Hepatomegaly and short stature in a 14-year-old with type 1 diabetes mellitus: case report

Author:

Fox Miriam T1,Tamaroff Jaclyn2,Percy Andrew G2,Baker Stephanie2,Altshuler Courtney2,Baluyot Mariju2,Stewart Rosalyn W23

Affiliation:

1. The Johns Hopkins University School of Medicine

2. The Johns Hopkins University School of Medicine, Department of Pediatrics

3. The Johns Hopkins University School of Medicine, Department of Medicine, Baltimore, MD, USA

Abstract

Abstract Background Mauriac syndrome is a rare consequence of poorly controlled insulin-dependent diabetes, characterized by hepatomegaly, growth failure, delayed onset of puberty, and cushingoid features. Case reports of patients with Mauriac syndrome are found infrequently in the literature given historic improvements in diabetes management due to readily available insulin therapy. Methods We describe a case of a 14-year-old girl who presented with acute onset abdominal pain, distention, and orthopnea. Results She had a history of poorly controlled insulin-dependent diabetes as well as short stature. Abdominal imaging revealed impressive hepatomegaly. Laboratory testing showed markedly elevated triglycerides and cholesterol. Mauriac syndrome was suspected and diagnosed by liver biopsy, which demonstrated significant glycogenic hepatopathy. Conclusions This case provides an illustrative example of Mauriac syndrome in a child who did not experience delayed onset of puberty and continued to have regular menses unlike what has been previously described. Furthermore, this case highlights the important consideration for significant dyslipidemia in patients with Mauriac syndrome and discusses the challenges of controlling insulin-dependent diabetes in the adolescent population.

Publisher

Oxford University Press (OUP)

Subject

Family Practice

Reference17 articles.

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2. Discovery of a genetic metabolic cause for Mauriac syndrome in type 1 diabetes;MacDonald;Diabetes,2016

3. Changes in liver volume from birth to adulthood: a meta-analysis;Johnson;Liver Transpl,2005

4. Fructosamine measurement for diabetes mellitus diagnosis and monitoring: a systematic review and meta-analysis protocol;Nansseu;BMJ Open,2015

5. Hepatic glycogenosis: an underdiagnosed complication of diabetes mellitus?;Julián;World J Diabetes,2015

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