Z Geburtshilfe Neonatol 2015; 219(04): 170-175
DOI: 10.1055/s-0035-1545341
Übersicht
© Georg Thieme Verlag KG Stuttgart · New York

Schwangerschaft bei Mukoviszidose – ein Überblick

Pregnancy and Cystic Fibrosis – An Overview
R. K. Michl*
1   Mukoviszidoszentrum für Kinder und Erwachsene, Klinik für Kinderheilkunde und Jugendmedizin, Univeritätsklinikum Jena, Jena
,
S. Mues*
2   Abteilung für Geburtshilfe, Universitätsklinikum Jena, Jena
,
J. G. Mainz
1   Mukoviszidoszentrum für Kinder und Erwachsene, Klinik für Kinderheilkunde und Jugendmedizin, Univeritätsklinikum Jena, Jena
,
U. R. Markert
2   Abteilung für Geburtshilfe, Universitätsklinikum Jena, Jena
› Author Affiliations
Further Information

Publication History

eingereicht 16 July 2014

angenommen nach Überarbeitung04 February 2015

Publication Date:
03 June 2015 (online)

Zusammenfassung

Die Lebenserwartung von Mukoviszidose-Patienten stieg dank intensiver Forschung und Therapie in den letzten 4 Jahrzehnten vom Vorschulalter auf heute über 40 Jahre. Entsprechend nimmt auch die Anzahl von Schwangerschaften an Mukoviszidose erkrankter Frauen zu. Häufig ist für Betroffene die Einschätzung der Konsequenzen einer Schwangerschaft auf ihre Erkrankung und die Auswirkung ihrer Erkrankung auf das heranwachsende Kind nicht möglich. Die Schwangerschaft einer an Mukoviszidose erkrankten Frau mit schlechter Lungenfunktion, reduziertem BMI und Komorbiditäten zeigt erwartungsgemäß mehr Komplikationen als eine Schwangerschaft von Patientinnen ohne entsprechende Risikofaktoren. Generell sind Verläufe mit bestem Outcome für Mutter und Kind unter exakter Planung und engmaschiger multidisziplinärer Betreuung zu erreichen. Ziel dieser zusammenfassenden Arbeit soll ein Überblick über die Risiken und Optionen einer Schwangerschaft bei Mukoviszidosepatientinnen sein.

Abstract

Life expectancy and quality of life of cystic fibrosis (CF) patients have been steadily increasing for many decades, due to intensified therapy and research. Correspondingly, the number of pregnancies in women with CF rises. Often it is not possible for the patients to assess the consequences of pregnancy in terms of their disease and the impact of their disease on the growing child. A pre-existing poor lung function, low body mass index, CF-related diabetes, chronic microbial colonisation, and transplanted lungs are the main risk factors for complications during pregnancy in CF. Generally, the best outcome for mother and child can be reached under exact planning and meshed multidisciplinary care. The purpose of this summary is to give a practical review of the risks and options associated with pregnancy in CF patients.

* Die Autoren Michl und Mues haben gleichen Anteil an der Erstellung des Manuskripts.


