Thrombotic microangiopathies

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在2018年3月27日 (二) 09:58所做的修訂版本 (編輯)
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*Risk factors *Risk factors
-**Pregnancy, pills, quinine, RT p’ts given ~OKT3 for rejection, calcineurin inhibitors (cyclosporine, tacrolimus), antiplatelets (ticlopidine and clopidogrel), HIV+**Pregnancy, pills, quinine, RT p'ts given ~OKT3 for rejection, calcineurin inhibitors (cyclosporine, tacrolimus), antiplatelets (ticlopidine and clopidogrel), HIV
*Clinical features *Clinical features
**Thrombocytopenic purpura, hemolytic anemia, fever, renal failure (esp. HUS), neurologic disturbances (esp. TTP) **Thrombocytopenic purpura, hemolytic anemia, fever, renal failure (esp. HUS), neurologic disturbances (esp. TTP)
第10行: 第10行:
*2nd classification *2nd classification
**Adult HUS/TTP **Adult HUS/TTP
-***Familial: ~ADAMTS13 metalloprotease (that cleaves large multimers of von Willebrand's factor) deficiency+***Familial: ADAMTS13 metalloprotease (that cleaves large multimers of von Willebrand's factor) deficiency
***Sporadic ***Sporadic
-****Some p'ts have antibody to ~ADAMTS13 or thrombospondin receptor on endothelial cells+****Some p'ts have antibody to ADAMTS13 or thrombospondin receptor on endothelial cells
****Some p'ts have plasminogen-activator inhibitor 1 ↑ ****Some p'ts have plasminogen-activator inhibitor 1 ↑
**Childhood HUS **Childhood HUS

在2018年3月27日 (二) 09:58所做的修訂版本

  • Risk factors
    • Pregnancy, pills, quinine, RT p'ts given ~OKT3 for rejection, calcineurin inhibitors (cyclosporine, tacrolimus), antiplatelets (ticlopidine and clopidogrel), HIV
  • Clinical features
    • Thrombocytopenic purpura, hemolytic anemia, fever, renal failure (esp. HUS), neurologic disturbances (esp. TTP)
  • Pathology
    • Endotheliosis with fibrin thrombi
  • 1st classification
  • 2nd classification
    • Adult HUS/TTP
      • Familial: ADAMTS13 metalloprotease (that cleaves large multimers of von Willebrand's factor) deficiency
      • Sporadic
        • Some p'ts have antibody to ADAMTS13 or thrombospondin receptor on endothelial cells
        • Some p'ts have plasminogen-activator inhibitor 1 ↑
    • Childhood HUS
      • Escherichia coli O157:H7 (occasionally Shigella dysenteriae) causing enterohemorrhagic diarrhea
  • Treatment
    • Daily plasmapheresis
    • Patients with childhood HUS from infectious diarrhea are not given antibiotics, as antibiotics are thought to accelerate the release of the toxin and the diarrhea is usually self-limited.