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1.
Cytotherapy ; 2024 May 08.
Artigo em Inglês | MEDLINE | ID: mdl-38795114

RESUMO

BACKGROUND AIMS: ABO incompatibility does not hinder bone marrow transplantation (BMT), but it has been associated with worse outcomes and additional adverse events. This study aimed to verify the impact of incompatible red blood cells (iRBCs) in allogeneic BMT and to determine a safe number of iRBCs to be infused. METHODS: We compared ABO-incompatible (iABO) allogeneic BMT (n = 42) with ABO-compatible allogeneic BMT (n = 44) and evaluated the impact of the number of infused iRBCs on outcomes and adverse events. RESULTS: The iABO patients demonstrated delayed time to transfusion independence at 30 days and 60 days, increased requirement for red blood cell (RBC) transfusion and greater hemolysis signals and incidence of pure red cell aplasia. Neutrophil/platelet engraftment, length of hospitalization post-transplant, platelet units required, graft-versus-host disease occurrence and overall survival were similar in both groups. Patients in the iABO group received 1.03 × 1010 iRBCs/kg (range, 0.36-3.88). Infusion of iRBCs >1.0 × 1010 /kg was related to graft failure or death before neutrophil engraftment or platelet engraftment or both as well as increased plasma requirement and increased creatinine. Our results also suggest that antibody titers impact the transplantation scenario. CONCLUSIONS: The iABO transplantation showed some unfavorable outcomes. It is important to monitor the value of iRBCs to be infused, considering the recipient antibody titers. We propose using the number of iRBCs (iRBCs/kg) as a dose parameter with regard to infused iRBCs. Further studies are necessary to clarify the maximum safe number of iRBCs in iABO transplants.

2.
J Pediatr Hematol Oncol ; 45(7): 416-422, 2023 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-37539993

RESUMO

BACKGROUND: Erythropoietic protoporphyria (EPP) is a rare inherited disease of heme biosynthesis resulting in the accumulation of protoporphyrin, characterized by liver failure in a minority of cases. Although liver transplant (LT) is the therapeutic strategy for advanced hepatic disease, it does not correct the primary defect, which leads to recurrence in liver graft. Thus, hematopoietic stem cell transplantation (HSCT) is an approach for treating EPP. METHODS: We aim to describe the first sequential LT and HSCT for EPP performed in Latin America, besides reviewing the present-day literature. RESULTS: The patient, a 13-year-old female with a history of photosensitivity, presented with symptoms of cholestatic and hepatopulmonary syndrome and was diagnosed with EPP. Liver biopsy demonstrated cirrhosis. She was submitted to a successful LT and showed improvement of respiratory symptoms. However, she had disease recurrence on the liver graft. She underwent a myeloablative HSCT using a matched unrelated donor, conditioning with BuCy (busulfan and cyclophosphamide), and GvHD (graft vs. host disease) prophylaxis with ATG (thymoglobulin), tacrolimus and methotrexate. Neutrophil engraftment occurred on D+18. She has presented mixed chimerism, but normalization of PP levels, being 300 days after HSCT, in good state of health and normal liver function. CONCLUSIONS: Consecutive LT and HSCT for EPP is a procedure that has been described in 10 cases in the literature and, even though these patients are a highly diversified population, studies have shown favorable results. This concept of treatment should be considered in patients with established liver disease.


Assuntos
Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Hepatopatias , Transplante de Fígado , Protoporfiria Eritropoética , Feminino , Humanos , Adolescente , Transplante de Medula Óssea , Protoporfiria Eritropoética/terapia , Protoporfiria Eritropoética/patologia , Transplante de Células-Tronco Hematopoéticas/métodos , Transplante de Fígado/métodos , Condicionamento Pré-Transplante
3.
Transfus Apher Sci ; 62(5): 103741, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37271665

RESUMO

Autoimmune hemolytic anemia (AIHA) is characterized by hemolysis caused by autoantibodies. However, many patients do not respond to therapies and may have an unfavorable outcome. It has been hypothesized that patients with AIHA and alloantibodies have a lower survival compared to patients with this disease and without alloimmunization. To this end, the clinical and laboratory profile was described and sought to identify features associated with survival in patients with AIHA. This is a single-site retrospective observational study that included patients (children, adolescents, adults and elderly) diagnosed with AIHA from January 2000 to June 2019. Epidemiological data, laboratory tests, treatment response, alloantibody and autoantibody profile, red cell transfusion and clinical course were analyzed. Survival analysis was performed using Kaplan-Meier curves and Cox proportional hazards regression. The study included 138 patients, mostly caucasians and female. The median age at diagnosis was 48.5 years (0.16-88) and 82 (59.4 %) patients had secondary AIHA. In addition, 33 % (25/75) of subjects had alloantibodies at the time of AIHA diagnosis and 40 % (16/40) detected alloantibody emergence later. The overall 10-year survival rate was 51 % (median follow-up was 39 months). Monocytosis, IgM class autoantibody and Direct Antiglobulin Test (DAT) intensity had a significant impact on predicting mortality in this population. On the other hand, alloimmunization at diagnosis and after did not affect survival in this population.


