• 文章类型: Case Reports
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    背景:尽管长效红细胞生成刺激剂(ESA)的广泛使用促进了慢性肾脏病(CKD)患者贫血的改善,预后改善尚未得到充分证实.缺铁与心血管疾病(CVD)的发展有关,红细胞生成刺激剂引起的相对铁缺乏可能阻止预后的改善。因此,我们研究了在使用转铁蛋白饱和度(TSAT)的长效红细胞生成刺激剂治疗期间铁缺乏与心血管事件之间的关系,比铁蛋白更不容易发炎。
    方法:本研究包括1040例非透析依赖性CKD患者,年龄≥20岁,肾小球滤过率<60mL/min/1.73m2,血红蛋白<11g/dL,接受达贝泊汀阿尔法治疗96周。这些患者是在BRIGHTEN试验中招募的,一个多中心,prospective,进行的观察性研究旨在评估临床上治疗非透析依赖性CKD贫血时对darbepoetinalfa的红细胞生成刺激剂耐药性。使用Kaplan-Meier方法评估转铁蛋白饱和度与心血管事件累积发生率之间的关联。要计算危险比(HR),使用95%置信区间(CI)和Cox比例风险模型。
    结果:心血管事件的生存曲线分析表明转铁蛋白饱和度≥30%的患者预后明显更好,调整后的风险比为0.34(95%置信区间0.22-0.52)。分层分析显示,转铁蛋白饱和度为30-40%的患者发生心血管事件的风险明显低于转铁蛋白饱和度为20-30%的患者。即使多变量校正后的风险比为0.33(95%置信区间0.21-0.54).
    结论:在使用长效红细胞生成刺激剂治疗的患者中,CKD和转铁蛋白饱和度为30-40%的患者的心血管事件明显少于转铁蛋白饱和度为20-30%的患者。因此,从红细胞生成刺激剂反应性和减少心血管事件的角度来看,维持较高的转铁蛋白饱和度可能是有用的.
    BACKGROUND: Although the widespread use of long-acting erythropoiesis-stimulating agents (ESAs) has facilitated the improvement of anemia in patients with chronic kidney disease (CKD), the improvement in prognosis has not been fully demonstrated. Iron deficiency is associated with the development of cardiovascular diseases (CVDs), and the relative iron deficiency induced by erythropoiesis-stimulating agents may prevent the improvement of prognosis. Therefore, we investigated the association between iron deficiency and cardiovascular events during long-acting erythropoiesis-stimulating agent therapy using transferrin saturation (TSAT), which is less susceptible to inflammation than ferritin.
    METHODS: This study included 1040 patients with non-dialysis-dependent CKD, aged ≥ 20 years, with a glomerular filtration rate < 60 mL/min/1.73 m2 and hemoglobin < 11 g/dL, who were treated with darbepoetin alfa for 96 weeks. The patients were recruited in the BRIGHTEN Trial, a multicenter, prospective, observational study conducted to evaluate erythropoiesis-stimulating agent resistance to darbepoetin alfa in treating anemia in non-dialysis-dependent CKD in a clinical setting. The association between transferrin saturation and the cumulative incidence of cardiovascular events was evaluated using the Kaplan-Meier method. To calculate the hazard ratio (HR), 95% confidence intervals (CI) and the Cox proportional hazards model were used.
    RESULTS: Survival curve analysis for cardiovascular events indicated that patients with transferrin saturation ≥ 30% had a significantly better prognosis, with an adjusted hazard ratio of 0.34 (95% confidence interval 0.22-0.52). Stratified analysis revealed that patients with transferrin saturation of 30-40% had a significantly lower risk of cardiovascular events than those with transferrin saturation of 20-30%, even after a multivariate-adjusted hazard ratio of 0.33 (95% confidence interval 0.21-0.54).
    CONCLUSIONS: Patients with CKD and transferrin saturation of 30-40% had significantly fewer cardiovascular events than those with transferrin saturation of 20-30% among patients treated with long-acting erythropoiesis-stimulating agents. Therefore, it may be useful to maintain higher transferrin saturation from the viewpoint of erythropoiesis-stimulating agent responsiveness and the reduction of cardiovascular events.
