Skip to main content

Advertisement

SpringerLink
Log in

Successful management with C1-inhibitor concentrate of hereditary angioedema attacks during two successive pregnancies: a case report

  • Case Report
  • Published:
Archives of Gynecology and Obstetrics Aims and scope Submit manuscript

Abstract

Backgroud

Hereditary angioedema (HAE) is a rare genetic disorder caused by a deficiency of the plasma protein C1 inhibitor (C1-INH). HAE is characterised by the onset of angioedema, which may develop in one or several organs, and may last from a few hours to several days. Oedema of the upper airway can be life-threatening. As a result of hormonal changes, some women experience more frequent angioedema attacks during pregnancy. During pregnancy, antifibrinolytic agents should only be used with caution, and attenuated androgens are contraindicated; therefore, replacement therapy with C1-INH concentrate represents one of few therapeutic options, but it is not widely documented.

Case study

We report the first case study of the successful management with regular infusions of C1-INH concentrate, of two successive pregnancies in a patient with HAE. During the second half of the first pregnancy, C1-INH was administered on demand at home. For the second pregnancy, on demand treatment was intensified to prophylactic therapy, with once or twice weekly infusions from the middle of the second trimester in order to efficiently control the frequent attacks.

Conclusions

This report illustrates that HAE can be successfully managed during pregnancy with C1-INH infusions at home. Since the number of crises may vary between pregnancies, the treatment regimen must be adapted to the patient’s need.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Agostoni A, Cicardi M (1992) Hereditary and acquired C1-inhibitor deficiency: biological and clinical characteristics in 235 patients. Medicine (Baltimore) 71:206–215

    CAS  Google Scholar 

  2. Agostoni A, Aygoren-Pursun E, Binkley KE, Blanch A, Bork K, Bouillet L, Bucher C, Castaldo AJ, Cicardi M, Davis AE, De Carolis C, Drouet C, Duponchel C, Farkas H, Fay K, Fekete B, Fischer B, Fontana L, Fust G, Giacomelli R, Groner A, Hack CE, Harmat G, Jakenfelds J, Juers M, Kalmar L, Kaposi PN, Karadi I, Kitzinger A, Kollar T, Kreuz W, Lakatos P, Longhurst HJ, Lopez-Trascasa M, Martinez-Saguer I, Monnier N, Nagy I, Nemeth E, Nielsen EW, Nuijens JH, O’Grady C, Pappalardo E, Penna V, Perricone C, Perricone R, Rauch U, Roche O, Rusicke E, Spath PJ, Szendei G, Takacs E, Tordai A, Truedsson L, Varga L, Visy B, Williams K, Zanichelli A, Zingale L (2004) Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. J Allergy Clin Immunol 114:S51–S131

    Article  PubMed  Google Scholar 

  3. Altman AD, McLaughlin J, Schellenberg R, Penner C, Arbour L, Tsang P, Ballem P, Lim KI (2006) Hereditary angioedema managed with low-dose danazol and C1 esterase inhibitor concentrate: a case report. J Obstet Gynaecol Can 28:27–31

    PubMed  Google Scholar 

  4. Bockers M, Bork K (1987) Contraception and pregnancy in hereditary angioedema. Dtsch Med Wochenschr 112:507–509

    PubMed  CAS  Google Scholar 

  5. Bork K, Barnstedt SE (2001) Treatment of 193 episodes of laryngeal edema with C1 inhibitor concentrate in patients with hereditary angioedema. Arch Intern Med 161:714–718

    Article  PubMed  CAS  Google Scholar 

  6. Bork K, Siedlecki K, Bosch S, Schopf RE, Kreuz W (2000) Asphyxiation by laryngeal edema in patients with hereditary angioedema. Mayo Clin Proc 75:349–354

    Article  PubMed  CAS  Google Scholar 

  7. Bork K, Fischer B, Dewald G (2003a) Recurrent episodes of skin angioedema and severe attacks of abdominal pain induced by oral contraceptives or hormone replacement therapy. Am J Med 114:294–298

    Article  CAS  Google Scholar 

  8. Bork K, Hardt J, Schicketanz KH, Ressel N (2003b) Clinical studies of sudden upper airway obstruction in patients with hereditary angioedema due to C1 esterase inhibitor deficiency. Arch Intern Med 163:1229–1235

