The Experts below are selected from a list of 258 Experts worldwide ranked by ideXlab platform

Giuseppe Remuzzi - One of the best experts on this subject based on the ideXlab platform.

  • Hemolytic Uremic Syndrome in Pregnancy and Postpartum
    Clinical journal of the American Society of Nephrology : CJASN, 2017
    Co-Authors: Alexandra Bruel, David J. Kavanagh, Marina Noris, Yahsou Delmas, Edwin K.s. Wong, Elena Bresin, François Provôt, Vicky Brocklebank, Caterina Mele, Giuseppe Remuzzi
    Abstract:

    Background Pregnancy is associated with various forms of thrombotic microangiopathy, including Hemolytic Uremic Syndrome. A previous small French study suggested that pregnancy-associated Hemolytic Uremic Syndrome was to be included in the spectrum of atypical Hemolytic Uremic Syndrome linked to complement alternative pathway dysregulation. Design, setting, participants, & measurements We sought to retrospectively analyze the presentation, outcome, and frequency of complement alternative pathway gene variants in a larger international (France, United Kingdom, Italy) cohort of patients with pregnancy-associated Hemolytic Uremic Syndrome. Results Eighty-seven patients with pregnancy-associated Hemolytic Uremic Syndrome were included. Hemolytic Uremic Syndrome occurred mainly during the first pregnancy (58%) and in the postpartum period (76%). At diagnosis, 56 (71%) patients required dialysis. Fifty-six (78%) patients underwent plasma exchanges, 21 (41%) received plasma infusions, and four (5%) received eculizumab. During follow-up (mean duration of 7.2 years), 41 (53%) patients reached ESRD, 15 (19%) had CKD, and 18 (28%) patients experienced Hemolytic Uremic Syndrome relapse. Twenty-four patients (27%) received a kidney transplant and a recurrence of Hemolytic Uremic Syndrome occurred in 13 (54%) patients. Variants in complement genes were detected in 49 (56%) patients, mainly in the CFH (30%) and CFI genes (9%). Conclusions Pregnancy-associated Hemolytic Uremic Syndrome and atypical Hemolytic Uremic Syndrome nonrelated to pregnancy have the same severity at onset and during follow-up and the same frequency of complement gene variants.

  • atypical Hemolytic Uremic Syndrome
    The New England Journal of Medicine, 2009
    Co-Authors: Marina Noris, Giuseppe Remuzzi
    Abstract:

    The Hemolytic–Uremic Syndrome, which is characterized by nonimmune Hemolytic anemia, thrombocytopenia, and renal impairment, occurs most frequently in young children. Most cases are secondary to infection with Escherichia coli O157:H7 and other Shiga-toxin–producing strains. However, approximately 10% of cases are atypical and not associated with infection. This article reviews current concepts about the pathobiology of atypical Hemolytic–Uremic Syndrome and its diagnosis and management.

  • Atypical Hemolytic–Uremic Syndrome
    The New England journal of medicine, 2009
    Co-Authors: Marina Noris, Giuseppe Remuzzi
    Abstract:

    The Hemolytic–Uremic Syndrome, which is characterized by nonimmune Hemolytic anemia, thrombocytopenia, and renal impairment, occurs most frequently in young children. Most cases are secondary to infection with Escherichia coli O157:H7 and other Shiga-toxin–producing strains. However, approximately 10% of cases are atypical and not associated with infection. This article reviews current concepts about the pathobiology of atypical Hemolytic–Uremic Syndrome and its diagnosis and management.

Marie-noëlle Peraldi - One of the best experts on this subject based on the ideXlab platform.

  • ESCHERICHIA COLI AND THE Hemolytic-Uremic Syndrome
    The New England journal of medicine, 1996
    Co-Authors: Eric Rondeau, Marie-noëlle Peraldi
    Abstract:

    The Hemolytic–Uremic Syndrome is characterized by nonimmune Hemolytic anemia, thrombocytopenia, and acute renal failure.1 Extrarenal manifestations, particularly neurologic signs, may be present, indicating that the disease process is not limited to the kidney. As in the closely related disorder of thrombotic thrombocytopenic purpura, the pathologic hallmark of the disease is thrombotic microangiopathy. The Hemolytic–Uremic Syndrome is the leading cause of acute renal failure in young children, but it occurs in adults as well, sometimes together with gastrointestinal infection and bloody diarrhea. Other forms of the Syndrome in adults are related to pregnancy, the postpartum state, oral-contraceptive use, malignant hypertension, cancer . . .

