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在两名患有Ia型糖原贮积病的青春期前生长迟缓女孩中,使用低剂量二氮嗪治疗导致了追赶性生长。

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

作者信息

Nuoffer J M, Mullis P E, Wiesmann U N

机构信息

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

出版信息

J Inherit Metab Dis. 1997 Nov;20(6):790-8. 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.

摘要

两名原本处于青春期前、患有Ia型糖原贮积病且身材矮小的女孩,分别接受了低剂量二氮嗪(每天3 - 4.8毫克/千克)治疗,疗程分别为7年和4年。接受该治疗后,两名女孩均出现了显著的追赶生长。这似乎是由于二氮嗪使餐后血糖正常化得以延长,以及空腹乳酸酸中毒减轻所致。

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