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Am J Physiol Renal Physiol ; 314(3): F454-F461, 2018 03 01.
Article in English | MEDLINE | ID: mdl-29167171

ABSTRACT

Hemolytic uremic syndrome (HUS) is major global health care issue as it is the leading cause of acute kidney injury in children. It is a triad of acute kidney injury, microangiopathic hemolytic anemia, and thrombocytopenia. In recent years, major advances in our understanding of complement-driven inherited rare forms of HUS have been achieved. However, in children 90% of cases of HUS are associated with a Shiga toxin-producing enteric pathogen. The precise pathological mechanisms in this setting are yet to be elucidated. The purpose of this review is to discuss advances in our understanding of the pathophysiology underlying HUS and identify the key questions yet to be answered by the scientific community.


Subject(s)
Acute Kidney Injury/etiology , Atypical Hemolytic Uremic Syndrome/etiology , Complement Activation , Complement System Proteins/immunology , Escherichia coli Infections/microbiology , Shiga-Toxigenic Escherichia coli/pathogenicity , Thrombotic Microangiopathies/etiology , Acute Kidney Injury/genetics , Acute Kidney Injury/immunology , Acute Kidney Injury/microbiology , Animals , Atypical Hemolytic Uremic Syndrome/genetics , Atypical Hemolytic Uremic Syndrome/immunology , Atypical Hemolytic Uremic Syndrome/microbiology , Complement Activation/genetics , Complement System Proteins/genetics , Genetic Predisposition to Disease , Humans , Phenotype , Prognosis , Risk Factors , Thrombotic Microangiopathies/genetics , Thrombotic Microangiopathies/immunology , Thrombotic Microangiopathies/microbiology
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