An enteric-coated high-buffered pancrelipase reduces steatorrhea in patients with cystic fibrosis: a prospective, randomized study.

Objective: Enteric-coated (EC) high-buffered (2.5 mEq [2.5 mmol] bicarbonate per capsule) pancrelipase microsphere enzymes were compared to EC-nonbuffered pancreatic enzymes for efficacy in reducing steatorrhea in patients with cystic fibrosis.

Effects of early cholesterol intake on cholesterol biosynthesis and plasma lipids among infants until 18 months of age.

Background: The endogenous cholesterol fractional synthesis rate (FSR) is related inversely to infant dietary cholesterol at 4 months of age; however, it remains to be established whether this effect is permanent, possibly contributing to later hypercholesterolemia.

Objective: To determine whether levels of dietary cholesterol in infancy induced changes in FSR and plasma lipid levels that persisted at 18 months.

Methods: A prospective clinical trial was conducted with 47 infants, from their first week of life until 18 months of age, who received human milk (HM) until weaned (n = 15) or were randomized to receive modified cow's milk formula (MCF) with added cholesterol (n = 15) or cow's milk formula (CF) (n = 17) for 12 months.

Impact of liver transplantation on HRQOL in children less than 5 years old.

Our primary goal was to assess health related quality of life (HRQOL) at transplantation and 1 yr after transplantation in pediatric liver transplant patients aged less than 5 years. We conducted a prospective longitudinal study of HRQOL in pediatric liver transplant recipients, aged less than 5 years to define the impact of liver transplantation on HRQOL and identify factors that predict HRQOL after transplantation. The infant toddler health status questionnaire (ITHQ) was completed at the time of listing for liver transplantation and at 6 and 12 months after liver transplantation.

Effect of triglyceride structure on fecal excretion of 13C-labeled triglycerides.

Objective: The aim of this work was to determine the effects of specific changes in the structure of (13)C-labeled triglyceride (TG*) on its fecal excretion relative to total stool fat excretion determined simultaneously in patients with reduced exocrine pancreatic function.

Methods: A series of 47 studies were conducted in 26 young cystic fibrosis (CF) patients and 11 adult patients with chronic pancreatitis over a five year period. Each test consisted of ingesting a single high fat test meal containing both (13)C-labeled triglyceride (TG*) and dysprosium chloride (DyCl(3)) a nonabsorbable marker of intestinal transit; in most studies the food colorant brilliant blue (FD&C blue #1) was administered along with the DyCl(3).

Dysprosium chloride as a nonabsorbable gastrointestinal marker for studies of stable isotope-labeled triglyceride excretion in man.

Objective: The aim of this work was to determine if dysprosium chloride (DyCl(3)) is a suitable nonabsorbable marker for studies of labeled-triglyceride excretion in cystic fibrosis patients allowing excretion to be determined accurately after analysis of one or two stools.

Methods: A series of 66 absorption studies were conducted in 36 young cystic fibrosis patients over a five year period. All tests consisted of ingesting a single test meal containing both (13)C-labeled triglyceride (TG*) and DyCl(3); in most studies the food colorant brilliant blue (FD&C blue #1) was administered along with the DyCl(3).

Risk of hearing impairment in pediatric liver transplant recipients: a single center study.

As survival rates following liver transplantation have increased, health care providers must assess the impact of transplantation on dimensions other than traditional medical measures. Hearing impairment may adversely impact social, emotional, cognitive, academic, and speech and language development. We hypothesized that children who undergo liver transplantation are at risk for hearing impairment due to exposure to ototoxic drugs.

Health-related quality of life in pediatric liver transplant recipients: A single-center study.

The goals of the present study were (1) to measure health-related quality of life (HRQOL) in pediatric liver transplantation (LT), (2) to identify demographic and clinical factors that correlate with HRQOL, and (3) to compare two instruments that have been used to measure HRQOL in children and adolescents. We conducted a single-center cross-sectional study of 77 pediatric LT recipients ages 5 to 18 years, all of whom had had LT at least 6 months previously. We used the Child Health Questionnaire Parent Form 50 (CHQPF50) and the PedsQL4.