Syndrome de Turner et diabete insulinodependant

Background. - Cases of diabetes mellitus associated with Turner syndrome are non-insulin-dependent. We report a case of insulin-dependent diabetes mellitus (IDDM) with Turner syndrome. Case report. - An 11 year-old girl with Turner syndrome was investigated for glucose tolerance that was normal with glycosylated hemoglobin at 5.1%. The patient was then given growth hormone plus oxandrolone until the age of 15 yr 3 mo; estrogen replacement was initiated at 15 yr 9 mo and progesterone 6 months later. At the age of 16.5 yrs, the patient developed manifestations of diabetes mellitus with polyuria, polydipsia, weight loss. Blood glucose concentration was 415 mg/dl with ketoacidosis. She was given insulin; at that time, blood insulin level was 7 mU/ml, C-peptide 0.8 ng/ml and glycosylated hemoglobin Alc 8.2%. Islet cell auto-antibodies were positive ( 1 80) and histocompatibility antigens were A1, A2, B14, B18, CW7, DR7, DR11, DQ7. After 6 months of treatment, blood insulin and C-peptide after IV glucagon were nil. There was no familial case of IDDM. Conclusions. - This case of IDDM seems to be the first reported in Turner syndrome. This IDDM could be a manifestation of autoimmunity.