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December 1, 1981Journal of Inherited Metabolic Disease

Familial AMP deaminase deficiency with skeletal muscle type I atrophy and fatal cardiomyopathy

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Key result

Muscular AMP deaminase deficiency is linked to skeletal myopathy and dilated cardiomyopathy, suggesting autosomal dominant inheritance.

Why the study?

Familial muscular AMP deaminase deficiency associated with skeletal muscle type I atrophy and dilated cardiomyopathy was identified but not well characterized.

Population

Two siblings with skeletal myopathy and dilated cardiomyopathy

Design

Case series

Authors

HSH. R. ScholteHBH. F. M. BuschILI. E. M. Luyt‐Houwen

Discussion

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Member takes

Overview

Alerts clinicians to possible cardiac involvement in AMPD deficiency; leaves open confirmation of autosomal dominant inheritance.

Structured PICO

P
Population
Two siblings suffering from a skeletal myopathy (characterized by type I fibre atrophy) and a dilated cardiomyopathy.
O
Outcome
Identification of muscular AMP deaminase deficiency and inheritance pattern

Reports a familial case of muscular AMP deaminase deficiency presenting with skeletal myopathy and fatal dilated cardiomyopathy, suggesting autosomal dominant inheritance.

Cite This Study

Scholte et al. (1981) studied this question. Muscular AMP deaminase deficiency was associated with skeletal myopathy, type I fiber atrophy, and dilated cardiomyopathy in two siblings, suggesting autosomal dominant inheritance.

synapsesocial.com/papers/6aaa3df81a1d6c962db43e7fhttps://doi.org/10.1007/bf02263644
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Also Consider

Synapse has enriched one closely related paper. Consider it for comparative context:

  1. 1Myoadenylate Deaminase Deficiency: A New Disease of Muscle1978 · 266 citations