Assuntos
Anemia Hemolítica Autoimune , Adolescente , Adulto , Idoso , Criança , Feminino , Humanos , Masculino , Anemia Hemolítica Autoimune/diagnóstico , Autoanticorpos , Transfusão de Eritrócitos , Isoanticorpos , Estudos Retrospectivos , Pessoa de Meia-Idade
4.
Genet Mol Biol ; 46(2): e20220230, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37216649

RESUMO

Hereditary Hemochromatosis is a disorder characterized by iron deposition in several organs and hyperferritinemia. The most studied variants are linked to the HFE gene. In Brazil, surveys that characterize this population are scarce, with no sampling in the state of Rio Grande do Sul. Our objective is to carry out a data collection focusing on the profile of this population and the influence of the most frequently HFE variants. Two centers were enrolled: Hospital de Clínicas de Porto Alegre and Hospital São Vicente de Paulo. Patients with hyperferritinemia and undergoing phlebotomy were invited. Clinical data were collected, including HFE investigation. Among the descriptive data, the allele frequency of the C282Y variant (0.252) stands out, which differs from the national scenario. Systemic arterial hypertension was the most cited comorbidity. Differences between centers were observed, highlighting higher frequency of H63D cases in HSVP (p<0.01). Genotypes were stratified according to deleterious effect of C282Y variant. Higher transferrin saturation and number of phlebotomies were observed in the C282Y/C282Y cases (p<0.001). Positive family history for hyperferritinemia was more prevalent in compound heterozygotes (p<0.01). The results presented confirm the importance of encouraging such studies and reiterate the need for greater attention to this population.

5.
Eur Respir J ; 59(2)2022 02.
Artigo em Inglês | MEDLINE | ID: mdl-34244316

RESUMO

BACKGROUND: The effects of convalescent plasma (CP) therapy in hospitalised patients with coronavirus disease 2019 (COVID-19) remain uncertain. This study investigates the effect of CP on clinical improvement in these patients. METHODS: This is an investigator-initiated, randomised, parallel arm, open-label, superiority clinical trial. Patients were randomly (1:1) assigned to two infusions of CP plus standard of care (SOC) or SOC alone. The primary outcome was the proportion of patients with clinical improvement 28 days after enrolment. RESULTS: A total of 160 (80 in each arm) patients (66.3% critically ill, 33.7% severely ill) completed the trial. The median (interquartile range (IQR)) age was 60.5 (48-68) years; 58.1% were male and the median (IQR) time from symptom onset to randomisation was 10 (8-12) days. Neutralising antibody titres >1:80 were present in 133 (83.1%) patients at baseline. The proportion of patients with clinical improvement on day 28 was 61.3% in the CP+SOC group and 65.0% in the SOC group (difference -3.7%, 95% CI -18.8-11.3%). The results were similar in the severe and critically ill subgroups. There was no significant difference between CP+SOC and SOC groups in pre-specified secondary outcomes, including 28-day mortality, days alive and free of respiratory support and duration of invasive ventilatory support. Inflammatory and other laboratory marker values on days 3, 7 and 14 were similar between groups. CONCLUSIONS: CP+SOC did not result in a higher proportion of clinical improvement on day 28 in hospitalised patients with COVID-19 compared to SOC alone.


Assuntos
COVID-19 , Idoso , COVID-19/terapia , Humanos , Imunização Passiva , Masculino , Pessoa de Meia-Idade , Plasma , SARS-CoV-2 , Resultado do Tratamento , Soroterapia para COVID-19
6.
Dysphagia ; 37(5): 1183-1200, 2022 10.
Artigo em Inglês | MEDLINE | ID: mdl-34719729

RESUMO

The scientific scope of swallowing disorders in the neonatal and pediatric populations is growing exponentially; however, the preponderance of evidence for evaluation protocols has been concentrated in non-instrumental evaluations creating a lack of research about protocols for instrumental swallowing assessment. Thus, the purpose of this study was to systematically review the literature to identify and to report protocols used in instrumental assessments through videofluoroscopic swallow study (VFSS) and fiberoptic endoscopic evaluation of swallowing (FEES) in the neonatal and pediatric populations to support clinical decision making. The search strategy was applied in five online databases, no filters were applied to restrict languages or publication dates and the gray literature was reviewed. PRISMA statement was used to guide the construction of this review. The studies included validated and unvalidated protocols, the validated protocols had their risk of bias estimated using the QUADAS-2. In total, 13 studies were included in the final review, of these eleven assessed through QUADAS-2, and two classified with low risk of bias. One study is in the process of standardization and validation of an instrumental assessment protocol for swallowing in bottle-fed infants through VFSS. Information about validity and reliability of published protocols for instrumental evaluation in the neonatal and pediatric populations is limited. Therefore, further research is needs to development studies aiming to standardize and validate protocols for instrumental assessments in these populations.


