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1.
Clin Rev Allergy Immunol ; 66(2): 192-209, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38689103

RESUMO

This study aims to perform an extensive review of the literature that evaluates various factors that affect the survival rates of patients with severe combined immunodeficiency (SCID) after hematopoietic stem cell transplantation (HSCT) in developed and developing countries. An extensive search of the literature was made in four different databases (PubMed, Embase, Scopus, and Web of Science). The search was carried out in December 2022 and updated in July 2023, and the terms such as "hematopoietic stem cell transplantation," "bone marrow transplant," "mortality," "opportunistic infections," and "survival" associated with "severe combined immunodeficiency" were sought based on the MeSH terms. The language of the articles was "English," and only articles published from 2000 onwards were selected. Twenty-three articles fulfilled the inclusion criteria for review and data extraction. The data collected corroborates that early HSCT, but above all, HSCT in patients without active infections, is related to better overall survival. The universal implementation of newborn screening for SCID will be a fundamental pillar for enabling most transplants to be carried out in this "ideal scenario" at an early age and free from infection. HSCT with an HLA-identical sibling donor is also associated with better survival rates, but this is the least common scenario. For this reason, transplantation with matched unrelated donors (MUD) and mismatched related donors (mMRD/Haploidentical) appear as alternatives. The results obtained with MUD are improving and show survival rates similar to those of MSD, as well as they do not require manipulation of the graft with expensive technologies. However, they still have high rates of complications after HSCT. Transplants with mMRD/Haplo are performed just in a few large centers because of the high costs of the technology to perform CD3/CD19 depletion and TCRαß/CD19 depletion or CD34 + selection techniques in vitro. The new possibility of in vivo T cell depletion using post-transplant cyclophosphamide could also be a viable alternative for performing mMRD transplants in centers that do not have this technology, especially in developing countries.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Imunodeficiência Combinada Severa , Humanos , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Imunodeficiência Combinada Severa/terapia , Imunodeficiência Combinada Severa/mortalidade , Imunodeficiência Combinada Severa/diagnóstico , Prognóstico , Recém-Nascido , Lactente , Condicionamento Pré-Transplante/métodos
2.
J Allergy Clin Immunol Pract ; 10(7): 1805-1812, 2022 07.
Artigo em Inglês | MEDLINE | ID: mdl-35526778

RESUMO

BACKGROUND: Hereditary angioedema (HAE) is known for mortality when it is not treated properly. Many advances have occurred over the past decades that affected patients' lives. However, not all patient populations have access to the same diagnosis and treatment resources. OBJECTIVE: To evaluate mortality from HAE in a large cohort in a reference center in Brazil. Furthermore, the research intended to describe patients' life span, the asphyxia evolution, and factors related to the fatal outcome. METHODS: A cohort of 433 patients from 46 families was evaluated in this prospective and retrospective study. Families were organized in clusters and were given a verbal autopsy to arrange data collection for the deaths and analyze symptoms during life. Causes of death were classified as deaths from laryngeal edema (LE) or other causes. RESULTS: Of 433 patients evaluated, 254 were not given the diagnosis of HAE. A total of 75 fatal events were evaluated. Only 10 of 75 patients were given the diagnosis of HAE before death, and the HAE diagnosis was made after death in 65 of 75 patients using verbal autopsy. The final cause of death after the investigation was asphyxiation owing to LE in 39 of 75 (52%) and deaths owing to other causes in 36 of 75 (48%). Ten deaths had occurred in the past decade. Time from onset of symptoms to seeking medical assistance was a median of 4 hours, and the time to death was a median of 8 hours. Three patients received fresh-frozen plasma and none received medications specific to HAE attacks. Throat pain or discomfort was the most common symptom, experienced by 71.8% of patients. The most common mistaken diagnosis at the original death certificate was allergy or anaphylaxis. The life span of patients who died of LE was reduced by 20 years compared with those who died of other causes. CONCLUSIONS: Hereditary angioedema remains a threat to life in the studied population. The large number of patients who do not receive a diagnosis makes the situation even more severe and is responsible for most deaths. Death analyses add knowledge to an understanding of the diseases and their impact on patients' lives, improving the targeting of public health efforts.


