Objective: There is a paucity of knowledge about long-term outcome issues in the bladder-exstrophy-epispadias complex (BEEC). Adult male BEEC patients were investigated in respect of bladder and renal function, fertility, genital function and psychosocial facts.
Patients And Methods: In a cross-sectional study, 17 adult male BEEC patients (mean age 23.
Objectives: Genital and reproductive function have a strong effect on the quality of life of adolescent and adult male patients with the exstrophy-epispadias complex (EEC). According to the limited available data, early exstrophy reconstruction, as well as recurrent infectious or operative trauma, have been responsible for the low fertility rates.
Methods: We evaluated 21 adult male patients with EEC.
Objective: To evaluate the risk of testicular damage from testicular biopsies that are carried out for testicular sperm extraction (TESE) in infertile men.
Design: Prospective controlled clinical study.
Setting: Academic hospital.
Steroid 5alpha-reductase deficiency is a rare autosomal recessive disorder caused by mutations in the SRD5A2-gene, resulting in diminished dihydrotestosterone (DHT) formation and, hence, in a severe virilization deficit of the external genitalia in patients with 46,XY karyotype. The phenotype of affected individuals is variable and has been reported to range from completely female over genital ambiguity to normal male, depending on the type of mutation and its effect on enzyme activity. Here we report an adolescent 46,XY patient with predominantly female appearance, who had been gonadectomized in early infancy.
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