Guillain-Barre Syndrome

Cite this article as:
Aoife Fox. Guillain-Barre Syndrome, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.25051

A 2-year-old girl, Amy, attends the emergency department. Her father says that for the last 24 hours she has been refusing to walk. Prior to this, she was running amuck without difficulty. In the ED, you notice that she is now having difficulty crawling. She has no significant medical history but did have fever along with a runny nose and cough 2 weeks prior to her attendance which her parents managed with paracetamol at home.

 

What is Guillain-Barré Syndrome?

Guillain-Barré Syndrome (GBS) is a group of acute immune-mediated polyneuropathies.  It most commonly presents as an acute monophasic, paralyzing illness provoked by a preceding infection.

It is the most common cause of acute flaccid paralysis in children. The annual incidence is 0.34 to 1.34 cases per 100,000 in under the 18s, which makes it less common in children than in adults. It rarely occurs in children younger than 2 years, but when it does affect younger children because GBS is way off our radars, this can make it really tricky to diagnose. Boys are affected more often than girls.

 

What causes it?

It’s thought that an immune response cross-reacts with the myelin or axon of peripheral nerves due to molecular mimicry. Similar peptide sequences between the body’s own peptides and foreign peptides sometimes cause the immune system to get confused and attack its own tissues.  You probably knew that myasthenia gravis is due to auto-antibodies against the acetylcholine receptor but did you know that the receptor shares a 7 amino acid sequence with HSV, the herpes simplex virus? It’s thought that exposure to HSV may be the precipitant for myasthenia gravis.

Approximate 2/3 of patients give a history of an antecedent respiratory tract or GI infection. Campylobacter infection is the most commonly identified precipitant and can be demonstrated in as many as 30% of cases. Other infectious precipitants include CMV, EBV, Mycoplasma pneumoniae, and influenza-like illnesses.

Other suggested triggers include immunization, although there is no clear causal relationship several cases suggest an association, as well as one with trauma and surgery.

What about Guillain Barre and COVID?

There have been several case studies reporting GBS associated with SARS-CoV-2 during the COVID-19 pandemic. Given the small number of cases, it is unclear whether severe neurological deficits are typical features of COVID-19 associated GBS. An answer to the diagnostic conundrum of whether the respiratory compromise in COVID19-associated-GBS is due to coronavirus or muscle weakness is yet to be answered.

 

How can I recognize it?

GBS classically begins with paraesthesia in the extremities – fingers and toes –  followed by lower extremity symmetric, or modestly asymmetric, weakness that ascends up the body. In severe cases, the muscles of respiration are affected, in about 10-20% of children.

Cranial neuropathy can also occur, most commonly affecting facial nerves, causing bilateral facial weakness.

Autonomic dysfunction occurs in approximately half of children with GBS: cardiac dysrhythmias, orthostatic hypotension, hypertension, paralytic ileus, bladder dysfunction, sweating.

Physical exam typically reveals:

  • Symmetric weakness
  • Diminished or absent reflexes
  • Gait abnormalities
  • Sensory symptoms include pain, paraesthesia (reflecting nerve irritability)

Generally, children have shorter clinical courses and more complete recoveries in comparison to adults. A child’s function typically deteriorates for 2-4 weeks followed by a slow return of function over the coming weeks to months.

 

On examination, you find a quiet child who is otherwise acting appropriately. She is afebrile and the rest of her vitals are within normal limits. No bruises or rashes are observed on her skin and there is no evidence of trauma. Cardiovascular, respiratory, abdominal and ENT exams are unremarkable. Her extremities are warm and well perfused with normal pulses. There is no bony tenderness or deformities on palpation of her limbs. On neurological examination, she is moving all 4 limbs spontaneously. However, she will not bear weight or stand. Both her lower limbs are weak on exam. Her grip strength is reduced and when given a toy, it falls. Both upper and lower extremity reflexes are absent.

 

Subtypes

GBS most commonly presents in the classical way above: a mixed motor and sensory polyneuropathy with lower limb pain and ascending weakness. This is the classic Acute Inflammatory Demyelinating  Polyradiculopathy (AIDP), which accounts for 85 to 90% of cases in the developed world. But, there are a few other subtypes of GBS you should be aware of.

Acute Motor Axonal Neuropathy (AMAN), is a purely motor from of GBS, occurring mainly in Asia, Central and South America and associated with a preceding Campylobacter infection. Its clinical features are similar to AIDP, but respiratory failure is more common.

Acute Motor-Sensory Axonal Neuropathy (AMSAN) is similar to AMAN but with more sensory symptoms. The course tends to be prolonged and severe but is pretty uncommon in children.

Miller-Fisher syndrome is characterized by an external ophthalmoplegia, ataxia and muscle weakness with areflexia. It affects adults more commonly than children but should definitely be on your radar in a child presenting with cranial nerve and lower limb neurology.

