Radiological evaluation of the lungs in children with cystic fibrosis diagnosed during newborn screening examinations

2006
journal article
article
dc.abstract.enBackground: Cystic fibrosis is an inherited, autosomal, recessive disease. This disorder is caused by defects in the gene for cystic fibrosis transmembrane conductance regulator (CFTR), which encodes for a protein that functions as a chloride channel. Mutations in the gene for CFTR result in ion disorders, and consequently in disturbances of exocrine glands in the respiratory, gastrointestinal, and genitourinary tracts. Pulmonary involvement occurs in 90% of patients, and is the main cause of death. The diagnosis of CF in Poland is based on clinical symptoms and positive results of the sweat test. Diacrisis is usually reached late in the 3rd year of life. In 1999-2003, newborn screening examinations were performed at the Mother and Child Institute. The idea of these studies was to establish a diagnosis and begin treatment as early as possible, even in the asymptomatic period of the disease. The level of immunoreactive trypsynogen was determined in the blood of 4-6-day-old newborns, as well as the mutation of gene CFTR. The mean age of CF diagnosis was about 38 days. The aim of our study was to assess the influence of early commencement of treatment on the rapidity of progression of pulmonary involvement. Material/Methods: 59 children with CF diagnosed by screening were examined by chest radiography in various periods of the disease, the earliest in the neonatal period. Pulmonary involvement (hyperinflation, periobronchial thickening, pulmonary nodules, cysts, parenchymal density, atelectasis and fibrosus changes) were assessed according to Brasfield score. The control group consisted of 19 children with symptomatic CF, born in 1997-2003.They were also examined by chest radiography. Results: Various pulmonary changes were recognized in 42 children diagnosed by screening. In the control group pulmonary involvement was found in 16 children. In both groups progression was found in 28% of the children, but significant progression was seen in 7% of those children with a screening diagnosis, and in 25% of the children with symptomatic CF. Conclusions: An analysis of these results suggests that early diagnosis and early commencement of treatment do not prevent pulmonary involvement, but moderate the progression of the disease.pl
dc.contributor.authorIwanowska, Beatapl
dc.contributor.authorKopyś-Wiszniewska, Izabelapl
dc.contributor.authorSands, Dorotapl
dc.date.accession2019-07-10pl
dc.date.accessioned2019-07-10T14:55:40Z
dc.date.available2019-07-10T14:55:40Z
dc.date.issued2006pl
dc.date.openaccess0
dc.description.accesstimew momencie opublikowania
dc.description.additionalBibliogr. s. 23pl
dc.description.number2pl
dc.description.physical18-23pl
dc.description.versionostateczna wersja wydawcy
dc.description.volume71pl
dc.identifier.articleid451182pl
dc.identifier.eissn1899-0967pl
dc.identifier.issn1733-134Xpl
dc.identifier.projectROD UJ / OPpl
dc.identifier.urihttps://ruj.uj.edu.pl/xmlui/handle/item/78842
dc.identifier.weblinkhttp://archiwum.inforadiologia.pl/download/index/idArt/451182.htmlpl
dc.languagepolpl
dc.language.containerpolpl
dc.rightsUdzielam licencji. Uznanie autorstwa - Użycie niekomercyjne - Bez utworów zależnych 4.0 Międzynarodowa*
dc.rights.licenceCC-BY-NC-ND
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/legalcode.pl*
dc.share.typeotwarte czasopismo
dc.subject.encystic fibrosispl
dc.subject.ennewborn screeningpl
dc.subject.enX-ray examinationpl
dc.subtypeArticlepl
dc.titleRadiological evaluation of the lungs in children with cystic fibrosis diagnosed during newborn screening examinationspl
dc.title.alternativeOcena radiologiczna płuc u dzieci z mukowiscydozą rozpoznaną w wyniku badania przesiewowego noworodkówpl
dc.title.journalPolish Journal of Radiologypl
dc.typeJournalArticlepl
