Hostname: page-component-78c5997874-j824f Total loading time: 0 Render date: 2024-11-10T06:52:30.703Z Has data issue: false hasContentIssue false
  • English
  • Français

Aortic stenosis in a patient with Hurler's syndrome after bone marrow transplantation

Published online by Cambridge University Press:  25 January 2011

Naruhito Watanabe ,
Petros V. Anagnostopoulos  and
Anthony Azakie
Naruhito Watanabe
Affiliation:
Department of Surgery, Division of Pediatric Cardiac Surgery, UCSF Benioff Children's Hospital, University of California San Francisco, San Francisco, California, United States of America
Petros V. Anagnostopoulos
Affiliation:
Department of Surgery, Division of Pediatric Cardiac Surgery, UCSF Benioff Children's Hospital, University of California San Francisco, San Francisco, California, United States of America
Anthony Azakie*
Affiliation:
Department of Surgery, Division of Pediatric Cardiac Surgery, UCSF Benioff Children's Hospital, University of California San Francisco, San Francisco, California, United States of America
*
Correspondence to: Dr A. Azakie, MD, UCSF Benioff Children's Hospital, University of California San Francisco, 513 Parnassus Avenue, San Francisco, California 94143, United States of America. Tel: 415 476 3501, Fax: 415 476 9678; E-mail: Tony.Azakie@ucsfmedctr.org
Get access Rights & Permissions [Opens in a new window]

Abstract

We describe a case of severe aortic stenosis in a 16-year-old male with Hurler's syndrome who had prior bone marrow transplantation. The excised aortic valve leaflets showed characteristic pathologic findings of Hurler's syndrome. This is the first case report of aortic valve replacement in a patient with Hurler's syndrome treated with bone marrow transplantation that demonstrates progression of the aortic valve disease despite treatment.

Keywords

Mucopolysaccharidosisaortic valve replacementvalvular disease
Type
Brief Reports
Information
Cardiology in the Young , Volume 21 , Issue 3 , June 2011 , pp. 349 - 350
Copyright
Copyright © Cambridge University Press 2011

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Lowry, RB, Applegarth, DA, Toone, JR, MacDonald, E, Thunem, NY. An update on the frequency of mucopolysaccharide syndromes in British Columbia. Hum Genet 1990; 86: 389–390.Google Scholar
2. Moore, D, Connock, MJ, Wraith, E, Lavery, C. The prevalence of and survival in mucopolysaccharidosis I: Hurler, Hurler-Scheie, and Scheie syndromes in the UK. Orphanet J Rare Dis 2008; 3: 24.Google Scholar
3. Hobbs, JR, Hugh-Jones, K, Barrett, AJ, et al. Reversal of clinical features of Hurler's disease and biochemical improvement after treatment by bone-marrow transplantation. Lancet 1981; 2: 709–712.CrossRefGoogle ScholarPubMed
4. Gatzoulis, MA, Vellodi, A, Redington, AN. Cardiac involvement in mucopolysaccharidoses: effects of allogeneic bone marrow transplantation. Arch Dis Child 1995; 73: 259–260.CrossRefGoogle ScholarPubMed
5. Braunlin, EA, Stauffer, NR, Peters, CH, et al. Usefulness of bone marrow transplantation in the Hurler syndrome. Am J Cardiol 2003; 92: 882–886.CrossRefGoogle ScholarPubMed