Sirs,
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.
References
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 Dr Eckhard Korsch’s letter: SIDS as the consequence of an immunological burst
I thank Dr Korsch for his supportive and helpful comments with which I fully concur. I remain concerned by the general lack of appreciation by mainstream SIDS researchers of the essential requirement of congruency between risk factors (male gender, prone sleep position, contaminated sleeping surfaces, smoke exposure, lack of breast feeding, high birth order, etc.) and various staining findings of brainstem nuclei or other pathological findings such as intrathoracic petechiae. The silence from the mainstream sector in relation to my ideas is also of concern. Funding of mainstream SIDS research will continue unimpeded as long as facts set out in my papers are not publicised and addressed. Such funding is an unconscionable waste.
In a new paper accepted for publication in Frontiers in Pediatrics1 I have extended the infection paradigm to take into account the as yet unexplained risk factor of prone sleep position. It seems that only in the presence of infection does prone sleep position achieve significance. My thinking suggests a role for the Vagus in unfavourably tipping the homeostatic balance through neuroimmunological pathways.
Paul N. Goldwater
Reference:
1. Goldwater PN. SIDS, infection, prone sleep position & Vagal neuroimmunology. Front Pediatr 2017; doi:10.3389/fped.2017.00223
I thank Mr O’Hagan for his insightful comments. Indeed it is implicit in the ideas put forward in my paper that a specific infection is not the “cause” of SIDS but, rather, it is the immunological response to the infection (bacterial or viral) in the predisposed infant that results in SIDS as proposed by Dr Korsch with his suggested “immunological burst.”
As mentioned in my response to Dr Korsch, I have extended the infection paradigm to take into account the as yet unexplained risk factor of prone sleep position. (See my paper accepted for publication in Frontiers in Pediatrics.1) The presence of infection is a requirement for the effect of prone sleep position to prevail and suggests a role for the Vagus in unfavourably tipping the homeostatic balance through neuroimmunological pathways.
Paul N. Goldwater
Reference:
1. Goldwater PN. SIDS, infection, prone sleep position & Vagal neuroimmunology. Front Pediatr 2017; doi:10.3389/fped.2017.00223
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...
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 that affect adults and children, the latter with a markedly heterogeneous range of seizure types, syndromes, causes, treatments and outcomes, all of which can be used to provide an individualised care plan. Failure to do so in certain situations might constitute discrimination against children with an epilepsy.
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...
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 karyotypes from PPB tumours usually contain excessive material from chromosome 8, and an increased incidence of p53 mutations. It has been suggested these mutations differentiate simple CPAM from cases that will later undergo malignant transformation.(3)
We therefore felt it was valuable to report that malignancy does occur in this group of patients unexpectedly, with significant consequences. There may also be a role for testing genetic aspects of CPAMs; some data suggests p53 mutations within CPAMs may be a useful indicator of risk of PPB.
1. Cook J, Chitty L, De Coppi P, Ashworth M, Wallis C. The natural history of prenatally diagnosed congenital cystic lung lesions: long-term follow-up of 119 cases. Arch Dis Child 2017;102:798–803.
2. Congenital pulmonary airway malformation and sequestration: Two standpoints for a single condition. Fievet L, Natale C, D’Journo X, Coze S, Dubus J, Guys J, Thomas P, De Lagausie P. J Minim Access Surg. 2015; 11(2): 129–133.
3. Vargas S, Korpershoek E, Kozakewich H, de Krijger R, Fletcher J, Perez-Atayde A. Cytogenetic and p53 profiles in congenital cystic adenomatoid malformation: insights into its relationship with pleuropulmonary blastoma. Pediatr Dev Pathol. 2006;9(3):190-195
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...
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 to remain aware of the ongoing risks of this disease and benefits of immunisation against this potentially fatal bacterium.
References
1. Nainani V, Galal U, Buttery J, Snape MD. An increase in accident and emergency presentations for adverse events following immunisation after introduction of the group B meningococcal vaccine: an observational study. Arch Dis Child. 2017.
2. Parikh SR, Andrews NJ, Beebeejaun K, Campbell H, Ribeiro S, Ward C, et al. Effectiveness and impact of a reduced infant schedule of 4CMenB vaccine against group B meningococcal disease in England: a national observational cohort study. Lancet. 2016;388(10061):2775-82.
3. Public Health England. Meningococcal disease: laboratory confirmed cases in England. [Online]. Cited 7th September 2017. Available at: https://www.gov.uk/government/publications/meningococcal-disease-laborat...
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...
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 cognate (albeit more conservative with respect to children) with the recommendations of Royal Life Saving Society – Australia and other Australian water safety organisations (Table 1). We acknowledge that our advice, concerning therapeutic levels of medication and the duration of seizure-free periods, is arbitrary. It reflects our collective experience of seven decades of drowning research, extensive aquatic involvement and participation in national and international formulation of safety doctrines. We also acknowledge that children with persistent febrile convulsions may have lower risks when taken for recreational swimming; but at this stage of knowledge we opine that heightened surveillance is nevertheless required. We agree that there are differential risks associated with different epileptic conditions and pre-existent syndromes. Judgement concerning such risks must reside with a child’s treating physician, as every child is different.
