Nutrition for Pregnant Women Who Have Cystic Fibrosis

Published:November 20, 2012DOI:
      CYSTIC FIBROSIS (CF) IS THE MOST COMMON autosomal-recessive life-threatening disease in the United States.
      • Farrell P.M.
      • Rosenstein B.J.
      • White T.B.
      • et al.
      Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report.
      CF is caused by a defect on chromosome 7 in the area responsible for coding the cystic fibrosis transmembrane regulator (CFTR) protein.
      • Riordan J.R.
      • Rommens J.M.
      • Kerem B.-S.
      • et al.
      Identifications of the cystic fibrosis gene: Cloning and characterization of complementary DNA.
      More than 1,500 mutations of CFTR have been identified, leading to wide variability in expressed pathology.
      • Farrell P.M.
      • Rosenstein B.J.
      • White T.B.
      • et al.
      Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report.
      Disordered production or function of the CFTR causes abnormal transport of ions and water across cells in many organs of the body. This results in thick, tenacious mucus and organ damage, especially in the respiratory, gastrointestinal, endocrine, and reproductive systems.


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      S. H. Michel is an advanced practice dietitian, Clinical Nutrition/Cystic Fibrosis Center, The Children's Hospital of Philadelphia, Philadelphia, PA.


      D. H. Mueller is a tenured associate professor, Department of Nutrition Sciences, College of Nursing and Health Professions, Drexel University, Philadelphia, PA.