Hemolytic-Uremic Syndrome Follow-up

  • Author: Malvinder S Parmar, MB, MS, FRCP(C), FACP, FASN; Chief Editor: Emmanuel C Besa, MD   more...
 
Updated: Aug 26, 2010
 

Further Inpatient Care

Provide nutritional support during the acute illness in patients with hemolytic-uremic syndrome (HUS). Closely monitor electrolyte levels, renal function, and platelet counts.

Next

Further Outpatient Care

Monitor renal function and blood pressure, because as many as 80% of adults with hemolytic-uremic syndrome (HUS) require long-term dialysis or renal transplantation.

Ensure adequate blood pressure control and consider renin-angiotensin blockade with angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin-receptor blockers.

Early protein restriction may be needed in patients who develop residual chronic kidney disease after the acute phase.

Previous
Next

Transfer

The patient may need to be transferred to a tertiary care facility for specialized treatment (eg, plasma exchange, dialysis, ICU monitoring).

Previous
Next

Deterrence/Prevention

Because typical hemolytic-uremic syndrome (HUS) commonly occurs in epidemics, consider this possibility and inform health authorities to monitor for the possibility of index cases and to prevent the spread of disease in the community.

At present, prevention is the main approach to decreasing the morbidity and mortality associated with Stx-E coli infection.

Antibiotic treatment of children with E coli O157:H7 infection increases the risk of hemolytic-uremic syndrome (HUS) and should be avoided unless they have septicemia.[14]

Previous
Next

Complications

Complications may include the following:

  • Renal failure
  • Stroke
  • Coma
  • Seizures
  • Bleeding complications
Previous
Next

Prognosis

Stx-HUS prognosis is as follows:

  • Acute renal failure occurs in 55-70% of patients, but 85% recover renal function with supportive therapy.
  • Approximately 15-20% of children may develop hypertension 3-5 years after the onset of disease.
  • Recurrence with renal allografting is 10% or lower.

Non – Stx-HUS prognosis is as follows:

  • Patients collectively have a poor prognosis, and as many as 50-60% progress to ESRD (50% in those with the sporadic forms and 60% in those with the familial forms) or develop irreversible brain damage. About 25% die during the acute phase.
  • The recurrence rate in patients receiving renal transplants is as high as 50%, with graft loss occurring in more than 90% who have recurrence. Recurrence rates are higher in patients with HF1 mutation.

Factors predictive of poor prognosis are as follows:

  • Non – Stx-HUS
  • Prolonged oliguria or anuria
  • Severe hypertension (especially delayed onset of hypertension)
  • Involvement of medium-sized arteries
  • Severity of central nervous system (CNS) symptoms
  • Persistent consumption of clotting factors
  • Extensive glomerular involvement (>80%)
  • Age older than 5 years
Previous
Next

Patient Education

Advise patients to avoid eating raw or partially cooked meat. Educate patients on the proper treatment of drinking water. Educate patients about proper hygienic measures, especially in cattle fields and farms.

Previous
 
Contributor Information and Disclosures
Author

Malvinder S Parmar, MB, MS, FRCP(C), FACP, FASN  Assistant Professor (VPT), Faculty of Medicine, University of Ottawa; Associate Professor, Department of Internal Medicine, Northern Ontario School of Medicine; Consulting Physician, Timmins and District Hospital, Ontario, Canada

Malvinder S Parmar, MB, MS, FRCP(C), FACP, FASN is a member of the following medical societies: American College of Physicians, American Society of Nephrology, Canadian Medical Association, Ontario Medical Association, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Specialty Editor Board

Rodger L Bick, MD, PhD, FACP  Clinical Professor of Medicine, University of Texas Southwestern Medical Center; Director, Dallas and Pacific Thrombosis Hemostasis and Vascular Medicine Clinical Center

Rodger L Bick, MD, PhD, FACP is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Association of Blood Banks, American Cancer Society, American College of Angiology, American College of Physicians, American Geriatrics Society, American Heart Association, American Medical Association, American Society for Clinical Pathology, American Society of Hematology, Association of Clinical Scientists, California Medical Association, California Thoracic Society, International College of Angiology, International Society of Hematology, International Society on Thrombosis and Haemostasis, New York Academy of Sciences, and Southwest Oncology Group

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Ronald A Sacher, MB, BCh, MD, FRCPC  Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

Ronald A Sacher, MB, BCh, MD, FRCPC is a member of the following medical societies: American Association for the Advancement of Science, American Association of Blood Banks, American Clinical and Climatological Association, American Society for Clinical Pathology, American Society of Hematology, College of American Pathologists, International Society of Blood Transfusion, International Society on Thrombosis and Haemostasis, and Royal College of Physicians and Surgeons of Canada

Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Talecris Honoraria Board membership

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD  Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Clinical Oncology, American Society of Hematology, and New York Academy of Sciences

Disclosure: Nothing to disclose.

References

  1. Siegler RL. Management of hemolytic-uremic syndrome. J Pediatr. Jun 1988;112(6):1014-20. [Medline].

  2. Siegler R, Oakes R. Hemolytic uremic syndrome; pathogenesis, treatment, and outcome. Curr Opin Pediatr. Apr 2005;17(2):200-4. [Medline].

  3. Furlan M, Robles R, Galbusera M, et al. von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med. Nov 26 1998;339(22):1578-84. [Medline]. [Full Text].

  4. Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med. Nov 26 1998;339(22):1585-94. [Medline]. [Full Text].

  5. George JN. ADAMTS13, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome. Curr Hematol Rep. May 2005;4(3):167-9. [Medline].