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  • 文章类型: Journal Article
    近年来,由于基础科学和生物技术的重大进展,肾脏病学见证了对导致各种与肾脏疾病相关或引起肾脏疾病的机制的更深入的了解,为开发特定的治疗方法开辟了新的视角。这些新的可能性给医生带来了更多的挑战,他们在疾病表征和为个体患者选择正确的治疗方面面临新的复杂性。
    我们选择了四种治疗情况:慢性肾病(CKD)贫血,CKD心力衰竭,IgA肾病(IgAN)和膜性肾病(MN)。文献检索是通过PubMed进行的。
    在CKD中,贫血管理仍然具有挑战性;通常需要个性化的治疗方法。对于射血分数降低的CKD和心力衰竭患者,确定可以从特定治疗中受益的患者也是重要目标。IgAN的几种新疗法正在临床开发中;有趣的是,它们专门针对疾病的发病机制。将MN发病机制理解为自身免疫性疾病以及几种自身抗体的发现可以更好地表征患者。淋巴细胞计数的高敏感性技术打开了更个性化使用抗CD20疗法的可能性。
    UNASSIGNED: In recent years, thanks to significant advances in basic science and biotechnologies, nephrology has witnessed a deeper understanding of the mechanisms leading to various conditions associated with or causing kidney disease, opening new perspectives for developing specific treatments. These new possibilities have brought increased challenges to physicians, who face with a new complexity in disease characterization and selection the right treatment for individual patients.
    UNASSIGNED: We chose four therapeutic situations: anemia in chronic kidney disease (CKD), heart failure in CKD, IgA nephropathy (IgAN) and membranous nephropathy (MN). The literature search was made through PubMed.
    UNASSIGNED: Anaemia management remains challenging in CKD; a personalized therapeutic approach is often needed. Identifying patients who could benefit from a specific therapy is also an important goal for patients with CKD and heart failure with reduced ejection fraction. Several new treatments are under clinical development for IgAN; interestingly, they target specifically the pathogenetic mechanisms of the disease. The understanding of MN pathogenesis as an autoimmune disease and the discovery of several autoantibodies allows a better characterization of patients. High-sensible techniques for lymphocyte counting open the possibility of more personalized use of anti CD20 therapies.
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  • 文章类型: Journal Article
    建议采用基于曲线下面积(AUC24)的方法来指导万古霉素治疗药物监测(TDM),尽管存在相关风险,但仍普遍使用谷浓度。缺乏明确的毒性目标,这对于肾毒性风险较高的血液学患者很重要。目的是(1)评估基于波谷的TDM对急性肾损伤(AKI)发生率的影响,(2)树立万古霉素肾毒性阈值,(3)评估血液学患者达到万古霉素治疗目标的比例。回顾性数据收集了2020年4月至2021年1月期间接受万古霉素治疗的100名患有血液系统恶性肿瘤或再生障碍性贫血的成年患者。AKI的发生是根据血清肌酐浓度确定的,和个体药代动力学参数使用贝叶斯方法估计。进行受试者工作特征(ROC)曲线分析以评估药代动力学指标预测AKI发生的能力。基于AUC24/MIC≥400和确定的毒性阈值评估达到目标万古霉素暴露的患者比例。AKI发生率为37%。ROC曲线分析表明最大AUC24为644mg。治疗期间的h/L是AKI的重要预测因子。到治疗的第4天,29%的疗程有治疗性万古霉素暴露,只有62%的课程达到AUC24目标。鉴定的毒性阈值支持400-650mg的AUC24目标范围。h/L,假设MIC为1毫克/升,以优化万古霉素的疗效和减少毒性。这项研究强调了该人群中AKI的高发生率,并强调了从基于波谷的TDM过渡到基于AUC的方法以改善临床结果的重要性。
    An area-under-the-curve (AUC24)-based approach is recommended to guide vancomycin therapeutic drug monitoring (TDM), yet trough concentrations are still commonly used despite associated risks. A definitive toxicity target is lacking, which is important for hematology patients who have a higher risk of nephrotoxicity. The aims were to (1) assess the impact of trough-based TDM on acute kidney injury (AKI) incidence, (2) establish a vancomycin nephrotoxicity threshold, and (3) evaluate the proportion of hematology patients achieving vancomycin therapeutic targets. Retrospective data was collected from 100 adult patients with a hematological malignancy or aplastic anemia who received vancomycin between April 2020 and January 2021. AKI occurrence was determined based on serum creatinine concentrations, and individual pharmacokinetic parameters were estimated using a Bayesian approach. Receiver operating characteristic (ROC) curve analysis was performed to assess the ability of pharmacokinetic indices to predict AKI occurrence. The proportion of patients who achieved target vancomycin exposure was evaluated based on an AUC24/MIC ≥400 and the determined toxicity threshold. The incidence of AKI was 37%. ROC curve analysis indicated a maximum AUC24 of 644 mg.h/L over the treatment period was an important predictor of AKI. By Day 4 of treatment, 29% of treatment courses had supratherapeutic vancomycin exposure, with only 62% of courses achieving AUC24 targets. The identified toxicity threshold supports an AUC24 target range of 400-650 mg.h/L, assuming an MIC of 1 mg/L, to optimize vancomycin efficacy and minimize toxicity. This study highlights high rates of AKI in this population and emphasizes the importance of transitioning from trough-based TDM to an AUC-based approach to improve clinical outcomes.
