Updates in Pediatrics  


May 20, 2015   Vol. VI, Issue 20
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Could serum pentraxin 3 levels (PTX3) and IgM deposition in skin biopsies predict subsequent renal involvement in children with Henoch-Sch?nlein purpura (HSP)?

PTX3 is a new protein belonging to the family which includes C-reactive protein (CRP) and is a new biomarker for inflammatory vascular disease.

 

HSP is a systemic vasculitis characterized by deposition of immune complexes which present clinically with purpura etc. A small proportion of affected children develop chronic kidney disease.

 

A prospective cohort study of 60 children with confirmed HSP examined initial blood samples, PTX3 and skin biopsies, and the presence 18 months later of renal involvement.

 

HSP children who subsequently develop renal involvement (48.3%) have significantly higher serum PTX3 levels and more immunoglobulin A (IgA) and IgM deposition in skin biopsies.

 

Pediatric Nephrology 

Update in SIDS risk reduction - The Swedish experience  

"Sweden for the past decade has had an enviably low SIDS rate". In most high-income countries, however while SIDS rates have declined there has been an increase in the risk of other sudden and unexpected infant deaths such as "ill-defined deaths", and "death attributable to accidental suffocation and strangulation in bed". While other factors playing a role in reducing in SIDS death rates appear well established the issue of bed sharing in low risk situations remains somewhat controversial.

 

Though accidental infant deaths are higher for bed sharing, given the present state of knowledge it appears prudent to suggest that in addition to minimizing all other risk factors, infants <3 months of age should sleep in a separate sleep place than their parents, though in the same room and within sight, sound and touch of the adjacent patent's bed.

 

Acta Paediatrica 

Acute flaccid paralysis, cranial nerve dysfunction and enterovirus D68

A cluster outbreak of 12 children (median age 11.5 years) presented with a prodromal respiratory febrile illness followed 7 days later by an acute flaccid paralysis or acute cranial nerve (VI and VII) dysfunction with brain stem lesions on imaging on investigation 10 of 11 children had positive nasopharyngeal specimens for rhinovirus or enterovirus D68 (45%). Children with limb weakness have residual defects: cranial nerve function may improve.

 

Enterovirus D68 infection may be associated with significant neurological deficits.

 

The Lancet

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Lowe Syndrome (OCRL - "oculocerebrorenal").

 

Lowe Syndrome (described in 1952) is a rare X-linked multisystem disorder characterized by bilateral congenital cataracts (glaucoma in 50% of cases), hypotonia and areflexia, mental retardation and proximal tubular acidosis etc. (Fanconi-type renal tubular dysfunction).

 

OCRL has a major mutational defect (deletion of the OCRL gene), together with other abnormalities in several flanking patterns of nucleic acids (repeat elements) which results in a clinical feature continuum broader than previously described (e.g. with advanced kidney disease, thrombocytopenia, hypersomnia and/or hyperacusis).

 

Pediatric Nephrology 

Video Feature 

(via YouTube)
Henoch-Sch?nlein Purpura
Henoch-Sch?nlein Purpura

Recurrent infections? - Think also of a Leukocyte adhesion defect (LAD).

 

"LAD is a rear autosomal recessive disorder characterized by immunodeficiency resulting in recurrent infections". Three subtypes are described; LAD type III in addition to the adhesion defect presents with a Glanzmann-type thrombocytosis (abnormality associated with failure of platelet aggregation).

 

The only definitive treatment of LAD III is hemopoietic stem cell transplantation which while correcting the underlying defect, particularly if applied early, appears to have a high complication rate.

 

Journal of Pediatric Hematology/Oncology

Eating habits, bed time and sleep timing in primary school children and obesity.

 

"Sleep patterns have been associated with unhealthy eating habits and energy intake".

 

A study 236 children in which physical measurements and body composition, cardio respiratory and musculoskeletal fitness tests, eating habits and child bedtime and waking hours were noted, concludes:

 

1.   There is an association between waist circumference and fat mass in late sleepers.

2.   That bedtime and sleep timing appear to be associated with eating habits in primary school children.

3.   The above factors may be important when evaluating obese children.

 

Journal Developmental and Behavioral Pediatrics

Patterns of bruising in preschool children 


A prospective longitudinal study of 328 children (<6 years) noted weekly the number and location of bruising over a 12 week period. Data suggests:

 

1.   The most common sites of bruising in all age groups are below the knee, followed by "facial T" and head in pre-mobile

Obesity and Sleep Patterns
Obesity and Sleep Patterns

and early mobile infants.

2.   Ears, neck, buttocks, genitals and hands are rarely bruised (<1%).

3.   Neither gender, season or level of social deprivation are associated with significant bruising patterns (siblings however have more bruises!)

 

Archives of Diseases in Childhood 


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