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Nonsteroidal Anti-inflammatory Drug Induced Thrombotic Thrombocytopenic Purpura. 非甾体抗炎药引起的血栓性血小板减少性紫癜。
IF 3 Q2 Medicine Pub Date : 2013-11-14 eCollection Date: 2013-01-01 DOI: 10.4137/CMBD.S12843
Karlos Z Oregel, Jeremy Ramdial, Stefan Glück

A 21-year-old male presented to the emergency department after a 5-day history of recurrent vomiting and decreased urine output. History revealed ingestion of ibuprofen. During the diagnostic workup, the following was identified: white blood cell count 13.4 (×10(3)/mcL), hemoglobin 11.9 (×10(6)/mcL) with an MCV of 73 fL, hematocrit 34% and platelets were 31,000/mcL, sodium of 130 mmol/L, potassium of 5.1 mmol/L, chloride of 83 mmol/L, bicarbonate of 21 mmol/L, blood urea nitrogen of 184 mg/dL and creatinine of 19.1 mg/dL. He was later diagnosed with thrombotic thrombocytopenic purpura (TTP) based on the fact that he presented with most components of the TTP pentad (except for fever), which included altered mental status, acute kidney injury, thrombocytopenia, and evidence of red cell fragmentation and his ADAMTS13 level was found to be less than 10% prior to therapy. The patient then received plasma exchange, oral corticosteroids, and hemodialysis, which led to a full recovery of platelet count and renal function.

一名21岁男性在5天的反复呕吐和尿量减少后出现在急诊科。病史显示曾摄入布洛芬。在诊断过程中,确定了以下指标:白细胞计数13.4 (×10(3)/mcL),血红蛋白11.9 (×10(6)/mcL), MCV为73 fL,红细胞比容34%,血小板31000 /mcL,钠130 mmol/L,钾5.1 mmol/L,氯化物83 mmol/L,碳酸氢盐21 mmol/L,血尿素氮184 mg/dL,肌酐19.1 mg/dL。他后来被诊断为血栓性血小板减少性紫癜(TTP),因为他表现出TTP五相的大部分成分(发烧除外),包括精神状态改变、急性肾损伤、血小板减少和红细胞分裂的证据,治疗前发现他的ADAMTS13水平低于10%。患者接受血浆置换、口服皮质类固醇和血液透析治疗,血小板计数和肾功能完全恢复。
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引用次数: 7
Hemoglobinuria misidentified as hematuria: review of discolored urine and paroxysmal nocturnal hemoglobinuria. 血红蛋白尿误诊为血尿:变色尿和阵发性夜间血红蛋白尿的回顾。
IF 3 Q2 Medicine Pub Date : 2013-06-20 eCollection Date: 2013-01-01 DOI: 10.4137/CMBD.S11517
Prashant Veerreddy

Discolored urine is a common reason for office visits to a primary care physician and urology referral. Early differentiation of the type or cause of discolored urine is necessary for accurate diagnosis and prompt management. Paroxysmal nocturnal hemoglobinuria is a clonal disorder caused by acquired somatic mutations in the PIG-A gene on the X- chromosome of hemopoietic stem cells and leads to deficiency of surface membrane anchor proteins. The deficiency of these proteins leads to an increased risk of hemolysis of erythrocytes and structural damage of platelets, resulting in a clinical syndrome characterized by complement-mediated intravascular hemolytic anemia, bone marrow failure, and venous thrombosis. Patients with this clinical syndrome present with paroxysms of hemolysis, causing hemoglobinuria manifesting as discolored urine. This can be easily confused with other common causes of discolored urine and result in extensive urologic work-up. Three commonly confused entities of discolored urine include hematuria, hemoglobinuria, and myoglobinuria. Specific characteristics in a dipstick test or urinalysis can guide differentiation of these three causes of discolored urine. This article begins with a case summary of a woman presenting with cranberry-colored urine and a final delayed diagnosis of paryxysmal nocturnal hemoglobinuria. Her hemoglobinuria was misdiagnosed as hematuria, leading to extensive urologic work-up. The article also gives an overview of the approach to diagnosing and treating discolored urine.