Assuntos
Transtornos de Deglutição , Deglutição , Criança , Transtornos de Deglutição/diagnóstico por imagem , Fluoroscopia/métodos , Humanos , Lactente , Recém-Nascido , Reprodutibilidade dos Testes , Gravação em Vídeo/métodos
7.
Br J Haematol ; 195(5): 710-721, 2021 12.
Artigo em Inglês | MEDLINE | ID: mdl-34490616

RESUMO

Patients with acute myeloid leukaemia (AML) have a five-year survival rate of 28·7%. Natural killer (NK)-cell have anti-leukaemic activity. Here, we report on a series of 13 patients with high-risk R/R AML, treated with repeated infusions of double-bright (CD56bright /CD16bright ) expanded NK cells at an academic centre in Brazil. NK cells from HLA-haploidentical donors were expanded using K562 feeder cells, modified to express membrane-bound interleukin-21. Patients received FLAG, after which cryopreserved NK cells were thawed and infused thrice weekly for six infusions in three dose cohorts (106 -107 cells/kg/infusion). Primary objectives were safety and feasibility. Secondary endpoints included overall response (OR) and complete response (CR) rates at 28-30 days after the first infusion. Patients received a median of five prior lines of therapy, seven with intermediate or adverse cytogenetics, three with concurrent central nervous system (CNS) leukaemia, and one with concurrent CNS mycetoma. No dose-limiting toxicities, infusion-related fever, or cytokine release syndrome were observed. An OR of 78·6% and CR of 50·0% were observed, including responses in three patients with CNS disease and clearance of a CNS mycetoma. Multiple infusions of expanded, cryopreserved NK cells were safely administered after intensive chemotherapy in high-risk patients with R/R AML and demonstrated encouraging outcomes.


Assuntos
Antígeno CD56/análise , Imunoterapia Adotiva/métodos , Células Matadoras Naturais/transplante , Leucemia Mieloide Aguda/terapia , Receptores de IgG/análise , Adolescente , Adulto , Brasil/epidemiologia , Antígeno CD56/imunologia , Criança , Feminino , Proteínas Ligadas por GPI/análise , Proteínas Ligadas por GPI/imunologia , Doença Enxerto-Hospedeiro/etiologia , Humanos , Imunoterapia Adotiva/efeitos adversos , Células Matadoras Naturais/imunologia , Leucemia Mieloide Aguda/epidemiologia , Leucemia Mieloide Aguda/imunologia , Masculino , Pessoa de Meia-Idade , Estudo de Prova de Conceito , Receptores de IgG/imunologia , Adulto Jovem
8.
J Thromb Thrombolysis ; 52(2): 482-492, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-33599858

RESUMO

Previous studies have suggested that COVID-19 pneumonia is associated with an increased risk of venous thromboembolism (VTE). This study aimed to investigate the incidence of VTE among mechanically ventilated adults with COVID-19 pneumonia, compared to patients with respiratory failure related to other causes. Prospective study that enrolled critically ill adults with suspected COVID-19 pneumonia between June 2, 2020 and August 11, 2020. Critically ill adults with suspected COVID-19 pneumonia who required mechanical ventilation within 24 h after hospital admission were followed until death or hospital discharge. Sequential ultrasonography screening of the lower extremities and catheter insertion sites, as well as testing for plasma biochemical markers, were performed at the intensive care unit admission, day 3, day 7, and day 14. The primary outcome was a composite of deep venous thrombosis, pulmonary embolism, and thrombosis at the central catheter insertion sites. We enrolled 70 patients, including 57 patients with COVID-19 and 13 patients without COVID-19, and all patients completed follow-up. The incidence of the primary outcome was higher among patients with COVID-19 than among patients with respiratory failure related to other etiologies (36.8% vs. 0%, p = 0.023). Multivariate regression analysis revealed that VTE was independently associated with a COVID-19 diagnosis (odds ratio: 6.28, 95% confidence interval: 1.19-68.07) and D-dimer concentration (1-ng/mL increase, odds ratio: 1.15, 95% confidence interval: 1.05-1.30). The incidence of VTE was higher among critically ill mechanically ventilated patients, relative to among patients with respiratory failure related to other causes.


