title: Treatment with low-dose diazoxide in two growth-retarded prepubertal girls with glycogen storage disease type Ia resulted in catch-up growth.
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.
authors.affiliation: Division of Endocrinology and Metabolism, University Children's Hospital, Bern, Switzerland.
authors.forename: J M
authors.initials: JM
authors.lastname: Nuoffer
authors.forename: P E
authors.initials: PE
authors.lastname: Mullis
authors.forename: U N
authors.initials: UN
authors.lastname: Wiesmann
mesh_terms: D000140:Acidosis, Lactic / Q000150:complications / Q000188:drug therapy; D001786:Blood Glucose / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D003981:Diazoxide / Q000008:administration & dosage / Q000627:therapeutic use*; D005260:Female; D005502:Food; D005953:Glycogen Storage Disease Type I / Q000150:complications*; D006128:Growth; D006130:Growth Disorders / Q000097:blood / Q000188:drug therapy* / Q000209:etiology; D006801:Humans
chemical_list: D001786:Blood Glucose; D003981:Diazoxide