Advances in our understanding of the pathogenesis of hemolytic uremic syndromes

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 in...

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Bibliographic Details
Published in:American journal of physiology. Renal physiology 2018-03, Vol.314 (3), p.F454-F461
Main Authors: Bowen, E E, Coward, R J
Format: Article
Language:English
Subjects:
Acute Kidney Injury - etiology
Acute Kidney Injury - genetics
Acute Kidney Injury - immunology
Acute Kidney Injury - microbiology
Anemia
Animals
Atypical Hemolytic Uremic Syndrome - etiology
Atypical Hemolytic Uremic Syndrome - genetics
Atypical Hemolytic Uremic Syndrome - immunology
Atypical Hemolytic Uremic Syndrome - microbiology
Blood diseases
Children
Children & youth
Complement Activation - genetics
Complement System Proteins - genetics
Complement System Proteins - immunology
Escherichia coli Infections - microbiology
Genetic Predisposition to Disease
Hemolytic anemia
Hemolytic uremic syndrome
Humans
Kidney diseases
Kidneys
Pathogenesis
Phenotype
Prognosis
Review
Risk Factors
Shiga toxin
Shiga-Toxigenic Escherichia coli - pathogenicity
Thrombocytopenia
Thrombotic Microangiopathies - etiology
Thrombotic Microangiopathies - genetics
Thrombotic Microangiopathies - immunology
Thrombotic Microangiopathies - microbiology
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Summary: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.
ISSN:1931-857X
1522-1466
DOI:10.1152/ajprenal.00376.2017