Liver Transplantation and Development of Diabetes in an Adolescent Male With HNF1B Disease.

Mutations in the hepatocyte nuclear factor-1-beta () gene cause a variety of diseases in different organ systems. Mutations have been described as causing neonatal cholestasis, maturity-onset diabetes of the young (type 5), cortical renal cysts, urogenital abnormalities, liver dysfunction, and atrophy of the pancreas. We describe a male patient who presented with cholestatic liver disease in infancy which progressed by age 14 to end-stage liver disease due to HNF1B disease.

Monitoring enteral nutrition support tolerance in infants and children.

Enteral nutrition support is used extensively in the care of infants and children, both for acute and chronic conditions. Monitoring a child's tolerance of enteral feedings is an ongoing challenge. Monitoring routines vary significantly between institutions, practitioners, and patient settings, and a number of definitions are used for "intolerance.

Hand-assisted laparoscopic donor nephrectomy for pediatric kidney allograft recipients.

Laparoscopic donor nephrectomy (LDN) is the method of choice for procuring kidneys from living donors at many transplant centers. The aim of this study was to assess the feasibility as well as outcome of LDN in pediatric recipients. Twenty-two pediatric patients, 18-yr old or younger received kidneys procured by a hand-assisted LDN technique.