Pancreatic insufficiency, growth, and nutrition in infants identified by newborn screening as having cystic fibrosis.

To evaluate the impact of early pancreatic insufficiency on growth and nutritional status in cystic fibrosis, we studied 49 infants identified by a newborn screening program. Pancreatic insufficiency, determined by increased 72-hour fecal fat excretion, was present in 59% (23/39) of infants at diagnosis (7.0 +/- 0.8 weeks; mean +/- SEM). Before initiation of pancreatic enzyme replacement, growth and nutritional status of pancreatic-insufficient (n = 16) and pancreatic-sufficient (n = 13) infants were compared. Pancreatic-insufficient infants gained less weight from birth to diagnosis (13.4 +/- 3.4 vs 22.3 +/- 4.0 gm/day; p = 0.05), had decreased triceps skin-fold thicknesses (4.5 +/- 0.3 vs 6.1 +/- 0.4 mm; p less than 0.005), and had lower blood urea nitrogen (3.07 +/- 0.42 vs 4.62 +/- 0.65 mg/dl; p = 0.02) and albumin (2.99 +/- 0.14 vs 3.54 +/- 0.14 gm/dl; p less than 0.01) levels despite higher gross calorie (154 +/- 8 vs 116 +/- 13 kcal/kg per day; p less than 0.01) and protein intakes (2.81 +/- 0.21 vs 2.14 +/- 0.33 gm/kg per day; p = 0.03). Fecal nitrogen loss was correlated with fat loss (r = 0.79; p less than 0.001). Fat malabsorption was present in 79% (30/38) and 92% (33/36) of infants tested at 6 months and 12 months of age, respectively, indicating that pancreatic insufficiency persists and increases in frequency throughout infancy. We conclude that pancreatic insufficiency is prevalent in young infants with cystic fibrosis and has a significant impact on growth and nutrition.

Fat-soluble vitamins in infants identified by cystic fibrosis newborn screening.

Fat-soluble vitamin status was assessed in 36 infants diagnosed with cystic fibrosis by newborn screening in the Colorado Program. At the time of diagnosis of cystic fibrosis, 36% of infants were hypoalbuminemic, 21% had vitamin A deficiency, 35% had vitamin D deficiency, and 38% had vitamin E deficiency. None had vitamin K deficiency. Supplementation with pancreatic enzymes, a multiple vitamin preparation, and additional vitamin E was associated with normalization of serum albumin, retinol, and 25-hydroxyvitamin D and negative PIVKA testing at age 6 and 12 months. Several patients remained vitamin E deficient, but this was felt to be due to poor compliance. Biochemical evidence of fat-soluble vitamin deficiency is common before age 3 months in infants with CF and responds to supplementation in the first year of life.

Pre-breakfast plasma zinc concentrations: the effect of previous meals.

The aim of this study was to determine the effect of the time and size of the last meal of the previous day on pre-breakfast 0700 hours plasma zinc concentrations. Nine healthy women were each studied on four separate occasions when the same standard meal was taken either at 1800 hours (study 1); double meal at 1800 hours (study 2); at 2400 hours (study 3); or at 1800 hours and 2400 hours (study 4). Mean differences between 1800 hours (day 1) and 0700 hours (day 2) plasma zinc (mumol/L +/- SEM) were 1.6 +/- 0.3 for study one; 0.8 +/- 0.3 for study two; 0.1 +/- 0.3 for study three and 0.0 +/- 0.2 for study four. The differences for studies two, three and four were significantly less than the difference for study one (p less than 0.05). The difference for study four was also less than the difference for study two (p less than 0.05). Changes in time and size of the meals accounted for 59% of within-subject variation in 0700 hours plasma zinc concentrations. It is concluded that pre-breakfast 0700 hours plasma zinc concentrations are affected by the time and the size of the meal(s) consumed the previous evening.

Anthropometric evaluation of children with chronic liver disease.

To characterize type and age distribution of malnutrition and to determine the usefulness of anthropometric indices in children with chronic liver disease (CLD), 56 children (aged 1 mo-10 y) with CLD underwent anthropometric evaluation when they were clinically stable. Mean-height Z score was depressed, whereas mean-weight Z score was closer to normal and mean-weight/height Z score was normal in patients with extrahepatic biliary atresia, idiopathic neonatal hepatitis, and other liver disorders. Patients with arteriohepatic dysplasia showed more severe depression of all three variables. In all patients, triceps skinfold (TSF) thickness Z scores were significantly more depressed than were weight/height Z scores. Depressions of midarm-circumference and midarm-muscle-area Z scores were intermediate. Mean-head-circumference Z score was depressed in children aged less than 24 mo. We conclude that acute (wasting) and chronic (stunting) malnutrition are common in childhood CLD and that weight/height values underestimate the degree of acute malnutrition compared with TSF thickness, most likely because of the inflated patient weight caused by organomegaly.