Assuntos
COVID-19 , Estado Terminal , Pneumonia Viral , Embolia Pulmonar , Insuficiência Respiratória , Medição de Risco , Tromboembolia Venosa , Brasil/epidemiologia , COVID-19/diagnóstico , COVID-19/fisiopatologia , COVID-19/terapia , Teste para COVID-19/métodos , Cateteres Venosos Centrais/efeitos adversos , Estado Terminal/epidemiologia , Estado Terminal/terapia , Feminino , Produtos de Degradação da Fibrina e do Fibrinogênio/análise , Humanos , Incidência , Unidades de Terapia Intensiva/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Pneumonia Viral/etiologia , Pneumonia Viral/fisiopatologia , Pneumonia Viral/terapia , Estudos Prospectivos , Embolia Pulmonar/diagnóstico , Embolia Pulmonar/etiologia , Respiração Artificial/métodos , Respiração Artificial/estatística & dados numéricos , Insuficiência Respiratória/epidemiologia , Insuficiência Respiratória/etiologia , Insuficiência Respiratória/terapia , Medição de Risco/métodos , Medição de Risco/estatística & dados numéricos , Tromboembolia Venosa/sangue , Tromboembolia Venosa/epidemiologia , Tromboembolia Venosa/etiologia , Tromboembolia Venosa/terapia
9.
Hematol Oncol ; 37(1): 62-74, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30129104

RESUMO

Autologous transplantation continues to be the cornerstone of younger and fit multiple myeloma patients. It is known that frontline induction therapy before transplantation can influence post-transplant results. Therefore, best frontline treatment for transplant-eligible patients should be based on best available evidence to guide therapy. Furthermore, until now due to data scarcity, it was not possible to thoroughly compare lenalidomide to other regimens in this setting. We performed a systematic review and network (mixed treatment comparison) meta-analysis of 21 clinical trial publications, enrolling 6474 patients and comparing 11 different treatment frontline setting regimens regarding survival, response, and safety outcomes. OS analysis showed superiority of CRD (cyclophosphamide-lenalidomide-dexamethasone) over TD-based (thalidomide-dexamethasone, HR = 0.76,0.62-0.90), VAD-based (HR = 0.71,0.52-0.90), and Z-Dex (idarubicin-dexamethasone, HR = 0.37,0.17-0.76) regimens. Concerning PFS, VTD (bortezomib-thalidomide-dexametasone) showed superior results when compared with TD-based (HR = 0.66,0.51-0.84), VAD-based (HR = 0.61,0.46-0.82), Z-Dex (HR = 0.42,0.22-0.78), and high dose dexamethasone (Dex, HR = 0.62,0.41-0.90) regimens. Bortezomib/thalidomide regimens were not superior to lenalidomide, considering these outcomes. Also, concerning complete and overall response, VTD ranked first among other regimens, showing clear superiority over thalidomide-only containing protocols. Safety outcome evaluated infectious, cardiac, gastrointestinal, neurological, thrombotic, and hematological grade 3 to 4 adverse events. Risk of thrombotic events was higher with TAD (thalidomide-doxorubicin-dexamethasone), neurological with PAD (bortezomib-doxorubicin-dexamethasone), infectious with Dex, hematological with Z-Dex, gastrointestinal with VTD, and cardiac with PAD regimens. Our study endorses current recommendations on combined immunomodulatory drugs and proteasome inhibitors frontline regimens (in triplets) in transplant-eligible multiple myeloma patients, but also formally demonstrates the favorable performance of lenalidomide in overall and progression-free survival, when compared with bortezomib/thalidomide protocols.


Assuntos
Mieloma Múltiplo/diagnóstico , Mieloma Múltiplo/terapia , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Terapia Combinada , Gerenciamento Clínico , Feminino , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Transplante de Células-Tronco Hematopoéticas/métodos , Humanos , Masculino , Mieloma Múltiplo/mortalidade , Análise de Sobrevida , Resultado do Tratamento
10.
Vox Sang ; 114(8): 808-815, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31625182

RESUMO

BACKGROUND AND OBJECTIVES: Smokers currently have no defined restrictions for blood donation. However, cigarette smoke contains toxic substances such as carbon monoxide (CO) and trace elements that can affect the packed red blood cells (PRBCs) quality and safety of transfusion. This study evaluated the effects of smoking on the concentration of essential and trace elements and on carboxyhemoglobin (COHb) levels in PRBCs from smoker donors. MATERIALS AND METHODS: A matched case-control study was conducted to compare COHb levels, determined by the CO-oximetry method, and levels of trace (Cd, Pb, Cr, Ni, As and Hg) and essential (Ca, Mg, Cu, Fe, Mn, Mo, Se and Zn) elements evaluated by inductively coupled plasma mass spectrometry, in PRBCs from smoker (n = 36) and non-smoker (n = 36) donors at Hospital de Clínicas de Porto Alegre, Brazil. RESULTS: Mean COHb level was 14 times higher in the PRBCs obtained from smoker donors (5·9 [4·0-9·1] vs. 0·4 [0·2-0·8]%). Cadmium (1·0 [1·0-1·8] µg/l vs. undetectable) and lead (27 [21-36] vs. 19 [14-26] µg/l) levels were significantly higher in the PRBCs from smokers. Moreover, except for molybdenum, levels of all essential elements were lower in smoker PRBCs. CONCLUSION: The PRBCs donated by smokers contain toxic elements that are probably not safe for transfusion in children. Our results might support changes in the current guidelines of blood banks to improve the transfusion safety through inclusion of inquiry about smoking in the clinical screening, labelling and reserve PRBCs from smoker donors for adults or less critical recipients.


