Treatment with low-dose diazoxide in two growth-retarded prepubertal girls with glycogen storage disease type Ia resulted in catch-up growth

J M Nuoffer; P E Mullis; U N Wiesmann

doi:10.1023/a:1005319818015

Treatment with low-dose diazoxide in two growth-retarded prepubertal girls with glycogen storage disease type Ia resulted in catch-up growth

J Inherit Metab Dis. 1997 Nov;20(6):790-8. doi: 10.1023/a:1005319818015.

Authors

J M Nuoffer¹, P E Mullis, U N Wiesmann

Affiliation

¹ Division of Endocrinology and Metabolism, University Children's Hospital, Bern, Switzerland.

PMID: 9427147
DOI: 10.1023/a:1005319818015

Abstract

Two originally prepubertal girls suffering from glycogen storage disease type Ia and short stature were treated with low-dose diazoxide (3-4.8 mg/kg per day) for 7 and 4 years, respectively. Both showed an impressive catch-up growth following this treatment. This appeared to be due to prolongation of normoglycaemia after meals and reduction of fasting lactic acidosis by diazoxide.

Publication types

Case Reports

MeSH terms

Acidosis, Lactic / complications
Acidosis, Lactic / drug therapy
Blood Glucose / metabolism
Child
Child, Preschool
Diazoxide / administration & dosage
Diazoxide / therapeutic use*
Female
Food
Glycogen Storage Disease Type I / complications*
Growth Disorders / blood
Growth Disorders / drug therapy*
Growth Disorders / etiology
Growth*
Humans

Substances

Blood Glucose
Diazoxide