• 文章类型: Case Reports
    因子XI缺乏是一种罕见的疾病,具有不可预测的出血倾向。这里,我们报道了在反复进行髋关节翻修手术中,通过共振超声测量对弹性的声波估计成功地用于指导严重XI因子缺乏的患者的止血处理.不管使用新鲜的冷冻血浆,三次髋部手术中的第一次发生了严重出血.新鲜冷冻血浆的重复应用可使延长的活化部分凝血活酶时间和共振超声流变法凝块时间值正常化;因子XI活性增加到足够的水平。在第二和第三髋关节手术中没有发生明显的出血。在止血管理中使用共振超声流变学指导方法有可能通过确保足够的凝血和预防副作用来提高XI因子缺乏症患者接受手术的安全性。
    Factor XI deficiency is a rare disorder with an unpredictable bleeding tendency. Here, we report the successful use of the sonic estimation of elasticity via resonance sonorheometry for guiding the management of haemostasis in a patient with a severe factor XI deficiency in repeated revision hip surgeries. Regardless of an administration of fresh frozen plasma, a significant haemorrhage occurred at the first of three hip surgeries. The repeat application of fresh frozen plasma normalised the prolonged activated partial thromboplastin time and the resonance sonorheometry clot time values; the factor XI activity increased to a sufficient level. No significant bleeding occurred in the second and third hip surgery. Using a resonance sonorheometry guided approach in haemostasis management has the potential to improve safety for patients with factor XI deficiency undergoing surgery by ensuring sufficient clotting and preventing side effects.
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  • 文章类型: Case Reports
    背景:急性甲型肝炎感染在发展中国家的儿童中很常见。儿童的临床表现通常是无症状和黄疸,这是一种自限性感染。很少,它可能与胸腔积液等肝外并发症有关,无结石性胆囊炎,和腹水。
    方法:一名8岁的中东儿童出现腹痛,巩膜黄疸,尿液的黄色,食欲不振。在过去的两天里,腹胀。在进行诊断调查后,患儿被诊断为伴有双侧胸腔积液的HAV肝炎,无结石性胆囊炎,和腹水。他接受了补充维生素K和支持性肠胃外液体的保守治疗。4天后,观察到临床改善。
    结论:甲型肝炎感染表现为肝外表现,如胸腔积液,无结石性胆囊炎,腹水非常罕见,尤其是儿童。有一些报道称这些表现是孤立发生的,但是为了让他们根据我们的知识共存,这只在文献中报道了两个案例,这是第三种情况,所有这三种罕见的并发症同时出现在一个孩子身上。尽管HAV感染在儿童时期是一种无症状和自限性的病毒性疾病,它可以表现为罕见的肝外并发症,所以儿科医生应该意识到这种罕见的关联,以避免不必要的调查。
    BACKGROUND: Acute hepatitis A infection is common among children in developing nations. The clinical presentation in children is usually asymptomatic and anicteric, and it is a self-limiting infection. Rarely, it can be associated with extrahepatic complications such as pleural effusion, acalculous cholecystitis, and ascites.
    METHODS: An 8-year-old middle eastern child presented with abdominal pain, jaundice in the sclera, yellowish color of urine, and poor appetite. In the last two days, abdominal distension developed. After conducting diagnostic investigations, the child was diagnosed with HAV hepatitis associated with bilateral pleural effusion, acalculous cholecystitis, and ascites. He was managed conservatively with vitamin K supplementation and supportive parenteral fluids. After 4 days, clinical improvement was observed.
    CONCLUSIONS: Hepatitis A infections presented with extrahepatic manifestations like pleural effusion, acalculous cholecystitis, and ascites are very rare, especially in children. There have been some reports of these manifestations occurring in isolation, but for them to co-exist to our knowledge, this has only been reported in two cases in the literature, and this is the third case with all these three rare complications being presented simultaneously in a single child. Although HAV infection is an asymptomatic and self-limiting viral disease in childhood, it can manifest with rare extrahepatic complications, so pediatricians should be aware of this rare association to avoid unnecessary investigations.
