Oct. 19, 2016
Volume VII | Issue No. 42

Quality of life in Moyamoya disease (MMD)
MMD is a progressive cerebrovascular occlusive disease of the bilateral internal carotid arteries that leads to a compensatory abnormal vascular network at the base of the brain" ("puff of smoke"). While relatively uncommon in the USA it occurs frequently in the Far East (Japanese and Korean families). Its pediatric distribution peak occurs around 5 years of age (40 years in adults) and most children present with Transient Ischemic Attacks (TIAs) or cerebral infarction (frequently with headache and involuntary movements). Etiology is unknown and treatment is usually surgical.

A cross-sectional study of 30 children with MMD (67% presenting with stroke) indicates that even in the absence of stroke (and/or with mild symptoms) children with MMD have a lower quality of life than healthy controls and would benefit from psychosocial support.

Salivary streptococcus mutants (MS) and caries progression 
A retrospective cohort study of 200 preschoolers (mean age 3.3 years) followed for >5 years indicates that the presence of very high counts of MS (TNTC-"too numerous to count") at first dental visit increases 8 fold the risk of severity/having carious teeth, and further in spite of preventive dental care, results in having a 6 times greater chance of having cavities over the following 5 years.
Cognitive status and co-morbidities in teenagers with asthma
It appears that there is a significant positive correlation between higher cognitive score and prevalence of asthma in teenagers. Co-morbidities include; chronic rhinitis (35%); atopic dermatitis (2%); urticaria (1%); anaphylaxis (0.4%); chronic sinusitis (0.4%); overall overweight/underweight (20% and 3.0% respectively); irritable bowel disease (1%); and thyroid disorder (0.4%).

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Neurological follow-up of children treated for congenital heart disease
Cardiologists and cardiac surgeons continue to prolong the lives of children born with congenital heart disease (CHD). Recently (Circulation August 28, 2012) the American Heart Association (AHA), in response to the recognition of neurodevelopmental deficits in these patients, issued management algorithm guidelines to assist pediatricians in their formal periodic ongoing neurodevelopmental management of these children.

While the AHA guidelines for CHD follow-up encompasses surveillance, screening, evaluation, reevaluation and management of developmental disorders or disabilities, it appears that many pediatricians are unaware of this tool to screen and refer children who could potentially benefit from testing and/or intervention.

Video Feature
Moya Moya disease
Moya Moya disease
Prenatal maternal smoking and the risk of Tourette's syndrome (TS) and Chronic tic disorders (TS/CT)

Tourette's syndrome (George Gilles De La Tourette 1857-1904) is an inherited (though environmental factors are thought to play a role) neuropsychiatric disorder occurring in children (usually between 5-18 years of age) characterized by multiple (2 or more) motor tics (which wax and wane, can be suppressed temporarily and are preceded by an "urge") and at least one vocal outburst. It is part of a spectrum of tic disorders; chronic tic disorders differ from each other in terms of age of presentation, the type of tic present and how long the symptoms last.

It appears from an analysis of 73,073 singleton pregnancies that prenatal maternal smoking is associated with an increased risk of TS/CT, with or without comorbid psychiatric disorders.
Journal of the American Academy of Child and Adolescent Psychiatry 
Maternal and infant Secretory Immunoglobulin A (IgA) across the Peripartum period 

IgA is an antibody that plays a critical role in immune function in the mucous membranes". IgA has 2 subclasses (IgA1 and IgA2) and can exist in a dimeric form (a molecule composed of 2 identical simpler molecules) called secretory IgA (sIgA). Protected from proteolytic enzymes this is the main
immunoglobulin found in tears, saliva, sweat, colostrum and secretions from the genitourinary tract, gastrointestinal tract, prostate and respiratory epithelium.

A study of 51 mother-infant dyads which collected salivary sIgA from the mother in the third trimester and infant and maternal salivary, and maternal breast milk sIgA at 1, 3 and 6 months post-partum, indicates that while maternal salivary sIgA is stable, breast milk and infant sIgA decreases from 1 to 3 months post-partum.

Phalangeal neck fractures; "Buddy taping with a short-arm splint" vs. operative treatment

Phalangeal neck (the transition area between body and head of the phalanx) fractures are uncommon, occur almost exclusively in children, are difficult to treat and cause concern primarily because they are unstable and have a high risk of complications.

From a study of 32 patients with phalangeal neck fractures (<12 years of age) who either underwent reduction and conservative (buddy taping and short-arm splint) or surgical treatment, it appears that conservative management gives as good a result as surgery.

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