RESUMEN
Congenital adrenal hyperplasia [CAH] is a rare congenital disorder, which in cases of female genotype may result in virilization. Specific enzyme deficiencies in adrenocorticoid hormones biosynthetic pathway lead to excess androgen production causing virilization. Classic type presents early in infant life as salt losing or simple virilizing type, whereas non classic form presents late at puberty or in adult life. Depending on the type of classic CAH, type of adrenocorticoid deficiency, extent of virilization and genotype, surgical corrective procedures, glucocorticoid and mineralocorticoid replacement therapy are the mainstay of management. We present here a case of classic congenital adrenal hyperplasia of simple virilizing type, which presented later in childhood
Asunto(s)
Humanos , Femenino , Virilismo , Trastornos del Desarrollo Sexual , Clítoris/patologíaRESUMEN
Rabson-Mendenhall syndrome is a rare genetic disorder characterized by growth retardation, dysmorphisms, lack of subcutaneous fat, acanthosis nigricans, enlarged genitalia, hirsutism, dysplastic dentition, coarse facial features, paradoxical fasting hypoglycemia, postprandial hyperglycemia, extreme hyperinsulinemia and pineal hyperplasia. Herein, we described a 10-year-old girl with physical features of the Rabson-Mendenhall syndrome that was presented with polyuria. To our knowledge, this is the first report of the Rabson-Mendenhall syndrome from Iran
Asunto(s)
Humanos , Femenino , Diabetes Mellitus , Resistencia a la Insulina , Hirsutismo , Clítoris/patología , PoliuriaRESUMEN
In this work, 57 cases of clitoridal cysts were studied for their relation to circumcision and their sexuality. Different pathogenetic factors were identified. Sexuality was assessed by sexual questionnaire sheet. The development of clitoridal cyst was associated with a compensatory increase in all sex scores, so as to reach nearly the total sexuality gain recorded for the uncircumcised controls. The development of clitorolabial orientation after its previous loss by circumcision explains the occurrence of this unique phenomenon of stimulated sex following the formation of such cysts