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The Mauriac syndrome.

E Y Morrison, K McKenzie

    The West Indian Medical Journal
    |September 1, 1989
    PubMed
    Summary
    This summary is machine-generated.

    This case study describes a young patient with insulin-dependent diabetes who experienced complications including liver enlargement and ketoacidosis. The symptoms resolved with improved diabetes management, but the patient later died suddenly.

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    Area of Science:

    • Pediatric Endocrinology
    • Metabolic Disorders

    Background:

    • Presents a case of a young patient diagnosed with insulin-dependent diabetes at age 7.
    • Highlights the challenges in achieving stable glycemic control in pediatric diabetes.

    Observation:

    • The patient experienced recurrent hyperglycemic ketoacidosis and developed tender hepatomegaly.
    • Hepatomegaly regressed upon stabilization of diabetes, but the patient exhibited growth failure below the tenth percentile.
    • Sudden death occurred at age 12.5, with post-mortem findings of ascites, hepatomegaly, and fatty liver changes.

    Findings:

    • The clinical presentation, including high insulin requirements (2.3 IU/kg/day), hepatomegaly, unstable diabetes, and growth failure, strongly suggests Mauriac syndrome.
    • Fatty metamorphosis of the liver was noted on post-mortem examination.

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    Implications:

    • This case underscores the potential for severe complications in poorly controlled pediatric diabetes.
    • Emphasizes the importance of vigilant monitoring and management to prevent long-term sequelae like Mauriac syndrome.
    • Highlights the link between chronic hyperglycemia and hepatic complications in diabetic children.