Department of Cystic Fibrosis, National Heart and Lung Institute, Imperial College and Royal Brompton Hospital, London, UK.
Respir Med. 2009 Apr;103(4):629-35. doi: 10.1016/j.rmed.2008.10.011. Epub 2008 Nov 20.
The proportion of patients with cystic fibrosis (CF) who are middle-aged is increasing - and is likely to continue to do so. We surveyed a population of long-term CF survivors to assess their burden of illness and profile their disease characteristics.
A case series (n=112) of patients from one specialist centre who had reached their 40th birthday without transplantation. Hospital records and annual review data were examined.
The median age of the group was 43.1 years (range 40-71.1); 57% were men. 68% were diagnosed before 16 years of age. 30% were DeltaF508/DeltaF508, 76% having at least one DeltaF508 allele. When compared with the total adult CF population, the older patients were significantly less likely to have a DeltaF508 mutation or colonisation with Stenotrophomonas maltophilia and MRSA; but more likely to have pancreatic sufficiency, colonisation with Pseudomonas aeruginosa or allergic bronchopulmonary aspergillosis. On average they required less than one hospital admission a year; lung function and body mass index were relatively well preserved. Many were married and working.
We describe one of the largest surveys to date of CF patients aged more than 40 years. The full spectrum of disease is represented in this population and, importantly, 30% are DeltaF508 homozygous. Provision needs to be made for the healthcare needs of this increasing population of older patients.
患有囊性纤维化 (CF) 的中年患者比例正在增加——而且这种趋势可能还会继续。我们调查了一群长期 CF 幸存者，以评估他们的疾病负担，并分析他们的疾病特征。
这是一项来自一家专科中心的病例系列研究（n=112），研究对象均为在未进行移植的情况下已年满 40 岁的患者。我们对患者的医院记录和年度复查数据进行了检查。
该组患者的中位年龄为 43.1 岁（范围为 40-71.1 岁）；57%为男性。68%的患者在 16 岁之前被诊断出患有 CF。30%为 DeltaF508/DeltaF508 纯合子，76%至少携带一个 DeltaF508 等位基因。与成年 CF 患者的总体情况相比，年龄较大的患者携带 DeltaF508 突变或定植 Sten诺氏假单胞菌和耐甲氧西林金黄色葡萄球菌的可能性显著较低，但更有可能存在胰腺功能充足、定植铜绿假单胞菌或变应性支气管肺曲霉病的情况。平均而言，他们每年需要不到一次住院治疗；肺功能和体重指数相对较好。许多患者已婚且有工作。
我们描述了迄今为止最大规模的 CF 患者年龄超过 40 岁的调查之一。该人群代表了疾病的全貌，重要的是，30%的患者为 DeltaF508 纯合子。需要为这一不断增加的老年患者群体提供医疗保健需求。
