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囊性纤维化:新视角。70名25岁以上患者。

Cystic fibrosis: a new outlook. 70 patients above 25 years of age.

作者信息

Shwachman H, Kowalski M, Khaw K T

出版信息

Medicine (Baltimore). 1977 Mar;56(2):129-49.

PMID:846387
Abstract

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.

摘要

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。

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Cystic fibrosis: a new outlook. 70 patients above 25 years of age.囊性纤维化:新视角。70名25岁以上患者。
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引用本文的文献

1
Genetic and clinical features of patients with cystic fibrosis diagnosed after the age of 16 years.16岁以后确诊的囊性纤维化患者的遗传和临床特征。
Thorax. 1995 Dec;50(12):1301-4. doi: 10.1136/thx.50.12.1301.
2
Spontaneous bacterial peritonitis in cystic fibrosis.囊性纤维化患者的自发性细菌性腹膜炎
Gut. 1994 May;35(5):709-11. doi: 10.1136/gut.35.5.709.
3
An approach for treating the hepatobiliary disease of cystic fibrosis by somatic gene transfer.一种通过体细胞基因转移治疗囊性纤维化肝胆疾病的方法。
Proc Natl Acad Sci U S A. 1993 May 15;90(10):4601-5. doi: 10.1073/pnas.90.10.4601.
4
Arthritis in childhood cystic fibrosis.儿童囊性纤维化中的关节炎
Ir J Med Sci. 1982 Aug;151(8):253-4. doi: 10.1007/BF02940194.
5
Immunological abnormalities in cystic fibrosis: chicken or egg?囊性纤维化中的免疫异常:先有鸡还是先有蛋?
Thorax. 1980 Nov;35(11):801-6. doi: 10.1136/thx.35.11.801.
6
Utilization of human respiratory secretions by mucoid Pseudomonas aeruginosa of cystic fibrosis origin.源自囊性纤维化的黏液型铜绿假单胞菌对人呼吸道分泌物的利用。
Infect Immun. 1982 Aug;37(2):662-9. doi: 10.1128/iai.37.2.662-669.1982.
7
Influence of mucoid coating on clearance of Pseudomonas aeruginosa from lungs.黏液样包被对铜绿假单胞菌从肺部清除的影响。
Infect Immun. 1981 May;32(2):443-8. doi: 10.1128/iai.32.2.443-448.1981.
8
Respiratory tract disease and obstructive azoospermia.呼吸道疾病与梗阻性无精子症。
Thorax. 1983 Dec;38(12):929-33. doi: 10.1136/thx.38.12.929.
9
The pathology of meconium ileus equivalent.胎粪性肠梗阻等效症的病理学
J Clin Pathol. 1983 Nov;36(11):1292-7. doi: 10.1136/jcp.36.11.1292.
10
Pancreatitis as initial presentation of cystic fibrosis in young adults. A report of two cases.胰腺炎作为年轻成年人囊性纤维化的首发表现。两例报告。
Dig Dis Sci. 1983 Oct;28(10):874-8. doi: 10.1007/BF01317036.