Julia Hall and Abigail Lazenbury. Scurvy, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.33473
A three-year-old boy with speech and language delay presents to the Emergency Department with a unilateral atraumatic limp. He is initially managed as per the ‘Limping Child Pathway’. At his follow up review, he is now non-ambulatory, resistant to examination and holds his legs in a flexed position.
He has an extensive array of normal investigations including basic blood tests, pelvis and bilateral leg x-rays and abdominal ultrasound. He subsequently has an MRI which shows florid symmetrical increased signalling in his long bones with a wide non-specific differential. He develops a small patch of petechiae on his ear. Reviewing his history, it appears that his diet is extremely restrictive and made up of only lactose-free milk and cheesy biscuits.
Could this be scurvy?
Definition
Scurvy, or vitamin C deficiency, is a disease of malnutrition. It is one of the oldest nutritional deficiencies identified, and the source of one the world’s first randomized control trials. In 1753 James Lind published A Treatise of the Scurvy. Like a lot of research, this paper was widely ignored. It wasn’t until about 10 years later, when sailors got to try sauerkraut, wort, or syrup of oranges and lemons (all contain vitamin C) did the higher ups at the Admiralty begin to take notice.
Scurvy is rare in higher income countries and therefore often forgotten as a potential differential diagnosis.
Pathophysiology
Vitamin C, also known as ascorbic acid, is a water-soluble essential vitamin. It is found in citrus fruits, peppers, potatoes and broccoli. In babies, vitamin C is provided in the breast milk. Vitamin C has important roles in the body including wound healing, bone, cartilage and blood vessel maintenance and helps with the absorption of iron. It is also involved in fatty acid transport, neurotransmitter synthesis, prostaglandin metabolism and nitric oxide synthesis.
Dietary doses of up to 100mg/day can be absorbed in the distal small intestine. It is renally excreted and therefore the kidneys are important in regulating vitamin C concentration in the blood. The World Health Organisation recommended daily allowance for infants and children is 25mg per day.
The clinical manifestations of scurvy result from disordered pathways that utilise vitamin C, such collagen and connective tissue synthesis. Symptoms can occur after just one to three months of inadequate vitamin C intake.
Signs and symptoms
Early manifestations of scurvy are often non-specific. Consider the infant with fatigue, anorexia, weight loss and low grade fever – these are all early signs of scurvy but scurvy was unlikely to have made it into your differentials list. Petechiae can also be present – you probably ran an FBC and CRP. But petechiae, follicular hyperkeratosis and perifollicular haemorrhage are all cutaneous manifestations of scurvy, while gingival signs include swelling, bleeding and loss of teeth.
And consider the limping child. Arthralgia, limb and joint swellings, limp, inability to weight-bear are often the presenting features of children being brought to medical attention. Although rare, scurvy should be considered in the differential of the limping child once the more common causes have been ruled out.
Risk Factors
In children of the developed world, risk factors for developing scurvy are severe dietary restriction of fruit and vegetables. These can be secondary to autism, developmental delay and psychiatric disorders, as well as the extremely fussy child.
Diagnosis
Scurvy is a clinical diagnosis based on presentation of typical signs and symptoms alongside a dietary history of restrictive vitamin C intake for at least 1-3 months. Most laboratories cannot process ascorbic acid levels. If they can be measured then a level less than 11 umol/L would be considered deficient.
The diagnosis is, in the main, a clinical one. When a signs and symptoms respond to dietary changes or supplementation with Vitamin C then you know you are on the right track.
Classic bony signs on imaging include: periosteal oedema, sub-periosteal collections and sub-periosteal haematomas, lucent bands through long bone metaphyses, osteopenia and widening of the distal extremity of the femur.
Management
- Vitamin C supplementation orally for children with 100-300mg for one month or until full recovery.
- All children under five years of age are recommended to take a multivitamin (unless formula feeding as this is already fortified).
- Referral to a dietician for dietary education is imperative.
- Resolution of symptoms can start within 24 hours but may require a few weeks of treatment to fully resolve.
Two months later, the child was seen in paediatric outpatients running and jumping in the consulting room. They subsequently had an appointment with the dieticians who discussed dietary changes with the family and ensured that all calorie and nutrient requirements were being met. The child continued to have a restricted diet and will therefore need a multivitamin supplement long term.
Scurvy is a preventable, easily treatable disease which due to its non-specific symptoms is often misdiagnosed or carries a delay in diagnosis, with patients presenting to healthcare professionals on multiple occasions. A good nutritional history is key to diagnosis.
