[Mauriac syndrome--a rare complication of type 1 diabetes mellitus].
The treatment of children with type 1 diabetes mellitus has improved dramatically over the last few decades. The maintenance of acceptable metabolic control, nevertheless, remains challenging because the success of treatment is so dependent on patient compliance. Children with type 1 diabetes and poor metabolic control are at risk of developing Mauriac syndrome, a condition characterised by hepatomegaly, growth retardation and cushingoid features. A similar complication may occur in type-1 or type-2 adult diabetics; namely, glycogenic hepatopathy. We describe two children, a 12-year-old girl and a 16-year-old boy, who presented with classic symptoms of Mauriac syndrome. After metabolic control was achieved, reduction of hepatomegaly and the disappearance of cushingoid features were observed, proving the reversibility of the syndrome. Awareness that this syndrome still exists despite improved insulin therapy is crucial for earlier recognition and treatment.
Sophia Kinderziekenhuis, afd. IC kinderen, Erasmus MC, Rotterdam, the Netherlands. anne_schmetz@hotmail.com
Anne V N
AV
Schmetz
Mariëlle H
MH
Dekker-Maas
Marianne P
MP
den Breejen
Theo C J
TC
Sas
D000293:Adolescent; D001786:Blood Glucose / Q000378:metabolism; D002648:Child; D003922:Diabetes Mellitus, Type 1 / Q000150:complications* / Q000188:drug therapy; D042241:Early Diagnosis; D005260:Female; D006130:Growth Disorders / Q000175:diagnosis / Q000209:etiology*; D006529:Hepatomegaly / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D007004:Hypoglycemic Agents / Q000627:therapeutic use*; D007328:Insulin / Q000627:therapeutic use*; D008297:Male; D010349:Patient Compliance; D011379:Prognosis; D013577:Syndrome
D001786:Blood Glucose; D007004:Hypoglycemic Agents; D007328:Insulin