Mauriac syndrome in a 3-year-old boy

Endocr Pract. 2007 Jan-Feb;13(1):63-6. doi: 10.4158/EP.13.1.63.

Abstract

Objective: To report a case of Mauriac syndrome in a young child with poorly controlled type 1 diabetes mellitus.

Methods: We describe the typical features of Mauriac syndrome and review the clinical, laboratory, and ultrasound findings and follow-up results in our current patient.

Results: Hepatomegaly, growth impairment, and cushingoid features characterize Mauriac syndrome. Most frequently, it is seen in adolescents and young adults with a history of poor glycemic control. In our current patient, a 3-year-old boy with type 1 diabetes mellitus of 2 years' duration, Mauriac syndrome developed in the context of a disrupted social environment. His liver enzyme derangement, hepatomegaly, and growth improved once euglycemia was achieved.

Conclusion: Although Mauriac syndrome is currently uncommon, close monitoring of children with type 1 diabetes is important to maintain optimal glycemic control and prevent such an occurrence.

Publication types

  • Case Reports

MeSH terms

  • Child, Preschool
  • Diabetes Mellitus, Type 1 / diagnosis*
  • Diabetes Mellitus, Type 1 / drug therapy*
  • Diabetes Mellitus, Type 1 / physiopathology
  • Growth Disorders / diagnosis*
  • Growth Disorders / physiopathology
  • Hepatomegaly / diagnosis*
  • Hepatomegaly / physiopathology
  • Humans
  • Hyperglycemia / diagnosis
  • Hyperglycemia / drug therapy
  • Hyperglycemia / physiopathology
  • Hypoglycemic Agents / therapeutic use*
  • Insulin / therapeutic use*
  • Male
  • Syndrome

Substances

  • Hypoglycemic Agents
  • Insulin