Boro Hiya, Kubihal Suraj, Arora Saurabh, Kubihal Vijay, Tandon Nikhil
Endocrinology, Diabetes, and Metabolism, All India Institute of Medical Sciences, New Delhi, IND.
Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, IND.
Cureus. 2020 Dec 14;12(12):e12073. doi: 10.7759/cureus.12073.
Multiple endocrine neoplasia type 1 (MEN1) or Wermer's syndrome is a genetic disease characterized by involvement of multiple endocrine glands, primarily involving parathyroid, pancreas, and pituitary. Other additional features include foregut carcinoids; non-functioning adrenal tumors; and skin lesions such as lipomas, collagenomas, and angiofibromas. Here, we describe our experience in managing a patient who presented to us with spontaneous episodes of hypoglycemia and was diagnosed with insulinoma. Detailed clinical and biochemical evaluation unraveled the diagnosis of MEN1 in the patient and her family members who constituted a large kindred. This case highlights the importance for evaluation of MEN1 in a patient or his/her family members in the setting of clinical and biochemical suspicion. In addition, we have also discussed the utility of the latest diagnostic and therapeutic modalities for management of MEN1.
多发性内分泌腺瘤1型（MEN1）或韦默综合征是一种遗传性疾病，其特征是多个内分泌腺受累，主要累及甲状旁腺、胰腺和垂体。其他额外特征包括前肠类癌；无功能性肾上腺肿瘤；以及皮肤病变，如脂肪瘤、胶原瘤和血管纤维瘤。在此，我们描述了我们对一名因自发性低血糖发作前来就诊并被诊断为胰岛素瘤的患者的管理经验。详细的临床和生化评估揭示了该患者及其构成一个大家族的家庭成员中MEN1的诊断。该病例突出了在临床和生化怀疑的情况下对患者或其家庭成员进行MEN1评估的重要性。此外，我们还讨论了MEN1管理中最新诊断和治疗方式的实用性。
