Grant D B, Dunger D B, Burns E C
Acta Endocrinol Suppl (Copenh). 1986;279:340-5. doi: 10.1530/acta.0.112s340.
This paper reviews the outcome in 12 children with hyperinsulinaemic hypoglycaemia who first developed symptoms between the ages of 2 and 8 months and who were treated with diazoxide (5-20 mg/kg/day) for 2-13 years. Two cases required subtotal pancreatectomy at the ages of 5 and 10 years because of recurrent hypoglycaemia and one girl with severe retardation died at the age of 6 years while still on diazoxide therapy. Two patients aged 3.5 and 9 years are still on treatment and in 7 cases diazoxide was discontinued between the ages of 2.5 and 14 years, indicating that spontaneous remission can be expected in a high proportion of children with post-neonatal hyperinsulinaemic hypoglycaemia. Of the 9 children who started diazoxide within 3 months of the onset of symptoms, 5 are of normal intelligence and 4 are moderately retarded (IQs 63-71). In 3 children diazoxide was started 8 months to 3 years after the onset of symptoms; two are retarded (IQs 60-70) and the third was severely retarded and died aged 6 years.
本文回顾了12例高胰岛素血症性低血糖症患儿的治疗结果，这些患儿最初在2至8个月龄时出现症状，并接受二氮嗪（5 - 20毫克/千克/天）治疗2至13年。2例患儿因反复低血糖在5岁和10岁时接受了胰腺次全切除术，1例严重发育迟缓的女孩在6岁时仍在接受二氮嗪治疗期间死亡。2例分别为3.5岁和9岁的患儿仍在接受治疗，7例患儿在2.5至14岁之间停用了二氮嗪，这表明新生儿期后高胰岛素血症性低血糖症的患儿中，很大一部分有望自然缓解。在症状出现后3个月内开始使用二氮嗪的9名儿童中，5名智力正常，4名中度发育迟缓（智商63 - 71）。在3名儿童中，二氮嗪在症状出现8个月至3年后开始使用；其中两名发育迟缓（智商60 - 70），第三名严重发育迟缓，6岁时死亡。
