Diversity of <i>RH</i> and transfusion support in Brazilian sickle cell disease patients with unexplained Rh antibodies

Dinardo, Carla Luana; Kelly, Shannon; Dezan, Marcia Regina; Ribeiro, Ingrid Helena; Castilho, Shirley Lopes de; Schimidt, Luciana C; Valgueiro, Maria do Carmo; Preiss, Liliana; Custer, Brian; Sabino, Éster Cerdeira; Westhoff, Connie M.; Epidemiology, Nhlbi Recipient

doi:10.1111/trf.15479

Cited by 11 publications

(9 citation statements)

References 29 publications

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“…We then conducted full-text article review and extracted relevant information. Specifically, the following study characteristics were retrieved and coded: intervention type, duration, intervention setting, country, sample size, EBI therapies (e.g., disease modifying agents , supportive care agents analgesics , antibiotics , pertinent vaccines systemic treatments , iron chelators , patient/carer/population education and nutritional supplements including folate supplementation ) recommended by the USA National Heart, Lung, Blood Institute [ 28 ] and World Health Organization (WHO) SCD management guidelines [ 29 , 30 ] and statements inferring each implementation outcome as recommended by Proctor [ 20 ] were retrieved. We applied the definition of each implementation outcome to identify relevant information from the eligible articles.…”

Section: Methodsmentioning

confidence: 99%

Evidence-based interventions implemented in low-and middle-income countries for sickle cell disease management: A systematic review of randomized controlled trials

et al. 2021

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Background Despite ~90% of sickle cell disease (SCD) occurring in low-and middle-income countries (LMICs), the vast majority of people are not receiving evidence-based interventions (EBIs) to reduce SCD-related adverse outcomes and mortality, and data on implementation research outcomes (IROs) and SCD is limited. This study aims to synthesize available data on EBIs for SCD and assess IROs. Methods We conducted a systematic review of RCTs reporting on EBIs for SCD management implemented in LMICs. We identified articles from PubMed/Medline, Global Health, PubMed Central, Embase, Web of Science medical subject heading (MeSH and Emtree) and keywords, published from inception through February 23, 2020, and conducted an updated search through December 24, 2020. We provide intervention characteristics for each study, EBI impact on SCD, and evidence of reporting on IROs. Main results 29 RCTs were analyzed. EBIs identified included disease modifying agents, supportive care agents/analgesics, anti-malarials, systemic treatments, patient/ provider education, and nutritional supplements. Studies using disease modifying agents, nutritional supplements, and anti-malarials reported improvements in pain crisis, hospitalization, children’s growth and reduction in severity and prevalence of malaria. Two studies reported on the sustainability of supplementary arginine, citrulline, and daily chloroquine and hydroxyurea for SCD patients. Only 13 studies (44.8%) provided descriptions that captured at least three of the eight IROs. There was limited reporting of acceptability, feasibility, fidelity, cost and sustainability. Conclusion EBIs are effective for SCD management in LMICs; however, measurement of IROs is scarce. Future research should focus on penetration of EBIs to inform evidence-based practice and sustainability in the context of LMICs. Clinical trial registration This review is registered in PROSPERO #CRD42020167289.

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Section: Methodsmentioning

confidence: 99%

Evidence-based interventions implemented in low-and middle-income countries for sickle cell disease management: A systematic review of randomized controlled trials

et al. 2021

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“…New cohort members will be genotyped using the same Transfusion Medicine array used during REDS‐III. In addition to multiple analyses on the entire cohort, targeted studies, such as the Recipient Iron biomarkers to investigate iron overload; Rh Variants and Unexplained Antibody Study; and Standardized Echocardiography to Characterize Tricuspid Regurgitation and Pulmonary Hypertension in Chronically Transfused Patients are nested within the Brazil SCD cohort 45 …”