Organisation and Title
Policy reference to epilepsy
Royal Life Saving Society - Australia Epilepsy Medical Policy (2014)
The general rule is that persons suffering from epilepsy are medically eligible for all society awards, patrol duties and aquatic competition provided they have been free of seizures for two years. This is irrespective of whether medications are being taken or not. Where the seizure is the result of any of the following, a further six months must elapse without seizure before water activities can be resumed: a) medication omitted; (b) inadequate sleep; or (c) physical exhaustion. Where a seizure has occurred following withdrawal of medication on medical advice a minimum period of one month must elapse before water activities can be resumed. Where a seizure is the result of; alcohol abuse, head injury or brain surgery water activity may not be resumed for a further two years.
Guidelines (SU6) for Safe Pool Operation, RLSSA (2013)
Qualified lifeguards with a diagnosis of epilepsy are eligible to participate in lifeguard activity provided they have the approval of their medical attendant who is fully aware of the nature and duties of pool lifeguarding. The responsibility for a lifeguard’s management of their epilepsy at all times rests with them and their medical attendant.
Australasian Council for the Teaching of Swimming and Water Safety (AUSTSWIM) Teachers of Aquatics - Access and Inclusion Manual (2013)
A person with epilepsy should be under the care of a doctor and have their condition managed with medication. If this is the case, there is no reason why they should not participate in aquatic activity. Cold water conditions and high-intensity activities should be avoided. Long periods of immersion should also be limited. It is recommended an ‘observer’ be present to watch for signs of a pending episode and assist in the event of an epileptic fit.
Surf Life Saving Australia Seizures and Epilepsy Policy (2012)
Individuals diagnosed with a seizure disorder, child or adult, should not swim, surf, or paddle on craft at a beach; unless they have been free of seizures for at least one year. They should not swim, surf or paddle on craft for at least three months after cessation of medications. Where the seizure is the result of a clear precipitating cause that can be remediated, a further six months must elapse without seizure before swimming, surfing, craft paddling is resumed. Individuals with a seizure disorder should not swim, surf or paddle alone. Companions should be aware of the potential for seizures and the possible need for rescue. They should never hyperventilate for any reason and this is especially important prior to swimming and diving.
International Life Saving Federation MPS 17 – Seizures and Epilepsy Risks for Bathing, Swimming, Lifesaving and Lifeguarding (2016) 4
Bathtub use for children with epilepsy should have direct, close (in the room), and continuous supervision. Older children and adolescents with epilepsy should not bathe or shower with the bathroom door locked.
Epilepsy carries increased relative risk of submersion and drowning. Children and adults should be advised of this increased relative risk. Individuals with unstable, or potentially unstable epilepsy should avoid water activity until stability is re-established.
Epilepsy submersion and drowning risk is greatest in an identified high-risk group that includes: those with frequent (more than one per year) seizures; - those with unpredictable convulsive seizures; and/or those who have other disabilities.
Extra precautions and attention are warranted. This patient group should avoid water activities, or should participate in clear, shallow, still water, with a (securely fastened) personal flotation device that will support an unconscious person, and they should be within arm’s length of a capable support person.
Individuals with stable, controlled epilepsy and no other risk factors, who participate in recreational, instructional, and competitive water activities, should do so in supervised areas and with another capable person. Individuals with chronic disabilities who are intellectually and neurologically able, and medically stable should be encouraged to undertake swimming and lifesaving training.
References
1. Appleton RE. Seizures, safety and submersion: sense and sensibility. Archives of Disease in Childhood 2017;102:883-884.
2. Pearn, J. H., & Franklin, R. C. (2013). Disability and Drowning: Personal Experiences, Research, and Practicalities of Adapted Aquatics. International Journal of Aquatic Research & Education, 7(2). 157-162
3. Franklin RC, Pearn JH, Peden AE. Drowning fatalities in childhood: the role of pre-existing medical conditions. Archives of Disease in Childhood 2017;102:888-893.
4. International Life Saving Federation (2016) Medical Position Statement – MPS 17: Seizures and Epilepsy Risks for Bathing, Swimming, Lifesaving and Lifeguarding. Accessed via: http://www.ilsf.org/about/position-statements
Hello,
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.
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...
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 and neuro-disability assessments, including autism, but also provide medical services related to child protection and child abuse; children in care of the state (“looked after children”), adoption panels, child death inquiries, statutory advice for children with special educational needs (currently Education and Health Care Plan), special schools and other specialist areas which are developed locally. The UK community paediatrician cannot be accessed on demand and at short notice in the same way as a UK GP or an Israeli primary paediatrician are.
