is not much evidence to support the use of NHF compared to CPAP, although quite notably, the rate of adverse events was lower in the NHF group (8% vs 21%).

Bottom line: There is no clinical advantage to starting NHF as first-line therapy in an infant with moderate to severe bronchiolitis to avoid intubation. However, its adverse event profile and tolerability by infants might make NHF more appealing as first-line therapy.

When should I start NHF in the HDU?

Case 3: A 5-year old boy with cerebral palsy and epilepsy is admitted to the paediatric HDU bed with fever, cough and respiratory distress. On examination, he has moderate subcostal and intercostal recession and a respiratory rate of 45 bpm. His oxygen saturation in room air is 88%. Would you start nasal high flow?

In this older child with a complex past medical history, is starting NHF, compared to either standard oxygen or CPAP, beneficial in terms of avoiding the need for endotracheal intubation?

What does the evidence say?

A recent systematic review (Luo J et al. Journal of Pediatrics, 2019) is an obvious starting point. In the comparison of NHF versus SOT, 5 RCTs were included, although 2 were focussed on bronchiolitis (previously covered – Franklin et al and Kepreotes et al). The other 3 RCTs were small (Chisti et al. Lancet, 2015; Ergul et al. Eur J Pediatrics, 2018; Sittikharnka et al. Indian J Crit Care Med 2018) with just 300 patients in total. The overall risk ratio for intubation from these 3 studies alone (calculated specifically for this post) was 0.72 (95% CI 0.38, 1.36). Similarly, in the comparison of NHF versus CPAP, 4 RCTs were included but 2 were in bronchiolitic infants (covered earlier). The other two RCTs (Ramnarayan et al. Crit Care 2018; Chisti et al. Lancet 2015) included just 187 children. The overall risk ratio for intubation based on these two RCTs (calculated for this post) was 2.14 (95% CI 0.93, 4.92) indicating a tendency for a higher intubation rate with NHF in older children.

Bottom line: In an older child, intubation was not less frequent when NHF was used compared to SOT as first line therapy. There was a tendency for NHF to be associated with a higher intubation rate compared to CPAP.

Great – what is the best way to provide NHF?

Starting gas flow rate

Milesi et al showed in physiological studies in infants aged <6 months with bronchiolitis that the work of breathing is reduced considerably when the gas flow rate is set at nearly 2 L/kg/min. In their cohort of 21 infants (mean weight 4.3 kg), the measured work of breathing was lowest at a flow rate of 7 L/min (compared to 1, 4 and 6 L/min). Similarly, in children up to the age of 3 years with pneumonia, work of breathing was reduced by nearly 20% at a flow rate of 1.5 L/kg/min compared to 0.5 L/kg/min (Weiler et al. Journal of Pediatrics 2017). Usual adult flow rates range from 50-60 L/min.

In summary, the optimal gas flow rate does not increase in a linear fashion with increasing age/weight, instead it goes from nearly 2 L/kg/min in infancy to nearly 1 L/kg/min in young adults.

RCTs of different starting flow rates

There have been two RCTs comparing NHF flow rates in bronchiolitis (Yurtseven A et al. Ped Pulm 2019; Milesi et al. Intens Care Med 2018). In the former, 1 L/kg/min (n=88) was compared to 2 L/kg/min (n=80) in infants <24 months with clinical severe bronchiolitis presenting to the emergency department. The primary outcome was ‘treatment failure’ within 24 hours. There was no significant difference in treatment failure between the two flow rates (1 L/kg/min: 11.4%; 2 L/kg/min: 10%). The second RCT compared 2 L/kg/min (n=142) with 3 L/kg/min (n=144) in infants aged <6 months with moderate/severe bronchiolitis. The primary outcome was treatment failure within 48 hours. There was no significant difference in treatment failure between the two groups (2 L/kg/min: 38.7%; 3 L/kg/min: 38.9%).

A useful chart with suggested starting flow rates based on weight is used in the ongoing FIRST ABC clinical trial of NHF versus CPAP.

Nasal cannula size

There are different nasal cannula sizes available based on the manufacturer. The general rule of thumb is that the cannula prongs should be no more than 50% of the diameter of the nostril to avoid inadvertent occlusion of the nasal passages. It is also advisable to start the gas flow rate at a low rate and then increase gradually over 10-15 min to avoid patient discomfort. Pacifiers may be useful in babies to prevent mouth opening.

Weaning NHF

There are no RCTs comparing weaning strategies for NHF. Clinical practice is also highly variable – in a global survey of practice in over 1000 PICU professionals by Kawaguchi et al, 68% weaned the FiO2 first to a threshold value (e.g. 0.40) and then weaned the flow rate gradually, 11% weaned the FiO2 first to a threshold value (e.g. 0.40) and then stopped NHF, and 4% weaned the flow rate alone without weaning the FiO2. The FIRST ABC RCT algorithm for the weaning of NHF provides a weight-based approach to a one-step weaning process and suggested clinical thresholds for weaning and stopping NHF.

The take homes

  • Nasal high flow is a form of non-invasive respiratory support that sits somewhere between standard oxygen therapy and nasal CPAP.
  • In infants with mild bronchiolitis, there is no clinical (or cost) benefit in starting NHF as first-line treatment – rather, NHF is best used as a ‘rescue’ therapy after standard oxygen.
  • In infants with moderate/severe bronchiolitis, NHF may be a useful first-line therapy owing to its ease of use and since it is better tolerated by infants, however there is no clinical benefit compared to nasal CPAP.
  • In older children with respiratory failure, there is little RCT evidence to guide practice – however, there is no clear benefit of starting NHF over and above standard oxygen. NHF may be associated with a higher intubation rate compared to CPAP in older children.
  • There is no RCT evidence to support either 1, 2 or 3 L/kg/min NHF flow rates in infants with bronchiolitis; however, physiological evidence suggests that nearly 2 L/kg/min is associated with reduction in work of breathing. Suitable flow rates in older children approximate 1.5 L/kg/min and in young adults, 1 L/kg/min.
  • There is no RCT evidence to support one way of weaning over another – the most common practice seems to be to reduce FiO2 to below 0.40, followed by a reduction in the NHF flow rate.
  • Ongoing RCTs such as the FIRST ABC trial will help address the question whether NHF is non-inferior to CPAP in critically ill children.

The 46th Bubble Wrap

Cite this article as:
Currie, V. The 46th Bubble Wrap, Don't Forget the Bubbles, 2021. Available at:
https://dontforgetthebubbles.com/the-46th-bubble-wrap/

With millions upon millions of journal articles being published every year it is impossible to keep up.  Every month we ask some of our friends from PERUKI (Paediatric Emergency Research in UK and Ireland) to point out something that has caught their eye.

Article 1: Ten Tips for Breaking Bad News

Brouwer, M.A., Maeckelberghe, E.L.M., van der Heide, A., et al., Breaking bad news: what parents would like you to know (2020) Archives of Disease in Childhood Published Online First: 30 October 2020. doi: 10.1136/archdischild-2019-318398

What’s it about? 

Difficult conversations in paediatrics often revolve around conditions which reduce life expectancy, such as oncological, metabolic, cardiac and neurological diagnoses. In the case of new diagnoses, difficult discussions may happen in the emergency department – an environment that is not designed for sensitive and long (ideally interruption free) discussions. 

This article reviews the experiences of parents who were involved in difficult conversations surrounding their child’s care or condition and provides practical advice on how to provide empathic, timely and optimal communication.

Based in the Netherlands, the authors recruited bereaved and non-bereaved parents of children aged between 1 and 12 years with life threatening conditions. Between November 2016 and October 2018, face to face interviews with the parents were conducted and transcribed verbatim.

Using transcripts, key themes and ten clear barriers to the communication of bad news were identified. The authors then reviewed the transcripts at length to identify positive feedback when breaking bad news; with the key aspects being where and when conversations took place, who was present for the conversation, and the honesty and information that was given.

Why does it matter? 

Breaking bad news or caring conversations are part of our every day work. But for the families and children receiving the information, the high emotional and practical significance means that they remember these conversations for a long, long time. The onus on us as professionals is to develop and grow the insight and skill to thoughtfully, effectively and compassionately communicate during these conversations.

Clinically Relevant Bottom Line:

Communication skills remain the cornerstone of medical practice. Feedback from patients and family often revolves around communication with the team caring for them, and whether it was “good” or “bad”. This article highlights some important factors to optimise communication when breaking bad news, which can be (and should be) utilised on a daily basis.

Reviewed by: Tina Abi Abdallah

Article 2: Risk of traumatic intracranial haemorrhage in children with bleeding disorders

Bressan, S., Monagle, P., Dalziel, S.R., Borland, M.L., Phillips, N., Kochar, A., Lyttle, M.D., Cheek, J.A., Neutze, J., Oakley, E., Dalton, S., Gilhotra, Y., Hearps, S., Furyk, J., Babl, F.E. (2020). Risk of traumatic intracranial haemorrhage in children with bleeding disorders. J Paediatr Child Health, 56: 1891-1897.

What’s it about? 

This multi-centre prospective observational study aimed to assess the rate of  CT use and frequency of diagnosing intracranial haemorrhage (ICH) on CT, in children with bleeding disorders presenting with head trauma.

20 137 children were evaluated in Australian and New Zealand EDs for head trauma, with or without bleeding disorders between April 2011 and November 2014. Congenital or acquired bleeding disorders were present in 0.5% of this population. Head CT use was significantly higher in children with bleeding disorders than those without (3 in 10 vs 1 in 10) despite the latter group presenting more frequently with severe mechanisms of head injury. Children with bleeding disorders who received CT were more likely to present with milder mechanisms of injury as well as clinical signs of vomiting and abnormal behaviour reported by parents, compared to children with bleeding disorders who did not receive CT scans . Only one child with a bleeding disorder had an ICH requiring neurosurgical intervention and no children without CT imaging had evidence of ICH on follow-up.

Why does it matter? 

Minor head injuries present frequently to paediatric EDs. Children with bleeding disorders are at increased risk of ICH following a minor head injury than those without bleeding disorders. Patients with severe haemophilia are reported to have the highest risk of traumatic ICH within this heterogenous disease group. It is important to detect ICH early in order to avoid long term disability and potentially fatal outcomes whilst balancing the decision for imaging against the risks of repeated radiation exposure. Previous clinical decision rules have supported ED clinicians in making judgements on CT use for paediatric head injuries but there is little evidence or guidance on its use for children with bleeding disorders.

Clinically Relevant Bottom Line:

The low incidence of ICH in children with bleeding disorders receiving CT imaging suggests that CT scans may not be routinely necessary in children with congenital or acquired bleeding disorder. The authors suggest a more selective approach to CT decision-making, combining a period of clinical observation with the severity of injury mechanism and the underlying bleeding disorder, rather than a “CT all” strategy.

However, the study is limited in its analysis by the low number of children with bleeding disorders. It would also be interesting to note from Bressan et al.’s study whether the rate of CT use varied with patients’ GCS scores or age of presentation, given the wide age window of children < 18 years.

 Nonetheless, current head injury rules such as PECARN were designed with the explicit exclusion of children with bleeding disorders. This study can therefore support the development of targeted neuroimaging guidelines for children with bleeding disorders.

Reviewed by: Ivy Jiang

Article 3: Can we safely send paediatric head injuries home from triage?

Aldridge, P., Castle, H., Phillips, C., Russell, E., Guerrero-Luduena, R., Rout, R. (2020). Head home: a prospective cohort study of a nurse-led paediatric head injury clinical decision tool at a district general hospital. Emergency Medicine Journal.

What’s it all about?

Head injuries are a common presentation to emergency departments internationally. Recent Australian data has shown that in >19,000 attendances with head injury only 3 in 100 had a traumatic brain injury on CT or a clinically important brain injury.

This study group set out to assess whether children under 17 years could safely be discharged by triage nurses following a pre-set clinical decision tool (HIDATq- Head Injury Discharge At Triage questionnaire). HIDATq was developed using PECARN and NICE guidelines. For a recap on Head Injury Decision tools see Anna Ing’s ‘Head Injury- who to scan?’ on DFTB.  HIDATq was implemented over a 6-month period in children who presented with a head or facial injury to a DGH in the UK.

Over 1700 patients were assessed, and data was analysed retrospectively. 61% were HIDATq negative and 1 in 5 of these patients were felt to be safe for discharge from triage without further investigation or management. A further 3 out of 10 children in the HIDATq negative patients were found to be eligible for discharge following minor wound management. 4 % of patients underwent CT scans (only 1 patient from the HIDATq negative group).

Why does it matter?

Head injuries are a common presentation to the paediatric ED. This study has revealed a patient group who might be eligible, using this screening tool, for a safe discharge from triage that would potentially have a large impact on ED crowding and pressures.

Clinically Relevant Bottom Line:

There were no adverse outcomes and the clinical decision tool used produced a high sensitivity and specificity for determining the need for CT after head injury. More than half of the children who had a negative HIDATq were potentially suitable for a safe discharge from triage.

This study did however have a highly selective population- it was not a major trauma centre so by default likely to have had less severe presentations of head injury. A larger multi centre trial is needed to provide validation for the tool. However, this study provides a useful starting point and identifies possible ways to improve patient management and ED departmental pressures.

Reviewed by: Brent Stevenson

Article 4: Should POC blood ketones be used as a triage tool to assess dehydration and predict likely admission?

Durnin, S., Jones, J., Ryan, E., Howard, R., Walsh, S., Dawkins, I., Blackburn, C., O’Donnell, S.M. and Barrett, M.J., 2020. The utility of ketones at triage: a prospective cohort study. Archives of Disease in Childhood105(12), pp.1157-1161.

What’s it about?

This is a small non-blinded prospective cohort study looking at 198 patients aged 1m-5yrs over a 12-month period. The eligibility criteria were presentation with vomiting/diarrhea/decreased fluid intake or clinical concerns of possible hypoglycaemia. Patients had finger prick blood ketones measured at triage, along with a Gorelick 4-point dehydration score. Repeat ketone measurement at 4hrs later or at discharge, clinical assessment and a 10-point Gorelick dehydration score (a Gorelick score is a validated tool to predict significant dehydration for children aged 1 month to 5 years).

The authors found a weak correlation between POC ketone level and the 10-point Gorelick dehydration scale (a more detailed assessment) and no correlation between POC ketones and Gorelick 4-point dehydration scale score.

Ketone level at triage was not predictive of admission however repeat measurement at 4hrs was weakly predictive; meaning, a larger proportion of the discharged cohort showed a reduction in ketones after rehydration compared to the admitted cohort.

Why does it matter?

