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.
Chen
Y T
YT
Department of Pediatrics, Duke University Medical Center, Durham, NC 27710.
Bazzarre
C H
CH
Lee
M M
MM
Sidbury
J B
JB
Coleman
R A
RA
D000293:Adolescent; D001827:Body Height; D001835:Body Weight; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D005947:Glucose / Q000008:administration & dosage*; D005953:Glycogen Storage Disease Type I / Q000097:blood / Q000178:diet therapy* / Q000503:physiopathology; D006801:Humans; D007441:Intubation, Gastrointestinal; D008297:Male; D013213:Starch / Q000008:administration & dosage* / Q000009:adverse effects; D013997:Time Factors
D013213:Starch; D005947:Glucose