  • ESCHERICHIA COLI AND THE Hemolytic-Uremic Syndrome
    The New England journal of medicine, 1996
    Co-Authors: Eric Rondeau, Marie-noëlle Peraldi
    Abstract:

    The Hemolytic–Uremic Syndrome is characterized by nonimmune Hemolytic anemia, thrombocytopenia, and acute renal failure.1 Extrarenal manifestations, particularly neurologic signs, may be present, i...

Diana Karpman - One of the best experts on this subject based on the ideXlab platform.

  • Pathogenesis of Shiga Toxin-Associated Hemolytic Uremic Syndrome
    Pediatric Research, 2001
    Co-Authors: François Proulx, Ernest G Seidman, Diana Karpman
    Abstract:

    The aim of this review is to examine recent advances in experimental and clinical research relevant to the pathogenesis of diarrhea-associated Hemolytic Uremic Syndrome with special reference to histopathologic findings, virulence factors of Shiga toxin-producing Escherichia coli , the host response, and the prothrombotic state. Despite significant advances during the past decade, the exact mechanism by which Shiga toxin-producing E. coli leads to Hemolytic Uremic Syndrome remains unclear. Factors such as Shiga toxin, lipopolysaccharide, the adhesins intimin and E. coli -secreted proteins A, B, and D, the 60-MD plasmid, and enterohemolysin likely contribute to the pathogenesis. Data on the inflammatory response of the host, including leukocytes and inflammatory mediators, are updated. The pathogenesis of the prothrombotic state leading to thrombocytopenia secondary to endothelial cell damage and platelet activation is also discussed. A hypothetical sequence of events from ingestion of the bacteria to the development of full-blown Hemolytic Uremic Syndrome is proposed.

Marina Noris - One of the best experts on this subject based on the ideXlab platform.

  • Hemolytic Uremic Syndrome in Pregnancy and Postpartum
    Clinical journal of the American Society of Nephrology : CJASN, 2017
    Co-Authors: Alexandra Bruel, David J. Kavanagh, Marina Noris, Yahsou Delmas, Edwin K.s. Wong, Elena Bresin, François Provôt, Vicky Brocklebank, Caterina Mele, Giuseppe Remuzzi
    Abstract:

    Background Pregnancy is associated with various forms of thrombotic microangiopathy, including Hemolytic Uremic Syndrome. A previous small French study suggested that pregnancy-associated Hemolytic Uremic Syndrome was to be included in the spectrum of atypical Hemolytic Uremic Syndrome linked to complement alternative pathway dysregulation. Design, setting, participants, & measurements We sought to retrospectively analyze the presentation, outcome, and frequency of complement alternative pathway gene variants in a larger international (France, United Kingdom, Italy) cohort of patients with pregnancy-associated Hemolytic Uremic Syndrome. Results Eighty-seven patients with pregnancy-associated Hemolytic Uremic Syndrome were included. Hemolytic Uremic Syndrome occurred mainly during the first pregnancy (58%) and in the postpartum period (76%). At diagnosis, 56 (71%) patients required dialysis. Fifty-six (78%) patients underwent plasma exchanges, 21 (41%) received plasma infusions, and four (5%) received eculizumab. During follow-up (mean duration of 7.2 years), 41 (53%) patients reached ESRD, 15 (19%) had CKD, and 18 (28%) patients experienced Hemolytic Uremic Syndrome relapse. Twenty-four patients (27%) received a kidney transplant and a recurrence of Hemolytic Uremic Syndrome occurred in 13 (54%) patients. Variants in complement genes were detected in 49 (56%) patients, mainly in the CFH (30%) and CFI genes (9%). Conclusions Pregnancy-associated Hemolytic Uremic Syndrome and atypical Hemolytic Uremic Syndrome nonrelated to pregnancy have the same severity at onset and during follow-up and the same frequency of complement gene variants.

  • atypical Hemolytic Uremic Syndrome
    The New England Journal of Medicine, 2009
    Co-Authors: Marina Noris, Giuseppe Remuzzi
    Abstract:

    The Hemolytic–Uremic Syndrome, which is characterized by nonimmune Hemolytic anemia, thrombocytopenia, and renal impairment, occurs most frequently in young children. Most cases are secondary to infection with Escherichia coli O157:H7 and other Shiga-toxin–producing strains. However, approximately 10% of cases are atypical and not associated with infection. This article reviews current concepts about the pathobiology of atypical Hemolytic–Uremic Syndrome and its diagnosis and management.