Assessing dehydration is an inexact science and an accurate POC test for dehydration would simplify and potentially improve patient care. The Gorelick 4-point scale has previously shown to be oversensitive for assessing percentage dehydration but scales are better than unstructured assessment. This study rules out blood ketones as a tool for assessment of degree of dehydration or for predicting admission in this patient group.

The bottom line

Blood ketones are of little use as a triage tool for assessing degree of dehydration or predicting hospital admission in children with reduced fluid intake / D&V. There is no benefit to routine measurement of blood ketones at triage in patients with dehydration who do not have concerns about potential DKA.

Reviewed by: Sarah Reynolds

Article 5: Is loop-mediated isothermal amplification a useful tool for early identification of invasive meningococcal disease?

Waterfield, T., Lyttle, M.D., McKenna, J., Maney, J.A., Roland, D., Corr, M., Woolfall, K., Patenall, B., Shields, M. and Fairley, D., 2020. Loop-mediated isothermal amplification for the early diagnosis of invasive meningococcal disease in children. Archives of Disease in Childhood105(12), pp.1151-1156.

What’s it about?

A point of care test: Loop mediated isothermal amplification (LAMP) is a potential test for early identification of invasive meningococcal disease (MD).  (LAMP) is a form of rapid nucleic acid amplification and a commercially available LAMP test (using oropharyngeal swabs) can test for all serotypes of Neisseria meningitidis. This study looked to evaluate the diagnostic accuracy of LAMP for identifying invasive (MD) in children and to compare LAMP testing with more familiar tests like CRP and white cell counts (WCC).

 263 children under the age 18 with fever and signs or symptoms of meningococcal septicaemia were included over a 2-year period in 3 ED’s across the UK. 97% of participants were appropriately vaccinated as per UK vaccination schedule with over 1 in 2 of these children having had the Men B vaccination and over 2 in 3 children who had received the Men C vaccine.

Less than 2 per 100 children had confirmed cases of invasive MD. There were 14 positive LAMP tests, and all the confirmed cases of invasive MD were within these. In all the children with negative LAMP tests NONE had invasive MD. The LAMP test in this population performed better than other more commonly used tests (CRP, WCC or neutrophil counts).

Why does it matter?

Whilst vaccination programmes have thankfully made invasive MD more uncommon, it is still a significant cause of morbidity and mortality in children when it does occur. 

Early diagnosis is challenging, meaning potential overuse of broad-spectrum antibiotics or false reassurance for the clinician.

A point of care test for N. meningitidis, which is easy to do, with a low false negative rate has the potential to change this. However, false positives, meaning asymptomatic carriage must be considered.  The study included mostly young children and adolescents who are known to have higher asymptomatic carriage rates of N. meningitis, meaning false positive could be higher in this group.  It has the potential to be used as an adjuvant to PCR and blood culture, but the optimum patient group selection is yet to be determined and it could not be used as a rule out test in low prevalence areas like the UK.

Clinically Relevant Bottom Line:

LAMP testing for IMD, is a potentially useful test to identify children with invasive MD rapidly. However, clinical utility is yet to be determined.

Reviewed by: Sarah Kapur

If we have missed out on something useful or you think other articles are absolutely worth sharing, please add them in the comments!

That’s it for this month. Many thanks to all of our reviewers who have taken the time to scour the literature so you don’t have to.

Conversations about constipation

Cite this article as:
Chris Dadnam. Conversations about constipation, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.31554

Like most of you I have to deal with the issues of constipation within the ED or CAU environment and most of the time it’s not the reason the child attends the department! This can then lead to a series of awkward questions and issues that parents may ask that we need to consider in order to provide safe, useful and most of all, worthwhile advice! 

So let’s go through these questions ask issues:

1. My child has a UTI, why are you talking to me about constipation? 

This is something I get asked not only when a UTI is diagnosed but other clinical conditions including; appendicitis, bedwetting, incontinence, urinary retention, obstruction, etc…. it is important that parents understand the implications of constipation, not only from a pain and symptoms point of view but also the complications surrounding it. Indeed, many parents also struggle to understand how their child, who is rolling around in agony, is only suffering from constipation (you can literally feel them questioning your medical acumen). 

During these tough times I always mention two key points. Firstly, the fact that your bowel covers the majority of your abdomen. A build up of wind and solid matter in the bowels can bring about severe griping abdominal pain, when pressing against sensitive nerves. As it covers a lot of your abdomen, when full, it will compress other structures like your child’s bladder leading to urinary infections, incontinence and retention. The second point is that stools are like a toxin your body wants to expel, when it remains in your bowels it can get into small structures like your child’s appendix and cause it to become inflamed and that leads to appendicitis.

Also, the longer the stools sit in the colon the more water is absorbed leading to harder, solid stools. This will cause a blockage and lead to vomiting and obstruction which may require surgery. 

Once parents have a better understanding of these points they’re less likely to roll their eyes at constipation! 

2. How are they constipated, they go every day? 

This in fairness is a good question, one that used to throw me quite a bit in my early paediatric years, but let’s break it down. Constipation is not simply the length of time between going to the toilet, rather it is the build up and insufficient clearance of stools in your bowels. With this in mind, a child can go daily and pass small amounts of stool but still have a backlog of faecal matter in their bowels. Therefore also question the time spent on the toilet, straining and pain during defecation. These are all signs of constipation. If you can, ask your young patients too! 

3. They already drink plenty of fluids

Don’t let this answer fool you, explore the parents’ meaning of fluids. When we are saying it, we mean clear liquids like water and squash (preferably sugar-free), but for the parents, it’s anything from water and tea, to milk and hot chocolates. Now there is a myth that milk makes us constipated but that’s simply not the case….well not entirely. Milk is indeed a liquid and it doesn’t make us constipated but it’s thicker and takes longer to digest (hence why in surgery they stress only clear liquids 4hrs before). Milk fills up the child and so reduces their intake of solid food which will be full of fibre. 

Always remember, parents may say they’ve cut out all the milk, but this may have been substituted for milky teas and hot chocolates, so double check! 

In terms of managing the milk, appreciate the difficulty the family is about to face. Wean the milk down slowly, starting with the bottles in the middle of the day, then the morning bottle and finally the night bottle. Milk shouldn’t be stopped entirely, having a 250-500ml glass of milk daily is perfectly fine. 

4. They eat a really good diet 

Whenever I get this response, I immediately think they haven’t and 99% of the time I’m right……says a lot. 

Again, this is either due to a misconception as to what a good diet is, or they don’t think it’s an important issue so they simply brush it off with this generic statement so that they can get to the medication that will actually help. Another quick way to check is to just ask the child. They normally find it much more difficult to turn a blind eye.

I always try to tackle this in one of two ways:

Tell me what they eat?

– it’s surprising how many children don’t have breakfast or any of the three square meals a day. If they do, just add in tips when you can.

Breakfast; dried fruits in cereal (especially raisins), don’t switch the cereal completely but rather mix in an all bran, so they’re still getting their tasty favourites, but now with added fibre. Toast – if it’s white bread, freeze it; it’ll keep longer and by placing it straight into the toaster means that the strands of carbs, fats and protein are bound together and form fibre.

Lunch; Try to include salad into sandwiches. If the parents say they don’t like salad then how do they expect their children too! Encourage healthy eating in the parents as well, to form positive connotations for their kids.

Dinner; any sauce can hide a multitude of veggies if blended or chopped fine enough – so get them cooking and where possible get them to encourage their kids to join in. If they cook it themselves, they’ll appreciate the food and, for some reason, enjoy it more……probably a labour of love! And it’s a great time to leave out and pick on a bowl of fresh berries or grapes, the more accessible things are the more they get eaten.

Do they eat all their fruit and vegetables?

This again leads to a classic ‘Yes’ response – which falsely reassures a lot of healthcare professionals. In truth, it’s a vague and rather inadequate question to ask. If I told you that my child eats peeled apples and pears, has a glass of orange juice and then eats loads of veggies which I boil until soft… It might make you think twice about the goodness they’re actually getting.  So I always ask – Do they eat the skins of the fruit and vegetables? How do you prepare them? The skin of most fruits and vegetables holds the majority of fibre along with different vitamins and minerals required. In all honesty, if you are peeling apples and pears, all you’re left with is sugar and water, so I tell parents to give their child the peel instead! 

Again with veggies, I tend to suggest for microwaving or steaming as people tend to overcook them when they boil them. They need a crunch as that equals fibre. Root vegetables (potatoes, sweet potatoes, carrots, butternut squash, celeriac, parsnips) – all these lovely fibre rich foods – contain most of the fibre in their skins. I tell parents to roast them, long and slow – they’ll taste better (caramelizes the sugars in the veg/skin) so children will prefer them! 

Be mindful of smoothies and fruit juices. Yes, they can count for 1 of our 5 a day (soon to be 10 a day) but they can have little to no fibre, especially with the models that separate out the pulp. The pulp is fibre!! Try to get them to have whole fruits instead or 1 x 250ml glass of fruit smoothie a day with the pulp. 

5. I have tried all this and it doesn’t work 

Before you dismiss this answer make sure you look over the medical background again (cystic fibrosis, hypothyroid disease, Hirschsprung’s). Ask these all-important questions:

  • When did they have their very first poo? It should be within the first 48 hours. Then double-check it was a good amount – small smears don’t count.
  • Have they had issues with weight gain and prolonged issues with chest problems (in cystic fibrosis, LRTIs tend to happen towards the end of their first year of life).
  • Did they have a Guthrie / heel prick test. Any developmental delay? A large soft spot on their head?

In all of these conditions, the child would have always had an issue with constipation since birth, so don’t miss them. 

Once covered, it’s important to go through what they have tried….. most parents will only have been given a packet of Movicol and told to get on with it. Look at the summary section to see how to structure a constructive management plan.

6. I’m scared they’ll starve so I give them what they want. 

How many of us have been told this with little Jonny sitting there looking larger than life?

In general, throughout the developed world, children are unlikely to starve if their parents are trying to feed them a healthy balanced diet. There are caveats to this:-

  • Autistic children or children with textural issues. 
  • Children with a background of eating disorders (bulimia or anorexia). 

These children will need extra support and input from community and nutritional teams. 

All the other children will always put up a fight (normally a good one!) but then their bodies will give in and want food. This is an important step for parents to understand, especially when the child is too young to go out and get food themselves. Make sure you tell the parents this won’t be a simple task, and the main reason children normally win, is that carers will be busy and won’t have time to tackle this problem. It’s a quick fix to give them something just so that they know they’ve eaten…….then the habit starts. I always tell parents, wait until you have a week off and prepare yourself/ yourselves for a bumpy ride. Have a united front, it’s no good if one parent plays the ‘strict/ bad guy’ whilst the other literally feeds the problem behind their back. Prepare meals and hide away the unhealthy processed snacks (or don’t buy them in the first place) and leave fruit out. Again, get the child involved in cooking, build a healthy connection with food and make it fun. Children will most likely throw tantrums at the start, but remind them that eventually their child’s body will give in and they will come for food, most likely with a grumpy face. 

Just make sure they’re hydrated with clear fluids! And NO milky substitutes. Remind the parents they’re not bad people and this will help fix things in the long run.

7. Movicol doesn’t work and I don’t want it to make their bowels lazy 

This age-old answer…..makes you wonder why we bother using Movicol? More often than not they’ve not prepared it correctly, despite the instructions being on the box. Honestly, the ways parents use Movicol; sprinkle on cereal, mix into snacks or food, add to tea….the list goes on!

Movicol is only effective when it is bound with water. After this, the parent can then mix it with a small amount of any other liquid or flavouring. Make sure they don’t add it to a litre bottle of squash as the child will have to drink the whole lot. Also, this means they don’t need the flavoured versions (which taste vile – remember when they made you try them in medical school!). 

Another myth is that “it’ll make their bowels lazy.” Explain that Movicol is not a stimulant, it is an osmotic diuretic and acts to drive the water you mixed it with into the child’s stool to make them softer and easier to pass. With this in mind, even stimulant laxatives won’t make your bowels lazy. I always say, they can be on it for the rest of their lives, it’ll never make their bowels lazy – that tends to reassure parents.

8. I tried laxatives before and they suddenly had diarrhoea so I stopped using it. 

This answer may again throw you into thinking that the laxative has done a great job in under 72 hours and fixed a months worth of constipation… it sounds too good to be true?? Well, it is. The big problem here is, if clinicians don’t pre-warn the carers what might happen after starting a laxative, it can lead to long term mistrust in both the medication and in our advice.

I always start off by setting the day to start. Aim to start the laxative at the end of the week, a Thursday or Friday, to avoid accidents in the school. They will deter the child from ever trying them again.

Once we know when to take them, always triple check they’re using them correctly… mix with water first, then add to a small volume of any other liquid for taste.

Finally, but most importantly, the change in stool. Referring to the Bristol stool chart (the only card I carry around!), I explain the child will start with Type1-3 stools. Then, they’ll have what looks like diarrhoea, brown watery smelly stools, but, of course, it’s overflow. Take the time to talk about why this happens; the Movicol is slowly moving through the hard stools, like rain trickling down a wall, in their child’s bowel. The Movicol/water mix will initially run over it but over time their stools start to soften. 

The next step, again important due to risk of pain, is the big logs. And big means big! I’ve had parents say they’ve used shears to cut up these stools in the toilet. This is essentially the wall slowly being emptied out. 

Once this is over, they will finally have the soft mushy stools. The laxative story should not end there! It is important to mention this ‘wall of stool’ has caused the bowels to stretch. This will lead to a build-up of stools again as the child won’t know when they need to go. This is reservoir constipation. It can take months to revert back to normal so I always advise to continue on with the laxatives and reduce (but not stop) the maintenance daily dose down if the child is passing clear watery liquid. Usually, treatment should continue for at least three months to treat reservoir constipation (although in some children it can be longer).

9. They don’t like my cooking. They’re vegetarian now, I’m not. 

It doesn’t have to be vegetarian, of course, this is just what an angry mum said to me once about her daughter. Parents will mention the difficulties of preparing food they’re not used to cooking, I always suggest ‘get your child involved‘. This is the perfect time to do something together (bonus points as well in tackling mental health and isolation. It gets the family talking). The child will appreciate their intent and willingness to give their lifestyle a try, which will build confidence in the relationship as well. They can get a cookbook, go online (it’s all free and easily accessible these days) and adapt their cooking style. Again. this will make their child feel more involved and interested in cooking and eating healthy foods. 

10. They don’t like fruit and vegetables. 

I think we’ve all suffered from hating vegetables and fruit at some point in our lives. I remember hating tomatoes and peppers, so I feel for any parent tackling this problem. 

There are several factors to contend with here:

  • Their child not liking the fruit because it’s unknown to them or feels texturally unsatisfactory.
  • Having access to other more ‘enjoyable’ foods such as biscuits, chocolates and crisps around the house which they can graze on and avoid these unwanted bags of goodness. 
  • Watching older siblings and parents, and copying them.

