Mauriac syndrome--a modern reality

J Pediatr Endocrinol Metab. 2010 Mar;23(3):311-3. doi: 10.1515/jpem.2010.23.3.311.

Abstract

We report two cases of adolescent females with poorly controlled diabetes mellitus who were found to have gross hepatomegaly on annual review. With the additional findings of short stature (in one case), delayed puberty and a Cushingoid habitus they were diagnosed with Mauriac syndrome. Within our diabetes service we have incorporated regular abdominal examinations for all children and young people with long standing, poorly controlled diabetes (HbA1c persistently >9.5%). A brief review of the literature is included.

Publication types

  • Case Reports
  • Letter

MeSH terms

  • Adolescent
  • Diabetes Mellitus, Type 1* / drug therapy
  • Diagnosis, Differential
  • Female
  • Hepatomegaly*
  • Humans
  • Puberty, Delayed*
  • Syndrome