    Article  Google Scholar 

  9. Bork K, Meng G, Staubach P, Hardt J (2005) Treatment with C1 inhibitor concentrate in abdominal pain attacks of patients with hereditary angioedema. Transfusion 45:1774–1784

    Article  PubMed  CAS  Google Scholar 

  10. Bouillet L, Ponard D, Drouet C, Jullien D, Massot C (2003) Angioedema and oral contraception. Dermatology 206:106–109

    Article  PubMed  CAS  Google Scholar 

  11. Chappatte O, de Swiet M (1988) Hereditary angioneurotic oedema and pregnancy. Case reports and review of the literature. Br J Obstet Gynaecol 95:938–942

    PubMed  CAS  Google Scholar 

  12. Chhibber G, Cohen A, Lane S, Farber A, Meloni FJ, Schmaier AH (1990) Immunoblotting of plasma in a pregnant patient with hereditary angioedema. J Lab Clin Med 115:112–121

    PubMed  CAS  Google Scholar 

  13. Cicardi M, Bergamaschini L, Marasini B, Boccassini G, Tucci A, Agostoni A (1982) Hereditary angioedema: an appraisal of 104 cases. Am J Med Sci 284:2–9

    Article  PubMed  CAS  Google Scholar 

  14. Cox M, Holdcroft A (1995) Hereditary angioneurotic oedema: current management in pregnancy. Anaesthesia 50:547–549

    Article  PubMed  CAS  Google Scholar 

  15. Cunningham DS, Jensen JT (1991) Hereditary angioneurotic edema in the puerperium. A case report. J Reprod Med 36:312–313

    PubMed  CAS  Google Scholar 

  16. de Serres J, Groner A, Lindner J (2003) Safety and efficacy of pasteurized C1 inhibitor concentrate (Berinert P) in hereditary angioedema: a review. Transfus Apher Sci 29:247–254

    Article  PubMed  Google Scholar 

  17. Fay A, Abinun M (2002) Current management of hereditary angio-oedema (C’1 esterase inhibitor deficiency). J Clin Pathol 55:266–270

    PubMed  CAS  Google Scholar 

  18. Ferlazzo B, Barrile A, Bonanno D, Crisafi A, Ferrari U, Quattrocchi P, Sorge R (1990) Clinical contribution to the problem of correlations between hereditary angioneurotic edema and pregnancy. Minerva Ginecol 42:351–356

    PubMed  CAS  Google Scholar 

  19. Frank MM, Gelfand JA, Atkinson JP (1976) Hereditary angioedema: the clinical syndrome and its management. Ann Intern Med 84:580–593

    PubMed  CAS  Google Scholar 

  20. Galan HL, Reedy MB, Starr J, Knight AB (1996) Fresh frozen plasma prophylaxis for hereditary angioedema during pregnancy. A case report. J Reprod Med 41:541–544

    PubMed  CAS  Google Scholar 

  21. Gompels MM, Lock RJ, Morgan JE, Osborne J, Brown A, Virgo PF (2002) A multicentre evaluation of the diagnostic efficiency of serological investigations for C1 inhibitor deficiency. J Clin Pathol 55:145–147

    Article  PubMed  CAS  Google Scholar 

  22. Gompels MM, Lock RJ, Abinun M, Bethune CA, Davies G, Grattan C, Fay AC, Longhurst HJ, Morrison L, Price A, Price M, Watters D (2005) C1 inhibitor deficiency: consensus document. Clin Exp Immunol 139:379–394

    Article  PubMed  CAS  Google Scholar 

  23. Juers M, Gröner A (2004) Virus safety of current plasma-derived C1-INH: the Aventis Behring experience. J Allergy Clin Immunol 114:S96–S98

    Google Scholar 

  24. Karim Y, Griffiths H, Deacock S (2004) Normal complement C4 values do not exclude hereditary angioedema. J Clin Pathol 57:213–214

    Article  PubMed  CAS  Google Scholar 

  25. Klarmann D, Kreuz W, Joseph-Steiner J, Ehrenforth S (1996) Hepatitis C and pasteurized C1-inhibitor concentrate. Transfusion 36:84–85

