Quality-of-Life Impairment in Neurofibromatosis Type 1

Autor: Emmanuel Ecosse, Jean Revuz, Pierre Wolkenstein, Jacques Zeller, Alain Leplège
Rok vydání: 2001
Předmět:
Zdroj: Archives of Dermatology. 137
ISSN: 0003-987X
Popis: Background Neurofibromatosis type 1 affects quality of life (QoL) through association with severe complications, impact on cosmetic features, and uncertainty of the effects of the disorder. Objective To evaluate the impact of the severity and visibility of neurofibromatosis type 1 on QoL. Design Monocenter, cross-sectional study. Setting One French academic dermatological and neurofibromatoses clinic. Patients A total of 128 adult patients with neurofibromatosis type 1. Main Outcome Measures Evaluation of severity and visibility using, respectively, the Riccardi and Ablon scales. Evaluation of skin disease–specific and general QoL using, respectively, Skindex-France and SF-36 (Short Form 36 health survey) profiles controlled for sex, age, severity, and visibility. Results In a multiple regression model controlling for sex, age, and visibility, visibility remained independently associated with the alteration of 3 aspects of the skin disease–specific QoL (Skindex-France): emotions, physical symptoms, and functioning ( P = .03, P = .009, and P = .002, respectively). Patients with more severe neurofibromatosis reported more effects on the following domains of their general health QoL (SF-36): physical function, bodily pain, general health perception, and vitality ( P = .006, P = .03, P = .01, and P = .04, respectively). Conclusions Neurofibromatosis type 1 has a significant impact on QoL through alteration of health and appearance. The consequences of visibility and severity from the viewpoint of patients can be evaluated using Skindex and the SF-36, respectively.
Databáze: OpenAIRE