 

How can it be diagnosed?

The initial diagnosis of GBS is based on the history and clinical exam – be suspicious of a child with lower limb weakness, weak reflexes and a preceding illness. Use investigations to confirm your suspicion.

CSF

CSF protein above 45mg/dL with a normal WCC count is present in 50-66% of patients in the first week after symptoms onset and ≥75% of patients in the third week. This disconnect between protein and white cells is called albuminocytologic dissociation.

Gadolinium-enhanced MRI of Spine

MRI will show contrast enhancement of the spinal nerve roots, cauda equina or cranial nerve roots. These changes aren’t specific to the GBS, but can be helpful in the correct clinical setting.

Nerve conduction studies

This is the most specific and sensitive test available for GBS, abnormal in up to 90% of cases. The test can be technically difficult in small children.

Antibodies

Antibodies against GQ1b (the ganglioside component of a nerve) are present in the vast majority of patients with Miller-Fisher syndrome.

 

In the emergency department, you send baseline bloods (FBC, U&E, LFTs and CRP) which are all normal and organize a CT head under sedation which is unremarkable. After getting consent from her parents you perform a lumbar puncture. The CSF appears clear. It has no red blood cells, 2 white blood cells and CSF glucose is within the normal limits but her protein is mildly elevated. No organisms were seen on gram stain and cultures had no growth after 5-days. You refer her to the neurology team for further investigation.

 

What else could it be?

The differential diagnosis of GBS is long.

 

Brain

  • Bilateral strokes
  • Acute disseminated encephalomyelitis
  • Acute cerebellar ataxia syndrome
  • Psychogenic symptoms

Spine

  • Anterior spinal artery syndrome
  • Compressive myelopathy
  • Transverse myelitis
  • Poliomyelitis
  • Infectious causes of acute myelitis

Peripheral nervous system

  • Chronic inflammatory demyelinating polyneuropathy
  • Critical illness polyneuropathy
  • Infection-related radiculitis (e.g. HIV, CMV, Lyme disease)
  • Thiamine deficiency
  • Toxins: biologic toxins (diphtheria), heavy metals (arsenic)
  • Vasculitis
  • Metabolic and electrolyte disorders (e.g. hypoglycaemia, hypophosphatemia)

Neuromuscular junction

  • Botulism
  • Myasthenia gravis
  • Neuromuscular blocking agents

Muscle

  • Acute inflammatory myopathies (e.g. dermatomyositis, polymyositis)
  • Acute viral myositis
  • Acute rhabdomyolysis
  • Critical illness myopathy
  • Metabolic myopathies (e.g. hypokalaemia, hyperkalaemia)
  • Mitochondrial myopathies

 

What is the treatment?

The mainstay of treatment is supportive management including close monitoring of motor, autonomic and respiratory function as well as pain management and prevention of immobility complications, such as pressure ulcers. ICU admission for mechanical ventilation will be required in 10-20% of kids. This is more likely to be needed in children with:

  • rapidly increasing weakness,
  • bulbar dysfunction,
  • bilateral facial weakness or

In addition IV immunoglobulin (IVIG) and plasmapheresis (plasma exchange) can be used in children with severe, progressive GBS (i.e. worsening respiratory status or need for mechanical ventilation, rapidly progressing weakness, inability to walk unaided or significant bulbar weakness).

IVIG  is typically preferred to plasmapheresis in children due to its better safety record and ease of administration

Plasmapheresis can be useful in bigger children where technically it is more feasible to perform. However, there are no reliable studies to suggest one has better efficacy than the other in children.

 

During her admission, Amy has a Gadolinium-enhanced MRI of the spine and nerve conduction studies which are consistent with the acute inflammatory demyelinating polyradiculopathy (ADIP) subtype of GBS. She is given IVIG. She does not develop any respiratory complications. On discharge after three weeks, her weakness is greatly improved and completely resolves over the next two months.

 

Bottom line

  • Clinical examination is key – do not forget to examine reflexes!
  • Always ask about recent viral illnesses.
  • GBS is the most common cause of acute flaccid paralysis in children and 10-20% will require mechanical ventilation.

 

Selected references

Bloch SA, Akhavan M, Avarello J. Weakness and the Inability to Ambulate in a 14-Month-Old Female: A Case Report and Concise Review of Guillain-Barre Syndrome. Case Rep Emerg Med [Internet]. 2013 [cited 2020 Apr 11];2013. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3572648/

 Yuki N, Hartung H-P. Guillain–Barré Syndrome. New England Journal of Medicine [Internet]. 2012 Jun 14 [cited 2020 Apr 5];366(24):2294–304. Available from: https://doi.org/10.1056/NEJMra1114525

Rudant J, Dupont A, Mikaeloff Y, Bolgert F, Coste J, Weill A. Surgery and risk of Guillain-Barré syndrome: A French nationwide epidemiologic study. Neurology. 2018 25;91(13):e1220–7.

