Management of pediatric patients with refractory constipation who fail cecostomy.

Background: Antegrade continence enema (ACE) is a recognized therapeutic option in the management of pediatric refractory constipation. Data on the long-term outcome of patients who fail to improve after an ACE-procedure are lacking.

Purpose: To describe the rate of ACE bowel management failure in pediatric refractory constipation, and the management and long term outcome of these patients.

Relationship of proximal renal tubular dysgenesis and fetal liver injury in neonatal hemochromatosis.

Renal tubular dysgenesis has been reported in isolated cases of neonatal hemochromatosis (NH). We hypothesized that fetal liver injury in NH impairs proximal renal tubular development via impaired hepatic angiotensinogen (AGT) elaboration. Morphometric analyses were performed of postmortem liver and kidney sections of cases of proven NH and postconception age-matched controls for renal proximal tubule density, hepatocyte mass, and hepatic AGT expression.