    Article  PubMed  CAS  Google Scholar 

  26. Landres B (2001) Hereditary angioedema. Proc UCLA Healthc 5:25–28

    Google Scholar 

  27. Logan RA, Greaves MW (1984) Hereditary angio-oedema: treatment with C1 esterase inhibitor concentrate. J R Soc Med 77:1046–1048

    PubMed  CAS  Google Scholar 

  28. Longhurst HJ, Carr S, Khair K (2006) C1-inhibitor concentrate home therapy for hereditary angioedema: a viable, effective treatment option. Clin Exp Immunol 147:11–17

    Google Scholar 

  29. McGlinchey PG, McCluskey DR (2000) Hereditary angioedema precipitated by estrogen replacement therapy in a menopausal woman. Am J Med Sci 320:212–213

    Article  PubMed  CAS  Google Scholar 

  30. McGlinchey PG, Golchin K, McCluskey DR (2000) Life-threatening laryngeal oedema in a pregnant woman with hereditary angioedema. Ulster Med J 69:54–57

    PubMed  CAS  Google Scholar 

  31. Nathani F, Sullivan H, Churchill D (2006) Pregnancy and C1 esterase inhibitor deficiency: a successful outcome. Arch Gynecol Obstet 274:381–384

    Article  PubMed  Google Scholar 

  32. Nielsen EW, Gran JT, Straume B, Mellbye OJ, Johansen HT, Mollnes TE (1996) Hereditary angio-oedema: new clinical observations and autoimmune screening, complement and kallikrein-kinin analyses. J Intern Med 239:119–130

    Article  PubMed  CAS  Google Scholar 

  33. Perkins W, Downie I, Keefe M, Chisholm M (1995) Cutaneous necrosis in pregnancy secondary to activated protein C resistance in hereditary angioedema. J R Soc Med 88:229P–230P

    PubMed  CAS  Google Scholar 

  34. Rusicke E, Martinez-Saguer I, Aygoren-Pursun E, Kreuz W (2006) Home treatment in patients with hereditary angioedema (HAE). J Allergy Clin Immunol 117:S180

    Article  Google Scholar 

  35. van der Klooster JM, Schelfhout LJ, Grootendorst AF, Zweers PG (2002) Recurrent attacks of angioedema ascribed to the use of estrogen preparations and a pregnancy (hereditary angioedema type 3). Ned Tijdschr Geneeskd 146:1599–1602

    PubMed  Google Scholar 

  36. Visentin DE, Yang WH, Karsh J (1998) C1-esterase inhibitor transfusions in patients with hereditary angioedema. Ann Allergy Asthma Immunol 80:457–461

    Article  PubMed  CAS  Google Scholar 

  37. Visy B, Fust G, Varga L, Szendei G, Takacs E, Karadi I, Fekete B, Harmat G, Farkas H (2004) Sex hormones in hereditary angioneurotic oedema. Clin Endocrinol (Oxf) 60:508–515

    Article  CAS  Google Scholar 

  38. Warin RP, Cunliffe WJ, Greaves MW, Wallington TB (1986) Recurrent angioedema: familial and oestrogen-induced. Br J Dermatol 115:731–734

    Article  PubMed  CAS  Google Scholar 

  39. Winnewisser J, Rossi M, Spath P, Burgi H (1997) Type I hereditary angio-oedema. Variability of clinical presentation and course within two large kindreds. J Intern Med 241:39–46

    Article  PubMed  CAS  Google Scholar 

  40. Yip J, Cunliffe WJ (1992) Hormonally exacerbated hereditary angioedema. Australas J Dermatol 33:35–38

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgments

I am particularly grateful to my patient, who very kindly agreed to be the subject of this case report, and took great care and diligence in accurately recording the data under sometimes difficult circumstances.

Author information

Authors and Affiliations

  1. Haemostasis and Thrombosis Unit, Department of Haematology, Cliniques Universitaires Saint-Luc, Catholic University of Louvain, Avenue Hippocrate 10, 1200, Brussels, Belgium

    Cedric Hermans

Authors
  1. Cedric Hermans
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Cedric Hermans.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Hermans, C. Successful management with C1-inhibitor concentrate of hereditary angioedema attacks during two successive pregnancies: a case report. Arch Gynecol Obstet 276, 271–276 (2007). https://doi.org/10.1007/s00404-007-0329-1

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00404-007-0329-1

Keywords

Search

Navigation