Mutational analysis of NOG in esophageal atresia and tracheoesophageal fistula patients.

Purpose: The NOG protein is a secretory antagonist of bone morphogenetic proteins (BMPs). Nog-/- mouse embryos demonstrate proximal esophageal atresia (EA) and distal tracheoesophageal fistula (TEF) compatible with the most common configuration of EA/TEF observed in humans. Four microdeletions that span the NOG locus at 17q22 have been described in human patients having EA/TEF.

Prospective, longitudinal evaluation of health related quality of life in the pediatric spina bifida population undergoing reconstructive urological surgery.

Purpose: Spina bifida, the most frequent permanently debilitating birth defect, results in major urological problems of voluntary bladder control and bowel function, which may impair quality of life. We prospectively assessed quality of life in patients with spina bifida using child and parent reports simultaneously. This study had 3 goals, that is to 1) document baseline health related quality of life in patients with spina bifida preoperatively, 2) study health related quality of life, reporting differences between parents and children, and 3) study changes in health related quality of life prospectively at preoperative and postoperative intervals.

Elective intestinal operations in infants and children without mechanical bowel preparation: a pilot study.

Background/purpose: Preoperative mechanical bowel preparation (MBP) for elective intestinal operations has been a long accepted practice. However, MBP is often unpleasant and time-consuming for patients, and clinical trials in adults have not shown improved outcomes. We conducted this pilot study to test whether omitting MBP before elective intestinal operations in infants and children would increase the risk of infectious or anastomotic complications.