Selected references on scurvy
Agarwal A, Shaharyar A, Kumar A et al. Scurvy in pediatric age group- A disease often forgotten? Journal of clinical orthopaedics and trauma. 2015; 6(2): 101-7 https://doi:10.1016/j.jcot.2014.12.003
Alqanatish JT, Alqahtani F, Alsewairi WM, Al-Kenaizan S. Childhood Scurvy: an unusual case of refusal to walk in a child. Pediatric Rheumatology 2015; 13(1): 23 https://doi:10.1186/s12969-015-0020-1
Chalouhi C, Nicolas N, Vegas N et al. Scurvy: A New Old Cause of Skeletal Pain in Young Children. Frontiers in Pediatrics. 2020; 8:8 doi:10.3389/fped.2020.00008
De Ioris MA, Geremia C, Diamanti A et al. Risks of inadequate nutrition in disabled children: four cases of scurvy. Archives of Disease in Childhood. 2016; 101(9): 871 https://doi:10.1136/archdischild-2016-310911, https://doi:10.1136/archdischild-2016-310911
Kitcharoensakkul M, Schulz CG, Kassel R et al. Scurvy revealed by difficulty walking: three cases in young children. Journal of Clinical Rheumatology: practical report on rheumatic and musculoskeletal disease. 2014; 20(4): 224-228 https://doi: 10.1097/RHU.0000000000000101
Noble JM, Mandel A, Patterson MC, Scurvy and rickets masked by chronic neurologic illness. Pediatrics. 2007; 119(3): e783-90 https://doi:10.1542/peds.2006-107
Pazirandeh S, Burns D. Overview of water-soluble vitamins. In: D, Seres, L, Kunins eds. UpToDate. 2020. Waltham, UpToDate [ Accessed 28th February 2021 ] Available from https://www.uptodate.com/contents/overview-of-water-solube-vitamins
Ratanachy EK, Sukswai P, Jeerathanyasakun Y, Wngtapradit L. Scurvy in pediatric patients: a review of 28 cases. Journal of the Medical Association of Thailand. 2003; 86(3): S734-S740
Weinstein M, Babyn P, Zlotkin S. An orange a day keeps the doctor away: scurvy in the year 2000. Pediatrics. 2001; 108 (3): E55. https://doi:10.1542/peds.108.3.e55
World Health Organization. Scurvy and its prevention and control in major emergencies [online]. World Health Organisation, 1999 [Viewed 28th February 2021]. Available from: https://apps.who.int/iris/bitstream/handle/10665/66962/WHO_NHD_99.11.pdf?ua=1
All paediatricians are complicit in delivering a racist healthcare service
Zeshan Qureshi and Anna Rose. All paediatricians are complicit in delivering a racist healthcare service, Don't Forget the Bubbles, 2021. Available at:
https://doi.org/10.31440/DFTB.33938
We’re supposed to be the nice ones. The friendly, fun, caring and supportive speciality – right? We’re the ones who have teddies on our stethoscopes and know all the words to Disney songs. In the hospital, we’d like to think we’re the Good Guys – but maybe we’re not when it comes to race.
The COVID-19 pandemic has been a monumental challenge to the NHS and has, undoubtedly, showcased the everyday heroism of our staff. It has also thrown a sharp light onto the ongoing racial inequalities in our society and healthcare systems. Racial disparities in the pandemic have been widely documented – and make for sobering reading. Analysis of national hospital data suggests that people of black and other minority backgrounds are up to twice as likely to die from the virus, as compared to white Britons – with some groups, such as black African-born men living in Britain, having an even higher risk [1]. Despite this, we have little doubt that the major impetus for the unprecedented emergency measures, national lockdowns, and political obsession was not the deaths of the poor, the ethnic minorities, or those in low and middle-income countries – but the perceived threat to wealthy, predominantly white, Westerners.
In an eerie parallel to the racial inequalities highlighted by the pandemic, the past year has also seen racial tensions in the USA reach boiling point. Following several high-profile incidents of police brutality, there was an eruption of social unrest and protest in America and around the world. The systemic disregard of black lives is not just written in blood on American pavements. It is written into the systems that surround us in our everyday working lives. As a speciality, and in the NHS as a whole, we must confront these engrained systemic inequalities, if we are to provide truly equitable care to all of our patients.
In this blog series, we will examine how clinical outcomes for common paediatric conditions are worse for children from minority ethnic backgrounds. Stillbirth, low birth weight and preterm birth are all more common in minority groups as compared to white northern European populations [2,3,4 ]. Outcomes for common chronic conditions, such as asthma and type 2 diabetes, are also worse for children within minority groups [5,6]. This could be because care received by children with chronic conditions is worse. Non-white children with renal failure are less likely to pre-emptively receive a renal transplant, for example [7]. There are also complex social and environmental roots to these adverse health outcomes – such as increased poverty in non-White groups [8] — and we will try to investigate these issues in more detail.
