Anemia, hemolytic

贫血,溶血
  • 文章类型: Case Reports
    甲型肝炎病毒(HAV)感染通常表现为自限性疾病,但它可以引起衰弱的症状和很少暴发性肝炎(急性肝衰竭),这往往是致命的。世卫组织估计,2016年全球有7134人死于甲型肝炎(占病毒性肝炎死亡率的0.5%)。在不到1%的急性病毒性肝炎病例中观察到暴发性肝衰竭。噬血细胞性淋巴组织细胞增生症(HLH)是由于异常免疫激活而导致的过度炎症和组织破坏的临床综合征。由病毒感染(也称为病毒相关的噬血细胞综合征)引起的获得性HLH最常见的是与EB病毒和巨细胞病毒(CMV)相关。HAV相关的HLH很少报道。在甲型肝炎病例中,轻度至中度溶血并非闻所未闻,这通常是免疫介导的。这里,我们介绍一个30多岁的男人的案子,急性病毒性肝炎暴露了G6PD缺乏症,后来进展为急性肝功能衰竭,HLH和肾衰竭。
    Hepatitis A virus (HAV) infection typically presents as a self-limiting illness but it can cause debilitating symptoms and rarely fulminant hepatitis (acute liver failure), which is often fatal. WHO estimates that in 2016, 7134 persons died from hepatitis A worldwide (accounting for 0.5% of the mortality due to viral hepatitis). Fulminant hepatic failure is observed in less than 1% of cases of acute viral hepatitis A. Haemophagocytic lymphohistiocytosis (HLH) is a clinical syndrome of excessive inflammation and tissue destruction owing to abnormal immune activation. Acquired HLH due to viral infections (also known as virus-associated haemophagocytic syndrome) is most commonly associated with Epstein-Barr virus and cytomegalovirus (CMV). HAV-associated HLH has been rarely reported. Haemolysis of mild to moderate degree is not unheard of in cases of hepatitis A, which is often immune-mediated. Here, we present the case of a man in his 30s, with G6PD deficiency unmasked by acute viral hepatitis A, which later on progressed to hyperacute liver failure, HLH and renal failure.
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  • 文章类型: Journal Article
    This paper analyzes the pathogenesis, clinical characteristics, treatment measures and prognosis of a case of methemoglobin and hemolytic anemia caused by acute nitrogen trifluoride poisoning. The patient with occupational exposure to nitrogen trifluoride was treated immediately after the onset of illness, methemoglobin was monitored and a comprehensive examination was conducted. After comprehensive analysis, it was considered that acute nitrogen trifluoride poisoning could cause methemoglobinemia, hemolytic anemia and liver injury. The patient was disengaged and given symptomatic treatment such as oxygen therapy, methylene blue, low-dose methylpredrone, vitamin C and reduced glutathione. The prognosis of the patient is good, which provides a reference for the clinical treatment and occupational health examination of nitrogen trifluoride poisoning.
    本文对1例急性三氟化氮中毒致高铁血红蛋白血症、溶血性贫血患者的发病过程、临床特点、治疗措施及预后进行分析。患者职业暴露三氟化氮,发病后立即就诊,监测高铁血红蛋白并进行全面检查,综合分析后考虑急性三氟化氮中毒致高铁血红蛋白血症、溶血性贫血及肝损伤。脱离接触,给予氧疗、亚甲蓝、小剂量甲泼尼龙、维生素C、还原性谷胱甘肽等对症治疗,患者预后较好,为三氟化氮中毒的临床救治及职业健康检查提供参考。.
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  • 文章类型: Journal Article
    转录因子的SP/KLF家族带有三个C端C2H2锌指,这些锌指由两个接头散布,使DNA与9-10bp的基序结合。该家族的创始成员KLF1的突变,很常见。接头2中的错义突变导致轻度表型。然而,当与功能丧失突变共同遗传时,它们导致严重的非球形细胞溶血性贫血。我们通过将Klf1+/-小鼠与在接头-2中带有错义突变的Klf1H350R/+小鼠杂交来产生这种疾病的小鼠模型。Klf1H350R/-小鼠表现出严重的溶血而没有地中海贫血。RNA-seq显示编码跨膜和细胞骨架蛋白的基因表达缺失,但不是globins.ChIP-seq显示DNA结合特异性没有变化,而是全球亲和力的降低,这是用重组蛋白和体外结合试验证实的。这项研究为锌指转录因子中的接头突变如何导致与功能丧失突变引起的表型不同提供了新的见解。
    The SP/KLF family of transcription factors harbour three C-terminal C2H2 zinc fingers interspersed by two linkers which confers DNA-binding to a 9-10 bp motif. Mutations in KLF1, the founding member of the family, are common. Missense mutations in linker two result in a mild phenotype. However, when co-inherited with loss-of-function mutations, they result in severe non-spherocytic hemolytic anemia. We generate a mouse model of this disease by crossing Klf1+/- mice with Klf1H350R/+ mice that harbour a missense mutation in linker-2. Klf1H350R/- mice exhibit severe hemolysis without thalassemia. RNA-seq demonstrate loss of expression of genes encoding transmembrane and cytoskeletal proteins, but not globins. ChIP-seq show no change in DNA-binding specificity, but a global reduction in affinity, which is confirmed using recombinant proteins and in vitro binding assays. This study provides new insights into how linker mutations in zinc finger transcription factors result in different phenotypes to those caused by loss-of-function mutations.
