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BMJ Open ◽  
2021 ◽  
Vol 11 (12) ◽  
pp. e046293
Author(s):  
Xianding Wang ◽  
Saifu Yin ◽  
Turun Song ◽  
Zhongli Huang ◽  
Yu Fan ◽  
...  

IntroductionIt remains unclear whether kidney transplantation (KT) from hepatitis B surface antigen (HBsAg) +donors to HBsAg− recipients (D(HBsAg+)/R(HBsAg−)) provides comparable transplant outcomes without hepatitis B virus (HBV) transmission compared with D(HBsAg−)/R(HBsAg−) KT. Moreover, no consensus has been reached for standardised prophylaxis regimens to prevent HBV transmission after D(HBsAg+)/R(HBsAg−) KT. We developed stratified prophylaxis regimens, including pretransplant antiviral treatment of donors, and pretransplant hepatitis B vaccination and post-transplant antiviral treatment of recipients, based on donors’ and recipients’ HBV serological characteristics. However, the safety and efficacy of stratified prophylaxis regimens remains unknown.Methods and analysisWe are conducting a prospective, multicentre, observational study. Between September 2020 and December 2023, 100 cases of (D(HBsAg+)/R (HBsAg−)) KT will be recruited from four university-affiliated hospitals with a follow-up at least 2 years. They will naturally receive stratified prophylaxis regimens or routine prophylaxis based on clinical experience to compare the efficacy and safety of these two regimens in (D(HBsAg+)/R(HBsAg−)) KT. The primary outcome will be post-transplant HBV infection to evaluate safety, defined as post-transplant HBsAg−→+or HBV DNA−→+. The composite endpoint of prevention failure will be also an endpoint of safety (any one of HBsAg−→+, HBV DNA−→+, HB e antigen−→+, HB e antibody−→+ and HB c antibody−→+). The efficacy will be evaluated by transplant outcomes, including death-censored graft survival, patient survival, acute rejection, delayed graft function and kidney graft function.Ethics and disseminationThis study will be registered as a clinical audit at each participating hospital and has obtained approval from the Ethics Committee of West China Hospital (reference: 2020-683, 8 September 2020).Trial registration numberNCT04562051.


2021 ◽  
Vol 5 (02) ◽  
pp. 57-60
Author(s):  
Nishat Mahzabin ◽  
Md. Akhlak-Ul- Islam ◽  
Kazi Mohammad Kamrul Islam ◽  
Khaza Amirul Islam ◽  
Md. Arif-Ur- Rahman ◽  
...  

Background: Hb-E/Beta thalassaemia is a congenital haemoglobin disorder which is a compound heterozygous state consists of qualitative disorder like Hb E variant & quantitative Hb disorder caused by genetic mutation of Beta chain. Objective: The aim of the study was to identify the beta gene mutation in Hb E/Beta thalassaemia. Method: A total of 32 diagnosed Hb E/Beta thalassaemia patients were included in this cross-sectional study from May 2019 to July 2020. Genetic analysis was done by sanger sequencing. Results: In this observational study, we found 13 different types of Beta gene mutations. Heterozygous for IVS 1-5(G>C) mutation was most frequent (53.1%). Conclusion: Genetic mutation is the confirmatory diagnosis for thalassaemia as well as one of the main factors for clinical expression. Mutation pattern also varies according to the geographical distribution. So, this study shows the frequently found mutation in Bangladesh and should carry out routinely to point out phenotypic expression.


2021 ◽  
Vol 15 (11) ◽  
pp. 2893-2896
Author(s):  
Nada Sikander ◽  
Shabnam Bashir ◽  
Rija Tariq ◽  
Furqan Sabir ◽  
Samia Akhtar ◽  
...  

