Endocrine Abstracts

A male patient aged 63 years old was admitted to the emergency department with the complaints of polydipsia, polyurea and dizziness. He had hypertension which was on a calcium channel blocker and his hypertension was in control. His plasma glucose was measured as 564 mg/dl and hospitalized due to his new onset diabetes. His family history showed that, his mother, uncle, aunt and all six brothers were diagnosed as diabetes mellitus. His uncle, aunt and aunts children were using oral antidiabetics. His system query showed that he had pollakuria and nocturiafor the last 3 months. His physical examination was normal except diminished skin turgor tonus. His laboratory was normal except blood glucose of 564 mg/dl, HbA1c 8.2%, urea 50 mg/dl, creatinin od 1.34 mg/dl, urine ketone bodies were +++, urine glucose 500 mg/dl. He was treated with i.v. saline, and insulin. On the follow up basal-bolus regimen was initiated. He had hypoglycemias due to four units bolus insulin and 14 units basal insulin glargine so bolus insulin was stopped and vildagliptine-metformin and insulin glargine was added. He had hypoglycemias as well and insulin glargine was stooped and gliclazide MR 60 mg was started and discharged. After 2 weeks his HbA1c was 6.9%, fasting plasma glucose was 122 mg/dl so vildagliptine-metformin was stopped and gliclazide was tapered to 30 mg/day. After 3 months his fasting blood glucose was 98 mg/dl and HA1c was 5.1%. His peripheral blood analysis was consistent with HNF1A gene and diagnosed as MODY 3. Transcription factor defects are the most common reasons of the MODY. Insulin secretion is decreased up to 85% but is sensitive to sulfonylureas. Low dose sulfonylureas generally control glycemia very well. But diabetic ketoasidosis is a very rare complication for MODY 3.