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Órgano Oficial del Instituto Nacional de Pediatría

2018, Number 6

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Acta Pediatr Mex 2018; 39 (6)

Mauriac syndrome: a case report

Pérez-Laínez SE, Rueda-Ochoa H, Deraz-Cabral S, Simental-Mendía LE

Language: Spanish
References: 16
Page: 349-354
PDF size: 1186.63 Kb.


ABSTRACT

Introduction: Mauriac syndrome is an uncommon condition characterized by growth retardation, hepatomegaly and obesity in children with type 1 diabetes.
Case Report: We describe an 11 years old female patient with type 1 diabetes of 8 years duration treated with glargine and rapid-acting insulin. She presented gradually increased abdominal volume of approximately 3 months duration and poor glycemic control over 6 months. In addition, she exhibited polydipsia and polyuria. Physical examination revealed growth retardation, pubertal delay and hepatomegaly. Markers of viral or autoimmune hepatitis were negatives. Finally, liver biopsy reported abundant glycogen deposition.
Conclusion: Although the Mauriac syndrome is an unusual condition, it should be suspected in patients with uncontrolled type 1 diabetes in order to establish a timely diagnosis and treatment.


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