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  • 文章类型: Case Reports
    我们在此介绍了在TAVI最初接受医学治疗后记录的假体副瓣膜渗漏的情况。这导致耐受性差的溶血性贫血。通过对TAVI瓣膜进行后扩张,我们能够纠正这种假体旁瓣膜渗漏,效果良好,随访不复杂。
    We present here a case of documented paraprosthetic valvular leak following TAVI treated medically initially. This led to a poorly tolerated hemolytic anemia. We were able to correct this paraprosthetic valvular leak by a postdilation of the TAVI valve with a good result and uncomplicated follow-up.
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  • 文章类型: Journal Article
    在这项研究中,我们报告了我们实验室对1例接受羟氯喹治疗的重症COVID-19患者的血浆样本进行的分析前挑战.这很重要,因为,在接受羟氯喹治疗的重症COVID-19患者中,在没有溶血的情况下,血浆样品可以具有较高的溶血指数,影响钾和其他分析物的报告结果。
    In this study, we report a preanalytical challenge noted in our laboratory on plasma samples from a critically ill COVID-19 patient treated with hydroxychloroquine. This is significant because, in critically ill COVID-19 patients on hydroxychloroquine, plasma samples can have a high measured haemolysis index in the absence of haemolysis, with the impact on reporting the results for potassium and other analytes.
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  • 文章类型: Letter
    暂无摘要。
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  • 文章类型: Case Reports
    斑疹伤寒,流行的热带感染,有时可能表现为不寻常的并发症。这里,我们介绍了一个年轻人的案例,他在过去的三天里发烧,并从那天早上开始出现深色尿液。在调查中,我们发现了血管内溶血性贫血.通过细致的检查,在他的右臀部发现了一个黑色坏死病变(焦痂),斑疹伤寒感染的病理标志。用口服多西环素100mg开始治疗,每天两次。随后,通过ELISA和焦痂组织的PCR分析,斑疹伤寒IgM阳性结果证实了斑疹伤寒的诊断。患者对口服多西环素反应良好,在接下来的几天内症状缓解。此病例突出了与斑疹伤寒感染相关的严重血管内溶血。
    Scrub typhus, a prevalent tropical infection, may sometimes manifest with unusual complications. Here, we present the case of a young man who was admitted to our facility with a fever for the past 3 days and passage of dark-coloured urine since that morning. On investigation, we identified intravascular haemolytic anaemia. Through meticulous examination, a black necrotic lesion (eschar) was discovered on his right buttock, a pathognomonic sign of scrub typhus infection. Treatment was initiated with oral doxycycline 100 mg two times a day. Subsequently, diagnosis of scrub typhus was confirmed through positive results from scrub typhus IgM via ELISA and PCR analysis from the eschar tissue. The patient responded well to oral doxycycline and his symptoms resolved within the next few days. This case highlights severe intravascular haemolysis associated with scrub typhus infection.
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  • 文章类型: Case Reports
    A 19-year-old male patient with high-risk acute B-cell lymphoblastic leukemia received haploidentical stem cell transplantation. He developed anemia repeatedly and parvovirus B19 nucleic acid was positive in blood plasma. The patient was diagnosed with cold agglutinin syndrome and multiple organ dysfunction including respiratory failure and hepatitis. In the conflict between viral infection and the treatment of cold agglutinin syndrome, we provided supportive treatment, complement inhibitors to control hemolysis, and antiviral therapy. After timely glucocorticoid and immunosuppressant therapy, the patient had achieved a good response.
    患者男性,19岁,高危急性B淋巴细胞白血病行单倍体异基因造血干细胞移植,移植后多次出现贫血,检测血细小病毒B19核酸阳性,随后出现冷凝集素综合征、多器官功能障碍(呼吸衰竭、肝脏损害等)。在病毒感染和冷凝集素综合征治疗矛盾情况下充分给予支持治疗、应用补体抑制剂控制溶血、坚持抗病毒治疗,适时加用糖皮质激素及免疫抑制剂,最终获得了较好的治疗效果。.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    棕色隐居蜘蛛叮咬会导致皮肤坏死等严重反应,溶血性贫血,多器官衰竭,可能会危及生命.已报道治疗性血浆置换为此类病例提供临床益处。在这份报告中,我们介绍了一例棕色隐士蜘蛛咬伤的病例,该病例通过治疗性血浆置换成功治疗,并将其与以前的病例报告进行了比较。
    Brown recluse spider bites can lead to severe reactions such as skin necrosis,hemolytic anemia, and multiorgan failure, which can be life-threatening. Therapeutic plasma exchange has been reported to provide clinical benefit for such cases. In thisreport, we present a case of a brown recluse spider bite that was successfully treated with therapeutic plasma exchange and compare it with previous case reports.
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