My child also has CSID. She is about to be 8 yrs old and has been on
Sucraid for the last 6-7 yrs. I am looking for an alternative to this
medication as sometimes we cannot get the medication and have to go a
strict diet until we can get some again. I will look into this alternative
and post results later.
We read with interest the review by Amirthalingam[1] and colleagues
of the potential value of a UK varicella vaccination programme. They cite
Blumental[2] and colleagues' article in the same issue which assessed the
burden of varicella and outlined some of the known complications, such as
bacterial skin and soft tissue infections, pneumonia, and neurological
complications including meningitis and encephalitis. The Blumen...
We read with interest the review by Amirthalingam[1] and colleagues
of the potential value of a UK varicella vaccination programme. They cite
Blumental[2] and colleagues' article in the same issue which assessed the
burden of varicella and outlined some of the known complications, such as
bacterial skin and soft tissue infections, pneumonia, and neurological
complications including meningitis and encephalitis. The Blumental study
included complications occurring up to 21 days after onset of the
chickenpox rash. We wish to highlight further potential complications that
are possibly excluded from that definition but may be a significant
benefit of vaccination.
There is evidence that varicella infection is associated with an
increased risk of focal cerebral arteriopathy and arterial ischemic stroke
(AIS). In a UK study of incidence, Thomas[3] and colleagues identified 49
children with AIS following chickenpox and found that, in children, the
incidence of AIS during the following 6 months was four times that of
controls (summary incidence ratio = 4.07; 95% CI 1.96-8.45). The estimated
incidence ratio in adults was 2.13 (95% CI 1.05-4.36). Considering
varicella vaccination in a cohort of more than 3 million children, Donahue
and colleagues found no association with ischemic stroke.[4]
We believe that neurological complications of AIS, which can occur
many weeks after infection, were not considered in the review by
Amirthalingam and colleagues. Most of the complications that they reported
are transient or readily treatable, although meningoencephalitis can leave
long-term neurodevelopmental sequelae. There is a high risk of long-term
disability from childhood AIS and this places significant financial burden
on healthcare systems. We believe that this relatively uncommon but
important complication should be considered when determining the cost-
effectiveness of a varicella vaccination programme for the UK.
References
1- Amirthalingam G, Ramsay M. Should the UK introduce a universal
childhood varicella vaccination programme? Arch Dis Child. 2016
Jan;101(1):2-3.
2- Blumental S, Sabbe M, Lepage P; Belgian Group for Varicella.
Varicella paediatric hospitalisations in Belgium: a 1-year national
survey. Arch Dis Child. 2016 Jan;101(1):16-22
3- Thomas SL, Minassian C, Ganesan V, Langan SM, Smeeth L. Chickenpox
and risk of stroke: a self-controlled case series analysis. Clin Infect
Dis. 2014 Jan;58(1):61-8.
4- Donahue JG, Kieke BA, Yih WK, Berger NR, McCauley JS, Baggs J,
Zangwill KM, Baxter R, Eriksen EM, Glanz JM, Hambidge SJ, Klein NP, Lewis
EM, Marcy SM, Naleway AL, Nordin JD, Ray P, Belongia EA; Vaccine Safety
DataLink Team. Varicella vaccination and ischemic stroke in children: is
there an association? Pediatrics. 2009 Feb;123(2):e228-34.
The ADC Archivist recently reported that Freedman et al had revealed
that "old-fashioned clinical examination" missed about 20% of cases of
significant dehydration in children.[1] Their assessment of this work was
not surprising because the meta-analysis in the Journal of Pediatrics
carries the headline message that even the "most accurate, noninvasive"
methods could only "identify dehydration suboptimally", and it was a...
The ADC Archivist recently reported that Freedman et al had revealed
that "old-fashioned clinical examination" missed about 20% of cases of
significant dehydration in children.[1] Their assessment of this work was
not surprising because the meta-analysis in the Journal of Pediatrics
carries the headline message that even the "most accurate, noninvasive"
methods could only "identify dehydration suboptimally", and it was a high
quality analysis which only included studies that had accurately
quantified the degree of dehydration by serial weighings.[2] However,
Freedman et al's conclusions are misleading because they only selected
papers for analysis that had evaluated a rapid triaging tool, and none
which had undertaken standard full clinical examinations.
The four papers that qualified for Freedman et al's statistical
reanalysis had used the 'Clinical Dehydration' and 'Gorelick' scores to
detect dehydration secondary to gastroenteritis. The individual components
of these tests were not mentioned in their meta-analysis paper, but either
can be performed quickly on a fully-clothed infant in less than a minute.
They rely on scoring (a) the child's general appearance (seeking signs of
thirst, restlessness, lethargy and irritability, drowsiness, limpness,
cold, sweatiness, or coma), (b) whether the eyes look sunken, (c) if the
tongue feel moist, and (d) if tears are reduced or absent, all on simple
scales. They do not include any of the following components of routine
clinical examinations: capillary refill time, pulse rate and volume,
respiratory pattern, peripheral coolness, or skin turgor. As such, these
authors are not entitled to list their triage-type scoring as being the
"most accurate, noninvasive" clinical tests for dehydration. By presenting
their data as they did, Freedman et al may have produced a false-
impression among paediatricians about the sensitivity of full, careful
clinical examinations for evaluating fluid-balance status, and by
reviewing it as they did the ADC Archivist may have inadvertantly
perpetuated this confusion.
