Revisit of a rare complication of type 1 diabetes mellitus: Mauriac syndrome

摘要

Mauriac syndrome is an uncommon complication of poorly-controlled type 1 diabetes mellitus (T1DM) reported in children 13-17 years of age. It manifests as delayed growth, hepatomegaly, elevated liver enzymes and serum lipids, and glycogen accumulation in hepatocytes. Common presenting features include short stature, growth retardation, moon facies, protuberant abdomen and proximal muscle wasting. Since the advent of long- and intermediate-acting insulin, this syndrome is now rarely encountered.We report the case of a 16-year-old male who had a 13-year history of T1 DM and was diagnosed with Mauriac syndrome. He was treated with biphasic isophane insulin and recovered - good glycaemic control was maintained, his height increased from 128cm to 141.5cm, hepatomegaly disappeared with normal liver enzymes, and the onset of pubertal spurt at eight months' follow up was shown.in conclusion, ensuring compliance with insulin therapy, and providing supportive treatment, nutritional support and good follow-up care can help to mitigate most of the complications relating to Mauriac syndrome.