How to manage puberty and prevent fertility disorders in men with CAH?

Hedi L Claahsen-van der Grinten

doi:10.1016/j.ando.2022.04.005

How to manage puberty and prevent fertility disorders in men with CAH?

Ann Endocrinol (Paris). 2022 Jun;83(3):186-187. doi: 10.1016/j.ando.2022.04.005. Epub 2022 Apr 15.

Author

Hedi L Claahsen-van der Grinten¹

Affiliation

¹ Radboud University Medical Centre, Department of Pediatric Endocrinology., Nijmegen, The Netherlands. Electronic address: Hedi.Claahsen@radboudumc.nl.

PMID: 35436502
DOI: 10.1016/j.ando.2022.04.005

Abstract

Gonadal dysfunction is an adverse outcome in patients with congenital adrenal hyperplasia (CAH), which may become apparent already during puberty. In males, gonadal dysfunction can be caused by primary gonadal failure due to testicular adrenal rest tumours (TART), and by secondary gonadal failure due to poor hormonal control. Yearly evaluation for TART using ultrasonography is recommended from the start of puberty or even earlier when poor hormonal control is present. We recommend yearly evaluation of gonadal function by measuring LH, FSH, testosterone, and inhibin B. When TART is present, cryopreservation of semen should be considered as soon as possible.

Keywords: 21OH deficiency; Congenital adrenal hyperplasia; Testiculars adrenal rest tumors; hypogonadism; infertility.

MeSH terms

Adrenal Hyperplasia, Congenital* / complications
Adrenal Rest Tumor* / etiology
Adrenal Rest Tumor* / pathology
Fertility
Humans
Male
Puberty
Testicular Neoplasms* / etiology
Testicular Neoplasms* / pathology