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  • 文章类型: Journal Article
    背景:镰状细胞病(SCD)是撒哈拉以南非洲的一种主要遗传病,包括毛里塔尼亚。胎儿血红蛋白(HbF)可以影响病理生理学,缓和临床过程,并为SCD的治疗提供了前景。本研究旨在探讨BCL11A基因中的单核苷酸多态性(SNPs)对毛里塔尼亚镰状细胞(HbSS)患者HbF水平和血液学参数的影响。
    方法:对565例疑似SCD患者进行全血细胞计数评估。进行聚合酶链反应(PCR)-限制性片段长度多态性以鉴定HbSS,并对50例镰状细胞患者的内含子2中的rs4671393(A>G)和rs11886868(C>T)和BCL11A基因3个UTR区的rs1052520(G>A)进行基因分型。
    结果:研究人群中HbSS的患病率为8.8%(50/565),HbF水平的平均值(±标准差)为15.0%(±6.0%)。测序显示存在三种基因型:AA(13.6%),AG(46.6%),GG(39.6%)在rs4671393;CC(17.6%),CT(48.7%),rs11886868中的TT(33.6%)。来自HbSS个体的所有样品在rs1052520等位基因中显示野生型基因型。次要等位基因A(rs4671393)和C(rs11886868)的患病率分别为37%和39%,分别。rs4671393SNP与升高的HbF之间存在统计学上的显着关联(p=0.034)(平均12.72±6.26%)。
    结论:对毛里塔尼亚SCD患者BCL11A基因座中三个SNP的研究显示rs4671393等位基因与HbF水平显著相关。需要进一步的研究来探索BCL11A基因座中的其他SNP,并调查据报道调节HbF水平的其他遗传标记。例如HBS1L-MYB和Xmn1-HBG2,以改善毛里塔尼亚这种潜在威胁生命的疾病的管理。
    BACKGROUND: Sickle cell disease (SCD) is a major heritable genetic disease in sub-Saharan Africa, including Mauritania. Fetal hemoglobin (HbF) can affect the pathophysiology, moderate the clinical course, and offer prospects for curative treatment of SCD. This study aimed to investigate the influence of single nucleotide polymorphisms (SNPs) in the BCL11A gene on the levels of HbF and hematological parameters in Mauritanian sickle cell (HbSS) patients.
    METHODS: Complete blood count was assessed in 565 patients suspected to have SCD. Polymerase chain reaction (PCR)-restriction fragment length polymorphism was performed to identify the HbSS, and sequencing was used for genotyping three SNPs: rs4671393 (A>G) and rs11886868 (C>T) in the intron 2 and rs1052520 (G>A) in the 3\'UTR regions of the BCL11A gene in 50 sickle cell patients.
    RESULTS: The prevalence of HbSS among the study population was 8.8% (50/565), and the mean (± standard deviation) of HbF level was 15.0% (± 6.0%). Sequencing showed the presence of three genotypes: AA (13.6%), AG (46.6%), GG (39.6%) in rs4671393; CC (17.6%), CT (48.7%), and TT (33.6%) in rs11886868. All samples from HbSS individuals displayed a wild-type genotype in the rs1052520 allele. The prevalence of minor alleles A (rs4671393) and C (rs11886868) were 37% and 39%, respectively. There was a statistically significant association (p = 0.034) between rs4671393 SNP and elevated HbF (mean 12.72 ± 6.26%).
    CONCLUSIONS: The study of three SNPs in the BCL11A locus in Mauritanian patients with SCD showed a significant association of rs4671393 allele with the HbF level. Further research is needed to explore additional SNPs in the BCL11A locus and investigate other genetic markers reported to modulate HbF levels, such as HBS1L-MYB and Xmn1-HBG2, to improve the management of this potentially life-threatening condition in Mauritania.