尿液变色是去初级保健医生和泌尿科转诊的常见原因。早期鉴别尿液变色的类型或原因是准确诊断和及时处理的必要条件。阵发性夜间血红蛋白尿是一种克隆性疾病,由造血干细胞X染色体上的猪- a基因获得性体细胞突变引起,并导致表面膜锚蛋白缺乏。这些蛋白的缺乏导致红细胞溶血和血小板结构损伤的风险增加,导致补体介导的血管内溶血性贫血、骨髓衰竭和静脉血栓形成为特征的临床综合征。这种临床综合征的患者表现为阵发性溶血,引起血红蛋白尿,表现为尿液变色。这很容易与尿液变色的其他常见原因混淆,并导致广泛的泌尿系统检查。三种常被混淆的变色尿包括血尿、血红蛋白尿和肌红蛋白尿。在试纸试验或尿液分析的具体特征可以指导这三种原因的尿变色的区分。这篇文章开始与一个案例总结的妇女表现为蔓越莓色尿和最后延迟诊断为阵发性夜间血红蛋白尿。她的血红蛋白尿被误诊为血尿,导致广泛的泌尿系统检查。文章还概述了尿液变色的诊断和治疗方法。
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引用次数: 32
Idiopathic thrombocytopenia with iron deficiency anemia. 特发性血小板减少症伴缺铁性贫血。
IF 3 Q2 Medicine Pub Date : 2013-04-14 eCollection Date: 2013-01-01 DOI: 10.4137/CMBD.S11371
Ramy Ibrahim, Jaffar Ahmad Alhilli, Tyler T Cooper, Irina Dashkova, Judah Guy, Anjula Gandhi, Mohammad Zaman

We report a rare case of anemia and thrombocytopenia as a result of uterine fibroid and adenomyosis, complicated by immune thrombocytopenic purpura (ITP). Symptoms were presented as menorrhagia and metrorrhagia in a 34-year-old African American woman, who was later treated with blood and platelet transfusion and iron therapy with steroids. Uterine fibroids are commonly found to cause hematologic disturbances such as anemia and reactive thrombocytosis and, less commonly, thrombocytopenia. Moreover, such hematologic disturbances are secondary to heavy and irregular uterine bleeding, which is typically presented. A previous uterine fibroid diagnosis was made and reconfirmed by pelvic and transvaginal ultrasound to exclude other locoregional pathologies. ITP was suggested by Coombs test and several other serologies, leading to confirmation via bone marrow biopsy. In a previous case study, we reported positive responses in hemotecrit and platelet count after the introduction of iron therapy to an iron-depleted middle-aged female presenting severe anemia and thrombocytopenia.1.

我们报告一例罕见的贫血和血小板减少由于子宫肌瘤和子宫腺肌病,并发免疫性血小板减少性紫癜(ITP)。一名34岁的非裔美国妇女出现月经过多和子宫出血的症状,后来接受了输血和血小板输注以及类固醇铁治疗。子宫肌瘤通常会引起血液学紊乱,如贫血和反应性血小板增多症,以及较少出现的血小板减少症。此外,这种血液学紊乱是继发于大量和不规则的子宫出血,这是典型的表现。既往子宫肌瘤的诊断,并再次确认盆腔和经阴道超声排除其他局部病理。Coombs试验和其他几种血清学提示ITP,可通过骨髓活检确诊。在之前的一个案例研究中,我们报道了一名出现严重贫血和血小板减少症的缺铁中年女性在引入铁治疗后,其血凝指标和血小板计数呈阳性反应。
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引用次数: 5
Clinical and Hematological Features of 335 Patients of Chronic Myelogenous Leukemia Diagnosed at Single Centre in Northern Pakistan 巴基斯坦北部单一中心诊断的335例慢性骨髓性白血病患者的临床和血液学特征
IF 3 Q2 Medicine Pub Date : 2012-01-01 DOI: 10.4137/CMBD.S10578
F. A. Bhatti, Suhaib Ahmed, N. Ali
There are no studies regarding analysis of clinical and haematological features of chronic myelogenous leukemia (CML) from Pakistan. This study analyzes the data of patients suffering from CML, reporting to a major referral Institute in Northern Pakistan in the past 6 years and 3 months. CML constitutes approximately 80% of all myeloproliferative disorders, with a peak incidence between 21-50 years of age, and a male:female ratio of 2:1. Anaemia and massive splenomegaly were the main clinical features found in 92% and 47% patients respectively. There was significant correlation between anaemia and WBC counts with degree of splenomegaly. Three percent of all CML patients presented as de novo accelerated phase, and another 3% presented as blast crises without any previous history of chronic phase. The ratio of myeloid and lymphoid blast crisis was 2:1. Median duration of chronic phase in patients on hydroxyurea treatment was 6 years. Thirty six percent of patients in chronic phase of CML belonged to intermediate and high risk according to Sokal and Hasford scoring systems. In contrast to the Caucasian populations where the peak incidence of the disease is in 6th to 7th decade, CML occurs in Pakistan in a much younger population, with a broad peak between 21-50 years of age. Patients present in fairly advanced disease because of poor access to health care facilities, due to non-affordability and lack of health insurance coverage.
