Recent advances in cystic fibrosis

被引：47

作者：

Doull, IJM ^{[1
]}

机构：

[1] Univ Wales Hosp, Dept Child Hlth, Cyst Fibrosis Resp Unit, Cardiff CF14 4XW, S Glam, Wales

来源：

ARCHIVES OF DISEASE IN CHILDHOOD | 2001年 / 85卷 / 01期

关键词：

cystic fibrosis; management;

D O I：

10.1136/adc.85.1.62

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

The median life expectancy for cystic fibrosis is now over 30 years, and it is projected that in newborn infants it will become more than 40 years. The identification of the cystic fibrosis gene and its product, cystic fibrosis transmembrane conductance regulator (CFTR), has widened the spectrum of the disease from the classical case of the infant with cystic fibrosis to the elderly childless man with unexplained bronchiectasis. There is increasing evidence of the advantages of newborn screening for cystic fibrosis and subsequent specialist care. Management concentrates on optimising nutritional status and preventing lung infection and inflammation.

引用

页码：62 / 66

页数：5

共 37 条

[1] Cationic lipid-mediated CFTR gene transfer to the lungs and nose of patients with cystic fibrosis:: a double-blind placebo-controlled trial [J].

Alton, EWFW ;

Stern, M ;

Farley, R ;

Jaffe, A ;

Chadwick, SL ;

Phillips, J ;

Davies, J ;

Smith, SN ;

Browning, J ;

Davies, MG ;

Hodson, ME ;

Durham, SR ;

Li, D ;

Jeffery, PK ;

Scallan, M ;

Balfour, R ;

Eastman, SJ ;

Cheng, SH ;

Smith, AE ;

Meeker, D ;

Geddes, DM .

LANCET, 1999, 353 (9157) :947-954

[2] Lower airway inflammation in infants and young children with cystic fibrosis [J].

Armstrong, DS ;

Grimwood, K ;

Carlin, JB ;

Carzino, R ;

Gutierrez, JP ;

Hull, J ;

Olinsky, A ;

Phelan, EM ;

Robertson, CF ;

Phelan, PD .

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 1997, 156 (04) :1197-1204

[3] Lung transplantation and life extension in children with cystic fibrosis [J].

Aurora, P ;

Whitehead, B ;

Wade, A ;

Bowyer, J ;

Whitmore, P ;

Rees, PG ;

Tsang, VT ;

Elliott, MJ ;

de Leval, M .

LANCET, 1999, 354 (9190) :1591-1593

[4] Status of gene therapy for cystic fibrosis lung disease [J].

Boucher, RC .

JOURNAL OF CLINICAL INVESTIGATION, 1999, 103 (04) :441-445

[5] Recent advances in management of acute leukaemia [J].

Chessells, JM .

ARCHIVES OF DISEASE IN CHILDHOOD, 2000, 82 (06) :438-442

[6] MUTATIONS IN THE CYSTIC-FIBROSIS GENE IN PATIENTS WITH CONGENITAL ABSENCE OF THE VAS-DEFERENS [J].

CHILLON, M ;

CASALS, T ;

MERCIER, B ;

BASSAS, L ;

LISSENS, W ;

SILBER, S ;

ROMEY, MC ;

RUIZROMERO, J ;

VERLINGUE, C ;

CLAUSTRES, M ;

NUNES, V ;

FEREC, C ;

ESTIVILL, X .

NEW ENGLAND JOURNAL OF MEDICINE, 1995, 332 (22) :1475-1480

[7] Relation between mutations of the cystic fibrosis gene and idiopathic pancreatitis [J].

Cohn, JA ;

Friedman, KJ ;

Noone, PG ;

Knowles, MR ;

Silverman, LM ;

Jowell, PS .

NEW ENGLAND JOURNAL OF MEDICINE, 1998, 339 (10) :653-658

[8] Recombinant human DNase (rhDNase) in cystic fibrosis: is it cost effective? [J].

Conway, SP .

ARCHIVES OF DISEASE IN CHILDHOOD, 1997, 77 (01) :1-3

[9] Incidence, population, and survival of cystic fibrosis in the UK, 1968-95 [J].

Dodge, JA ;

Morison, S ;

Lewis, PA ;

Coles, EC ;

Geddes, D ;

Russell, G ;

Littlewood, JM ;

Scott, MT .

ARCHIVES OF DISEASE IN CHILDHOOD, 1997, 77 (06) :493-496

[10] Elective versus symptomatic antibiotic treatment in cystic fibrosis patients with chronic Pseudomonas infection of the lungs [J].

Elborn, JS ;

Prescott, RJ ;

Stack, BHR ;

Goodchild, MC ;

Bates, J ;

Pantin, C ;

Ali, N ;

Shale, DJ ;

Crane, M .

THORAX, 2000, 55 (05) :355-358

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