EARLY CORRECTION OF HYPOSPADIAS IN GIRL WITH A DISORDER OF SEX DEVELOPMENT. CLINICAL CASE


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Resumo

The presented clinical case of a girl with a salt-wasting form of congenital adrenal hyperplasia in combination with chronic recurrent infection and lower urinary tract dysfunction demonstrates the need to change conventional two-staged approach to surgical feminization in favor of a one-stage intervention in order to prevent a progression of genitourinary complications. After controlling for the underlying condition, the one-stage feminization was performed, including modified tightening introitoplasty using a Passerini-Glazel flap and a correction of hypertrophic clitoris and labia minora. Good short- and long-term results were achieved.

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Sobre autores

A. Anikiev

FGBU National Medical Research Center of Endocrinology of the Ministry of Health of the Russian Federation

Email: anikieal70@gmail.com
Ph.D., urologist at the Department of Pediatric Surgery Moscow, Russia

N. Kalinchenko

FGBU National Medical Research Center of Endocrinology of the Ministry of Health of the Russian Federation

Email: kalinnat@rambler.ru
Ph.D., senior researcher, physician at the Department of Thyroidology, Reproductive and Somatic Development Moscow, Russia

E. Volod’ko

FGBOU DPO «Russian Medical Academy for Postgraduate Education» of the Ministry of Health of the Russian Federation

Email: okulov20@yandex.ru
MD, professor at the Department of Pediatric Surgery Moscow, Russia

D. Brovan

FGBU National Medical Research Center of Endocrinology of the Ministry of Health of the Russian Federation

Email: brovin-dn@yandex.ru
Ph.D., Head of the Department of Pediatric Surgery Moscow, Russia

A. Okulov

FGBOU DPO «Russian Medical Academy for Postgraduate Education» of the Ministry of Health of the Russian Federation

Email: okulov20@yandex.ru
MD, professor at the Department of Pediatric Surgery Moscow, Russia

O. Bezlepkina

FGBU National Medical Research Center of Endocrinology of the Ministry of Health of the Russian Federation

Email: olgabezlepkina@mail.ru
MD, professor, Deputy Director for clinical care and scientific work Moscow, Russia

Bibliografia

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  3. Aguirre J.M.E., Cadena Y., Lopez P-J., et.al. Feminizing genitoplasty in adrenal congenital hyperplasia: one or two surgical steps? Arch Esp Urol. 2009;62(9):724-730.
  4. Joint LWPES/ESPE CAH Working Group. Consensus Statement on 21-Hydroxylase Deficiency from The Lawson Wilkins Pediatric Endocrine Society and The European Society for Paediatric Endocrinology. The Journal of Clinical Endocrinology & Metabolism. 2002;87(9):4048-4053. doi: 10.1210/jc.2002-020611
  5. Yankovic F., Cherian A., Steven L., et.al. Current practice in feminizing surgery for congenital adrenal hyperplasia; a specialist survey. J Pediatr Urol. 2013;9(6):1103-1107. doi: 10.1016/j.jpurol.2013.03.013.
  6. Davies M.C., Crouch N.S., Woodhouse C.R., Creighton S.M. Congenital adrenal hyperplasia and lower urinary tract symptoms. BJU Int. 2005;95:1263-1266. doi: 10.1111/j.1464- 410X.2005.05516.x.
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  8. Hayashi G.Y., Carvalho D.F., de Miranda M.C., et.al. Neonatal 17-hydroxyprogesterone levels adjusted according to age at sample collection and birthweight improve the efficacy of congenital adrenal hyperplasia newborn screening. Clin Endocrinol (Oxf). 2017;86(4):480- 487. doi: 10.1111/cen.13292.

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