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CMAJ : Canadian Medical Association Journal logoLink to CMAJ : Canadian Medical Association Journal
. 1993 Sep 15;149(6):809–813.

Cystic fibrosis in pregnancy.

N E Kent 1, D F Farquharson 1
PMCID: PMC1485377  PMID: 8374843

Abstract

OBJECTIVE: To review the outcomes of pregnancies in women with cystic fibrosis (CF) and to address issues pertinent to the obstetric care of such women. DATA SOURCES: English-language case reports and case series published from 1960 to 1991 identified through a search of MEDLINE and Index Medicus. The terms of reference were "cystic fibrosis" and "pregnancy". Not all the reports reviewed addressed all the outcomes under consideration. STUDY SELECTION: A total of 20 reports citing cases of pregnancy in women with CF. DATA EXTRACTION: Outcomes included the number of spontaneous abortions, pregnancies continued beyond 20 weeks, preterm deliveries, maternal deaths at 6 months and 2 years after delivery and perinatal deaths. Breast-feeding was addressed. Measures to assess the severity of maternal disease included the mean age at diagnosis of CF, weight gain during pregnancy, pulmonary function studies if available and the need for pancreatic enzyme replacement therapy. DATA SYNTHESIS: Of 217 pregnancies in 162 women spontaneous abortion occurred in 10 (4.6%). Pregnancy progressed beyond 20 weeks in 81.6% of cases; 24.3% of the deliveries were preterm. The maternal death rate did not exceed that among age-related women with CF who were not pregnant. The rate of perinatal death was 7.9%. Breast milk was not hypernatremic. Poor outcomes were associated with a weight gain of less than 4.5 kg and a forced vital capacity of less than 50% of the predicted value. CONCLUSIONS: Premature labour and delivery remain a significant risk for pregnant women with CF, contributing to a high rate of perinatal death. Maternal illness and death result from deteriorating pulmonary function. Breast-feeding is not contraindicated. Attention to energy intake and pulmonary function is important.

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Selected References

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  1. Alpert S. E., Cormier A. D. Normal electrolyte and protein content in milk from mothers with cystic fibrosis: an explanation for the initial report of elevated milk sodium concentration. J Pediatr. 1983 Jan;102(1):77–80. doi: 10.1016/s0022-3476(83)80293-5. [DOI] [PubMed] [Google Scholar]
  2. Arboit J. M., Gildengers E. Breast-feeding and hypernatremia. J Pediatr. 1980 Aug;97(2):335–336. doi: 10.1016/s0022-3476(80)80522-1. [DOI] [PubMed] [Google Scholar]
  3. Bitman J., Hamosh M., Wood D. L., Freed L. M., Hamosh P. Lipid composition of milk from mothers with cystic fibrosis. Pediatrics. 1987 Dec;80(6):927–932. [PubMed] [Google Scholar]
  4. Canny G. J., Corey M., Livingstone R. A., Carpenter S., Green L., Levison H. Pregnancy and cystic fibrosis. Obstet Gynecol. 1991 Jun;77(6):850–853. [PubMed] [Google Scholar]
  5. Cohen L. F., di Sant'Agnese P. A., Friedlander J. Cystic fibrosis and pregnancy. A national survey. Lancet. 1980 Oct 18;2(8199):842–844. doi: 10.1016/s0140-6736(80)90183-x. [DOI] [PubMed] [Google Scholar]
  6. Cole B. N., Seltzer M. H., Kassabian J., Abboud S. E. Parenteral nutrition in a pregnant cystic fibrosis patient. JPEN J Parenter Enteral Nutr. 1987 Mar-Apr;11(2):205–207. doi: 10.1177/0148607187011002205. [DOI] [PubMed] [Google Scholar]
  7. Corkey C. W., Newth C. J., Corey M., Levison H. Pregnancy in cystic fibrosis: a better prognosis in patients with pancreatic function? Am J Obstet Gynecol. 1981 Aug 1;140(7):737–742. doi: 10.1016/0002-9378(81)90732-8. [DOI] [PubMed] [Google Scholar]
  8. Friedman A. J., Haseltine F. P., Berkowitz R. L. Pregnancy in a patient with cystic fibrosis and idiopathic thrombocytopenic purpura. Obstet Gynecol. 1980 Apr;55(4):511–514. [PubMed] [Google Scholar]
  9. Hyde N. H., Harrison D. M. Intrathecal morphine in a parturient with cystic fibrosis. Anesth Analg. 1986 Dec;65(12):1357–1358. [PubMed] [Google Scholar]
  10. Larsen J. W., Jr Cystic fibrosis and pregnancy. Obstet Gynecol. 1972 Jun;39(6):880–883. [PubMed] [Google Scholar]
  11. Novy M. J., Tyler J. M., Shwachman H., Easterday C. L., Reid D. E. Cystic fibrosis and pregnancy. Report of a case, with a study of pulmonary function and arterial blood gases. Obstet Gynecol. 1967 Oct;30(4):530–536. [PubMed] [Google Scholar]
  12. Palmer J., Dillon-Baker C., Tecklin J. S., Wolfson B., Rosenberg B., Burroughs B., Holsclaw D. S., Jr, Scanlin T. F., Huang N. N., Sewell E. M. Pregnancy in patients with cystic fibrosis. Ann Intern Med. 1983 Nov;99(5):596–600. doi: 10.7326/0003-4819-99-5-596. [DOI] [PubMed] [Google Scholar]
  13. Pittard W. B., 3rd, Sorensen R. U., Schnatz P. T. Pregnancy outcome in mothers with cystic fibrosis: normal neonatal immune responses. South Med J. 1987 Mar;80(3):344–346. doi: 10.1097/00007611-198703000-00017. [DOI] [PubMed] [Google Scholar]
  14. Plotz E. J., Patterson P. R., Streit J. H. Pregnancy in a patient with cystic fibrosis (mucoviscidosis) and diabetes mellitus. Am J Obstet Gynecol. 1967 Aug 15;98(8):1105–1110. doi: 10.1016/0002-9378(67)90036-1. [DOI] [PubMed] [Google Scholar]
  15. Rosenow E. C., 3rd, Lee R. A. Cystic fibrosis and pregnancy. JAMA. 1968 Jan 15;203(3):227–229. [PubMed] [Google Scholar]
  16. Shiffman M. L., Seale T. W., Flux M., Rennert O. R., Swender P. T. Breast-milk composition in women with cystic fibrosis: report of two cases and a review of the literature. Am J Clin Nutr. 1989 Apr;49(4):612–617. doi: 10.1093/ajcn/49.4.612. [DOI] [PubMed] [Google Scholar]
  17. Valenzuela G. J., Comunale F. L., Davidson B. H., Dooley R. R., Foster T. C. Clinical management of patients with cystic fibrosis and pulmonary insufficiency. Am J Obstet Gynecol. 1988 Nov;159(5):1181–1183. doi: 10.1016/0002-9378(88)90441-3. [DOI] [PubMed] [Google Scholar]
  18. Welch M. J., Phelps D. L., Osher A. B. Breast-feeding by a mother with cystic fibrosis. Pediatrics. 1981 May;67(5):664–666. [PubMed] [Google Scholar]
  19. Whitelaw A., Butterfield A. High breast-milk sodium in cystic fibrosis. Lancet. 1977 Dec 17;2(8051):1288–1288. doi: 10.1016/s0140-6736(77)92697-6. [DOI] [PubMed] [Google Scholar]

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