dspace.entity.typePublication
dc.abstract.enpl
Background: Cystic fibrosis is an inherited, autosomal, recessive disease. This disorder is caused by defects in the gene for cystic fibrosis transmembrane conductance regulator (CFTR), which encodes for a protein that functions as a chloride channel. Mutations in the gene for CFTR result in ion disorders, and consequently in disturbances of exocrine glands in the respiratory, gastrointestinal, and genitourinary tracts. Pulmonary involvement occurs in 90% of patients, and is the main cause of death. The diagnosis of CF in Poland is based on clinical symptoms and positive results of the sweat test. Diacrisis is usually reached late in the 3rd year of life. In 1999-2003, newborn screening examinations were performed at the Mother and Child Institute. The idea of these studies was to establish a diagnosis and begin treatment as early as possible, even in the asymptomatic period of the disease. The level of immunoreactive trypsynogen was determined in the blood of 4-6-day-old newborns, as well as the mutation of gene CFTR. The mean age of CF diagnosis was about 38 days. The aim of our study was to assess the influence of early commencement of treatment on the rapidity of progression of pulmonary involvement. Material/Methods: 59 children with CF diagnosed by screening were examined by chest radiography in various periods of the disease, the earliest in the neonatal period. Pulmonary involvement (hyperinflation, periobronchial thickening, pulmonary nodules, cysts, parenchymal density, atelectasis and fibrosus changes) were assessed according to Brasfield score. The control group consisted of 19 children with symptomatic CF, born in 1997-2003.They were also examined by chest radiography. Results: Various pulmonary changes were recognized in 42 children diagnosed by screening. In the control group pulmonary involvement was found in 16 children. In both groups progression was found in 28% of the children, but significant progression was seen in 7% of those children with a screening diagnosis, and in 25% of the children with symptomatic CF. Conclusions: An analysis of these results suggests that early diagnosis and early commencement of treatment do not prevent pulmonary involvement, but moderate the progression of the disease.
dc.contributor.authorpl
Iwanowska, Beata
dc.contributor.authorpl
Kopyś-Wiszniewska, Izabela
dc.contributor.authorpl
Sands, Dorota
dc.date.accessionpl
2019-07-10
dc.date.accessioned
2019-07-10T14:55:40Z
dc.date.available
2019-07-10T14:55:40Z
dc.date.issuedpl
2006
dc.date.openaccess
0
dc.description.accesstime
w momencie opublikowania
dc.description.additionalpl
Bibliogr. s. 23
dc.description.numberpl
2
dc.description.physicalpl
18-23
dc.description.version
ostateczna wersja wydawcy
dc.description.volumepl
71
dc.identifier.articleidpl
451182
dc.identifier.eissnpl
1899-0967
dc.identifier.issnpl
1733-134X
dc.identifier.projectpl
ROD UJ / OP
dc.identifier.uri
https://ruj.uj.edu.pl/xmlui/handle/item/78842
dc.identifier.weblinkpl
http://archiwum.inforadiologia.pl/download/index/idArt/451182.html
dc.languagepl
pol
dc.language.containerpl
pol
dc.rights*
Udzielam licencji. Uznanie autorstwa - Użycie niekomercyjne - Bez utworów zależnych 4.0 Międzynarodowa
dc.rights.licence
CC-BY-NC-ND
dc.rights.uri*
http://creativecommons.org/licenses/by-nc-nd/4.0/legalcode.pl
dc.share.type
otwarte czasopismo
dc.subject.enpl
cystic fibrosis
dc.subject.enpl
newborn screening
dc.subject.enpl
X-ray examination
dc.subtypepl
Article
dc.titlepl
Radiological evaluation of the lungs in children with cystic fibrosis diagnosed during newborn screening examinations
dc.title.alternativepl
Ocena radiologiczna płuc u dzieci z mukowiscydozą rozpoznaną w wyniku badania przesiewowego noworodków
dc.title.journalpl
Polish Journal of Radiology
dc.typepl
JournalArticle
dspace.entity.type
Publication
Affiliations

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