Journal Information
Vol. 79. Issue 3.
Pages 202-203 (July - September 2014)
Vol. 79. Issue 3.
Pages 202-203 (July - September 2014)
Clinical image in Gastroenterology
DOI: 10.1016/j.rgmxen.2014.09.002
Open Access
Pierre Mauriac syndrome and uncontrolled type 1 diabetes mellitus
Síndrome de Pierre Mauriac y diabetes mellitus tipo 1 descontrolada
D. Espinoza-Peraltaa, F.J. Gutierrez-Llamasb, I. García-Juárezc,
Corresponding author

Corresponding author: Calle Vasco de Quiroga No 15, Departamento de Gastroenterología, Sección 16, Distrito Federal, México, CP 14000.
a Departamento de Endocrinología y Metabolismo, Instituto Nacional de Ciencias Médicas y Nutrición «Salvador Zubirán», Mexico City, Mexico
b Departamento de Patología, Instituto Nacional de Ciencias Médicas y Nutrición «Salvador Zubirán», Mexico City, Mexico
c Departamento de Gastroenterología, Instituto Nacional de Ciencias Médicas y Nutrición «Salvador Zubirán», Mexico City, Mexico
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A 26-year-old woman was diagnosed with type 1 diabetes mellitus (DM1) at the age of 5 years. Her disease was chronically uncontrolled despite multiple insulin regimens, with HbA1c levels of 12%, and she presented with retinopathy and peripheral neuropathy. Clinical symptoms of one-month duration were characterized by nausea, vomiting, and abdominal pain. Physical examination revealed very short stature, overweight, Cushingoid facies, and hepatomegaly. Liver function tests only showed increased transaminases (ALT 158 IU, AST 192 IU), and AF 265 IU. Viral or autoimmune etiology was ruled out. A noncontrast abdominal computed tomography (CT) scan revealed hepatomegaly with a shiny aspect (fig. 1).

Figure 1

Percutaneous liver biopsy revealed normal architecture with mild changes due to fat infiltration (fig. 2A). Periodic acid-Schiff (PAS) stain showed abundant glycogen deposits (fig. 2B) that disappeared after diastase digestion (fig. 2C).

Figure 2

Ultrastructural evaluation confirmed the presence of nuclear and cytoplasmic glycogen deposits (fig. 2D).

Pierre Mauriac syndrome was described in 1930 and is characterized by retarded growth, a Cushingoid appearance, hepatomegaly, and hypertransaminasemia, mainly in chronically uncontrolled DM1 patients. Diagnosis requires a high index of clinical suspicion and is corroborated through liver biopsy; staining techniques such as PAS show the presence of glycogen, which disappears with the application of diastase, and steatosis is occasionally observed.

The patient's hepatomegaly and hypertransaminasemia were resolved after 4 months of optimum glycemic control, with HbA1c levels of 7%.

Financial disclosure

No financial support was received in relation to this article.

Conflict of interest

The authors declare that there is no conflict of interest.

Please cite this article as: Espinoza-Peralta D, Gutierrez-Llamas FJ, García-Juárez I. Síndrome de Pierre Mauriac y diabetes mellitus tipo 1 descontrolada. Revista de Gastroenterología de México. 2014;79:202–203.

Copyright © 2014. Asociación Mexicana de Gastroenterología
Revista de Gastroenterología de México

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