Puberty in Patients with Ovotesticular DSD: Evaluation of 20 Patients and Review of the Literature.
Pediatr Endocrinol Rev
; 17(3): 243-249, 2020 Jun.
Article
em En
| MEDLINE
| ID: mdl-32741155
ABSTRACT
BACKGROUND:
Ovotesticular Difference of Sex Development (OT DSD) is a rare condition characterized by histologic demonstration of ovarian and testicular tissue in the same individual. Descriptions in literature usually do not include long term follow-up data.OBJECTIVES:
The aim of this study is to describe clinical, biochemical and histological findings, as well as long term outcomes (including onset and progression of puberty) in patients with OT DSD.RESULTS:
In a retrospective study of 31 patients, findings include predominantly male gender assignment at the time of referral (54.8%) and subsequent female gender of rearing (54.8%). The most frequent karyotype was 46,XX (58.1%). Ovotestis was the most frequent gonad (48.4%) Puberty could be evaluated in 20 patients, being spontaneous in 12 of them. Four patients with partial gonadectomy in infancy were able to enter female puberty spontaneously.CONCLUSION:
It was observed that patients who preserved gonadal tissues were able to enter puberty spontaneously.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Transtornos do Desenvolvimento Sexual
/
Transtornos Ovotesticulares do Desenvolvimento Sexual
Tipo de estudo:
Observational_studies
Limite:
Female
/
Humans
/
Male
Idioma:
En
Revista:
Pediatr Endocrinol Rev
Assunto da revista:
ENDOCRINOLOGIA
/
PEDIATRIA
Ano de publicação:
2020
Tipo de documento:
Article