Type I glycogen storage disease: nine years of management with cornstarch.

Long-term effects of cornstarch (CS) therapy on biochemical values and physical growth in children with type I glycogen storage disease (GSD I) were compared to those of children receiving continuous nocturnal nasogastric glucose feedings (CNG). Only patients who had received more than 5 years of dietary therapy (either CS or CNG) were evaluated. Six patients (five female, age 13.5 years +/- 1.3, range 11.7-16.5 years) received CS (1.75-2.5 g/kg, four times daily) and seven patients (five female, age 9.6 +/- 2.5 years, range 7.3-14.8 years) received CNG. Blood glucose, lactate, cholesterol and triglyceride levels were not significantly different between the two methods of treatment. All patients maintained linear growth rates normal for their age. The standard deviation score of height after 6.7 +/- 1.6 years (range 5-9 years) of CS treatment was -1.29 +/- 0.59 and after 8.8 +/- 2.4 years (range 7-14 years) of CNG was -1.24 +/- 0.63. These values did not differ significantly from each other or from the target height, an estimate of genetic potential for height as determined from parental heights. With the exceptions of diarrhea, increased flatulence and excess weight gain, there were no adverse effects of CS after 9 years of treatment. Our data suggests that cornstarch is a simple, effective and safe therapy for GSD I.

Reversal of skin test anergy during maintenance hemodialysis by protein and calorie supplementation.

The effect of protein and calorie supplementation on the immune function of two maintenance hemodialysis patients was assessed. Before nutritional supplementation, both patients were anergic to four skin test antigens and had low relative percentages and absolute number of T lymphocytes. After 3 months of nutritional supplements both patients responded to in vivo skin testing to at least two antigens and in both patients, the relative percentage and absolute number of T lymphocytes increased. These two cases illustrate that the defect in cell-mediated immunity and impaired delayed cutaneous hypersensitivity which is known to occur in hemodialysis patients may be a reversible manifestation of protein-calorie malnutrition.