Independent genetic determinants of pancreatic and pulmonary status in cystic fibrosis.
The concordance of pancreatic and pulmonary status among siblings with cystic fibrosis, the cystic fibrosis genotype, and non-genetic factors were analysed in adult patients with cystic fibrosis. Genetic factors were more important than non-genetic factors in affecting the severity of pancreatic and lung disease. The genetic factors were independent of each other and did not invariably cosegregate with specific mutations at the cystic fibrosis locus.
Santis
G
G
Department of Cystic Fibrosis, Royal Brompton and National Heart Hospital, London, UK.
Osborne
L
L
Knight
R A
RA
Hodson
M E
ME
D000328:Adult; D000483:Alleles; D001798:Blood Proteins / Q000235:genetics; D040501:Calgranulin A; D002874:Chromosome Mapping; D003550:Cystic Fibrosis / Q000150:complications / Q000235:genetics* / Q000503:physiopathology; D004247:DNA / Q000032:analysis*; D010188:Exocrine Pancreatic Insufficiency / Q000209:etiology / Q000235:genetics* / Q000503:physiopathology; D005260:Female; D005541:Forced Expiratory Volume; D006720:Homozygote; D006801:Humans; D008171:Lung Diseases / Q000209:etiology / Q000235:genetics* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009154:Mutation / Q000235:genetics*; D010641:Phenotype; D016133:Polymerase Chain Reaction; D011110:Polymorphism, Genetic
D001798:Blood Proteins; D040501:Calgranulin A; C026422:cystic fibrosis serum factor; D004247:DNA