Assuntos
Doadores de Sangue/estatística & dados numéricos , Fumantes/estatística & dados numéricos , Fumar/sangue , Oligoelementos/sangue , Reação Transfusional/epidemiologia , Adulto , Bancos de Sangue/normas , Estudos de Casos e Controles , Eritrócitos/química , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Fumar/epidemiologia
11.
Eur Arch Otorhinolaryngol ; 275(9): 2325-2331, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30008140

RESUMO

PURPOSE: To assess the impact of balloon laryngoplasty on clinical and surgical outcomes in pediatric patients with acute subglottic stenosis. METHODS: Two case series were included and compared. The first group included patients treated initially either with tracheostomy (if severe symptoms) or with close follow-up (if mild symptoms). Those children underwent re-evaluation and specific treatment of their stenosis with laser incisions or open surgeries some weeks later. The other group included children treated initially with balloon laryngoplasty, reflecting a shift in surgical practice after 2009. Data as success of the procedure, mean hospital stay, mean pediatric intensive care unit (PICU) stay, post-procedure fever, need of antibiotics, procedure-related complications, and deaths were assessed and compared between both cohorts. RESULTS: The sample comprised 38 pediatric patients aged 0-5 years. Fifteen children were treated before 2009, of who 10 (66.7%) required tracheostomy soon after the diagnosis. Ultimately, 13 (86.6%) underwent laryngotracheal reconstruction. Twenty-three children were treated after 2009 and the success rate in these patients treated primarily with balloon laryngoplasty was 82.6%. Of these, only 3 (13%) required tracheostomy and 1 (4.3%) required further open laryngotracheal reconstruction. Patients treated by balloon laryngoplasty underwent fewer procedures under general anesthesia and had a lower burden of treatment-related morbidity, as denoted by shorter PICU stay, less antibiotic use, earlier postoperative resumption of oral feeding, and a lower incidence of postoperative complications and fever. CONCLUSION: When used for management of acute laryngeal stenosis, balloon laryngoplasty is associated with a high success rate, presenting lower morbidity than open surgery.


Assuntos
Laringoplastia/efeitos adversos , Laringoestenose/cirurgia , Complicações Pós-Operatórias/epidemiologia , Traqueostomia/efeitos adversos , Doença Aguda , Pré-Escolar , Estudos de Coortes , Constrição Patológica/cirurgia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Resultado do Tratamento
12.
Eur Arch Otorhinolaryngol ; 274(7): 2871-2876, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28439690

RESUMO

The objective of this study is to determine the incidence of post-extubation acute laryngeal lesions in a pediatric intensive care unit (PICU) and potential risk factors. Children, aged 28 days to 5 years, admitted to the PICU who required endotracheal intubation for at least 24 h were enrolled. Exclusion criteria were a previous intubation, history of laryngeal disease, current or past tracheostomy, the presence of craniofacial malformations and patients considered on palliative care. All patients underwent flexible fiber-optic laryngoscopy (FFL) not later than 8 h after extubation. A blinded researcher identified and classified laryngeal lesions based on recorded media. 231 children were enrolled between November 2005 and December 2015. At FFL examination, 102 children (44.15%) presented moderate to severe laryngeal lesions. On a multivariable analysis, we found that for each additional day with repositioning of the endotracheal tube, there was an increase of 7.3% (RR 95% CI 1.012-1.137; P = 0.018) on the baseline risk of developing moderate to severe acute laryngeal lesions. Furthermore, for each additional dose of sedation per day of intubation, there was also an increase of 3.5% on the same baseline risk (RR 95% CI 1.001-1.070; P = 0.041). The amount of tube repositioning episodes and the need for extra doses of sedation (as a proxy for possible agitation) were found to be associated with acute laryngeal lesions. Adequate sedation and minimized tube repositioning should be pursued to possibly prevent the development of post-extubation airway compromise.


