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Rev. peru. pediatr ; 61(1): 16-21, ene.-mar. 2008. ilus, tab
Article in Spanish | LILACS, LIPECS | ID: lil-503017

ABSTRACT

Objetivo: Estudiar las características clínicas y hormonales de pacientes portadores del síndrome de insensibilidad a andrógenos. Material y Métodos: Se han estudiado 20 casos de pacientes portadores del síndrome de insensibilidad a andrógenos atendidos en el servicio de Endocrinología del Instituto Especializado de Salud del Niño. Se aplicó los criterios de Quigley para evaluar el grado de ambigüedad sexual.Resultados: Se encontró 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 últimos grados corresponden al fenotipo femenino (SIA completo). En los pacientes con formas de SIA incompleto se encontró relación T/DHT >17, lo que equivale a discreta falla en la 5α-reductasa, lo que no sucedió con los pacientes con SIA completo. Conclusiones: El síndrome de insensibilidad a andrógenos por mostrar diversos grados de ambigüedad sexual amerita estudios genéticos, hormonales, diagnóstico por imágenes y exploración quirúrgica que permitan una orientación en la determinación del género.


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 Niño (Institute of Child Health - Lima, Perú). 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.


Subject(s)
Humans , Male , Female , Child , Androgen-Insensitivity Syndrome , Phenotype , Analytical Epidemiology , Retrospective Studies , Case-Control Studies
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