  • Atypical Hemolytic–Uremic Syndrome
    The New England journal of medicine, 2009
    Co-Authors: Marina Noris, Giuseppe Remuzzi
    Abstract:

    The Hemolytic–Uremic Syndrome, which is characterized by nonimmune Hemolytic anemia, thrombocytopenia, and renal impairment, occurs most frequently in young children. Most cases are secondary to infection with Escherichia coli O157:H7 and other Shiga-toxin–producing strains. However, approximately 10% of cases are atypical and not associated with infection. This article reviews current concepts about the pathobiology of atypical Hemolytic–Uremic Syndrome and its diagnosis and management.

  • Thrombomodulin mutations in atypical Hemolytic-Uremic Syndrome.
    The New England journal of medicine, 2009
    Co-Authors: Mieke Delvaeye, Marina Noris, Astrid De Vriese, Charles T. Esmon, Naomi L. Esmon, Gary L. Ferrell, Jurgen Del-favero, Stephane Plaisance, Bart Claes, Diether Lambrechts
    Abstract:

    BACKGROUND The Hemolytic-Uremic Syndrome consists of the triad of microangiopathic Hemolytic anemia, thrombocytopenia, and renal failure. The common form of the Syndrome is triggered by infection with Shiga toxin-producing bacteria and has a favorable outcome. The less common form of the Syndrome, called atypical Hemolytic-Uremic Syndrome, accounts for about 10% of cases, and patients with this form of the Syndrome have a poor prognosis. Approximately half of the patients with atypical Hemolytic-Uremic Syndrome have mutations in genes that regulate the complement system. Genetic factors in the remaining cases are unknown. We studied the role of thrombomodulin, an endothelial glycoprotein with anticoagulant, antiinflammatory, and cytoprotective properties, in atypical Hemolytic-Uremic Syndrome. METHODS We sequenced the entire thrombomodulin gene (THBD) in 152 patients with atypical Hemolytic-Uremic Syndrome and in 380 controls. Using purified proteins and cell-expression systems, we investigated whether thrombomodulin regulates the complement system, and we characterized the mechanisms. We evaluated the effects of thrombomodulin missense mutations associated with atypical Hemolytic-Uremic Syndrome on complement activation by expressing thrombomodulin variants in cultured cells. RESULTS Of 152 patients with atypical Hemolytic-Uremic Syndrome, 7 unrelated patients had six different heterozygous missense THBD mutations. In vitro, thrombomodulin binds to C3b and factor H (CFH) and negatively regulates complement by accelerating factor I-mediated inactivation of C3b in the presence of cofactors, CFH or C4b binding protein. By promoting activation of the plasma procarboxypeptidase B, thrombomodulin also accelerates the inactivation of anaphylatoxins C3a and C5a. Cultured cells expressing thrombomodulin variants associated with atypical Hemolytic-Uremic Syndrome had diminished capacity to inactivate C3b and to activate procarboxypeptidase B and were thus less protected from activated complement. CONCLUSIONS Mutations that impair the function of thrombomodulin occur in about 5% of patients with atypical Hemolytic-Uremic Syndrome.

Donald J. Weaver - One of the best experts on this subject based on the ideXlab platform.

  • Hemolytic Uremic Syndrome with simultaneous Shiga toxin producing Escherichia coli and complement abnormalities.
    BMC pediatrics, 2014
    Co-Authors: Nicole Mccoy, Donald J. Weaver
    Abstract:

    Hemolytic Uremic Syndrome is a common cause of acute kidney injury in children. In children, Hemolytic Uremic Syndrome is most commonly associated with gasterointestinal infections caused by Shiga toxin-producing Escherichia coli or other enteric organisms. Although less common, atypical Hemolytic Uremic Syndrome is triggered by multiple factors and portends a significantly worse prognosis with a high rate of recurrence. Here we discuss the case of a 10 year old Caucasian male presenting with thrombocytopenia, anemia, and acute kidney injury. This case highlights the clinical challenges in diagnosing and managing patients with Hemolytic Uremic Syndrome. Because of similarity in symptoms, differentiating Shiga toxin-producing Escherichia coli associated Hemolytic Uremic Syndrome and atypical Hemolytic Uremic Syndrome can be challenging. However, because of the increased morbidity and mortality of atypical Hemolytic Uremic Syndrome, early detection and initiation of therapy are critical. Providers must have a heightened suspicion in order to initiate supportive care or disease directed therapy in the case of atypical Hemolytic Uremic Syndrome.