Download PDF
CC BY 4.0 · Rev Bras Ginecol Obstet 2019; 41(04): 230-235
DOI: 10.1055/s-0039-1678613
Original Article
Thieme Revinter Publicações Ltda Rio de Janeiro, Brazil

Maternal and Perinatal Outcomes in Pregnant Women with Cystic Fibrosis

Desfechos maternos e perinatais em gestantes portadoras de fibrose cística
Gilmar de Souza Osmundo Junior 
2   Faculty of Medicine, Division of Pneumology, Heart Institute, Hospital das Clínicas da Universidade de São Paulo, SP, Brazil
,
Rodrigo Abensur Athanazio
2   Faculty of Medicine, Division of Pneumology, Heart Institute, Hospital das Clínicas da Universidade de São Paulo, SP, Brazil
,
Samia Zahi Rached
1   Faculty of Medicine, Division of Obstetrics, Department of Obstetrics and Gynecology, Hospital das Clínicas da Universidade de São Paulo, SP, Brazil
,
Rossana Pulcineli Vieira Francisco
1   Faculty of Medicine, Division of Obstetrics, Department of Obstetrics and Gynecology, Hospital das Clínicas da Universidade de São Paulo, SP, Brazil
› Author Affiliations
Further Information

Publication History

06 October 2018

08 January 2019

Publication Date:
07 March 2019 (online)

   Permissions and Reprints

Abstract

Objectives To assess the perinatal and maternal outcomes of pregnant women with cystic fibrosis (CF) and severe lung impairment.

Methods This was a series of cases aiming to review the maternal and fetal outcomes in cases of singleton pregnant women with a diagnosis of CF. We have included all of the cases of singleton pregnancy in patients with CF who were followed-up at the obstetrics department of the Medical School of the Universidade de São Paulo, between 2003 and 2016. The exclusion criteria were the unattainability of the medical records of the patient, and delivery at other institutions. A forced expiratory volume in 1 second < 50% was considered as severe lung impairment. We have also analyzed data regarding maternal hospitalization and respiratory exacerbations (REs).

Results Pregnant women with CF and severe lung impairment did not present an association with spontaneous prematurity, fetal growth restriction or fetal demise. All of the cases involved multiple RE episodes requiring antibiotic therapy. The median (range) of events per patient was of 4 (2–4) events.

Conclusion Cystic fibrosis patients with severe lung impairment may achieve successful term pregnancies. However, pregnancies of women with CF are frequently complicated by REs, and this population may require hospital admission during the course of the pregnancy. Cystic fibrosis patients should be followed by a specialized team with experience in treating respiratory diseases.

Resumo

Objetivo Avaliar os desfechos maternos e perinatais de gestações em mulheres portadoras de fibrose cística (FC) e disfunção pulmonar grave.

Métodos Série de casos visando a avaliação dos desfechos maternos e perinatais em gestações únicas de mulheres com diagnóstico de FC. Foram incluídos todos os casos de gestações únicas de pacientes com FC que tiveram acompanhamento no departamento de obstetrícia da Faculdade de Medicina da Universidade de São Paulo, no período de 2003 a 2016. Os critérios de exclusão foram não disponibilidade do prontuário da paciente e parto em outro serviço. Disfunção pulmonar grave foi definida como presença de volume expiratório forçado em 1 segundo < 50%. Foram analisados também os dados referentes a exacerbações respiratórias e internações maternas.

Resultados Gestação em mulheres portadoras de FC com disfunção pulmonar grave não se associaram com prematuridade espontânea, restrição de crescimento fetal ou óbito fetal. Todos os casos apresentaram múltiplos episódios de exacerbações respiratórias necessitando de antibioticoterapia. A mediana de eventos por pacientes (intervalo) foi de 4 (2–4) eventos.

Conclusão Mulheres portadoras de FC com disfunção pulmonar grave podem evoluir com gestações de termo bem sucedidas. Entretanto, gestações nestas pacientes são frequentemente complicadas por exacerbações respiratórias, necessitando de internação. Gestantes portadoras de FC devem ser acompanhadas por uma equipe especializada com experiência no manejo de doenças respiratórias.

