以低血糖昏迷就诊的多发性内分泌腺瘤1型四例并文献复习

目的 探讨以低血糖昏迷就诊的多发性内分泌腺瘤1型(MENI型)的临床特点及诊治经验.方法 对因复低血糖昏迷住院、经内分泌激素和影像检查及手术确诊为MEN1型的4例患者临床资料进行分析.结果 (1)4例患者均具备Whipple三联征,影像榆查显示胰腺内占位性病变,手术病理证实为胰岛素瘤,其中2例为多发性.(2)1例合并垂体瘤、甲状旁腺瘤(术后复发)和肾上腺皮质腺瘤,1例合并垂体瘤、甲状旁腺瘤(2个)和肾卜腺结节增生,1例合并垂体瘤和甲状旁腺瘤,另1例合并垂体瘤及可疑甲状旁腺瘤.结论 (1)对于胰岛素瘤患者,应仔细检查是否存在MENl型.(2)对于MEN1型患者,手术时应关注甲状旁腺瘤(或增生)和胰岛素瘤是否为多发及异位等情况.术后应定期随访以监测病变的复发和新病变发生.

Multiple endocrine neoplasia type 1 presenting as hypoglycemic coma :a report of four cases and review of literatures

Objective To investigate the clinical characteristics of multiple endocrine neoplasia type 1(MEN1)patients presenting with hypoglycemic coma as chief manifestation and the related clinical experience in diagnosis and therapy.Methods We analyzed the clinical data of 4 patients who were hospitMized because of recurrent hypoglycemic coma and diagnosed as having MEN1 by endocrinolgical,radiological and pathological examinations.Results In the 4 cases of Whipple trilogy,radiological examination showed occupying lesion in the pancreas and pathological examination confirmed the diagnosis of insulinoma.In 2 cases the insulinomas were multiple.In this series,one cage was complicated with pituitary adenoma,parathyroidoma(recurrent after operation)and adrenocortical adenoma,one case with pituitary adenoma,parathyroidoma(2 tumors)and adrenal nodular hyperplasia,one case with pituitary adenoma and parathyroidoma,and the remaining one with pituitary adenoma and suspectable parathyroidoma.Conclusions For patients with insulinoma,MEN1 should be considered.In patients with MEN1,the presence of multiple or ectopic Darathymid adenomas(or hyperplasia)and insulinomas should be inspected during operation.After operation,examinations should be regularly performed to identify whether the diseases relapse or new endocrine neoplasias occur.