Hicks CW, Kay B, Worley SE, Moodley M. A clinical picture of Guillain-Barré syndrome in children in the United States. J Child Neurol. 2010 Dec;25(12):1504–10.

Dimachkie MM, Barohn RJ. Guillain-Barré Syndrome and Variants. Neurol Clin [Internet]. 2013 May [cited 2020 Apr 5];31(2):491–510. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3939842/

Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barré syndrome. The Lancet [Internet]. 2016 Aug 13 [cited 2020 Apr 5];388(10045):717–27. Available from: https://www.sciencedirect.com/science/article/pii/S0140673616003391

Ryan MM. Pediatric Guillain-Barré syndrome. Curr Opin Pediatr. 2013 Dec;25(6):689–93.

Hughes RAC, Wijdicks EFM, Barohn R, Benson E, Cornblath DR, Hahn AF, et al. Practice parameter: Immunotherapy for Guillain–Barré syndrome. Neurology. 2003 Sep 23;61(6):736.

He’s always sick: ENT infections and immunodeficiency

Cite this article as:
Alasdair Munro. He’s always sick: ENT infections and immunodeficiency, Don't Forget the Bubbles, 2019. Available at:
https://doi.org/10.31440/DFTB.20243

Otis is a 3yr old boy presenting to the emergency department with fever, and purulent discharge from his left ear. He otherwise looks well, however, his mother mentions this is his third ear infection since he was born, and he always seems to have a cough and a cold. She asks you if there could be a problem with his immune system?

Some children seem to have constant ear, nose or throat infections during childhood. We know that for a small, but important minority of children this may be the presenting feature of a primary immunodeficiency. Let’s look at how these may present, when to think of it, and what to do about it.

Primary immunodeficiency is rare

It’s worth stating from the outset, that the majority of children with recurrent ENT infections will not have a primary immunodeficiency. There is a relatively common phenomenon called “physiological immunodeficiency of infancy”, where-by there is a natural nadir in immunoglobulin levels as maternal immunoglobulin fades, and the child’s own immune system has only just become able to produce immunoglobulin for itself. This is at its lowest between 3-6 months and normally resolves by age 1. However, fully developed protection against encapsulated organisms doesn’t reach maturity until between 2-5 years, and IgA production doesn’t reach adult levels until adolescence. It can be completely normal for young children to suffer 4-11 respiratory infections a year (depending on exposure, e.g. siblings, nursery, etc.)

When should I suspect immunodeficiency?

When considering the characteristics of infections that should trigger suspicion for immunodeficiency, we should be thinking about:

More severe infections than is usual

Combined immunodeficiency disorders (affecting both cellular and humoral immunity), such as severe combined immunodeficiency (SCID), present in the first 3-6 months with severe, life-threatening infection. Unusually aggressive infections should prompt further investigation

Infections with unusual organisms

Infections with certain pathogens can point towards specific diagnoses, including respiratory infections with Pseudomonas aeruginosa (think cystic fibrosis or primary ciliary dyskinesia), oral/oesophageal candidiasis (think HIV or chronic granulomatous disease), upper respiratory infections with Pneumocystis carinii (think HIV or other T cell deficiencies) or recurrent otitis/sinusitis with Neisseria meningitidis (think complement deficiency).

Finally, to a lesser extent:

Frequency of infection

This is the least predictive of immunodeficiency, given the discussion above. Very frequent sinopulmonary infections in younger children with encapsulated bacteria can be the presenting feature of the rare condition X-linked agammaglobulinaemia (XLA: boys who produce no immunoglobulins). In late childhood and adolescence, the same presentation in a milder form may be a sign of combined, variable immunodeficiency (CVID), which is a heterogeneous group of disorders of antibody production.

Other, rare conditions include chronic granulomatous disease (CGD) which may present with deep abscesses of the outer ear or mastoid, or HIV presenting with recurrent otitis media (normally with other associated features)

 

When to refer

Some general guidelines have been produced by the Jeffrey Modell foundation for when to consider referral for immunodeficiency workup:

  • Four of more new ear infections within 1 year
  • Two or more serious sinus infections within 1 year
  • Two or more months on antibiotics with little effect
  • Two or more pneumonias within 1 year
  • Failure of an infant to gain weight or grow properly
  • Recurrent, deep skin or organ abscesses
  • Persistent thrush in the mouth or fungal infection on skin
  • Need for intravenous antibiotics to clear infections
  • Two or more deep-seated infections including septicaemia
  • A family history of primary immunodeficiency

Although having a low specificity, they provide a useful framework when thinking of children with more severe infections than usual.

 

Should I do some tests?

If considering referral, there are definitely some basic tests are useful to do first (if the child is severely unwell, don’t wait for tests to refer).