We will also explore how paediatrics has normalized white Northern European genetics, physiology and behaviour, leading to biased clinical decision making. Normalization of one ethnic group has lead to the classification of other normal values (in both the laboratory and social sense) as pathological or inappropriate. In other words – you are only normal if you are white and normal. Neutrophil counts are often lower in black babies [9]. Rather than reporting ethnically normal ranges babies often end up having multiple blood tests due to a lack of awareness of the variation. Parents get told that their neutrophil count is low, but it’s acceptable for a black baby (rather than categorically stating that their count is normal). Worst still, it might be classified as a disease – benign ethnic neutropenia – despite not being associated with increased morbidity or mortality.
Within medical education, we are guilty of peddling irrelevant and outdated racial and religious stereotypes. These hold little educational value, but risk enforcing dangerous bias within our future doctors. Any paediatrician would be able to tell you about the association between Tay-Sachs disease and Ashkenazi heritage, or sickle cell disease and sub-Saharan Black Africans. Such associations are often over-simplified and over-emphasized, to the point of creating a disease-ridden caricature, particularly in exam questions. Most of these stereotyped conditions are very rare, and over-emphasis during medical school risks blinkering us to more common diagnoses. We’ll explore how racial bias is ingrained in medical education in the UK, and try to come up with some ideas on how we can improve MedEd to be more diverse and inclusive in the future.
There also seems to be a disproportionate concern that those from Muslim backgrounds might be consanguineous, and that we need to ask about this even when it is not relevant to the presenting complaint. Conversely, when genetic testing is being sent off, a detailed family tree needs to be drawn. It should include details of any consanguinity – yet it seems that a white family is less likely to be asked. As first or second cousin marriages are no longer a social norm in the UK, they have become defined by pathological associations with genetic conditions, such as inborn errors of metabolism. And whilst there are, of course, differences in the prevalence of disease alleles in different populations, and an increased risk of recessive disorders in families with intergenerational consanguinity, it does not automatically follow that a child from a Muslim background has a recessive disorder, or that a white British child does not. The same considerations need to be given to other cultural practices that might be different to the social norms of Northern and Western Europe. Putting children on a vegetarian diet is often classed as a ‘restrictive diet’ – despite the fact that it is only restrictive based on traditional Western standards – and might, in fact, hold health benefits [10].
Finally, in our series, we will examine how systemic racism within the health service tolerates – and sometimes even facilitates – the unacceptable behaviours demonstrated by some parents. One thing that sets paediatrics apart from adult medicine is that patients are almost never seen alone, and a parent is often required to deliver care. This can present a dilemma to staff when confronted with a racist parent. Any punishment directed towards the parents might directly harm their child. We will explore how guidelines should be developed to help clinicians handle racist parents, whilst minimizing the effect on the clinical care of our patients.
It can be painful for us – as individuals and as a speciality – to consider that we might be complicit in a racist system that ultimately leads to poorer health outcomes for some children. Just because something is painful, does not mean we shouldn’t do it. We hope that you’ll join us for this series of short articles, as we try to explore how we can begin to move from a white-centric healthcare system to a child-centred one.
Selected references
1) The IFS Deaton Review. Are some ethnic groups more vulnerable to COVID-19 than others?
2) Gardosi J, et al. (2013). Maternal and fetal risk factors for stillbirth: population-based study. BMJ 346:f108.
3) Kelly Y, et al. (2008). Why does birthweight vary among ethnic groups in the UK? Findings from the Millenium Cohort Study. Journal of Public Health, 31:131–137.
4) Aveyard P, et al (2002). The risk of preterm delivery in women of different ethnic groups. British Journal of Obstetrics and Gynaecology 109:894-899.
5) Asthma UK (2018) On the Edge: How inequality affects people with asthma. Available at www.asthma.org.uk
6) RCPCH (2020) State of Child Health: Diabetes. Available at www.rcpch.ac.uk
7) Plumb LA et al. (2021) Associations between Deprivation, Geographic Location, and Access to Pediatric Kidney Care in the United Kingdom. CJASN. 16:194-203.
8) Office for National Statistics (2020) Child poverty and education outcomes by ethnicity. Available at www.ons.gov.uk.
9) Haddy TB, Rana SR, Castro O. (1999) Benign ethnic neutropenia: what is a normal absolute neutrophil count? J Lab Clin Med. 133:15-22.
10) Kalhoff H. et al (2021) Vegetarian Diets in Children—Some Thoughts on Restricted Diets and Allergy. International Journal of Clinical Medicine. 12:43-60.