References
1. Archivist. Assessing dehydration. Archives of Diesease in
Childhood 2015;100:999.
2. Freedman SB, Vandermeer B, Milne A, Hartling L. Diagnosing clinically
significant dehydration in children with acute gastroenteritis using
noninvasive methods: a meta-analysis. Journal of Pediatrics 2015;166:908-
16.
Monika Bajaj and Amaka Offiah are to be commended for their
thoughtful and helpful review of the benefits and risks of skeletal
imaging in cases of suspected child abuse.(1) The diagnosis of child
abuse is a complex process which requires an evidence-informed approach
combining clinical acumen with collaborative multi-agency working.
Skeletal imaging, including CT scans, provide a valuable tool for the
clinician, but,...
Monika Bajaj and Amaka Offiah are to be commended for their
thoughtful and helpful review of the benefits and risks of skeletal
imaging in cases of suspected child abuse.(1) The diagnosis of child
abuse is a complex process which requires an evidence-informed approach
combining clinical acumen with collaborative multi-agency working.
Skeletal imaging, including CT scans, provide a valuable tool for the
clinician, but, as Bajaj and Offiah point out, is not without its risks.
The clinician must take a lead in informing the parents and other
professionals of the potential benefits of imaging, the inherent risks,
and the statutory responsibilities under which we work.
The concept of informed consent in such situations is problematic.
What reasonable parent will subject their child to a potentially harmful
procedure to rule out abuse which they 'know' has not happened?
Conversely, what reasonable parent, having abused their child, will
consent to a test which may help to prove that abuse? Parents must be
informed of the small but real risks associated with skeletal imaging and
that these need to be balanced against the clinical imperative to identify
or exclude injury and the statutory duty to investigate cases of possible
harm. Where parents do not give their consent to such imaging, the case
needs to be discussed with the multi-disciplinary team, and a decision
made as to whether to work with the increased uncertainty inherent in not
having a skeletal survey or CT scan, or whether to seek a court order to
obtain such investigations outwith parental consent.
Such decisions need to be made in the light of the known short- and
long-term harms caused by child abuse. These include a small risk of
fatality from severe physical abuse, and the much more prevalent ongoing
harm suffered by children living in contexts of ongoing physical or
emotional abuse and neglect. The risk of fatality, while clearly
significant, should not be overstated. Our current analysis of Serious
Case Reviews in England from 2009-14 suggests an average of 28-33 deaths
per year directly caused by child abuse (Sidebotham, unpublished data).
In their article, Bajaj and Offiah state that 'Data from Child Death
Reviews has identified "deliberately inflicted injury, abuse or neglect"
as the single largest category of childhood deaths with modifiable factors
in England.'(2) In fact, these data show that this is the category with
the highest proportion of deaths considered modifiable (65% compared to
20% overall). However, of the 784 child deaths for which child death
overview panels considered there to be modifiable factors present, only 28
(3.6%) were due to deliberately inflicted injury, abuse or neglect. This
compares to 185 sudden unexpected and unexplained deaths (24% of all
deaths with modifiable factors present); 178 deaths from perinatal or
neonatal events (23%); and 145 (18%) from trauma and other external
factors. Far from being the 'single largest category of childhood deaths
with modifiable factors', deaths from child abuse make up a very small
proportion of those child deaths which we, as a society, may be able to
prevent.
References
1. Bajaj M, Offiah AC. Imaging in suspected child abuse: necessity or
radiation hazard? Arch Dis Child. 2015;100(12):1163-8.
2. Department for Education. Child death reviews: year ending 31st March
2012. London: Department for Education, 2013.
This editorial is a very helpful review of the current state of the
debate.
I am concerned that zoster is under diagnosed in childhood because of
lack of familiarity in both primary and secondary care. Anecdotally it is
not uncommon in a paediatric unit, in otherwise well children, but does
cause significant concern and use of resources. This needs to be
accurately captured as it may shift the economic modelling...
This editorial is a very helpful review of the current state of the
debate.
I am concerned that zoster is under diagnosed in childhood because of
lack of familiarity in both primary and secondary care. Anecdotally it is
not uncommon in a paediatric unit, in otherwise well children, but does
cause significant concern and use of resources. This needs to be
accurately captured as it may shift the economic modelling used to decide
whether to go ahead with varicella vaccination.
In their excellent review on the hypoglycaemia in childhood the
authors suggest that for the management of the hyperinsulinaemic
hypoglycaemia (HH) diazoxide is the first-line therapy (1). Patients who
do not respond to diazoxide may respond to the octreotide but the efficacy
of this is often limited by tachyphylaxis. Mutations in ABCC8 and KCNJ11
are associated with severe HH that is unresponsive to...