Full blood count

This is useful for ANY suspected immunodeficiency. Persistent lymphopaenia in a child <2yrs should prompt screening for SCID.

NB: It can be normal to have transient lymphopaenia or neutropaenia in isolation in young children following a viral illness. Incidental neutropaenia does not need repeat testing if there are no concerns about underlying immunodeficiency.

Immunoglobulins

IgG, IgM and IgA levels are useful to investigate children with recurrent ENT/airway infections.

It is also worth considering an HIV test if symptoms are consistent, but ensure you have a discussion with parents before testing.

If both FBC and immunoglobulins are normal in the setting of recurrent infections, it is perfectly acceptable to wait for 3 -6 months to see if the condition improves before referral.

 

Conclusions

  • Primary immunodeficiencies are rare but important, and ENT infections may be the presenting feature
  • The severity of infection and presence of opportunistic pathogens are a much stronger predictor than the frequency of infections
  • Basic tests such as FBC and Immunoglobulins should be performed in children prior to/pending referral if they are not severely unwell
  • Consider investigation and referral for primary immunodeficiency early in children with severe infections and failure to thrive, or those with family history

Further reading: https://www.entmasterclass.com/ENT_Journal_2019_Interactive.pdf page 9

Transient hypogammaglobulinaemia of infancy

Cite this article as:
Clementine David. Transient hypogammaglobulinaemia of infancy, Don't Forget the Bubbles, 2019. Available at:
https://doi.org/10.31440/DFTB.17301

A 14-month-old boy is brought to your GP clinic by his mother with recurrent upper respiratory tract infections. His mother reports that he attends full time day care and he is ‘constantly sick.’ You note that one of your colleagues has seen him twice for acute otitis media this year.

Kawasaki’s Disease

Cite this article as:
Alyssa Courtney. Kawasaki’s Disease, Don't Forget the Bubbles, 2017. Available at:
https://doi.org/10.31440/DFTB.12282
A four-year-old Japanese boy was brought into Emergency with 5 days of fevers, non-exudative bilaterally injected sclerae, erythematous pharynx and irritability.

Wondering about the possibility of Kawasaki Disease, I turned to check the 2017 update of the American Heart Association Scientific Statement, focusing on considering a diagnosis of Incomplete Kawasaki Disease.

 

Bottom line:

  • Unchanged diagnostic criteria of complete Kawasaki Disease (KD)
  • Refined algorithm for evaluation of suspected incomplete KD (15-20% of cases)
  • Recommended ECHO at diagnosis, and repeated at 1-2 weeks and 4-6 weeks after treatment
  • Unchanged acute management– Intravenous immunoglobulin (IVIG) single dose 2g/kg over 10-12 hours. Ideally prior to day 10. Some countries continue to use high dose aspirin for varying durations.
  • Additional therapeutic options are outlined for the 10-20% with persistent or recurrent fever
  • New model of KD vasculopathy

 

What is Kawasaki Disease?

An acute, self-limited febrile illness of unknown cause, predominantly in children <5 years. It is the most common cause of acquired heart disease in developed countries. Without pathognomonic tests, we need to detect it clinically!

 

Epidemiology

  • Most common in Japan (where it was first described) with an annual incidence of 264.8 per 100 000 children in 2012. The estimated incidence in North America is 25 cases per 100 000 children <5 years of age per year. Australia has one of the lowest reported rates (3.7 per 100 000 <5 years of age), equivalent to 50–60 cases Australia-wide per year. It is likely that the current Australian incidence is higher.
  • Highest relative risk is in Asian children, especially of Japanese ancestry
  • The ratio of males to females is 1.5:1
  • Predominantly affects children 6 months to 4 years
  • Predisposing factors have been reported inconsistently
  • In Japan, the recurrence rate is 3%, and the relative risk in siblings is ten-fold higher

 

What is the aetiology?

We have no idea why…. BUT the resultant systemic inflammation leads to associated clinical findings: liver (hepatitis), lung (interstitial pneumonitis), gastrointestinal tract (abdominal pain, vomiting, diarrhoea, gallbladder hydrops), meninges (aseptic meningitis, irritability), heart (myocarditis, pericarditis, valvulitis), urinary tract (pyuria), pancreas (pancreatitis), and lymph nodes (lymphadenopathy).

A new model of Kawasaki disease vasculopathy involves three processes impacting muscular arteries. The first is a necrotising arteritis, followed by subacute/chronic vasculitis. The final process is luminal myofibroblastic proliferation.

 

How do we diagnose it in Australia?

