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Aneurysm of the aortic root in the setting of Wiskott-Aldrich syndrome

Published online by Cambridge University Press:  01 April 2009

Masamichi Ono ,
Heidi Goerler  and
Thomas Breymann
Masamichi Ono*
Affiliation:
Division of Cardiothoracic, Transplantation, and Vascular Surgery, Hannover Medical School, Hannover, Germany
Heidi Goerler
Affiliation:
Division of Cardiothoracic, Transplantation, and Vascular Surgery, Hannover Medical School, Hannover, Germany
Thomas Breymann
Affiliation:
Division of Cardiothoracic, Transplantation, and Vascular Surgery, Hannover Medical School, Hannover, Germany
*
Correspondence to: Masamichi Ono, MD. Division of Cardiothoracic, Transplantation, and Vascular Surgery, Hannover Medical School, Carl-Neuberg-Str. 1, 30625 Hannover, Germany. Tel: +49-511-532 9397; Fax: +49-511-532 9832; E-mail: Ono.Masamichi@mh-hannover.de
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Abstract

Wiskott-Aldrich syndrome is a rare X-linked disease, associated with immunodeficiency, infections, thrombocytopaenia, and eczema. Aortitis and formation of aneurysms have also been described. We describe here our experience with a 7-year-boy with this syndrome. He survived replacement of the aortic root because of an aneurysmal ascending aorta, and subsequent bone marrow transplantation.

Keywords

Aortic valvecongenital cardiac surgerybone marrow transplantation
Type
Brief Report
Information
Cardiology in the Young , Volume 19 , Issue 2 , April 2009 , pp. 212 - 215
Copyright
Copyright © Cambridge University Press 2009

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References

1.Perry, GS, Spector, BD, Schumann, LM. The Wiskott-Aldrich syndrome in the United States and Canada (1892–1979). J Pediatr 1980; 97: 72–78.CrossRefGoogle ScholarPubMed
2.McCluggage, WG, Armstrong, DJ, Maxwell, RJ, Ellis, PK, McCluskey, DR. Systemic vasculitis and aneurysm formation in the Wiskott-Aldrich syndrome. J Clin Pathol 1999; 52: 390–392.Google Scholar
3.Johnston, SL, Unsworth, DJ, Dwight, JF, Kennedy, CT. Wiskott-Aldrich syndrome, vasculitis and critical aortic dilatation. Acta Pediatr 2001; 90: 1346–1348.Google Scholar
4.van Son, JAM, O’Marcaigh, AS, Edwards, WD, Julsrud, PR, Danielson, GK. Successful resection of thoracic aortic aneurysms in Wiskott-Aldrich syndome. Ann Thorac Surg 1995; 60: 685–687.CrossRefGoogle Scholar
5.Narayan, P, Alwair, H, Bryan, AJ. Surgical resection of sequential thoracic aortic aneurysms in Wiskott-Aldrich syndrome. Interact Cardiovasc Thorac Surg 2004; 3: 346–348.Google Scholar
6.Bernabeu, E, Josa, M, Nomdedeu, B, et al. One-step surgical approach of a thoracic aortic aneurysm in Wiskott-Aldrich syndrome. Ann Thorac Surg 2007; 83: 1537–1538.Google Scholar
7.Boztug, K, Baumann, U, Ballmaier, M, et al. Large granular lymphocyte proliferation and revertant mosaicism: two rare events in a Wiskott-Aldrich syndrome patient. Haematologica 2007; 92: e43–e45.CrossRefGoogle Scholar
8.Rose, C, Castillo, E, Wessel, A, et al. Morphological and functional MRI studies after correction of aortic isthmus stenosis. Z Kardiol 2002; 91: 161–168.Google Scholar
9.Mullen, CA, Anderson, KD, Blaese, RM. Splenectomy and/or bone marrow transplantation in the management of the Wiskott-Aldrich syndrome: long term follow up of 62 cases. Blood 1993; 82: 2961–2966.Google Scholar
10.Vricella, LA, Williams, JA, Revekes, WJ, et al. Early experience with valve-sparing aortic root replacement in children. Ann Thorac Surg 2005; 80: 1622–1627.CrossRefGoogle ScholarPubMed