急性血小板減少性紫斑病
WordNet
- of critical importance and consequence; "an acute (or critical) lack of research funds"
- having or demonstrating ability to recognize or draw fine distinctions; "an acute observer of politics and politicians"; "incisive comments"; "icy knifelike reasoning"; "as sharp and incisive as the stroke of a fang"; "penetrating insight"; "frequent penetrative observations" (同)discriminating, incisive, keen, knifelike, penetrating, penetrative, piercing, sharp
- extremely sharp or intense; "acute pain"; "felt acute annoyance"; "intense itching and burning" (同)intense
- having or experiencing a rapid onset and short but severe course; "acute appendicitis"; "the acute phase of the illness"; "acute patients"
- of an angle; less than 90 degrees
- any of several blood diseases causing subcutaneous bleeding (同)peliosis
PrepTutorEJDIC
- (先の)『鋭い』,とがった / (痛み・感情などが)『激しい』,強い / (知力・感覚などが)『鋭い』,鋭敏な / (事態が)重大な / (病気が)急性の / (音が)高い,鋭い / 鋭角の
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English Journal
- Alterations in immune cell subsets and their cytokine secretion profile in childhood idiopathic thrombocytopenic purpura (ITP).
- Talaat RM1, Elmaghraby AM, Barakat SS, El-Shahat M.Author information 1Molecular Biology Department, Genetic Engineering and Biotechnology Research Institute (GEBRI), University of Sadat City, Sadat City, Egypt.AbstractImmune thrombocytopenic purpura (ITP) is acquired autoimmune disease in children characterized by the breakdown of immune tolerance. This work is designed to explore the contribution of different lymphocyte subsets in acute and chronic ITP children. Imbalance in the T helper type 1 (Th1)/Th2 cytokine secretion profile was investigated. The frequency of T (CD3(+) , CD4(+) , CD8(+) ) and B (CD19(+) ) lymphocytes, natural killer (NK) (CD16(+) 56(+) ) and regulatory T (Treg ) [CD4(+) CD25(+high) forkhead box protein 3 (FoxP3)(+) ] cells was investigated by flow cytometry in 35 ITP children (15 acute and 20 chronic) and 10 healthy controls. Plasma levels of Th1 cytokines [interferon (IFN-γ) and tumour necrosis factor (TNF-α)] and Th2 [interleukin (IL)-4, IL-6 and IL-10)] cytokines were measured using enzyme-linked immunosorbent assay (ELISA). The percentage of Treg (P < 0·001) and natural killer (NK) (P < 0·001) cells were significantly decreased in ITP patients compared to healthy controls. A negative correlation was reported between the percentage of Treg cells and development of acute (r = -0·737; P < 0·01) and chronic (r = -0·515; P < 0·01) disease. All evaluated cytokines (IFN-γ, TNF-α, IL-4, IL-6 and IL-10) were elevated significantly in ITP patients (P < 0·001, P < 0·05, P < 0·05, P < 0·05 and P < 0·001, respectively) compared to controls. In conclusion, our data shed some light on the fundamental role of immune cells and their related cytokines in ITP patients. The loss of tolerance in ITP may contribute to the dysfunction of Tregs . Understanding the role of T cell subsets will permit a better control of autoimmunity through manipulation of their cytokine network.
- Clinical and experimental immunology.Clin Exp Immunol.2014 May;176(2):291-300. doi: 10.1111/cei.12279.
- Immune thrombocytopenic purpura (ITP) is acquired autoimmune disease in children characterized by the breakdown of immune tolerance. This work is designed to explore the contribution of different lymphocyte subsets in acute and chronic ITP children. Imbalance in the T helper type 1 (Th1)/Th2 cytokin
- PMID 24460857
- Expression of a structurally constrained von Willebrand factor variant triggers acute thrombotic thrombocytopenic purpura in mice.
- Morioka Y1, Casari C, Wohner N, Cho S, Kurata S, Kitano A, Christophe OD, Lenting PJ, Li R, Denis CV, Prévost N.Author information 1Career-Path Unit for Young Life Scientists, Kyoto University, Kyoto, Japan;AbstractThrombotic thrombocytopenic purpura (TTP) is a life-threatening disease that presents with thrombocytopenia, disseminated thrombosis, haemolytic anemia and organ dysfunction. The etiology of TTP has revealed that patients share a deficiency in plasma protease ADAMTS13, the enzyme responsible for cleaving ultra-large von Willebrand factor (VWF) multimers into non-thrombogenic fragments. Therefore, existing TTP mouse models were developed by targeted disruption of the ADAMTS13 gene. ADAMTS13-/- mice are mostly asymptomatic in the absence of a trigger, as redundant proteases appear to take on VWF processing. As an alternative approach to creating one such model, we have devised a strategy based on the expression of a cleavage resistant VWF mutant in mice. The creation of a disulfide bond within the A2 domain of VWF was found to render VWF multimers resistant to proteolysis by plasma proteases under flow. Furthermore, mice expressing the mVWF/p.S1494C-p.A1534C mutant present with symptoms characteristics of acute TTP such as thrombocytopenia, red cell shredding, accumulation of VWF-rich thrombi in the microvasculature and advanced TTP symptoms such as renal dysfunction and splenomegaly. Because this model appears to faithfully emulate the pathophysiology of TTP, it should prove most useful in the study of microangiopathic diseases and their treatment.
