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Hemolytiskt uremiskt syndrom och trombotisk trombocytopen purpura. Nya rön om EHEC, komplementmutationer och ADAMTS13.

Karpman, Diana LU (2008) In Läkartidningen 105(15). p.1096-1101
Abstract
Thrombotic microangiopathy is the pathological lesion found in hemolytic uremic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP) as well as other conditions. It manifests as damaged endothelial cells in small vessels with deposits of platelet plugs, occlusion of vessels, decreased blood flow and secondary organ insufficiency. HUS is characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. It may be associated with gastrointestinal infection with Shiga toxin-producing bacteria (enterohemorrhagic Escherichia coli or Shigella dysenteriae) causing a prodrome of diarrhea followed in some cases by HUS. Atypical HUS may be hereditary and recurrent, and may, in certain cases, be associated with... (More)
Thrombotic microangiopathy is the pathological lesion found in hemolytic uremic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP) as well as other conditions. It manifests as damaged endothelial cells in small vessels with deposits of platelet plugs, occlusion of vessels, decreased blood flow and secondary organ insufficiency. HUS is characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. It may be associated with gastrointestinal infection with Shiga toxin-producing bacteria (enterohemorrhagic Escherichia coli or Shigella dysenteriae) causing a prodrome of diarrhea followed in some cases by HUS. Atypical HUS may be hereditary and recurrent, and may, in certain cases, be associated with dysregulation of the alternative pathway of complement. TTP is characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, renal and CNS manifestations. It has been associated with deficient activity of ADAMTS13, the von Willebrand factor cleaving protease. This review will focus on the classification, symptoms, pathology, pathogenesis, investigation, management and treatment of these conditions. (Less)
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keywords
ADAM Proteins: blood, ADAM Proteins: genetics, ADAM Proteins: immunology, Biological Markers: blood, Complement System Proteins: genetics, Enterohemorrhagic Escherichia coli: pathogenicity, Hemolytic-Uremic Syndrome: diagnosis, Hemolytic-Uremic Syndrome: etiology, Hemolytic-Uremic Syndrome: therapy, Kidney Cortex: pathology, Purpura, Thrombotic Thrombocytopenic: diagnosis, Thrombotic Thrombocytopenic: etiology, ADAM Proteins: deficiency, Thrombotic Thrombocytopenic: therapy, Thrombosis: diagnosis, Thrombosis: etiology
in
Läkartidningen
volume
105
issue
15
pages
1096 - 1101
publisher
Sveriges Läkarförbund
external identifiers
  • pmid:18561752
  • scopus:42649137892
ISSN
0023-7205
language
Swedish
LU publication?
yes
id
dcb22119-70bb-48e9-9050-27df196efd24 (old id 1168721)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/18561752?dopt=Abstract
http://ltarkiv.lakartidningen.se/artNo34933
date added to LUP
2008-07-04 10:55:43
date last changed
2017-01-01 07:46:28
@article{dcb22119-70bb-48e9-9050-27df196efd24,
  abstract     = {Thrombotic microangiopathy is the pathological lesion found in hemolytic uremic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP) as well as other conditions. It manifests as damaged endothelial cells in small vessels with deposits of platelet plugs, occlusion of vessels, decreased blood flow and secondary organ insufficiency. HUS is characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. It may be associated with gastrointestinal infection with Shiga toxin-producing bacteria (enterohemorrhagic Escherichia coli or Shigella dysenteriae) causing a prodrome of diarrhea followed in some cases by HUS. Atypical HUS may be hereditary and recurrent, and may, in certain cases, be associated with dysregulation of the alternative pathway of complement. TTP is characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, renal and CNS manifestations. It has been associated with deficient activity of ADAMTS13, the von Willebrand factor cleaving protease. This review will focus on the classification, symptoms, pathology, pathogenesis, investigation, management and treatment of these conditions.},
  author       = {Karpman, Diana},
  issn         = {0023-7205},
  keyword      = {ADAM Proteins: blood,ADAM Proteins: genetics,ADAM Proteins: immunology,Biological Markers: blood,Complement System Proteins: genetics,Enterohemorrhagic Escherichia coli: pathogenicity,Hemolytic-Uremic Syndrome: diagnosis,Hemolytic-Uremic Syndrome: etiology,Hemolytic-Uremic Syndrome: therapy,Kidney Cortex: pathology,Purpura,Thrombotic Thrombocytopenic: diagnosis,Thrombotic Thrombocytopenic: etiology,ADAM Proteins: deficiency,Thrombotic Thrombocytopenic: therapy,Thrombosis: diagnosis,Thrombosis: etiology},
  language     = {swe},
  number       = {15},
  pages        = {1096--1101},
  publisher    = {Sveriges Läkarförbund},
  series       = {Läkartidningen},
  title        = {Hemolytiskt uremiskt syndrom och trombotisk trombocytopen purpura. Nya rön om EHEC, komplementmutationer och ADAMTS13.},
  volume       = {105},
  year         = {2008},
}