1212 e-Letters

published between 2016 and 2019

  • Response to Seizures, safety and submersion: sense and sensibility

    I am grateful for the clarification of one specific point made in the original paper published in Archives of Disease in Childhood by Richard Franklin, John Pearn and Amy Peden (Drowning fatalities in childhood: the role of pre-existing medical conditions. Archives of Disease in Childhood 2017; 102:888-93). This relates to their recommendations on swimming safely that reflected both their collective experiential opinion, as well as the recommendations of authorities such as the ‘Royal Life Saving Society – Australia’ and other Australian water safety organisations. Understandably, these authorities will have a significant adult bias and one could – and reasonably should – question some of their criteria, both in terms of ‘seizures’ (i.e. what type of 'seizure') and seizure-frequency. I would challenge the comment made by the International Life Saving Federation in which they state: “Epilepsy submersion and drowning risk is greatest in an identified high-risk group that includes: those with frequent (more than one per year) seizures….”; the majority of paediatricians and paediatric neurologists and probably adult physicians that treat people with epilepsy would not define “frequent” as more than one seizure per year; by definition this would include two seizures per year. My point remains that doctors, and the many different authorities to which they provide expert advice, should no longer consider and cite epilepsy as a single disorder but as a group of disorders...

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  • Recent identification of pleuropulmonary blastoma following non-urgent resection of a cystic pulmonary airway malformation.

    We read with interest the recent paper by Cook et al(1) reporting their experience with 119 cases of cystic pulmonary airway malformation (CPAM); in which no reported cases showed malignant change. The potential for malignant transformation of CPAM is well-described but extremely rare. Type 1 can predispose to mucinous bronchiolo-alveolar carcinoma in adults, type 2 are associated with pleuropulmonary blastoma (PPB)(2). We describe a case of PPB diagnosed histologically following non-urgent resection of a CPAM.
    A 10-month old boy was routinely referred by primary care with episodic wheeze and shortness of breath. He was thriving with no clinical stigmata nor symptoms of malignancy, and no relevant family history.
    Examination noted reduced air entry to the left lung; chest x-ray showed extensive left sided hyperlucency with mediastinal shift. Urgent chest CT with contrast demonstrated a very large multi-septated cystic malformation arising in the left lower lobe, with no systemic arterial supply. He was referred to the paediatric thoracic surgeons.
    Four months later he had reduced exercise tolerance and one brief admission for pneumonia. Surgical excision took place one year after CT imaging. Resection was uncomplicated and subsequent histological identification of a type 2 CPAM with PPB was unexpected. There were nodules containing malignant spindle epithelioid cells and he has since commenced chemotherapy.
    Previous papers have shown that karyoty...

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  • Immunisation against group B meningococcal disease: clear benefits to infants in the UK.

    Nainani V, Gulal U, Buttery J, Snape MD

    Word count: 234

    As authors of a recent article demonstrating an increase in Accident and Emergency presentations for acute vaccine reactions following the introduction of the group B meningococcal vaccine (4CMenB) into the UK infant immunisation schedule in 2015 (1), we welcome correspondence from Mukherjee et al emphasising the ongoing risk of invasive meningococcal disease (IMD) in this country.

    These data give a local perspective to the national Public Health England surveillance data demonstrating a 50% reduction in group B meningococcal disease following introduction of the 4CMenB vaccine (2). Despite immunisation with 4CMenB being 82.9% effective against group B invasive meningococcal disease in infants, there were still 56 cases in England in the year to March 2017 in under 1 year olds, and a further 119 cases in 1 to 4 year olds (an age group that currently includes both immunised and unimmunised cohorts) (2) (3). In the context of the epidemiology of meningococcal disease in the UK, the benefits of immunisation with 4CMenB to infants clearly outweigh any risks of a transient febrile reaction. The current 4CMenB immunisation campaign is not expected to induce herd immunity, therefore invasive meningococcal bacteria will continue to circulate in the community and unimmunised infants remain at increased risk of invasive meningococcal disease compared to their immunised peers. Parents and clinicians need t...

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  • Response to Seizures, safety and submersion: sense and sensibility

    The Editorial by Professor Richard Appleton, ‘Seizures, safety and submersion: sense and sensibility’ addresses crucial points relating to children with epilepsy and their optimal, but safe, participation in aquatic activities 1. All agree that the goal is to ensure that children from all backgrounds and with pre-existent medical conditions grow up to have a ‘normal and unrestricted life’ 1. Aquatics are an important activity for all children in both developed and developing nations 2. Our study was undertaken specifically to determine the relative risk of different medical conditions 3. Like the other studies quoted by Professor Appleton, pre-existent epilepsy has been found to pose an increased risk of drowning by a factor of between 2 and 10. The absence of other pre-morbid diagnoses may either reflect a selection bias in that parents are not allowing those children to participate in aquatics or that parents recognise the hazards and put in place appropriate safety strategies. Differential aquatic exposure rates, specific for different pre-existent medical conditions, are unknown; and therefore denominators which define specific risks remain unknown. In our paper we recommend that ‘Children with epilepsy may swim with safety if drug levels are in the therapeutic range, the child has been seizure-free for 6-12 months and compensatory extra supervision is in place’ (3). These reflect the opinions of the authors, but are generally cognat...

