Document Detail

Prevalence and Characterization of Cardiac Involvement in Hunter Syndrome.
MedLine Citation:
PMID:  21529823     Owner:  NLM     Status:  Publisher    
OBJECTIVES: To assess the prevalence of cardiovascular signs and symptoms in a large group of patients with Hunter syndrome, an X-linked metabolic disorder caused by a deficiency of the lysosomal enzyme iduronate-2-sulfatase. STUDY DESIGN: The Hunter Outcome Survey was established to characterize the natural history of Hunter syndrome and to assess the response to enzyme replacement therapy. Echocardiographic and electrocardiographic examination results were available for 102 patients who were enzyme replacement therapy-naïve in the Hunter Outcome Survey (median age at examination, approximately 8 years) as of Jan 23, 2009. RESULTS: The most common cardiovascular finding was valve disease, which was present in 63% of patients. Left ventricular hypertrophy (defined as left ventricular mass indexed to height(2.7) ≥50 g/m(2.7)) was found in 48% of patients <18 years old. Elevated blood pressure (defined as a Z score ≥2 for systolic blood pressure or diastolic blood pressure) was present in 25% of patients <18 years old. Other findings included abnormal heart frequency (7%), arrhythmia (5%), and congestive heart failure (6%). CONCLUSIONS: Treating physicians should be aware of the early emergence of cardiovascular manifestations in patients with Hunter syndrome so that appropriate treatment can be initiated.
Christoph Kampmann; Michael Beck; Isabelle Morin; James P Loehr
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-4-27
Journal Detail:
Title:  The Journal of pediatrics     Volume:  -     ISSN:  1097-6833     ISO Abbreviation:  -     Publication Date:  2011 Apr 
Date Detail:
Created Date:  2011-5-2     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0375410     Medline TA:  J Pediatr     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2011 Mosby, Inc. All rights reserved.
Division of Cardiology and Division of Lysosomal Storage Diseases, University Children's Hospital, Mainz, Germany.
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