UIP Masthead

June 3, 2015   Vol. VI, Issue 22
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Phenotypic variability in childhood skeletal muscle sodium channelopathies

"Channelopathies are diseases caused by disturbed function of ion channel subunits or the proteins that regulate them". They may be congenital (from a mutation/s in the encoding gene) or acquired (from e.g. an "autoimmune attack on an ion channel"). There are a large number of clinical abnormalities known to be caused by channelopathies; many more presently under investigation. The SCN4A (called "sodium channel, voltage gated, type IV alpha subunit) belongs to the family of genes which transport sodium ions into cells and are found in skeletal muscle.


Mutations of the SCN4A gene (located on the long arm of chromosome 17 at position 23.3) causes several skeletal muscle abnormalities (e.g. hyper and hypokalemic periodic paralysis, paramyotonia congenita, potassium aggravated myotonia, one form of myasthenia gravis, etc). Variability in clinical presentation (phenotypic heterogeneity) may be confusing because of overlapping presentations in childhood. Treatment for some conditions exist.


Pediatric Neurology 

Physical and neurodevelopmental outcomes in children with single-ventricle circulation (hypoplastic left heart syndrome - HLHS)

A study of 58 patients with HLHS and 44 non-HLHS patients with single ventricle physiology surviving >10 years indicates that HLHS survivors have  greatly reduced exercise tolerance, more educational issues (41% vs. 23%), & increased behavioral abnormalities (autism or ADHD) (12% vs. 0%) requiring greater multidisciplinary team involvement.


Archives of Diseases in Childhood 

Asynchronous milk ejection in the  human lactating breast.

"Milk production is under the influence of autocrine control" - with a rate of synthesis decreasing as the breast fills with milk. It has been assumed that milk ejection occurs in all alveoli simultaneously; though animal studies indicate that full alveoli eject milk earlier than less filled ones.


A study using ultrasound videos of milk duct flow during pumping indicates that flow from the two separate main milk ducts differ substantially (by 2-8 seconds) and the timing of myoepithelial cell response differs resulting in heterogeneity of alveolar milk emptying.


Journal of Human lactation 



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Factors predictive of second recurrence of clubfoot treated by Ponseti casting


The Ponseti treatment a standard painless, fast, successful and cost-effective method of managing clubfoot, (repopularized in the 2000's ; originally described in the 1950's) uses gentle corrective mechanical manipulation of soft tissues (held in position by a plaster cast after each manipulation) to gradually stretch and reduce the deformity towards normal. Recurrence is common and tibialis anterior tendon transfer (TATT) is

Club Foot
Club Foot

frequently used to correct this. Recurrence after TATT may also occur (secondary recurrences).


In a retrospective review of 60 patients with 85 club feet, 20% developed recurrences after TATT. Young age and brace noncompliance are primarily responsible for secondary recurrences.


Journal of Pediatric Orthopaedics 

Excess weight gain, bronchiolitis and asthma in preschool children


A prospective study of 151 former bronchiolitic patients followed to age 6.45 years (mean) evaluated weights (BMI), height, asthma symptoms and medication for asthma, over time. 20% of infants became "overweight" and 6% "obese".


Asthma is more common in "overweight" than normal weight post-bronchiolitic preschool and early school age children (obesity is associated predominantly with "currently" asthmatic children).


Pediatric Pulmonology

Video Feature

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Hypoplastic left heart syndrome
Hypoplastic left heart syndrome

Safety and efficacy of Pimecrolimus in Atopic Dermatitis (AD)


Pimecrolimus (Elidel) is an immunomodulating agent available as a 1% cream (PIM) for the treatment of eczema. Although topical steroids (TCS's) are often prescribed for atopic dermatitis, compliance is often poor due to concerns over side effects.


An open label five year study of 2,418 infants with mild to moderate AD half randomized to receive either TCS's or PIM & where long term efficacy/safety were documented, indicates that:


1.   Both TCS and PIM have rapid onsets; >50% of patients achieving successful treatment by three weeks, with >95% and 85% respectively maintaining success for > 5yrs.

2.   Adverse events are similar for both treatments.

3.   Neither treatment demonstrates abnormalities in humoral or cellular immunity.

4.   PIM is steroid-sparing and may be valuable as a first-line treatment of mild to moderate AD.



Collaborative care for children with Attention-Deficit/Hyperactivity Disorder (ADHD) symptoms   


A randomized comparative study which evaluated "standard care management with decision support" to an  "enhanced care arm", where care managers were also trained in motivational and parent management techniques  to help parents engage in the child's treatment, address their own mental health needs and manage challenging child behaviors, indicates "enhanced arm" participants achieve superior management of hyperactivity/impulsivity, improved social skills and less oppositionality.



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