Iram Musharaf, Aslam M, Waqar Hussain.
Mauriac Syndrome.
Proceeding Shaikh Zayed Postgrad Med Inst Jan ;15(2):103-4.

A 16 yrs old male presented with failure to gain height for 4 years and generalized swelling of the body for l week. The patient was a known case of type I diabetes mellitus for 13 years, diagnosed at the age of 3 years when he had polyuric. He was currently on combination insulin therapy but with poor glycemic control and no regular follow ups. On examination he was found to be short statured. His height was 141 cm and weight was 42 kg, both of them were less than 5th centile for his age. His blood pressure was 140/100 mmHg. He had stage 3 limited joint mobility, hyperpigmentation and hypertrophy of the injection site at left arm, periorbital swelling, ankle edema and stage 2 sexual maturation. He also had hepatomegaly but rest of the examination including the eye was normal. His investigations showed blood glucose level of 402 mg/dl. with +3 proteinuria. His liver function tests were normal BUN=40, creatinine=31ng/dl and total protein=5.2gm/dl, albumin=2.3 gm/dl. He also had high levels of Cholesterol= 355mg/dl, triglyceride=219, LDL=246 mg/dl. Thyroid function tests were normal. On X rays, the bone age was found to be of 13 years. Ultra sound abdomen showed an enlarged liver with fatty changes. A final diagnosis of uncontrolled diabetes was made along with Mauriac syndrome, lipohypertrophy and suspected diabetic nephropathy.

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