Reiterer F, Gamillscheg A, Ritschl E, Müller W D, Schwingshandl J, Borkenstein M, Klos J, Ratschek M, Fellbaum C
Department für Neonatologie, Universitäts-Kinderklinik Graz.
Padiatr Padol. 1990;25(1):25-31.
A now 10 month old female infant suffered from persistent non ketotic neonatal hypoglycemia despite continuous intravenous application of glucose (greater than 10 mg/kg/min). There was only a transient response of blood glucose after intravenous administration of glucagon and prednisolon. Biochemical findings indicated hyperinsulinismus (insulin level of 26 mE/ml during hypoglycemia). Oral diazoxid treatment in high doses (22 mg/kg) stopped hypoglycemia episodes for several days but the newborn remained glucose infusion depended. Finally the treatment had to be interrupted because of vomiting. At the age of 4 1/2 weeks a subtotal pancreatectomy was performed. The histological examination of the pancreas confirmed the clinically suspected diagnosis of nesidioblastosis. After pancreatectomy the infant required insulin therapy. Since six months the girl is without insulin in a good condition. Despite periods of arrested head growth before pancreatectomy the psychomotoric development is normal.
一名现10个月大的女婴，尽管持续静脉输注葡萄糖（大于10毫克/千克/分钟），仍患有持续性非酮症性新生儿低血糖症。静脉注射胰高血糖素和泼尼松龙后，血糖仅有短暂反应。生化检查结果提示高胰岛素血症（低血糖期间胰岛素水平为26微国际单位/毫升）。大剂量口服二氮嗪治疗（22毫克/千克）可使低血糖发作停止数天，但新生儿仍依赖葡萄糖输注。最后，由于呕吐，治疗不得不中断。在4.5周龄时进行了次全胰腺切除术。胰腺组织学检查证实了临床怀疑的胰岛细胞增殖症诊断。胰腺切除术后，婴儿需要胰岛素治疗。自6个月以来，女孩无需胰岛素，状况良好。尽管在胰腺切除术前有头部生长停滞期，但精神运动发育正常。
