Clinical characterization of cardiovascular abnormalities associated with feline mucopolysaccharidosis I and VI

Summary Objective: The purpose of this study was to define the cardiovascular abnormalities present in young and adult cats affected with the lysosomal storage diseases mucopolysaccharidosis (MPS) I and MPS VI. Method: Eighteen cats affected with MPS I and 10 cats affected with MPS VI were evaluated...

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Bibliographic Details
Published in:	Journal of inherited metabolic disease Vol. 31; no. 3; pp. 424 - 431
Main Authors:	Sleeper, M. M., Kusiak, C. M., Shofer, F. S., O’Donnell, P., Bryan, C., Ponder, K. P., Haskins, M. E.
Format:	Journal Article
Language:	English
Published:	Dordrecht Springer Netherlands 01-06-2008 Springer Blackwell Publishing Ltd
Subjects:	Animals Biochemistry Biological and medical sciences Carbohydrates (enzymatic deficiencies). Glycogenosis Cardiovascular System - pathology Cardiovascular System - physiopathology Cats Echocardiography Electrocardiography Errors of metabolism Human Genetics Internal Medicine Medical genetics Medical sciences Medicine Medicine & Public Health Metabolic Diseases Mucopolysaccharidosis I - pathology Mucopolysaccharidosis I - physiopathology Mucopolysaccharidosis VI - pathology Mucopolysaccharidosis VI - physiopathology Original Article Pediatrics Enzyme Replacement Therapy Lysosomal Storage Disease Aortic Dilatation Aortic Valve Dermatan Sulfate Characterization Association Nutrition Diseases of the osteoarticular system Genetics Metabolic diseases Anomaly Carbohydrate Circulatory system Enzymopathy Maroteaux mucopolysaccharidosis Genetic disease
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Summary:	Summary Objective: The purpose of this study was to define the cardiovascular abnormalities present in young and adult cats affected with the lysosomal storage diseases mucopolysaccharidosis (MPS) I and MPS VI. Method: Eighteen cats affected with MPS I and 10 cats affected with MPS VI were evaluated by physical examination, electrocardiography and echocardiography. Electrocardiography (ECG) was performed on all MPS I and 9 of the MPS VI cats. Twelve unaffected cats underwent complete examinations for comparison purposes. Results: No cardiovascular abnormalities were noted on physical examination. Measured ECG intervals were normal in affected cats; however, sinus arrhythmia was noted more frequently than in the unaffected cats. Significant echocardiographic abnormalities included aortic valve thickening, regurgitation and aortic root dilation. Significant mitral valve thickening was also noted. The severity of changes increased in older affected cats. Conclusion: As affected animals increased in age, more cardiac abnormalities were found with increasing severity. Significant lesions included the mitral and aortic valves and ascending aorta, but myocardial changes were not recognized. MPS I and MPS VI cats have similar cardiovascular findings to those seen in children and constitute important models for testing new MPS therapies.
Bibliography:	Competing interests: None declared Communicating editor: Guy Besley ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	0141-8955 1573-2665
DOI:	10.1007/s10545-008-0821-1