Características clínicas y hormonales del síndrome de insensibilidad a andrógenos

Objetivo: Estudiar las caracteristicas clinicas y hormonales de pacientes portadores del sindrome de insensibilidad a androgenos. Material y Metodos: Se han estudiado 20 casos de pacientes portadores del sindrome de insensibilidad a androgenos atendidos en el servicio de Endocrinologia del Instituto Especializado de Salud del Nino. Se aplico los criterios de Quigley para evaluar el grado de ambiguedad sexual.Resultados: Se encontro 10/18 (55,55%) de casos de grado III, 4/18 (22,22%) de grado IV, 2/18 (11,11%) de grado V, 1/18 (5,55%) de grado VI y 1/18 (5,55%) de grado VII, estos dos ultimos grados corresponden al fenotipo femenino (SIA completo). En los pacientes con formas de SIA incompleto se encontro relacion T/DHT >17, lo que equivale a discreta falla en la 5α-reductasa, lo que no sucedio con los pacientes con SIA completo. Conclusiones: El sindrome de insensibilidad a androgenos por mostrar diversos grados de ambiguedad sexual amerita estudios geneticos, hormonales, diagnostico por imagenes y exploracion quirurgica que permitan una orientacion en la determinacion del genero. Objective: Assessment of the clinical and hormonal characteristics of patients with androgen insensitivity syndrome (AIS). Materials and methods: 20 cases of AIS were studied in the Endocrinology Unit of the Instituto Especializado del Salud del Nino (Institute of Child Health - Lima, Peru). Quigley criteria was used to assess ambiguous genitalia. Results: According this criteria the findings were the following: 10/18 (55,55%) of cases were stage III, 4/18 (22,22%) were stage IV, 2/18 (11,11%) were stage V, 1/18 (5,55%) were stage VI and 1/18 (5,55%) were stage VII. The last two stages had female phenotype (complete AIS). In patients with incomplete AIS the ratio testosterone/dihydrotestosterone was less than 17, showing a mild failure of 5α-reductasa. This finding was not present in the cases with complete AIS. Conclusions: Due to the different degrees of ambiguous genitalia, the patients with AIS need genetic, hormone, images studies and surgical exploration to have the necessary criteria to suggest the most adequate therapy for each case.