MCH Updates in Pediatrics Masthead  

        Volume IV
          Issue 16
                                                                         South Florida's  only licensed  free-standing
                                                                           specialty hospital  exclusively for children ...                                                              April 17, 2013 
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Outcomes of congenital esophageal atresia (OA) repair

OA, with or without an associated trachea-oesophageal fistula is the most common esophageal malformation, occurring in 1 in 2000-5000 live births.

 

An 8.8 year follow up review of 130 consecutive infants with OA indicates that a majority (87%) had a primary end-to-end anastomotic repair with esophageal continuity occurring in all patients and 94% of them successfully orally fed. Overall mortality was 2%.

 

Alagille Syndrome and Craniosynostosis  

Alagille Syndrome is most frequently (70%) caused by an autosomal mutation of the JAGGED 1 gene on

chromosome 20p2.  

Clinically, children present with growth retardation, characteristic facies, (broad forehead, widely spaced eyes and a straight nose), vertebral defects ("butterfly vertebrae"), ocular and cardiac abnormalities and chronic obstructive jaundice associated with a paucity of bile ducts. The JAG1 gene appears to participate in cranial suture formation.

 

A report of a fairly uncommon presentation of a child with Alagille syndrome who presented with Craniosynostosis and possible raised intracranial pressure, pruritus and abnormal liver enzymes, reminds all of the associated features of this genetic abnormality.


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Obesity, non-alcoholic liver disease (NAFLD) and atherosclerosis in children  

 

NAFLD was diagnosed (by ultrasound & exclusion of infection & metabolic causes) and utilized in 157 obese children (mean age 11.3 yrs.) to document carotid intimal thickness (c-IMT) and liver enzymes, and compare them to a matched non-obese group.

 

Obese children and adolescents with NAFLD have significant thickening of their carotid artery placing them at risk for the early development of atherosclerosis. As liver enzymes may or may not be elevated in these children, a liver ultrasound should be considered to diagnose NAFLD in all obese children/adolescents.


Acta Paediatrica  

Video Feature
New gene sites discovered affecting nonalcoholic fatty liver disease (NAFLD)
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New gene sites discovered affecting nonalcoholic fatty liver disease (NAFLD)
University of Michigan Health System  via YouTube

Exclusively breast fed infants and vitamin D status

 

WHO policy recommends that all infants be exclusively breast fed until 6 months of age. As New Zealand has no policy on daily Vit. D supplementation to prevent rickets, a study of breast fed infants at 2-3 months of age which measured serum 25-hydroxy-Vitamin D (25(OH)D), was undertaken.

 

Vit. D deficiency is prevalent in exclusively breast fed infants who do not receive daily supplementation of 400 IU of vitamin D.

 

Diagnosing cerebral shunt malfunctioning by cerebral arterial oxygen saturation      

 

Cerebral regional oxygen saturation (rSO2) using near infra-red spectroscopy represents tissue perfusion and oxygen saturation. 131 malfunctioning shunt subjects with raised intracranial pressure whose rSO2's were measured indicates increased variability of regional cerebral rSO2 readings with shunt malfunction.

 

The American Journal of Emergency Medicine 

Scoliosis in patients with Fontan circulation  

 

The Fontan-type operation is utilized in the definitive management of infants with congenital single-ventricle hearts.

 

A retrospective follow up study of 194 patients (median age: 1.3 years) who had Fontan surgery and who 3.4 years later had  

reviewable spinal radiographs, indicates 9.8% of them develop scoliosis with a female to male ratio 5:3. The majority of patients have the major curve to the right. Scoliosis risk increases with each year of age.

  

Archives of Disease in Childhhood 

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