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Debrancher Enzyme Deficiency (DBD) (Also known as Cori's or Forbes' Disease)
Definition - one of a group of muscle diseases
that interfere with the processing of carbohydrates to draw energy from food
Cause - a genetic defect in the debrancher enzyme, which affects the
breakdown of glycogen, the stored form of glucose (sugar)
Onset - childhood to adulthood
Symptoms - principally affects the liver, causing swelling of the liver,
slowing of growth, low blood sugar levels and, sometimes, seizures; heart can
be affected
Progression - slow except in severe, infantile form
Inheritance - autosomal recessive, or caused by the contribution of a
defective gene from each parent
