Pediatric Hemolytic Uremic Syndrome Medication

  • Author: Robert S Gillespie, MD, MPH; Chief Editor: Craig B Langman, MD   more...
 
Updated: Sep 26, 2011
 

Medication Summary

Supportive care remains the mainstay of therapy for hemolytic-uremic syndrome (HUS). Medications such as antihypertensives, diuretics, anticonvulsants, and analgesics are indicated to treat specific symptoms or complications of hemolytic-uremic syndrome. These medications are have not clearly demonstrated to alter the disease process.

Eculizumab is the first treatment approved by the US Food and Drug Administration (FDA) in September 2011 for adults and children with atypical hemolytic uremic syndrome (aHUS).

Unfortunately, several agents that in theory should ameliorate hemolytic-uremic syndrome have failed to do so in clinical trials. These include thrombolytic agents (eg, heparin, urokinase), platelet inhibitors (eg, aspirin, dipyridamole), and a Shiga toxin (Stx)–binding agent (ie, Synsorb-Pk). Current evidence does not support use of these medications.

Corticosteroids are not useful in diarrhea-associated hemolytic-uremic syndrome (D+ HUS). They may be of value in non–diarrhea-associated hemolytic-uremic syndrome (D- HUS) if the patient has an autoimmune-produced inhibitor of ADAMTS13. Clinical testing for inhibitors is available but has a long turnaround time. Corticosteroid therapy may be initiated presumptively in patients with unexplained D- hemolytic-uremic syndrome.

Limited case reports describe using intravenous immune globulin (IVIG) in patients with D- hemolytic-uremic syndrome associated with organ transplantation. IVIG does not have a role in hereditary D- hemolytic-uremic syndrome or in D+ hemolytic-uremic syndrome.

Plasma therapies are covered in Treatment. They are indicated only for treatment of D- hemolytic-uremic syndrome, or possibly D+ hemolytic-uremic syndrome with associated CNS involvement.

Studies have shown that antibiotics given to patients with diarrhea due to E coli 0157:H7 increase the risk of developing hemolytic-uremic syndrome.[22] A theory proposed to explain this finding is that antibiotic therapy causes rapid large-scale bacterial lysis with massive release of Stx, overwhelming host defense mechanisms. Whether antibiotics affect the course of established hemolytic-uremic syndrome remains unknown. Patients with E coli 0157 colitis usually clear the infection spontaneously.

Most pediatric nephrologists do not routinely use antibiotics in patients with D+ hemolytic-uremic syndrome, based on a theoretical concern it could exacerbate the disease process.[23] However, antibiotics should be used when indicated according to clinical judgment. Examples include patients having suspected or documented bacteremia, urinary tract infection, or sepsis.

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Monoclonal Antibodies, Endocrine

Class Summary

Eculizumab is a monoclonal antibody indicated for atypical hemolytic uremic syndrome to inhibit complement-mediated thrombotic microangiopathy; effectiveness is based on the effects on thrombotic microangiopathy and renal function. It is not indicated for patients with Shiga toxin Escherichia coli – related hemolytic uremic syndrome (STEC-HUS).

View full drug information

Eculizumab (Soliris)

 

Monoclonal blocking antibody to complement protein C5; inhibits cleavage to C5a and C5b, thus preventing terminal complement complex C5b-9, thereby preventing RBC hemolysis. Inhibits terminal complement mediated intravascular hemolysis in PNH patients and complement-mediated thrombotic microangiopathy (TMA) in patients with aHUS.

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Contributor Information and Disclosures
Author

Robert S Gillespie, MD, MPH  Department of Pediatrics, Cook Children's Medical Center

Robert S Gillespie, MD, MPH is a member of the following medical societies: American Society of Nephrology, American Society of Pediatric Nephrology, and Texas Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Craig S Wong, MD, MPH  Assistant Professor, Division of Pediatric Nephrology, Department of Pediatrics, University of New Mexico School of Medicine; Director of Pediatric Kidney Transplantation, Division of Pediatric Nephrology, Department of Pediatrics, University of New Mexico Transplant Services, Children's Hospital of New Mexico

Craig S Wong, MD, MPH is a member of the following medical societies: American Society of Nephrology and American Society of Pediatric Nephrology

Disclosure: Nothing to disclose.

Ronald D Prauner  MD, Assistant Professor of Pediatrics, F Edward Herbert School of Medicine, Uniformed Services of the Health Sciences; Assistant Deputy Commander for Medicine; Fort Belvoir Community Hospital, Fort Belvoir, VA; Consultant to the Army Surgeon General for Pediatric Subspecialties; Staff Pediatric Hematologist-Oncologist, Fort Belvoir Community Hospital

Ronald D Prauner is a member of the following medical societies: American Academy of Pediatrics, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, and Christian Medical & Dental Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard Neiberger, MD, PhD  Director of Pediatric Renal Stone Disease Clinic, Associate Professor, Department of Pediatrics, Division of Nephrology, University of Florida College of Medicine and Shands Hospital

Richard Neiberger, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Federation for Medical Research, American Medical Association, American Society of Nephrology, American Society of Pediatric Nephrology, Christian Medical & Dental Society, Florida Medical Association, International Society for Peritoneal Dialysis, International Society of Nephrology, National Kidney Foundation, New York Academy of Sciences, Shock Society, Sigma Xi, Southern Medical Association, Southern Society for Pediatric Research, and Southwest Pediatric Nephrology Study Group

Disclosure: The Osler Institute Honoraria Speaking and teaching

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Luther Travis, MD  Professor Emeritus, Departments of Pediatrics, Nephrology and Diabetes, University of Texas Medical Branch School of Medicine

Luther Travis, MD is a member of the following medical societies: Alpha Omega Alpha, American Federation for Medical Research, International Society of Nephrology, and Texas Pediatric Society

Disclosure: Nothing to disclose.

Howard Trachtman, MD  Program Director, Pediatrics Research, Schneider Children's Hospital, Department of Pediatrics, Division of Nephrology, Professor, Albert Einstein College of Medicine

Howard Trachtman, MD is a member of the following medical societies: American Society of Hypertension, American Society of Nephrology, American Society of Pediatric Nephrology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Craig B Langman, MD  The Isaac A Abt, MD, Professor of Kidney Diseases, Northwestern University, The Feinberg School of Medicine; Division Head of Kidney Diseases, Children's Memorial Hospital

Craig B Langman, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, and International Society of Nephrology

Disclosure: Merck Grant/research funds None; NIH Grant/research funds None; Raptor Pharmaceuticals, Inc Grant/research funds None; Alexion Pharmaceuticals, Inc. Grant/research funds None

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author Tamara Biega, MD, to the original writing and development of this article.

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Peripheral blood smear in hemolytic-uremic syndrome (HUS) showing many schistocytes and RBC fragments due to hemolysis, and relatively few platelets reflective of thrombocytopenia.
 
 
 
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