Kaneko Keizo, Satake Chihiro, Yamamoto Junpei, Takahashi Hironori, Sawada Shojiro, Imai Junta, Yamada Tetsuya, Katagiri Hideki
Department of Diabetes and Metabolism, Tohoku University Hospital, Sendai 980-8575, Japan.
Endocr J. 2017 Mar 31;64(3):369-374. doi: 10.1507/endocrj.EJ16-0245. Epub 2017 Jan 6.
Fulminant type 1 diabetes is characterized by remarkably rapid and complete β-cell destruction. The established diagnostic criteria include the occurrence of diabetic ketosis soon after the onset of hyperglycemic symptoms, elevated plasma glucose with relatively low HbA1c at the first visit, and extremely low C-peptide. Serum C-peptide levels remain extremely low over a prolonged period. A 26-year-old-man with diabetic ketosis was admitted to our hospital. His relatively low HbA1c (7.6%), despite marked hyperglycemia (593 mg/dL) with marked ketosis, indicated abrupt onset. Islet-related autoantibodies were all negative. His data at onset, including extremely low serum C-peptide (0.11 ng/mL), fulfilled the diagnostic criteria for fulminant type 1 diabetes. However, his fasting serum C-peptide levels subsequently showed substantial recovery. While fasting C-peptide stayed below 0.30 ng/mL during the first two months post onset, the levels gradually increased and thereafter fluctuated between 0.60 ng/mL and 0.90 ng/mL until 24 months post onset. By means of multiple daily insulin injection therapy, his glycemic control has been well maintained (HbA1c approximately 6.0%), with relatively small glycemic fluctuations evaluated by continuous glucose monitoring. This clinical course suggests that, despite the abrupt diabetes onset with extremely low C-peptide levels, substantial numbers of β-cells had been spared destruction and their function later showed gradual recovery. Diabetes has come to be considered a much more heterogeneous disease than the present subdivisions suggest. This case does not fit into the existing concepts of either fulminant type 1 or ketosis-prone diabetes, thereby further highlighting the heterogeneity of idiopathic type 1 diabetes.
暴发性1型糖尿病的特点是β细胞迅速且完全破坏。既定的诊断标准包括高血糖症状发作后不久出现糖尿病酮症、初诊时血糖升高但糖化血红蛋白（HbA1c）相对较低以及C肽极低。血清C肽水平在较长时间内一直极低。一名患有糖尿病酮症的26岁男性入住我院。尽管他血糖显著升高（593mg/dL）且伴有明显酮症，但HbA1c相对较低（7.6%），提示起病急骤。胰岛相关自身抗体均为阴性。他起病时的数据，包括极低的血清C肽（0.11ng/mL），符合暴发性1型糖尿病的诊断标准。然而，他的空腹血清C肽水平随后出现了显著恢复。在起病后的头两个月，空腹C肽水平一直低于0.30ng/mL，之后逐渐升高，直至起病后24个月，其水平在0.60ng/mL至0.90ng/mL之间波动。通过每日多次胰岛素注射治疗，他的血糖控制良好（HbA1c约为6.0%），通过持续葡萄糖监测评估，血糖波动相对较小。这一临床过程表明，尽管糖尿病起病急骤且C肽水平极低，但仍有大量β细胞未被破坏，其功能随后逐渐恢复。糖尿病已被认为是一种比目前分类更为异质性的疾病。该病例既不符合暴发性1型糖尿病的现有概念，也不符合易发生酮症的糖尿病的概念，从而进一步凸显了特发性1型糖尿病的异质性。