 
  • Literatur

  • 1 Riordan JR, Rommens JM, Kerem B et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 1989; 245: 1066-1073
  • 2 Poulou M, Fylaktou I, Fotoulaki M et al. Cystic fibrosis genetic counseling difficulties due to the identification of novel mutations in the CFTR gene. Journal of cystic fibrosis: official journal of the European Cystic Fibrosis Society 2012; DOI: 10.1016/j.jcf.2012.01.004.
  • 3 Ballmann MaS Christina Hrsg. CF Manual. 2nd edition. Aufl: UNI-MED; 2008: 192
  • 4 Dinwiddie R. Anti-inflammatory therapy in cystic fibrosis. Journal of cystic fibrosis: official journal of the European Cystic Fibrosis Society 2005; 4 (Suppl. 02) 45-48
  • 5 Edenborough FP. Women with cystic fibrosis and their potential for reproduction. Thorax 2001; 56: 649-655
  • 6 Umlawska W, Sands D, Zielinska A. Age of menarche in girls with cystic fibrosis. Folia histochemica et cytobiologica/Polish Academy of Sciences, Polish Histochemical and Cytochemical Society 2010; 48: 185-190
  • 7 Johannesson M, Gottlieb C, Hjelte L. Delayed puberty in girls with cystic fibrosis despite good clinical status. Pediatrics 1997; 99: 29-34
  • 8 Neinstein LS, Stewart D, Wang CI et al. Menstrual dysfunction in cystic fibrosis. Journal of adolescent health care: official publication of the Society for Adolescent Medicine 1983; 4: 153-157
  • 9 Stead RJ, Hodson ME, Batten JC et al. Amenorrhoea in cystic fibrosis. Clinical endocrinology 1987; 26: 187-195
  • 10 Lyon A, Bilton D. Fertility issues in cystic fibrosis. Paediatric respiratory reviews 2002; 3: 236-240
  • 11 Oppenheimer EA, Case AL, Esterly JR et al. Cervical mucus in cystic fibrosis: a possible cause of infertility. American journal of obstetrics and gynecology 1970; 108: 673-674
  • 12 Schram CA, Stephenson AL, Hannam TG et al. Cystic fibrosis (cf) and ovarian reserve: A cross-sectional study examining serum anti-mullerian hormone (amh) in young women. Journal of cystic fibrosis: official journal of the European Cystic Fibrosis Society 2014; DOI: 10.1016/j.jcf.2014.09.008.
  • 13 Lissens W, Liebaers I. The genetics of male infertility in relation to cystic fibrosis. Bailliere's clinical obstetrics and gynaecology 1997; 11: 797-817
  • 14 Tsang A, Moriarty C, Towns S. Contraception, communication and counseling for sexuality and reproductive health in adolescents and young adults with CF. Paediatric respiratory reviews 2010; 11: 84-89
  • 15 Zentler-Munro PL. Cystic fibrosis – a gastroenterological cornucopia. Gut 1987; 28: 1531-1547
  • 16 Dowsett J. An overview of nutritional issues for the adult with cystic fibrosis. Nutrition 2000; 16: 566-570
  • 17 Wilson DC, Pencharz PB. Nutrition and cystic fibrosis. Nutrition 1998; 14: 792-795
  • 18 Whitty JE. Cystic fibrosis in pregnancy. Clinical obstetrics and gynecology 2010; 53: 369-376
  • 19 Matel JL. Nutritional management of cystic fibrosis. JPEN Journal of parenteral and enteral nutrition 2012; 36: 60S-67S
  • 20 Kondo A, Kamihira O, Ozawa H. Neural tube defects: prevalence, etiology and prevention. International journal of urology: official journal of the Japanese Urological Association 2009; 16: 49-57
  • 21 Bailey LB. New standard for dietary folate intake in pregnant women. The American journal of clinical nutrition 2000; 71: 1304S-1307S
  • 22 Borowitz D, Robinson KA, Rosenfeld M et al. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. The Journal of pediatrics 2009; 155: S73-S93
  • 23 Edenborough FP, Borgo G, Knoop C et al. Guidelines for the management of pregnancy in women with cystic fibrosis. Journal of cystic fibrosis: official journal of the European Cystic Fibrosis Society 2008; 7 (Suppl. 01) S2-S32
  • 24 Stephenson A, Brotherwood M, Robert R et al. Increased vitamin A and E levels in adult cystic fibrosis patients after lung transplantation. Transplantation 2005; 79: 613-615
  • 25 Sitrin MD, Lieberman F, Jensen WE et al. Vitamin E deficiency and neurologic disease in adults with cystic fibrosis. Annals of internal medicine 1987; 107: 51-54
  • 26 Bye AM, Muller DP, Wilson J et al. Symptomatic vitamin E deficiency in cystic fibrosis. Archives of disease in childhood 1985; 60: 162-164
  • 27 Carr SB, McBratney J. The role of vitamins in cystic fibrosis. Journal of the Royal Society of Medicine 2000; 93 (Suppl. 38) 14-19
  • 28 Davis PB, di Sant'Agnese PA. Diagnosis and treatment of cystic fibrosis. An update. Chest 1984; 85: 802-809
  • 29 Hubert D. [Cystic fibrosis in adults]. La Revue du praticien 2003; 53: 158-162
  • 30 Corey M, Gaskin K, Durie P et al. Improved prognosis in CF patients with normal fat absorption. Journal of pediatric gastroenterology and nutrition 1984; 3 (Suppl. 01) S99-S105
  • 31 Folkesson A, Jelsbak L, Yang L et al. Adaptation of Pseudomonas aeruginosa to the cystic fibrosis airway: an evolutionary perspective. Nature reviews Microbiology 2012; 10: 841-851
  • 32 Porter LA, Goldberg JB. Influence of Neutrophil Defects on Burkholderia cepacia Complex Pathogenesis. Frontiers in cellular and infection microbiology 2011; 1: 9
  • 33 Govan JR, Hughes JE, Vandamme P. Burkholderia cepacia: medical, taxonomic and ecological issues. Journal of medical microbiology 1996; 45: 395-407
