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Aortic dissection and cystinosis: is there any relationship?

Published online by Cambridge University Press:  11 May 2017

Masih Tajdini ,
Mehdi Bayati  and
Ali Vasheghani-Farahani
Masih Tajdini*
Affiliation:
Tehran Heart Center and School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
Mehdi Bayati
Affiliation:
Tehran Heart Center and School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
Ali Vasheghani-Farahani
Affiliation:
Tehran Heart Center and School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
*
Correspondence to: M. Tajdini, MD, Cardiology Resident, Tehran Heart Center, Tehran University of Medical Sciences, North Kargar Ave., Tehran 1411713138, Iran. Tel: +98 21 8802 9256; Fax: +98 21 8802 9702; E-mail: [email protected]
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Abstract

Cystinosis is a rare, autosomal-recessive genetic disorder. The kidneys are commonly involved, as there is cystinosin protein malfunction, and nephropathic cystinosis ensues. Although cardiac and vascular involvements are rare, we describe a unique case of aortic dissection in a 25-year-old female with cystinosis. We discuss the possible aetiologies of aortic dissection in this condition.

Keywords

Cystinosisaortic dissectionyoung adult
Type
Brief Report
Information
Cardiology in the Young , Volume 27 , Issue 7 , September 2017 , pp. 1434 - 1436
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Copyright
© Cambridge University Press 2017 

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References

1. Shotelersuk, V, Larson, D, Anikster, Y, et al. CTNS mutations in an American-based population of cystinosis patients. Am J Hum Genet 1998; 63: 1352–1362.Google Scholar
2. Nesterova, G, Gahl, WA. Cystinosis: the evolution of a treatable disease. Pediatr Nephrol 2013; 28: 51–59.Google Scholar
3. Kaddourah, A, Uthup, S, Madueme, P, et al. Prevalence and predictors of aortic dilation as a novel cardiovascular complication in children with end-stage renal disease. Clin Nephrol 2015; 83: 262.Google Scholar
4. Edelman, M, Silverstein, D, Strom, J, Factor, SM. Cardiomyopathy in a male with cystinosis. Cardiovasc Pathol 1997; 6: 43–47.CrossRefGoogle Scholar
5. Ueda, M, O’Brien, K, Rosing, DR, et al. Coronary artery and other vascular calcifications in patients with cystinosis after kidney transplantation. Clin J Am Soc Nephrol 2006; 1: 555–562.CrossRefGoogle ScholarPubMed
6. Fikar, CR. Acute aortic dissection in children and adolescents: diagnostic and after‐event follow‐up obligation to the patient and family. Clin Cardiol 2006; 29: 383–386.Google Scholar
7. Groothoff, JW, Lilien, MR, van de Kar, NC, et al. Cardiovascular disease as a late complication of end-stage renal disease in children. Pediatr Nephrol 2005; 20: 374–379.Google Scholar
8. Strayer, DS. Cystinosis and a dissecting aortic aneurysm in a 7-year-old boy. Am J Dis Child 1979; 133: 436–438.Google Scholar
9. Golledge, J, Norman, PE. Current status of medical management for abdominal aortic aneurysm. Atherosclerosis 2011; 217: 57–63.Google Scholar
10. Hagan, PG, Nienaber, CA, Isselbacher, EM, et al. The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. JAMA 2000; 283: 897–903.Google Scholar