Chapter 26
Hemolytic Uremic Syndrome
Asaf Lebel,
Amrit Kirpalani,
Christoph Licht,
Asaf Lebel
Division of Nephrology and Cell Biology Program, Research Institute, The Hospital for Sick Children, Toronto, ON, Canada
Search for more papers by this authorAmrit Kirpalani
Division of Nephrology and Cell Biology Program, Research Institute, The Hospital for Sick Children, Toronto, ON, Canada
Search for more papers by this authorChristoph Licht
Division of Nephrology and Cell Biology Program, Research Institute, The Hospital for Sick Children, Toronto, ON, Canada
Department of Paediatrics, University of Toronto, Toronto, ON, Canada
Search for more papers by this authorAsaf Lebel,
Amrit Kirpalani,
Christoph Licht,
Asaf Lebel
Division of Nephrology and Cell Biology Program, Research Institute, The Hospital for Sick Children, Toronto, ON, Canada
Search for more papers by this authorAmrit Kirpalani
Division of Nephrology and Cell Biology Program, Research Institute, The Hospital for Sick Children, Toronto, ON, Canada
Search for more papers by this authorChristoph Licht
Division of Nephrology and Cell Biology Program, Research Institute, The Hospital for Sick Children, Toronto, ON, Canada
Department of Paediatrics, University of Toronto, Toronto, ON, Canada
Search for more papers by this authorBook Editor(s):Jonathan C. Craig MBChB, DipCH, MMed(Clin Epi), PhD, FAHMS,
Donald A. Molony MD,
Giovanni F.M. Strippoli MD, PhD, MPH, MM (Epi),
Jonathan C. Craig MBChB, DipCH, MMed(Clin Epi), PhD, FAHMS
Matthew Flinders Distinguished Professor Vice President and Executive Dean
College of Medicine and Public Health, Flinders University, Adelaide, Australia
Search for more papers by this authorDonald A. Molony MD
Professor of Medicine Distinguished Teaching Professor of the University of Texas System
Division of Renal Diseases and Hypertension AND Center for Clinical Research and Evidence-based Medicine, McGovern Medical School University of Texas, Houston, TX, USA
Search for more papers by this authorGiovanni F.M. Strippoli MD, PhD, MPH, MM (Epi)
Professor of Nephrology Adjunct Professor of Epidemiology
Department of Emergency and Organ Transplantation – University of Bari, Bari, Italy
School of Public Health, University of Sydney, Sydney, NSW, Australia
Search for more papers by this authorFirst published: 18 November 2022
Summary
This chapter focuses on the epidemiology, pathophysiology, clinical manifestations, diagnosis, prognosis, and treatment of two entities of the Thrombotic microangiopathy (TMA) spectrum: Shiga toxin-producing Escherichia coli-associated hemolytic uremic syndrome (STEC-HUS) and complement-mediated TMA. STEC-HUS is more frequent in children than in adults, with median age around two years, although adults have more severe disease with higher mortality. The common pathologic basis of all TMA is injury to the vascular endothelium with subsequent microvascular thrombosis, which leads to consumptive thrombocytopenia, microangiopathichemolytic anemia, and kidney and other organs injury. The clinical symptoms of TMA reflect anemia, thrombocytopenia, and renal or other organ involvement, and include pallor, fatigue, shortness of breath, oliguria, and edema. HUS is an entity under the umbrella of TMA. The most common cause, STEC-HUS, has long been considered as independent of the rest of this spectrum of diseases as it has a defined trigger and known secondary cause of microangiopathy.
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