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Syndrome reference sheet
Generated 13 Oct 2026
Endocrinology · Endocrinology / Diabetes

Mauriac syndrome

Also known as: Glycogenic hepatopathy with growth failure in diabetes

Growth failure, hepatomegaly and delayed puberty in poorly controlled type 1 diabetes.

Medical specialty
Endocrinology
Subspecialty
Endocrinology / Diabetes
Sex & age distribution
Male: affected; Female: affected; Children: yes, children and adolescents
Gene(s) involved
None recorded

Etiology & mechanism

Chronic hyperglycemia with glycogen deposition in liver.

Gene(s) involved (0)

No gene recorded.

Symptoms & signs (6)

cushingoid featuresdelayed pubertyelevated liver enzymeshepatomegalypoor glycemic controlshort stature
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