Display options
Share it on

Indian J Crit Care Med. 2017 Feb;21(2):102-104. doi: 10.4103/ijccm.IJCCM_341_16.

Complements Spurned: Our Experience with Atypical Hemolytic Uremic Syndrome.

Indian journal of critical care medicine : peer-reviewed, official publication of Indian Society of Critical Care Medicine

Vidya S Nagar, Rudrarpan Chaterjee, Ankita Sood, Basavaraj Sajjan, Aniruddha Kaushik, Sameer V Vyahalkar

Affiliations

  1. Department of General Medicine, Grant Medical College and Sir JJ Group of Hospital, Mumbai, Maharashtra, India.
  2. Department of Nephrology, Grant Medical College and Sir JJ Group of Hospital, Mumbai, Maharashtra, India.

PMID: 28250608 PMCID: PMC5330049 DOI: 10.4103/ijccm.IJCCM_341_16

Abstract

Atypical hemolytic uremic syndrome (aHUS) is a rare disorder resulting from a dysregulated activation of the alternative pathway of the complement system. It results in significant morbidity and mortality if not diagnosed and treated promptly. It lends itself to myriad renal and extrarenal manifestations, all potentially disabling. Eculizumab, a monoclonal antibody to complement C5 is now the widely accepted norm for treatment. However, in resource-limited settings, plasma exchange if instituted early may be as beneficial. We report a case of aHUS treated with extended plasma exchange with excellent results. Critical care monitoring is essential for the management of the disease in view of a tendency to develop multiple complications. Long-term immunosuppression may be successful in maintaining remission.

Keywords: Atypical hemolytic uremic syndrome; complement system; eculizumab; plasma exchange; thrombotic microangiopathy

Conflict of interest statement

There are no conflicts of interest.

References

  1. Mol Immunol. 2013 Dec 15;56(3):199-212 - PubMed
  2. Semin Nephrol. 2013 Nov;33(6):508-30 - PubMed
  3. Pediatr Nephrol. 2010 Dec;25(12):2431-42 - PubMed
  4. N Engl J Med. 2009 Oct 22;361(17):1676-87 - PubMed
  5. Clin J Am Soc Nephrol. 2010 Oct;5(10):1844-59 - PubMed
  6. Orphanet J Rare Dis. 2011 Sep 08;6:60 - PubMed
  7. Br J Haematol. 2010 Jan;148(1):37-47 - PubMed
  8. Clin J Am Soc Nephrol. 2013 Mar;8(3):407-15 - PubMed
  9. Pediatr Nephrol. 2013 May;28(5):827-30 - PubMed
  10. Clin J Am Soc Nephrol. 2013 Apr;8(4):554-62 - PubMed

Publication Types