Re-emergence of a rare syndrome: A case of mauriac syndrome
A 15-year-old Type 1 diabetic boy was referred to us for evaluation of short stature. He was diagnosed to have T1DM, following an episode of diabetic ketoacidosis (DKA) 8 years back, and was started on premix (30/70) insulin. He was irregular with the treatment, with 4 hospitalisations for DKA till date.\nExamination showed that he was significantly short for his age, height 131 cms (less than 3rd percentile), weight 28 kg (less than 3rd percentile) and body mass index of 16.31. Secondary sexual characteristic A1, P1, testicular volume less than 4 ml bilaterally. He had abdominal fat deposition [Figures and ] and liver was palpable clinically 5 cms below costal margin [] with no splenomegaly or free-fluid. There were no signs of chronic liver failure.\nInvestigations showed haemoglobin of 13.5 gm/dl. Liver function tests, renal function tests, and urine routine examination were normal. Ultrasound abdomen showed liver enlargement 15.5 cms with increased echotexture, with normal spleen and portal vein and no free-fluid. On evaluation for diabetic status, he had an HbA1c of 10.3%, urine microalbumin 70 μg/gm of creatinine, and fundus showing background diabetic retinopathy. He had a bone age of 10.2 years, (Tanner Whitehouse 2) thyroid-stimulating hormone (TSH) of 2.4 mIU/ml and T4 of 8.8 microgm/dl. His growth hormone (GH) stimulation test and overnight dexa methasone suppression test were normal (after blood glucose control and testosterone priming).\nBased on the clinical history and investigations, the final diagnosis of Mauriac syndrome was made and the patient was advised tight control of sugars. He was switched over to basal bolus regime, with Glargine in the night and three doses of short-acting insulin before meals. He was follow-up for 6 months. He had shown reduction in hepatomegaly and a height gain of 3 cms. After that he was lost to follow-up.