巴基斯坦慢性髓性白血病(CML)的临床和血液学特征分析尚未见报道。本研究分析了过去6年零3个月向巴基斯坦北部一家主要转诊机构报告的CML患者的数据。CML约占所有骨髓增生性疾病的80%,发病率高峰在21-50岁之间,男女比例为2:1。92%的患者表现为贫血,47%的患者表现为脾肿大。贫血和白细胞计数与脾肿大程度有显著相关性。3%的CML患者表现为新生加速期,另外3%表现为没有任何慢性期病史的突发危象。髓母细胞危象与淋巴母细胞危象的比例为2:1。接受羟基脲治疗的患者慢性期的中位持续时间为6年。根据Sokal和Hasford评分系统,慢性粒细胞白血病(CML)慢性期有36%的患者属于中高风险。高加索人群的发病率高峰出现在第6至第7个10岁,与此相反,CML在巴基斯坦发生在更年轻的人群中,在21至50岁之间广泛出现高峰。由于负担不起医疗费用和缺乏医疗保险,患者难以获得医疗保健设施,病情相当严重。
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引用次数: 11
Clinicopathological Features and Outcomes of T- and NK-Cell Lymphomas in European Russia 俄罗斯欧洲地区T细胞和nk细胞淋巴瘤的临床病理特征和预后
IF 3 Q2 Medicine Pub Date : 2012-01-01 DOI: 10.4137/CMBD.S7804
Y. Vinogradova, I. Kaplanskaya, R. S. Samoilova, I. Vorobiev, B. Zingerman, Y. Sidorova, N. Shklovskiy-Kordi, L. G. Aitova, D. C. Maryin, J. Morris, L. Varticovski, A. Vorobiev
With the introduction of the revised World Health Organization Classification of Tumors of Hematopoietic and Lymphoid Tissues in 2001, many patients had to be re-evaluated for the correct diagnosis of T- and NK-cell lymphomas. Because some T-cell malignancies are associated with poor outcome, it is important to identify subsets of patients that may benefit from novel or more intensive therapies. The purpose of this study was to determine, for the first time, the relative frequencies, pathological features and outcomes of patients with T- and NK-cell lymphomas in a predominantly Russian Slavic population. We identified 291 patients with a diagnosis of T- and NK-cell malignancies treated at our Center between 2000-2008. In applying the revised WHO classification, we confirmed the diagnosis and had complete clinical follow up and pathological information on 264 cases that were included in the analysis. We found some differences in frequency of several subsets as compared with previously published reports, including younger age of onset and relatively higher incidence of T-LGL in our patients. We also confirm that intensive treatment regimens of advanced stage PTCL and ALK—ALCL led to considerable improvement in response rates, but not in the overall survival.