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  • 文章类型: Journal Article
    背景与目的急性髓系白血病(AML)是儿童和成人普遍存在的异质性和侵袭性血液恶性肿瘤,占全球急性白血病病例的很大比例。我们的研究旨在揭示印度南部三级政府医院新诊断的AML病例的人口统计学和临床特征以及风险分层。方法我们进行了一项横断面研究,涉及临床血液科221例AML患者,拉吉夫·甘地政府总医院和马德拉斯医学院,钦奈,泰米尔纳德邦从2020年1月到2022年12月。所有数据均从医院患者病历数据库中收集。全面分析临床病史,合并症,实验室,风险分层,并进行化疗方案。纳入研究的患者是13岁以上的AML新诊断病例,我们排除了所有复发病例。结果41~50岁年龄组患者比例最高(22.2%),并且在该队列中有显着的男性优势(55.7%)。占领方面,31%的研究人口是农民,其次是家庭主妇(16.3%)。虽然在191例(86.4%)中没有发现AML的可识别危险因素,4.1%曾接受过化疗,3.6%患有骨髓增生异常综合征(MDS)。50例(22.6%)出现高尿酸血症,而8.6%患有肿瘤溶解综合征(TLS)。大约53.8%的病例属于AML的中等风险类别。87.3%的AML患者接受标准诱导化疗。结论对AML的地区人口统计数据和临床表现的认识和了解将有助于早期发现,及时转介,并开始治疗,从而进一步改善靶向治疗和造血干细胞移植时代的患者预后。
    Background and objective Acute myeloid leukemia (AML) is a heterogeneous and aggressive blood malignancy prevalent among both children and adults, accounting for a significant proportion of acute leukemia cases worldwide. Our study aimed to shed light on the demographic and clinical profile and risk stratification of newly diagnosed AML cases at a tertiary care government hospital in South India. Methods We conducted a cross-sectional study involving 221 patients with AML in the Department of Clinical Hematology, Rajiv Gandhi Government General Hospital and Madras Medical College, Chennai, Tamil Nadu from January 2020 to December 2022. All data were collected from the hospital database of patients\' medical records. A thorough analysis of clinical history, comorbidities, laboratories, risk stratification, and chemotherapy regimen was performed. The patients included in the study were newly diagnosed cases of AML over the age of 13 years, and we excluded all the relapsed cases. Results The highest proportion of patients were in the age group of 41-50 years (22.2%), and there was a significant male predominance (55.7%) in the cohort. Occupationwise, 31% of the study population were farmers, followed by housewives (16.3%). While no identifiable risk factors for AML were found in 191 cases (86.4%), 4.1% had undergone previous chemotherapy, and 3.6% had myelodysplastic syndrome (MDS). Hyperuricemia was noted in 50 cases (22.6%) while 8.6% had tumor lysis syndrome (TLS). About 53.8% of cases fell in the intermediate risk category of AML. Standard induction chemotherapy was administered in 87.3% of cases of AML. Conclusions Gaining awareness and knowledge about the regional demographic data and clinical presentation of AML will aid in the early detection, prompt referral, and initiation of treatment, thereby further improving patient outcomes in the era of targeted therapy and hematopoietic stem cell transplantation.
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  • 文章类型: Journal Article
    Aplastic anemia is a rare disease with a large differential diagnosis, including neoplastic origin as well as congenital bone marrow failure syndromes. Investigations must be quick and precise. Treatment depends on the patient\'s age and consists of immunosuppression treatment or allogeneic bone marrow transplantation. Because of the risk of progression to other hematological diseases, a close specialized follow-up is recommended.
    L’anémie aplasique est une maladie rare avec un diagnostic différentiel large, comprenant des maladies d’origine néoplasique ainsi que les syndromes d’insuffisance médullaire congénitale. Les investigations doivent être rapides et précises. Le traitement dépend de l’âge du patient et consiste en une immunosuppression plus ou moins sévère ou une allogreffe de moelle osseuse. En raison du risque d’évolution vers d’autres maladies hématologiques, un suivi spécialisé rapproché est préconisé.