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  • 文章类型: Case Reports
    一名65岁的男性,患有多种合并症,最近被诊断为糖尿病肾病,在艾司西酞普兰因抑郁情绪开始后出现上下肢皮疹。临床评估和皮肤活检证实皮肤小血管血管炎(CSVV),提示停药和口服甲基强的松龙治疗。皮疹的解决在一周内实现。这种罕见的艾司西酞普兰诱导的CSVV病例强调了及时识别和管理药物诱导的CSVV的重要性,并增加了有关选择性5-羟色胺再摄取抑制剂相关CSVV的有限文献。
    A 65-year-old male with multiple comorbidities and recently diagnosed with diabetic kidney disease developed upper and lower extremity rash following escitalopram initiation for his depressive mood. Clinical assessment and skin biopsy confirmed cutaneous small-vessel vasculitis (CSVV), prompting drug discontinuation and oral methylprednisolone therapy. The resolution of the rash was achieved within a week. This rare case of CSVV induced by escitalopram highlights the importance of timely recognition and management of drug-induced CSVV and adds to the limited literature on selective serotonin reuptake inhibitor-associated CSVV.
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  • 文章类型: Case Reports
    药物性免疫性血小板减少症是一种以血小板加速破坏为标志的不良反应。在癌症治疗中,血小板减少症还有许多其他原因,包括化疗药物引起的骨髓抑制,感染,和癌症的进展;药物性血小板减少容易被误诊或忽视。这里,我们介绍了一例有混合性结缔组织疾病病史的卵巢癌患者,该患者接受了手术,然后接受了紫杉醇治疗,顺铂,和贝伐单抗.患者在第六个周期后出现急性孤立性血小板减少症。血清抗血小板抗体测试显示针对糖蛋白IIb的抗体。在我们分析了这个病人的整个治疗过程之后,假定药物诱导的免疫性血小板减少症,贝伐单抗被推测为最可能的药物.血小板减少症最终使用重组人血小板生成素成功治疗,泼尼松,和重组人白细胞介素-11。本文总结了现有关于贝伐单抗诱导的免疫性血小板减少症的文献,并讨论了药物诱导的免疫性血小板减少症的相关机制和触发因素。本病例强调了贝伐单抗诱导免疫介导的血小板减少症的潜力,强调需要提高对自身免疫性疾病或自身免疫激活状态的警惕,这些疾病或自身免疫激活状态是癌症治疗中罕见药物诱导的免疫性血小板减少症的合理触发因素。
    Drug-induced immune thrombocytopenia is an adverse reaction marked by accelerated destruction of blood platelets. In cancer therapy, thrombocytopenia has many other causes including bone marrow suppression induced by chemotherapeutic agents, infection, and progression of cancer; drug-induced thrombocytopenia can easily be misdiagnosed or overlooked. Here, we present a case of an ovarian cancer patient with a history of mixed connective tissue disease who underwent surgery followed by treatment with paclitaxel, cisplatin, and bevacizumab. The patient developed acute isolated thrombocytopenia after the sixth cycle. Serum antiplatelet antibody testing revealed antibodies against glycoprotein IIb. After we analyzed the whole therapeutic process of this patient, drug-induced immune thrombocytopenia was assumed, and bevacizumab was conjectured as the most probable drug. Thrombocytopenia was ultimately successfully managed using recombinant human thrombopoietin, prednisone, and recombinant human interleukin-11. We provide a summary of existing literature on immune thrombocytopenia induced by bevacizumab and discuss related mechanisms and triggers for drug-induced immune thrombocytopenia. The present case underscores the potential of bevacizumab to induce immune-mediated thrombocytopenia, emphasizing the need for heightened vigilance towards autoimmune diseases or an autoimmune-activated state as plausible triggers for rare drug-induced immune thrombocytopenia in cancer therapy.
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  • 文章类型: Case Reports
    获得性血友病A(AHA)是一种罕见的自身免疫性疾病,其特征是出现特异性靶向凝血因子VIII的抑制剂,经常导致严重出血。
    我们对一名68岁男性患者的病历进行了回顾性分析,该患者接受了阿达木单抗诱导的AHA。
    患者接受阿达木单抗,肿瘤坏死因子抑制剂抗体,作为他治疗类风湿性关节炎的一部分.病人的临床旅程,以严重出血和凝血障碍为特征,通过应用重组因子VIIa(rFVIIa)和CyDRi方案有效地管理。
    该案例强调了在接受包括阿达木单抗治疗在内的类风湿关节炎疾病改善治疗的出血症状患者中,及时进行凝血评估的重要性。考虑到AHA罕见但危及生命的性质。此外,本报告对药物诱导的AHA的现有文献进行了广泛的综述,特别强调与免疫调节药物有关的病例。通过这种双管齐下的方法,我们的报告旨在提高医疗保健提供者对这种严重并发症的理解和认识,促进及时诊断和干预。
    UNASSIGNED: Acquired Hemophilia A (AHA) is a rare autoimmune disorder characterized by the emergence of inhibitors that specifically target coagulation Factor VIII, frequently resulting in severe bleeding episodes.
