Shwachman H, Kowalski M, Khaw K T
Medicine (Baltimore). 1977 Mar;56(2):129-49.
We have presented a group of 70 patients over 25 years of age as of January 1, 1975, who were diagnosed as having cystic fibrosis (CF) at various ages from birth to 28 years. Some patients were diagnosed late in life in spite of classical symptoms and a characteristic history of the disease. Patients with CF present with a broad spectrum of symptoms which vary considerably in severity. This older age group represents approximately 10% of our current roster of patients with CF. This disease is no longer confined to early life and will continue to be seen with increasing frequency in older patients. Our experience with this disease indicates that a number of affected individuals can attain adulthood and find a useful place in society. The quality of life provided to these adults is illustrated in their intellectual, socioeconomic achievements and marital life. Our aim has been to recognize the disease as early in life as possible and to provide a comprehensive medical program which includes all aspects of health care.
我们呈现了一组70名患者,截至1975年1月1日,他们年龄超过25岁,在从出生到28岁的不同年龄段被诊断为患有囊性纤维化(CF)。尽管有典型症状和该病的特征性病史,但一些患者在生命后期才被诊断出来。CF患者表现出广泛的症状,严重程度差异很大。这个年龄较大的群体约占我们目前CF患者名单的10%。这种疾病不再局限于早年,在老年患者中将会越来越频繁地出现。我们对这种疾病的经验表明,一些受影响的个体能够成年并在社会中找到有用的位置。这些成年人的生活质量体现在他们的智力、社会经济成就和婚姻生活中。我们的目标是尽早识别这种疾病,并提供一个包括医疗保健各个方面的综合医疗计划。