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  • 文章类型: Journal Article
    慢性贫血是一个重大的健康问题,特别是在育龄妇女中。月经失血等因素,分娩,营养不足,紧密间隔的怀孕,反复的消化道出血会增加贫血的风险。这项研究调查了当前的避孕方法是否与育龄印度妇女的贫血有关。
    来自全国家庭健康调查第五轮的横截面数据,于2019-21年进行,用于本次调查(NFHS-5)。我们的分析中只包括非妊娠和非闭经妇女,最终得到673,094名15-49岁女性的分析样本。采用双变量交叉表和多变量逻辑回归分析数据。
    贫血患病率为57%,调整后的回归模型发现,任何避孕方法的使用与妇女的血红蛋白状态之间均无显著关联。使用传统避孕方法的妇女有1.08(95%置信区间,1.048-1.113)患贫血的几率更高。在现代方法中,除注射剂外,所有其他方法-例如宫内节育器(IUD),屏障使用,和绝育-与使用避孕药的女性相比,贫血的几率更高。
    这项研究探讨了印度现代避孕药与血红蛋白水平之间的关系,揭示注射剂与贫血几率显着降低有关,而传统避孕药和其他现代方法与贫血呈正相关.这些发现促使政策制定者将重点放在减少贫血和安全避孕上。需要更多的研究来指导决策,鉴于文献很少。
    UNASSIGNED: Chronic anaemia is a significant health concern, particularly among women of childbearing age. Factors such as menstrual blood loss, childbirth, inadequate nutrition, closely spaced pregnancies, and recurrent gastrointestinal bleeding increase the risk of anaemia. This study investigated whether current contraceptive methods are associated with anaemia in Indian women of reproductive age.
    UNASSIGNED: Cross-sectional data from the fifth round of the National Family Health Survey, conducted in 2019-21, were used for this investigation (NFHS-5). We included only non-pregnant and non-amenorrhoeic women in our analysis, resulting in a final analytical sample of 673,094 women aged 15-49. Bivariate cross-tabulations and multivariable logistic regression were employed to analyse the data.
    UNASSIGNED: The prevalence of anaemia was 57%, and the adjusted regression models found no significant association between the use of any contraceptive methods and women\'s haemoglobin status. Women using traditional contraceptive methods had 1.08 (95% confidence interval, 1.048-1.113) times higher odds of having anaemia. Among the modern methods, other than injectables, all other methods-such as an intrauterine device (IUD), barrier use, and sterilisation-were associated with higher odds of anaemia compared to women who used contraceptive pills.
    UNASSIGNED: This study explored the relationship between modern contraceptives and haemoglobin levels in India, revealing that injectables were associated with a notable reduction in the odds of anaemia, whereas traditional contraceptives and other modern methods exhibited positive associations with anaemia. These findings prompt policymakers to focus on anaemia reduction and safe contraceptives. More research is needed to inform decisions, given the scant literature.
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  • 文章类型: Case Reports
    背景:结核病(TB),全世界死亡的主要原因之一,在土著人民中发病率较高。尽管不常见,自身免疫性溶血性贫血(AIHA)已被认为是发展分枝杆菌感染的风险条件,作为免疫抑制治疗的结果。TB,反过来,可能是继发感染的诱发因素。
    方法:这里我们介绍一个来自哥伦比亚的28岁土著妇女的案例,先前诊断为AIHA和肺结核。尽管有各种治疗方法,治疗和医疗干预,患者在多种原因导致的严重髓质再生症后死亡,包括免疫抑制治疗的继发性骨髓毒性和继发性播散性感染,金黄色葡萄球菌感染,肺炎克雷伯菌和光滑念珠菌,被鉴定为耐药微生物。一起,这导致了严重的临床并发症.尸检时诊断为侵袭性曲霉病。
    结论:本报告提出了AIHA的罕见发现,其次是TB,并强调了应对共感染的巨大挑战,特别是耐药病原体。它还旨在促使政府和公共卫生当局将注意力集中在预防上,结核病的筛查和管理,特别是在脆弱的社区中,比如土著人。
    BACKGROUND: Tuberculosis (TB), one of the leading causes of death worldwide, has a higher incidence among indigenous people. Albeit uncommon, autoimmune hemolytic anemia (AIHA) has been deemed a risk condition to develop mycobacterial infection, as a result of the immunosuppressive treatments. TB, in turn, can be a predisposing factor for secondary infections.
    METHODS: Here we present a case of a 28-year-old indigenous woman from Colombia, previously diagnosed with AIHA and pulmonary TB. Despite various treatments, therapies and medical interventions, the patient died after severe medullary aplasia of multiple causes, including secondary myelotoxicity by immunosuppressive therapy and secondary disseminated infections, underlining infection by Staphylococcus aureus, Klebsiella pneumoniae and Candida glabrata, which were identified as drug-resistant microorganisms. Together, this led to significant clinical complications. Invasive aspergillosis was diagnosed at autopsy.
    CONCLUSIONS: This report presents a rarely finding of AIHA followed by TB, and highlights the great challenges of dealing with co-infections, particularly by drug resistant pathogens. It also aims to spur governments and public health authorities to focus attention in the prevention, screening and management of TB, especially among vulnerable communities, such as indigenous people.
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