    UNASSIGNED: We conducted a retrospective analysis of the medical records of a 68-year-old male patient who presented with adalimumab-induced AHA.
    UNASSIGNED: The patient received adalimumab, a tumor necrosis factor inhibitor antibody, as part of his treatment for rheumatoid arthritis. The patient\'s clinical journey, characterized by intense bleeding and coagulopathy, was effectively managed with the application of recombinant Factor VIIa (rFVIIa) and the CyDRi protocol.
    UNASSIGNED: The case emphasizes the importance of prompt coagulation assessment in patients with bleeding symptoms receiving disease-modifying therapy for rheumatoid arthritis that includes adalimumab therapy, considering the rare yet life-threatening nature of AHA. Additionally, this report provides an extensive review of the existing literature on drug-induced AHA, with a special emphasis on cases linked to immunomodulatory medications. Through this two-pronged approach, our report aims to enhance understanding and awareness of this severe complication among healthcare providers, promoting timely diagnosis and intervention.
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  • 文章类型: Journal Article
    维生素缺乏症K与抑郁和自杀有关,但是流行病学研究很少。本研究旨在探讨维生素K与抑郁症和自杀企图之间的关系。
    这是一项回顾性横断面研究,涉及146例有自杀企图史的患者和149例无自杀企图史的患者。甲状腺激素水平,血脂谱,炎性细胞因子,和维生素被测量。
    有自杀企图的受试者表现为FT4,TC显着下降,维生素D,和维生素K,但CRP水平升高。在这些变量中,维生素K对抑郁症患者的自杀企图有更好的诊断价值,灵敏度为0.842,特异性为0.715。相关分析表明,维生素K与FT4、TC、LDL,和sdLDL。多因素分析显示,血清维生素K水平可预测抑郁症患者的自杀企图(OR=0.614,P=0.004,95%CI0.153-0.904)。此外,维生素K和自杀企图之间的负相关也注意到部分FT4,CRP,和维生素D地层分析。
    我们的研究表明,低维生素K水平与抑郁症患者的自杀企图有关,表明维生素K缺乏可能是抑郁症的生物学危险因素。
    UNASSIGNED: Hypovitaminosis K has been linked to depression and suicide, but epidemiological research is scarce. This study aimed to explore the association among vitamin K with depression and suicidal attempts.
    UNASSIGNED: This was a retrospective cross-sectional study involving 146 cases with a history of suicidal attempts and 149 subjects without a lifetime history of suicidal attempts. The levels of thyroid hormones, lipid profile, inflammatory cytokines, and vitamins were measured.
    UNASSIGNED: Subjects who had suicidal attempts presented with a significant decrease in FT4, TC, vitamin D, and vitamin K but increased CRP levels. In these variables, vitamin K has a better diagnostic value for suicidal attempts in depressed patients, with a sensitivity of 0.842 and a specificity of 0.715. Correlation analysis suggested that vitamin K was significantly and positively related to FT4, TC, LDL, and sdLDL. Multivariate analysis showed that serum vitamin K level predicts suicidal attempts in depressive patients (OR = 0.614, P = 0.004, 95% CI 0.153-0.904). Moreover, a negative correlation between vitamin K and suicidal attempts was also noted for partial FT4, CRP, and vitamin D strata analysis.
    UNASSIGNED: Our study suggests that low vitamin K levels were correlated with suicidal attempts in patients with depression, indicating that vitamin K deficiency might be a biological risk factor for depression.
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  • 文章类型: Case Reports
    长效抗凝血杀鼠剂使人类中毒,被称为超级华法林,导致难以管理的凝血病。我们介绍了一名42岁的男子在自杀未遂中摄入了有毒剂量的灭鼠剂的案例,随着鼻出血的发展,INR为11.6,需要住院治疗。七天来,进行了凝血测试的串行控制,优化不同剂量的维生素K补充剂。该案例强调了这种类型的抗凝剂的效力和延长的半衰期(大约六周),这需要定期的临床控制和令人满意的治疗依从性。
    Human intoxication by long-acting anticoagulant rodenticides, known as superwarfarins, causes coagulopathy that is difficult to manage. We present the case of a 42-year-old man who ingested a toxic dose of rodenticide in a suicide attempt, evolving with epistaxis, INR of 11.6, and needing hospitalization. For seven days, serial controls of coagulation tests were carried out, with optimization of different doses of Vitamin K supplementation. The case highlights this type of anticoagulant\'s potency and prolonged half-life (approximately six weeks), which requires regular clinical control and satisfactory treatment adherence.