Assuntos
Hipnóticos e Sedativos/uso terapêutico , Intubação Intratraqueal , Doenças da Laringe , Laringe , Ajuste de Prótese/efeitos adversos , Brasil/epidemiologia , Pré-Escolar , Feminino , Humanos , Doença Iatrogênica/epidemiologia , Doença Iatrogênica/prevenção & controle , Lactente , Unidades de Terapia Intensiva Pediátrica/estatística & dados numéricos , Intubação Intratraqueal/efeitos adversos , Intubação Intratraqueal/instrumentação , Intubação Intratraqueal/métodos , Doenças da Laringe/diagnóstico , Doenças da Laringe/epidemiologia , Doenças da Laringe/etiologia , Laringoscopia/métodos , Laringe/diagnóstico por imagem , Laringe/lesões , Masculino , Respiração Artificial/métodos , Fatores de Risco
13.
J Craniofac Surg ; 28(7): e697-e700, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28891901

RESUMO

Maxillary hypoplasia (MH) is a rare cause of respiratory dysfunction in infants and may occur in association with genetic abnormalities or as an isolated condition. It is included in the differential diagnosis of congenital nasal obstruction. This paper seeks to report a case series of infants with MH, discuss methods for its diagnosis, and compare computed tomography (CT) measurements of nasal cavities of infants with MH and without craniomaxillofacial abnormalities. The therapeutic approach in each patient is also described. All infants with MH admitted to a tertiary hospital between 2012 and 2015 were included. Baseline nasal endoscopy was performed at bedside. The width of the infants' nasal cavities was measured by a radiologist with experience in CT scanning of facial bones. Control patients were infants of matched sex and similar age who underwent head CT scanning for various reasons. Overall, 8 infants with MH and 8 controls were assessed. All nasal cavity dimensions of infants with MH were significantly smaller than those of control subjects. The authors conclude that the diagnosis of MH should be considered in infants with nasal obstruction and nasal cavity narrowing at nasal endoscopy.


Assuntos
Micrognatismo/diagnóstico por imagem , Cavidade Nasal/diagnóstico por imagem , Obstrução Nasal/diagnóstico por imagem , Diagnóstico Diferencial , Feminino , Humanos , Lactente , Masculino , Tomografia Computadorizada por Raios X
14.
Hematol Oncol ; 33(4): 212-9, 2015 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-25363800

RESUMO

Previous meta-analyses suggested that acute myeloid leukaemia induction regimens containing idarubicin (IDA) or high-dose daunorubicin (HDD) induce higher rates of complete remission (CR) than conventional-dose daunorubicin (CDD), with a possible benefit in overall survival. However, robust comparisons between these regimens are still lacking. We conducted a mixed treatment comparison meta-analysis regarding these three regimens. Mixed treatment comparison is a statistical method of data summarization that aggregates data from both direct and indirect effect estimates. Literature search strategy included MEDLINE, EMBASE, Cochrane, Scielo and LILACS, from inception until August 2013 and resulted in the inclusion of 17 trials enrolling 7258 adult patients. HDD [relative risk (RR) 1.13; 95% credible interval (CrI) 1.02-1.26] and IDA (RR 1.13; 95% CrI 1.05-1.23) showed higher CR rates than CDD. IDA also led to lower long-term overall mortality rates when compared with CDD (RR 0.93, 95% CrI 0.86-0.99), whereas HDD and CDD were no different (RR 0.94, 95% CrI 0.85-1.02). HDD and IDA comparison did not reach statistically significant differences in CR (RR 1.00; 95% CrI 0.89-1.11) and in long-term mortality (RR 1.01, 95% CrI 0.91-1.11). IDA and HDD are consistently superior to CDD in inducing CR, and IDA was associated with lower long-term mortality. On the basis of these findings, we recommend incorporation of IDA and HDD instead of the traditional CDD as standard treatments for acute myeloid leukaemia induction. The lack of HDD benefit on mortality, when compared with CDD in this study, should be cautiously addressed, because it may have been susceptible to underestimation because of statistical power limitations.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Daunorrubicina/uso terapêutico , Idarubicina/uso terapêutico , Leucemia Mieloide Aguda/tratamento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Daunorrubicina/administração & dosagem , Intervalo Livre de Doença , Feminino , Humanos , Idarubicina/administração & dosagem , Leucemia Mieloide Aguda/mortalidade , Leucemia Mieloide Aguda/patologia , Masculino , Indução de Remissão
15.
Braz J Otorhinolaryngol ; 89(2): 254-263, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-35680554

