Study of 'Vascular Competence' Profile and Endothelial Activation in the Hemolytic Uremic Syndrome in Children and Adults
Study of 'Vascular Competence' Profile and Endothelial Activation in the Hemolytic Uremic Syndrome in Children and Adults
The Hemolytic Uremic Syndrome (HUS) is a rare thrombotic microangiopathy (TMA), affecting
 both children and adults. HUS is characterized by the abnormal occurrence of diffuse
 thrombosis in the microcirculation resulting in the occurrence of ischemic events affecting
 especially the kidneys and is associated with hemolytic anemia. One of the major problems
 encountered in the management of HUS is the absence of reliable marker of treatment response
 or relapse; conventional hematological markers being too insensitive to judge therapeutic
 efficacy or identify early relapse. Data from the literature suggest that the endothelial
 cell is a major target of this syndrome. Our hypothesis is that an initial micro-endothelial
 activation plays a critical role in the initiation and / or relapse of the disease.The main
 objective of this study is to define a "vascular competence" profile in a population of
 patients with typical or atypical HUS; both in the acute phase and in remission of the
 disease.
The Hemolytic Uremic Syndrome (HUS) is a rare thrombotic microangiopathy (TMA), affecting
 both children and adults. HUS is characterized by the abnormal occurrence of diffuse
 thrombosis in the microcirculation resulting in the occurrence of ischemic events affecting
 especially the kidneys and is associated with hemolytic anemia.Its prognosis is severe, with
 a mortality of 1% in children, 10% in adults and the occurrence of renal failure in 50% of
 cases.In its typical form, HUS occurs in the aftermath of a diarrheic intestinal infection by
 bacteria which produce a Shiga toxin. In its unusual shape, which affects both children and
 adults, there are genetic abnormalities alternate way of regulating complement proteins
 explaining frequent relapses.One of the major problems encountered in the management of HUS
 is the absence of reliable marker of treatment response or relapse; conventional
 hematological markers being too insensitive to judge therapeutic efficacy or identify early
 relapse. Data from the literature suggest that the endothelial cell is a major target of this
 syndrome. Our hypothesis is that an initial micro-endothelial activation plays a critical
 role in the initiation and / or relapse of the disease through the sudden release of high
 molecular weight ultralarge von Willebrand factor (UL-vWHf) and procoagulant endothelial
 microparticles.The main objective of this study is to define a "vascular competence" profile
 in a population of patients with typical or atypical HUS; both in the acute phase and in
 remission of the disease.
Inclusion Criteria:

 -  Patients with a clinical diagnosis of typical or atypical HUS in acute phase

 Exclusion Criteria:

 -  Patient with positive serological screening test for infection with the HIV.

 -  Patients with a history of cancer.

 -  Patients who have undergone organ transplantation or bone marrow.

 -  Patient with a vivid picture of autoimmune thrombotic thrombocytopenic purpura