Literature DB >> 900085

The effect of early diagnosis and treatment in cystic fibrosis: a seven-year study of 16 sibling pairs.

D M Orenstein, T F Boat, R C Stern, A S Tucker, E L Charnock, L W Matthews, C F Doershuk.   

Abstract

Data on 16 sibling pairs with cystic fibrosis were analyzed to test the hypothesis that early treatment of this condition improves prognosis. Younger siblings' conditions were diagnosed before 1 year of age, usually before the onset of pulmonary disease. Older siblings' conditions were diagnosed after 1 year of age and after the onset of pulmonary disease. Although the sibling pairs received similar treatment, comparison at 7 years of age showed that the younger siblings had significantly better chest roentgenogram scores, total clinical scores, residual lung volumes, and ratios of residual volume to total lung volume. Younger siblings also required fewer hospital admissions to control their lung disease. The results suggest that, in general, early initiation of therapy is beneficial for patients with cystic fibrosis.

Entities:  

Mesh:

Year:  1977        PMID: 900085     DOI: 10.1001/archpedi.1977.02120220039005

Source DB:  PubMed          Journal:  Am J Dis Child        ISSN: 0002-922X


  15 in total

1.  With commentary.

Authors:  P D Phelan
Journal:  Arch Dis Child       Date:  1982-10       Impact factor: 3.791

Review 2.  Neonatal screening--should we or shouldn't we?

Authors:  P H Weller; J V West
Journal:  J R Soc Med       Date:  1991       Impact factor: 5.344

3.  Screening for cystic fibrosis in the newborn by meconium analysis.

Authors:  H C Ryley; L M Neale; T D Brogan; P T Bray
Journal:  Arch Dis Child       Date:  1979-02       Impact factor: 3.791

4.  Sweat tests and flucloxacillin.

Authors:  J Williams; P D Griffiths; A Green; P H Weller
Journal:  Arch Dis Child       Date:  1988-07       Impact factor: 3.791

5.  Satisfactory screening for cystic fibrosis with the BM meconium procedure.

Authors:  R T Evans; A J Little; A E Steel; J M Littlewood
Journal:  J Clin Pathol       Date:  1981-08       Impact factor: 3.411

6.  Cystic fibrosis in blacks in Washington, DC: fifteen years' experience.

Authors:  N Prapphal; S B Fitzpatrick; P Getson; R Fink; R O'Donnell; H Chaney
Journal:  J Natl Med Assoc       Date:  1989-03       Impact factor: 1.798

Review 7.  Newborn screening for cystic fibrosis.

Authors:  Jack K Sharp; Michael J Rock
Journal:  Clin Rev Allergy Immunol       Date:  2008-12       Impact factor: 8.667

8.  Vitamin status in treated patients with cystic fibrosis.

Authors:  P J Congden; G Bruce; M M Rothburn; P C Clarke; J M Littlewood; J Kelleher; M S Losowsky
Journal:  Arch Dis Child       Date:  1981-09       Impact factor: 3.791

9.  Genetic and clinical features of patients with cystic fibrosis diagnosed after the age of 16 years.

Authors:  K H Gan; W P Geus; W Bakker; C B Lamers; H G Heijerman
Journal:  Thorax       Date:  1995-12       Impact factor: 9.139

10.  Neonatal screening for cystic fibrosis in Wales and the West Midlands: clinical assessment after five years of screening.

Authors:  S Chatfield; G Owen; H C Ryley; J Williams; M Alfaham; M C Goodchild; P Weller
Journal:  Arch Dis Child       Date:  1991-01       Impact factor: 3.791

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