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  • Ryanodine Receptor mutation

    After reviewing some cases presenting to Bristol with Rhabdomyolysis I wondered if this child had genetics sent for Ryanodine Receptor gene (RYR gene). This can cause malignant hyperthermia and in our case the boy resented with muscle pain on exertion and recurrent rhabdomyolysis. The article below is useful.
    Chan EK, Kornberg AJ, Ryan MM, A diagnostic approach to recurrent myalgia and rhabdomyolysis in children. Archives of Disease in Childhood 2015;100:793-797.

  • Community paediatrics in Israel - spotlight on terminology

    Confusion may arise in the minds of UK-based readers of this article due to the terminology used in this article, which differs slightly from the notion of “community paediatrics” in the UK.

    Whereas in the UK all children receive primary care from a General Practitioner (GP), who then makes referrals to secondary and tertiary level specialists as required, the Israeli “community paediatrician” referred to in this article is actually a primary care paediatrician, who delivers all aspects of medical care to the infant and child, much as the GP in the UK does for both adults and children.

    The majority of children and young people in Israel receive primary medical care from a fully qualified paediatrician who has achieved consultant (or specialist) status with a minimum of 4.5-5 years of general paediatric training. Only a minority of children and young people in Israel receive primary medical care from a Family Physician (equivalent to the UK “GP”). Paediatricians in Israel can work either in primary care or as paediatricians with an additional sub-specialty in hospitals, or can work in both settings in parallel (often working part or full time in hospital and moonlighting in primary care).

    In the UK, the “community paediatrician” is a second tier specialist who deals in specific medical areas , and accepts referrals from the primary medical carer (GP), via a selective referral system. Community paediatricians in the UK usually provide neuro-development...

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  • Bruising in children with bleeding disorders - Limitations
    Mallinath Chakraborty

    Dear Sir,

    We would like to congratulate the authors of this excellent observational study. For paediatricians, and for medico-legal professionals, data from this study would be invaluable in their practice.

    While the authors have discussed most of the limitations of their study, we would like to point out a few more which have come to our notice.

    1) Controls were recruited only from South Wal...

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  • Re:Bruising in children with bleeding disorders - Limitations
    Peter Collins

    Dear Editors

    We thank Drs Chakraborty and Morris for their interest in our study. We acknowledge that the children without bleeding disorders were only recruited in south Wales whilst those with bleeding disorders were recruited in centres around the UK. Given the data available we are not able to comment on whether children are likely to bruise differently dependent on where they live.

    We agree that...

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  • SIDS as the consequence of an immunological burst

    In his recent review “Infection: the neglected paradigm in SIDS research” Goldwater (2017) demonstrated that the infection model is the key pathological finding and the key epidemiological risk factor in SIDS. He reasoned that future research regarding the process how the microbiome shapes the immune system in infancy, will close remaining gaps in the knowledge about these tragic events. The well-known and worldwide similar distribution of age of SIDS-death with a clear peak between the 2nd and 4th month (AAP 2005, 2016) likewise supports this infection hypothesis. In this time slot the battle between microbial colonizing of the dermal and the mucosal tissue, including pathogens as well as microbiome building bacteria (Gensollen 2016) and the proceeding of the infant’s immature to a mature immune system (Basha 2014, Elahi 2013), potentially complicated by viral infections, opens a wide window for an immunological burst. In the neonate with little immunological memory the innate and adaptive immune system (immune cells, cytokines, antibodies, etc.) starts to mature rapidly in the first three months of his life (Basha 2014). Additionally CD71+ erythroid cells, which are enriched in the newborn period and which have actively immunosuppressive and immunomodulatory properties, vanish during the first months, leaving the infant exceedingly susceptible to infections (Elahi 2014).
    At the same time the passive protection by transplacentally transferred maternal antibodies, w...

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  • Yes, parental consent is important. But it is not the only factor.

    We thank Dr Neefjes for engaging so thoroughly with our research, which focuses on an important area of care that has a significant impact on parents, children and clinicians. We readily agree that early discussions about end-of-life care might be beneficial in giving parents more time to explore treatment options. Further research could explore whether earlier discussions would be acceptable and of benefit. In our research we found, unsurprisingly, that parents want the best for their children and that clinicians want to do the best for their patients. Reducing the chance for relationships to become ‘adversarial’ is in our view a good aim where possible. It is true that, if parents feel that they are no longer able to defend the best interests of their children, this loss of empowerment may precipitate conflict. However, it was apparent in our research that parents with whom we spoke did not think of their rights of consent in absolute terms. Instead they thought that their rights to consent were complex and not necessarily absolute in that they sometimes amounted to the power to agree to– or disagree with –a narrow range of options (including, in some cases, in relation to decisions to withdraw or withhold treatment). This is not to say that all other parents share this opinion (we lack evidence for such a general empirical claim), but more that the nuance in the way that these parents perceived consent may distinguish parental consent from consent in other populations....

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