Impact of hypertensive emergency and rare complement variants on the presentation and outcome of atypical hemolytic uremic syndrome.
Adult
Antibodies, Monoclonal, Humanized
/ therapeutic use
Atypical Hemolytic Uremic Syndrome
/ drug therapy
Complement C3
/ antagonists & inhibitors
Complement Inactivating Agents
/ therapeutic use
Emergencies
Female
Follow-Up Studies
Genetic Variation
Humans
Hypertension
/ complications
Male
Prognosis
Retrospective Studies
Survival Rate
Journal
Haematologica
ISSN: 1592-8721
Titre abrégé: Haematologica
Pays: Italy
ID NLM: 0417435
Informations de publication
Date de publication:
12 2019
12 2019
Historique:
received:
21
01
2019
accepted:
18
03
2019
pubmed:
21
3
2019
medline:
5
8
2020
entrez:
21
3
2019
Statut:
ppublish
Résumé
A typical hemolytic uremic syndrome (aHUS) is a prototypic thrombotic microangiopathy attributable to complement dysregulation. Hypertensive emergency, characterized by elevation of systolic (>180 mmHg) or diastolic (>120 mmHg) blood pressure together with end-organ damage, can cause thrombotic microangiopathy which may mimic aHUS. We retrospectively evaluated the clinical, biological and complement genetic characteristics of 76 and 61 aHUS patients with and without hypertensive emergency, respectively. Patients with hypertensive emergency-aHUS were more frequently males, with neurological involvement, and a slightly higher hemoglobin level. At least one rare complement variant was identified in 51.3% (39/76) and 67% (41/61) patients with or without hypertensive emergency, respectively (
Identifiants
pubmed: 30890598
pii: haematol.2019.216903
doi: 10.3324/haematol.2019.216903
pmc: PMC6959192
doi:
Substances chimiques
Antibodies, Monoclonal, Humanized
0
Complement C3
0
Complement Inactivating Agents
0
eculizumab
A3ULP0F556
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
2501-2511Informations de copyright
Copyright© 2019 Ferrata Storti Foundation.
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