Thiamine responsive megaloblastic anemia syndrome, an autosomal recessive inherited disorder characterized by a triad of anemia, diabetes mellitus and sensorineural deafness is caused by a deficiency of a thiamine transporter protein. The disorder is rare and has not been reported from our community which has high background of consanguinity. We report a six years old girl who presented with diabetes mellitus which remitted after thiamine replacement. The girl in addition had sensorineural deafness, reinopathy, atrial septal defect and megaloblastic anemia which responded to high doses of thymine. This is the first case reported from Kashmir valley and third from India. The presentation and management in such cases is discussed.

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Diabetic myonecrosis is a rare and underdiagnosed complication of long-standing, uncontrolled diabetes. It usually occurs in patients with long-standing diabetes in the presence of microvascular complications. Thigh muscles are more commonly affected and the usual presentation is thigh swelling with or without pain, systemic features being rare. Magnetic resonance imaging is the gold standard for diagnosis. Most patients recover spontaneously with bed rest, adequate analgesia and good glycemic control. We present a case of recurrent myonecrosis of adductor muscles of the thigh in a patient with long standing type 2 diabetes mellitus who recovered with conservative management.