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.
Article
de En
| WPRIM
| ID: wpr-225588
Bibliothèque responsable:
WPRO
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.
Mots clés
Texte intégral:
1
Indice:
WPRIM
Sujet Principal:
Thromboembolie
/
Odds ratio
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Enregistrements
/
Études rétrospectives
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Facteurs de risque
/
Courbe ROC
/
Aire sous la courbe
/
Dyspnée
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Estimation de Kaplan-Meier
/
République de Corée
Type d'étude:
Etiology_studies
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limites du sujet:
Adolescent
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Adult
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Aged
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Aged80
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Child
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Female
/
Humans
/
Male
Pays comme sujet:
Asia
langue:
En
Texte intégral:
Journal of Korean Medical Science
Année:
2016
Type:
Article