RESUMO

OBJECTIVES: To evaluate the bacterial microbiome found in tracheostomy cannulas of a group of children diagnosed with glossoptosis secondary to Robin Sequence (RS), and its clinical implications. METHODS: Pediatric patients were enrolled in the study at the time of the cannula change in the hospital. During this procedure, the removed cannula was collected and stored for amplicon sequencing of 16s rRNA. DNA extraction was performed using DNeasy PowerBiofilm Kit (QIAGEN® ‒ Cat nº 24000-50) while sequencing was performed with the S5 (Ion S5™ System, Thermo Fisher Scientific), following Brazilian Microbiome Project (BMP) protocol. RESULTS: All 12 patients included in the study were using tracheostomy uncuffed cannulas of the same brand, had tracheostomy performed for over 1-year and had used the removed cannula for approximately 3-months. Most abundant genera found were Aggregatibacter, Pseudomonas, Haemophilus, Neisseria, Staphylococcus, Fusobacterium, Moraxella, Streptococcus, Alloiococcus, and Capnocytophaga. Individual microbiome of each individual was highly variable, not correlating to any particular clinical characteristic. CONCLUSION: The microbiome of tracheostomy cannulas is highly variable, even among patients with similar clinical characteristics, making it challenging to determine a standard for normality.


Assuntos
Microbiota , Traqueostomia , RNA Ribossômico 16S/genética , Cânula , Microbiota/genética , Brasil
16.
Braz J Otorhinolaryngol ; 88(5): 657-662, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-33272833

RESUMO

INTRODUCTION: Inadequate drooling can cause serious clinical, functional and social problems. Validated questionnaires to evaluate drooling impact on quality of life are lacking in Brazilian Portuguese. OBJECTIVES: To translate and validate the drooling impact scale to Brazilian Portuguese. METHODS: The drooling impact scale was translated to Brazilian Portuguese and back- translated to English to assess potential conceptual differences. Brazilian Portuguese version of drooling impact scale was applied to a 40 patients' sample of sialorrhea presenting pediatric patients (up to 20 years of age). Chronbach's alpha, exploratory factorial analysis and confirmatory factorial analysis were then proceeded with data collected. RESULTS: The mean drooling impact scale value for the whole population was 51.77 (SD = 16.13). The internal consistency obtained with Cronbach's alpha indicated a value of 0.72 for the entire sample. The Bartlett's test of sphericity was significant (p <  0.0001), confirming correlation among variables tested. Kaiser-Meyer-Olkin measure of sampling adequacy revealed a value of 0.72, indicating that the correlation matrix was reasonably suitable for factor analysis. Regarding exploratory factorial analysis, parallel analysis suggested a two-factor solution that was used for confirmatory factorial analysis. The first factor was responsible for 33.78% of the variance with an Eigenvalue of 3.38. The second factor explained 16.1% of the variance with an Eigenvalue of 1.61. At confirmatory factorial analysis, the two-factor model showed consistently better adjustments parameters than the one-factor model. CONCLUSION: The drooling impact scale has been successfully translated to Brazilian Portuguese language, showing adequate internal validity. Validation of this instrument allows physicians and other personnel involved in the care of these patients to perform a better management of patients experiencing drooling. With this tool, we are now able to guide routines and provide guidelines both before and after the different kinds of treatments in order to improve the general well-being of the patient and his family.


Assuntos
Sialorreia , Brasil , Criança , Humanos , Idioma , Psicometria , Qualidade de Vida , Reprodutibilidade dos Testes , Sialorreia/diagnóstico , Inquéritos e Questionários , Traduções
17.
Hematol Transfus Cell Ther ; 44(4): 519-525, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-34824032

RESUMO

INTRODUCTION: Urgent blood component transfusions may be life-saving for patients in hemorrhagic shock. Measures to reduce the time taken to provide these transfusions, such as uncrossmatched transfusion or abbreviated testing, are available. However, transport time is still an additional delay and the use of a pneumatic tube system (PTS) may be an alternative to shorten the transport time of blood components. OBJECTIVES: To assess pneumatic tube system transportation of blood components based on a validation protocol. METHODS: Pre- and post-transport quality control laboratory parameters, visual appearance, transport time and temperature of the packed red blood cells (RBCs), thawed fresh plasma (TFP), cryoprecipitate (CR), and platelet concentrate (PC) were evaluated. Parameters were compared between transport via pneumatic tube and courier. RESULTS: A total of 23 units of RBCs, 50 units of TFP, 30 units of CR and ten units of PC were evaluated. No statistically significant differences were found between pre- and post-transport laboratory results. There was also no difference in laboratory parameters between transport modalities (PTS versus courier). All blood components transported matched regulatory requirements for quality criteria. The temperature during transport remained stable and the transport time via PTS was significantly shorter than the courier's transport time (p < 0.05). CONCLUSION: The PTS was considered a fast, safe and reliable means of transportation for blood components, also securing quality prerequisites.

