Shimizu I, Makino H, Imagawa A, Iwahashi H, Uchigata Y, Kanatsuka A, Kawasaki E, Kobayashi T, Shimada A, Maruyama T, Hanafusa T
Department of Internal Medicine, Ehime Prefectural Imabari Hospital, Imabari 794-0006, Japan.
J Clin Endocrinol Metab. 2006 Feb;91(2):471-6. doi: 10.1210/jc.2005-1943. Epub 2005 Nov 8.
The objective of this study was to characterize the clinical and immunogenetic features of Japanese pregnancy-associated fulminant type 1 diabetes (PF). A group of patients with PF was compared with a group of patients of child-bearing age with fulminant type 1 diabetes that was not associated with pregnancy (NPF) in a nationwide survey conducted from 2000-2004.
The clinical characteristics of the 22 patients in the PF group were compared with those of the 48 patients in the NPF group. Human leukocyte antigen (HLA) class II DR and DQ genotyping of 17 PF and 20 NPF patients was performed.
Arterial pH was significantly lower (P = 0.0366), and amylase values tended to increase in PF patients compared with NPF patients (P = 0.0515). In 22 PF patients, 18 developed disease during pregnancy (26.3 wk; range, 7-38), whereas four cases occurred immediately after delivery (10.5 d; range, 7-14 d). Twelve cases that developed during pregnancy resulted in stillbirth (67%), and five of the six fetal cases that survived were delivered by cesarean section. The haplotype frequency of HLA DRB10901-DQB10303 in PF was significantly higher than those in NPF (P = 0.0244) and controls (P = 0.0001), whereas that of DRB10405-DQB10401 in NPF was significantly higher than those in PF (P = 0.0162) and controls (P < 0.0001).
The clinical symptoms of PF patients were more severe than those of NPF patients, and the prognosis of their fetuses was extremely poor. The type 1 diabetes-susceptible HLA class II haplotype is distinct in PF and NPF patients, suggesting that different HLA haplotypes underlie the presentation of PF or NPF.
本研究的目的是对日本妊娠相关暴发性1型糖尿病（PF）的临床和免疫遗传学特征进行描述。在2000年至2004年开展的一项全国性调查中，将一组PF患者与一组非妊娠相关暴发性1型糖尿病（NPF）的育龄患者进行了比较。
将PF组的22例患者的临床特征与NPF组的48例患者的临床特征进行了比较。对17例PF患者和20例NPF患者进行了人类白细胞抗原（HLA）II类DR和DQ基因分型。
与NPF患者相比，PF患者的动脉血pH值显著更低（P = 0.0366），淀粉酶值有升高趋势（P = 0.0515）。在22例PF患者中，18例在孕期发病（26.3周；范围7 - 38周），而4例在产后立即发病（10.5天；范围7 - 14天）。孕期发病的12例导致死产（67%），存活的6例胎儿中有5例通过剖宫产分娩。PF患者中HLA DRB10901 - DQB10303单倍型频率显著高于NPF患者（P = 0.0244）和对照组（P = 0.0001），而NPF患者中DRB10405 - DQB10401单倍型频率显著高于PF患者（P = 0.0162）和对照组（P < 0.0001）。
PF患者的临床症状比NPF患者更严重，其胎儿预后极差。1型糖尿病易感的HLA II类单倍型在PF和NPF患者中不同，提示不同的HLA单倍型是PF或NPF表现的基础。
