\fˈɔːbz dɪzˈiːz], \fˈɔːbz dɪzˈiːz], \f_ˈɔː_b_z d_ɪ_z_ˈiː_z]\
Definitions of FORBES DISEASE
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An autosomal recessive metabolic disorder due to deficient expression of amylo-1,6-glucosidase (one part of the glycogen debranching enzyme system). The clinical course of the disease is similar to that of glycogen storage disease type I, but milder. Massive hepatomegaly, which is present in young children, diminishes and occasionally disappears with age. Levels of glycogen with short outer branches are elevated in muscle, liver, and erythrocytes. Six subgroups have been identified, with subgroups Type IIIa and Type IIIb being the most prevalent.
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