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Medicine C.V.S 359694d9

Cardiomyopathy may be seen in all of the following except

A
Duchenne muscular dystrophy
B
Friedrich's ataxia
C
Type IUI glycogen storage disease
D
Alkaptonuria
High-Yield Explanation
Friedreich ataxia (FA) is an autosomal recessively inherited neurodegenerative disease that most often presents in childhood or in young adulthood. A substantial propoion of patients with FA also develop a cardiomyopathy that usually presents as left ventricular hyperophy (FA-CM Duchenne Muscular Dystrophy (DMD) is a rare, X linked condition with progressive muscle weakness and accompanying cardiomyopathy. ... Becker Muscular Dystrophy, like DMD, is caused by dystrophin mutations and often also includes dilated cardiomyopathy but with variable progression Glycogen storage disease type III (GSD III) due to debranching enzyme deficiency presenting usually with hepatomegaly and hypoglycemia may be responsible for severe cardiomyopathy which is often fatal. Current treatment of GSD III is based on frequent high-carbohydrate meals that have no effect on the cardiomyopathy. Ref Davidson 23rd edition pg 445

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