Open AccessExtra-renal manifestations of atypical hemolytic uremic syndrome
Author(s) -
Cassandra Formeck,
Agnieszka SwiateckaUrban
Publication year - 2018
Publication title -
pediatric nephrology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.831
H-Index - 107
eISSN - 1432-198X
pISSN - 0931-041X
DOI - 10.1007/s00467-018-4039-7
Subject(s) - medicine , atypical hemolytic uremic syndrome , nephrology , intensive care medicine , incidence (geometry) , complement system , disease , eculizumab , immunology , physics , antibody , optics
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.