There was a rubella epidemic in 1964–1965 in Okinawa, Japan. By 2004, 280 subjects were diagnosed with congenital rubella syndrome (CRS). All 280 patients followed over this 40-year period developed cataracts, sensory deafness, and/or heart disease. We measured islet cell surface antibody (ICSA), islet cell antibody (ICA), and anti-GAD65, IA-2, and insulin antibodies. Three (1.1%) (patients 1, 2, and 3) of these 280 CRS patients developed type 1 diabetes (positive autoantibodies to pancreatic β-cells). Patients 1 and 2 had diabetic ketoacidosis and type 1 diabetes. Patient 3 was diagnosed with diabetes at 18 years of age and later found to have type 1 diabetes. Endogenous insulin response at the onset of diabetes was maintained in two patients (2 and 3). However, all three patients later required insulin administration. They had autoantibodies to pancreatic β-cells and developed type 1 diabetes.
Patient 1 is a 40-year-old woman born in 1965. She had cataracts, sensory deafness, and mutism. At 18 years of age, her fasting plasma glucose was 68 mg/dl. At 21 years of age, she developed diabetic ketoacidosis and was diagnosed with type 1 diabetes. She had ICSA, ICA, anti-GAD65Ab, and anti-IA2Ab, as well as goiter and thyroid-antibodies. She had Hashimoto’s thyroiditis and developed hypothyroidism, for which she was treated with thyroxine.
Patient 2 is a 40-year-old woman born in 1965. She had cataracts, sensory deafness, and mutism. At 13 years of age, in May 1979, she complained of thirst and polyuria and developed diabetic ketoacidosis. She was treated with insulin. In September 1979, she again developed diabetic ketoacidosis. She was diagnosed with and treated for type 1 diabetes. She had ICSA.
Patient 3 is a 40-year-old man born in 1965. He had cataracts, sensory deafness, and mutism. He also had atrial septal defect. He was diagnosed with diabetes at 18 years of age. He later developed type 1 diabetes and is being treated with insulin. He had antibodies to ICSA and anti-GAD65Ab.
Patients 1 and 2 had diabetic ketoacidosis and were diagnosed with type 1 diabetes, and Patient 3 was found to have diabetes upon screening, later developed insulin-dependent diabetes, and now requires insulin. All three subjects developed type 1 diabetes. The age of diabetes onset was 21, 13, and 18 years, respectively. They had autoantibodies to pancreatic β-cells and type 1 diabetes. Total amount of insulin required ranged from 32 to 47 units per day. Patient 1 had Hashimoto’s hypothyroidism.
Although the prevalence of diabetes in CRS was reported to be 20% in Caucasians (1), it is 1.1% in Japanese. The prevalence is 20 times higher in Caucasians than in Japanese in subjects with CRS, as seen in those without CRS (22–29 cases per 100,000 in Scandinavians, Canadians, and Scots and 1.5 per 100,000 in Japanese) (2). The present study was undertaken according to the principles of the Declaration of Helsinki. Informed consent was obtained from all participants.