随着2001年世界卫生组织修订的《造血和淋巴组织肿瘤分类》的引入,许多患者必须重新评估才能正确诊断T细胞淋巴瘤和nk细胞淋巴瘤。由于一些t细胞恶性肿瘤与不良预后相关,因此确定可能从新的或更强化的治疗中受益的患者亚群是很重要的。本研究的目的是首次确定以俄罗斯斯拉夫人口为主的T细胞和nk细胞淋巴瘤患者的相对频率、病理特征和预后。在2000-2008年期间,我们确定了291例诊断为T细胞和nk细胞恶性肿瘤的患者。在应用修订后的WHO分类时,我们对纳入分析的264例病例进行了确诊和完整的临床随访和病理信息。与之前发表的报告相比,我们发现几个亚群的频率存在一些差异,包括发病年龄更年轻和患者中T-LGL的发病率相对较高。我们还证实,晚期PTCL和ALK-ALCL的强化治疗方案在缓解率方面有相当大的改善,但在总生存期方面没有改善。
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引用次数: 0
Pattern of AST and ALT changes in Relation to Hemolysis in sickle cell Disease 镰状细胞病中与溶血相关的AST和ALT变化模式
IF 3 Q2 Medicine Pub Date : 2011-01-01 DOI: 10.4137/CMBD.S3969
K. Nsiah, V. Dzogbefia, Daniel Ansong, A. Akoto, H. Boateng, D. Ocloo
Background Elevated aminotransferase levels are commonly associated with compromised hepatic integrity from various insults. In sickle cell disease, aspartate transaminase (AST) is also released via intravascular hemolysis. This study was done to determine the pattern of changes in AST and alanine transaminase (ALT), in particular the AST:ALT ratio, and to relate these to the hemolytic state, which we consider to be more important than hepatic and cardiac dysfunction in some individuals with sickle cell disease. Methods Serum aminotransferase levels were measured in 330 subjects with sickle cell disease, as well as hemoglobin, reticulocytes, and lactate dehydrogenase. The AST:ALT ratio was designated as a hemolytic marker, and simple and multivariate regression analyses were carried out between this ratio and other hemolytic markers. Results Mean AST and ALT levels were 48.24 % 27.78 and 26.48 % 22.73 U/L, respectively. However, for 49 subjects without sickle cell disease, mean AST and ALT levels were the same, ie, 23.0 U/L. In the subjects with sickle cell disease, the increases in AST levels were far higher than for ALT, supporting its release via intravascular hemolysis. In 95.8% of the subjects with sickle cell disease, the AST:ALT ratio was > 1, but our results did not suggest overt malfunctioning of the liver and heart in the majority of subjects. Conclusion Regression analyses support the use of the AST:ALT ratio as a hemolytic marker, because it has an inverse association with the hemoglobin level. Whether in steady state or in crisis, provided hepatic and cardiac integrity has not been compromised, subjects with sickle cell disease would have higher AST levels due to the hemolytic nature of the condition. This is the first report highlighting the AST:ALT ratio in sickle cell disease.
背景:转氨酶水平升高通常与各种损伤引起的肝完整性受损有关。在镰状细胞病中,天冬氨酸转氨酶(AST)也通过血管内溶血释放。本研究是为了确定AST和丙氨酸转氨酶(ALT)的变化模式,特别是AST:ALT的比值,并将其与溶血状态联系起来,我们认为在一些镰状细胞病患者中,溶血状态比肝功能和心功能障碍更重要。方法测定330例镰状细胞病患者血清转氨酶、血红蛋白、网织红细胞和乳酸脱氢酶水平。选择AST:ALT比值作为溶血标志物,并将该比值与其他溶血标志物进行简单多元回归分析。结果AST、ALT平均水平分别为48.24%、27.78、26.48%、22.73 U/L。而在49例无镰状细胞病的受试者中,AST和ALT的平均水平相同,均为23.0 U/L。在镰状细胞病患者中,AST水平的升高远高于ALT水平,支持其通过血管内溶血释放。在95.8%的镰状细胞病患者中,谷丙转氨酶与谷丙转氨酶的比值为bb0.1,但我们的结果并未表明大多数患者存在明显的肝脏和心脏功能障碍。结论回归分析支持使用AST:ALT比值作为溶血标志物,因为它与血红蛋白水平呈负相关。无论处于稳定状态还是危重状态,只要肝和心脏的完整性未受到损害,镰状细胞病患者由于其溶血性,其AST水平均较高。这是第一个强调镰状细胞病中AST:ALT比值的报告。
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引用次数: 25
C1 Esterase Inhibitor (Human) for the Treatment of Acute Hereditary Angioedema C1酯酶抑制剂(人)治疗急性遗传性血管性水肿
IF 3 Q2 Medicine Pub Date : 2011-01-01 DOI: 10.4137/CMBD.S4090
S. Valle, A. T. França, R. Campos, A. Grumach
Hereditary angioedema (HAE) is a relatively rare disease characterized by acute episodes of swelling. These swellings can be disfiguring, painful and life-threatening. Since the symptoms occur in different areas and most patients experience a delay in their diagnosis, resulting in unnecessary suffering and dangerous situations. HAE can have a tremendous impact on the quality of life. The major genetic deficiency in this disorder is either an absent or nonfunctional C1INH which regulates the complement, fibrinolitic, kalikrein and plasmin pathways.
