Haddad M J, Mathew P M
Surgical Services Division, Saudi Aramco Medical Services Organization, Saudi Aramco-Dhahran Health Center, Saudi Arabia.
Eur J Pediatr Surg. 1996 Apr;6(2):82-5. doi: 10.1055/s-2008-1066477.
A retrospective analysis of data on all infants with persistent neonatal hyperinsulinism (PNH) in our hospital during a nine-year period (July 1983 to June 1992) was carried out. The extent of pancreatectomy and outcome based on long-term follow-up were reviewed. Fourteen of the 18 cases, diagnosed at our facility according to the established criteria of PNH, underwent surgery. Two patients who were managed medically because of milder disease and reluctance of parents for surgery and two patients who were diagnosed and operated upon outside our facility were excluded from this study. One patient, operated upon in 1985, needed re-surgery after 95% pancreatectomy because of recurrent hypoglycaemia unresponsive to medical treatment. This case influenced our management in a subsequent patient in whom we performed total pancreatectomy as an initial procedure. Both these patients are still diabetic. Three more patients had recurrence of hypoglycaemia after 95% pancreatectomy, two were managed successfully with a short course of diazoxide, the third had associated carnitine deficiency and his hypoglycaemia resolved after a short period of carnitine therapy. The first patient in our series had 85% pancreatectomy, the other 11 patients had 95% pancreatectomy as an initial procedure. The 12 patients are normoglycaemic and off all medication. We conclude that 95% pancreatectomy as an initial procedure for PNH offers the best prognosis and that recurrence of hypoglycaemia does occur following 95% pancreatectomy and can easily be managed medically.
我们对我院在九年期间（1983年7月至1992年6月）所有持续性新生儿高胰岛素血症（PNH）婴儿的数据进行了回顾性分析。回顾了胰腺切除术的范围以及基于长期随访的结果。根据PNH既定标准在我院确诊的18例病例中，有14例接受了手术。因病情较轻且家长不愿手术而接受药物治疗的2例患者以及在我院外确诊并接受手术的2例患者被排除在本研究之外。1985年接受手术的1例患者，在95%胰腺切除术后因反复低血糖且药物治疗无效而需要再次手术。该病例影响了我们对后续1例患者的治疗，我们对该患者首次进行了全胰腺切除术。这2例患者仍患有糖尿病。另外3例患者在95%胰腺切除术后出现低血糖复发，其中2例通过短期使用二氮嗪成功治疗，第3例伴有肉碱缺乏，在短期肉碱治疗后低血糖得到缓解。我们系列中的首例患者进行了85%胰腺切除术，其他11例患者首次进行了95%胰腺切除术。这12例患者血糖正常，无需任何药物治疗。我们得出结论，95%胰腺切除术作为PNH的初始治疗方法预后最佳，95%胰腺切除术后确实会发生低血糖复发，且通过药物治疗很容易控制。
