1531 e-Letters

  • Platelet transfusions in haemolytic uraemic syndrome

    I read with great interest the article entitled “Treatment and management of children with haemolytic uraemic syndrome” by Walsh and Johnson recently published in Archives of Disease in Childhood (1). In this review, the authors quoted a study performed by our team that investigated the effect of platelet transfusions in children with haemolytic uraemic syndrome (HUS) (2). The main finding of this study is that we did not find statistically significant evidence of worse disease in children who received platelets; however, the authors of the current review outlined that there was a trend towards prolonged need for dialysis among patients who received platelets. A close analysis of this point showed that transfused patients required dialysis for a median of 7 (2-22) days whereas those not transfused for 10 (2-30) days. Therefore, unlike their statement, comparison of these values (p = 0.08) shows a trend towards shorter duration of dialysis in the group receiving platelets. Despite this result, we still suggest that platelet transfusion should be minimized or avoided if possible in patients with HUS.


    1. Walsh PR, Johnson S. Treatment and management of children with haemolytic uraemic syndrome. Arch Dis Child 2017; Sep 12. [Epub ahead of print]
    2. Balestracci A, Martin SM, Toledo I, et al. Impact of platelet transfusions in children with post-diarrheal hemolytic uremic syndrome. Pediatr Nephrol 2013;28:919–25.

  • 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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  • 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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  • Tertium non datur

    Dr. Goldwater's review once again reflects a suberb understanding of SIDS and related phenomena. He consistently presents information to his readers in a most interesting and objectively accurate and well-written set of steps, which are typically precise, factual, and to the point. For example, he ends the review by observing as follows:
    " If multiple causes were involved, then it would be reasonable to expect a variety of pathological findings. This demonstrably is not the case. There is a fixed pattern to the vast majority of cases. The crux of the argument against broad polycausality of SIDS is the consistent pathological picture (usually in more than 90% of cases) "
    " In moving forward, SIDS researchers should be asking the following questions: (1) Does my hypothesis take into account the key pathological findings in SIDS? (2) Is my hypothesis congruent with the key epidemiological risk factors? (3) Does the hypothesis link questions (1) and (2) This review has shown that infection meets these questions appropriately and researchers in this area deserve acknowledgement and funding support. There remain gaps in our knowledge with regard to the infection model, but it is clear that other lines of research are not making the grade ...."
    Note that while Dr. Goldwater has provided a formidable case on behalf of supporting and funding infection related SIDS research, and also given that there unquestionably is a relationshi...

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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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  • 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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  • Policy reviews 5 years after valuable in holding to account

    Lemer has very usefully carried out a 5 year review of policy implementation. Policy is only as good as the receivers at the other end and these change frequently along with an ever changing political and economic landscape.Thus the exercise is valuable in not only taking stock but also reminding those in power of an independent review process with recommendations which should transcend governments. Sadly , the focus on funding education of the workforce (recommendations 10-12) do not appear to have been a priority and without this foundation, we will not move ahead sufficiently fast with a child and family friendly service. The Children and Young Persons Outcomes Framework is similarly the result of much work in a previous government and must not be allowed to whither on the vine. Perhaps we should regularly remind policy makers in the current administration of the value of persistence with other such initiatives which have a broad professional consensus and can be dusted off and re-badged as necessary to tempt politicians to move the goal posts a little closer to what is required to optimize child health? Lets see how far we have got in another 5 years.

  • Is Bexsero® (MenB vaccine) effective in preventing invasive meningococcal disease? Experience of a tertiary hospital in the UK

    Is Bexsero® (MenB vaccine) effective in preventing invasive meningococcal disease? Experience of a tertiary hospital in the UK. Novel meningococcus serogroup B vaccine (Bexsero®) was introduced in UK national immunisation programme on 1 September 2015. All babies born from July 2015 were offered the vaccine alongside other routine immunisations and all babies born in May 2015 were offered Bexsero® as a one-off catch-up. Bexsero® is estimated to protect against 73–88% of MenB strains causing invasive meningococcal disease (IMD) in England and Wales1,2. Among the diseases preventable by immunisation, IMD remains a high public profile illness deserving the most rigorous consideration because of its rapid and severe onset, high mortality rate and burden of sequelae. Epidemiological data suggest that infants in the first year of life experience the highest risk of infection peaking at around 5 months and declining thereafter. We continue to observe IMD in the first year of life despite the introduction of Bexsero® in our national immunisation programme (Table 1). This retrospective data was obtained as part of service evaluation at Central Manchester University Hospital Foundation Trust from our microbiology department. We are one of the biggest integrated Children's hospitals in the UK providing a wide range of services for the North West region and have over 220,000 patient visits each year. The epidemiological year starts from July to June, rath...

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  • A false equivalency between CFS/ME and CDF

    The title of the paper and the majority of the introduction imply that the study is about adolescents with CFS/ME. However, the final sentence of the introduction undermines that objective: “As children in our study were not examined by a physician, we have used the term ‘chronic disabling fatigue’ (CDF) rather than CFS/ME to indicate chronic fatigue that is disabling.”

    Those children may have had a variety of different diseases that cause prolonged fatigue, yet we are led to believe that a study of their collective conditions can somehow add to the body of literature on a specific disease process. CFS/ME is a highly contentious disease with a great deal of conflicting evidence and hypotheses; answers as to its exact nature and cause are as yet to be determined. By publishing a study of patients who are so poorly defined as to be undefined, Archives of Disease in Childhood has further muddied already murky waters. The addition of this study to the body of literature is not only unhelpful, but is actively detrimental to the pursuit of answers for patients with this highly disabling disease.

    How are ADC or the authors able to justify publishing a study that to all appearances is about CFS/ME, yet fails to properly assess if any of the study participants actually have CFS/ME?