Mauriac syndrome

Mauriac Syndrome is a rare complication of Type 1 diabetes that was first described by the French pediatrician Maurice Mauriac in 1930. It is characterized by growth retardation, delayed puberty, and hepatomegaly in children and adolescents with poorly controlled diabetes.

Symptoms and Signs

The primary symptoms of Mauriac Syndrome include hepatomegaly (enlarged liver), Cushingoid features (such as a round face and obesity), growth retardation, and delayed puberty. Other symptoms may include dwarfism, osteoporosis, and elevated liver enzymes.

Causes

Mauriac Syndrome is caused by poor control of blood sugar levels in individuals with Type 1 diabetes. The exact mechanism is not fully understood, but it is believed to involve the effects of chronic hyperglycemia on the liver and other organs.

Diagnosis

The diagnosis of Mauriac Syndrome is based on the presence of characteristic symptoms in a patient with poorly controlled Type 1 diabetes. Laboratory tests may show elevated liver enzymes and abnormal liver function. Imaging studies such as ultrasound or computed tomography (CT) scan may reveal hepatomegaly.

Treatment

The primary treatment for Mauriac Syndrome is improved control of blood sugar levels. This may involve changes to the patient's insulin regimen, diet, and exercise habits. In some cases, treatment may also include medications to manage symptoms such as osteoporosis or elevated liver enzymes.

Prognosis

With proper management of blood sugar levels, the prognosis for Mauriac Syndrome is generally good. However, long-term complications can occur if the condition is not properly managed, including liver disease and bone loss.

See Also

• Type 1 diabetes
• Hepatomegaly
• Cushingoid

NIH genetic and rare disease info

• NIH info on Mauriac syndrome

Mauriac syndrome is a rare disease.

Mauriac syndrome Resources
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