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Because glycogen storage disease type IA (GSD-IA) is characterized by recurrent episodes of hypoglycemia that promote a marked elevation in blood triglyceride levels, we evaluated plasma lipid levels in 12 patients with GSD-IA on a regular basis. Six of the 12 patients had plasma fatty acid composition measured; because of possible essential fatty acid deficiency, urinary prostaglandin excretion was also measured. All patients had triglyceride levels between 1440 and 6120 mg/dl (16.25 to 69.09 mmol/L) before treatment. After treatment to promote blood glucose levels of 75 to 85 mg/dl (4.2 to 4.7 mmol/L), triglyceride levels in each of 11 patients were between 189 +/- 31 (2.13 +/- 0.35 mmol/L) and 510 +/- 60 mg/dl (5.76 +/- 0.68 mmol/L). The lipoprotein fatty acid composition in six patients showed a substantial elevation in C16:0, C16:1 omega 7, and C18:1 omega 9, but no increase in C20:3 omega 9 (the fatty acid that characteristically increases in essential fatty acid deficiency). In addition, each of the six patients had normal 24-hour urinary excretion of prostaglandin. One patient, whose triglyceride levels remained elevated despite dietary treatment, was given either clofibrate, lovastatin, niacin, or fish oil. With the exception of lovastatin, these agents produced a decrease in triglyceride values for 1 to 2 months; however, by 3 months triglycerides reached pretreatment levels. Combined treatment with clofibrate and niacin resulted in a sustained decrease in plasma triglyceride levels for 4 months. The findings indicate that dietary management of GSD-IA is usually associated with improvements in triglyceride levels; however, patients maintain triglyceride values between 300 and 500 mg/dl (3.38 to 5.65 mmol/L). No patient had biochemical evidence of essential fatty acid deficiency.

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Because total parenteral nutrition with vitamins added to the glucose-amino acid mixture is often associated with a reduction in blood levels of vitamin A (retinol) during the routine treatment of many very low birth weight (VLBW) infants (less than 1500 gm), and because retinol losses in the plastic delivery system can be prevented by adding the vitamins to an intravenous lipid emulsion, seven VLBW infants with a mean birth weight of 900 gm (range 450 to 1360 gm) were given 40% of a unit dose vial, per kilogram of body weight, of a multivitamin preparation (M.V.I. Pediatric) (280 micrograms retinol; 160 IU vitamin D; 2.8 mg tocopherol; 0.68 mg riboflavin) in a lipid emulsion, Intralipid. After treatment with the intralipid-vitamin mixture for 19 to 28 days, plasma vitamin A (retinol) concentrations increased significantly from 11.0 +/- 0.76 (mean +/- SEM) before intralipid to 19.2 +/- 0.97 micrograms/dl after the intralipid-vitamin mixture (p less than 0.01); 25-hydroxyvitamin D concentrations increased from an initial value of 12.6 +/- 2.6 to 20.2 +/- 1.9 mg/dl (p less than 0.01); alpha-tocopherol concentrations increased from an initial value of 0.31 +/- 0.06 to 2.44 +/- 0.13 mg/dl (p less than 0.01); and riboflavin levels increased from 64.1 +/- 7.8 ng/ml to concentrations between 20 and 100 times the initial level. Erythrocyte riboflavin levels increased from 71.8 +/- 14 initially to 166 +/- 41 ng/gm hemoglobin, and erythrocyte flavin-adenine dinucleotide levels increased similarly from 972 +/- 112 initially to 2005 +/- 294 ng/gm hemoglobin. These results show that the addition of M.V.I. Pediatric to Intralipid decreases the extensive in vivo loss of retinol and is associated with an increase in plasma retinol concentrations in VLBW infants. The daily doses of vitamins D (160 IU/kg) and E (2.8 mg/kg) appear sufficient, but the dose of vitamin A (280 micrograms/kg) is insufficient to raise blood levels of all infants into the normal range. The current dose of riboflavin is excessive and may be harmful.

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Epidermal growth factor is a polypeptide that stimulates proliferation and differentiation of a variety of cell types, including the developing intestinal epithelium; it is the agent in human milk that induces mitosis in human fibroblast culture. We systematically evaluated the EGF content of milk from 20 women delivering prematurely and from 11 women delivering at term. In preterm mothers, the concentration of EGF was 70 +/- 5 ng/ml (mean +/- SEM), with no significant change during seven weeks of lactation. EGF concentration in milk of term mothers was 68 +/- 19 ng/ml (mean +/- SEM). No diurnal variation in the concentration was found. Total EGF content was closely correlated with the volume of milk expressed, suggesting a passive transport from the circulation. These observations confirm that a substantial amount of EGF is present in human milk and that EGF concentrations are not affected by duration of gestation, time of day, or duration of lactation.

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An increased incidence of chronic nonspecific diarrhea has been coincident with popularization of orally administered fluid-electrolyte therapy for management of diarrhea, and led up to postulate than an increase in fluid intake might be related to this increased incidence. Of 105 referred patients, 85 were found to have no clinical or laboratory evidence of malabsorption. Forty of these patients had characteristic features of CNSD: diarrhea for at least three weeks, normal growth, and no evidence of enteric pathogens. An outpatient study evaluated fecal output, dietary energy-protein intake, and nonprotein fluid intake. Patients were separated into two groups whose fluid intakes were highly different: group A, 196 +/- 32 ml/kg/day, and group B, 91 +/- 15 ml/kg/day (P less than 0.001). The nonprotein fluid intake was then reduced to 90 ml/kg/day with no change in diet. Evaluation at two weeks and again at six to eight weeks showed a decrease in stool frequency (from four to ten per day to zero to three per day) and increase in stool consistency in all patients in group A, but no significant change in stool patterns in group B. Our findings suggest a cause-and-effect relationship between excessive fluid intake and some cases of CNSD.

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We report 10 children with chronic relapsing pancreatitis. These patients can be divided into three groups, based on their clinical history, manifestations, and radiographic findings. Group 1 includes four patients with hereditary pancreatitis; these patients have had recurrent abdominal pain since early childhood, and have a positive family history for pancreatitis. Group 2 includes two patients with clinical and radiographic findings similar to those in patients with hereditary pancreatitis but without a family history of pancreatitis. Group 3 includes four patients with fibrosing pancreatitis who had symptoms and signs of obstructive jaundice. Our report emphasizes three points: (1) that chronic pancreatitis does occur in young children and is most commonly caused by hereditary pancreatitis or fibrosing pancreatitis; (2) that endoscopic retrograde cholangiopancreatiography is a safe and valuable tool for the study of pancreatic and common bile ducts; and (3) that surgical intervention is indicated to drain the pancreatic duct in patients with hereditary pancreatitis, and sphincterotomy is an effective therapy for patients with fibrosing pancreatitis.

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