Congleton J, Hodson M E, Duncan-Skingle F
Department of Cystic Fibrosis, National Heart and Lung Institute, London, UK.
Thorax. 1996 Sep;51(9):936-40. doi: 10.1136/thx.51.9.936.
Cystic fibrosis is an inherited condition with a high mortality and morbidity. The aims of this study were to assess quality of life in a population of adults with cystic fibrosis, to compare quality of life with published scores from a healthy population and other patient groups, and to examine the relation between quality of life and other measured clinical variables.
Patients over 16 years of age attending an adult cystic fibrosis outpatient clinic were surveyed at a time when they were clinically stable. A self-complete questionnaire was administered which comprised the Nottingham Health Profile (NHP) together with six additional questions related to cystic fibrosis.
Completed questionnaires were obtained from 240 subjects (100 women) of median age 26 years (range 16-56). Mean (SD) forced expiratory volume in one second (FEV1) was 49 (26)% predicted, forced vital capacity (FVC) was 68 (26)% predicted, and the FEV1:FVC ratio was 59 (16)%. In this cross sectional study different patterns of perceived quality of life were seen in men and women. In part 1 of the NHP there was an age related trend compared with norms in men, with more distress/disability in the dimensions of emotion, sleep, and social isolation in the older age groups. In women there was no age related trend in the degree of distress/disability compared with norms. The mean score was different from norms in the dimensions of pain, emotion and sleep. For the patients with cystic fibrosis as a whole the scores in part 1 were comparable with published scores of patients with minor non-acute conditions. Scores in part 2 of the NHP for men were different from norms in six of the seven areas of daily living (all except home life). For women the scores were different from norms in the areas of looking after the home, social life, hobbies, and holidays. There were correlations between several of the quality of life dimensions and other measured variables such as FEV1, breathlessness score, and the time spent on home treatment.
Men and women with cystic fibrosis have different patterns of perceived quality of life, and there is an age related trend of perceived quality of life in men in some dimensions. Quality of life scores in this group, as assessed by the NHP, are similar to those reported in subjects with minor non-acute conditions.
囊性纤维化是一种遗传性疾病，死亡率和发病率都很高。本研究的目的是评估成年囊性纤维化患者群体的生活质量，将其生活质量与健康人群及其他患者群体已公布的评分进行比较，并研究生活质量与其他测量的临床变量之间的关系。
对就诊于成人囊性纤维化门诊的16岁以上患者在临床稳定时进行调查。发放一份自我填写的问卷，其中包括诺丁汉健康量表（NHP）以及六个与囊性纤维化相关的附加问题。
共获得240名受试者（100名女性）填写完整的问卷，中位年龄26岁（范围16 - 56岁）。一秒用力呼气量（FEV1）的平均（标准差）预计值为49（26）%，用力肺活量（FVC）为68（26）%，FEV1:FVC比值为59（16）%。在这项横断面研究中，男性和女性的生活质量感知模式不同。在NHP的第1部分，与男性的正常标准相比存在年龄相关趋势，年龄较大的组在情绪、睡眠和社会隔离维度上有更多的痛苦/残疾。与正常标准相比，女性在痛苦/残疾程度上没有年龄相关趋势。疼痛、情绪和睡眠维度的平均得分与正常标准不同。对于整个囊性纤维化患者群体，第1部分的得分与轻度非急性疾病患者已公布的得分相当。NHP第2部分中男性在七个日常生活领域中的六个领域（除家庭生活外）的得分与正常标准不同。女性在家庭照料、社交生活、爱好和假期领域的得分与正常标准不同。生活质量的几个维度与其他测量变量之间存在相关性，如FEV1、呼吸急促评分和家庭治疗时间。
囊性纤维化男性和女性的生活质量感知模式不同，男性在某些维度上的生活质量感知存在年龄相关趋势。通过NHP评估，该组的生活质量得分与轻度非急性疾病受试者报告的得分相似。