Carnitine status of pediatric patients on continuous ambulatory peritoneal dialysis.

Plasma carnitine and the effect of oral carnitine supplementation on serum triglycerides was studied in 12 pediatric patients receiving continuous ambulatory peritoneal dialysis (CAPD). Baseline evaluation of all patients included plasma carnitine and serum triglyceride values. Following randomization into two groups, only group 2 patients received oral L-carnitine supplementation, 100 mg/kg/day, for 2 months. The initial laboratory evaluation was repeated at the conclusion of the study. Plasma carnitine values were also determined from a control population. Mean baseline plasma carnitine concentrations of group 1 (39.8 +/- 8.0 nmol/ml) and group 2 (45.2 +/- 10.3 nmol/ml) patients were not significantly different from each other or from the control population. Serum triglyceride values were elevated in both groups (group 1 - 206.5 +/- 100.0 mg/dl; group 2 - 279.3 +/- 74.5 mg/dl). After 2 months, the mean plasma carnitine concentration of group 2 patients increased to 147.7 +/- 84.1 nmol/ml, significantly greater than the value of group 1, 32.8 +/- 8.0 nmol/ml (p less than 0.004). However, no significant change in the serum triglyceride level was noted in either group. We conclude that the plasma carnitine status of pediatric patients receiving CAPD is normal and that oral carnitine supplementation does not lead to the resolution of hypertriglyceridemia.

Fat-soluble-vitamin status during the first year of life in infants with cystic fibrosis identified by screening of newborns.

We investigated the fat-soluble-vitamin status during the first year of life in 36 infants with cystic fibrosis (CF) consecutively identified by screening of newborns. At initial evaluation (at age 51.0 +/- 26.7 d) 36% of patients were hypoalbuminemic, 21% had low serum retinol, 35% had low serum 25-hydroxyvitamin D. 38% had low serum alpha-tocopherol and low ratios of serum vitamin E to total lipids, and none had elevated protein in vitamin K absence (PIVKA). Hypoalbuminemia was more common in breast-fed than in formula-fed infants. Seventy-two-hour fecal fat excretion correlated inversely with serum alpha-tocopherol. Treatment with oral pancreatic enzyme supplements, a multiple vitamin, and additional vitamin E was associated with normalization of serum albumin, retinol, and 25-hydroxyvitamin D and negative PIVKA at age 6 and 12 mo. Approximately 10% of patients remained vitamin E deficient. Biochemical evidence of fat-soluble-vitamin deficiencies is common before age 3 mo in patients with CF and, except for vitamin E, these deficiencies corrected with standard therapy.

Post-prandial and daily changes in plasma zinc.

The objective of this study was to determine changes in plasma zinc over a 24-hour period and the relationship of these changes to meals. Hourly plasma zinc concentrations were measured in 12 normal adult women for 24 consecutive hours. The subjects were fed standard meals at 0700 hours, 1200 hours and 1700 hours. A decline in plasma zinc started 1/2 to 1 1/2 hours after each meal, reaching a low point 3-4 hours after each meal. During the night there was a progressive increase in plasma zinc concentrations averaging (1.54 micrograms/dl)h-1 for 11 hours. The lowest value of the day at 2100 hours was 22% lower than the highest value at 0730 hours. These results indicate that meals are the primary factor and could be the only factor responsible for variations in an individual's plasma zinc during any one day.

A unique approach to peritoneal dialysis in infants.

Little recorded experience exists concerning the use of chronic peritoneal dialysis in the infant. We herein report the results of a multidisciplinary approach to two infants who were treated with a unique modification of "short-dwell" peritoneal dialysis. A single-bag technique was devised in the hopes of reducing glucose absorption and protein losses while concomitantly permitting a simplified manual method of cycling peritoneal dialysis. The desired nutritional needs of the infants consisted of a caloric intake of 140 calories/kg/d and a protein intake of 3.0 to 4.0 g/kg/d, a goal that often required administration through a nasogastric tube. The timely initiation of peritoneal dialysis and the strict adherence to the prescribed dietary regimen was associated with continued growth in both patients. Repeated developmental assessments of each child were conducted and revealed normal development in one patient and mild delay, but gradual improvement, in the other.