先天性高胰岛素血症胰腺次全切除术后3例

Therapeutic Effect of Subtotal Pancreatectomy on Three Patients with Congenital Hyperinsulinism

目的对3例先天性高胰岛素血症(CHI)患儿胰腺次全切除术后的疗效进行分析,并对胰腺次全切除术治疗CHI的可行性进行探讨。方法选取2002年3月-2011年9月由本院收治并行胰腺次全切除术治疗的3例CHI患儿为研究对象,对其临床资料及诊疗经过进行回顾性分析,对胰腺次全切除术治疗CHI的疗效进行研究。结果 3例CHI患儿手术前均患有严重的低血糖症,空腹血糖分别为1.9～3.3 mmol·L-1、1.1～3.7 mmol·L-1、0.9～3.1 mmol·L-1,且均伴有高胰岛素血症(低血糖发作时胰岛素水平分别高达50.10 mIU·L-1、82.80 mIU·L-1、21.62 mIU·L-1)。行胰腺次全切除术后,3例患儿空腹血糖分别上升至5.4～8.2 mmol·L-1、2.3～4.3 mmol·L-1、2.5～8.0 mmol·L-1;空腹C-肽和胰岛素均降至正常水平;术后均未发生持续性高血糖及胃肠吸收障碍等严重并发症。结论对二氮嗪治疗无效的CHI患儿,胰腺次全切除术是行之有效的治疗方法之一。鉴于目前国内尚无技术区分弥散型和局灶型CHI,术前应向患儿家长充分告知术后可能发生的糖尿病等严重并发症。

Objective To analyze the therapeutic effect of subtotal pancreatectomy on 3 patients with congenital hyperinsulinism（CHI）, so as to explore the feasibility of applying pancreatectomy to treat CHI. Methods Three patients with CHI,who were hospitalized in endocri-nological unit of Beijing Children＇s Hospital from Mar. 2002 to Sep. 2011, received subtotal pancreatectorny and selected as research subjects. A retrospective study was done for the clinical data and the treatment procedures of the 3 patients. Results All of 3 cases suffered from severe hypoglycemia before surgical operation. The fasting blood glucoses were 1.9 - 3.3 mmol· L^-1, 1.1 - 3.7 mmol· L^-1 and 0.9 - 3.1 mmol· L^-1 respectively, accompanied with hyperinsulinism（ when hypoglycemia occurred, the insulin levels of the 3 patients were 50.10 mIU· L^-1, 82.80 mIU· L^-1 and 21.62 mIU· L^-1, respectively）. After operation, the fasting blood glucoses of the 3 patients raised to 5.4 - 8.2 mmol· L^-1 ,2.3-4.3 mmol· L^-1 ,and 2.5 - 8.0 mmol· L^-1 respectively. Both of the fasting insulin and C - peptide reduced to normal levels. There were no serious complications such as persistent hyperglycemia and gastrointestinal malabsorption, etc. after operation. Conclusions Subtotal pancreatectomy is an effective treatment for the CHI patients unresponsive to diazoxide treatment. In view of the fact that there are no relative techniques to distinguish diffuse from focal CHI currently in China, a fully information including the possible risk of occurring diabetes and other serious complications after the operation should be provided to the parents before surgery.