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血栓性血小板减少紫癜1例

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哈尔滨医科大学学报 1991年第5期25卷 380页

作者：陈学新哈尔滨医科大学二院血液病研究所

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血栓性血小板减小性紫癜1例

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临床荟萃 2003年第5期18卷 236-236页

作者：杨喆习杰英梁长安西安交通大学第一医院血液科陕西西安710061 西安交通大学第一医院血液科陕西扶风722200 扶风县医院内三科

患者男,52岁,教师,头痛、鼻塞7天,全身皮肤紫癜2天入院.

关键词：紫癜血栓性血小板减少性

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上腹部不适烧灼感皮肤淤斑全身黄染

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中国实用内科杂志 2007年第12期27卷 989-991页

作者：吴玉红刘春燕付蓉宋嘉王珺邵宗鸿天津医科大学总医院血液科天津300052

1病例资料患者男,62岁.因上腹不适,烧灼感4 d,于2006-07-12住天津医科大学总医院高干科.入院时体温正常,皮肤黏膜轻度黄染,仝身未见出血点,上腹部轻压痛,血常规示红细胞(RBC)4．59×l0~(12)／L,血红蛋白(Hb)143 g／L,白细胞(WBC)8．... 详细信息

1病例资料患者男,62岁.因上腹不适,烧灼感4 d,于2006-07-12住天津医科大学总医院高干科.入院时体温正常,皮肤黏膜轻度黄染,仝身未见出血点,上腹部轻压痛,血常规示红细胞(RBC)4．59×l0~(12)／L,血红蛋白(Hb)143 g／L,白细胞(WBC)8．8×10~9／L,血小板(PI )66×10~9／L,肝功能异常,直接及间接胆红素升高,给予制酸、护肝等对症处理.2 d后患者出现烦躁、呕吐,呕吐物中带血,血尿(+),无血便,体温38．O℃ ,皮肤巩膜黄染逐渐加重,Hb降至97 g／L,PLT降至41×10~9／L,诊断“上消化道出血”.予禁食、留置胃管、抗炎、血小十反和血浆输注,症状无好转.疑诊咀栓性血小板减少性紫癜(_丌P),为行血浆置换于7月17日转入血液科.既往有卜二指肠球部溃疡史10年.体检：体温37．5℃ ,脉搏90／min,呼吸18／min,血压128／80 mm Hg(1 mm Hg=0．133 kPa).神志清楚,消瘦,精神萎靡,中度贫m貌,全身皮肤重度黄染,四肢可见散在出血点、淤斑,浅表淋巴结尢肿大,巩膜重度黄染.左鼻腔中带有一条胃管,有少量暗红色的引流液.颈软,颈静脉无怒张,胸骨无压痛,双肺呼吸音清,未闻及干湿哕音.心率90／min,律齐,尤杂音.腹软,肝脾双肾未及,腹部未及包块,移动性浊音(一),生理反射存在,病理反射未引出.

关键词：紫癜血栓性血小板减少性胃癌转移性血浆置换

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英文现刊输血医学文献题录汉译选辑

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国际输血及血液学杂志 1992年第5期 319-322页

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ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases

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BLOOD 2005年第4期106卷 1262-1267页

作者： Rieger, M Mannucci, PM Hovinga, JAK Herzog, A Gerstenbauer, G Konetschny, C Zimmermann, K Scharrer, I Peyvandi, F Galbusera, M Remuzzi, G Böhm, M Plaimauer, B Lämmle, B Scheiflinger, F Baxter BioSci Biomed Res Ctr Orth Austria Maggiore Hosp Angelo Bianchi Bonomi Hemophilia & Thrombosis Ctr Milan Italy Maggiore Hosp Fdn Luigi Villa Dept Internal Med & Dermatol IRCCS Milan Italy Univ Milan Milan Italy Univ Hosp Inselspital Dept Hematol Bern Switzerland Univ Hosp Inselspital Cent Hematol Lab Bern Switzerland Mario Negri Inst Pharmacol Res I-24100 Bergamo Italy Univ Hosp Dept Hemostaseol Frankfurt Germany Osped Riuniti Bergamo Div Nephrol & Dialysis Bergamo Italy

Autoantibodies neutralizing human ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motif), the metalloprotease that physiologically cleaves von Willebrand factor, are a major cause of severe deficiency of the protease and of acquired thrombotic thrombocytopenic purpura (TTP). We evaluated prevalence of anti-ADAMTS13 antibodies in 59 patients with thrombotic microangiopathies (TMAs) and in 160 patients with immunologic or thrombocytopenic diseases different from TTP, using an enzyme-linked immunosorbent assay (ELISA). Immunoglobulin G (IgG) antibodies directed against ADAMTS13 were found in 97% of untreated patients with acute acquired TMA who had plasma levels of ADAMTS13 activity below 10%. The corresponding prevalence of IgM antibodies was 11%. In contrast, anti-ADAMTS13 antibodies of G or M isotypes were detected in 20% of patients with TMA with ADAMTS13 activity above 10%. The ELISA was more sensitive than the standard functional inhibitor assay for detecting antibodies against ADAMTS13. Patients with thrombocytopenia from various causes (n = 50), systemic lupus erythematosus (SLE;n = 40), and the antiphospholipid antibody syndrome (APS;n = 55) had prevalences of IgG antibodies of 8%, 13%, and 5% respectively, only slightly higher than the prevalence in 111 healthy donors (4%). A rather high prevalence of anti-ADAMTS13 IgM antibodies was found in patients with SLE and APS (18% each). The clinical significance of IgM antibodies in these groups is unclear. In conclusion, the ELISA method detected anti-ADAMTS13 IgG antibodies in a very large proportion of patients with acquired TMA associated with severe ADAMTS13 deficiency, and was more sensitive than the inhibitor assay.