Keywords

cystic fibrosis - pregnancy complications - respiratory infections

Palavras-chave

fibrose cística - complicações na gravidez - infecções respiratórias

Contributors

Osmundo Junior G. S., Athanazio R. A., Rached S. Z. and Francisco R. P. V. contributed with the project and the interpretation of data, the writing of the article, the critical review of the intellectual content and with the final approval of the version to be published.


 

  • References

  • 1 Rosenstein BJ, Cutting GR. ; Cystic Fibrosis Foundation Consensus Panel. The diagnosis of cystic fibrosis: a consensus statement. J Pediatr 1998; 132 (04) 589-595 . Doi: 10.1016/S0022-3476(98)70344-0
    CrossrefPubMedGoogle Scholar
  • 2 Raskin S, Pereira-Ferrari L, Reis FC. , et al. Incidence of cystic fibrosis in five different states of Brazil as determined by screening of p.F508del, mutation at the CFTR gene in newborns and patients. J Cyst Fibros 2008; 7 (01) 15-22 . Doi: 10.1016/j.jcf.2007.03.006
    CrossrefPubMedGoogle Scholar
  • 3 Rowe SM, Miller S, Sorscher EJ. Cystic fibrosis. N Engl J Med 2005; 352 (19) 1992-2001 . Doi: 10.1056/NEJMra043184
    CrossrefPubMedGoogle Scholar
  • 4 Ratjen F, Döring G. Cystic fibrosis. Lancet 2003; 361 (9358): 681-689 . Doi: 10.1016/S0140-6736(03)12567-6
    CrossrefPubMedGoogle Scholar
  • 5 Cystic Fibrosis Foundation. Patient Registry 2015: Annual Data Report . Bethesda, MD: Cystic Fibrosis Foundation; 2016
    Google Scholar
  • 6 Dodge JA, Lewis PA, Stanton M, Wilsher J. Cystic fibrosis mortality and survival in the UK: 1947-2003. Eur Respir J 2007; 29 (03) 522-526 . Doi: 10.1183/09031936.00099506
    CrossrefPubMedGoogle Scholar
  • 7 Fair A, Griffiths K, Osman LM. . The Collaborative Group of Scottish Adult CF Centres Attitudes to fertility issues among adults with cystic fibrosis in Scotland. Thorax 2000; 55 (08) 672-677 . Doi: 10.1136/thorax.55.8.672
    CrossrefPubMedGoogle Scholar
  • 8 Siegel B, Siegel S. Pregnancy and delivery in a patient with cystic fibrosis of the pancreas. Obstet Gynecol 1960; 16: 438-440
    PubMedGoogle Scholar
  • 9 Thorpe-Beeston JG, Madge S, Gyi K, Hodson M, Bilton D. The outcome of pregnancies in women with cystic fibrosis–single centre experience 1998-2011. BJOG 2013; 120 (03) 354-361 . Doi: 10.1111/1471-0528.12040
    CrossrefPubMedGoogle Scholar
  • 10 Gilljam M, Antoniou M, Shin J, Dupuis A, Corey M, Tullis DE. Pregnancy in cystic fibrosis. Fetal and maternal outcome. Chest 2000; 118 (01) 85-91 . Doi: 10.1378/chest.118.1.85
    CrossrefPubMedGoogle Scholar
  • 11 Cohen LF, di Sant'Agnese PA, Friedlander J. Cystic fibrosis and pregnancy. A national survey. Lancet 1980; 2 (8199): 842-844 . Doi: 10.1016/S0140-6736(80)90183-X
    CrossrefPubMedGoogle Scholar
  • 12 Goss CH, Rubenfeld GD, Otto K, Aitken ML. The effect of pregnancy on survival in women with cystic fibrosis. Chest 2003; 124 (04) 1460-1468 . Doi: 10.1378/chest.124.4.1460