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  • 文章类型: Journal Article
    背景:获得性血友病-A(AHA)是一种罕见但可能危及生命的受损凝血障碍,其特征是针对凝血因子VIII的自身抗体的发展。流感疫苗触发的AHA仅有少数病例报告。这里,我们报告了一例流感疫苗接种后由于AHA引起的严重出血性疾病,已成功治疗。
    方法:患者因几个严重,接受流感疫苗接种后,瘀伤逐渐恶化。因此,患者因鼻腔出血需要插管,损害了气道,腹膜后出血伴休克。
    方法:通过凝血因子测定证实了AHA,包括凝血活性和抗体测试,这可能是由流感疫苗接种引发的。
    方法:服用低剂量环磷酰胺和氢化可的松,直至活化部分凝血活酶时间显示正常水平。给予凝血因子VIIa,同时积极输血以解决失血问题。
    结果:上呼吸道出血消退,出血倾向已纠正至正常。患者顺利地从呼吸机上断奶,并从危重疾病中康复。然后她在第19天出院。
    结论:可以立即进行活化部分凝血活酶时间混合试验,以建立重度凝血病的初步鉴别诊断和治疗方案。AHA可能是由疫苗接种引发的,具有自身抗体激活和分子模拟的假设;这种机制需要进一步研究。
    BACKGROUND: Acquired hemophilia-A (AHA) is a rare but potentially life-threatening impaired coagulation disorder characterized by the development of autoantibodies against clotting factor VIII. Only a few case reports have been experienced with influenza vaccine-triggered AHA. Here, we report a case of severe hemorrhagic disorder due to AHA following influenza vaccine, which was successfully treated.
    METHODS: The patient presented to the emergency department because of several severe, progressively worsening bruises after receiving the influenza vaccination. Consequently, the patient required intubation due to nasal-oral bleeding, which compromised the airway, and retroperitoneal hemorrhage with shock also developed.
    METHODS: AHA was confirmed through a coagulation factor assay, including coagulation activity and antibody testing, which is possibly triggered by influenza vaccination.
    METHODS: Low-dose cyclophosphamide and hydrocortisone were prescribed until activated partial thromboplastin time showed normal levels. Coagulation factor VIIa was administered, and aggressive blood transfusion was carried out concurrently to address the blood loss.
    RESULTS: The upper airway bleeding subsided and bleeding tendencies had been corrected to normal. The patient was smoothly weaned from the ventilator and recovered from critical illness. She was then discharged on the 19th day.
    CONCLUSIONS: The activated partial thromboplastin time mixing test can be performed immediately to establish the initial differential diagnosis and treatment plan for severe coagulopathy. AHA may be triggered by vaccination, with the hypothesis of activation of autoantibodies and molecular mimicry; this mechanism should be further studied.
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  • 文章类型: Case Reports
    背景:慢性肉芽肿病(CGD)是一种罕见的免疫缺陷性疾病,其特征是吞噬功能受损,导致反复感染和肉芽肿形成。NADPH氧化酶复合物成分的基因突变,比如CYBB,NCF1、NCF2和CYBA基因,有助于发病机制。此病例报告探讨了与CGD相关的可能的眼部和血液学并发症。
    方法:一个有玻璃体切除术史的6岁女孩,膜切开术,由于先天性失明(诊断为脉络膜视网膜病变),激光治疗因全身性瘀斑和血小板减少而被转诊至医院。诊断检查最初建议慢性免疫性血小板减少性紫癜(ITP)。随后入院显示有坏死伤口,尿路感染,和复发性血小板减少症。怀疑免疫缺陷,CGD测试,进行了硝基蓝四唑(NBT)和二氢罗丹明(DHR)。她有一个低DHR(6.7),她的NBT测试为阴性(0.0%)。她的整个外显子组测序结果证实常染色体隐性CGD具有纯合NCF1突变。
    结论:该病例强调了CGD的各种临床表现,包括复发性血小板减少症和可能的早发性眼部受累。诊断挑战突出了涉及血液学家的多学科方法的重要性。免疫学家,和眼科医生进行准确的诊断和管理。在CGD中ITP的罕见共存强调了免疫缺陷和自身免疫之间的复杂联系,需要量身定制的治疗策略。
    BACKGROUND: Chronic Granulomatous Disease (CGD) is a rare immunodeficiency disorder characterized by impaired phagocytic function, leading to recurrent infections and granuloma formation. Genetic mutations in NADPH oxidase complex components, such as CYBB, NCF1, NCF2, and CYBA genes, contribute to the pathogenesis. This case report explores the possible ocular and hematologic complications associated with CGD.
