title: Fulminant Type 1 diabetes in a pregnant woman as an initial manifestation of the insulin autoimmune syndrome.
abstract: Fulminant Type 1 diabetes is a subtype of Type 1 diabetes characterized by (1) abrupt onset of diabetes, (2) very short duration of hyperglycaemia with mildly elevated HbA(1c) (< 69 mmol/mol, 8.5%), (3) rapid progression to diabetic ketoacidosis, (4) very low C-peptide level, and (5) often associated with elevated serum pancreatic enzymes, and absence of diabetes-related autoantibodies. We encountered a case of fulminant Type 1 diabetes that developed with an initial manifestation of the insulin autoimmune syndrome and rapidly progressed to diabetic ketoacidosis during pregnancy. A 31-year-old Korean woman presented with recurrent sudden onset of sweating and change of consciousness during sleep at 19 weeks gestation. During a 72-h fasting test, hypoglycaemia (1.72 mmol/l) occurred at 4 h after the start of the test. At that time, there was a high insulin level (370.2 μU/ml), a paradoxically low C-peptide level (0.01 nmol/l) and a positive insulin autoantibody test. An oral glucose tolerance test revealed postprandial hyperglycaemia. She was initially diagnosed as the insulin autoimmune syndrome. On the day 5 of admission, she developed diabetic ketoacidosis. Her HbA(1c) was 62 mmol/mol (7.8%). The rapid progression of diabetic ketoacidosis altered the diagnosis to fulminant Type 1 diabetes. This case differed from typical fulminant Type 1 diabetes because it presented with hypoglycaemia, and positive insulin and anti-phospholipid antibody tests. Her HLA typing was HLA-DQA1*0302, 0501, HLA-DRB1*0301 (DR3), 0901(DR9). Her glucose level was subsequently very well controlled with multiple insulin injections and she successfully delivered a healthy baby.
authors.affiliation: Department of Endocrinology and Metabolism, Kyung Hee University School of Medicine, Seoul, Korea.
authors.forename: H S
authors.initials: HS
authors.lastname: Kim
authors.forename: T-Y
authors.initials: TY
authors.lastname: Lee
authors.forename: E Y
authors.initials: EY
authors.lastname: Kim
authors.forename: J H
authors.initials: JH
authors.lastname: Choi
authors.forename: S Y
authors.initials: SY
authors.lastname: Kim
authors.forename: Y-C
authors.initials: YC
authors.lastname: Hwang
authors.forename: J-H
authors.initials: JH
authors.lastname: Kang
authors.forename: K J
authors.initials: KJ
authors.lastname: Ahn
authors.forename: H Y
authors.initials: HY
authors.lastname: Chung
authors.forename: I-K
authors.initials: IK
authors.lastname: Jeong
mesh_terms: D000328:Adult; D017152:Antibodies, Antiphospholipid / Q000097:blood; D001323:Autoantibodies / Q000097:blood*; D001327:Autoimmune Diseases / Q000097:blood / Q000276:immunology*; D002096:C-Peptide / Q000097:blood; D003922:Diabetes Mellitus, Type 1 / Q000150:complications / Q000276:immunology*; D016883:Diabetic Ketoacidosis / Q000276:immunology*; D018450:Disease Progression; D005260:Female; D005951:Glucose Tolerance Test; D006442:Glycated Hemoglobin / Q000378:metabolism; D059848:HLA-DQ alpha-Chains / Q000097:blood; D059811:HLA-DRB1 Chains / Q000097:blood; D006801:Humans; D007004:Hypoglycemic Agents / Q000008:administration & dosage / Q000276:immunology*; D007231:Infant, Newborn; D007328:Insulin / Q000008:administration & dosage / Q000276:immunology*; D011247:Pregnancy; D011256:Pregnancy Outcome; D011254:Pregnancy in Diabetics / Q000097:blood / Q000276:immunology*; D013577:Syndrome
chemical_list: D017152:Antibodies, Antiphospholipid; D001323:Autoantibodies; D002096:C-Peptide; D006442:Glycated Hemoglobin; D059848:HLA-DQ alpha-Chains; D059811:HLA-DRB1 Chains; D007004:Hypoglycemic Agents; D007328:Insulin; C069050:HLA-DQA1 antigen; C553502:HLA-DRB1*03:01 antigen; C517652:hemoglobin A1c protein, human