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. |
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@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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