Ageing in Cystic Fibrosis and Long-term Survival

被引：32

作者：

Simmonds, N. J. ^{[1
]}

机构：

[1] Univ London Imperial Coll Sci Technol & Med, Royal Brompton Hosp, Dept Cyst Fibrosis, London SW3 6NA, England

来源：

PAEDIATRIC RESPIRATORY REVIEWS | 2013年 / 14卷

关键词：

Adults; Ageing; Cystic fibrosis; Survival; DISEASE; AGE; DIAGNOSIS; PHENOTYPE; GENOTYPE; MUTATION;

D O I：

10.1016/j.prrv.2013.01.007

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

The demographics of cystic fibrosis continue to change with adults outnumbering children in most developed countries. Median predicted survival is now over 40 years and 7.6% are aged >40 years. Patients surviving beyond 40 cover the full spectrum of disease from homozygous F508del to single organ disease. Differences in the characteristics of older patients are recognised, but generally patients diagnosed in adulthood are still at risk of accelerated lung function decline. Improved survival brings new challenges, including a rising rate of CF co-morbidities such as diabetes, in addition to other medical problems such as renal impairment and ototoxicity. (C) 2013 Elsevier Ltd. All rights reserved.

引用

页码：6 / 9

页数：4

共 20 条

[1] Cystic fibrosis of the pancreas and its relation to celiac disease - A clinical and pathologic study [J].

Andersen, DH .

AMERICAN JOURNAL OF DISEASES OF CHILDREN, 1938, 56 (02) :344-399

[2]

[Anonymous], 2012, PAT REG ANN DAT REP

[3] MILD CYSTIC-FIBROSIS AND NORMAL OR BORDERLINE SWEAT TEST IN PATIENTS WITH THE 3849+10 KB C-]T MUTATION [J].

AUGARTEN, A ;

KEREM, BS ;

YAHAV, Y ;

NOIMAN, S ;

RIVLIN, Y ;

TAL, A ;

BLAU, H ;

BENTUR, L ;

SZEINBERG, A ;

KEREM, E ;

GAZIT, E .

LANCET, 1993, 342 (8862) :25-26

[4] Association between socioeconomic status, sex, and age at death from cystic fibrosis in England and Wales (1959 to 2008): cross sectional study [J].

Barr, Helen L. ;

Britton, John ;

Smyth, Alan R. ;

Fogarty, Andrew W. .

BMJ-BRITISH MEDICAL JOURNAL, 2011, 343

[5]

Cystic Fibrosis Trust, 2011, CYST FIBR DAT REP 20

[6] Cystic fibrosis: terminology and diagnostic algorithms [J].

De Boeck, K. ;

Wilschanski, M. ;

Castellani, C. ;

Taylor, C. ;

Cuppens, H. ;

Dodge, J. ;

Sinaasappel, M. .

THORAX, 2006, 61 (07) :627-635

[7] Cystic fibrosis mortality and survival in the UK: 1947-2003 [J].

Dodge, J. A. ;

Lewis, P. A. ;

Stanton, M. ;

Wilsher, J. .

EUROPEAN RESPIRATORY JOURNAL, 2007, 29 (03) :522-526

[8] An international/multicentre report on patients with cystic fibrosis (CF) over the age of 40 years [J].

Hodson, Margaret E. ;

Simmonds, Nicholas J. ;

Warwick, Warren J. ;

Tullis, Elizabeth ;

Castellani, Carlo ;

Assael, Baroukh ;

Dodge, John A. ;

Corey, Mary .

JOURNAL OF CYSTIC FIBROSIS, 2008, 7 (06) :537-542

[9] THE RELATION BETWEEN GENOTYPE AND PHENOTYPE IN CYSTIC-FIBROSIS - ANALYSIS OF THE MOST COMMON MUTATION (DELTA-F508) [J].

KEREM, E ;

COREY, M ;

KEREM, BS ;

ROMMENS, J ;

MARKIEWICZ, D ;

LEVISON, H ;

TSUI, LC ;

DURIE, P .

NEW ENGLAND JOURNAL OF MEDICINE, 1990, 323 (22) :1517-1522

[10]

Maisonneuve P, 2012, J NATL CANC I

← 1 2 →