遗传性血管性水肿(HAE)是一种相对罕见的疾病,其特征是急性发作性肿胀。这些肿胀可能会毁容、疼痛并危及生命。由于症状出现在不同的部位,而且大多数患者的诊断出现延误,导致不必要的痛苦和危险情况。HAE可对生活质量产生巨大影响。这种疾病的主要遗传缺陷是缺乏或无功能的C1INH,它调节补体、纤溶蛋白、钾化酶和纤溶蛋白途径。
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引用次数: 1
Safety and Efficacy of Argatroban in the Management of Heparin-Induced Thrombocytopenia 阿加曲班治疗肝素性血小板减少症的安全性和有效性
IF 3 Q2 Medicine Pub Date : 2011-01-01 DOI: 10.4137/CMBD.S5118
B. Saugel, R. Schmid, W. Huber
Heparin-induced thrombocytopenia (HIT) is a life-threatening adverse reaction to heparin therapy that is characterized by thrombocytopenia and an increased risk of venous and arterial thrombosis. According to guidelines, in patients with strongly suspected or confirmed HIT all sources of heparin have to be discontinued and an alternative, nonheparin anticoagulant for HIT treatment must immediately be started. For both the prophylaxis of thrombembolic events in HIT and the treatment of HIT with thrombosis the direct thrombin inhibitor argatroban is approved in the United States. The objective of this review is to describe the mechanism of action and the pharmacokinetic profile of argatroban, to characterize argatroban regarding its safety and therapeutic efficacy and to discuss its place in therapy in HIT.
肝素诱导的血小板减少症(HIT)是肝素治疗的一种危及生命的不良反应,其特征是血小板减少和静脉和动脉血栓形成的风险增加。根据指南,对于强烈怀疑或确诊为HIT的患者,必须停止使用所有来源的肝素,并立即开始使用替代的非肝素抗凝剂治疗HIT。对于HIT患者血栓形成事件的预防和HIT合并血栓形成的治疗,直接凝血酶抑制剂阿加曲班已在美国获得批准。本综述的目的是描述阿加曲班的作用机制和药代动力学特征,描述阿加曲班的安全性和治疗效果,并讨论其在HIT治疗中的地位。
{"title":"Safety and Efficacy of Argatroban in the Management of Heparin-Induced Thrombocytopenia","authors":"B. Saugel, R. Schmid, W. Huber","doi":"10.4137/CMBD.S5118","DOIUrl":"https://doi.org/10.4137/CMBD.S5118","url":null,"abstract":"Heparin-induced thrombocytopenia (HIT) is a life-threatening adverse reaction to heparin therapy that is characterized by thrombocytopenia and an increased risk of venous and arterial thrombosis. According to guidelines, in patients with strongly suspected or confirmed HIT all sources of heparin have to be discontinued and an alternative, nonheparin anticoagulant for HIT treatment must immediately be started. For both the prophylaxis of thrombembolic events in HIT and the treatment of HIT with thrombosis the direct thrombin inhibitor argatroban is approved in the United States. The objective of this review is to describe the mechanism of action and the pharmacokinetic profile of argatroban, to characterize argatroban regarding its safety and therapeutic efficacy and to discuss its place in therapy in HIT.","PeriodicalId":43083,"journal":{"name":"Clinical Medicine Insights-Blood Disorders","volume":"16 1","pages":""},"PeriodicalIF":3.0,"publicationDate":"2011-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86433325","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 2
