Mauriac Syndrome: A Rare Hepatic Glycogenosis with Metabolic Bone Disease in Poorly Controlled Type 1 Diabetes

Turki Alharthi Samaher Ismail Nouf Alghofaili Saleh Alghamdi Elham Bin Abbas

We report a case of 20 year old female, diagnosed with type 1 diabetes mellitus (T1DM) when she was 5 years of age when she presented with Diabetic ketoacidosis, she had poor glycemic control as she had multiple hospital admissions due to diabetic ketoacidosis. During adolescence she presented with delay puberty, short stature and hepatomegaly. Which end diagnosing this patient by liver biopsy as Mauriac syndrome. Learning points • Mauriac Syndrome (MS) is a rare complication that occurs in patients with type 1 diabetes mellitus. • Herein, a patient with MS presented with additional features including low bone density and delayed puberty. • The early recognition and management of this syndrome may improve patient outcomes. • Continuous glucose monitoring may be an appropriate approach for treating these patients to improve clinical outcomes and avoid complications.