Therefore, the title of the article “Community paediatrics in Israel” actually refers to a system of primary care for children and young people, with on-demand access for parents or children, rather than to a specialist second-tier service. However the changes described in the article refer to expansion of the existing training to incorporate additional expertise in topics such as mental health, behaviour and ADHD, which are increasing in prevalence and demand for service (in Israel as in the UK).
Another term in the article which might cause confusion is “resident” or residency, which in the UK would be equivalent to “registrar”.
The first paragraph in the article ends in a statement that “management of paediatric problems in the community (Israel) is done by paediatricians who had not completed subspecialty training”. This actually means that they are fully trained consultant paediatricians, but are not necessarily certified in a subspecialty, such as paediatric respiratory, neonatology, neurology or haematology, etc.
Whereas in the UK not all paediatricians are at consultant (specialist) grade (i.e. “non career grade paediatricians”), in Israel the only non-consultant grade paediatricians working in hospitals or primary care are those who are in paediatric training (registrars by UK terminology , or “residents” in USA terminology), which culminates in achieving status of specialist /consultant.
Paediatricians in the UK are usually either “community, “acute” (hospital general paediatricians), or specialist in paediatric sub-specialities (mostly based in tertiary level hospital settings).
In Israel many paediatric subspecialties are available both in hospitals and in the community, some of which require a referral from the primary paediatrician, but others can be accessed directly by parent initiative (for example paediatric orthopaedic surgeons or neurologists).
All people permanently residing in Israeli (I have avoided the word “resident” here) are covered by national health insurance, however, as opposed to the single system (NHS) operating in the UK, in Israel four competing public health organisations (Health Funds) operate, delivering a basic universal “basket of services” , and competing with each other by offering increasing levels of excellence or diversity of additional services.
This comment will, I hope, clarify some of the information presented in the article by Porter et al.
Dr Naomi Gerson-Sofer
Consultant Community Paediatrician
Oxleas Foundation Trust
(Formerly Director of Child Development Centre in the Western Galilee Hospital, Israel)
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.
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 Wales, while cases were
multi-centre. This could give rise to inter-centre variability, which
needs to be looked at and accounted for.
2) Validation of training of carers was done on children with non-
bleeding disorders only. This should have been attempted on children with
bleeding disorders as well, as their reporting form the basis of this
study.
3) While sexes were balanced in children with non-bleeding disorders,
children with bleeding disorders were dominated by males. Differences in
type or duration of activity could be a confounding factor in comparing
these groups. While this is difficult to correct without introducing
further bias, this needs to be considered as a factor for adjustment in
models, if possible.
Sirs,
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.
References
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 Dr Eckhard Korsch’s letter: SIDS as the consequence of an immunological burst
I thank Dr Korsch for his supportive and helpful comments with which I fully concur. I remain concerned by the general lack of appreciation by mainstream SIDS researchers of the essential requirement of congruency between risk factors (male gender, prone sleep position, contaminated sleeping surfaces, smoke exposure, lack of breast feeding, high birth order, etc.) and various staining findings of brainstem nuclei or other pathological findings such as intrathoracic petechiae. The silence from the mainstream sector in relation to my ideas is also of concern. Funding of mainstream SIDS research will continue unimpeded as long as facts set out in my papers are not publicised and addressed. Such funding is an unconscionable waste.
In a new paper accepted for publication in Frontiers in Pediatrics1 I have extended the infection paradigm to take into account the as yet unexplained risk factor of prone sleep position. It seems that only in the presence of infection does prone sleep position achieve significance. My thinking suggests a role for the Vagus in unfavourably tipping the homeostatic balance through neuroimmunological pathways.
Paul N. Goldwater
Reference:
1. Goldwater PN. SIDS, infection, prone sleep position & Vagal neuroimmunology. Front Pediatr 2017; doi:10.3389/fped.2017.00223
Conflict of interest
None declared.
Response to Edward J O’Hagan: Tertium non datur
I thank Mr O’Hagan for his insightful comments. Indeed it is implicit in the ideas put forward in my paper that a specific infection is not the “cause” of SIDS but, rather, it is the immunological response to the infection (bacterial or viral) in the predisposed infant that results in SIDS as proposed by Dr Korsch with his suggested “immunological burst.”
As mentioned in my response to Dr Korsch, I have extended the infection paradigm to take into account the as yet unexplained risk factor of prone sleep position. (See my paper accepted for publication in Frontiers in Pediatrics.1) The presence of infection is a requirement for the effect of prone sleep position to prevail and suggests a role for the Vagus in unfavourably tipping the homeostatic balance through neuroimmunological pathways.
Paul N. Goldwater
Reference:
1. Goldwater PN. SIDS, infection, prone sleep position & Vagal neuroimmunology. Front Pediatr 2017; doi:10.3389/fped.2017.00223
Conflict of interest
None declared.
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...
Show MoreWe 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.
Show MoreA 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...
Nainani V, Gulal U, Buttery J, Snape MD
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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...
Show MoreThe 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...
Show MoreHello,
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.
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...
Show MoreDear 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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