- Blood.Blood.2014 Apr 8. [Epub ahead of print]
- Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease that presents with thrombocytopenia, disseminated thrombosis, haemolytic anemia and organ dysfunction. The etiology of TTP has revealed that patients share a deficiency in plasma protease ADAMTS13, the enzyme responsible for cle
- PMID 24713928
- Persistence of circulating ADAMTS13-specific immune complexes in patients with acquired thrombotic thrombocytopenic purpura.
- Ferrari S1, Palavra K, Gruber B, Kremer Hovinga JA, Knöbl P, Caron C, Cromwell C, Aledort L, Plaimauer B, Turecek PL, Rottensteiner H, Scheiflinger F.Author information 1Hanspeter_Rottensteiner@baxter.com.AbstractAnti-ADAMTS13 autoantibodies are the main cause of acquired thrombotic thrombocytopenic purpura. Binding of these antibodies to ADAMTS13 eventually results in the formation of antigen-antibody immune complexes. Circulating ADAMTS13-specific immune complexes have been described in patients with acquired thrombotic thrombocytopenic purpura, although the prevalence and persistence of these immune complexes over time have hitherto remained elusive. Here, we analyzed a large cohort of patients with acquired thrombotic thrombocytopenic purpura for the presence of free and complexed anti-ADAMTS13 antibodies. In the acute phase (n=68), 100% of patients had free IgG antibodies and 97% had ADAMTS13-specific immune complexes. In remission (n=28), 75% of patients had free antibodies (mainly IgG) and 93% had ADAMTS13-specific immune complexes. Free antibodies were mainly of subclasses IgG1 and IgG4, whereas IgG4 was by far the most prevalent in ADAMTS13-specific immune complexes. Comparison of ADAMTS13 inhibitor and anti-ADAMTS13 IgG (total and subclasses) antibody titers in acute phase and in remission samples showed a statistically significant decrease in all parameters in remission. Although non-significant, a trend towards reduced or undetectable titers in remission was also observed for ADAMTS13-specific immune complexes of subclasses IgG1, IgG2 and IgG3. No such trend was discernible for IgG4; IgG4 immune complexes persisted over years, even in patients who had been treated with rituximab and who showed no features suggesting relapse.
- Haematologica.Haematologica.2014 Apr;99(4):779-87. doi: 10.3324/haematol.2013.094151. Epub 2013 Nov 15.
- Anti-ADAMTS13 autoantibodies are the main cause of acquired thrombotic thrombocytopenic purpura. Binding of these antibodies to ADAMTS13 eventually results in the formation of antigen-antibody immune complexes. Circulating ADAMTS13-specific immune complexes have been described in patients with acqui
- PMID 24241492
Japanese Journal
- 症例報告 TPO受容体作動薬を使用し抗凝固療法への移行が可能であった下肢深部静脈血栓症合併慢性ITP急性増悪
- 血液疾患に対するアフェレシス療法の臨床評価(<特集>アフェレシス療法における臨床評価方法について)
- 上田 恭典
- 日本アフェレシス学会雑誌 33(2), 123-128, 2014-05-31
- … Plasma exchange is the most important procedure to treat thrombotic microangiopathy, including acquired idiopathic thrombotic thrombocytopenic purpura, and atypical hemolytic uremic syndrome. …
- NAID 110009823175
- 症例報告 低用量エルトロンボパグで寛解を維持できた高齢者 : 急性特発性血小板減少性紫斑病の1例
Related Links
- Many children with acute ITP have an increased number of normal or atypical lymphocytes on the peripheral smear, reflecting a recent viral illness. Clumps of platelets on a peripheral smear prepared from ...
- ... and no therapy in childhood acute immune thrombocytopenic purpura. J Pediatr. 1993 Dec. 123(6):989-95. . Borst F, Keuning JJ, van Hulsteijn H, Sinnige H, Vreugdenhil G. High-dose dexamethasone as a first- and second . . ...
★リンクテーブル★
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- 英
- acute thrombocytopenic purpura
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- (疾患)急性の、急性型の、急性的な。(形状が)鋭い、鋭角の。(感覚、才知などが)鋭い。明敏な、鋭い眼識のある。
- 関
- acutely、quick、sharp
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血小板減少性紫斑病