Typical and Atypical Hemolytic Uremic Syndrome in the Critically Ill

Carlos L Manrique-Caballero; Sadudee Peerapornratana; Cassandra Formeck; Gaspar Del Rio-Pertuz; Hernando Gomez Danies; John A Kellum

doi:10.1016/j.ccc.2019.11.004

Typical and Atypical Hemolytic Uremic Syndrome in the Critically Ill

Crit Care Clin. 2020 Apr;36(2):333-356. doi: 10.1016/j.ccc.2019.11.004. Epub 2020 Jan 31.

Authors

Carlos L Manrique-Caballero¹, Sadudee Peerapornratana², Cassandra Formeck³, Gaspar Del Rio-Pertuz¹, Hernando Gomez Danies¹, John A Kellum⁴

Affiliations

¹ Center for Critical Care Nephrology, Department of Critical Care Medicine, University of Pittsburgh School of Medicine, 3347 Forbes Avenue Suite 220, Pittsburgh, PA 15213, USA; The CRISMA (Clinical Research, Investigation and Systems Modeling of Acute Illness) Center, Department of Critical Care Medicine, University of Pittsburgh School of Medicine, 3550 Terrace Street, Scaife Hall, Suite 600, Pittsburgh, PA 15213, USA.
² Center for Critical Care Nephrology, Department of Critical Care Medicine, University of Pittsburgh School of Medicine, 3347 Forbes Avenue Suite 220, Pittsburgh, PA 15213, USA; The CRISMA (Clinical Research, Investigation and Systems Modeling of Acute Illness) Center, Department of Critical Care Medicine, University of Pittsburgh School of Medicine, 3550 Terrace Street, Scaife Hall, Suite 600, Pittsburgh, PA 15213, USA; Excellence Center for Critical Care Nephrology, Division of Nephrology, Department of Medicine, Chulalongkorn University, 1873 Rama 4 Road, Pathumwan, Bangkok 10330, Thailand; Department of Laboratory Medicine, Chulalongkorn University, 1873 Rama 4 Road, Pathumwan, Bangkok 10330, Thailand.
³ Center for Critical Care Nephrology, Department of Critical Care Medicine, University of Pittsburgh School of Medicine, 3347 Forbes Avenue Suite 220, Pittsburgh, PA 15213, USA; The CRISMA (Clinical Research, Investigation and Systems Modeling of Acute Illness) Center, Department of Critical Care Medicine, University of Pittsburgh School of Medicine, 3550 Terrace Street, Scaife Hall, Suite 600, Pittsburgh, PA 15213, USA; Department of Nephrology, Children's Hospital of Pittsburgh of UPMC, 4401 Penn Avenue, Floor 3, Pittsburgh, PA 15224, USA.
⁴ Center for Critical Care Nephrology, Department of Critical Care Medicine, University of Pittsburgh School of Medicine, 3347 Forbes Avenue Suite 220, Pittsburgh, PA 15213, USA; The CRISMA (Clinical Research, Investigation and Systems Modeling of Acute Illness) Center, Department of Critical Care Medicine, University of Pittsburgh School of Medicine, 3550 Terrace Street, Scaife Hall, Suite 600, Pittsburgh, PA 15213, USA. Electronic address: kellum@pitt.edu.

PMID: 32172817
DOI: 10.1016/j.ccc.2019.11.004

Abstract

Hemolytic uremic syndrome is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Disseminated intravascular coagulation, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome have a similar clinical presentation. Diagnostic needs to be prompt to decrease mortality, because identifying the different disorders can help to tailor specific, effective therapies. However, diagnosis is challenging and morbidity and mortality remain high, especially in the critically ill population. Development of clinical prediction scores and rapid diagnostic tests for hemolytic uremic syndrome based on mechanistic knowledge are needed to facilitate early diagnosis and assign timely specific treatments to patients with hemolytic uremic syndrome variants.

Keywords: Atypical HUS; Complement-mediated HUS; Critically ill; Microangiopathy.

Publication types

Review

MeSH terms

Algorithms
Atypical Hemolytic Uremic Syndrome / diagnosis
Atypical Hemolytic Uremic Syndrome / etiology
Atypical Hemolytic Uremic Syndrome / genetics
Atypical Hemolytic Uremic Syndrome / therapy
Critical Illness*
Diagnosis, Differential
Early Diagnosis
Hemolytic-Uremic Syndrome / diagnosis*
Hemolytic-Uremic Syndrome / etiology*
Hemolytic-Uremic Syndrome / genetics
Hemolytic-Uremic Syndrome / therapy
Humans
Prognosis
Risk Factors
Shiga Toxin / toxicity

Substances

Shiga Toxin