  6. Haspel RL, Jarolím P. The "cutting" edge: von Willebrand factor-cleaving protease activity in thrombotic microangiopathies. Transfus Apher Sci. Apr 2005;32(2):177-84. [Medline].

  7. Blackall DP, Marques MB. Hemolytic uremic syndrome revisited: Shiga toxin, factor H, and fibrin generation. Am J Clin Pathol. Jun 2004;121 suppl:S81-8. [Medline].

  8. Tarr PI, Gordon CA, Chandler WL. Shiga-toxin-producing Escherichia coli and haemolytic uraemic syndrome. Lancet. Mar 19-25 2005;365(9464):1073-86. [Medline].

  9. Caprioli J, Bettinaglio P, Zipfel PF, et al. The molecular basis of familial hemolytic uremic syndrome: mutation analysis of factor H gene reveals a hot spot in short consensus repeat 20. J Am Soc Nephrol. Feb 2001;12(2):297-307. [Medline]. [Full Text].

  10. Fremeaux-Bacchi V, Kemp EJ, Goodship JA, Dragon-Durey MA, Strain L, Loirat C, et al. The development of atypical haemolytic-uraemic syndrome is influenced by susceptibility factors in factor H and membrane cofactor protein: evidence from two independent cohorts. J Med Genet. Nov 2005;42(11):852-6. [Medline]. [Full Text].

  11. Edey MM, Mead PA, Saunders RE, et al. Association of a factor H mutation with hemolytic uremic syndrome following a diarrheal illness. Am J Kidney Dis. Mar 2008;51(3):487-90. [Medline].

  12. Lapeyraque AL, Wagner E, Phan V, et al. Efficacy of plasma therapy in atypical hemolytic uremic syndrome with complement factor H mutations. Pediatr Nephrol. Aug 2008;23(8):1363-6. [Medline].

  13. McGannon CM, Fuller CA, Weiss AA. Different classes of antibiotics differentially influence Shiga toxin production. Antimicrob Agents Chemother. Sept 2010;54(9):3790-3798.

  14. Wong CS, Jelacic S, Habeeb RL, Watkins SL, Tarr PI. The risk of the hemolytic-uremic syndrome after antibiotic treatment of Escherichia coli O157:H7 infections. N Engl J Med. Jun 29 2000;342(26):1930-6. [Medline]. [Full Text].

  15. Cattran DC. Adult hemolytic-uremic syndrome: successful treatment with plasmapheresis. Am J Kidney Dis. Jan 1984;3(4):275-9. [Medline].

  16. Gordon LI, Kwaan HC, Rossi EC. Deleterious effects of platelet transfusions and recovery thrombocytosis in patients with thrombotic microangiopathy. Semin Hematol. Jul 1987;24(3):194-201. [Medline].

  17. Hakim RM, Schulman G, Churchill WH Jr, Lazarus JM. Successful management of thrombocytopenia, microangiopathic anemia, and acute renal failure by plasmapheresis. Am J Kidney Dis. Mar 1985;5(3):170-6. [Medline].

  18. Harkness DR, Byrnes JJ, Lian EC, Williams WD, Hensley GT. Hazard of platelet transfusion in thrombotic thrombocytopenic purpura. JAMA. Oct 23-30 1981;246(17):1931-3. [Medline].

  19. Kwaan HC. Miscellaneous secondary thrombotic microangiopathy. Semin Hematol. Jul 1987;24(3):141-7. [Medline].

  20. Loirat C. Treatment of childhood hemolytic-uremic syndrome with immunoglobulins [abstract]. Pediatr Nephrol. 1991;5:C39.

  21. Loirat C, Baudouin V, Sonsino E, Mariani-Kurdjian P, Elion J. Hemolytic-uremic syndrome in the child. Adv Nephrol Necker Hosp. 1993;22:141-68. [Medline].

  22. Misiani R, Appiani AC, Edefonti A, Gotti E, Bettinelli A, Giani M, et al. Haemolytic uraemic syndrome: therapeutic effect of plasma infusion. Br Med J (Clin Res Ed). Nov 6 1982;285(6351):1304-6. [Medline]. [Full Text].

  23. Morel-Maroger L, Kanfer A, Solez K, Sraer JD, Richet G. Prognostic importance of vascular lesions in acute renal failure with microangiopathic hemolytic anemia (hemolytic-uremic syndrome): clinicopathologic study in 20 adults. Kidney Int. May 1979;15(5):548-58. [Medline].

  24. Ponticelli C, Rivolta E, Imbasciati E, Rossi E, Mannucci PM. Hemolytic uremic syndrome in adults. Arch Intern Med. Mar 1980;140(3):353-7. [Medline].

  25. Ruggenenti P, Noris M, Remuzzi G. Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura. Kidney Int. Sep 2001;60(3):831-46. [Medline]. [Full Text].

 
Previous
Next
 
Photomicrograph (hematoxylin and eosin, original magnification ×25) shows diffuse thickening of the glomerular capillary wall with double contouring (arrow) and swelling of endothelial cells. Fibrin thrombi and packed red blood cells are visible in the lumina (arrowhead). Courtesy of Madeleine Moussa, MD, FRCPC, Department of Pathology, London Health Sciences Centre, London, Ontario, Canada.
Photomicrograph (periodic acid-Schiff, original magnification ×40) shows diffuse thickening of the glomerular capillary wall with double contouring (arrow) and swelling of endothelial cells. Courtesy of Madeleine Moussa, MD, FRCPC, Department of Pathology, London Health Sciences Centre, London, Ontario, Canada.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.