Registry of 919 patients with thrombotic microangiopathies across Japan: database of Nara Medical University during 1998-2008.

@article{Fujimura2010RegistryO9,
  title={Registry of 919 patients with thrombotic microangiopathies across Japan: database of Nara Medical University during 1998-2008.},
  author={Y. Fujimura and M. Matsumoto},
  journal={Internal medicine},
  year={2010},
  volume={49 1},
  pages={
          7-15
        }
}
BACKGROUND Thrombotic microangiopathies (TMAs) are pathological conditions characterized by generalized microvascular occlusion by platelet thrombi, thrombocytopenia, and microangiopathic hemolytic anemia. Two typical phenotypes of TMAs are hemolytic-uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). Severe deficiency of plasma ADAMTS13 activity (ADAMTS13: AC) is more specific for TTP, but not for HUS. Since 1998, our laboratory has functioned as a nationwide referral center… Expand
Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura): a cross-sectional analysis of the French national registry for thrombotic microangiopathy.
TLDR
Thrombotic thrombocytopenic purpura is a heterogeneous syndrome, and that features of the disease at presentation are strongly associated with the mechanisms of ADAMTS13 deficiency, which could have implications for the initial therapeutic management of patients with this disorder. Expand
Acquired Idiopathic ADAMTS13 Activity Deficient Thrombotic Thrombocytopenic Purpura in a Population from Japan
TLDR
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TLDR
58 patients (41 USS and 17 acquired TTP) who were diagnosed with a severe deficiency in ADAMTS13 activity during childhood are focused on, causing a paradigm shift in the concept of TTP. Expand
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The differential diagnosis of thrombotic microangiopathy (TMA) has become clearer following the establishment of the relationships between (1) diarrhea-associated hemolytic uremic syndrome (HUS) andExpand
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Current and Future Perspectives on ADAMTS13 and Thrombotic Thrombocytopenic Purpura.
TLDR
Novel diagnostic assays, targeted treatments, and the unraveling of both ADAMTS13 function and TTP pathophysiology should help to further improve the patients' quality of life. Expand
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TLDR
Long-term follow-up after the acute episode is critical to monitor for relapse and to diagnose and manage chronic sequelae of this disease. Expand
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TLDR
A case-oriented study that retrospectively analyzed the cases of acquired TTP patients treated in a single institution from 2000 to 2011 to provide a future direction for developing a more efficient treatment modality is presented. Expand
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TLDR
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Thrombotic microangiopathies: thrombotic thrombocytopenic purpura / hemolytic uremic syndrome.
  • M. G. Polito, G. Kirsztajn
  • Medicine
  • Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
  • 2010
TLDR
Although TMAs are highly heterogeneous pathological conditions, one-third of TMA patients have severe deficiency of ADAMTS13, and platelet transfusions are contraindicated, Plasma infusion or exchange is the only treatment of proven efficacy. Expand
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TLDR
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