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2020, Number 2

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Rev Cuba Endoc 2020; 31 (2)

The reproductive future of female-assigned patients with congenital adrenal hyperplasia

Espinosa RTM, Cruz LD, Agramonte MA, Vera PG, Domínguez AE

Language: Spanish
References: 48
Page: 1-18
PDF size: 359.53 Kb.


ABSTRACT

Introduction: Although in women with congenital adrenal hyperplasia, the achievement of normal gonadal function and fertility requires strict adherence to substitution treatment, this is not always sufficient and some degree of ovarian hyperandrogenism appears with a negative effect, which is evident since adolescence.
Objective: To characterize some factors related to sexual and reproductive health in patients with congenital adrenal hyperplasia and assigned as female.
Methods: A cross-sectional and observational-descriptive study was carried out, including all female-assigned patients with a diagnosis of congenital adrenal hyperplasia and who were treated at the Institute of Endocrinology from 2000 to 2019. The study explored demographic aspects, family history, as well as aspects related to sexual and reproductive health.
Results: The sample was made up of 47 patients, with current mean age of 14.76 ± 7.04 years and average age for starting treatment of 5.9 years. Predominance of classic clinical forms was verified in 25 patients (53.19 %), while 22 patients (46.80 %) presented nonclassical forms. Some degree of genital virilization manifested in 22 patients; of this group, 13 (59.1 %) had received genital surgery, four (8.5 %) received clitoroplasty at mean age of 2.8 ± 0.8 years, and nine (19, 1 %) received an approach combined with vaginoplasty. Of the 36 patients at reproductive age, 11 (37.9 %) reported having started sexual intercourse relations at an average age of 17.8 ± 3.9 years old.
Conclusions: It is important to consider that subfertility of women with congenital adrenal hyperplasia starts in the peripubertal years, a reason why it should be of permanent interest to the pediatric endocrinologist in order to improve their reproductive future.


REFERENCES

  1. Lekarev O, Lin-Su K, Vogiatzi M. Infertility and reproductive function in patients with congenital adrenal hyperplasia: pathophysiology, advances in management, and recent outcomes. Endocrinol. Metab. Clin. North Am. 2015;44(4):705-22.

  2. Witchel SF. Congenital Adrenal Hyperplasia. J. Pediatr. Adolesc. Gynecol. 2017; 30(5):520-34.

  3. Krone N, Wachter I, Stefanidou M, Roscher A, Schwarz P. Mothers with congenital adrenal hyperplasia and their children: outcome of pregnancy, birth and childhood. Clin. Endocrinolol. 2001;55(4):523-9.

  4. Weiss RV, Clapauch R. Female infertility of endocrine origin. Arq. Bras. Endocrinol. Metabol. 2014;58(2):144-52.

  5. Gastaud F, Bouvattier C, Duranteau L. Impaired sexual and reproductive outcomes in women with classical forms of congenital adrenal hyperplasia. J. Clin. Endocrinol. Metab. 2007;92(4):1391-6.

  6. Hagenfeldt K, Janson P, Holmdahl G. Fertility and pregnancy outcome in women with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Hum Reprod. 2008;23(7):1607-13.

  7. Kim JH, Choi JH, Kang E, Kim YM, Lee BH, Yoo HW. Long-term Consequences of Congenital Adrenal Hyperplasia due to Classic 21-hydroxylase Deficiency in Adolescents and Adults. Exp. Clin. Endocrinol. Diabetes. 2017; 125(3):196-201.

  8. Casteras A, Silva P De, Rumsby G, Conway GS. Reassessing fecundity in women with classical congenital adrenal hyperplasia (CAH): normal pregnancy rate but reduced fertility rate. Clin. Endocrinol. 2009;70(6):833-7.

  9. Slijper FM. Clitoral surgery and sexual outcome in intersex conditions. The Lancet. 2003;361(12):1236-7.

  10. Agramonte MA, Ledón L, Fabré RB, Espinosa RT, González FP, Carvajal MF, et al. Intersexualidad, necesidad del cambio en el paradigma de atención. Rev. Cubana Endocrinol. 2010;21(3):307-22.

  11. Agramonte MA, Ledón L, Fabré RB, Carvajal MF, Espinosa RT, González FP et al. Impacto sexual, psicosocial y quirúrgico de la cirugía genital en personas diagnosticadas de genitales ambiguos. Boletín CNSCS. 2011;(2). Disponible en: https://articulos.sld.cu/boletincnscs/archives/48

  12. Dickens BM. Management of intersex newborns: Legal and ethical developments. Int J Gynaecol Obstet. 2018;143(2):255-9.

