About The Medical Condition Debrancher Deficiencies
Find the definition of the medical term Debrancher Deficiencies. Debrancher Deficiencies defined and explained for easy understanding at the Medical Conditions from ClusterMed.info.
Debrancher Deficiencies
| Debrancher Deficiencies Medical Condition |
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What's The Definition Of The Medical Condition Debrancher Deficiencies?
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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