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

Kód výsledku v 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>

Nalezeny alternativní kódy

RIV/00216208:11140/16:10329453

Výsledek na webu

<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>

Jazyk výsledku

angličtina

Název v původním jazyce

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

Popis výsledku v původním jazyce

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

Název v anglickém jazyce

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

Popis výsledku anglicky

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

Druh

J_x - Nezařazeno - Článek v odborném periodiku (Jimp, Jsc a Jost)

CEP obor

EC - Imunologie

OECD FORD obor

—

Projekt

—

Návaznosti

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Rok uplatnění

2016

Kód důvěrnosti údajů

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

Název periodika

International Archives of Allergy and Immunology

ISSN

1018-2438

e-ISSN

—

Svazek periodika

170

Číslo periodika v rámci svazku

4

Stát vydavatele periodika

CH - Švýcarská konfederace

Počet stran výsledku

6

Strana od-do

251-256

Kód UT WoS článku

000388066200004

EID výsledku v databázi Scopus

2-s2.0-84990192011