Background: Hemoglobin D Iran is frequently misdiagnosed as Hb E or Hb D Punjab if only one method of screening is used. The objective of our study was to highlight the importance of using two different screening techniques in diagnosis of a hemoglobin variant, Hb D Iran in our case. Hematological parameters of heterozygous Hb D Iran and compound heterozygous β/Hb D Iran were also compared. Methods: A descriptive study was carried out on results of 52,379 subjects which were part of thalassemia extended family cascade screening from 36 districts of Punjab from October 2019-March 2021. Cases of Hb D Punjab and Hb E were run on both CE-HPLC (cation exchange-high performance liquid chromatography) and CZE (capillary zone electrophoresis). Resulting Hb D Iran cases were confirmed by ARMS-PCR (Amplification refractory mutation system-polymerase chain reaction). Results: Forty cases of Hb D Iran were detected out of 160 initially suspected Hb D Punjab cases and 126 Hb E cases. Diagnosis was confirmed by molecular analysis. Statistical significance was found between RBC count, MCV, MCH, Hb F and diagnosis of “heterozygous Hb D Iran” and “compound heterozygous for β/ Hb D Iran”. Conclusion: Hb D Iran can be easily missed and misdiagnosed as Hb E or Hb D Punjab, if two screening methods are not used. This maybe a reason why Hb D Iran remains unreported in our region. CBC and HPLC indices can also be suggestive if a case is of heterozygous D Iran or compound heterozygous β/Hb D Iran. Keywords: Hb D Iran, Hb E, Hb D Punjab, Cation exchange High performance liquid chromatography, Capillary zone electrophoresis


Author(s):  
Darren Sandean ◽  
Michail Samaras ◽  
Urjit Chatterji ◽  
Richard Power ◽  
Hafiz Qureshi
Keyword(s):  

Resumo Objetivo A anemia pré-operatória em pacientes ortopédicos, está associada a maiores taxas de transfusão de sangue alogênico, resultando em desfechos mais desfavoráveis. Até 25% dos pacientes encaminhados para cirurgia ortopédica de grande porte, apresentam algum grau de anemia. O controle adequado do sangue do paciente no perioperatório é essencial, a fim de reduzir as sequelas da anemia e a necessidade de transfusões. Avaliamos a eficácia do teste rápido, em conjunto com uma clínica especializada no tratamento da anemia pré-operatória, com o objetivo de detectar e tratar a anemia dos pacientes a serem submetidos a artroplastia total primária do quadril. Métodos A comparação das taxas totais de transfusão de sangue alogênico, foi realizada em pacientes submetidos a artroplastia total primária do quadril, antes e depois da implementação dos testes rápidos e da clínica especializada no tratamento da anemia pré-operatória ao longo de um ano. Também foi feita uma comparação entre os pacientes com anemia, que foram encaminhados para a clínica, com aqueles que não foram encaminhados. Foram revisados os níveis de hemoglobina pré-operatória, as taxas de transfusão de sangue alogênico e o tratamento clínico em 1.095 pacientes. Resultados Houve uma redução significativa nas taxas de transfusão, em pacientes submetidos à ATQ primária, caindo de 10,0% para 6,2% (p <0,05, teste χ2), após a implementação do teste rápido e da clínica especializada em anemia pré-operatória. A taxa de transfusão de sangue alogênico para pacientes anêmicos tratados na clínica foi de 6,7%, em comparação com 26,9% para pacientes anêmicos no pré-operatório, que não foram tratados na clínica (p <0,05, teste exato de Fisher). Em média, o tratamento na clínica aumentou a taxa de hemoglobina dos pacientes em 20 g/L, passando de 104 g/L para 124 g/L p <0,001. Conclusões O teste rápido, juntamente com a clínica especializada no tratamento da anemia pré-operatória, reduziu a necessidade de transfusão de sangue alogênico perioperatório, em pacientes submetidos a artroplastia total primária do quadril, proporcionando uma identificação rápida e um tratamento eficaz da anemia pré-operatória.


2021 ◽  
Author(s):  
Nishat Mahzabin ◽  
Md. Abdul Aziz ◽  
Md. Akhlak-Ul Islam ◽  
Nusrat Jahan ◽  
Md. Kamrul Hasan Sajib ◽  
...  

