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    Complex management of a patient with refractory primary erythromelalgia lacking a SCN9A mutation

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    Name:
    JPR-129661-complex-management- ...
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    Author
    Low, Sarah
    Robbins, Wendye
    Tawfik, Vivianne
    Affiliation
    Univ Arizona, Coll Med, Dept Internal Med, Banner Univ Med Ctr
    Issue Date
    2017-04
    Keywords
    erythromelalgia
    chronic pain
    genetic testing
    sodium channels
    ketamine
    
    Metadata
    Show full item record
    Publisher
    DOVE MEDICAL PRESS LTD
    Citation
    Complex management of a patient with refractory primary erythromelalgia lacking a SCN9A mutation 2017, Volume 10:973 Journal of Pain Research
    Journal
    Journal of Pain Research
    Rights
    © 2017 Low et al. This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms. php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/).
    Collection Information
    This item from the UA Faculty Publications collection is made available by the University of Arizona with support from the University of Arizona Libraries. If you have questions, please contact us at repository@u.library.arizona.edu.
    Abstract
    A 41-year-old woman presented with burning and erythema in her extremities triggered by warmth and activity, which was relieved by applying ice. Extensive workup was consistent with adult-onset primary erythromelalgia (EM). Several pharmacological treatments were tried including local anesthetics, capsaicin, ziconotide, and dantrolene, all providing 24-48 hours of relief followed by symptom flare. Interventional therapies, including peripheral and sympathetic ganglion blocks, also failed. Thus far, clonidine and ketamine have been the only effective agents for our patient. Genetic testing was negative for an EM-associated mutation in the SCN9A gene, encoding the Na(V)1.7 sodium channel, suggesting a mutation in an alternate gene.
    Note
    Open Access Journal
    ISSN
    1178-7090
    PubMed ID
    28490900
    DOI
    10.2147/JPR.S129661
    Version
    Final published version
    Sponsors
    Foundation for Anesthesia Education and Research (FAER)
    Additional Links
    https://www.dovepress.com/complex-management-of-a-patient-with-refractory-primary-erythromelalgi-peer-reviewed-article-JPR
    ae974a485f413a2113503eed53cd6c53
    10.2147/JPR.S129661
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