    CrossrefPubMedGoogle Scholar
  • 13 Girault A, Blanc J, Gayet V, Goffinet F, Hubert D. Maternal and perinatal outcomes of pregnancies in women with cystic fibrosis--A single centre case-control study. Respir Med 2016; 113: 22-27 . Doi: 10.1016/j.rmed.2016.02.010
    CrossrefPubMedGoogle Scholar
  • 14 Cheng EY, Goss CH, McKone EF. , et al. Aggressive prenatal care results in successful fetal outcomes in CF women. J Cyst Fibros 2006; 5 (02) 85-91 . Doi: 10.1016/j.jcf.2006.01.002
    CrossrefPubMedGoogle Scholar
  • 15 Tonelli MR, Aitken ML. Pregnancy in cystic fibrosis. Curr Opin Pulm Med 2007; 13 (06) 537-540 . Doi: 10.1097/MCP.0b013e3282f01120
    CrossrefPubMedGoogle Scholar
  • 16 Salvatore D, Buzzetti R, Baldo E. , et al. An overview of international literature from cystic fibrosis registries. Part 4: update 2011. J Cyst Fibros 2012; 11 (06) 480-493 . Doi: 10.1016/j.jcf.2012.07.005
    CrossrefPubMedGoogle Scholar
  • 17 Larsen Jr JW. Cystic fibrosis and pregnancy. Obstet Gynecol 1972; 39 (06) 880-883
    PubMedGoogle Scholar
  • 18 Edenborough FP, Borgo G, Knoop C. , et al; European Cystic Fibrosis Society. Guidelines for the management of pregnancy in women with cystic fibrosis. J Cyst Fibros 2008; 7 (Suppl. 01) S2-S32 . Doi: 10.1016/j.jcf.2007.10.001
    CrossrefPubMedGoogle Scholar
  • 19 Burden C, Ion R, Chung Y, Henry A, Downey DG, Trinder J. Current pregnancy outcomes in women with cystic fibrosis. Eur J Obstet Gynecol Reprod Biol 2012; 164 (02) 142-145 . Doi: 10.1016/j.ejogrb.2012.06.013
    CrossrefPubMedGoogle Scholar
  • 20 McMullen AH, Pasta DJ, Frederick PD. , et al. Impact of pregnancy on women with cystic fibrosis. Chest 2006; 129 (03) 706-711 . Doi: 10.1378/chest.129.3.706
    CrossrefPubMedGoogle Scholar
  • 21 Gillet D, de Braekeleer M, Bellis G, Durieu I. ; French Cystic Fibrosis Registry. Cystic fibrosis and pregnancy. Report from French data (1980-1999). BJOG 2002; 109 (08) 912-918 . Doi: 10.1111/j.1471-0528.2002.01511.x
    PubMedGoogle Scholar
  • 22 Moser C, Johannesson M, Song ZJ, Hougen HP, Kharazmi A, Høiby N. Altered cytokine response and increased degree of lung pathology in pregnant C3HIHeN mice with chronic Pseudomonas aeruginosa lung infection. Neth J Med 1999; 54: S70-S71 . Doi: 10.1016/S0300-2977(99)90236-X
    CrossrefPubMedGoogle Scholar
  • 23 Thompson LP, Pence L, Pinkas G, Song H, Telugu BP. Placental hypoxia during early pregnancy causes maternal hypertension and placental insufficiency in the hypoxic guinea pig model. Biol Reprod 2016; 95 (06) 128 . Doi: 10.1095/biolreprod.116.142273
    CrossrefPubMedGoogle Scholar
  • 24 Hegewald MJ, Crapo RO. Respiratory physiology in pregnancy. Clin Chest Med 2011; 32 (01) 1-13 . Doi: 10.1016/j.ccm.2010.11.001
    CrossrefPubMedGoogle Scholar
  • 25 Mighty HE. Acute respiratory failure in pregnancy. Clin Obstet Gynecol 2010; 53 (02) 360-368 . Doi: 10.1097/GRF.0b013e3181deb3f1
    CrossrefPubMedGoogle Scholar