Document Detail

Echocardiographic findings in some metabolic storage diseases.
MedLine Citation:
PMID:  7830328     Owner:  NLM     Status:  MEDLINE    
This study was planned to determine the presence and extent of cardiac involvement in metabolic storage diseases, including types I and II glycogenoses, Gaucher and Neimann-Pick diseases, galactosialidosis and mucopolysaccharidosis. M-mode, 2-dimensional and Doppler echocardiographic studies were used for the determination of left ventricular wall thickness, systolic function, anatomic derangement, valvular dysfunction and left ventricular diastolic function in the patient and control groups. In 19.35% of the cases valvular involvement, and in 59.26% thickening of the left ventricular wall due to metabolic storage, was found. Left ventricular systolic function was in the normal range. The diastolic mitral flow patterns of the cases showed obstructive type changes. It can be concluded that in this study, besides previously described cardiovascular changes, some new findings have been detected by means of echocardiography in patients with metabolic storage diseases.
F Senocak; M Sarçlar; S Ozkutlu
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Japanese heart journal     Volume:  35     ISSN:  0021-4868     ISO Abbreviation:  Jpn Heart J     Publication Date:  1994 Sep 
Date Detail:
Created Date:  1995-02-22     Completed Date:  1995-02-22     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0401175     Medline TA:  Jpn Heart J     Country:  JAPAN    
Other Details:
Languages:  eng     Pagination:  635-43     Citation Subset:  IM    
Department of Pediatric Cardiology, Hacettepe University, Ankara, Turkey.
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MeSH Terms
Child, Preschool
Echocardiography, Doppler
Gangliosidoses / ultrasonography
Gaucher Disease / ultrasonography
Glycogen Storage Disease / complications,  pathology,  ultrasonography*
Glycogen Storage Disease Type I / ultrasonography
Glycogen Storage Disease Type II / ultrasonography
Heart Valve Diseases / complications,  pathology,  ultrasonography
Mucopolysaccharidoses / ultrasonography
Myocardium / pathology
Ventricular Function, Left / physiology*

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