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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.
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.
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
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...
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...
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 relationship between the two, nevertheless he has not claimed that bacterial and/or viral infective agents are the actual cause of the fatalities.
At present, the foregoing actually comes to down to applying the 'Law of excluded middle'. Either infections are the cause of the SIDs or they are not. If they are, then Dr. Goldwater and other fellow travellers are hopefully treading the royal road to success. If not, and applying what his review clearly implies, then infection has to be a concominant variant. In other words, whatever causes SIDS also is responsible for the presentation of many, if not all of the accompanying pathological 'markers' of the 'syndrome', and with particular reference to infections being the predominant consideration. That aspect would involve the identification of a cause of death which also carries with it the inability to protect the body again infection in 90% of the SIDS cases
In that regard, meriting consideration and available on PubMed is a free PMC article. Entering PMC2647905 will screen up the text.
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....
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. Ethically, we also see a distinction between the right of a person to consent on their own behalf and their right to consent on another’s behalf. Further, we suggest, there is a serious risk that the interests of children can in some cases be diminished or under-emphasised (for example, in disputes that last many months). The prohibitive costs of legal action, fear of further undermining relationships, and a lack of confidence in the courts may all contribute to a desire not to seek legal judgment. It is because of all these factors, rather than believing it is representative of parental wishes as such, that we suggest parental assent might have the potential to balance competing factors better than parental consent.
Future research is certainly needed given that assent itself is (so far) poorly conceptualised in the wider bioethical literature. In our view, a key attribute of any future development would be to ensure that ‘parental assent’ would not itself precipitate early disputes by undermining trust in the decision-making process. Currently, the vast majority of cases appear to be resolved sensitively, expeditiously and with due regard to the interests of both parents and children. Unfortunately, even where discussions between parents and clinicians are sensitive and take place at an early stage, in a small minority of cases this balance is lost and discussion stalls. While acknowledging the importance of building good relationships with early discussion, in these cases we consider the interests of both parents and children ultimately suffer. We suggest it is vitally important to seek to address– and hopefully avoid –this.
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...
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, which contribute to early defense against pathogenic organisms, decreases significantly (Waaijenborg 2013).
In consideration of that high complexity and numerous possibilities of interaction between various parts of this immune ontogeny it is comprehensible that, in case of inauspicious conditions, an overwhelming sepsis/septic shock, i.e. SIDS as the maximum credible accident might occur.
Thus, the infection model should not only be considered in further research “solving the tragic enigma of SIDS” (Goldwater 2017) but it also stresses the importance of all infection preventive recommendations, such as breast feeding, infant sleep location and exposures to smoking.
References:
American Academy of Pediatrics Task Force on Sudden Infant Death Syndrome. The changing concept of sudden infant death syndrome: diagnostic coding shifts, controversies regarding the sleeping environment, and new variables to consider in reducing risk. Pediatrics. 2005 Nov;116(5):1245-55.
TASK FORCE ON SUDDEN INFANT DEATH SYNDROME. SIDS and Other Sleep-Related Infant Deaths: Updated 2016 Recommendations for a Safe Infant Sleeping Environment. Pediatrics. 2016 Nov;138(5). pii: e20162938.
Basha S1, Surendran N, Pichichero M. Immune responses in neonates. Expert Rev Clin Immunol. 2014 Sep;10(9):1171-84.
Elahi S, Ertelt JM, Kinder JM, Jiang TT, Zhang X, Xin L, Chaturvedi V, Strong BS, Qualls JE, Steinbrecher KA, Kalfa TA, Shaaban AF, Way SS. Immunosuppressive CD71+ erythroid cells compromise neonatal host defence against infection. Nature. 2013 Dec 5;504(7478):158-62.
Elahi S. New insight into an old concept: role of immature erythroid cells in immune pathogenesis of neonatal infection. Front Immunol. 2014 Aug 12;5:376, 1-7.
Gensollen T, Iyer SS, Kasper DL, Blumberg RS. How colonization by microbiota in early life shapes the immune system. Science. 2016 Apr 29;352(6285):539-44
Goldwater PN. Infection: the neglected paradigm in SIDS research. Arch Dis Child. 2017 Aug;102(8):767-772.
Waaijenborg S, Hahne SJ, Mollema L, et al. Waning of maternal antibodies against measles, mumps, rubella, and varicella in communities with contrasting vaccination coverage. The Journal of infectious diseases. 2013; 208(1):10–16.
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. 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...
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, rather than January to December. Most forms of IMD, like many infectious diseases, display seasonal variation and reach a peak during the winter. Looking at epidemiological years means that every winter peak always falls within one year rather than being divided between two consecutive calendar years. ‘Unvaccinated’ cases (Table 1) had all routine vaccinations apart from Bexsero® (before September 2015). The ‘Vaccinated’ group includes those who ‘supposedly’ received Bexsero® (after September 2015). The assumption was made that all patients born from July 2015, received Bexsero®; their vaccination records were not checked. There are uncertainties about how effective it will be in protecting against invasive meningococcal disease3. Good active population-based sentinel surveillance would allow gathering important information also about the capability of the new vaccine to disrupt carrier chain and reach herd immunity.
REFERENCES
1.Frosi G, et al., Bactericidal antibody against a representative epidemiological meningococcal serogroup B panel confirms that MATS underestimates 4CMenB vaccine strain coverage. Vaccine, 2013.
2.Vogel, U., et al., Predicted strain coverage of a meningococcal multicomponent vaccine (4CMenB) in Europe: a qualitative and quantitative assessment. Lancet Infect Dis, 2013. 13(5): p. 416-25.
3.Andrews SM, Pollard AJ. A vaccine against serogroup B Neisseria meningitidis: dealing with uncertainty. The Lancet infectious diseases. 2014;14(5): 426–34. doi: 10.1016/S1473-3099(13)70341-4 [PubMed]
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.
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.
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...
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 MoreDr. 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:
Show More" 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...
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....
Show MoreIn 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).
Show MoreAt the same time the passive protection by transplacentally transferred maternal antibodies, w...
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. 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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