Platelet Gene Expression as a Biomarker Risk Stratification Tool in Acute Myocardial Infarction: A Pilot Investigation 血小板基因表达作为急性心肌梗死的生物标志物风险分层工具:一项试点研究
IF 3 Q2 Medicine Pub Date : 2010-01-01 DOI: 10.4137/CMBD.S5005
D. Calverley, I. Casserly, Qamrul G. Choudhury, T. Phang, B. Gao, J. Messenger, M. Geraci
Platelets play a major role in the pathophysiology of acute myocardial infarction (AMI). Recent evidence reveals megakaryocyte-derived platelet pre-mRNA is spliced to mRNA and then translated into functional proteins in response to external stimulation. An exon microarray analyzes pre-mRNA alternative splicing and is thus applicable for studying gene expression in the anucleate platelet. We hypothesized a subset of megakaryocyte/platelet genes exists that are significantly over or underexpressed in AMI compared with stable coronary artery disease (CAD), yielding a gene expression profile for further study. Microarray analysis employing platelet mRNA was used to generate gene expression data in the above two patient groups. Unsupervised hierarchical clustering has revealed an expression profile that includes 95 over- or under-expressed genes depicted in a heat map where separation of both sets takes place. This preliminary study reveals a platelet-based gene expression signature that differentiates between AMI and stable CAD, and further study may yield a prognostic tool for a future AMI event in atherosclerosis risk factor-based subsets of CAD patients.
血小板在急性心肌梗死(AMI)的病理生理中起着重要作用。最近的证据表明,巨核细胞衍生的血小板前mRNA与mRNA剪接,然后翻译成功能蛋白,以响应外部刺激。外显子微阵列分析前mrna选择性剪接,因此适用于研究无核血小板中的基因表达。我们假设存在一个巨核细胞/血小板基因亚群,与稳定型冠状动脉疾病(CAD)相比,在AMI中显著过表达或过表达,从而产生一个基因表达谱,供进一步研究。采用血小板mRNA微阵列分析生成上述两组患者的基因表达数据。无监督的分层聚类揭示了一种表达谱,其中包括在热图中描述的95个过度表达或表达不足的基因,其中两组分离发生。这项初步研究揭示了一种基于血小板的基因表达特征,可以区分AMI和稳定性CAD,进一步的研究可能会为基于动脉粥样硬化危险因素的CAD患者亚群中未来AMI事件的预后提供工具。
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引用次数: 5
Pulsed and Tissue Doppler Echocardiographic Changes in Patients with Thalassemia Major 重型地中海贫血患者的脉冲和组织多普勒超声心动图变化
IF 3 Q2 Medicine Pub Date : 2010-01-01 DOI: 10.4137/CMBD.S4377
Taysir Garadah, S. Kassab, Najat Mahdi, Ahmed A. Abu-Taleb, A. Jamsheer
Background Doppler echocardiographic studies of left ventricle (LV) systolic and diastolic function in patients with β-Thalassemia Major (β-TM) had shown different patterns of systolic and diastolic dysfunction. Aim This cross-sectional study was designed to study the LV systolic and diastolic function in patients with β-TM using Pulsed Doppler (PD) and Tissue Doppler (TD) echocardiography. Methods All patients were evaluated clinically and by echocardiography, The study included patients with β-TM (n = 38, age 15.7 ± 8.9 years) compared with an age-matched control group (n = 38, age 15.9 ± 8.9 years). The pulse Doppler indices were normalized for age and heart