To this, I normally offer a number of solutions but be mindful that parents will have busy lives around their child’s eating habits so it has to be a conscious effort at a convenient time, like over the weekend or annual leave.  

Firstly, hide unhealthy snacks or simply reduce the amount you buy, what they don’t see they don’t know…. out of sight, out of mind technique.

Secondly, I always tell carers that they and older siblings are role models. If you’re not eating it, why should they? There should be a united front by the parents. 

Finally, get cooking and get your child involved. Any child who cooks will appreciate the food they’ve made and the sense of achievement, even if it doesn’t taste nice, they’ll love it. It’s a great time to chat over a bowl of fresh berries…

My own enthusiasm then tends to kick in. I like to say “Get creative in the kitchen!” I’ve mentioned simple things for example: make flapjacks and throw in lots of dried fruits; raisins, dates, apricots, prunes – all-natural sweetness with skinned nuts & oats. Freeze smoothies with the pulp into ice-lollies. Fruit crumbles with honey and oats… With vegetables, always remember to steam or microwave them, they need the crunch. Again if kids don’t like them…. Chop them up/ blend them and throw them into sauces, pies and mixed into other dishes. If it’s the taste they don’t like, again mixed into gravy or a tasty sauce will fix that problem! 

It is important to mention the importance of a healthy balanced diet. Food is your best medicine. This is can be true for managing many conditions; anaemia , skin problems, poor immunity, nail and hair problems..you get my point. A varied diet holds the key to a lot of management plans, and it’s important to mention this even when the child is on supplements. A classic example is the parent says we’ve fixed the iron problem with iron tablets, but they fail to realise, without vitamin C your body can’t absorb the iron through the small intestine. Therefore, they’ll be questioning why their child remains anaemic in months to come. 

11. They’ve gone back into nappies as they’re scared of using the toilet. 

This is an important issue. It’ll mean the child will probably have problems with incontinence which may be affecting their social life such as staying over at a friends houses. Yet another reason why it needs tackling. 

Always start with asking what happened? More often than not it’s a series of bad habits and untimely events that have led to a regression in the family’s good practice. It happens to the best of us. Reassure everyone and give them the positive reinforcement that they’ve identified a problem that needs to be solved. Then offer the advice below. Take your time with these parents, it would have taken a lot for them to come into an acute setting to seek advice, so try to give them some. 

A framework for managing constipation.

This is ultimately an important topic that you have or, no doubt, will see at some point in your paediatric career. Knowing how to manage it is a core skill. 

I always frame my management in 3 steps: 

1. Diet and fluid intake – take the points from the above questions. Ultimately, the parents control the diet and food at home. They, and older siblings, are the child’s role models so what they eat will influence the child’s diet too. Remind them that food is their family’s best medicine so they need to get it right. Cancel out the milky drinks, cook smart & healthy and don’t forget clear fluids.

2. Toileting – our constipation module covers this but key points; get them into a routine (20 to 30mins after dinner – to sit on the toilet). Make the toilet a fun place with all their toys and gadgets and don’t forget to get them to blow the bubbles. Optimise positioning with knees above bums when sitting, using a footstool. 

3. Medication – ensure parents are giving them correctly – mixing with water first then adding to any other liquid for taste. Make sure this isn’t a full bottle, as they’ll have to drink the lot!. Movicol doesn’t make your bowels lazy. If they’re on a disimpaction regime, think about the volume they’ll be drinking each time. It may be better to split it into thrice daily doses instead. Briefly touch on the sequence of stool changes to reduce misconceptions of overflow and diarrhoea. Lastly start Movicol towards the end of the week, Thursday or Friday to avoid accidents at school. 

End with: 

  • Referencing the ERIC constipation website. It’s a great tool for constipation and bedwetting. It talks to the parent and child, so easy to understand and explain.
  • It will take time for things to fall into place. There is no quick fix. There will be tantrums, sleepless nights and days you’ll want to give in. Hang in there and once you’re sorted you’ll wish you’d done it sooner!

Bubble Wrap PLUS – January

Cite this article as:
Anke Raaijmakers. Bubble Wrap PLUS – January, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.31763

Happy New Year! Most of us have a long list of good intentions for the new year. We hope that keeping up with literature in 2021 is one of them. Here is a new Bubble Wrap Plus, our monthly paediatric Journal Club List provided by Professor Jaan Toelen of the University Hospitals in Leuven (Belgium). This comprehensive list of ‘articles to read’ comes from 34 journals, including Pediatrics, The Journal of Pediatrics, Archives of Disease in Childhood, JAMA Pediatrics, Journal of Paediatrics and Child Health, NEJM, and many more.

This month’s list features answers to intriguing questions such as: ‘Is child abuse more prevalent during the pandemic?’, ‘Can the smartphone replace the Bristol Stool Chart?’, ‘Does ‘one hour’ matter in giving antibiotics in septic shock?’, ‘Does social distancing lead to fewer URTIs?’, ‘Are ketones useful at triage?’ and ‘Should we worry about invasive infections in children with OMA?’.

You will find the list is broken down into four sections:

2. Original clinical studies

Physiological responses to facemask application in newborns immediately after birth.

Gaertner VD, et al. Arch Dis Child Fetal Neonatal Ed. 2020 Dec 9

Kawasaki Disease Shock Syndrome versus Septic Shock: Early Differentiating Features Despite Overlapping Clinical Profiles.

Power A, et al. J Pediatr. 2020 Dec 5:S0022-3476(20)31482-7.

Invasive Bacterial Infections in Afebrile Infants Diagnosed With Acute Otitis Media.

McLaren SH, et al. Pediatrics. 2020 Dec 7:e20201571.

Invasive Bacterial Infections in Afebrile Infants With Otitis Media: Worry Less but Still Worry.

Ravera J, et al. Pediatrics. 2020 Dec 7:e2020039602.

Machine Learning Supports Automated Digital Image Scoring of Stool Consistency in Diapers.

Ludwig T, et al. J Pediatr Gastroenterol Nutr. 2020 Dec 1.

Use of insulin pump therapy is associated with reduced hospital-days in the long-term: a real-world study of 48,756 pediatric patients with type 1 diabetes.

Auzanneau M, et al. Eur J Pediatr. 2020 Dec 1.

Prescription of acid inhibitors in infants: an addiction hard to break.

Levy EI, et al. Eur J Pediatr. 2020 Dec;179(12):1957-1961.

Use of anti-reflux medications in infants under 1 year of age: a retrospective drug utilization study using national prescription reimbursement data.

O’Reilly D, et al. Eur J Pediatr. 2020 Dec;179(12):1963-1967.

Fool me once… treatment exposure to achieve remission in pediatric IBD.

Van Limbergen JE, et al. Eur J Pediatr. 2020 Dec;179(12):1921-1924.

Infliximab in young paediatric IBD patients: it is all about the dosing.

Jongsma MME, et al. Eur J Pediatr. 2020 Dec;179(12):1935-1944.

 

Diarrheal Deaths After the Introduction of Rotavirus Vaccination in 4 Countries.

Paternina-Caicedo A, et al.Pediatrics. 2020 Dec 30:e20193167.

Emergency Visits and Hospitalizations for Child Abuse During the COVID-19 Pandemic.

Kaiser SV, et al. Pediatrics. 2020 Dec 30:e2020038489.

Association Between Abnormal Fetal Head Growth and Autism Spectrum Disorder.

Regev O, et al. J Am Acad Child Adolesc Psychiatry. 2020 Dec 27:S0890-8567(20)32215-2.

High flow in children with respiratory failure: A randomised controlled pilot trial – A paediatric acute respiratory intervention study.

Franklin D, et al. J Paediatr Child Health. 2020 Dec 30.

Early Skin-to-skin Care with a Polyethylene Bag for Neonatal Hypothermia: A Randomized Clinical Trial.

Travers CP, et al. J Pediatr. 2020 Dec 26:S0022-3476(20)31574-2.

Atypical bartonellosis in children: What do we know?

Lemos AP, et al. J Paediatr Child Health. 2020 Dec 10.

Alternative Cerebral Fuels in the First Five Days in Healthy Term Infants: The Glucose in Well Babies (GLOW) Study.

Harris DL, et al. J Pediatr. 2020 Dec 26:S0022-3476(20)31573-0.

Improving Toddlers’ Healthy Eating Habits and Self-regulation: A Randomized Controlled Trial.

Nix RL, et al. Pediatrics. 2020 Dec 28:e20193326.

Bench-top comparison of thermometers used in newborn infants.

Dunne EA, et al. Arch Dis Child Fetal Neonatal Ed. 2020 Dec 28:fetalneonatal-2020-320123.

Pediatric Emergency Department Visits at US Children’s Hospitals During the COVID-19 Pandemic.

DeLaroche AM, et al. Pediatrics. 2020 Dec 23:e2020039628.

Sustained Lung Inflations During Neonatal Resuscitation at Birth: A Meta-analysis.

Kapadia VS, et al. Pediatrics. 2020 Dec 23:e2020021204.

Association between Baseline Cortisol Serum Concentrations and the Effect of Prophylactic Hydrocortisone in Extremely Preterm Infants.

Renolleau C, et al. J Pediatr. 2020 Dec 23:S0022-3476(20)31567-5.

Delayed Administration of Antibiotics beyond the First Hour of Recognition is Associated with Increased Mortality Rates in Children with Sepsis/Severe Sepsis and Septic Shock.

Sankar J, et al. J Pediatr. 2020 Dec 23:S0022-3476(20)31545-6.

Association of Cesarean Delivery with Childhood Hospitalization for Infections before 13 Years of Age.

Auger N, et al. J Pediatr. 2020 Dec 21:S0022-3476(20)31546-8.

Effect of a Sepsis Screening Algorithm on Care of Children with False-Positive Sepsis Alerts.

Baker AH, et al. J Pediatr. 2020 Dec 21:S0022-3476(20)31543-2.

Rates of Presentation, Treatments and Serious Neurologic Disorders Among Children and Young Adults Presenting to US Emergency Departments With Headache.

Ramgopal S, et al. J Child Neurol. 2020 Dec 24:883073820979137.

COVID-19 in children treated with immunosuppressive medication for kidney diseases.

Marlais M, et al. Arch Dis Child. 2020 Dec 21:archdischild-2020-320616.

Audit on awake anorectal manometry: tolerability in children.

Athanasakos E, et al. Arch Dis Child. 2020 Dec 21:archdischild-2020-321083.

A Graded Approach to Intravenous Dextrose for Neonatal Hypoglycemia Decreases Blood Glucose Variability, Time in the Neonatal Intensive Care Unit, and Cost of Stay.

Sen S, et al. J Pediatr. 2020 Dec 15:S0022-3476(20)31507-9.

Association of Childhood Growth Hormone Treatment With Long-term Cardiovascular Morbidity.

Tidblad A, et al. JAMA Pediatr. 2020 Dec 21:e205199.

Cardiovascular Disease in Former Pediatric Recipients of Growth Hormone: Another Look at Growth Hormone Safety.

Grimberg A. JAMA Pediatr. 2020 Dec 21:e205232.

Deamidated Gliadin Antibodies: Do They Add to Tissue Transglutaminase-IgA Assay in Screening For Celiac Disease?

Abdulrahim A, et al. J Pediatr Gastroenterol Nutr. 2020 Dec 16;Publish Ahead of Print.

Acute Kidney Injury Associated with Late-Onset Neonatal Sepsis: A Matched Cohort Study.

Coggins SA, et al. J Pediatr. 2020 Dec 16:S0022-3476(20)31505-5.

Autism spectrum disorder and kidney disease.

Clothier J, et al. Pediatr Nephrol. 2020 Dec 19.

Bronchopulmonary dysplasia and postnatal growth following extremely preterm birth.

Dassios T, et al. Arch Dis Child Fetal Neonatal Ed. 2020 Dec 17:fetalneonatal-2020-320816.

Long-Term Survival and Causes of Death in Children with Trisomy 21 After Congenital Heart Surgery.

Peterson JK, et al. J Pediatr. 2020 Dec 23:S0022-3476(20)31568-7.

Follow-up Blood Cultures in Children With Staphylococcus aureus Bacteremia.

Cardenas-Comfort C, et al. Pediatrics. 2020 Dec;146(6):e20201821.

Motor Impairment in Children With Congenital Heart Defects: A Systematic Review.

Bolduc ME, et al. Pediatrics. 2020 Dec;146(6):e20200083.

Hospitalised infants due to falls aged less 12 months in New South Wales from 2002 to 2013.

Cooray N, et al. J Paediatr Child Health. 2020 Dec;56(12):1885-1890.

Risk of traumatic intracranial haemorrhage in children with bleeding disorders.

Bressan S, et al. J Paediatr Child Health. 2020 Dec;56(12):1891-1897.

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome – Is It Related to Ethnicity? An Israeli Multicenter Cohort Study.

Amarilyo G, et al. J Pediatr. 2020 Dec;227:268-273.

Association Between Fat Mass in Early Life and Later Fat Mass Trajectories.

de Fluiter KS, et al. AMA Pediatr. 2020 Dec 1;174(12):1141-1148.

Complementary and Alternative Medicine Use in Pediatric Functional Abdominal Pain Disorders at a Large Academic Center.

Ciciora SL, et al. J Pediatr. 2020 Dec;227:53-59.e1.

Effect of Social Distancing Due to the COVID-19 Pandemic on the Incidence of Viral Respiratory Tract Infections in Children in Finland During Early 2020.

Kuitunen I, et al. Pediatr Infect Dis J. 2020 Dec;39(12):e423-e427.

Universal screening of high-risk neonates, parents, and staff at a neonatal intensive care unit during the SARS-CoV-2 pandemic.

Cavicchiolo ME, et al. Eur J Pediatr. 2020 Dec;179(12):1949-1955.

Risk Factors for Orthostatic Hypertension in Children.

Hu Y, et al. J Pediatr. 2020 Dec;227:212-217.e1.

Neurodevelopmental outcomes after moderate to severe neonatal hypoglycemia.

Rasmussen AH, et al. Eur J Pediatr. 2020 Dec;179(12):1981-1991.

The utility of ketones at triage: a prospective cohort study.

Durnin S, et al. Arch Dis Child. 2020 Dec;105(12):1157-1161.

Safety and Immunogenicity of Two RNA-Based Covid-19 Vaccine Candidates.

Walsh EE, et al. N Engl J Med. 2020 Dec 17;383(25):2439-2450.

Does detection of fetal growth restriction improve neonatal outcomes?

Selvaratnam RJ, et al. J Paediatr Child Health. 2020 Dec 14.

Children and young adults with spinal muscular atrophy treated with nusinersen.