Assuntos
Angioedemas Hereditários , Edema Laríngeo , Angioedemas Hereditários/complicações , Angioedemas Hereditários/diagnóstico , Angioedemas Hereditários/mortalidade , Asfixia , Brasil/epidemiologia , Humanos , Edema Laríngeo/etiologia , Edema Laríngeo/mortalidade , Estudos Prospectivos , Estudos Retrospectivos
3.
Oral Surg Oral Med Oral Pathol Oral Radiol ; 127(6): e118-e122, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30954493

RESUMO

Acinetobacter baumannii infection of skin and soft tissues is uncommon and usually associated with trauma. The present report describes 2 pediatric cases of cellulitis in the orofacial region, caused by A. baumannii infection with a fatal outcome. A 12-year-old male patient, diagnosed with acute promyelocytic leukemia, presented with an ulcerated lesion on the lip suggestive of local trauma. The condition progressed to cellulitis, epithelial necrosis, and nonspecific vesicles and blisters. The second case occurred in a 10-year-old male patient with a diagnosis of Burkitt lymphoma. The patient's condition progressed to World Health Organization Grade IV mucositis and cellulitis. In both cases, hemoculture was positive for multidrug-resistant A. baumannii. In conclusion, A. baumannii should be considered a potentially multidrug-resistant pathogen in the presence of skin and soft tissue cellulitis. Ulcerated oral lesions may place hospitalized pediatric patients at risk for A. baumannii infection.


Assuntos
Infecções por Acinetobacter , Acinetobacter baumannii , Celulite (Flegmão) , Antibacterianos , Criança , Infecção Hospitalar , Humanos , Masculino
4.
Int J Cardiol ; 248: 280-285, 2017 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-28712559

RESUMO

BACKGROUND: The status of intrinsic left ventricular (LV) contractility in patients with isolated rheumatic mitral stenosis (MS) has been debated. The acute changes in loading conditions after percutaneous mitral valvuloplasty (PMV) may affect LV performance. We aimed to examine the acute effects of PMV on LV function and identify factors associated with LV ejection fraction (LVEF) changes, and determinants of long-term events following the procedure. METHODS: One hundred and forty-two patients who underwent PMV for symptomatic rheumatic MS (valve area of 0.99±0.3cm2) were prospectively enrolled. LV volumes and LVEF were measured by three-dimensional (3D) echocardiography. Long-term outcome was a composite endpoint of death, mitral valve (MV) replacement, repeat PMV, new onset of atrial fibrillation, and stroke. RESULTS: The mean age was 42.3±12.1years, and 125 patients were women (88%). After PMV, LVEF increased significantly (51.4 vs 56.5%, p<0.001), primary due to a significant increase in LV end-diastolic volume (65.8mL vs 67.9mL, p=0.002), and resultant increase in the stroke volume (33.9mL vs 39.6mL, p<0.001). Changes in cardiac index and systolic pulmonary artery pressure were associated with LVEF changes after PMV. During a mean follow-up period of 30.8months, 28 adverse clinical events were observed. Postprocedural mitral regurgitation, MV area, and mean gradient were independent predictors of composite endpoints. CONCLUSIONS: In patients with rheumatic MS, PMV resulted in a significant improvement in LV end-diastolic volume, stroke volume and consequently increased in LVEF. Changes in cardiac index and systolic pulmonary artery pressure were associated with LVEF changes after PMV. The predictors of long-term adverse events following PMV were post-procedural variables, including mitral regurgitation, valve area, and mean gradient.


Assuntos
Valvuloplastia com Balão/tendências , Ecocardiografia Tridimensional/tendências , Estenose da Valva Mitral/diagnóstico por imagem , Cardiopatia Reumática/diagnóstico por imagem , Função Ventricular Esquerda/fisiologia , Adulto , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Estenose da Valva Mitral/fisiopatologia , Estenose da Valva Mitral/terapia , Estudos Prospectivos , Cardiopatia Reumática/fisiopatologia , Cardiopatia Reumática/terapia , Resultado do Tratamento
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