rate. Results Compared with control patients, M-Mode showed that patients with β-TM have thicker LV septal wall index (0.659 ± 0.23 vs. 0.446 ± 0.219 cm, P ≤ 0.001), posterior wall index (0.659 ± 0.235 vs. 0.437 ± 0.214 cm, P ≤ 0.01), and larger LVEDD index is (3.99 ± 0.48 vs. 2.170 ± 0.57 mm. P = 0.035). Pulsed Doppler showed high LV trans-mitral E wave velocity (70.818 ± 10.139 vs. 57.532 ± 10.139, p = 0.027) and E/A ratio (1.54 vs. 1.23, P ≤ 0.01). The duration of Deceleration time (DT) and isovolumic relaxation time (IVRT) were significantly shorter in patients with β-TM (150.234 ± 20.0.23 vs. 167.123 ± 19.143 msec, P ≤ 0.01) and (60.647 ± 6.77 vs. 75.474 ± 5.83 msec, P ≤ 0.001), respectively. The ratio of transmitral E wave velocity to the tissue Doppler E wave at the basal septal mitral annulus E/Em– was significantly higher in β-TM group (14.024 ± 2.29 vs. 12.132 ± 1.82, P ≤ 0.01). The Tissue Doppler systolic velocity (Sm) and the early diastolic velocity (Em) were significantly lower in β-TM group compared to control (4.31 ± 1.2 cm/s vs. 6.95 ± 2.1, P ≤ 0.01 and 4.31 ± 2.7 cm/s vs. 5.82 ± 2.5, P ≤ 0.01) respectively. The tricuspid valve velocity was significantly higher than controls (2.993 ± 0.569 vs. 1.93 ± 0.471 m/sec, respectively, P ≤ 0.01). However, the LVEF% and fractional shortening were normal with no significant difference in both groups. Conclusion In this study, patients with β-thalassemia major compared with controls, have significantly thicker LV wall, and larger LV cavity and LV diastolic filling indices suggestive of restrictive pattern with a higher tricuspid valve velocity. These data showed that left ventricle diastolic indices are compromised initially in patients with β-thalassemia major.
背景β-地中海贫血(β-TM)患者左心室(LV)收缩和舒张功能的多普勒超声心动图研究显示不同类型的收缩和舒张功能障碍。目的应用脉冲多普勒(PD)和组织多普勒(TD)超声心动图研究β-TM患者左室收缩和舒张功能。方法选取β-TM患者(n = 38,年龄15.7±8.9岁)与年龄匹配的对照组(n = 38,年龄15.9±8.9岁)进行临床及超声心动图评价。脉搏多普勒指数按年龄和心率归一化。结果与对照组相比,M-Mode显示β-TM患者左室间隔壁指数(0.659±0.23 vs 0.446±0.219 cm, P≤0.001)较厚,后壁指数(0.659±0.235 vs 0.437±0.214 cm, P≤0.01)较大,LVEDD指数为(3.99±0.48 vs 2.170±0.57 mm)。P = 0.035)。脉冲多普勒显示左室经二尖瓣E波速度高(70.818±10.139比57.532±10.139,p = 0.027), E/A比高(1.54比1.23,p≤0.01)。β-TM患者的减速时间(DT)和等容松弛时间(IVRT)均显著缩短(150.234±20.0.23∶167.123±19.143 msec, P≤0.01)和(60.647±6.77∶75.474±5.83 msec, P≤0.001)。β-TM组基底隔二尖瓣环透射E波速度与组织多普勒E波速度之比(14.024±2.29∶12.132±1.82,P≤0.01)显著高于β-TM组。β-TM组组织多普勒收缩速度(Sm)和早期舒张速度(Em)明显低于对照组(4.31±1.2 cm/s比6.95±2.1,P≤0.01)和4.31±2.7 cm/s比5.82±2.5,P≤0.01)。三尖瓣速度显著高于对照组(分别为2.993±0.569和1.93±0.471 m/sec, P≤0.01)。然而,LVEF%和分数缩短正常,两组无显著差异。结论与对照组相比,β-地中海贫血患者左室壁明显增厚,左室腔和左室舒张充盈指数明显增大,三尖瓣瓣速增高,提示限制性型。这些数据表明,β-地中海贫血患者左心室舒张指数最初受损。
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引用次数: 16
期刊
Clinical Medicine Insights-Blood Disorders
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