Osredkar D, et al. Eur J Paediatr Neurol. 2020 Dec 4;30:1-8.

Effect of Early Targeted Treatment of Ductus Arteriosus with Ibuprofen on Survival Without Cerebral Palsy at 2 years in Infants with Extreme Prematurity: A Randomized Clinical Trial.

Rozé JC, et al. J Pediatr. 2020 Dec 8:S0022-3476(20)31488-8.

Time Course of Coronary Artery Aneurysms in Kawasaki Disease.

Tsuda E, et al. J Pediatr. 2020 Dec 7:S0022-3476(20)31484-0.

4. Case reports

Chewing gum bezoar as an unexpected cause of acute intestinal obstruction.

Sinopidis X, et al. J Paediatr Child Health. 2020 Dec 29.

Unusual presentation of coeliac disease with idiopathic intracranial hypertension.

Pathmanandavel K, et al. J Paediatr Child Health. 2020 Dec 21.

Case 39-2020: A 29-Month-Old Boy with Seizure and Hypocalcemia.

Virkud YV, et al. N Engl J Med. 2020 Dec 17;383(25):2462-2470.

A Gut Feeling: Abdominal Symptoms as an Initial Presentation of EVALI.

Wekon-Kemeni C, et al. Pediatrics. 2020 Dec 30:e20193834.

Sequential Retinal Hemorrhages in an Asymptomatic Child.

Ho DK, et al. J Pediatr. 2020 Dec;227:319-320.

If we have missed out on something useful or you think other articles are absolutely worth sharing, please add them in the comments!

The 45th Bubble Wrap

Cite this article as:
DFTB, T. The 45th Bubble Wrap, Don't Forget the Bubbles, 2021. Available at:
https://dontforgetthebubbles.com/the-45th-bubble-wrap/

With millions upon millions of journal articles being published every year it is impossible to keep up.  Every month we ask some of our friends from PERUKI (Paediatric Emergency Research in UK and Ireland) to point out something that has caught their eye.

Bubble Wrap PLUS – December 2020

Cite this article as:
Anke Raaijmakers. Bubble Wrap PLUS – December 2020, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.31117

Can’t get enough of Bubble Wrap? The Bubble Wrap Plus is a monthly paediatric journal club reading list from Anke Raaijmakers working with Professor Jaan Toelen and his team of the University Hospitals in Leuven. This comprehensive list is developed from 34 journals, including major and subspecialty paediatric journals. We suggest this list can help you discover relevant or interesting articles for your local journal club or simply help you to keep a finger on the pulse of paediatric research.

This month’s list features answers to intriguing questions such as: ‘Is shivering a relevant clinical sign in diagnosing serious bacterial infection?’, ‘Is a bulging fontanelle a good clinical marker for bacterial meningitis?’, ‘What is the value of follow-up blood cultures in children with S aureus bacteraemia?’, ‘What is a recurrence rate of infantile haemangioma after propranolol therapy?’ and ‘What is the prevalence of UTI in infants with URTI?’.

You will find the list is broken down into four sections:

1.Reviews and opinion articles

Understanding primary ciliary dyskinesia and other ciliopathies.

Horani A, et al. J Pediatr. 2020 Nov 23:S0022-3476(20)31452-9.

Opening doors: suggested practice for medical professionals for when a child might be close to telling about abuse.

Marchant R, et al. Arch Dis Child. 2020 Nov 24:archdischild-2020-320093.

‘I just wanted someone to ask me’: when to ask (about child sexual abuse).

Debelle G, et al. Arch Dis Child. 2020 Nov 24:archdischild-2019-317033.

Pediatrician Guidance in Supporting Families of Children Who Are Adopted, Fostered, or in Kinship Care.

Jones VF, et al. Pediatrics. 2020 Nov 23:e2020034629.

When a Family Seeks To Exclude Residents From Their Child’s Care.

Largent EA, et al. Pediatrics. 2020 Nov 5:e2020011007.

Paediatric pancreatic diseases.

Coffey MJ, et al. J Paediatr Child Health. 2020 Nov;56(11):1694-1701.

Coeliac disease in childhood: An overview.

Bishop J, et al. J Paediatr Child Health. 2020 Nov;56(11):1685-1693.

Neonatal liver disease.

Evans HM, et al. J Paediatr Child Health. 2020 Nov;56(11):1760-1768.

Liver disease in the older child.

Ee LC. J Paediatr Child Health. 2020 Nov;56(11):1702-1707.

Diagnosis and management of severe sepsis in the paediatric patient.

Farrell CA. Paediatr Child Health. 2020 Nov 2;25(7):475-476.

Clinical management of sickle cell liver disease in children and young adults.

Kyrana E, et al. Arch Dis Child. 2020 Nov 11:archdischild-2020-319778.

Routine Neuroimaging of the Preterm Brain.

Hand IL, et al. Pediatrics. 2020 Nov;146(5):e2020029082.

Patent Ductus Arteriosus of the Preterm Infant.

Hamrick SEG, et al. Pediatrics. 2020 Nov;146(5):e20201209.

2. Original clinical studies

Shivering has little diagnostic value in diagnosing serious bacterial infection in children: a systematic review and meta-analysis.

Vandenberk M, et al. Eur J Pediatr. 2020 Nov 11.

Adverse Childhood Experiences and School Readiness Among Preschool-Aged Children.

Jackson DB, et al. J Pediatr. 2020 Nov 23:S0022-3476(20)31435-9.

Follow-up Blood Cultures in Children With Staphylococcus aureus Bacteremia.

Cardenas-Comfort C, et al. Pediatrics. 2020 Nov 25:e20201821.

Multisystem Inflammatory Syndrome in Children: An International Survey.

Bautista-Rodriguez C, et al. Pediatrics. 2020 Nov 24:e2020024554.

Implementing a new method of group toilet training in daycare centres: a cluster randomised controlled trial.

Van Aggelpoel T, et al. Eur J Pediatr. 2020 Nov 23.

Clinical Experience with Performing Esophageal Function Testing in Children.

van Lennep M, et al. J Pediatr Gastroenterol Nutr. 2020 Nov 20.

Assessment of 135 794 Pediatric Patients Tested for Severe Acute Respiratory Syndrome Coronavirus 2 Across the United States.

Bailey LC, et al. JAMA Pediatr. 2020 Nov 23.

Randomized Controlled Trial of High-Flow Nasal Cannula in Preterm Infants After Extubation.

Uchiyama A, et al. Pediatrics. 2020 Nov 19:e20201101.

Adenovirus Infection-associated Central Nervous System Disease in Children.

Zhang XF, et al. Pediatr Infect Dis J. 2020 Nov 16.

Cost-effectiveness of Interventions to Increase HPV Vaccine Uptake.

Spencer JC, et al. Pediatrics. 2020 Nov 16:e20200395.

Recurrence rate of infantile hemangioma after oral propranolol therapy.

Frongia G, et al. Eur J Pediatr. 2020 Nov 13.

Somatic symptom and related disorders in a tertiary paediatric hospital: prevalence, reach and complexity.

Wiggins A, et al. Eur J Pediatr. 2020 Nov 13.

Use of a Procalcitonin-guided Antibiotic Treatment Algorithm in the Pediatric Intensive Care Unit.

Katz SE, et al. Pediatr Infect Dis J. 2020 Nov 10.

Abusive Head Trauma in Day Care Centers.

Rey-Salmon C, et al.  Pediatrics. 2020 Nov 10:e2020013771.

Low-dose or no aspirin administration in acute-phase Kawasaki disease: a meta-analysis and systematic review.

Chiang MH, et al. Arch Dis Child. 2020 Nov 10:archdischild-2019-318245.

Bulging fontanelle in febrile infants as a predictor of bacterial meningitis.

Takagi D, et al. Eur J Pediatr. 2020 Nov 9.

Smoking Intention and Progression From E-Cigarette Use to Cigarette Smoking.

Owotomo O, et al. Pediatrics. 2020 Nov 9:e2020002881.

Acute viral bronchiolitis as a cause of pediatric acute respiratory distress syndrome.

Ghazaly MMH, et al. Eur J Pediatr. 2020 Nov 7:1-6.

Predictors of hospital readmission in infants less than 3 months old.

Mace AO, et al. J Paediatr Child Health. 2020 Nov 6.

The Effectiveness of Working Memory Training for Children With Low Working Memory.

Spencer-Smith M, et al. Pediatrics. 2020 Nov 6:e20194028.

Survival and causes of death in extremely preterm infants in the Netherlands.

van Beek PE, et al. Arch Dis Child Fetal Neonatal Ed. 2020 Nov 6:

Longitudinal Association Between Participation in Organized Sport and Psychosocial Development in Early Childhood.

Neville RD, et al. J Pediatr. 2020 Nov 3:S0022-3476(20)31376-7.

In children with a facial port-wine stain, what facial distribution warrants screening for glaucoma?

Mehan A, et al. Arch Dis Child. 2020 Nov 5:archdischild-2020-319931.

New diagnostic approach of the different types of isolated craniosynostosis.

Kronig SAJ, et al. Eur J Pediatr. 2020 Nov 5.

Eating disorders double and acute respiratory infections tumble in hospitalised children during the 2020 COVID shutdown on the Gold Coast.

Jones PD, et al. J Paediatr Child Health. 2020 Nov 5.

Nephrolithiasis during the first 6 months of life in exclusively breastfed infants.

Yılmaz N, et al. Pediatr Nephrol. 2020 Nov 5.

Perceptions of non-successful families attending a weight-management clinic.

Cox JS, et al. Arch Dis Child. 2020 Nov 2:archdischild-2020-319558.

How Are They Doing? Neurodevelopmental Outcomes at School Age of Children Born Following Assisted Reproductive Treatments.

Farhi A, et al. J Child Neurol. 2020 Nov 2:883073820967169.

Breastfeeding and Infections in Early Childhood: A Cohort Study.

Christensen N, et al. Pediatrics. 2020 Nov;146(5):e20191892.

Gut Microenvironment and Bacterial Invasion in Paediatric Inflammatory Bowel Diseases.

Zaidi D, et al. J Pediatr Gastroenterol Nutr. 2020 Nov;71(5):624-632.

The Gut Microbiome and the Triple Environmental Hit Concept of Inflammatory Bowel Disease Pathogenesis.

Kellermayer R, et al. J Pediatr Gastroenterol Nutr. 2020 Nov;71(5):589-595.

Feasibility Study of a New Magnetic Resonance Imaging Mini-capsule Device to Measure Whole Gut Transit Time in Paediatric Constipation.

Sharif H, et al. J Pediatr Gastroenterol Nutr. 2020 Nov;71(5):604-611.

Prevalence of Urinary Tract Infection in Febrile Infants With Upper Respiratory Tract Symptomatology.

Bolivar P, et al. Pediatr Infect Dis J. 2020 Nov;39(11):e380-e382.

Macrolide and Nonmacrolide Resistance with Mass Azithromycin Distribution.

Doan T, et al. N Engl J Med. 2020 Nov 12;383(20):1941-1950.

4. Case reports

Unusual position of the umbilicus in a neonate.

Tsoi SK, et al. Arch Dis Child Fetal Neonatal Ed. 2020 Nov 20:fetalneonatal-2020-321003.

Acholic stools and a small gallbladder: Not always a case of biliary atresia.

Singh H, et al. J Paediatr Child Health. 2020 Nov;56(11):1812-1813.

An unexpected revelation in a child with recurrent severe headaches.

Loke KY, et al. J Paediatr Child Health. 2020 Nov 13.

If we have missed out on something useful or you think other articles are absolutely worth sharing, please add them in the comments!

PEM Adventures Chapter 2

Cite this article as:
Team PEM Adventures. PEM Adventures Chapter 2, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.30926

Are you ready for another PEM adventure? This time the stakes are a little higher. Join us on another journey (with an inbuilt time travel machine) as we manage Grace…

Teenager holding mobile phone

Meet Grace. Grace is a 15-year-old vegetarian environmental activist. She’s thrilled because she’s recently hit a TikTok following of 10,000 – social media is SO the way to spread the word.

She spent yesterday at an illegal climate strike rally outside parliament. Buoyed up on the adrenaline of a thrilling protest, she and some buddies went back to her friend, Zak’s house where they celebrated in style with vodka pops. But this morning, horrified by the fact Grace was impossible to wake, Zak called the emergency services.

Meanwhile, you’ve just fished a pea out of a child’s ear when the red phone rings. Hearing the pre-alert, you mobilise the team and prep a bay in resus. Minutes later, Grace is wheeled in with Zak in tow and she’s transferred to a trolley.

Whiteboard containing vital signs

Your SHO, Lucy, does a primary survey:

  • A: Tolerating an oropharyngeal airway. No stridor or stertor.
  • B: Self-ventilating in 15L O2 via a non-rebreathe mask. Respiratory rate is a bit raised but her chest is clear and she doesn’t have any other signs of respiratory distress.
  • C: Warm and well perfused, heart rate 68 with normal heart sounds and normal pulse volume. Blood pressure is 115/70 and capillary refill time is less than 2 seconds.
  • D: GCS 7, made up of M4, V2, E1. Pupils are size 3 bilaterally and normally reactive to light. Tone is generally low but reflexes are normal and plantars are down going.
  • E: No rashes, no bruises and Grace is currently afebrile.

Lucy gets Grace’s mum’s number from Zak and phones her to get a bit more information. Grace is a healthy adolescent with no significant past medical history. She’s not on any medications, is not allergic to anything and is fully vaccinated. She’s been completely well with no fever, cough, coryza, or any other symptoms. She did have a cold sore a few months ago – could that be relevant?

Grace’s parents, who had gone away for the first time since covid-lockdown lifted, are running to the train station to make their way back home.

Back in resus, you put in a cannula, and send off some bloods: FBC, U&E, LFT, CRP, blood culture and an alcohol level.

Her venous gas shows a pH of 7.47, pCO2 of 2.7, bicarb 14, lactate of 2.7 and normal glucose.

Blood gases showing respiratory alkalosis

That’s odd, you think to yourself, a respiratory alkalosis with some metabolic compensation. You pause for a second and work through your list of possible causes.

  1. Could this be a central cause of hyperventilation? A bleed? A tumour? A meningoencephalitis? You put up a request for CT brain. 
  2. Could this be a respiratory cause? Asthma? Pneumonia? Pneumothorax? Better get a chest x-ray too.
  3. Could this be sepsis? You prescribe ceftriaxone and add acyclovir. There was that coldsore after all…
  4. Pregnancy?
  5. Endocrine or hypermetabolic cause? Maybe DKA? No… her blood sugar’s normal. Or thyrotoxicosis?
  6. Maybe it’s something toxicological? You remember, from your undergraduate days, learning that salicylates cause a respiratory alkalosis.

