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

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>

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
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Czech description
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Type
J<sub>x</sub> - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)
CEP classification
EC - Immunology
OECD FORD branch
—

Project
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Continuities
I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year
2016
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

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