Diagnostic Criteria

Fever for 5 days or more (typically high spiking (>39°C to 40°C) and remittent)

Plus 4/5 of:

  • polymorphous rash (usually within 5 days of fever onset)
  • bilateral (non-purulent) conjunctival injection (usually begins shortly after fever onset and often spares the limbus, an avascular zone around the iris)
  • mucous membrane changes e.g. reddened or dry cracked lips, strawberry tongue, diffuse redness of oral or pharyngeal mucosa (oral ulcers and pharyngeal exudates are not consistent with KD)
  • peripheral changes, e.g. erythema of the palms or soles, oedema of the hands or feet, and in convalescence desquamation
  • cervical lymphadenopathy (> 15 mm diameter, usually unilateral, single, non-purulent and painful in the anterior cervical chain)

AND exclusion of diseases with a similar presentation

  • Staphylococcal infection (e.g. scalded skin syndrome, toxic shock syndrome)
  • Streptococcal infection (e.g. scarlet fever, toxic shock-like syndrome not just isolation from throat)
  • Measles
  • Viral exanthems
  • Steven’s Johnson syndrome
  • Drug reactions
  • Juvenile rheumatoid arthritis.

Trickily, these children may have a concurrent viral infection, often adenovirus. Adenovirus is more likely with exudative pharyngitis and conjunctivitis and positive PCR assay. Kawasaki disease is more likely with erythema/swelling of hands and feet, a strawberry tongue, and a desquamating groin rash.

Inflammation and crusting of a recent Bacille-Calmette-Guérin (BCG) injection site may occur.

Consider an alternative diagnosis to Kawasaki Disease if there is exudative conjunctivitis, exudative pharyngitis, ulcerative intraoral lesions, bullous or vesicular rash, generalized adenopathy, or splenomegaly.

 

What is Incomplete Kawasaki Disease?

Scarily, this is so easily missed. They make up 15-20% of all cases!!
Patients with incomplete KD, particularly those <6 months of age and older children, may experience significant delays in diagnosis and these children are at high risk of developing coronary artery abnormalities.

Consider KD if:

  • Infants <6 months old with prolonged fever and irritability
  • Infants with prolonged fever and unexplained aseptic meningitis
  • Infants or children with prolonged fever and unexplained or culture-negative shock
  • Infants or children with prolonged fever and cervical lymphadenitis unresponsive to antibiotic therapy
  • Infants or children with prolonged fever and retropharyngeal oroparapharyngeal phlegmon unresponsive to antibiotic therapy

Evaluation of suspected incomplete Kawasaki disease (via McCrindle BW et al. 2017)

What can we investigate?

As suggested by RCH Melbourne guidelines, all patients should have

  • ASOT / Anti DNAase B
  • Echocardiography (at least twice: at initial presentation and, if negative, again at 6 – 8 weeks).
  • Platelet count (marked thrombocytosis common in the second week of illness)
  • Consider Mycoplasma

In addition, findings can provide support when considering Incomplete Kawasaki Disease – refer to the above algorithm.

Evolution of laboratory findings via Tremoulet et al.

  • KD is unlikely if ESR, CRP, and platelet count are normal after day 7 of illness.
  • Low WBC and lymphocyte predominance suggests an alternative diagnosis
  • Leukocytosis is typical in the acute stage, with granulocyte predominance
  • Normocytic, normochromic anaemia is common during inflammation
  • CRP and ESR elevation is nearly universal, CRP normalizes more quickly with inflammation resolution. ESR is elevated by IVIG therapy.
  • Minimally elevated ESR in the setting of severe clinical disease should prompt investigation for disseminated intravascular coagulation.
  • Thrombocytosis is a characteristic feature that generally doesn’t occur until the second week, peaking in the third week, normalizing by 4 to 6 weeks
  • Thrombocytopenia can be a sign of disseminated intravascular coagulation and is a risk factor for the development of coronary artery abnormalities
  • Mild to moderate elevations in serum transaminases or gammaglutamyl transpeptidase occur in 40% to 60% of patients, and mild hyperbilirubinemia occurs in ≈10%.
  • Hypoalbuminaemia is common and associated with more severe and more prolonged acute disease
  • Urinalysis may show pyuria in up to 80% of children, non-specific for KD
  • In children who undergo lumbar puncture, ≈30% demonstrate pleocytosis with a mononuclear cell predominance, normal glucose levels, and generally normal protein levels

 

Pitfalls

Fever and pyuria in an infant or young child may be diagnosed as a urinary tract infection, with subsequent development of rash, red eyes, and red lips attributed to an antibiotic reaction. Irritability and a culture-negative pleocytosis of the cerebrospinal fluid in an infant with prolonged fever suggestive of aseptic meningitis (or if antibiotics have been given, partially treated meningitis) may cause a diagnosis of KD to be overlooked. Cervical lymphadenitis as the primary clinical manifestation can be misdiagnosed as having bacterial adenitis. Gastrointestinal symptoms are considered for surgical causes, other physical findings of KD can be overlooked.

 

What is the treatment?