关键词： Autoantibodies Metalloendopeptidases Purpura Thrombotic Thrombocytopenic 自身抗体紫癜血栓性血小板减少性

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Complement factor H mutations and gene polymorphisms in haemolytic uraemic syndrome: the C-257T, the A2089G and the G2881T polymorphisms are strongly associated with the disease

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HUMAN MOLECULAR GENETICS 2003年第24期12卷 3385-3395页

作者： Caprioli, J Castelletti, F Bucchioni, S Bettinaglio, P Bresin, E Pianetti, G Gamba, S Brioschi, S Daina, E Remuzzi, G Noris, M Mario Negri Inst Pharmacol Res Clin Res Ctr Rare Dis Aldo & Cele Dacco I-24020 Ranica Italy Osped Riuniti Bergamo Azienda Osped Unit Nephrol & Dialysis I-24100 Bergamo Italy

Mutations in complement factor H (HF1) gene have been reported in non-Shiga toxin-associated and diarrhoea-negative haemolytic uraemic syndrome (D-HUS). We analysed the complete HF1 in 101 patients with HUS, in 32 with thrombotic thrombocytopenic purpura (TTP) and in 106 controls to evaluate the frequency of HF1 mutations, the clinical outcome in mutation and non-mutation carriers and the role of HF1 polymorphisms in the predisposition to HUS. We found 17 HF1 mutations (16 heterozygous, one homozygous) in 33 HUS patients. Thirteen mutations were located in exons XXII and XXIII. No TTP patient carried HF1 mutations. The disease manifested earlier and the mortality rate was higher in mutation carriers than in non-carriers. Kidney transplants invariably failed for disease recurrences in patients with HF1 mutations, while in non-mutated patients half of the grafts were functioning after 1 year. Three HF1 polymorphic variants were strongly associated with D-HUS: -257T (promoter region), 2089G (exonXIV, silent) and 2881T (963Asp, SCR16). The association was stronger in patients without HF1 mutations. Two or three disease-associated variants led to a higher risk of HUS than a single one. Analysis of available relatives of mutated patients revealed a penetrance of 50%. In 5/9 families the proband inherited the mutation from one parent and two disease-associated variants from the other, while unaffected carriers inherited the protective variants. In conclusion HF1 mutations are frequent in patients with D-HUS (24%). Common polymorphisms of HF1 may contribute to D-HUS manifestation in subjects with and without HF1 mutations.

关键词： Complement Factor H Hemolytic-Uremic Syndrome Mutation Polymorphism (Genetics) 补体因子H 溶血尿毒症综合征突变多态现象(遗传学) 紫癜血栓性血小板减少性 Mutation Complement Factor H Gene polymorphism CFH gene Hemolytic-Uremic Syndrome Genetic Polymorphism

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Thrombotic microangiopathy and hypothermia in an HIV-positive patient: Importance of cytomegalovirus infection

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SCANDINAVIAN JOURNAL OF INFECTIOUS DISEASES 2004年第3期36卷 234-236页

作者： Rerolle, JP Canaud, G Fakhouri, F Aaron, L Morelon, E Peraldi, MN Kreis, H Bruneel, MFM Hop Necker Enfants Malad Serv Transplantat Renale Dept Renal Transplantat F-75743 Paris 15 France Hop Necker Enfants Malad Dept Nephrol F-75743 Paris France Hop Necker Enfants Malad Dept Infect Dis F-75743 Paris 15 France

Cytomegalovirus (CMV) infection is a well-known frequent complication in HIV-infected patients. We report a case of thrombotic microangiopathy and severe hypothermia in a 21-y-old woman positive for HIV infection. CMV infection was diagnosed by PCR. The symptoms completely resolved after ganciclovir therapy which supports the role of CMV as a causative agent.

