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Jeseoak M.,Comenius University | Banovein P.,Comenius University | Freiberger T.,Center for Cardiovascular and Transplantation Surgery
Lekarsky Obzor | Year: 2013

Hereditary angioedema (HAE) is rare, potentially life-threating disease which is caused by the deficiency of C1-inhibitor (C1-INH) and is a part of complement immunodeficiencies. Clinically it is characterized by the recurrent angioedema of skin and/or mucosa of gastrointestinal tract or upper airways. Half of the patients manifest the clinical symptoms during the childhood. The diagnosis is confirmed by the laboratory examination and in uncertain cases also through the genetic analysis. The treatment consists from prophylaxis and the therapy of acute attacks. Understanding the molecular basis of HAE, new targeted therapies are available. Patients and methods: We reported the group of paediatric and adolescent patients with confirmed diagnosis of hereditary angioedema from 6 families. In our Centre, we are taking care about 10 patients of paediatric or adolescent (or today early adult) age. From our group 7 patients have HAE type I, 2 types II and 1 girl has HAE type III. Results: The mean age onset of the symptoms was 12.94±5.52 years with the confirmation of HAE at the age of 11.75±7.56 years. The delay in the diagnosis between the clinical onset of the symptoms and final confirmation was 1.05±1.21 years. All the patients (except from 1 asymptomatic girl and girl with HAE type III) had decreased C4 concentrations (0.05±0.04 g/L). We found a significant negative correlation between the attacks frequency and functional analysis of C1-INH in patients with HAE type I. Conclusions: All the cases of recurrent asymmetric angioedema without pruritus, lasting for several hours or days, which are painful and with poor response to the antiallergic treatment should be evaluated for the HAE by the specialist. In case of positive family history for HAE in first-degree relative, the selected screening for HAE in the other members should be performed as soon as possible. Source


Novotny T.,Masaryk University | Leinveber P.,St Annes University Hospital | Hnatkova K.,Imperial College London | Reichlova T.,St Annes University Hospital | And 13 more authors.
Journal of Electrocardiology | Year: 2014

Background Repolarization processes in female and male are different. This study provided pilot data on automatic measurements of QT intervals in heart transplant (HT) recipients stratified according to the sex of the recipient and the donor.Methods and results The following groups were analyzed: Group A - 20 males with male heart, group B - 14 females with male heart, group C - 13 females with female heart, group D - 11 males with female heart, group E - 20 healthy males, and group F - 20 healthy females. Twelve-lead electrocardiograms were digitally captured during autonomic provocative test of five postural 8-minute stages - supine, unsupported sitting, supine, unsupported standing, and supine. Fridericia formula was used for heart rate correction together with a generic correction for QT/RR hysteresis. Neither female nor male HT recipients exhibit any differences in QTc interval duration related to the sex of the donor. There was, however, a trend towards longer QTc intervals in female HT recipients compared to male HT recipients irrespective of the sex of the donor. The QTc differences between healthy control females and males were highly statistically significant proving the assay sensitivity of the study.Conclusion The available pilot data suggest that in HT patients, the sex of the donor has little influence on the QTc interval of the transplanted heart. © 2014 Elsevier Inc. All rights reserved. Source


Novotny T.,Masaryk University | Leinveber P.,St Anne iversity Hospital | Hnatkova K.,Imperial College London | Reichlova T.,St Anne iversity Hospital | And 13 more authors.
Journal of Electrocardiology | Year: 2014

Background: Repolarization processes in female and male are different. This study provided pilot data on automatic measurements of QT intervals in heart transplant (HT) recipients stratified according to the sex of the recipient and the donor. Methods and results: The following groups were analyzed: Group A-20 males with male heart, group B-14 females with male heart, group C-13 females with female heart, group D-11 males with female heart, group E-20 healthy males, and group F-20 healthy females. Twelve-lead electrocardiograms were digitally captured during autonomic provocative test of five postural 8-minute stages-supine, unsupported sitting, supine, unsupported standing, and supine. Fridericia formula was used for heart rate correction together with a generic correction for QT/RR hysteresis. Neither female nor male HT recipients exhibit any differences in QTc interval duration related to the sex of the donor. There was, however, a trend towards longer QTc intervals in female HT recipients compared to male HT recipients irrespective of the sex of the donor. The QTc differences between healthy control females and males were highly statistically significant proving the assay sensitivity of the study. Conclusion: The available pilot data suggest that in HT patients, the sex of the donor has little influence on the QTc interval of the transplanted heart. © 2014 Elsevier Inc. All rights reserved. Source

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