Sept. 6, 2017
Volume VIII |  Issue No. 36

Inattention and poor eye contact is not all Autism
Autism Spectrum Disorders (ASD's) have three types of clinical symptoms (deficits in social communication, social interaction and restricted repetitive patterns of behavior or activity).

"Absence seizures" (previously called "petit mal" seizures) are a type of brief (few seconds) generalized seizure that causes short periods of lapses in awareness (sometimes with staring - "blanking out" and frequently mistaken in children for daydreaming).

Absence seizures may be difficult to diagnose in children with ADS, and are rarely reported. Two case presentations illustrate that stereotypic and inattentive behavior considered to be part of ASD may in fact be absence seizures. An EEG should be routine in all children diagnosed with ASD.

Vascular endothelial growth factors A (VEGF-A) and congenital renal lesions in children with urinary tract infections
VEGF-A (a gene located on chromosome 6) has a wide spectrum of molecular functions which impact biological processes (e.g. vasculogenesis, cell maturation, outflow tract morphogenesis, mammary areolar development, etc.).
A study examined (VEGF-A)-460 C/T functional gene polymorphisms (different forms of a single gene) and renal abnormalities in 76 children with their first urinary tract infection (UTI) and 63 uninfected children.

In children with UTIs, the C-allele polymorphism of VEGF-A gene is associated with probable congenital hypodysplastic (small and malformed) kidneys. Differing VEGF genotypes or T alleles do not appear to be associated with urinary reflux or other urinary tract anomalies.

Risk factors for mortality before age 18 years in Cystic Fibrosis (CF)
A study examined a variety of risk factors at age 6-8 years for childhood death before 18 years in children diagnosed with CF.

Risk of death from CF (median age 13.4 years) identified at 6-8 years of age include: female sex, unknown CFTR genotype, minority race or ethnicity in the USA, low socioeconomic status, pseudomonas aeruginosa positivity (on >2 cultures), and weight <50 percentile.

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Celiac disease (CD) and Non-celiac sensitivity 

Celiac disease is a chronic, small intestinal immune-mediated enteropathy initiated by exposure to dietary gluten in genetically predisposed individuals and characterized by specific autoantibodies against tissue transglutaminase 2 (anti-IgA-(TG2), endomysium (EMA) and/or deamidated gliadin peptide (IgG DGP)". While many carry the genotype (HLA-DQ2 or HLA-DQ8) few (2-3%) develop CD. Affecting primarily young white children (any age and race) CD presents with gastrointestinal (GIT) signs or symptoms, extra-intestinal signs or symptoms, or both.

Non-celiac gluten sensitivity patients are those who have similar signs and/or symptoms with gluten ingestion with symptomatic improvement on its withdrawal. At present there are no biomarkers to aid in its diagnosis.

An excellent review article outlines the current evidence regarding screening, diagnosis and treatment of both CD and non-celiac gluten sensitivity.

See related video HERE>>
Video Feature
Absence Seizures
Absence Seizures
A decade of pediatric tracheostomies
A retrospective review over a ten year period of 426 children (median age 1.5 years) admitted to a pediatric Intensive Care Unit who underwent a tracheostomy for a variety of reasons indicates:
  1. 23% die (75th percentile survival time 5.9 years).
  2. Patients undergoing tracheotomy for airway obstruction are least likely to die (vs. those with acquired neurologic disease who are most likely to die).
  3. 50% are de-cannulated at 1-2 years.
  4. 62% remain cannulated with children with congenital neurologic disease least likely to be decannulated.
  5. >50% of tracheotomy patients are discharged with a tracheotomy in place on some form of mechanical support.
Body mass index (BMI) and Vigorous Physical Activity (VPA) in children and adolescents
Childhood obesity has steadily increased since the 1980s, and it is estimated that 170 million children (under the age of 18 years) worldwide are overweight. Obese children tend to become obese adults which results in an increased risk for diabetes and cardiovascular disease, stroke and several cancers.

Data from a multicenter, multi-country, multi-phase, cross-sectional self-reporting questionnaire study (from parents and adolescents) on height, weight and reported VPA of 214,706 children (ages 6-7 years) and 362,091 adolescents (13-14 years of age) was utilized to examine the relationship between calculated BMI and VPA.

Reported vigorous physical activity alone does not appear to decrease obesity in children or adolescents (in fact VPA may be associated with a higher BMI). Overweight/obesity is multifactorial in origin and changes in other variables in conjunction with VPA is required to manage the increased BMI seen in children and adolescents.

Wide variability in caloric density of expressed human milk
From an analysis of protein, carbohydrate and fat content plus calculated caloric density of human expressed breast milk from 64 samples (24 mothers) it appears that only 34% of samples contain 20kcal/oz. (average 17.9% with wide variability).
The presumption that human breast milk contains 20kcal/oz. may lead to under nutrition in "at risk" infants. Nutritional strategies to optimize caloric intake should be individualized.
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7th annual MCHF5K Run/Walk 
September 16, 2017 | Coral Gables City Hall

     Join our Miami Children's Health Foundation 5K Run/Walk and come together for the children of Nicklaus Children's Hospital. Our largest annual community event, the MCHF5K is designed to increase awareness of Nicklaus Children's pediatric subspecialties and showcase our cutting-edge research, work, talent and the dedication of our physicians and staff.
     Last year's top male runner overall was Joshua Estrada (16:22), and top female overall runner was Julie West (21:12). The top 'FUN'raising teams were Nico's Promise ($13,739) and Team Alberto ($11,007).
     The MCHF5K is the Foundation's largest annual community event, featuring several 'FUN'raising teams who raise awareness and funds for current and former patients of Nicklaus Children's Hospital. The event brought together participants from all over South Florida looking to make a difference.
To participate in the 7th Annual MCHF5K, visit