Pediatric Hemolytic Uremic Syndrome Follow-up

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

Further Outpatient Care

  • Diarrhea-associated hemolytic-uremic syndrome (D+ HUS)
    • Patients recovering from D+ hemolytic-uremic syndrome should have regular follow-up until their symptoms have resolved and their hemoglobin, platelet counts and renal function have returned to normal.
    • Beyond that, no consensus is noted regarding frequency of follow-up or testing required. Preliminary data suggest many survivors may have persistent, subclinical renal injury, putting them at risk for future development of hypertension, proteinuria, and/or chronic renal disease.
      • All patients should have their blood pressure checked at each medical encounter.
      • The authors suggest annual follow-up with a nephrologist, with consideration of annual urinalysis, urine microalbumin, serum creatinine, and fasting glucose levels on an annual basis.
      • Counsel patients on the importance of a healthy lifestyle, with regular exercise, healthy diet, and avoidance of tobacco and obesity. These measures are beneficial for all patients but especially those at higher risk for future renal disease.
  • Non–diarrhea-associated hemolytic-uremic syndrome (D- HUS)
    • Patients with streptococcal-associated hemolytic-uremic syndrome have a low risk of recurrence and should have follow-up as outlined for D+ hemolytic-uremic syndrome above.
    • Patients with idiopathic or genetically mediated D- hemolytic-uremic syndrome usually have a persistent and relapsing course, and most require frequent and lifelong nephrology follow-up.
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Inpatient & Outpatient Medications

  • Patients with persistent hypertension require antihypertensives.
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Transfer

  • Transfer may be required if the patient requires care or services not available at the patient's facility, such as pediatric specialist consultation, pediatric intensive care, dialysis or plasma exchange.
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Deterrence/Prevention

  • General preventive measures
    • Avoid ingestion of raw or undercooked meat.
    • Avoid unpasteurized milk and cheese.
    • Practice good hand-washing technique, especially during outbreaks of diarrhea.
    • Wash hands well after touching livestock, farm animals or "petting zoo" animals. Supervise children to ensure good technique.
    • Avoid taking antidiarrheal or antimotility agents for diarrhea. Avoid taking antibiotics for diarrhea unless under the management of a physician.
    • Seek medical care immediately for bloody diarrhea.
  • Preventive measures for medical practitioners
    • Avoid antibiotic treatment of patients with possible GI E coli 0157:H7 infection, unless other clinical factors require antibiotic therapy.[22]
    • Use ample parenteral volume expansion with isotonic ("normal") saline in patients with suspected E coli 0157:H7 infection (eg, those with bloody diarrhea). Early recognition is important.
    • A study has shown that early and ample rehydration with isotonic saline is associated with a lower risk of developing oligoanuric renal failure.[2]
      • Many patients who received this therapy still developed hemolytic-uremic syndrome, but they had a less severe course, with shorter lengths of stay and fewer patients requiring dialysis.
      • Ake and colleagues recommend that patients with suspected E coli 0157:H7 infection be admitted for inpatient therapy, using intravenous isotonic saline for both maintenance and replacement fluid requirements, avoiding use of hypotonic fluids.
      • The authors of this article concur with this advice.
      • Trials of oral rehydration, normally an appropriate practice, should be avoided in this situation due to the risk of prolonged renal hypoperfusion.
    • Monitor fluid status, intake and output closely because renal function may change rapidly, requiring adjustments to fluid therapy. Use potassium supplementation with great caution.
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Complications

  • Renal system
    • Renal insufficiency
    • Renal failure
    • Hypertension
  • CNS
    • Mental retardation
    • Seizures
    • Focal motor deficit
    • Optic atrophy
    • Cortical blindness
    • Learning disability
  • Endocrine system
    • Diabetes mellitus
    • Pancreatic exocrine insufficiency
  • GI system - Intestinal necrosis
  • Cardiac system - Congestive heart failure
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Prognosis

  • D+ hemolytic-uremic syndrome
    • Most patients with D+ hemolytic-uremic syndrome who receive the appropriate treatment have a good recovery. Recurrence is very rare. Poor prognostic indicators include the following:
      • Elevated WBC count at diagnosis
      • Prolonged anuria
      • Severe prodromal illness
      • Severe hemorrhagic colitis with rectal prolapse or colonic gangrene
      • Severe multisystemic involvement
      • Persistent proteinuria
    • The long-term prognosis for survivors of childhood D+ hemolytic-uremic syndrome remains unknown. A five-year follow-up of a cohort of patients showed no difference in blood pressure and slightly higher rates of microalbuminuria compared with controls.[24] The patients also had lower glomerular filtration rates (GFRs) as measured by cystatin C but not as measured by serum creatinine levels. Other studies have shown similar findings. Continued long-term follow-up studies are needed to help determine whether survivors have residual subclinical renal injury that could manifest itself later in life. At present, patients should be counseled on avoiding risk factors for renal disease (eg, tobacco use, obesity, hypertension) and the importance of continued medical follow-up.
  • D- hemolytic-uremic syndrome: The prognosis is more guarded than for D+ hemolytic-uremic syndrome. Patients with D- hemolytic-uremic syndrome typically have frequent relapses and a higher risk of progression to end-stage renal disease (ESRD).
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Patient Education

  • Diet
    • Low-salt diet to decrease risk of hypertension
    • Diet high in iron and folic acid content to help recover from anemia
    • High-energy diet to help patient regain lost weight
  • Social worker or psychologist consultation to help the family cope with the illness
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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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