Independent genetic determinants of pancreatic and pulmonary status in cystic fibrosis

Lancet. 1990 Nov 3;336(8723):1081-4. doi: 10.1016/0140-6736(90)92566-z.

Abstract

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Alleles
  • Blood Proteins / genetics
  • Calgranulin A
  • Chromosome Mapping
  • Cystic Fibrosis / complications
  • Cystic Fibrosis / genetics*
  • Cystic Fibrosis / physiopathology
  • DNA / analysis*
  • Exocrine Pancreatic Insufficiency / etiology
  • Exocrine Pancreatic Insufficiency / genetics*
  • Exocrine Pancreatic Insufficiency / physiopathology
  • Female
  • Forced Expiratory Volume
  • Homozygote
  • Humans
  • Lung Diseases / etiology
  • Lung Diseases / genetics*
  • Lung Diseases / physiopathology
  • Male
  • Middle Aged
  • Mutation / genetics*
  • Phenotype
  • Polymerase Chain Reaction
  • Polymorphism, Genetic

Substances

  • Blood Proteins
  • Calgranulin A
  • cystic fibrosis serum factor
  • DNA