You add a salicylate level, and paracetamol for good measure, add thyroid function and ask for a catheter urine for beta HCG and a tox screen.  

But her catheter urine doesn’t give you any extra clues. Grace’s urine beta-HCG is negative, her tox screen is negative and her dip is negative.

The resus nurse gently touches your elbow and quietly says, “Do you want to call the anaesthetist?

Good question, you think to yourself. Her GCS is 7 and she’s tolerating the oropharyngeal airway, but she’s breathing well for herself at the moment. What do you want to do?

There are some compelling arguments not to intubate; Grace is maintaining her airway and she’s obtunded and may have seizures – if you give her a paralysing agent as part of her RSI you’ll never be able to tell. Sure, if you really want to monitor for seizure activity, AND you’re in a a tertiary centre with a PICU with capability of CFM or EEG monitoring, you could keep arguing you can monitor for seizure activity while she’s intubated and ventilated, but it takes a while to set up, and time is of the essence.

So you make the brave decision not to intubate. 

You later decide it was less brave and more foolhardy. While Grace is in CT she drops her GCS further and then has a respiratory arrest, which quickly deteriorates into cardiac arrest. The scanner is a terrible place for CPR. While you’re trying to run an arrest on a narrow CT bed you wish you could go back in time and make that choice again. Luckily for you, the inbuilt PEM adventures time travel machine can do just that. In you hop and whizz back to resus.

Close the toggle and this time click on the ‘intubate’ choice.

There are some compelling arguments not to intubate; Grace is maintaining her airway and she’s obtunded and may have seizures – if you give her a paralysing agent as part of her RSI you’ll never be able to tell if she’s seizing. 

But there’s something niggling you… Grace is heading for a CT scan and the LAST thing you need is for her to arrest in the scanner.

And yes, it’s true, there is a risk you could miss a seizure if she was paralysed, but you can give her a long-lasting anticonvulsant to prevent seizures. 

So… you decide to follow your gut and make the decision to intubate.

Thankfully the anaesthetist is nifty with a tube and she’s already drawn up the RSI drugs – fentanyl, ketamine and rocuronium in a 1:1:1 ratio (that’s fentanyl 1mcg/kg, ketamine 1mg/kg and rocuronium 1mg/kg). She’s intubated without difficulty. 

Grace has bilateral equal breath sounds and a mobile chest x-ray shows the tube to be in a good position, with clear lung fields and normal heart size. You mentally cross respiratory causes of an alkalosis off your list.

You’re doing great.

The anaesthetist asks you, “How should I ventilate Grace? Should I match her raised respiratory rate?

That’s a good question, you think to yourself. What should you do?

This is a very good question and you’re not sure you know the answer. Grace is hyperventilating for some reason, and maybe mimicking this is the right thing to do…

But, you’re worried about her ultra low pCO2. At 2.7 it’s likely to be causing cerebral vasoconstriction and hypoperfusion. It’s time to start some simple, proactive neuroprotective measures.

On reflection, you decide it would be better to slow Grace’s breathing so resolutely you turn back to the anaesthetist and ask him to SLOW Grace’s respiratory rate to keep her end tidal CO2 tightly between 4.5 and 5; you want to prevent secondary brain injury.

He nods his assent, while tilting the head of the bed up to 30 degrees.

But, remembering a great DFTB post by Costas Kanaris, you know you can do more than that to neuroprotect. As well as maintaining normocapnia and nursing her at 30 degrees head in line, Grace needs strict normothermia and hypoxia should be avoided at all costs. She needs vigilant glucose monitoring, tight circulatory monitoring and support and an anticonvulsant to prevent seizures. 

Close the toggle and move on to the next part of the story.

You think this through. The alkalotic pH doesn’t matter quite so much, what’s really troubling you is Grace’s pCO2. With a pCO2 of 2.7, there’ll be huge amount of cerebral vasoconstriction and hypoperfusion. It’s time to start some simple, proactive neuroprotective measures.

Resolutely you turn back to the anaesthetist and ask him slow Grace’s respiratory rate to keep her end tidal CO2 tightly between 4.5 and 5; you want to prevent secondary brain injury and so now’s the time to start some neuroprotection.

He nods his assent, while tilting the head of the bed up to 30 degrees.

But, remembering a great DFTB post by Costas Kanaris, you know you can do more than that to neuroprotect. As well as maintaining normocapnia and nursing her at 30 degrees head in line, Grace needs strict normothermia and hypoxia should be avoided at all costs. She needs vigilant glucose monitoring, tight circulatory monitoring and support and an anticonvulsant to prevent seizures. 

Great choice! Close the toggle and move on to the next part of the story.

With fortuitous timing, CT ring down to say they’re ready for Grace.

Satisfied that A, B and C are all stable, you turn to take the brake off the trolley when Lucy, your SHO, asks, “But do we only want a plain non-contrast CT?

That’s a good question, you think to yourself. Is that all I want? What neuroimaging will you choose?

“Yes”, you say to Lucy. “A non-con CT is quick and will show us most tumours and bleeds. She can have an MRI later to get a bit more detail.” 

But,” your SHO counters, “a non-con CT won’t always detect an ischaemic stroke. Perhaps we should ask for a CTA too?

You remember a case from a few weeks ago, a little boy called Tomas. You’d bookmarked the RCPCH Stroke in Childhood guideline on your phone. You quickly bring it up and Lucy’s right, the guideline says to consider stroke in children with focal neurology, speech disturbance, focal seizures, severe headache, cerebellar signs… and unexplained decreased conscious level.

Smiling gratefully at Lucy you pick up the phone and ask the radiologist if you can add a CTA. They say yes.

Minutes later, Grace has her CT with CTA… but it’s normal. No abscess… no tumour… no bleed… and no stroke.

Well that’s good news for Grace, you think to yourself, but it doesn’t give you any much-needed clues.

Great work. Close the toggle and move onto the next part of the story.

You know what”, you say to your SHO, “let’s ask for a contrast-enhanced CT. It’s still quick and will give us a little more detail than a non-con CT.

But,” she counters, “do you think we should be considering stroke in our differential? Perhaps we should ask for a CTA too?

You remember a case from a few weeks ago, a little boy called Tomas. You’d bookmarked the RCPCH Stroke in Childhood guideline on your phone. You quickly bring it up and Lucy’s right, the guideline says to consider stroke in children with focal neurology, speech disturbance, focal seizures, severe headache, cerebellar signs… and unexplained decreased conscious level.

Smiling gratefully at Lucy you pick up the phone and ask the radiologist if you can add a CTA. They say yes.

Minutes later, Grace has her CT with CTA… but it’s normal. No abscess… no tumour… no bleed… and no stroke.

Well that’s good news for Grace, you think to yourself, but it doesn’t give you any much-needed clues.

Great work. Close the toggle and move onto the next part of the story.

You know what”, you say to your SHO, “let’s ask for a CT plus CTA. The CT will show us most tumours and bleeds and she can have an MRI later for a bit more detail, but we should consider stroke in our differential, and to detect that we need to add angiography to our CT.

You think back to a case from a few weeks ago, a little boy called Tomas. You’d read the RCPCH Stroke in Childhood guideline and remember that it says to consider stroke in children with focal neurology, speech disturbance, focal seizures, severe headache, cerebellar signs… and unexplained decreased conscious level.

Smiling gratefully at Lucy you pick up the phone and ask the radiologist if you can add a CTA. They say yes.

Minutes later, Grace has her CT with CTA… but it’s normal. No abscess… no tumour… no bleed… and no stroke.

Well that’s good news for Grace, you think to yourself, but it doesn’t give you any much-needed clues.

Great work. Close the toggle and move onto the next part of the story.

You haven’t ruled out infection. So, when you’re back down in resus, you ask Lucy if she’d like to do the LP.

Really? Is that safe with her low GCS?” she questions. 

What do you think? Should you LP?

It’s fine,” you reply, “she doesn’t have physiological signs of raised ICP: she’s not bradycardic or hypertensive, she’s not posturing and she didn’t have focal neurology. Plus, her CT doesn’t look like there’s cerebral oedema.

Feeling reassured, Lucy picks up the spinal needle and performs an LP. 

But it’s not your finest decision. Grace cones and arrests. 

Luckily for you and Grace, there’s an inbuilt time travel function in your PEM adventure and you return back to resus just as your SHO asks if it’s safe to LP Grace.

You have a strange feeling of déjà vu, while a little voice tells you that although a normal CT is usually reliable for ruling out raised intracranial pressure, this isn’t failsafe and it might be safer to defer the LP for when she’s a little more stable. You’ve already started the ceftriaxone and acyclovir, so this time you decide that the LP can wait until she’s a bit more stable and can have an MRI first. 

Thank goodness for that time machine! Close this toggle and move onto the next part of the story.

Lucy’s right. Although a normal CT is usually reliable for ruling out raised ICP, this isn’t failsafe and there’s no rush to get CSF now. You’ve already started ceftriaxone and acyclovir anyway. And when she’s a bit more stable she can have an MRI to check the LP’s safe. The LP can wait for now.

Great teamwork! Close the toggle and continue the next part of the story.

You’re still not sure what’s causing Grace’s low GCS though. Maybe the bloods will help. So you log in to the computer to check Grace’s results.

Results showing a mild transaminitis

Huh, you think to yourself. Grace’s FBC and CRP are normal; it’s sounding less and less like infection.

Her urea is low and her liver enzymes are raised, with a slightly prolonged INR.

Her salicylate and alcohol levels are undetectable. This isn’t feeling so toxicological anymore.

You mull this over with Lucy. Maybe this is a viral picture. There was that cold sore…

Just then Maureen, the ED cleaner, pops her head into the office. “Might this be of any use?” she asks. She’s holding the RCPCH Decreased Conscious level guideline.

You quickly flick through. Bloods… imaging… you’ve done pretty much everything it suggests. But then you take a closer look at the list of bloods it suggests. And there, in black and white, it says ammonia.

Of course!” you say out loud. “That would explain the respiratory alkalosis!

You draw off an ammonia sample, get it on ice and ask Raymond, the dashing porter, to run it down to the lab. You give the lab a ring so they can get the machine primed.

While you’re waiting for the result to come back, Zak comes running over. He’s just been looking in Grace’s backpack for her mobile and found a high protein Diet book. Apparently she’s been trying to lose weight for TikTok. Could it be relevant?

The cogs begin to whir… Hang on a minute… A high protein diet in a vegetarian environmental activist?

The lab phones with Grace’s ammonia level.

It’s over 500! And normal is less than 40.

It all falls into place. Selective vegetarian… Recent protein load… Raised transaminases… High ammonia… This is all beginning to sound a bit metabolic.

But what should you do about that ammonia? As far as you can see, the DeCon guidance only tells you to take it, not what you do when it comes back at over 10 times the upper limit of normal.

Just a sec,” says Lucy scrolling through her mobile phone, “The British Inherited Metabolic Disease Group have got this covered. They’ve produced a whole range of easy access emergency guidelines, including this one, for the management of an undiagnosed hyperammonaemia.”

It says, turn off protein catabolism by giving a 10% dextrose bolus followed by a dextrose infusion to provide an alternative energy source. If her glucose climbs, add insulin but don’t reduce the dextrose – otherwise, she’ll just start breaking down more protein. And, finally, mop up that ammonia with scavengers like phenylbutyrate and sodium benzoate.

The words ‘ammonia scavengers’ remind you of another post you read on Don’t Forget The Bubbles, about the different types of metabolic conditions, how they present and the various treatment strategies. You make a mental note to read it again later to remind yourself of the differences between an amino acid and organic acid.

Meanwhile, you hastily prescribe…

  •       A 2ml/kg bolus of 10% dextrose
  •       a dextrose infusion
  •       And those ammonia scavengers, sodium benzoate and sodium phenylacetate

Grace is subsequently diagnosed as having a urea cycle disorder. You’re amazed to discover that although most diagnoses are made in neonates, diagnoses are sometimes made in adolescents and adults presenting encephalopathic after a big protein load or when catabolic, such as after trauma, childbirth, major surgery, major haemorrhage, critical illness, rapid weight loss or simply after switching to a high protein diet. This is particularly true for ornithine transcarbamylase (OTC) deficiency, which although is X-linked, can present in symptomatic female OTC carriers. Little diagnostic clues include autoselective vegetarianism (that protein makes them feel a bit ‘ugh’) and subtle or behavioural difficulties from chronic low-level hyperammonaemia.

You bookmark a fantastic review article to read later and flick back through your undergraduate biochemistry textbook to remind yourself about urea cycle defects… and hastily close it again when you remember how little you knew even then, at the prime of your undergrad years.

Wow, what a shift. You pack up your stethoscope and head home, reflecting on your day as you walk to the bus stop.

Grace has taught you the importance of…

Reaching for the RCPCH DeCon guideline when looking after a child with an unexplained low GCS.

Not ever forgetting to send an ammonia in an encephalopathic child, young person or even adult; these tricksy urea cycle disorders can present in adulthood. If the ammonia comes back high, BIMDG have a handy guideline telling you exactly what to do.

And, remembering that a normal CT does not ALWAYS rule out raised ICP. In a child with low GCS, put away that LP needle and neuroprotect instead.

But what happened to Grace? Let’s jump in the time travel machine and find out…

Your epic diagnosis of a late presenting metabolic disorder was the talk of the ED. The RCPCH DeCon poster was put up in the ED staff room and from that point onwards, everyone remembered to check an ammonia in a patient presenting with an unexplained low GCS. 

Lucy was nominated as employee of the month. This shift was a pivotal moment in her career as she decided PEM was her vocation.

The ammonia scavengers did the trick and Grace made a full recovery.

Grace focussed her efforts on reducing plastic waste in hospital and successfully petitioned for the introduction of plastic-free PPE, reducing plastic waste during the COVID-19 pandemic by an incredible 275%.

She hit 3 million TikTok followers (and you’re one of them).

This PEM adventure wouldn’t have been possible without some help from some amazing people. Thank you to Roshni Vara, Consultant in Paediatric Inherited Metabolic Disease at the Evelina London, Costas Kanaris, PICU and retrieval consultant at the Royal Manchester Children’s Hospital and Jon Lillie, PICU and retrieval consultant at the Evelina London Children’s Hospital.

Here are some of their wise words of advice…

As Costas says in The N of 1 matters, we’ve outlined our take on Grace’s case and how we’d manage her in our own resus bays. Medicine’s not always so clear cut and there are often different approaches to the same problem, but this is our consensus on minimising risk using, as Costas says, a rational, evidence-based and pharmacologically prudent approach (I love that phrase Costas!)

Should we intubate Grace?