关键词： hypothermia natural HIV Cytomegalovirus Infections cisplatin/methotrexate/vinblastine 巨细胞病毒感染紫癜血栓性血小板减少性 Seroconversion Cytomegalovirus Hypothermia Thrombotic Microangiopathies HIV Cytomegalovirus Infections

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Evolution of ADAMTS13 antibodies in a fatal case of thrombotic thrombocytopenic purpura

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AMERICAN JOURNAL OF HEMATOLOGY 2008年第10期83卷 815-817页

作者： Dong, Lingli Chandrasekaran, Visalam Zhou, Wenhua Tsai, Han-Mou New York Blood Ctr Dept Educ New York NY 10021 USA Montefiore Med Ctr Unified Div Hematol Bronx NY 10467 USA

In a patient with fatal thrombotic thrombocytopenic purpura, the inhibitory activity of antibodies against ADAMTS13 rapidly escalated to extremely high levels despite daily plasma exchange and corticosteroid therapy. This increase was found to be because of a combination of higher antibody concentration and potency. Furthermore, during her course of the disease, the percentage of IgG(1) antibody progressively decreased whereas that of IgG(2) antibody increased, suggesting Th1-type cytokine response. These changes suggest that the course of TTP may be exacerbated by complex immune reactions. Further characterization of the factors contributing to this exacerbation may have important pathogenetic and therapeutic implications.

关键词： ADAM Proteins Adolescent Adrenal Cortex Hormones Autoantibodies Enzyme Inhibitors 肾上腺皮质激素类自身抗体酶抑制剂致命性结局血浆交换紫癜血栓性血小板减少性 Enzyme Inhibitors Adolescent characterization factor Adrenal Cortex Hormones Plasma Exchange steroid treatment ADAM Proteins ADAMTS13 gene fatality Autoantibodies Thrombotic Thrombocytopenic Purpura

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71-year-old woman with fever and altered mental status

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MAYO CLINIC PROCEEDINGS 2007年第2期82卷 237-240页

作者： Chee, Cheng E. Mohr, David N. Mayo Clin Coll Med Div Gen Internal Med Mayo Sch Grad Med Educ Rochester MN 55905 USA

A 71-year-old woman with a history of type 2 diabetes mellitus, hypertension, and hyperlipidemia was hospitalized at our institution because of a sudden change in mental status. Two months previously, she had been hospitalized elsewhere for nausea, vomiting, and diarrhea. At that time, a duodenal ulcer and acute renal failure attributed to volume depletion-were diagnosed, although renal biopsies performed during hospitalization showed focal segmental glomerulosclerosis with collapsing features, acute tubular necrosis, and thrombotic microangiopathy with glomerular capillary wall remodeling (double contours). No medication was administered for the renal failure. A dialysis catheter was placed and dialysis initiated, but the patient\'s symptoms persisted. During the next 2 months, she experienced an 11.25-kg weight loss. Two days before the current admission, the patient noted neck and shoulder pain after sleeping on a new pillow and took benzodiazepine for symptom relief. During the 24 hours before admission, her daughter noted a change in behavior, somnolence, and during the last few hours before presentation, agitation and confusion. The patient did not have chills, dysuria, or pain.

关键词： Aged Endocarditis Bacterial Female Hemolytic-Uremic Syndrome Humans 老年人心内膜炎细菌性溶血尿毒症综合征紫癜血栓性血小板减少性葡萄球菌感染 altered mental status Focal Segmental Glomerulosclerosis Shoulder Pain dialysis catheter Thrombotic Microangiopathies Endocarditis Acute Kidney Tubular Necrosis Hemolytic-Uremic Syndrome Patient symptoms

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Rituximab prevents recurrence of thrombotic thrombocytopenic purpura:: a case report

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BLOOD 2005年第3期106卷 925-928页

作者： Galbusera, M Bresin, E Noris, M Gastoldi, S Belotti, D Capoferri, C Daina, E Perseghin, P Scheiflinger, F Fakhouri, F Grünfeld, JP Pogliani, E Remuzzi, G Mario Negri Inst Pharmacol Res Clin Res Ctr Rare Dis I-24125 Bergamo Italy Osped San Gerardo Unit Hematol & Immunohematol Monza Italy Osped San Gerardo Ctr Blood Transfus Monza Italy Baxter Biosci Biomed Res Ctr A-2304 Orth Austria Hop Necker Enfants Malad Nephrol Unit Paris France Osped Riuniti Bergamo Unti Nephrol & Dialysis Azienda Osped Bergamo Italy

Thrombotic thrombocytopenic purpura (TTP) is a rare disorder of small vessels that is associated with deficiency of the von Willebrand factor-cleaving protease, ADAMTS13. The presence of anti-ADAMTS13 autoantibodies is considered a factor predisposing to relapses. Despite close monitoring and intensive plasma treatment, in these patients acute episodes are still associated with substantial morbidity and mortality rates, and the optimal therapeutic option should be prevention of relapses. This study was conducted in a patient with recurrent TTP due to high titers of ADAMTS13 inhibitors, who used to have 2 relapses of TTP a year. The study compared the standard treatment plasma exchange with rituximab. Results documented that plasma exchange had only a small transient effect on ADAMTS1 3 activity and inhibitors;on the contrary, prophylaxis with rituximab was associated with disappearance of anti-ADAMTS13 antibodies, a progressive recovery of protease activity, and it allowed the patient to maintain a disease-free state during a more than 2-year follow-up.

关键词： Antibodies Monoclonal Purpura Thrombotic Thrombocytopenic 抗体单克隆紫癜血栓性血小板减少性

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