18.
Hematol Transfus Cell Ther ; 44(3): 379-385, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-33775585

RESUMO

INTRODUCTION: Thromboembolic events occur due to an imbalance in the hemostasis and some factors associated with this condition can be inherited. In order to evaluate the frequency of genotypes considered to be common hereditary risk factors for thrombophilia associated with venous thrombosis (g.1691G > A and g.20210G > A) and hyperhomocysteinemia (g.677C > T and g.1298A > C), samples from voluntary healthy blood donors at the Hospital de Clínicas de Porto Alegre were tested. METHODS: We examined 325 blood samples from blood donors collected from October 2017 to July 2018. Blood was collected on filter paper and the DNA was extracted for single nucleotide polymorphisms (SNPs) analysis using the qualitative real time polymerase chain reaction. RESULTS: The calculated frequencies of each genetic variant in heterozygosity were 4% for the FV gene (g.1691G > A), 4% for the F2 gene (g.20210G > A) and 42% and 39% for methylenetetrahydrofolate reductase (MTHFR), g.677C > T and g.1298A > C, respectively. Only the genetic variants of MTHFR were found in homozygosity, with frequencies of 14% and 6% (g.677C > T and g.1298A > C), respectively. DISCUSSION: Altogether, these results describe the frequencies of genetic variants associated with venous thrombosis and hyperhomocysteinemia in the analyzed group and are important to enhance our current knowledge about the genetic profiles of Brazilian blood donors.

19.
Hum Immunol ; 81(5): 197-201, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32067841

RESUMO

Immunological platelet refractoriness occurs when polytransfused patients develop antibodies against donors' HLA class I antigens, HPA (human platelet antigens) and few cases against both systems. Flow cytometry crossmatch with the patient serum against platelets from several donors can determine whether the refractoriness is or is not of immunological origin. Patients with moderate sensitization will be given transfusions from donors with a negative platelets crossmatch; those who are hypersensitized will need to have antibodies assessed against a reactivity panel (RP) for HLA class I and HPA. The patient must be typed for HLA and HPA in order to identify best donors. We have compiled a list of 500 donors registered at our blood bank with known HLA and HPA profiles. Pre-transfusion crossmatch is performed against donors selected virtually, transfusing those who are negative. We analyzed 75 patients with refractoriness, 67% (50/75) of whom had anti-HLA or anti-HPA antibodies and 56% (28/50) were hypersensitized, with RP ≥ 80%. The diagnosis of the immunological refractoriness and the compatibility between donor and recipient allowed efficient transfusions for all patients.


Assuntos
Anticorpos/imunologia , Antígenos de Plaquetas Humanas/imunologia , Tipagem e Reações Cruzadas Sanguíneas/métodos , Plaquetas/imunologia , Antígenos HLA/imunologia , Antígenos de Histocompatibilidade Classe I/imunologia , Histocompatibilidade , Doadores de Sangue , Humanos , Contagem de Plaquetas , Transfusão de Plaquetas/métodos , Estudos Retrospectivos , Trombocitopenia/terapia , Reação Transfusional
20.
Blood Transfus ; 18(1): 40-48, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31855151

RESUMO

BACKGROUND: Red blood cells from smoking donors can have more lesions from oxidative stress, decreasing the benefits of blood transfusion. We aimed to explore the effect of cigarette smoking on the oxidative status of packed red blood cells (PRBCs) prior to storage. MATERIALS AND METHODS: We compared serum vitamin C, plasmatic malondialdehyde (MDA), and non-protein thiol groups (GSH) levels in PRBCs, as well glutathione peroxidase (GPx) and glutathione s-transferase (GST) activity in PRBCs from smoking (n=36) and non-smoking (n=36) donors. We also correlated urinary cotinine levels with these parameters. RESULTS: Cigarette smoking was associated with decreased serum levels of vitamin C and GPx, and increased GST activity in PRBCs. We found negative correlations between cotinine, GPx activity and vitamin C levels, and a positive correlation between cotinine and GST activity. DISCUSSION: Cigarette smoking changed antioxidant defences of PRBCs prior to storage and these parameters are correlated with cotinine levels. Increased RBC antioxidants such as GST may reflect an exposure to oxidants during erythropoiesis. Because of the inability of mature RBCs to resynthesise antioxidants, PRBCs from smokers may have higher risk of storage lesions than those from non-smoker donors.


Assuntos
Doadores de Sangue , Fumar Cigarros/sangue , Eritrócitos/metabolismo , Adulto , Idoso , Antioxidantes/análise , Ácido Ascórbico/sangue , Cotinina/urina , Eritrócitos/química , Eritrócitos/enzimologia , Feminino , Glutationa Peroxidase/sangue , Glutationa Transferase/sangue , Humanos , Masculino , Malondialdeído/sangue , Pessoa de Meia-Idade , Estresse Oxidativo/efeitos dos fármacos , Compostos de Sulfidrila/sangue
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