Section: Transfusion Recipient Outcomesmentioning

confidence: 99%

The Recipient Epidemiology and Donor Evaluation Study‐IV‐Pediatric (REDS‐IV‐P): A research program striving to improve blood donor safety and optimize transfusion outcomes across the lifespan

et al. 2022

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Background The Recipient Epidemiology and Donor Evaluation Study‐IV‐Pediatric (REDS‐IV‐P) is a new iteration of prior National Heart, Lung, and Blood Institute (NHLBI) REDS programs that focus on improving transfusion recipient outcomes across the lifespan as well as the safety and availability of the blood supply. Study Design and Methods The US program includes blood centers and hospitals (22 including 6 free‐standing Children's hospitals) in four geographic regions. The Brazilian program has 5 participating hemocenters. A Center for Transfusion Laboratory Studies (CTLS) and a Data Coordinating Center (DCC) support synergistic studies and activities over the 7‐year REDS‐IV‐P program. Results The US is building a centralized, vein‐to‐vein (V2V) database, linking information collected from blood donors, their donations, the resulting manufactured components, and data extracts from hospital electronic medical records of transfused and non‐transfused patients. Simultaneously, the Brazilian program is building a donor, donation, and component database. The databases will serve as the backbone for retrospective and prospective observational studies in transfusion epidemiology, transfusion recipient outcomes, blood component quality, and emerging blood safety issues. Special focus will be on preterm infants, patients with sickle cell disease, thalassemia or cancer, and the effect of donor biologic variability and component manufacturing on recipient outcomes. A rapid response capability to emerging safety threats has resulted in timely studies related to Severe Acute Respiratory Syndrome Corona Virus‐2 (SARS‐CoV‐2). Conclusions The REDS‐IV‐P program endeavors to improve donor‐recipient‐linked research with a focus on children and special populations while also maintaining the flexibility to address emerging blood safety issues.

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“…The Rh blood group system plays a pivotal role in the field of blood transfusion and severe hemolytic disease of fetus/newborn due to its high polymorphism and strong immunogenicity (1)(2)(3). Except for the common RhD-positive and -negative phenotypes, populations exhibit numerous variants, including weak D, partial D, and Del types.…”

Section: Introductionmentioning

confidence: 99%

The Significance of RHD Genotyping and Characteristic Analysis in Chinese RhD Variant Individuals

et al. 2021

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BackgroundRhD is the most important and complex blood group system because of its highly polymorphic and immunogenic nature. RhD variants can induce immune response by allogeneic transfusion, organ transplantation, and fetal immunity. The transfusion strategies are different for RhD variants formed by various alleles. Therefore, extensive investigation of the molecular mechanism underlying RhD variants is critical for preventing immune-related blood transfusion reactions and fetal immunity.MethodsRhD variants were collected from donors and patients in Zhejiang Province, China. The phenotypes were classified using the serologic method. The full coding regions of RHD gene were analyzed using the PCR-SBT method. The multiplex ligation-dependent probe amplification (MLPA) assay was used to analyze the genotype and gene copy number. SWISS-MODLE and PyMOL software were used to analyze 3D structures of RhD caused by the variant alleles. The effect of non-synonymous substitutions was predicted using Polymorphism Phenotyping algorithm (PolyPhen-2), Sorting Intolerant From Tolerant (SIFT), and Protein Variation Effect Analyzer (PROVEAN) software.ResultsIn the collected RhD variants, 28 distinct RHD variant alleles were identified, including three novel variant alleles. RH-MLPA assay is advantageous for determining the copy number of RHD gene. 3D homology modeling predicted that protein conformation was disrupted and may explain RhD epitope differential expression. A total of 14 non-synonymous mutations were determined to be detrimental to the protein structure.DiscussionWe revealed the diversity of RHD alleles present in eastern Chinese RhD variants. The bioinformatics of these variant alleles extended our knowledge of RhD variants, which was crucial for evaluating their impact to guide transfusion support and avoid immune-related blood transfusion reactions.

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Diversity of RH and transfusion support in Brazilian sickle cell disease patients with unexplained Rh antibodies

Cited by 11 publications

References 29 publications

Evidence-based interventions implemented in low-and middle-income countries for sickle cell disease management: A systematic review of randomized controlled trials

Evidence-based interventions implemented in low-and middle-income countries for sickle cell disease management: A systematic review of randomized controlled trials

The Recipient Epidemiology and Donor Evaluation Study‐IV‐Pediatric (REDS‐IV‐P): A research program striving to improve blood donor safety and optimize transfusion outcomes across the lifespan

The Significance of RHD Genotyping and Characteristic Analysis in Chinese RhD Variant Individuals

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