Grace is self-ventilating but the fact that she is tolerating an oropharyngeal airway means some of her airway reflexes have gone. Scanning a child with a GCS of 8 or less, without securing the airway, puts them at risk. If they vomit, they aspirate. If they stop breathing and arrest in the scanner, the CT room is one of the least fun places to run an arrest, perhaps second only to an elevator. Are there any counter-arguments? Yes, and they’re soft.  One is “this patient is encephalopathic/obtunded and may have seizures; if the child starts fitting we won’t be able to tell as they’ll be paralysed”.  Costas says he usually stands his ground and says that if someone is worried about seizures then the child can be given a long-acting antiepileptic. Levetiracetam is his preference, although phenytoin would work just as well unless there’s suspicion of an overdose of an arrhythmogenic agent. The last thing you need is to tip this child into an arrhythmia.

When should a lumbar puncture be performed in a child with a decreased conscious level?

CT is a useful tool for ruling out raised intracranial pressure before proceeding to lumbar puncture. And we’d agree. But Grace has a low GCS and this changes the picture.

If we take a look at the full RCPCH DeCon guideline it dedicates a whole section to answering the question about LP in decreased conscious level. So, let’s start there.

The DeCon guideline advises a lumbar puncture if your differentials are viral encephalitis or tuberculous meningitis and advises that we consider lumbar puncture when our differentials are bacterial meningitis, sepsis, or the cause of the low GCS is not known. This is cloaked with the phrase “when no acute contraindications exist” and this is key. So what are those contraindications?

  • Signs of raised intracranial pressure: dilated pupil(s), abnormal pupil reaction to light, bradycardia, hypertension or abnormal breathing pattern.
  • A GCS equal to or less than 8, or a deteriorating GCS
  • Focal neurology
  • A seizure lasting more than 10 minutes with a GCS less than 13
  • Shock or clinical evidence of meningococcal disease
  • CT or MRI suggesting obstruction of the CSF pathways by blood, pus, tumour or coning.

What’s the evidence? Well, it’s mostly been derived by expert opinion, and there aren’t many people who’d dispute them.

But what about when you have a normal CT? The radiologists can look for midline shift and for signs of impending herniation by assessing the position of the cerebellar tonsils. So, surely that can rule out raised ICP, allowing an LP to be done?

The DeCon guideline quotes a study published in 2000 that showed that in 124 CT scans from 65 children with traumatic brain injury, CT had an excellent sensitivity of detecting raised ICP of 99.1%, with a specificity of 78.1%. But, a 2019 revision to the guideline says that no further evidence about the sensitivity or specificity of CT in detecting raised ICP in children has been found. None. Although the sensitivity in the one quoted study was very high, it was felt that one study, in children with traumatic brain injury, could not be extrapolated to all children with a decreased conscious level. And so the guideline states that a normal CT scan does not exclude raised ICP. If other contraindications are present, don’t use a normal CT to justify LP.

What does this mean in practice? Well, in a child with a GCS of 8 or less, like Grace, there’s no rush to do an LP. It’s unlikely to change your management acutely in the ED. Her infection can be treated empirically and once she’s more stable, and you have more information including, potentially, an MRI, she can then have an LP for PCR.

What neuroimaging should we do?

That’s a good question, answered beautifully by an article by Hayes et al, published in 2018. Although this article focuses on neuroimaging for headaches, it has a great section on when you might choose each type of scan.

We’d all agree that the ideal imaging to look for a brain tumour is an MRI. It gives excellent detail about the brain tissue as well as other intracranial soft tissues and the extra-axial CSF spaces.

But, if you want a quick answer, or your access to MR is difficult, a non-contrast CT can be performed easily from the ED. If there’s no possibility of a later MR, then contrast-enhanced CT might be better as it gives more detail, but it’s more radiation – this is one for discussion with the radiologist.

CT is very sensitive in detecting blood, and it can be done quickly, in an emergent setting from the ED. So, in children with thunderclap headache, when you want to exclude subarachnoid hemorrhage, a non-contrast CT will be your first choice scan. If blood is detected, then add in arterial imaging: CT or MR angiography (CTA or MRA). Contrast is injected and images taken in the arterial phase.

CTA or MRA are also useful in the investigation of suspected stroke. In practice, you need an answer fast, particularly if the child’s within the thrombolysis window and could be a candidate if there’s evidence of ischaemic stroke, so a CTA is a more practical scan. The CT component looks for blood or large areas of parenchymal infarct, while the angiography looks for filling defects in the arteries that could indicate a thrombus.

If you’re looking for intracranial extension of infection, such as from an orbital cellulitis, mastoiditis or a brain abscess, then a contrast-enhanced CT will highlight suppurative collections.

And if you suspect a venous sinus thrombosis, such as in children with coagulopathies, sickle cell disease, infective spread from meningitis / mastoiditis / sinusitis, or secondary to dehydration or renal failure? Then you need to look at the venous spaces. CT or MR venography (CTV or MRV), when contrast is injected and images obtained in the venous phase, will give you the answers you need.

And what ARE the causes of a respiratory alkalosis?

There are a few! Here are the main ones:

  • Central: brain tumours, meningoencephalitis; stroke
  • Respiratory: asthma, pneumonia, pneumothorax, PE
  • Sepsis
  • Pregnancy
  • Endocrine and hypermetabolic cause: DKA, thyrotoxicosis
  • Toxicology: salicylates 
  • Hyperammonemia: liver and metabolic disorders 
infographic of causes of respiratory alklosis

We would LOVE your feedback about these DFTB PEM adventures so if you can spare a minute, please complete our survey at www.tiny.cc/DFTBpemadventure or use your smartphone to let the QR code take you straight there. We timed ourselves completing it and it takes less than a minute. Thank you.

Select references

The management of children and young people with an acute decrease in conscious level. A nationally developed evidence-based guideline for practitioners. RCPCH. 2015 update, with 2019 revisions. Management of children and young people with an acute decrease in conscious level – Clinical guideline | RCPCH

Undiagnosed Hyperammonaemia. Diagnosis and Immediate Management. British Inherited Metabolic Disease Group. Last reviewed 2017. The major causes are as follows (bimdg.org.uk)

Hirsch, W., Beck, R., Behrmann, C. et al. Reliability of cranial CT versus intracerebral pressure measurement for the evaluation of generalised cerebral oedema in children. Pediatric Radiology 30, 439–443 (2000). https://doi.org/10.1007/s002470000255

Expert Panel on Pediatric Imaging:, Hayes LL, Palasis S, Bartel TB, Booth TN, Iyer RS, Jones JY, Kadom N, Milla SS, Myseros JS, Pakalnis A, Partap S, Robertson RL, Ryan ME, Saigal G, Soares BP, Tekes A, Karmazyn BK. ACR Appropriateness Criteria® Headache-Child. J Am Coll Radiol. 2018 May;15(5S):S78-S90. doi: 10.1016/j.jacr.2018.03.017. PMID: 29724429.

Mitani H, Mochizuki T, Otani N, Tanaka H, Ishimatsu S. Ornithine transcarbamylase deficiency that developed at the age of 19 years with acute brain edema. Acute Med Surg. 2016;3(4):419-423. doi:10.1002/ams2.214

Summar ML, Barr F, Dawling S, Smith W, Lee B, Singh RH, Rhead WJ, Sniderman King L, Christman BW. Unmasked adult-onset urea cycle disorders in the critical care setting. Crit Care Clin. 2005 Oct;21(4 Suppl):S1-8. doi: 10.1016/j.ccc.2005.05.002. PMID: 16227111.

Kanaris C, Ghosh A, Partington CG389(P) A case for early ammonia testing in all encephalopathic patients: female patients with x-linked ornithine transcarbamylase deficiency. Archives of Disease in Childhood 2015;100:A158-A159. http://dx.doi.org/10.1136/archdischild-2015-308599.343

Summar, Marshall. (2005). Presentation and management of urea cycle disorders outside the newborn period. Critical Care Clinics. 21. IX-IX. 10.1016/j.jccc.2005.08.004.

Bubble Wrap PLUS – October/November 2020

Cite this article as:
Grace Leo. Bubble Wrap PLUS – October/November 2020, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.30866

Can’t get enough of Bubble Wrap? The Bubble Wrap Plus is a monthly paediatric journal club reading list from Anke Raaijmakers working with Professor Jaan Toelen & his team of the University Hospitals in Leuven. This comprehensive list is developed from 34 journals, including major and subspecialty paediatric journals. We suggest this list can help you discover relevant or interesting articles for your local journal club or simply help you to keep a finger on the pulse of paediatric research.

This month’s list features answers to intriguing questions such as: ‘How do children respond to masked paediatricians?’, ‘Are the youngest children in a classroom at increased risk of being medicated for ADHD?’, ‘Does neonatal paracetamol treatment lead to problems later in life?’, ‘Are probiotics useful for treating IBS?’ and ‘Is digital media exposure early in life associated with autism?’.

You will find the list is broken down into four sections:

1.Reviews and opinion articles

Multisystem Inflammatory Syndrome in Children and SARS-CoV-2 Serology.

Zeichner SL, et al. Pediatrics. 2020 Sep 24:e2020032888.

Update on congenital cytomegalovirus infection: Prenatal prevention, newborn diagnosis, and management.

Barton M, et al. Paediatr Child Health. 2020 Sep 16;25(6):395-396.

Do probiotics in pregnancy reduce the risk of group B streptococcal colonisation?

Jois RS, et al. J Paediatr Child Health. 2020 Sep;56(9):1468-1472.

Trajectories of Lung Function in Infants and Children: Setting a Course for Lifelong Lung Health.

Jordan BK, et al. Pediatrics. 2020 Sep 16:e20200417.

Principles of immunisation in children with solid organ transplant.

Kelgeri C, et al. Arch Dis Child. 2020 Sep 16:archdischild-2020-319822

What time interval is needed between the administration of live attenuated vaccines?

Zimmermann P, et al. Arch Dis Child. 2020 Sep 8:archdischild-2020-320091.

For Victims of Fatal Child Abuse, Who Has the Right to Consent to Organ Donation?

Deutsch SA, et al. Pediatrics. 2020 Sep;146(3):e20200662.

Advocating for Children During the COVID-19 School Closures.

Masonbrink AR, et al. Pediatrics. 2020 Sep;146(3):e20201440.

2. Original clinical studies

Coronavirus infection in neonates: a systematic review.

Trevisanuto D, et al. Arch Dis Child Fetal Neonatal Ed. 2020 Sep 17:fetalneonatal-2020-319837.

Susceptibility to SARS-CoV-2 Infection Among Children and Adolescents Compared With Adults: A Systematic Review and Meta-analysis.

Viner RM, et al. JAMA Pediatr. 2020 Sep 25.

Frequency of Children vs Adults Carrying Severe Acute Respiratory Syndrome Coronavirus 2 Asymptomatically.

Milani GP, et al. JAMA Pediatr. 2020 Sep 14.

Masked paediatricians during the COVID-19 pandemic and communication with children.

Shack AR, et al. J Paediatr Child Health. 2020 Sep;56(9):1475-1476.

Possible Coronavirus Disease 2019 Pandemic and Pregnancy: Vertical Transmission Is Not Excluded.

Marzollo R, et al. Pediatr Infect Dis J. 2020 Sep;39(9):e261-e262.

Probable Vertical Transmission of SARS-CoV-2 Infection.

Demirjian A, et al. Pediatr Infect Dis J. 2020 Sep;39(9):e257-e260.

Prepubertal gynecomastia is not always idiopathic: case series and review of the literature.

Laimon W, et al. Eur J Pediatr. 2020 Sep 25.

Reducing Antibiotic Prescribing in Primary Care for Respiratory Illness.

Kronman MP, et al. Pediatrics. 2020 Sep;146(3):e20200038

Surfactant use in late preterm infants: a survey among Belgian neonatologists.

Cornette L, et al. Eur J Pediatr. 2020 Sep 24.

Magnet Ingestions in Children Presenting to Emergency Departments in the United States 2009-2019: A Problem in Flux.

Reeves PT, et al. J Pediatr Gastroenterol Nutr. 2020 Sep 22.

E-cigarette or Vaping-Associated Acute Lung Injury and Hemophagocytic Lymphohistiocytosis.

Derespina KR, et al. Pediatrics. 2020 Sep 23:e20193664.

Resuscitation of non-vigorous neonates born through meconium-stained amniotic fluid: post policy change impact analysis.

Oommen VI, et al..Arch Dis Child Fetal Neonatal Ed. 2020 Sep 22:fetalneonatal-2020-319771.

Value of serial echocardiography in diagnosing Kawasaki’s disease.

Hörl M, et al. Eur J Pediatr. 2020 Sep 21.

Children’s Relative Age and ADHD Medication Use: A Finnish Population-Based Study.

Vuori M, et al. Pediatrics. 2020 Sep 21:e20194046.

Brain Morphology Associated With Obsessive-Compulsive Symptoms In 2,551 Children From the General Population.

Weeland CJ, et al..J Am Acad Child Adolesc Psychiatry. 2020 Sep 16:S0890-8567(20)31837-2.

The biological and social determinants of childhood obesity: comparison of two cohorts 50 years apart.

Robinson N, et al. J Pediatr. 2020 Sep 16:S0022-3476(20)31164-1.

Lung ultrasound features predict admission to the neonatal intensive care unit in infants with transient neonatal tachypnoea or respiratory distress syndrome born by caesarean section.

Poerio A, et al. Eur J Pediatr. 2020 Sep 19.

Clinical and Epidemiologic Characteristics of Pertussis in Hospitalized Children: A Prospective and Standardized Long-term Surveillance Study.

Zumstein J, et al. Pediatr Infect Dis J. 2020 Sep 16.

Long-term Cognitive, Psychological, and Health Outcomes Associated With Child Abuse and Neglect.

Strathearn L, et al. Pediatrics. 2020 Sep 17:e20200438

Childhood Adversity and Health After Physical Abuse.

Campbell KA, et al. Pediatrics. 2020 Sep 16:e20200638.

Intravenous paracetamol for neonates: long-term diseases not escalated during 5 years of follow-up.

Juujärvi S, et al. Arch Dis Child Fetal Neonatal Ed. 2020 Sep 17:fetalneonatal-2020-319069.

Use of probiotics in the treatment of functional abdominal pain in children-systematic review and meta-analysis.

Trivić I, et al. Eur J Pediatr. 2020 Sep 17.

Preliminary study on the feasibility of a two-stage screening strategy for otitis media with effusion in children.

Yang J, et al. J Paediatr Child Health. 2020 Sep 17.

Longitudinal Changes in Early Nasal Microbiota and the Risk of Childhood Asthma.

Toivonen L, et al. Pediatrics. 2020 Sep 15:e20200421

Where are they now? An autism follow-up study.

