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Treatment with low‐dose diazoxide in two growth‐retarded prepubertal girls with glycogen storage disease type Ia resulted in catch‐up growth
Author(s) -
Nuoffer J. M.,
Mullis P. E.,
Wiesmann U. N.
Publication year - 1997
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1023/a:1005319818015
Subject(s) - diazoxide , lactic acidosis , endocrinology , medicine , glycogen , glycogen storage disease , acidosis , metabolic acidosis , insulin
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.