Dong Ai Mei, Yuan Zhen Fang, Zhang Hong, Gao Yan Ming, Guo Xiao Hui
Department of endocrinology, Peking University First Hospital, Beijing 100034, China.
Beijing Da Xue Xue Bao Yi Xue Ban. 2009 Oct 18;41(5):590-2.
Adults-onset nesidioblastosis, as a differential diagnosis of organic hyperinsulinemic hypoglycemia, is very rare and has been recognised as "noninsulinoma pancreatogenous hypoglycaemia syndrome (NIPHS)". Here we described an extremely rare case of NIPHS in an elderly type 2 diabetes mellitus with insulin therapy. A 84-year old male was diagnosed as type 2 diabetes six years ago and switched from an oral hypoglycemic drug to pro-mixed insulin treatment 3 years ago. According to medical records, he had good-glucose control over few hypoglycemia. He was admitted to hospital due to frequent fasting hypoglycaemic episodes and comas despite withdrawal of any anti-diabetes drugs and continuous infusion of homogenate meal at night. Lab test showed low fasting glucose level and inappropriate high insulin/C-peptide level, and anti-insulin antibody was negative. A characteristic of hyperinsulinemic hypoglycemia with high C-peptide level was consistent with the possible diagnosis of insulinoma, but localizing studies including computed tomography of the abdomen and somatostatin receptor scintigraphy were negative. Surgical exploration by the palpation and intraoperative ultrasonography failed to detect any mass in the pancreas and 70% distal pancreatectomy was performed. Histological examination of the resected pancrease revealed an increased number and size of islets consistent with nesidioblastosis. After transient decline, his serum insulin travelled back to the level before pancreaectomy, but recurrent fasting hypoglycemia was mild and controlled by regular night eating postpancreaectomy.
成人隐匿性自身免疫性糖尿病,作为器质性高胰岛素血症性低血糖症的鉴别诊断,非常罕见,已被确认为“非胰岛素瘤性胰源性低血糖综合征(NIPHS)”。在此,我们描述了一例极为罕见的NIPHS病例,患者为一名接受胰岛素治疗的老年2型糖尿病患者。一名84岁男性6年前被诊断为2型糖尿病,3年前从口服降糖药改为预混胰岛素治疗。根据病历记录,他血糖控制良好,很少发生低血糖。尽管停用了所有抗糖尿病药物并在夜间持续输注匀浆膳食,但他仍因频繁的空腹低血糖发作和昏迷入院。实验室检查显示空腹血糖水平低,胰岛素/C肽水平异常升高,且抗胰岛素抗体为阴性。高胰岛素血症性低血糖伴高C肽水平的特征与胰岛素瘤的可能诊断一致,但包括腹部计算机断层扫描和生长抑素受体闪烁显像在内的定位检查均为阴性。通过触诊和术中超声进行的手术探查未在胰腺中发现任何肿块,遂进行了70%的远端胰腺切除术。切除胰腺的组织学检查显示胰岛数量和大小增加,符合胰岛细胞增生症。术后血清胰岛素短暂下降后又回升至胰腺切除术前的水平,但复发性空腹低血糖症状较轻,胰腺切除术后通过规律的夜间进食得到控制。