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.

• MeSH
• agamaglobulinemie krev   imunologie   MeSH
• dítě MeSH
• Fontanova operace škodlivé účinky   MeSH
• imunofenotypizace MeSH
• imunoglobulinové izotypy krev   MeSH
• lidé MeSH
• lymfopenie krev   imunologie   MeSH
• počet lymfocytů MeSH
• předškolní dítě MeSH
• těžká kombinovaná imunodeficience diagnóza   etiologie   MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• Názvy látek
• imunoglobulinové izotypy MeSH

Immediate and delayed hypersensitivity reactions can play a role in the pathogenesis of atopic dermatitis (AD). We tested 71 patients (median age 5 years) with AD for hypersensitivity to grass and birch pollen, Dermatophagoides pteronyssinus, and Dermatophagoides farinae using atopy patch test (APT), skin prick test (SPT), and specific IgE measurement. The sensitivity (SE) and specificity (SP) of the tests were calculated on the basis of personal history of AD exacerbation, clinical AD score (SCORAD) changes, and the number of days with need for topical anti-inflammatory treatment (AITD) in relation to exposure to the allergens being tested. APT was positive in 45 patients, mostly to D. farinae (n=37). SPT and/or specific IgE were positive in 42 subjects, in most cases to grass and birch pollen (n=29). SE of APT reached 33%-56% for history, 33% for SCORAD, and 0%-60% for AITD; SP of APT was comparable for all three assessment standards (history, SCORAD, and AITD) (48%-67%). SE of SPT/specific IgE was higher for history (26%-63%) than for the other two standards of assessment (0%-67%); SP of SPT/specific IgE was also highest for history (69%-91%), and lower for SCORAD (59%-87%) and AITD (65%-80%). AD is often associated with hypersensitivity; its influence on AD, however, is clinically significant only in a minor group of patients. While personal history and SCORAD changes present themselves as possible standards in the evaluation of clinically relevant hypersensitivity in AD patients, the anti-inflammatory treatment days (AITD) appears to be unsuitable for this purpose.

• Publikační typ
• časopisecké články MeSH

The aim of this study was to confirm or refute the difference between efficacy of long-term specific immunotherapy (SIT) with standardized allergen vaccine consisting of six grass pollens (oat grass, orchard grass, fescue, rye grass, timothy grass, and rye) administered either by sublingual or by supralingual route. To investigate clinical and immunologic changes, 51 patients of a previous 1-year double-blind, placebo-controlled, randomized study were enrolled in an open randomized study that continued over the next 3 years. Sublingual or supralingual immunotherapy (SLIT) was performed in the same way, keeping the drops under or on the tongue, respectively, for 1-2 minutes before swallowing them. Data about symptoms scores and rescue medication intake during grass pollen seasons, as well as skin-prick test results, levels of specific IgG, and IgE antibodies were collected after each pollen season. It was clearly shown that both routes of administration were effective, leading to a significant decrease of clinical symptoms of grass pollen allergy after SIT lasting 3-4 years. No statistically significant difference between sublingually and supralingually treated patients was observed at the end of the study. Adverse effects were limited to a small number of generally mild local and/or systemic reactions with no significant difference between both administration ways of SIT. The significant therapeutic effect of both SLIT and supralingual immunotherapy lasting 3-4 years was clearly achieved. Despite no significant difference between efficacy of both administration ways of SIT, the onset of sublingual SIT effect seems to be slightly faster than that of supralingual SIT.

• MeSH
• alergeny aplikace a dávkování   MeSH
• aplikace sublinguální * MeSH
• desenzibilizace imunologická metody   MeSH
• dítě MeSH
• dospělí MeSH
• dvojitá slepá metoda MeSH
• imunoglobulin E krev   MeSH
• imunoglobulin G krev   MeSH
• kožní testy MeSH
• lékové formy MeSH
• lidé středního věku MeSH
• lidé MeSH
• lipnicovité imunologie   MeSH
• mladiství MeSH
• mladý dospělý MeSH
• placebo MeSH
• pyl imunologie   MeSH
• rozvrh dávkování léků MeSH
• sezónní alergická rýma imunologie   terapie   MeSH
• výsledek terapie MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• randomizované kontrolované studie MeSH
• Názvy látek
• alergeny MeSH
• imunoglobulin E MeSH
• imunoglobulin G MeSH
• lékové formy MeSH
• placebo MeSH