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1.
Dermatol Ther (Heidelb) ; 11(5): 1469-1480, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34269966

RESUMO

Epidermolysis bullosa (EB) is a hereditary genetic skin disorder, classified as a type of genodermatosis, which causes severe, chronic skin blisters associated with painful and potentially life-threatening complications. Currently, there is no effective therapy or cure for EB. However, over the past decade, there have been several important advances in treatment methods, which are now approaching clinical application, including gene therapy, protein replacement therapy, cell therapy (allogeneic fibroblasts, mesenchymal stromal cells), bone marrow stem cell transplant, culture/vaccination of revertant mosaic keratinocytes, gene editing/engineering, and the clinical application of inducible pluripotent stem cells. Tissue engineering scientists are developing materials that mimic the structure and natural healing process to promote skin reconstruction in the event of an incurable injury. Although a cure for EB remains elusive, recent data from animal models and preliminary human clinical trials have raised the expectations of patients, clinicians, and researchers, where modifying the disease and improving patients' quality of life are now considered attainable goals. In addition, the lessons learned from the treatment of EB may improve the treatment of other genetic diseases.

2.
Transplant Proc ; 52(8): 2477-2479, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32434743

RESUMO

Biliary complications are one of the most serious and dangerous complications following liver transplantation. Factors that may determine their occurrence are still being assessed. The retrospective analysis of 239 consecutive liver transplantations (LT) performed between January 2013 and December 2018 was conducted in compliance with the Helsinki Congress and the Istanbul Declaration. We divided recipients into 2 groups depending on whether biliary complications occurred. The first (biliary complication [BC group]) consisted of patients who developed biliary complications (n = 41) and the second (nonbiliary complications [NBC group]) without them (n = 198). Demographic and statistical data analysis showed no differences between the groups in terms of age, Model for End-Stage Liver Disease with sodium serum concentration (MELD-Na) score, and average cold or warm ischemia time. In comparison, estimated intraoperative blood loss, 1341 mL in the NBC and 1399 mL in the BC, was not significantly different, as were the number of transfused red blood cells (RBC) units, which were respectively 1.7 and 2.1 (P = ns). The recipients' hemoglobin levels just before surgery were (11.5 g/dL vs 11.6 g/dL; P = ns) and after transplantation (9.8 g/dL vs 9.8 g/dL; P = ns). Eleven patients died within 30 days of transplantation. This group was characterized by a higher MELD-Na score (25 vs 17; P = .01), lower pretransplant hemoglobin level (10 g/dL vs 11.6 g/dL; P = .02), and the number of transfused RBC units (3.3 vs 1.7; P = .01). However, there was no correlation between intraoperative blood loss, the number of transfused RBC units, pre- and postoperative hemoglobin levels, and the incidence of biliary complications after LT. Lower pretransplant hemoglobin levels and a higher amount of intraoperatively transfused blood products were associated with a higher fatality rate after LT.


Assuntos
Perda Sanguínea Cirúrgica , Transfusão de Sangue , Transplante de Fígado/efeitos adversos , Complicações Pós-Operatórias/epidemiologia , Adulto , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos
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