  • 34 Horsley A, Jones AM. Antibiotic treatment for Burkholderia cepacia complex in people with cystic fibrosis experiencing a pulmonary exacerbation. Cochrane Database Syst Rev 2012; 10: CD009529
  • 35 Smyth A. Prophylactic antibiotics in cystic fibrosis: a conviction without evidence?. Pediatric pulmonology 2005; 40: 471-476
  • 36 Ewald N, Kaufmann C, Raspe A et al. Prevalence of diabetes mellitus secondary to pancreatic diseases (type 3c). Diabetes/metabolism research and reviews 2012; 28: 338-342
  • 37 Moran A, Brunzell C, Cohen RC et al. Clinical care guidelines for cystic fibrosis-related diabetes: a position statement of the American Diabetes Association and a clinical practice guideline of the Cystic Fibrosis Foundation, endorsed by the Pediatric Endocrine Society. Diabetes care 2010; 33: 2697-2708
  • 38 O'Riordan SM, Dattani MT, Hindmarsh PC. Cystic fibrosis-related diabetes in childhood. Hormone research in paediatrics 2010; 73: 15-24
  • 39 Lanng S. Glucose intolerance in cystic fibrosis patients. Paediatric respiratory reviews 2001; 2: 253-259
  • 40 Brennan AL, Geddes DM, Gyi KM et al. Clinical importance of cystic fibrosis-related diabetes. Journal of cystic fibrosis: official journal of the European Cystic Fibrosis Society 2004; 3: 209-222
  • 41 Curran DR, McArdle JR, Talwalkar JS. Diabetes mellitus and bone disease in cystic fibrosis. Seminars in respiratory and critical care medicine 2009; 30: 514-530
  • 42 Alicandro G, Battezzati PM, Battezzati A et al. Insulin secretion, nutritional status and respiratory function in cystic fibrosis patients with normal glucose tolerance. Clin Nutr 2012; 31: 118-123
  • 43 Lanng S, Thorsteinsson B, Nerup J et al. Influence of the development of diabetes mellitus on clinical status in patients with cystic fibrosis. European journal of pediatrics 1992; 151: 684-687
  • 44 Moran A, Pekow P, Grover P et al. Insulin therapy to improve BMI in cystic fibrosis-related diabetes without fasting hyperglycemia: results of the cystic fibrosis related diabetes therapy trial. Diabetes care 2009; 32: 1783-1788
  • 45 Bismuth E, Laborde K, Taupin P et al. Glucose tolerance and insulin secretion, morbidity, and death in patients with cystic fibrosis. The Journal of pediatrics 2008; 152: 540-545 545, e541
  • 46 Koch C, Rainisio M, Madessani U et al. Presence of cystic fibrosis-related diabetes mellitus is tightly linked to poor lung function in patients with cystic fibrosis: data from the European Epidemiologic Registry of Cystic Fibrosis. Pediatric pulmonology 2001; 32: 343-350
  • 47 Hardin DS, Rice J, Cohen RC et al. The metabolic effects of pregnancy in cystic fibrosis. Obstet Gynecol 2005; 106: 367-375
  • 48 Metzger BE, Lowe LP, Dyer AR et al. Hyperglycemia and adverse pregnancy outcomes. The New England journal of medicine 2008; 358: 1991-2002
  • 49 Kleinwechter H, Schafer-Graf U, Buhrer C et al. Gestational diabetes mellitus (GDM) diagnosis, therapy and follow-up care: Practice Guideline of the German Diabetes Association (DDG) and the German Association for Gynaecology and Obstetrics (DGGG). Experimental and clinical endocrinology & diabetes: official journal, German Society of Endocrinology [and] German Diabetes Association 2014; 122: 395-405
  • 50 Braun AT, Merlo CA. Cystic fibrosis lung transplantation. Current opinion in pulmonary medicine 2011; 17: 467-472
  • 51 Budev MM, Arroliga AC, Emery S. Exacerbation of underlying pulmonary disease in pregnancy. Critical care medicine 2005; 33: S313-S318
  • 52 Gyi KM, Hodson ME, Yacoub MY. Pregnancy in cystic fibrosis lung transplant recipients: case series and review. Journal of cystic fibrosis: official journal of the European Cystic Fibrosis Society 2006; 5: 171-175
  • 53 Kulak CA, Borba VZ, Kulak Jr. J et al. Osteoporosis after transplantation. Current osteoporosis reports 2012; 10: 48-55
  • 54 Aris RM, Neuringer IP, Weiner MA et al. Severe osteoporosis before and after lung transplantation. Chest 1996; 109: 1176-1183
  • 55 Kotloff RM, FitzSimmons SC, Fiel SB. Fertility and pregnancy in patients with cystic fibrosis. Clinics in chest medicine 1992; 13: 623-635
  • 56 Larsen Jr JW. Cystic fibrosis and pregnancy. Obstet Gynecol 1972; 39: 880-883
  • 57 Burden C, Ion R, Chung Y et al. Current pregnancy outcomes in women with cystic fibrosis. European journal of obstetrics, gynecology, and reproductive biology 2012; 164: 142-145
  • 58 Edenborough FP, Mackenzie WE, Stableforth DE. The outcome of 72 pregnancies in 55 women with cystic fibrosis in the United Kingdom 1977–1996. BJOG: an international journal of obstetrics and gynaecology 2000; 107: 254-261
  • 59 Odegaard I, Stray-Pedersen B, Hallberg K et al. Maternal and fetal morbidity in pregnancies of Norwegian and Swedish women with cystic fibrosis. Acta obstetricia et gynecologica Scandinavica 2002; 81: 698-705
  • 60 Huffmyer JL, Littlewood KE, Nemergut EC. Perioperative management of the adult with cystic fibrosis. Anesthesia and analgesia 2009; 109: 1949-1961
  • 61 Faden Y, Gruslin A. Vaginal delivery of a patient with severe cystic fibrosis. International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and Obstetrics 2005; 91: 256-257
  • 62 NIH Consensus conference. Optimal calcium intake. NIH Consensus Development Panel on Optimal Calcium Intake. JAMA: the journal of the American Medical Association 1994; 272: 1942-1948