We’re aiming to prevent important coronary artery abnormalities. Timely (as soon as possible, ideally within 10 days) IVIG treatment reduces the incidence of coronary artery aneurysms (defined from absolute luminal dimensions) from 25% to 4%. Studies with additional therapies to IVIG have not substantially reduced this residual risk of 4%. Adverse effects are rare but include Coomb’s positive haemolytic anaemia and aseptic meningitis. The measles, mumps, and varicella vaccine should be deferred for 11 months unless at high risk (seek advice, may need repeat vaccination). If the diagnosis is delayed, IVIG should still be given (after the tenth day of illness) IF there is presence of fever, or continued elevation of ESR or CRP>3, indicating ongoing inflammation. Aspirin is used with the theoretical rationale of reducing coronary artery aneurysms (although there is no well-established evidence for this). In Australia, a dose of 3-5mg/kg daily from diagnosis until cardiology review at 6 weeks is routine. The newly released statement advises the administration of moderate to high-dose (80–100 mg/kg/day) aspirin is reasonable until the patient is afebrile. Patients should receive a seasonal influenza vaccination.

Fever usually resolves within 36 hours after IVIG infusion has been completed; if not, the patient is considered to have resistance to IVIG. 10-20% of patients will not respond to the single IVIG treatment dose. There is minimal data to support therapeutic agents for the child with IVIG resistance. Repeating the IVIG dose, 3 days of high-dose pulsed steroids, or 2-3 weeks of tapering prednisolone are all options. There are lower levels of evidence for infliximab and cyclosporine.

 

Coronary artery abnormalities

An angiographic study of 1100 patients showed coronary artery lesions in 24%, with aneurysms in 8% and a number of patients with stenoses and occlusions. Valvular regurgitation is usually mild to moderate in severity and resolves prior to follow-up. MR can occur after the acute stage from myocardial ischaemia. Patients after KD have been shown to have functional and anatomic abnormalities of the aorta with unknown long-term implications. Myocarditis is common during the acute illness but complete resolution is expected. Risk stratification for long-term management is based primarily on maximal coronary artery luminal dimensions, normalized as Z scores, and is calibrated to both past and current involvement. Patients with aneurysms require life-long and uninterrupted cardiology follow-up.

 

What is the prognosis?

  1. The case fatality rate is <0.1% in Japan, virtually all from cardiac sequelae.
  2. Peak mortality occurs 15 to 45 days after onset of fever, during which time well-established coronary artery vasculitis occurs concomitantly with marked elevation of the platelet count and a hypercoagulable state
  3. Coronary artery aneurysms from KD account for 5% of acute coronary syndromes (ACS) in adults <40 years of age

 

In Summary:

  • Be aware of the diagnostic criteria of complete Kawasaki Disease (KD)
  • Highest relative risk in Asian children, especially Japanese ancestry
  • Always consider incomplete KD (15-20% of cases) and refer to the algorithm if concerns, there are pitfalls!
  • Liaise with cardiology regarding an ECHO
  • Unchanged acute management– Intravenous immunoglobulin (IVIG) single dose 2g/kg over 10-12 hours. Ideally prior to day 10. Some countries continue to use high dose aspirin for varying durations.
  • Additional therapeutic options are outlined for the 10-20% with persistent or recurrent fever, minimal evidence for these
  • New model of KD vasculopathy but we are still in the dark regarding aetiology
  • Coronary artery aneurysms from KD account for approximately 5% of acute coronary syndromes (ACS) in adults <40 years of age

 

 Selected references

McCrindle BW, Rowley AH, Newburger JW, et al. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. Circulation2017;Mar 29

Yim D, Curtis N, Cheung M, Burgner D. Update on Kawasaki disease: Epidemiology, aetiology and pathogenesis. Journal of Paediatrics and Child Health 49 (2013) 704–708

Clinical Practice Guidelines, Royal Children’s Hospital, Melbourne

Kim DS, Kawasaki Disease. Yonsei Medical Journal. 47 (2006) (6): 759–72. PMC 2687814 PMID 17191303. doi:10.3349/ymj.2006.47.6.759

Yim D, Curtis N, Cheung M, Burgner D. An update on Kawasaki disease II: Clinical features, diagnosis, treatment and outcomes. Journal of Paediatrics and Child Health 49 (2013) 614–623

Tremoulet AH, Jain S, Chandrasekar D, Sun X, Sato Y, Burns JC. Evolution of laboratory values in patients with Kawasaki disease. Pediatr Infect Dis J. 2011;30:1022–1026

Dengler LD, Capparelli EV, Bastian JF, Bradley DJ, Glode MP, Santa S, Newburger JW, Baker AL, Matsubara T, Burns JC. Cerebrospinal fluid profile in patients with acute Kawasaki disease. Pediatr Infect Dis J. 1998;17:478–481

 

Does consuming peanuts in infancy reduce the likelihood of allergy?