In their excellent review on the hypoglycaemia in childhood the
authors suggest that for the management of the hyperinsulinaemic
hypoglycaemia (HH) diazoxide is the first-line therapy (1). Patients who
do not respond to diazoxide may respond to the octreotide but the efficacy
of this is often limited by tachyphylaxis. Mutations in ABCC8 and KCNJ11
are associated with severe HH that is unresponsive to medical treatment
with diazoxide and octreotide. The treatment option for patients with
medically unresponsive forms of diffuse HH is a subtotal pancreatectomy,
but some patients who have undergone surgery continue to have recurrent
hypoglycemia, whereas diabetes mellitus and exocrine pancreatic
insufficiency develop in others (1).
The authors of the review make no mention of the possible use of the
mammalian target of rapamycin (mTOR) inhibitor sirolimus recently reported
to be effective and safe for the severe, diffuse form of HH that had been
unresponsive to maximal doses of diazoxide and octreotide (2-5). No major
adverse reactions were observed during the 1-year follow-up period in the
first 4 cases described (2). This finding is reinforced by the latest
cases report of others infant (3,4) and older children (5).
The suggested method of action of sirolimus includes the reduction of
beta-cell proliferation, inhibition of insulin production, and induced
peripheral insulin resistance (4). The sirolimus-sensitive mTORC1 pathway
is present in the exocrine pancreas and the relatively sirolimus-resistant
IGF1R/mTORC2/Akt pathway is overexpressed in the beta-cells, thereby
suggesting that sirolimus is effective in treating diffuse HH by reducing
the transdifferentiation of exocrine elements to insulin-producing cells
(5).
Even if further studies are needed, primarily to identify long-term
sequelae and side effects, sirolimus appears to be a promising therapeutic
option to treat children with severe HH unresponsive to diazoxide and
octreotide.
Federico Marchetti, Vanna Graziani
Department of Paediatrics, S. Maria delle Croci Hospital, Ravenna,
Italy.
48121 Ravenna, Italy
e-mail: fedemarche@tin.it
References
1. Ghosh A, Banerjee I, Morris AAM. Recognition, assessment and
management of hypoglycaemia in childhood Arch Dis Child Published Online
First: 30 December 2015 doi:10.1136/archdischild-2015-308337
2. Senniappan S, Brown RE, Hussain K. Sirolimus in severe
hyperinsulinemic hypoglycemia. N Engl J Med 2014;370(25):2448-9.
3. Abraham MB, Shetty VB, Price G, et al. Efficacy and safety of
sirolimus in a neonate with persistent hypoglycaemia following near-total
pancreatectomy for hyperinsulinaemic hypoglycaemia. J Pediatr Endocrinol
Metab 2015;28(11-12):1391-8
4. Meder U, Bokodi G, Balogh L, et al. Severe hyperinsulinemic
hypoglycemia in a neonate: response to sirolimus therapy. Pediatrics.
2015;136(5):e1369-72
5. Minute M, Patti G, Tornese G, Faleschini E, Zuiani C, Ventura A.
Sirolimus therapy in congenital hyperinsulinism: a successful experience
beyond infancy. Pediatrics. 2015;136(5):e1373-6
My child also has CSID. She is about to be 8 yrs old and has been on Sucraid for the last 6-7 yrs. I am looking for an alternative to this medication as sometimes we cannot get the medication and have to go a strict diet until we can get some again. I will look into this alternative and post results later.
Conflict of Interest:
None declared
We read with interest the review by Amirthalingam[1] and colleagues of the potential value of a UK varicella vaccination programme. They cite Blumental[2] and colleagues' article in the same issue which assessed the burden of varicella and outlined some of the known complications, such as bacterial skin and soft tissue infections, pneumonia, and neurological complications including meningitis and encephalitis. The Blumen...
The ADC Archivist recently reported that Freedman et al had revealed that "old-fashioned clinical examination" missed about 20% of cases of significant dehydration in children.[1] Their assessment of this work was not surprising because the meta-analysis in the Journal of Pediatrics carries the headline message that even the "most accurate, noninvasive" methods could only "identify dehydration suboptimally", and it was a...
Monika Bajaj and Amaka Offiah are to be commended for their thoughtful and helpful review of the benefits and risks of skeletal imaging in cases of suspected child abuse.(1) The diagnosis of child abuse is a complex process which requires an evidence-informed approach combining clinical acumen with collaborative multi-agency working. Skeletal imaging, including CT scans, provide a valuable tool for the clinician, but,...
This editorial is a very helpful review of the current state of the debate.
I am concerned that zoster is under diagnosed in childhood because of lack of familiarity in both primary and secondary care. Anecdotally it is not uncommon in a paediatric unit, in otherwise well children, but does cause significant concern and use of resources. This needs to be accurately captured as it may shift the economic modelling...
Dear Editor
In their excellent review on the hypoglycaemia in childhood the authors suggest that for the management of the hyperinsulinaemic hypoglycaemia (HH) diazoxide is the first-line therapy (1). Patients who do not respond to diazoxide may respond to the octreotide but the efficacy of this is often limited by tachyphylaxis. Mutations in ABCC8 and KCNJ11 are associated with severe HH that is unresponsive to...
Pages