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Extra-renal manifestations of atypical hemolytic uremic syndrome

Abstract

Atypical hemolytic uremic syndrome (aHUS) is a rare and complex disease resulting from abnormal alternative complement activation with a wide range of clinical presentations. Extra-renal manifestations of aHUS can involve many organ systems, including the peripheral and central nervous, gastrointestinal, cardiovascular, integumentary, pulmonary, as well as the eye. While some of these extra-renal manifestations occur in the acute phase of aHUS, some can also occur as long-term sequelae of unopposed complement activation. Extra-renal symptoms are observed in approximately 20% of patients with aHUS, with the incidence of specific organ system complications ranging from a few case reports to 50% of described patients. Careful monitoring for extra-renal involvement is critical in patients with aHUS, as prompt evaluation and management may decrease the risk of high morbidity and mortality associated with aHUS.

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Correspondence to Cassandra Formeck.

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Dr. Cassandra Formeck - funded by NIDDK T32 training grant.

Dr. Agnieszka Swiatecka-Urban - funded by NIH grant R56HL127202.

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Answers to the Questions:

1. b; 2. d; 3. b; 4. e; 5. e

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Formeck, C., Swiatecka-Urban, A. Extra-renal manifestations of atypical hemolytic uremic syndrome. Pediatr Nephrol 34, 1337–1348 (2019). https://doi.org/10.1007/s00467-018-4039-7

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  • DOI: https://doi.org/10.1007/s00467-018-4039-7

Keywords

  • Atypical hemolytic-uremic syndrome (aHUS)
  • Thrombotic microangiopathy (TMA)
  • Extra-renal manifestations
  • Gastrointestinal complications
  • Cardiovascular complications
  • Neurologic complications