Definition:
Forbes Disease occurs in 1 for every 100,000 live births. It is also called Cori's type III glycogenosis. It was Gerty Theresa Radnitz Cori and Gilbert Burnett Forbes who developed an accurate description of this condition. This rare disease is due to a missing enzyme which causes abnormal glycogen levels to be absorbed by the body, namely the liver and the skeleton musculature.
Diagnosis:
People suspected of having this condition will need to have their enzyme levels tested, measured, and monitored. Genetic testing and screening may also be required.
Treatment:
Doctors may recommend dietary changes like increase in protein consumption. A high protein diet helps facilitate gluconeogenesis. Patients may be prescribed cornstarch in between meals or at night time. This helps avoid blood sugar levels to go down. In grave cases, liver transplants will be needed.
Symptoms and Signs:
Those who suffer from Forbes Disease show different symptoms. Those symptoms for GSD IIIa include muscle and liver involvement. Those symptoms for GSD IIIb have liver involvement but no muscle involvement. Symptoms of the disease generally present during infancy and usually include hypoglycemia. Others are hepatomegaly and muscular disease, cardiomyopathy, and hypotonia. A number of adult patients develop cirrhosis. Sufferers are observed to have excessive craving for carbohydrates and sweets.
Causes:
Forbes Disease is inherited, this is of Autosomal recessive inheritance and patients are frequent in Israel.
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