McDonald J, et al. J Paediatr Child Health. 2020 Sep 15.

Use of procalcitonin and C-reactive protein in the diagnosis of bacterial infection in infants with severe bronchiolitis.

Alejandre C, et al. Eur J Pediatr. 2020 Sep 14.

Association Between Congenital Cytomegalovirus and the Prevalence at Birth of Microcephaly in the United States.

Messinger CJ, et al. JAMA Pediatr. 2020 Sep 14.

Human Herpes Virus 6 Detection in Children With Suspected Central Nervous System Infection.

Cosme I, et al. Pediatr Infect Dis J. 2020 Sep 7

Urine collection methods and dipstick testing in non-toilet-trained children.

Diviney J, et al. Pediatr Nephrol. 2020 Sep 12

Reassessing the Use of Proton Pump Inhibitors and Histamine-2 Antagonists in Critically Ill Children: A Systematic Review and Meta-Analysis.

Jie Yao DW, et al. J Pediatr. 2020 Sep 8:S0022-3476(20)31139-2

Early Neurodevelopmental Trajectories for Autism Spectrum Disorder in Children Born Very Preterm.

Chen LW, et al. Pediatrics. 2020 Sep 8:e20200297

Childhood Obesity is Associated with Poor Academic Skills and Coping Mechanisms.

Gill N, et al. J Pediatr. 2020 Sep 4:S0022-3476(20)31129-X.

Sounding the Alarm on Sleep: A Negative Association Between Inadequate Sleep and Flourishing.

Tsao HS, et al. J Pediatr. 2020 Sep 2:S0022-3476(20)31116-1.

Intravenous paracetamol in comparison with ibuprofen for the treatment of patent ductus arteriosus in preterm infants: a randomized controlled trial.

Dani C, et al. Eur J Pediatr. 2020 Sep 4.

Multidisciplinary approach to paediatric aerodigestive disorders: A single-centre longitudinal observational study.

Fuladi A, et al. J Paediatr Child Health. 2020 Sep 4

Did children ‘stay safe’? Evaluation of burns presentations to a children’s emergency department during the period of COVID-19 school closures.

Mann JA, et al. Arch Dis Child. 2020 Sep 3:archdischild-2020-320015.

Increased IgE-Mediated Food Allergy With Food Protein-Induced Allergic Proctocolitis.

Martin VM, et al. Pediatrics. 2020 Sep;146(3):e20200202

Machine Learning To Predict Serious Bacterial Infections in Young Febrile Infants.

Ramgopal S, et al. Pediatrics. 2020 Sep;146(3):e20194096.

Machine Learning and Clinical Prediction Rules: A Perfect Match?

Chamberlain JM, et al. Pediatrics. 2020 Sep;146(3):e2020012203.

Parent Involvement in Adolescent Obesity Treatment: A Systematic Review.

Bean MK, et al. Pediatrics. 2020 Sep;146(3):e20193315

Social/digital media exposure early in life associated with autistic symptoms.

Chonchaiya W. J Pediatr. 2020 Sep;224:179-183

What is the effectiveness and safety of different interventions in the management of drooling in children with cerebral palsy?

Khajuria S, et al. Arch Dis Child. 2020 Sep;105(9):906-910.

Varicella Zoster Reactivation Causing Aseptic Meningitis in Healthy Adolescents: A Case Series And Review Of The Literature.

Barry R, et al. Pediatr Infect Dis J. 2020 Sep;39(9):e278-e282

How Much Is Too Much? Examining the Relationship Between Digital Screen Engagement and Psychosocial Functioning in a Confirmatory Cohort Study.

Przybylski AK, et al. J Am Acad Child Adolesc Psychiatry. 2020 Sep;59(9):1080-1088.

4. Case reports

Penny Ingestion: Can We Really Manage Expectantly?

Krasaelap A, et al. J Pediatr Gastroenterol Nutr. 2020 Sep;71(3):e101-e102

Do not trust your gut: A rare cause of oedema in a 3-year-old.

Cilento SV, et al. J Paediatr Child Health. 2020 Sep 11

Rectal prolapse but it is not just a rectal prolapse.

Al Mughaizwi T, et al. J Paediatr Child Health. 2020 Sep 5.

Spontaneous Pneumomediastinum: Unusual Cause of Sore Throat.

Kumai T, et al. J Pediatr. 2020 Sep;224:176-177.

A Persistent “Pimple” in a 5-Year-Old Girl.

Bell KA, et al. J Pediatr. 2020 Sep;224:172-173.

If we have missed out on something useful or you think other articles are absolutely worth sharing, please add them in the comments!

Gastroenteritis

Cite this article as:
Angharad Griffiths. Gastroenteritis, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.28790

Conor is a 10 kg, 13 month-old boy who’s presented to the ED with a 24-hour history of diarrhoea and vomiting.  He has had 5 episodes of non-bloody, non-bilious vomits. Since waking up this morning has two episodes of loose/watery non-bloody malodorous stools. They have not ‘flooded’ the nappy but were quite large.  He is taking sips of fluid (mixtures of water, milk, and juice being offered) and has only eaten half a digestive biscuit so far today.  He has had a fairly large wet nappy last night, but not since, though it’s now difficult to tell as his last nappy was dirty.  He is alert and looking around while being carried but is upset on leaving his mother’s arms.  He cries with tears, has a normal heart rate but his mother is worried about his dry lips.  She was told by a healthcare worker neighbour that he will “need a drip”. CRT, HR, and BP are normal.  His temperature is 37.8.  His nappy is dry and has been on for 3 hours now.  His capillary glucose measurement is 3.2.  You decide he’s probably mildly dehydrated.

Introduction

Gastroenteritis (GE) is the presence or diarrhoea or vomiting (or both) that may or may not be accompanied by fever, abdominal pain and anorexia.  Diarrhoea is the passage of excessively liquidy or frequent stools with liquid high water content.  Although often felt to be a common minor illness presentation, it is a major cause of childhood mortality and morbidity, causing millions of deaths worldwide in children in low and middle-income countries; of all child deaths from gastroenteritis 78% occur in Africa and South-East Asia. 

Gastroenteritis accounts for a huge proportion of GP and ED presentations. In Europe, acute gastroenteritis the third commonest cause of hospital admission, accounting for between 4-17% of admissions.  In Australia, gastroenteritis caused by rotavirus alone accounts for 115,000 GP visits, 22,000 ED visits and 10,000 hospital admissions a year, with an estimated cost of 30m Australian Dollars (£12m, €18m).  In the UK, 20% of GP consultations in the under 5’s are for GE.

It is imperative that the child with gastroenteritis is differentiated from more sinister causes of vomiting.  The presence of diarrhoea is reassuring but doesn’t exclude other intra-abdominal causes.  The same can be said for pain out of proportion with gastroenteritis, distension, peritoneal signs or localised tenderness.

Most cases are not associated with complications but when complications do occur, the commonest are electrolyte disturbance and metabolic acidosis.  Supplementary fluids through oral or intravenous routes are the most effective way to avoid these complications.

Gastroenteritis in low and middle-income countries can present differently, has different aetiologies, is often managed differently, and is a larger burden to healthcare systems in general than in high-income countries.  This post will focus on gastroenteritis in high-income countries. For more information about comparisons of guidelines across the world; Vecchio et al (2016) is an interesting read.

This is not meant to provide a clinical practice guideline; rather an overview of the illness.  Many (if not all!) paediatric emergency departments or general paediatric units have their own guidelines.

Pathophysiology

Worldwide, the commonest causes are viral pathogens, most commonly rotaviruses and noroviruses.  Viral infections cause damage to the small bowel enterocytes with resultant low-grade fevers and watery diarrhoea – classically without blood.  Rotavirus strains are seasonal and vary within different geographical areas.  The peak age for these infections is between 6 months and 2 years.  Children with poor nutrition are at higher risk of acquiring gastroenteritis and developing dehydration and complications.

Children with bacterial gastroenteritis are more likely to have bloody stool.
Escherichia coli and Shigella dysenteriae can be complicated by haemolytic uraemic syndrome (HUS).  This is an acute onset, microangiopathic haemolytic anaemia, thrombocytopaenia, acute renal impairment and multisystem involvement.  (Just to confuse things, HUS can present in the absence of bloody diarrhoea.)

Pathogens can be generalised into four groups:

  • Viral (70% of cases): Rotavirus, Norovirus, Adenovirus, Enterovirus
  • Bacterial (10-20% of cases): Campylobacter jejuni, Salmonella spp, Escherichia coli, Shigella spp, Yersinia enterocolitica.
  • Protozoa (unusual, accounting for <10%): Cryptosporidium, Giardia lamblia, Entamoeba histolytica
  • Helminths (very unusual): Strongyloides stercoralis

Transmission

Pathogens are spread mainly via the faeco-oral route, acquired by ingesting contaminated food or drink.  Water may be contaminated with bacteria, viruses, or protozoa. Undercooked (or inappropriately stored/cooked) meats and seafood are common culprits of bacterial pathogens.  Bacterial contaminants can produce toxins (e.g. Bacillus cereus in re-warmed rice or Staphylococcus aureus in ice-cream).

Pathogens causing gastroenteritis can also be transmitted without the patient being symptomatic.

Assessment

Gastroenteritis is a clinical diagnosis.  Enquire about sick/infectious contacts and potential sources (recent travel, food).  Enquire about the frequency of symptoms and intake of fluids.  Note the frequency of urination.  Note other things that may cause diarrhoea e.g. recent use of enteral antibiotics or chronic constipation with overflow diarrhoea the presenting feature. 

In the presence of signs such as high fever, long duration of symptoms, severe abdominal pain or bilious vomiting; review the diagnosis and do not immediately label as gastroenteritis.

Oral hydration fluids

Most children are not dehydrated and can tolerate oral fluids and so can be managed at home.  Take a look at Nikki Abela’s DFTB19 talk on top tips for a high yield dehydration assessment.

When children are only mildly to moderately dehydrated, as a general rule they can be treated with oral / enteral rehydration with low osmolality oral rehydration solution (ORS).  Worldwide, ORS is recognised as first line therapy and treating mild to moderate dehydration with enteral rehydration is supported by the WHO, European Society for Paediatric Gastroenterology and the American Academy of Paediatrics. The WHO recommends a low osmolality (hypo-osmolar) solution, usually containing sodium, potassium, chloride, carbohydrate (glucose) and a base.  Low osmolarity solutions reduce the need for IV fluids, reduce stool output and reduce vomiting frequency.

But… a major limitation to the use of ORS is its taste – and this is where apple juice comes in. For minimally dehydrated patients, half-strength apple juice is associated with fewer treatment failures compared to ORS and could suit as a more palatable alternative.  Take a look at a sweet summary (pun intended!) of the “apple juice trial”.

Breastfeeding should continue and a child can be supplemented with ORS if this is needed.  Children can go back to a normal diet after the illness has passed.

Enteral (oral / NG) versus IV hydration

Most studies show that enteral rehydration with ORS is just as effective as IV hydration in mild to moderate dehydration with a 2006 Cochrane analysis concluding that enteral rehydration is as effective if not better than IV rehydration with fewer adverse events and a shorter hospital stay.  It is also less invasive (even with NG placement) and anecdotally satisfaction is greater amongst parents.  It is very safe.

Enteral rehydration only fails in approximately 1 in 20-25 children.

Barriers to oral rehydration include unfamiliarity with the benefits, misconception that it takes longer than IV therapy, and that it has a high failure rate.

Contraindications to enteral rehydration include haemodynamic instability, abdominal distension, concern over ileus, absent bowel sounds, or impaired airway reflexes.

IV therapy is more invasive and involves placing and maintaining IV access.  There are also iatrogenic complications including electrolyte disturbance should inappropriate fluids / composition / volume / rate be used. 

But… in severely dehydrated children, put away the ORS and apple juice. They will need IV rehydration as first line.

Antiemetics

How can we support enteral fluids? Well, children who receive Ondansetron are less likely to vomit, have greater oral intake and are less likely to require IV hydration.  A Cochrane review demonstrates that Ondansetron also increases the proportion of children who stop vomiting when compared to placebo [RR1.4] and reduces the proportion of children needing IV therapy (and therefore admission rate) [RR 0.41].  Median length of stay is also shorter in the ED. 

Reported side effects are rare with very few reported side effects other than a few cases of increased frequency of diarrhoea.

Antiemetics alleviate vomiting by acting on the ChemoReceptor Trigger Zone and vomiting centre.  Ondansetron is a 5HT3 receptor antagonist.  This class of antiemetics have fewer adverse effects (than dopamine antagonists, anticholinergics, antihistamines and corticosteroids) and can be safely used in children.  The NICE guideline discusses its off-licence use (at time of publication it’s licence was for post-operative nausea and vomiting and chemotherapy induced vomiting).

Ondansetron prolongs the QT interval.  Recommendations are it should be avoided in those with long QT and should be used in caution where there may be electrolyte imbalance (severe dehydration) or on other QT-prolonging medication.

Ondansetron is relatively cheap  £1.71 for 10 4mg tablets and is available in oro-dispersible form (though these are much more expensive at £36 for 10x4mg tablets) and liquid (£36.82 for 40mg [50ml] bottle).

Probiotics

An ESPGHAN working group position paper on the use of probiotics in acute paediatric gastroenteritis concludes that:

  • Effects seen in clinical trial are probiotic strain specific (this makes ‘trial-life’ difficult to replicate in ‘real-life’).
  • A lack of evidence now doesn’t mean that there won’t be evidence sometime in the future. 
  • Safety profile of certain strains cannot be extrapolated to other strains.
  • Studies that report benefits in certain doses in certain settings have insufficient evidence to support a health benefit at lower doses and different setting.

…the jury’s still out.

Other therapies

Antibiotics and anti-diarrhoeal agents aren’t routinely recommended in the management of paediatric gastroenteritis.

For gastroenteritis in high income countries, the WHO does not recommend adding zinc to a treatment regimen (it is for gastroenteritis in low and middle income countries). 

Investigations

Routine lab testing in mild and moderate gastroenteritis is of little value in these patients and should be avoided unless clinically indicated.

This goes for stool samples too.  Stool cultures are not routinely indicated in immunocompetent children with non-bloody diarrhoea.

Confirmation of viral gastroenteritis after the child has been discharged from the ED, and likely on the road to recovery at home, adds very little to (A) the clinical diagnosis of viral gastroenteritis in the ED, (B) the management plan and (C) the clinical outcome. 

Should the investigation influence management, then stool sampling may be of benefit.  This could be applicable where an outbreak may be suspected in school or creche, where there may be a public health benefit.

Stool samples should be sent in cases of bloody diarrhoea, immunodeficiency and recent foreign travel.

