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Secondary Combined Immunodeficiency on Pediatric Patients after the Fontan Operation: Three Case Reports

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00669806%3A_____%2F16%3A10329453" target="_blank" >RIV/00669806:_____/16:10329453 - isvavai.cz</a>

  • Alternative codes found

    RIV/00216208:11140/16:10329453

  • Result on the web

    <a href="http://dx.doi.org/10.1159/000449163" target="_blank" >http://dx.doi.org/10.1159/000449163</a>

  • DOI - Digital Object Identifier

    <a href="http://dx.doi.org/10.1159/000449163" target="_blank" >10.1159/000449163</a>

Alternative languages

  • Result language

    angličtina

  • Original language name

    Secondary Combined Immunodeficiency on Pediatric Patients after the Fontan Operation: Three Case Reports

  • Original language description

    The Fontan operation or total cavopulmonal connection (TCPC) is a palliative surgical correction of rare and complex inborn cardiac malformations that are characterized by uni-ventricular circulation. Protein-losing enteropathy (PLE) develops in 4-13% of patients after the Fontan procedure. Fontan-related PLE leads to secondary combined immunodeficiency marked by hypogammaglobulinemia and predominantly CD4(+) lymphocytopenia. Here, we present 3 case reports of patients with secondary immunodeficiency after the Fontan operation. The severity of hypogammaglobulinemia correlated with the nature of the infectious complications; however, clinical manifestations of T cell deficiency such as severe viral or opportunistic infections were not observed. The clinical consequences of the secondary combined immunodeficiency were modified by immunoglobulin replacement treatment and antibiotic prophylaxis. Heart transplantation led to the resolution of PLE signs and the restitution of IgG levels in 1 transplanted patient. Our experience shows that the immunological follow-up was delayed in all 3 patients. We suggest that all patients should be followed regularly by a clinical immunologist after the Fontan surgery. (C) 2016 S. Karger AG, Basel

  • Czech name

  • Czech description

Classification

  • Type

    J<sub>x</sub> - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)

  • CEP classification

    EC - Immunology

  • OECD FORD branch

Result continuities

  • Project

  • Continuities

    I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Others

  • Publication year

    2016

  • Confidentiality

    S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Data specific for result type

  • Name of the periodical

    International Archives of Allergy and Immunology

  • ISSN

    1018-2438

  • e-ISSN

  • Volume of the periodical

    170

  • Issue of the periodical within the volume

    4

  • Country of publishing house

    CH - SWITZERLAND

  • Number of pages

    6

  • Pages from-to

    251-256

  • UT code for WoS article

    000388066200004

  • EID of the result in the Scopus database

    2-s2.0-84990192011