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

J 2016

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

HLAVÁČKOVÁ, Eva, Martin LISKA, Hana JIČÍNSKÁ, Jiří NAVRÁTIL, Jiří LITZMAN et. al.

Základní údaje

Originální název

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

Autoři

HLAVÁČKOVÁ, Eva (203 Česká republika, domácí), Martin LISKA (203 Česká republika), Hana JIČÍNSKÁ (203 Česká republika), Jiří NAVRÁTIL (203 Česká republika) a Jiří LITZMAN (203 Česká republika, garant, domácí)

Vydání

International Archives of Allergy and Immunology, Basel, Karger, 2016, 1018-2438

Další údaje

Jazyk

angličtina

Typ výsledku

Článek v odborném periodiku

Obor

30102 Immunology

Stát vydavatele

Švýcarsko

Utajení

není předmětem státního či obchodního tajemství

Impakt faktor

Impact factor: 2.720

Kód RIV

RIV/00216224:14110/16:00091304

Organizační jednotka

Lékařská fakulta

DOI

http://dx.doi.org/10.1159/000449163

UT WoS

000388066200004

Klíčová slova anglicky

CD4+ lymphocytopenia; Fontan operation; Hypogammaglobulinemia; Protein-losing enteropathy; Secondary combined immunodeficiency

Štítky

EL OK

Příznaky

Mezinárodní význam, Recenzováno

Změněno: 10. 1. 2017 10:52, Ing. Mgr. Věra Pospíšilíková

Anotace

V originále

The Fontan operation or total cavopulmonal connection (TCPC) is a palliative surgical correction of rare and complex inborn cardiac malformations that are characterized by univentricular 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.

Návaznosti

MUNI/A/1183/2015, interní kód MU

Název: Patogeneze a léčba humorální imunodeficiencí

Investor: Masarykova univerzita, Patogeneze a léčba humorální imunodeficiencí, DO R. 2020_Kategorie A - Specifický výzkum - Studentské výzkumné projekty

Citovat

HLAVÁČKOVÁ, Eva, Martin LISKA, Hana JIČÍNSKÁ, Jiří NAVRÁTIL a Jiří LITZMAN. Secondary Combined Immunodeficiency in Pediatric Patients after the Fontan Operation: Three Case Reports. International Archives of Allergy and Immunology. Basel: Karger, 2016, roč. 170, č. 4, s. 251-256. ISSN 1018-2438. Dostupné z: https://dx.doi.org/10.1159/000449163.

@article{1357487,
   author = {Hlaváčková, Eva and Liska, Martin and Jičínská, Hana and Navrátil, Jiří and Litzman, Jiří},
   article_location = {Basel},
   article_number = {4},
   doi = {http://dx.doi.org/10.1159/000449163},
   keywords = {CD4+ lymphocytopenia; Fontan operation; Hypogammaglobulinemia; Protein-losing enteropathy; Secondary combined immunodeficiency},
   language = {eng},
   issn = {1018-2438},
   journal = {International Archives of Allergy and Immunology},
   title = {Secondary Combined Immunodeficiency in Pediatric Patients after the Fontan Operation: Three Case Reports},
   volume = {170},
   year = {2016}
}

TY  - JOUR
ID  - 1357487
AU  - Hlaváčková, Eva - Liska, Martin - Jičínská, Hana - Navrátil, Jiří - Litzman, Jiří
PY  - 2016
TI  - Secondary Combined Immunodeficiency in Pediatric Patients after the Fontan Operation: Three Case Reports
JF  - International Archives of Allergy and Immunology
VL  - 170
IS  - 4
SP  - 251-256
EP  - 251-256
PB  - Karger
SN  - 10182438
KW  - CD4+ lymphocytopenia
KW  - Fontan operation
KW  - Hypogammaglobulinemia
KW  - Protein-losing enteropathy
KW  - Secondary combined immunodeficiency
N2  - The Fontan operation or total cavopulmonal connection (TCPC) is a palliative surgical correction of rare and complex inborn cardiac malformations that are characterized by univentricular 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.
ER  -

HLAVÁČKOVÁ, Eva, Martin LISKA, Hana JIČÍNSKÁ, Jiří NAVRÁTIL a Jiří LITZMAN. Secondary Combined Immunodeficiency in Pediatric Patients after the Fontan Operation: Three Case Reports. \textit{International Archives of Allergy and Immunology}. Basel: Karger, 2016, roč.~170, č.~4, s.~251-256. ISSN~1018-2438. Dostupné z: https://dx.doi.org/10.1159/000449163.

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