Cite this article as:
Tessa Davis. Does consuming peanuts in infancy reduce the likelihood of allergy?, Don't Forget the Bubbles, 2015. Available at:
https://doi.org/10.31440/DFTB.6727

In a previous study, the authors of this paper found that Jewish children in the UK had 10 times the rate of peanut allergy as Jewish children in Israel. This also correlated with the earlier introduction of peanuts in Israel due to a tasty snack called Bamba.

bamba

Community needlestick injury in children

Cite this article as:
Henry Goldstein. Community needlestick injury in children, Don't Forget the Bubbles, 2014. Available at:
https://doi.org/10.31440/DFTB.5241

Alfie, 6, is playing at local playground under Mum’s watchful eye. He goes down the slide and jumps off, landing on his hands and feet. He starts to cry and shows his Mum a syringe lying in the bark and a needlestick injury of his left hand. Mum is distraught when she brings Alfie into your department. What next?

Bottom Line:

  • Needlestick injuries in the community are a source of great concern for parents.
  • There is one reported case of seroconversion of Hepatitis B in children.
  • The actual chance of viral transmission is very low.
  • Ensure your patient is immunised!
  • High risk patients should be discussed with your local infectious disease team for consideration of post-exposure prophylaxis.
  • Educate children not to handles needles – 2/3 CANSI’s are from intentional handling!

There is a risk of transmission of Hepatitis B, Hepatits C or HIV from a community-acquired needlestick injury (CANSI). Although the risk is very low, this is a source of significant concern to parents. Several studies lasting from several months to nearly two decades in length have looked at the epidemiology of CANSIs. These studies, undertaken in Melbourne, Montreal, Birmingham and Perth broadly agree that;

~65% CANSIs occur in boys

Mean age is around 6-8 years

In two-thirds of CANSIs, the syringe or needle was intentionally handled by the child

The most common site of injury was the hand

About a quarter of wounds bled

In the Melbourne study CANSIs often occurred in public places in parks (30%), in the street (18%), in carparks (5%) and at the beach (6%). In Montreal, CANSIs occurred predominantly in the street (30%) and in parks (24%). Whilst the obvious difference  is the lack of beaches in downtown Montreal, it’s also worth noting that a number of CANSIs must also occur in private residences.

So, what’s the risk?

Each of the papers described the baseline prevalence of HBV, HCV & HIV in their population, as well as the same prevalence within the IVDU community. None of the papers reviewed (total patients 416) reported any cases of seroconversion to Hep B, Hep C or HIV. There is a single case report of seroconversion to Hepatitis B after a CANSI in a child, reported in Barcelona in 1997. In 1999, Bowden et al, proposed conversion rates in the Victorian population to be around 6-30% for HepB, 0-7% for HepC and 0.4% HIV.

 

Although the risk is largely theoretical, factors that are considered to be high risk for acquired infection are:

  • known needle source user
  • needle user known to be infected
  • a deliberate assault
  • a large-volume injection
  • wide bore, hollow needle
  • blood in or on syringe
  • deep wound (vs superficial)

These children should be discussed with your local infectious diseases team for consideration of HIV post-exposure prophylaxis, after their initial management.

What is the initial management?

Wash the wound with soap and water.

Ensure the syringe/needle has been safely disposed

History of note:

  • Time, date and location of CANSI
  • Type of exposure?
  • What did the needle look like?
  • What kind of needle was it?
  • Is the child immunised? (specific details of each)
  • Were there other children around that may have an unreported CANSI?
  • Is this a high-risk exposure, as outlined above?

Take blood for HepB Surface antibody (HepB AbS) in a serum gel tube to store.

 

Consider tetanus vaccine +/- tetanus immunoglobulin.

Not required if immunised against tetanus in last five years.

If unimmunised, for immunoglobulin and vaccine.

Otherwise, if previously immunised, for booster dose.

 

Hepatitis B vaccination +/- Hep B immunoglobulin

If unimmunised, give first dose of vaccine and HepB Ig within 72 hours of exposure (in different limbs!)

If immunised, check titre & give booster.

 

Luckily, Alfie is immunised for both Hepatitis B and Tetanus. After a thorough wash of his hand, and some relatively obliging blood tests, he’s ready for home. His Mum asks if he needs any other medicine to reduce the risk of  “catching one of those viruses you mentioned.”

Post-exposure prophylaxis : Hepatits B immunoglobulin

There is a larger argument that there are risks associated with Hepatitis B immunoglobulin, including that of acquired infection, which must be weighted against the potential benefits of preventing a seroconversion when this may be highly unlikely in the first instance.

In the UK, Hep B immunoglobulin is only recommended in patients with exposure to known Hepatitis B source, although there is some leeway depending on the clinical circumstances. The Auckland District Health Board (ADHB), in NZ states “Administration of hepatitis immune globulin (HBIG) is not indicated if the child has completed a standard three-dose regimen of hepatitis B vaccination.” RCH Melbourne advises to offer HBIG to all unimmunised children with CANSI. There remains controversy in this component of management.

