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Predictive Factors of Mortality in Population of Patients with Paroxysmal Nocturnal Hemoglobinuria (PNH): Results from a Korean PNH Registry
Journal of Korean Medical Science ; : 214-221, 2016.
Artículo en Inglés | WPRIM | ID: wpr-225588
ABSTRACT
Paroxysmal nocturnal hemoglobinuria (PNH) is a progressive, systemic, life-threatening disease, characterized by chronic uncontrolled complement activation. A retrospective analysis of 301 Korean PNH patients who had not received eculizumab was performed to systematically identify the clinical symptoms and signs predictive of mortality. PNH patients with hemolysis (lactate dehydrogenase [LDH] > or = 1.5 x the upper limit of normal [ULN]) have a 4.8-fold higher mortality rate compared with the age- and sex-matched general population (P < 0.001). In contrast, patients with LDH < 1.5 x ULN have a similar mortality rate as the general population (P = 0.824). Thromboembolism (TE) (odds ratio [OR] 7.11; 95% confidence interval [CI] (3.052-16.562), renal impairment (OR, 2.953; 95% CI, 1.116-7.818) and PNH-cytopenia (OR, 2.547; 95% CI, 1.159-5.597) are independent risk factors for mortality, with mortality rates 14-fold (P < 0.001), 8-fold (P < 0.001), and 6.2-fold (P < 0.001) greater than that of the age- and sex-matched general population, respectively. The combination of hemolysis and 1 or more of the clinical symptoms such as abdominal pain, chest pain, or dyspnea, resulted in a much greater increased mortality rate when compared with patients with just the individual symptom alone or just hemolysis. Early identification of risk factors related to mortality is crucial for the management of PNH. This trial was registered at www.clinicaltrials.gov as NCT01224483.
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Texto completo: Disponible Índice: WPRIM (Pacífico Occidental) Asunto principal: Tromboembolia / Oportunidad Relativa / Sistema de Registros / Estudios Retrospectivos / Factores de Riesgo / Curva ROC / Área Bajo la Curva / Disnea / Estimación de Kaplan-Meier / República de Corea Tipo de estudio: Estudio de etiología / Estudio observacional / Estudio pronóstico / Factores de riesgo Límite: Adolescente / Adulto / Anciano / Aged80 / Niño / Femenino / Humanos / Masculino País/Región como asunto: Asia Idioma: Inglés Revista: Journal of Korean Medical Science Año: 2016 Tipo del documento: Artículo

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Texto completo: Disponible Índice: WPRIM (Pacífico Occidental) Asunto principal: Tromboembolia / Oportunidad Relativa / Sistema de Registros / Estudios Retrospectivos / Factores de Riesgo / Curva ROC / Área Bajo la Curva / Disnea / Estimación de Kaplan-Meier / República de Corea Tipo de estudio: Estudio de etiología / Estudio observacional / Estudio pronóstico / Factores de riesgo Límite: Adolescente / Adulto / Anciano / Aged80 / Niño / Femenino / Humanos / Masculino País/Región como asunto: Asia Idioma: Inglés Revista: Journal of Korean Medical Science Año: 2016 Tipo del documento: Artículo