Mauriac Syndrome Still Exists in Poorly Controlled Type 1 Diabetes: A Report of Two Cases and Literature Review

Mauriac syndrome is a very rare syndrome that occurs in poorly controlled type 1 diabetes mellitus with diabetic complications. Its cardinal features include delayed growth and puberty, hepatomegaly, and moon faces. These features were attributed mainly to insulin deficiency and sub-optimal diabetic management. Its incidence is decreasing due to the newer insulin formulation and intensive blood glucose control. Early recognition and management of this syndrome may improve the outcome of these patients. Recently, there are increasing reports of this syndrome. Here, we present the cases of two adolescent males with type 1 diabetes who presented with the classical features of Mauriac syndrome.

[1]  G. Salzano,et al.  Hepatomegaly and type 1 diabetes: a clinical case of Mauriac’s syndrome , 2019, Italian Journal of Pediatrics.

[2]  S. Stoker,et al.  Discovery of a Genetic Metabolic Cause for Mauriac Syndrome in Type 1 Diabetes , 2016, Diabetes.

[3]  Y. Vijay,et al.  Mauriac syndrome: A preventable complication of type 1 diabetes mellitus , 2014 .

[4]  K. Gupta,et al.  Re-emergence of a rare syndrome: A case of mauriac syndrome , 2013, Indian journal of endocrinology and metabolism.

[5]  J. Quintos,et al.  Mauriac syndrome: growth failure and type 1 diabetes mellitus. , 2008, Pediatric endocrinology reviews : PER.

[6]  J. Quintos,et al.  Mauriac syndrome in a 3-year-old boy. , 2007, Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists.

[7]  H. Bode,et al.  Stunted growth and hepatomegaly in diabetes mellitus. , 1977, The Journal of pediatrics.