• Login
    View Item 
    •   Home
    • UA Faculty Research
    • UA Faculty Publications
    • View Item
    •   Home
    • UA Faculty Research
    • UA Faculty Publications
    • View Item
    JavaScript is disabled for your browser. Some features of this site may not work without it.

    Browse

    All of UA Campus RepositoryCommunitiesTitleAuthorsIssue DateSubmit DateSubjectsPublisherJournalThis CollectionTitleAuthorsIssue DateSubmit DateSubjectsPublisherJournal

    My Account

    LoginRegister

    About

    AboutUA Faculty PublicationsUA DissertationsUA Master's ThesesUA Honors ThesesUA PressUA YearbooksUA Catalogs

    Statistics

    Display statistics

    Nephropathic cystinosis associated with cardiomyopathy: A 27-year clinical follow-up

    • CSV
    • RefMan
    • EndNote
    • BibTex
    • RefWorks
    Thumbnail
    Name:
    1471-2369-3-8.pdf
    Size:
    294.6Kb
    Format:
    PDF
    Download
    Author
    Dixit, Mehul
    Greifer, Ira
    Affiliation
    Division of Nephrology, Department of Pediatrics, University of Arizona, Tucson, USA
    Division of Nephrology, Department of Pediatrics, Albert Einstein College of Medicine, Bronx, USA
    Issue Date
    2002
    Keywords
    Nephropathic cystinosis
    Renal transplant
    Cardiomyopathy
    Pseudoaneurysm
    Long term survivor
    
    Metadata
    Show full item record
    Publisher
    BioMed Central
    Citation
    BMC Nephrology 2002, 3:8 http://www.biomedcentral.com/1471-2369/3/8
    Journal
    BMC Nephrology
    Rights
    © 2002 Dixit and Greifer; licensee BioMed Central Ltd. This is an Open Access article: verbatim copying and redistribution of this article are permitted in all media for any purpose, provided this notice is preserved along with the article's original URL.
    Collection Information
    This item is part of the UA Faculty Publications collection. For more information this item or other items in the UA Campus Repository, contact the University of Arizona Libraries at repository@u.library.arizona.edu.
    Abstract
    BACKGROUND:Nephropathic cystinosis is an autosomal recessive disease resulting from intracellular accumulation of cystine leading to multiple organ failure.CASE REPORT:We describe the clinical course of a patient managed from the age of six until his death at the age of 33 years. He underwent multiple surgery, including two renal transplants, developed transplant renal artery stenosis that was managed medically, and progressive heart failure at the age of 33 years. His death from a ruptured pseudoaneurysm associated with a restrictive cardiomyopathy is noteworthy. A limited cardiac autopsy revealed the presence of cystine crystals in interstitial cardiac histiocytes and one myocardial cell, along with 1000-fold higher tissue cystine content of the left ventricular myocardium compared to patients without cystinosis, suggesting the possibility of direct cystine mediated metabolic injury.
    EISSN
    1471-2369
    DOI
    10.1186/1471-2369-3-8
    Version
    Final published version
    Additional Links
    http://www.biomedcentral.com/1471-2369/3/8
    ae974a485f413a2113503eed53cd6c53
    10.1186/1471-2369-3-8
    Scopus Count
    Collections
    UA Faculty Publications

    entitlement

     
    The University of Arizona Libraries | 1510 E. University Blvd. | Tucson, AZ 85721-0055
    Tel 520-621-6442 | repository@u.library.arizona.edu
    DSpace software copyright © 2002-2017  DuraSpace
    Quick Guide | Contact Us | Send Feedback
    Open Repository is a service operated by 
    Atmire NV
     

    Export search results

    The export option will allow you to export the current search results of the entered query to a file. Different formats are available for download. To export the items, click on the button corresponding with the preferred download format.

    By default, clicking on the export buttons will result in a download of the allowed maximum amount of items.

    To select a subset of the search results, click "Selective Export" button and make a selection of the items you want to export. The amount of items that can be exported at once is similarly restricted as the full export.

    After making a selection, click one of the export format buttons. The amount of items that will be exported is indicated in the bubble next to export format.