Hence, if the decision is made to treat, give HBIG within 72hrs.

(In Australia, Hep B Immunoglobulin is provided by the Red Cross Blood Service.)

Give the HBIG in a different limb to the Hep B booster you’ve just administered!

Dosing:

<30kg – give 100 iu IM injection

>30kg – give 400 iu IM injection

 

Post-exposure prophylaxis: HIV

The papers reviewed had no reports of viral transmission of HIV from a CANSI. All mentioned antiretroviral therapy as potential post-exposure prophylaxis for HIV exposure. There were no clear guidelines on which children should be offered HIV-PEP; the ‘high-risk’ patients identified in the list above were more likely to receive prophylaxis. In the Montreal study, of the 210 patients who presented thereafter, an offer of prophylaxis to 87 patients (41.4%) was documented, and 82 (94.3%) of these patients accepted. Prophylaxis was zidovudine and lamivudine for 28 days in 74 patients (90.2%), additionally eight patients were also prescribed a protease inhibitor (nelfinavir, indinavir or ritonavir). Papenburg and colleagues go on to describe the rates of adverse effects from these medications. Consideration of HIV:PEP should be discussed with the local infectious diseases team.

 

Follow up & counselling

Although the risk of seroconversion is low, it’s important not to underestimate the stress a needlestick will place on the child and family. Provide reassurance that the risk of viral transmission from a CANSI is very low. Don’t forget some written information about completing a catch-up course of immunisation. Contact your local paediatric infectious disease team; they may be happy to provide additional follow-up or counselling. That being said, always consider the prevalence of the blood-borne viruses where you work! The majority of DFTB readers are working in the Australasian, United Kingdom & North American settings; within and without these areas, the prevalence of Hep B, C & HIV can vary considerably.

 

It’s also worth noting that the studies mentioned probably underestimate the rate of CANSIs; not all children with a needlestick injury will tell their parents, and likewise, not all parents whose child reports a needlestick injury will present for care.

Finally, there’s clearly a huge public health component of this issue. Papenburg et al. identified that in nearly two-thirds of cases, the child actively handled the needle; it’s important to teach children to avoid any discarded syringes or needles and to tell an adult.

 

References:

Russell FM.  Nash MC. A prospective study of children with community-acquired needlestick injuries in Melbourne.  Journal of Paediatrics & Child Health.  38(3):322-3, 2002 Jun. https://onlinelibrary.wiley.com/doi/10.1046/j.1440-1754.2002.t01-2-00859.x/abstract

Papenburg J.  Blais D.  Moore D.  Al-Hosni M.  Laferriere C.  Tapiero B.  Quach C. Pediatric injuries from needles discarded in the community: epidemiology and risk of seroconversion.  Pediatrics.  122(2):e487-92, 2008 Aug. https://www.academia.edu/942640/Pediatric_injuries_from_needles_discarded_in_the_community_epidemiology_and_risk_of_seroconversion

Celenza, A. et al. Audit of emergency department assessment and management of patients presenting with community-acquired needle stick injuries. Australian Health Review, 2011, 35, 57–62. https://www.ncbi.nlm.nih.gov/pubmed/21367332

Garc ́ıa-Algar O, Vall O. Hepatitis B virus infection from a needle stick. Pediatr Infect Dis J. 1997;16(11):1099 https://journals.lww.com/pidj/Citation/1997/11000/Hepatitis_B_Virus_Infection_From_A_Needle_Stick.27.aspx

Makwana N.  Riordan FA. Prospective study of community needlestick injuries.  Archives of Disease in Childhood.  90(5):523-4, 2005 May. https://adc.bmj.com/content/90/5/523.short

Bowden S, Druce J, Kelly H. Stability of blood-borne viruses in the environment and risk of infection. Victorian Infect. Dis. Bull. 1999; 2: 71–2. https://docs.health.vic.gov.au/docs/doc/D785EE77B8899CD1CA2578C4000219EA/$FILE/vidbv2i4.pdf

Starship Children’s Hospital, Auckland, NZ – Clinical Guidelines (Needlestick Injuries) https://www.adhb.govt.nz/starshipclinicalguidelines/Needlestick%20Injuries.htm

Decle, P. Post-Exposure Prophylaxis (PEP) guidelines for children and adolescents exposed to blood-borne viruses 06/08/2011 https://www.chiva.org.uk/professionals/health/guidelines/pep/young-pep-ref.html

Royal Children’s Hospital, Melbourne, Clinical Practice Guidelines – Needlestick Injury https://www.rch.org.au/clinicalguide/guideline_index/Needle_Stick_Injury/

Updated 5/11/2017: Corrected initial investigations from HepB Surface Antigen to Antibody. See comments below.