Please use this identifier to cite or link to this item: http://hdl.handle.net/10553/52078
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dc.contributor.authorVillegas, Anaen_US
dc.contributor.authorNúñez, Ramiroen_US
dc.contributor.authorGaya, Annaen_US
dc.contributor.authorCuevas-Ruiz, María Victoriaen_US
dc.contributor.authorBosch, José Miguelen_US
dc.contributor.authorCarral, Annaen_US
dc.contributor.authorArrizabalaga, Beatrizen_US
dc.contributor.authorGómez-Roncero, María Isabelen_US
dc.contributor.authorMora, Asunciónen_US
dc.contributor.authorBravo, Pilaren_US
dc.contributor.authorLavilla, Esperanzaen_US
dc.contributor.authorMonteserín, Carmenen_US
dc.contributor.authorHernández, Belénen_US
dc.contributor.authorMartínez-Barranco, Pilaren_US
dc.contributor.authorJarque, Isidroen_US
dc.contributor.authorUrquía, María Anunciaciónen_US
dc.contributor.authorGarcía-Donas, Gloriaen_US
dc.contributor.authorBrunet, Saluten_US
dc.contributor.authorGonzález, Fernando Ataulfoen_US
dc.contributor.authorUrbano, Álvaroen_US
dc.date.accessioned2018-11-25T17:17:04Z-
dc.date.available2018-11-25T17:17:04Z-
dc.date.issued2017en_US
dc.identifier.issn0939-5555en_US
dc.identifier.urihttp://hdl.handle.net/10553/52078-
dc.description.abstractParoxysmal nocturnal hemoglobinuria (PNH) is a rare, life-threatening blood disease. With the advent of eculizumab treatment, renal function has substantially improved, although no data from real-world clinical practice are available. An observational, retrospective, multicenter study was conducted in Spain on clinical data obtained from outpatient visits of patients with PNH (Spanish PNH Registry) who had experienced acute (ARF) or chronic (CRF) renal failure. Of the 128 patients registered (April 2014), 60 were diagnosed with classic PNH. Twenty-seven (45.0%) patients with a mean age of 48.5 (±16.2) years had renal failure, ARF or CRF, and were included in this study. Near half of the patients (n = 13; 48.1%) presented with ARF alone, 33.3% (n = 9) had CRF with episodes of ARF, while 18.5% (n = 5) were diagnosed with CRF alone. For patients with diagnosis of PNH and renal failure (n = 27), the median time to the first ARF episode was 6.5 (CI 95%; 2.2, 14.9) years, whereas the median to the diagnosis of CRF was 14.5 (CI 95%; 3.8, 19.2) years after the diagnosis of PNH. Patients with ARF (n = 22) were treated with eculizumab and did not experience new episodes of ARF, except for one patient with sepsis. Of the patients with CRF, two received treatment without experiencing further episodes of ARF. Sixteen patients who completed treatment (11 with ARF and 5 with ARF + CRF) recovered from the episode of ARF or from CRF. Of the remaining patients treated with eculizumab, one patient improved from stages III to II, three patients stabilized without showing disease progression, and one patient progressed from stages III to IV. Treatment with eculizumab in PNH patients has beneficial effects on renal function, preventing ARF and progression to CRF.en_US
dc.languageengen_US
dc.relation.ispartofAnnals of Hematologyen_US
dc.sourceAnnals of Hematology[ISSN 0939-5555],v. 96, p. 1727-1733 (Julio 2017)en_US
dc.subject32 Ciencias médicasen_US
dc.subject320504 Hematologíaen_US
dc.subject.otherParoxysmal nocturnal hemoglobinuria (PNH)en_US
dc.subject.otherAcute and chronic renal failureen_US
dc.subject.otherEculizumaben_US
dc.subject.otherGlomerular filtration rateen_US
dc.subject.otherRenal function statusen_US
dc.titlePresence of acute and chronic renal failure in patients with paroxysmal nocturnal hemoglobinuria: results of a retrospective analysis from the Spanish PNH Registryen_US
dc.typeinfo:eu-repo/semantics/articleen_US
dc.typeArticleen_US
dc.identifier.doi10.1007/s00277-017-3059-xen_US
dc.identifier.scopus85026903163-
dc.contributor.authorscopusid35494384900-
dc.contributor.authorscopusid8865319200-
dc.contributor.authorscopusid56228751100-
dc.contributor.authorscopusid57195305939-
dc.contributor.authorscopusid7402325631-
dc.contributor.authorscopusid6508307271-
dc.contributor.authorscopusid6602243797-
dc.contributor.authorscopusid6504107791-
dc.contributor.authorscopusid55827968400-
dc.contributor.authorscopusid57188550409-
dc.contributor.authorscopusid6602200160-
dc.contributor.authorscopusid6506243865-
dc.contributor.authorscopusid26430550900-
dc.contributor.authorscopusid55420965200-
dc.contributor.authorscopusid7003758640-
dc.contributor.authorscopusid57195305123-
dc.contributor.authorscopusid57195305731-
dc.contributor.authorscopusid55597087872-
dc.contributor.authorscopusid57194243771-
dc.contributor.authorscopusid7005068225-
dc.description.lastpage1733en_US
dc.description.firstpage1727en_US
dc.relation.volume96en_US
dc.investigacionCiencias de la Saluden_US
dc.type2Artículoen_US
dc.description.numberofpages7en_US
dc.utils.revisionen_US
dc.date.coverdateJulio 2017en_US
dc.identifier.ulpgcen_US
dc.contributor.buulpgcBU-MEDen_US
dc.description.sjr1,043-
dc.description.jcr2,845-
dc.description.sjrqQ1-
dc.description.jcrqQ2-
dc.description.scieSCIE-
item.grantfulltextnone-
item.fulltextSin texto completo-
crisitem.author.deptGIR Biopoética, Semiótica Cognitiva y Neuroestética-
crisitem.author.deptIU de Investigaciones Biomédicas y Sanitarias-
crisitem.author.deptDepartamento de Ciencias Médicas y Quirúrgicas-
crisitem.author.parentorgIU de Investigaciones Biomédicas y Sanitarias-
crisitem.author.fullNameBosch Benítez, José Miguel-
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