    METHODS: A 6-year-old girl with a history of vitrectomy, membranotomy, and laser therapy due to congenital blindness (diagnosed with chorioretinopathy) was referred to the hospital with generalized ecchymosis and thrombocytopenia. Diagnostic workup initially suggested chronic immune thrombocytopenic purpura (ITP). Subsequent admissions revealed necrotic wounds, urinary tract infections, and recurrent thrombocytopenia. Suspecting immunodeficiency, tests for CGD, Nitroblue tetrazolium (NBT) and dihydrorhodamine (DHR) were performed. She had a low DHR (6.7), and her NBT test was negative (0.0%). Her whole exome sequencing results confirmed autosomal recessive CGD with a homozygous NCF1 mutation.
    CONCLUSIONS: This case underscores the diverse clinical manifestations of CGD, including recurrent thrombocytopenia and possible early-onset ocular involvement. The diagnostic challenges highlight the importance of a multidisciplinary approach involving hematologists, immunologists, and ophthalmologists for accurate diagnosis and management. The rare coexistence of ITP in CGD emphasizes the intricate link between immunodeficiency and autoimmunity, requiring tailored therapeutic strategies.
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  • 文章类型: Journal Article
    背景:血栓性血小板减少性紫癜(TTP)是一种以广泛的血管凝血和出血为特征的综合征。它可以影响任何年龄的个体,但更常见于女性,特别是在怀孕期间。妊娠合并TTP是一种严重且进展迅速的疾病,通常被误诊为产科疾病,例如严重的先兆子痫或HELLP综合征。结合1例临床病例,加深对妊娠期TTP的认识。
    方法:一名20岁的患者,是怀孕1次出生0,32周的最后一次月经期,出现胸闷,体力活动3天后呼吸急促。
    方法:TTP。
    方法:目前,没有预防措施。及时诊断和治疗是有用的。血浆置换和治疗阻碍患者自身抗体,比如丙种球蛋白,甲基强的松龙,利妥昔单抗,环孢素有效。
    结果:患者生命体征稳定,正常检查结果,没有并发症。她出院后,我们继续监测她的进展。
    TTP的急性发作通常与妊娠有关,因为这是一个触发因素。及时识别,准确诊断,以及涉及血浆置换的综合治疗方法,免疫抑制剂,终止妊娠可以导致缓解和对大多数患者有利的前景。
    BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a syndrome characterized by widespread blood vessel clotting and bleeding. It can affect individuals of any age but is more commonly observed in females, particularly during pregnancy. Pregnancy combined with TTP is a critical and rapidly progressing condition that is often misdiagnosed as an obstetric disorder like severe preeclampsia or HELLP syndrome. To deepen the understanding of TTP during pregnancy with the help of a clinical case.
    METHODS: A 20-year-old patient, is pregnancy 1 birth 0, 32 weeks dated by her last menstrual period, presented chest tightness, and shortness of breath after physical activity for 3 days.
    METHODS: TTP.
    METHODS: At present, there are no preventive measures. Timely diagnosis and treatment are useful. Plasma exchange and treat to the patient hinder autoantibodies, such as gamma globulin, methylprednisolone, rituximab, and cyclosporine were effective.
    RESULTS: The patient exhibited stable vital signs, normal examination results, and experienced no complications. We continued to monitor her progress after she was discharged.
    UNASSIGNED: The acute onset of TTP is often associated with pregnancy, as it is a triggering factor. Timely identification, accurate diagnosis, and a comprehensive treatment approach involving plasma exchange, immunosuppressants, and the termination of pregnancy can lead to remission and a favorable outlook for the majority of patients.
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