A Novel Strategy for Hemolytic Uremic Syndrome: Successful Treatment With Thrombomodulin α

@article{Honda2013ANS,
  title={A Novel Strategy for Hemolytic Uremic Syndrome: Successful Treatment With Thrombomodulin $\alpha$},
  author={Takashi Honda and Shohei Ogata and Eri Mineo and Yukako Nagamori and Shinya Nakamura and Yuki Bando and Masahiro Ishii},
  journal={Pediatrics},
  year={2013},
  volume={131},
  pages={e928 - e933}
}
Hemolytic uremic syndrome (HUS) is a life-threatening infectious disease in childhood for which there is no confirmed therapeutic strategy. Endothelial inflammation leading to microthrombosis formation via complement activation is the main pathology of HUS. Thrombomodulin is an endothelial membrane protein that has anticoagulation and anti-inflammatory effects, including the suppression of complement activity. Recombinant human soluble thrombomodulin (rTM) is a novel therapeutic medicine for… 
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Efficacy of recombinant human soluble thrombomodulin for childhood hemolytic uremic syndrome
TLDR
The present results suggest that rhTM may be a safe and effective treatment for DIC complicated with HUS in children.
Management of hemolytic uremic syndrome
TLDR
The current understanding of the pathogenesis of HUS is reviewed and how this has led to advances in patient care is reviewed to improve outcomes in patients with atypical forms of H US.
Post‐transplant recurrence of atypical hemolytic uremic syndrome in a patient with thrombomodulin mutation
TLDR
The present report suggests that THBD mutations may favor‐relapse of aHUS after renal transplantation, and reports the case of a 19‐yr‐old man with aH US secondary to a THBD mutation that relapsed twice after two renal transplantations performed 12 yr apart.
Postinfectious Hemolytic Uremic Syndrome
TLDR
Treatment of STEC-HUS is supportive; more investigations are needed to evaluate the efficacy of putative preventive and therapeutic measures, such as non-phage-inducing antibiotics, volume expansion and anti-complement agents.
Effect of eculizumab and recombinant human soluble thrombomodulin combination therapy in a 7-year-old girl with atypical hemolytic uremic syndrome due to anti-factor H autoantibodies
TLDR
In conclusion, combination therapy of eculizumab and rTMD was effective for an aHUS patient, and this therapy may be helpful for improving the prognosis and long-term kidney function of aH US patients.
Thrombomodulin in disseminated intravascular coagulation and other critical conditions—a multi-faceted anticoagulant protein with therapeutic potential
TLDR
The accumulated knowledge of endogenous as well as recombinant thrombomodulin from basic to clinical aspects is summarized and future directions for this novel therapeutic agent are suggested.
Shiga Toxin-Associated Hemolytic Uremic Syndrome: A Narrative Review
TLDR
This work reviews the current knowledge of STEC virulence, how societies organize the prevention of human disease, and how physicians treat its potentially fatal complications, and focuses on STEC-induced hemolytic and uremic syndrome.
Guidelines for the management and investigation of hemolytic uremic syndrome
TLDR
The aim of this set of guidelines is to provide a support, tool for daily medical practice and to contribute to the standardization and accessibility of HUS-related medical care, as well as to improve level of safety for HUS patients.
Thrombomodulin and Endothelial Dysfunction: A Disease-Modifier Shared between Malignant Hypertension and Atypical Hemolytic Uremic Syndrome
TLDR
A woman presenting with malignant hypertension (MHT) and associated kidney, brain, cardiac, and hematological involvement with thrombotic microangiopathy on kidney biopsy had a documented mutation of the gene coding for TM, which was associated with both aHUS and an increased risk for venous and arterialThrombosis.
The efficacy of recombinant human soluble thrombomodulin for the treatment of shiga toxin-associated hemolytic uremic syndrome model mice.
  • K. Suyama, Y. Kawasaki, +5 authors M. Hosoya
  • Biology, Medicine
    Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
  • 2015
TLDR
The results indicate that rhTM might afford an efficacious treatment for t-HUS model mice via the inhibition of further thrombin formation and amelioration of hypercoagulant status.
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