  13. Nordenström A, Frisén L, Falhammar H, Filipsson H, Holmdahl G, Janson PO, et al. Sexual function and surgical outcome in women with CAH due to 21-hydroxylase deficiency. Clinical perspective and patient's perception. J. Clin. Endocrinol. Metab. 2010;85(8):3633-40.

  14. Wisnieswki AB, Migeon CJ, Malouf MA, Gearhart JP. Psychosexual outcome in women affected by congenital adrenal hyperplasia due to 21-hydroxylase deficiency. J Urol. 2004;171(6):497-501.

  15. Dittman RW, Kappes ME, Kappes MH. Sexual behavior in adolescent and adult females with congenital adrenal hyperplasia. Psychoneuroendocrinology. 1992;17(2):153-70.

  16. Agramonte Machado A, Espinosa Reyes T, Rodríguez Pérez M; Ledón Llanes L, González Fernández P, Carvajal Martínez F, et al. Psychosexual function of intersexed individuals with genital surgery: A Cuban study. En: Pereira H. Coming-out for LGBT, Psychology in the current international scenario. Lisboa. Editorial Henrique Pereira & Pedro Costa; 2013. p. 253-64.

  17. Defining sexual health. World Health Organization. Salud sexual. 2020. [actualizado: 02/10/2020; acceso: 02/10/2020]. Disponible en: Disponible en: https://www.who.int/reproductivehealth/topics/sexual_health/sh_definitions/en/

  18. World Health Organization. Infecciones de transmisión sexual. 2020. [actualizado: 02/10/2020; acceso: 02/10/2020]. Disponible en: Disponible en: https://www.who.int/topics/sexually_transmitted_infections/es/

  19. Institute of Isotopes. Testosterona RIA KIT. Budapest: IZOTOP; 2010.

  20. Witchel SF, Oberfield S, Rosenfield RL, Codner E, Bonny A, Ibanez L, et al. The Diagnosis of Polycystic Ovary Syndrome during Adolescence. Horm. Res. Paediatr. 2015;83:376-89. DOI: 10.1159/000375530

  21. Reichman DE, White PC, New MI, Rosenwaks Z. Fertility in patients with congenital adrenal hyperplasia. Fertil Steril. 2014;101(2):301-9.

  22. Larissa G. Gomes, Tania A. Bachega, Berenice B. Mendonca. Classic congenital adrenal hyperplasia and its impact on reproduction. Fertility and Sterility. 2019;111(1):7-12.

  23. Mulaikal RM, Migeon CJ, Rock JA. Fertility rates in female patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. N. Engl. J. Med. 1987;316(4):178-82.

  24. Han TS, Walker BR, Arlt W, Ross RJ. Treatment and health outcomes in adults with congenital adrenal hyperplasia. Nat. Rev. Endocrinol. 2014;10(2):115-24.

  25. Auchus RJ. Management considerations for the adult with congenital adrenal hyperplasia. Mol. Cell. Endocrinol. 2015;408:190-7.

  26. Zainuddin AA, Grover SR, Shamsuddin K, Mahdy AZ. Research on quality of life in female patients with congenital adrenal hyperplasia and issues in developing nations. J. Pediatr. Adolesc. Gynecol. 2013;26(6):296-304.

  27. Nermoen I, Husebye ES, Myhre AG, Løvås K. Classical congenital adrenal hyperplasia. Tidsskr Nor Laegeforen. 2017;137(7):540-3.

  28. Forest MG, Castro FL. Hiperplasia suprarrenal congénita. En: Pombo M. Tratado de Endocrinología Pediátrica, 3.ª Ed. Madrid: McGraw-Hill Interamericana; 2002. p. 970-1005.

  29. Gebara E, Fernández MA, Rojas E, Amina A, López MR. Hiperplasia suprarrenal congénita perdedora de sal en varones durante el período neonatal. ¿Es posible adelantarse a la emergencia metabólica? Arch. Argent. Pediatr. 2009;107(4):369-73.

  30. Cardosa MIR, Sotelo CN. Hiperplasia suprarrenal congénita: diagnóstico y tratamiento en 20 casos. Rev. Mex. Pediatr. 2007;74(6):251-6.

  31. Braga L, Lorenzo AJ, Tatsuo ES, Silva IN, Pippi Salle JL. Prospective evaluation of feminizing genitoplasty using partial urogenital sinus mobilization for Congenital Adrenal Hyperplasia. J. Urol. 2006;176:2199-2204.