How about tests for dehydration? Sadly there is no one test that correlates clinically with dehydration. Urine specific gravity in infants is unreliable because the kidney reaches adult concentrating abilities after the age of 1.  Also, the child often doesn’t begin urinating until rehydration has begun.

And glucose? Well, almost 10% of GE patients aged 1 month to 5 years in high income countries present with hypoglycaemia.  Risk factors for hypoglycaemia on presentation include a longer duration of vomiting and increased frequency of vomiting.  It would be reasonable to consider point of care glucose testing at triage for young children as identifying hypoglycaemia on clinical ground alone is difficult in this age group. 

Prevention

The key to reducing the burden (and generally for an all-round happier life!) is in the prevention of acute gastroenteritis.  Rotavirus vaccination is now commonplace thought the antibodies, the UK & Ireland and other countries around the world.  It is very effective.

In the home and in the ED…Handwashing, handwashing, handwashing!

Vaccination leads to a profound reduction in presentations and admissions and a fall in overall seasonal workload, often within the first year after the introduction of universal vaccination against rotavirus.  Even though only those under 1 year old are generally vaccinated, it has been shown to contribute to a significant herd effect with fewer cases than expected in older children. In Scotland, where initial vaccine uptake was 93- 94% during the first 2 years, annual rotavirus confirmed gastroenteritis cases fell by 84.7%, bed days reduced by 91% (from 325 to just 29), without any documented cases of intussusception.  Reductions were seen across all age groups despite only infants receiving the vaccine.  Similar results can be seen in other areas of the UK and Ireland.

The not to miss bits

  • Do not assume isolated vomiting in a child is gastroenteritis.  Consider other causes -these very widely from inborn errors of metabolism to diabetes mellitus, surgical obstruction to urinary tract infections. If you’d like to hear more, check out Dani’s talk on vomiting in children in DFTB Essentials.
  • Beware chronic diarrhoea in an infant – do they have malabsorption or is this a presentation of IBD or an immunodeficiency?
  • Beware the non-thriving child with diarrhoea.
  • And beware chronic diarrhoea.

But what happened to Conor?

Conor was given a cup of Dioralyte ORS and his favourite beaker filled with Dioralyte.  His mum was encouraged to give him syringes of 5 mls of Dioralyte frequently or for him to take sips from his beaker and was asked to document on a piece of paper how many he received.  He vomited after 30 minutes of this therapy.

You give him a dose of Ondansetron and place an NG tube and give him 100mls (10ml/kg) over 1 hour after deciding he does not need rapid rehydration but slightly more than normal maintenance.  He then receives maintenance volumes of Dioralyte via his NG, which he tolerates well and then starts to take his own sips from his beaker.

He does not vomit in the ED again, has one episode of loose stools, passes urine, and is tolerating fluids orally.  He’s smiling at you! You feel he can be discharged and council his mum regarding regular fluid intake, choice of fluids, of any red flags, and encouraged to return in the event of any concern.

Conor’s Dad calls to say that Conor’s 3 year old sister at home is now vomiting too!  But it’s OK – He’s not too worried about her and Conor’s Mum has advised his Dad to start giving her regular sips of Dioralyte at home…

References

Colletti JE, Brown KM, Sharieff GQ, Barata IA, Ishimine P. The Management of Children with Gastroenteritis and Dehydration in the Emergency Department. J Emerg Med [Internet]. 2010;38(5):686–98. Available from: https://dx.doi.org/10.1016/j.jemermed.2008.06.015

Elliott EJ. Acute gastroenteritis in children. Br Med J. 2007;334(7583):35–40.

Vecchio A Lo, Dias A, Berkley JA, Boey C, Cohen MB, Cruchet S, et al. Comparison of Recommendations in Clinical Practice Guidelines for Acute Gastroenteritis in Children. Gastroenterology. 2016;63(2):226–35.

Freedman SB, Willan AR, Boutis K, Schuh S. Effect of dilute apple juice and preferred fluids vs electrolyte maintenance solution on treatment failure among children with mild gastroenteritis: A randomized clinical trial. JAMA – J Am Med Assoc. 2016;315(18):1966–74.

BK F, A H, JC C. Enteral vs Intravenous regydration therapy for children with gastroenteritis: A meta-analysis of randomized controlled trials. Arch Paediatr Adolesc. 2004;158(1):483–90.

Hartling L, Bellemare S, Wiebe N, Kf R, Tp K, Wr C, et al. Oral versus intravenous rehydration for treating dehydration due to gastroenteritis in children (Review). 2006;

Fedorowicz Z, Jagannath V, Carter B. Antiemetics for reducing vomiting related to acute gastroenteritis in children and adolescents. [Internet]. Cochrane database of systematic reviews. 2011. Available from: https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD005506.pub5/full

NICE. Management of vomiting omiting in children and y young oung people with gastroenteritis : ondansetron. NICE GUIDELINES. 2014. p. 1–20.

Szajewska H, Guarino A, Hojsak I, Indrio F, Kolacek S, Shamir R, et al. Use of Probiotics for Management of Acute Gastroenteritis : A Position Paper by the ESPGHAN Working Group for Probiotics and Prebiotics. 2014;58(4):531–9.

Forrest R, Jones L, Willocks L, Hardie A, Templeton K. Impact of the introduction of rotavirus vaccination on paediatric hospital admissions , Lothian , Scotland : a retrospective observational study. 2017;323–7.

MARLOW RD, MUIR P, VIPOND I, TROTTER CL FA. Assessing the impacts from the first year of rotavirus vaccination in the UK. Arch Dis Child. 2015;100(Supl 3):A30.

The 44th Bubble Wrap

Cite this article as:
DFTB, T. The 44th Bubble Wrap, Don't Forget the Bubbles, 2020. Available at:
https://dontforgetthebubbles.com/the-44th-bubble-wrap/

With millions upon millions of journal articles being published every year it is impossible to keep up.  Every month we ask some of our friends from PERUKI (Paediatric Emergency Research in UK and Ireland) to point out something that has caught their eye.

Article 1: Neurodevelopmental outcomes at the edge of viability.

Metabolic presentations part 2: children and adolescents

Cite this article as:
Taciane Alegra. Metabolic presentations part 2: children and adolescents, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.28592

Neurological symptoms in a healthy teen

Jane, 14 years old, is brought in by ambulance, unconscious after a 20-minute generalized tonic-clonic seizure at home. She’s wheeled into resus, and while she has a cannula inserted, you take a history from her father. You learn that she has been a healthy child who’s never had a seizure before, with no chronic conditions, no history of drug abuse, no acute illness, and no sick contacts. She’s a vegetarian and enjoys dancing. She’s started a new ‘intermittent fasting diet’ and yesterday hadn’t eaten since brunch. She went to bed early and this morning her mother was woken early by strange sounds coming from Jane’s room and found her seizing on the floor. 

Her primary survey shows that she’s maintaining her airway, is tachypnoeic with oxygen saturations of 98% in air and clear lungs, a normal cardiovascular examination and a GCS of 10, with global hyperreflexia.

This adolescent has an acute onset of neurological symptoms. The differential diagnoses are broad, but her symptoms were precipitated by a new diet that required prolonged fasting. This case is a red flag for a metabolic condition! 

The RCPCH Decreased Consciousness (DeCon) guideline lays out an approach to the child with a decreased conscious level, including differentials, investigations and management (take a look at the DeCon poster and summary guidance). 

You send some bloods and, as suggested by the RCPCH DeCon guideline, you include an ammonia.

Some points to remember

Common things are common: sepsis, CNS infections, intoxication (prescribed and recreational drugs), and primary seizure disorders should all be considered here, but extend your differentials to conditions that can be individually rare but are common as a group: metabolic diseases.

All children presenting with a decreased conscious level, regardless of age, should have an ammonia sent as part of their initial investigation in ED… this could be a case of an undiagnosed urea cycle defect. 

In late onset urea cycle defects, acute metabolic encephalopathy develops following metabolic stress precipitated by a rapid increase in nitrogen load from: 

  • infection
  • trauma
  • rapid weight loss and auto-catabolism
  • increase in protein turnover from steroids
  • surgery and childbirth
  • or other precipitants of protein catabolism.

Adolescents and adults with an undiagnosed urea cycle defect may be completely fit and well, but may have chronic symptoms such as headache, cyclical vomiting, behavioural difficulties, psychiatric symptoms or mild learning difficulties.

They may be selective vegetarians, restricting their protein intake.

Between episodes patients are relatively well. However, acute presentations can be fatal or patients may be left with a neurological deficit.

For more information about cycle urea disorders, check out Metabolic presentations part 1.

The take home

Always send an ammonia in any child presenting with an acute encephalopathy or decreased GCS.

Disorders involving energy metabolism

Next up is Liz, a patient with a diagnosed metabolic disorder.

Liz is a 3-year-old girl from the countryside, who is visiting her grandmother in the city. She has had diarrhoea since yesterday and started vomiting last night. In the last 3 hours, she hasn’t been able to tolerate anything orally. There has been no fever or respiratory symptoms and she is passing urine as normal. Her 5-year-old cousin has similar symptoms. 

Her Grandmother informs you that Liz has MCAD deficiency and her emergency plan was tried at home, without success. Liz is not usually treated at your hospital and you don’t have her chart. Unfortunately, Liz’s grandmother didn’t bring the plan to the hospital. 

Liz looks tired and is mildly dehydrated, but smiles at you. Her heart sounds are normal and her chest is clear. She has increased bowel sounds, a soft abnormal with mild diffuse pain on deep palpation and no masses or organomegaly. She’s afebrile but tachycardic at 165, her capillary  refill time is 3 seconds, and her systolic BP is 104mmHg.

You put in a cannula and measure bedside glucose and ketones. Liz has a hypoketotic hypoglycaemia.

What is MCAD deficiency?

Medium-chain acyl-CoA dehydrogenase deficiency (MCADD) is the most common fatty acid oxidation disorder in Caucasians in Northern Europe and the United States. Most children are now diagnosed through newborn screening. In fatty acid oxidation disorders, the body can only partially break down fat.

Let’s recap some basic biochemistry: in prolonged fasting, the body’s normal response is to break down fat to create ketones, as an alternative source of energy. However, children with MCAD deficiency can’t produce large amounts of ketones, so their ketone response is not appropriate to the degree of hypoglycaemia. 

Clinical symptoms in a previously apparently healthy child with MCAD deficiency include hypoketotic hypoglycemia and vomiting that may progress to lethargy, seizures, and coma, triggered by a common illness. Hepatomegaly and liver disease are often present during an acute episode. These children appear well at birth and, if not identified through newborn screening, typically present between 3 and 24 months of age, although presentation even as late as adulthood is possible. The prognosis is excellent once the diagnosis is established and frequent feedings are instituted to avoid any prolonged periods of fasting (Merritt and Chang, 2019).

Children with fatty acid oxidation disorders (medium, long and short chain defects) have typical acylcarnitine patterns. This is one of the reasons acylcarnitines are sent as part of metabolic and hypoglycaemia work-ups.

What is the priority in acute presentations?

Children who have MCADD, like Liz, need extra calories when sick. The most important intervention is to give simple carbohydrates by mouth, such as glucose tablets or sweetened, non-diet beverages, or intravenously if needed to reverse catabolism and sustain anabolism. In Liz’s case, she’s vomiting all oral intake so cannot tolerate oral carbohydrates, so the intravenous route is necessary. 

The key priorities are:

  • Correct hypoglycaemia immediately with 200mg/kg glucose: 2 ml/kg of 10% glucose or 1ml/kg of 20% glucose, over a few minutes. 
  • Treat shock or circulatory compromise with a bolus of 20ml/kg 0.9% sodium chloride. 
  • Give maintenance fluids with potassium once the plasma potassium concentration is known and the child is passing urine. 

Where can you find resources?

The British Inherited Metabolic Disease Group, BIMDG, has specific guidance on their website.

Disorders involving storage of complex molecules

Mike is 12 years old, presenting to the ED with cough and fever. He has been coughing for 10 days, worse progressively in the last 5  and febrile for the last 3 days. He’s been lethargic since yesterday and even when afebrile he looks unwell. His appetite is poor and he has been “sipping some apple juice”. You learn from his mother that he has a condition called Mucopolysaccharidosis (MPS) type I and is receiving treatment with “the enzyme”. Every now and again, “he is chesty and needs to come to hospital”. 

You examine Mike. He’s pink and well hydrated, but looks sick. You notice that he is shorter than an average 12 year old boy, has hand contractures and coarse facial features. 

He has a soft systolic cardiac murmur with good pulse volume. On auscultating his chest you hear creps and rhonchi on the right side. He has mild hepatomegaly and an umbilical hernia. 

His temperature is 37.5ºC, heart rate is132, respiratory rate 30, and oxygen saturations are just 88% in air.

A bit about mucopolysaccharidoses (MPS)

In mucopolysaccharidosis disorders, the body is unable to break down mucopolysaccharide sugar chains. These mucopolysaccharide sugars build up in cells, blood and connective tissue: hence the name, ‘storage disorders’.

In general, most affected people appear healthy at birth and experience a period of normal development, followed by a decline in physical and/or mental function.

As the condition progresses, it may affect appearance; physical abilities; organ function; and, in most cases, cognitive development. 

Most cases are inherited in an autosomal recessive manner, although one specific form (Type II) follows an X-linked pattern of inheritance. 

Specific treatment can be provided via enzyme replacement therapy or haematopoietic stem cell transplantation in the early stages. 

Presently, enzyme replacement therapy is available for MPS I, II and VI and is given as an intravenous infusion either weekly or biweekly, depending on the disease. 

Both enzyme-replacement and haemotopoietic stem cell treatments still have gaps and few clinical trials supporting them. (rarediseases.info; Dornelles et.al, 2014).

What treatment should be started in the ED?

Patients with Mucopolysaccharidosis don’t require any emergency treatment in the ED for their underlying metabolic disease. They are, however, at increased risk of respiratory infections.

Mike is likely to have a community acquired pneumonia and needs to be treated accordingly with oxygen and antibiotics.

References

Adam , HH. Ardinger, RA. Pagon, S. E. Wallis, L. J. H. Bean, K. Stephens, & A. Amemiya (Eds.), GeneReviews® [online book]

Merritt JL,  Chang IJ. Medium-Chain Acyl-Coenzyme A Dehydrogenase Deficiency.  GeneReviews® [online book], June 2019. Available at  https://www.ncbi.nlm.nih.gov/books/NBK1424/

Genetic and Rare Diseases Information Center (GARD) https://rarediseases.info.nih.gov/diseases/7065/mucopolysaccharidosis 

Dornelles AD et al. Enzyme replacement therapy for Mucopolysaccharidosis Type I among patients followed within the MPS Brazil Network. Genet Mol Biol. 2014