Physical growth and development of children with type 1 glycogen-storage disease: comparison of the effects of long-term use of dextrose and uncooked cornstarch.
Thirteen patients with type 1 glycogen-storage disease (GSD-1) were studied to compare the effects on biochemical control and growth of 2 y of therapy with intermittent feedings of uncooked cornstarch (UCS) at the fasting glucose production rate and therapy with continuous overnight glucose (COG) and dextrose feedings during the day. Mean biochemical abnormalities for the groups were minimized but not normalized by either COG or UCS. Growth progressed normally when COG was started by 1.2 y of age and normal growth rate was maintained by UCS. Weight increased from 101 +/- 3% ideal body weight at onset of COG to 127 +/- 5% during COG and the first year of UCS therapy but did not increase further in the second year. When growth failure occurred before onset of COG [-3.7 SD score for chronological age (SDSCA)], only partial correction (-1.9 SDSCA) to genetic potential for height occurred. Intermittent feeding of UCS provides an effective alternative to COG for the treatment of GSD-1.
Wolfsdorf
J I
JI
Department of Medicine, Children's Hospital, Boston, MA 02115.
Rudlin
C R
CR
Crigler
J F
JF
D000293:Adolescent; D001786:Blood Glucose / Q000032:analysis; D001827:Body Height; D001835:Body Weight; D002648:Child; D002675:Child, Preschool; D002784:Cholesterol / Q000097:blood; D005260:Female; D005947:Glucose / Q000008:administration & dosage*; D005953:Glycogen Storage Disease Type I / Q000097:blood / Q000503:physiopathology*; D013006:Growth Hormone / Q000097:blood; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D007328:Insulin / Q000097:blood; D007773:Lactates / Q000097:blood; D008137:Longitudinal Studies; D008297:Male; D011446:Prospective Studies; D013213:Starch / Q000008:administration & dosage*; D014280:Triglycerides / Q000097:blood; D014527:Uric Acid / Q000097:blood
D001786:Blood Glucose; D007328:Insulin; D007773:Lactates; D014280:Triglycerides; D014527:Uric Acid; D013006:Growth Hormone; D013213:Starch; D002784:Cholesterol; D005947:Glucose; D006854:Hydrocortisone