Extra-renal manifestations of atypical hemolytic uremic syndrome.
Atypical Hemolytic Uremic Syndrome
/ complications
Cardiovascular Diseases
/ epidemiology
Chronic Disease
Complement Pathway, Alternative
Eye Diseases
/ epidemiology
Gastrointestinal Diseases
/ epidemiology
Humans
Incidence
Lung Diseases
/ epidemiology
Nervous System Diseases
/ epidemiology
Skin Diseases
/ epidemiology
Atypical hemolytic-uremic syndrome (aHUS)
Cardiovascular complications
Extra-renal manifestations
Gastrointestinal complications
Neurologic complications
Thrombotic microangiopathy (TMA)
Journal
Pediatric nephrology (Berlin, Germany)
ISSN: 1432-198X
Titre abrégé: Pediatr Nephrol
Pays: Germany
ID NLM: 8708728
Informations de publication
Date de publication:
08 2019
08 2019
Historique:
received:
22
03
2018
accepted:
01
08
2018
revised:
31
07
2018
pubmed:
16
8
2018
medline:
6
6
2020
entrez:
16
8
2018
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 30109445
doi: 10.1007/s00467-018-4039-7
pii: 10.1007/s00467-018-4039-7
pmc: PMC8627279
mid: NIHMS1503862
doi:
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
1337-1348Subventions
Organisme : NHLBI NIH HHS
ID : R01 HL090767
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL144539
Pays : United States
Organisme : NIDDK NIH HHS
ID : T32 DK091202
Pays : United States
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