Your browser doesn't support javascript.
loading
Surgical Therapy of 17α-hydroxylase Deficiency in 30 Patients / 中国医学科学院学报
Artigo em Inglês | WPRIM (Pacífico Ocidental) | ID: wpr-277940
Biblioteca responsável: WPRO
ABSTRACT
Objective To analyze the clinical features of 17α-hydroxylase deficiency and explore the appropriate timing and methods of surgical treatment. Methods We retrospectively analyzed the clinical data of patients with complete 17α-hydroxylase deficiency,containing Y chromosome material in their karyotype,adimitted to Peking Union Medical College Hospital from January 2004 to December 2014. Results Thirty patients with complete 17α-hydroxylase deficiency were included. Their social gender were all female and the mean age at diagnosis was (16.1±2.7) years. Twenty-six patients (86.7%) presented with primary amenorrhea and hypertension. The development of secondary sexual characteristics was poor and their uterus was absent. The levels of gonadotropin,progesterone,and adrenocorticotropic hormone were elevated in all patients and the levels of estradiol,testosterone,and cortisol were decreased. All patients had undergone laparoscopic gonadectomy. Most (86.7%) of the gonads were located in abdomen,while 13.3% were in inguinal canal. Histopathology confirmed that gonadal malignancy was obsetved in two patients (6.7%) one with leydig cell tumor and the other with sertoli cell tumor. Conclusions Patients with complete 17α-hydroxylase deficiency have specific clinical features. Early diagnosis and timely laparoscopic gonadectomy are critical to prevent gonadal malignancy.
Assuntos
Texto completo: Disponível Base de dados: WPRIM (Pacífico Ocidental) Assunto principal: Cirurgia Geral / Esteroide 17-alfa-Hidroxilase / Estudos Retrospectivos / Hiperplasia Suprarrenal Congênita / Amenorreia / Hipertensão / Cariotipagem Tipo de estudo: Estudo observacional / Estudo de rastreamento Limite: Adolescente / Feminino / Humanos Idioma: Inglês Revista: Acta Academiae Medicinae Sinicae Ano de publicação: 2016 Tipo de documento: Artigo
Texto completo: Disponível Base de dados: WPRIM (Pacífico Ocidental) Assunto principal: Cirurgia Geral / Esteroide 17-alfa-Hidroxilase / Estudos Retrospectivos / Hiperplasia Suprarrenal Congênita / Amenorreia / Hipertensão / Cariotipagem Tipo de estudo: Estudo observacional / Estudo de rastreamento Limite: Adolescente / Feminino / Humanos Idioma: Inglês Revista: Acta Academiae Medicinae Sinicae Ano de publicação: 2016 Tipo de documento: Artigo
...