title: Fulminant type 1 diabetes: a collaborative clinical cases investigation in China.
abstract: To clarify the detailed distribution, clinical and immunological features of patients with fulminant type 1 diabetes (F1D), we consecutively investigated 53 cases from nationwide 24 hospitals in China. After the clinical and immunological features were evaluated, we compared pregnancy-associated F1D (PF) with those of child-bearing age with F1D that was not associated with pregnancy in clinical characteristics. As a result, patients with F1D were reported from all over China, and there was no significant regional and seasonal preference of this disease. Around 34.0% (18/53) patients displayed low titers of autoantibodies against one or more autoantigens, including 12 cases positive for GADA, 2 for IA-2A, 4 for ZnT8A and 3 for both GADA and ZnT8A. The frequency of PF in female F1D was 34.6% (9/26). Among 9 PF patients, 8 (88.9%) developed F1D during pregnancy which resulted in stillbirth, while one had the onset of F1D after eutocia with her fetus survived. This study suggests that islet-associated autoimmunity may be involved in and contribute to the development of F1D. Pregnant women may be PF high-risk population, and the prognosis for the fetus is extremely poor in PF patients.
authors.affiliation: Diabetes Center, Institute of Metabolism and Endocrinology, Second Xiangya Hospital, Central South University, Changsha, China.
authors.forename: Shuoming
authors.initials: S
authors.lastname: Luo
authors.forename: Zhenyi
authors.initials: Z
authors.lastname: Zhang
authors.forename: Xia
authors.initials: X
authors.lastname: Li
authors.forename: Lin
authors.initials: L
authors.lastname: Yang
authors.forename: Jian
authors.initials: J
authors.lastname: Lin
authors.forename: Xiang
authors.initials: X
authors.lastname: Yan
authors.forename: Zhen
authors.initials: Z
authors.lastname: Wang
authors.forename: Chao
authors.initials: C
authors.lastname: Zheng
authors.forename: Gan
authors.initials: G
authors.lastname: Huang
authors.forename: Zhiguang
authors.initials: Z
authors.lastname: Zhou
mesh_terms: D000293:Adolescent; D000328:Adult; D001323:Autoantibodies / Q000276:immunology; D027682:Cation Transport Proteins / Q000276:immunology; D003922:Diabetes Mellitus, Type 1 / Q000453:epidemiology* / Q000208:ethnology / Q000276:immunology; D005260:Female; D005968:Glutamate Decarboxylase / Q000276:immunology; D006801:Humans; D008297:Male; D008875:Middle Aged; D011247:Pregnancy; D017027:Protein Tyrosine Phosphatases / Q000276:immunology; D055815:Young Adult; D000075703:Zinc Transporter 8
chemical_list: D001323:Autoantibodies; D027682:Cation Transport Proteins; C492522:SLC30A8 protein, human; D000075703:Zinc Transporter 8; D017027:Protein Tyrosine Phosphatases; D005968:Glutamate Decarboxylase