Abstract Background: Hemoglobin E/β-thalassemia is a common inherited hemoglobin disorder among South Asian countries. The phenotypically diverse presentation of the disease is often attributed to coinheritance of β-globin (HBB) gene mutations. The current study described the phenotype and genetic basis of Hb E/β-thalassemia patients and assessed its relation with clinical severity.Methods: A total of 32 patients were included in this cross-sectional study. Cases were confirmed by using capillary hemoglobin electrophoresis or high-performance liquid chromatography. Those with positive findings were further analyzed with clinical information and ancestral data either from the interview or medical records. Data collection was confined to May 2019 and July 2020. Gene sequencing was performed using Sanger’s sequencing method for mutational analysis, and Mahidol scoring was used to grade clinical severity.Result: A total of 13 heterozygous mutations were identified in the HBB gene. Of all, IVS-1-5 (G>C) (n=17, 53.1%) was the most common, and codon 30 (G>C) (n=4, 12.5%) was the second most common mutations. According to the Mahidol scoring system, 37.5% (n=12) were classified as phenotypically mild, 43.8% (n=14) as moderate and 18.8% (n=6) as severe. The IVS-1-5(G>C) mutation was found to be frequently associated with severe disease and showed no mild form.Conclusion: The present study described the clinical severity and its association with genetic mutations in hemoglobin E/β-thalassemia patients. This finding could guide individually tailored management strategies for this particular group of patients.


2021 ◽  
Author(s):  
Stella de Jesus Lourenço da Silva ◽  
Alícia De Souza Santos ◽  
Derly Rodrigues De Souza ◽  
Raquel Lira Lustosa Carvalho

Introdução: A anemia falciforme é uma doença causada por uma mutação na cadeia β da hemoglobina (Hb) que leva à produção da hemoglobina falciforme (HbS), resultando na falcização dos eritrócitos. Essa alteração pode causar crise vaso-oclusiva dolorosa, lesão vascular e dano ao órgão-alvo. O tratamento atual é feito com hidroxiuréia e L-glutamina, que tratam apenas os sintomas da doença, além disso, a hidroxiuréia apresenta efeitos adversos significativos. O voxelotor foi aprovado em 2019 pela US Food and Drug Administration. Esse medicamento forma uma ligação covalente reversível com a valina N-terminal da cadeia α da Hb e modula sua afinidade pelo oxigênio que, uma vez oxigenada, previne a falcização. Objetivo: Analisar a eficácia e os resultados obtidos na fase de teste deste medicamento através da análise de artigos disponíveis na literatura. Metodologia: A pesquisa foi realizada nas bases Biblioteca Virtual em Saúde, PubMed, SciELO e Google acadêmico. A seleção dos artigos foi realizada de acordo com título, resumo, palavras-chave e ano de publicação (2016-2021). Resultados: O voxelotor apresentou boa tolerância, perfil farmacocinético linear e meia-vida variando de 61 a 85 horas. O medicamento conseguiu aumentar os níveis de hemoglobina, reduzir os marcadores de hemólise de maneira dose-dependente e diminuiu os danos vasculares induzidos por hemólise em pacientes com anemia falciforme. Ademais, a droga inibiu a falcização das hemácias e diminuiu a viscosidade do sangue, reduzindo o risco de anemia e hemólise. Alguns pacientes apresentaram efeitos leves a moderados, como diarreia, náuseas e vômitos, entretanto, estes podem não estar diretamente relacionados ao tratamento. Conclusões: O voxelotor foi bem tolerado pelos usuários, aumentou os níveis de hemoglobina, a afinidade pelo oxigênio, diminuiu a hemólise em pacientes com anemia falciforme e exibiu apenas efeitos colaterais leves a moderados, mostrando-se ser um medicamento promissor no tratamento da anemia falciforme.


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