  32. Creighton SM. Long-term outcome of feminization surgery: the London experience. BJU Int. 2004;93(3):144.

  33. Minto CL, Liao LM, Woodhouse CR, Ransley PG, Creighton SM. The effect of clitoral surgery on sexual outcome in individuals who have intersex conditions with ambiguous genitalia: a cross-sectional study. The Lancet. 2003;12(361):1252-7.

  34. Speiser PW, Arlt W, Richard J. Auchus RJ, Baskin SL, Conway SG, et al. An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2018;103(11):4043-88.

  35. Santana Pérez F, Ovies Carballo G, Verdeja Valdés Ol. Características de la primera relación sexual en adolescentes en Ciudad de la Habana. Rev Cubana Salud Pública. 2006 [acceso: 02/10/2020]; 32(3). Disponible en: Disponible en: http://scielo.sld.cu/scielo.php?script=sci_arttext&pid=S0864-34662006000300006&lng=es&nrm=iso

  36. Peláez MJ. Anticoncepción en los adolescentes y adultos jóvenes. En: Peláez MJ. Métodos anticonceptivos. Actualidad y perspectivas para el nuevo milenio. La Habana: Editorial Científico- Técnica; 2002. p. 54-83.

  37. Rodríguez GG. La anticoncepción en Cuba, historia y perspectivas de los individuos. Algunos apuntes. Rev Nov Pob. 2017 [acceso: 02/10/2020]; 13(26):213-8. Disponible en: Disponible en: http://scielo.sld.cu/scielo.php?script=sci_arttext&pid=S1817-40782017000200017&lng=es&nrm=iso

  38. Morán C, Azziz R, Carmina E. 21-Hydroxylase- deficient non clasical adrenal hyperplasia is a progressive disorder: a multicenter study. Am. J. Obstet. Gynecol. 2000;183(6):1468-74.

  39. Montilva M. Postergación de la maternidad de mujeres profesionales jóvenes en dos metrópolis latinoamericanas. Revista Internac Filosof Iberoameric Teor Soc. 2008;13(41):69-79.

  40. Fuentes A, Jesa C, Devoto L, Angarita B, Galleguillos A, Torres A, et al. Postergación de la maternidad en Chile: Una realidad oculta. Rev. Med. Chile. 2010;138:1240-5.

  41. García AI, Alemán MM. Riesgos del embarazo en la edad avanzada. Rev. Cubana Obstet. Ginecol. 2013;39(2):120-7.

  42. Schwitzgebel VM, Tyrrell JB, Fitzgerald P, Kaplan SL. Normal females' infants born of mothers with classic congenital adrenal hyperplasia due to 21- Hydroxylase deficiency. J Clin. Endocrinol. Metab. 1999;84:930-6.

  43. Labartaa, E. Belloa, M. Ruiz-Echarrib, C. Ruedab, P. Martulc, E. Mayayoa, et al. Estado en la edad adulta y propuesta de optimización terapéutica de la hiperplasia suprarrenal congénita. An. Pediatr. 2003;58(Supl 2):S12-S34.

  44. Carvajal F, Montesinos T, Espinosa T, Navarrete CJ, Pérez GC. Forma no clásica de hiperplasia adrenal congénita en la niñez y adolescencia. Rev. Cubana Endocrinol. 2010;21(1):62-73.

  45. Stikkelbroeck N, Hermus A, Braat D, Otten B. Fertility in women with congenital adrenal hyperplasia due to 21-hydroxylase defficiency. Obstet. Gynecol. Surv. 2003;58(4):275-84.

  46. Kim JH, Choi JH, Kang E, Kim YM, Lee BH, Yoo HW. Long-term Consequences of Congenital Adrenal Hyperplasia due to Classic 21-hydroxylase Deficiency in Adolescents and Adults. Exp. Clin. Endocrinol Diabetes. 2017;125(3):196-201.

  47. Jääskeläinen J, Hippeläinen M, Kiekara O, Voutilainen R. Child rate, pregnancy outcome and ovarian function in females with classical 21-hydroxylase deficiency. Acta Obstet. Gynecol. Scand. 2000;79(8):687-92.

  48. Sowikowska-Hilczer J, Hirschberg AL, Claahsen-van der Grinten H, Reisch N, Bouvattier C, Thyen U, et al. Fertility outcome and information on fertility issues in individuals with different forms of disorders of sex development: findings from the dsd-LIFE study. Fertil Steril. 2017;108(5):822-31.




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