Anomalie de developpement sexuel : Un cas de pseudohermaphrodisme feminin ou anomalie du developpement sexuel XX

Abstract

Lepseudohermaphrodisme feminin (PHF) ou anomalie du developpement sexuel XX, etat intersexue rare, represente lfaffection la plus frequente des anomalies du developpement sexuel. La cause la plus frequente est lfhyperplasie congenitale des surrenales. Nous rapportons dans ce present travail un cas de pseudohermaphrodisme feminin ou anomalie du developpement sexuel XX. Il sfest agi dfun patient, de genre masculin, recu en consultation a lfage de 14 ans pour cryptorchidie bilaterale confirmee a lfexamen clinique. Lfanalyse cytogenetique a indique un caryotype normal de type feminin. Le dosage hormonal sanguin a indique un bloc en 11ƒÀ-OH. Lfanalyse de cette observation releve la negligence ou lfignorance des parents face aux pathologies neonatales graves ou le pronostic vital de lfenfant nfest pas engage mais aux lourdes consequences pour lfinsertion de ces enfants dans la societe.

Mots cles : Pseudohermaphrodisme feminin, anomalie du developpement sexuel XX, caryotype, sexe social.

Anomaly of sexual development: a case of feminin pseudohermaphrodism or anomaly of the sexual development XX

The feminine pseudohermaphrodism (FPH) or anomaly of the sexual development XX, rare intersexual state, represents the most frequent affection of the anomalies of the sexual development. The most frequent reason is the congenital hyperplasia of the adrenal. We return in this present work a case of feminine pseudohermaphrodism or anomaly of the sexual development XX. It was about a patient, of masculine kind, received in consultation at the age of 14 years for bilateral cryptorchidism confirmed to the clinical exam. The cytogenetic analysis indicated a normal karyotype of feminine type. The blood hormonal dosage indicated a block in 11ƒÀ-OH. The analysis of this observation raises the carelessness or the ignorance of the parents facing the pathologies serious neonatal where the child's vital prognosis is not hired but to the heavy consequences for the insertion of these children in the society.

Key words : Feminine pseudohermaphrodism, anomaly of the sexual development XX, karyotype, social sex.