UNASSIGNED: A 59-year-old woman with a 24-year history of T1D and recurrent GIST presented with frequent episodes of symptomatic hypoglycemia despite continuous reductions in her insulin therapy. Laboratory workup revealed undetectable insulin and C-peptide, low insulin-like growth factor (IGF) 1, normal IGF-2, and an elevated IGF-2:IGF-1 ratio. Medical management with prednisone alone and, later, in combination with octreotide did not reduce hypoglycemic episodes. Eventually, during hospitalization for severe hypoglycemia, she was treated and discharged with continuous intravenous dextrose infusion. She ultimately required around-the-clock glucose infusions, which helped her maintain what she believed was an acceptable quality of life during her remaining weeks.
UNASSIGNED: NICTH is characterized by excessive tumor production of IGF-2 or pro-IGF-2, leading to unrestricted glucose uptake in peripheral tissues and hypoglycemia. A diagnosis of NICTH can be made on the basis of low IGF-1 levels in the plasma with normal or elevated IGF-2. Tumor resection is the most definitive treatment for NICTH.
UNASSIGNED: This patient with T1D presented with resistant hypoglycemia due to recurrence of an enlarging GIST. She required discontinuation of all insulin therapy and continuous dextrose infusions to maintain euglycemia.
■一名59岁女性,有24年的T1D和复发性GIST病史,尽管她的胰岛素治疗持续减少,但仍表现为症状性低血糖发作。实验室检查显示检测不到胰岛素和C肽,低胰岛素样生长因子(IGF)1,正常的IGF-2和升高的IGF-2:IGF-1比率。单独使用泼尼松的医疗管理,稍后,与奥曲肽联用并不能减少低血糖发作.最终,在严重低血糖住院期间,她接受了持续静脉输注葡萄糖治疗并出院.她最终需要全天候注射葡萄糖,这帮助她在剩下的几周内保持了她认为可以接受的生活质量。
■NICTH的特征是肿瘤产生过多的IGF-2或pro-IGF-2,导致外周组织中葡萄糖摄取不受限制和低血糖。NICTH的诊断可以基于具有正常或升高的IGF-2的血浆中的低IGF-1水平来进行。肿瘤切除是NICTH最明确的治疗方法。
■该T1D患者由于GIST增大复发而出现耐药性低血糖。她需要停止所有胰岛素治疗和持续葡萄糖输注以维持血糖正常。