Elevated meconium lactase activity. Its use as a screening test for cystic fibrosis.

Screening of newborn infants could provide information needed to evaluate the effects of early treatment on the course and prognosis of cystic fibrosis (CF). Two procedures of screening meconium for CF were compared: increased albumin levels, detected by a commercial test strip; and increased lactase activity, detected by glucose production after incubation of meconium with lactose. Specimens positive by the lactase test were retested for albumin. Low-birth-weight infants accounted for more than half the positive test results with both procedures. Sweat chloride measurements were carried out only on infants whose meconium specimens had albumin concentration greater than 12 mg/g (wet weight), measured by radial immunodiffusion. Twelve infants with CF were identified through screening, six while testing 44,816 specimens by lactase activity test. Three additional infants with CF were missed because meconium specimens were negative to both tests.

Dietary supplement and nutrition in children with cystic fibrosis.

Assessment of nutritional status of patients with cystic fibrosis of the pancreas (CFP) showed that poor growth was associated with low concentrations of albumin, urea nitrogen, and cholesterol in serum and with elevated white blood cell (WBC) counts. Patients with CFP maintained weight approximately 1 standard deviation below the mean until 8 years, after which there was a progressive decline in growth rate compared to normal. A complete dietary supplement consisting of a beef serum hydrolysate, a glucose polymer, and medium-chain triglycerides was given to 15 patients for a year. Patients who received the diet showed significant gains in weight, significant increase in clinical score, significant increase in serum albumin level, and a significant drop in the WBC count compared to control patients who did not receive the supplement.