Turkish pediatric atypical hemolytic uremic syndrome registry: initial analysis of 146 patients

BESBAS, NESRİN; GULHAN, BORA; SOYLEMEZOGLU, Oguz; OZCAKAR, Zeynep; KORKMAZ, Emine; HAYRAN, KADİR; Ozaltin, FATİH

doi:10.1186/s12882-016-0420-6

Turkish pediatric atypical hemolytic uremic syndrome registry: initial analysis of 146 patients

BESBAS N., GULHAN B., SOYLEMEZOGLU O., OZCAKAR Z. B., KORKMAZ E., HAYRAN M., ...Daha Fazla

BMC NEPHROLOGY, cilt.18, 2017 (SCI-Expanded, Scopus)

Yayın Türü: Makale / Tam Makale
Cilt numarası: 18
Basım Tarihi: 2017
Doi Numarası: 10.1186/s12882-016-0420-6
Dergi Adı: BMC NEPHROLOGY
Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
Anahtar Kelimeler: Atypical hemolytic uremic syndrome, Turkish registry, Treatment, Outcome, Prognosis, COMPLEMENT INHIBITOR ECULIZUMAB, FACTOR-H MUTATIONS, CLINICAL PHENOTYPE, GENE, VARIANTS, CHILDREN, AHUS, DISCONTINUATION
Hacettepe Üniversitesi Adresli: Evet

Özet

Background: Atypical hemolytic uremic syndrome (aHUS) is a devastating disease with significant morbidity and mortality. Its genetic heterogeneity impacts its clinical presentation, progress, and outcome, and there is no consensus on its clinical management.