Objective To analyze the clinical,endocrinal and genetic features of 46,XX male syndrome. Methods Retrospectively collect and analyze the clinical data of 4 cases of 46,XX male sex reversal syndrome. Results Four patients were all sociopsychologically males.Among them,2 cases had cryptorchidism,3 hypospadia and 1 severe chordee with short urethra.Neither ovary nor uterus was detected through B ultrasonography or surgical exploration.Assessment of serum sex hormone suggested hypergonadotropic hypogonadism.Genetic detection indicated 46,XX karyotype as normal female's type. Conclusions 46,XX male has nearly normal male phenotype otherwise with testes dysgenesis and most are infertile.The target of treatment is to correct the urinogenital malformation and to compensate hormone defect with androgen.

Objective To study the risk factors which lead to increase in the incidence rate of hypospadias. Methods A case-control design was used.The mothers of 210 cases and 420 controls were interviewed by telephone or face-to-face,and the questionnaires were answered.Thirty probable risk factors were investigated.Statistical analysis was performed using univariate and multivariate unconditional Logistic regression analyses for evaluation of the data. Results All boys with hypospadias in the case group were of Han nationality.Only 5 cases had positive family history.Odds ratio (OR) of the factors investigated were:pregnant age (OR,38.63),threatened abortion (OR,4.71),progestin (OR,3.64),estrogen (OR,5.1),pesticide (OR,2.39),organic solvents (OR,2.12),season of pregnancy (OR,1.86) and detergent (OR, 1.87).But OR of frequency of abortion,weeks of gestation and birth weight were less than 1.The results of other 17 investigated factors were negative. Conclusions Threatened abortion,nonfull-term fetus,low birth weight and some environmental endocrine-disturbing chemicals ( pesticide,detergent,organic solvents,progestin and estrogen) may be risk factors associated with hypospadias.These environmental endocrine-disturbing chemicals may increase the incidence rate of hypospadias.

Objective To study and analyze the cause, prevention and treatment for complications in patients with serious hypospadias repaired by one-stage urethroplasty. Methods From 1987 to 2002,275 patients with serious hypospadias were repaired by one-stage urethroplasty, there were 35 cases had complications. The classifications were penoscrotal 148 cases, scrotal 95 cases and perineal 32 cases. The lengths of new urethras were from 3.0 to 8.4 cm, the mean was (4.1?0.7) cm. Thirty-two cases were received endocrinotherapy before urethroplasty. Results The rate of complications was 12.7% for 1～3 years following survey. There were urethral fistulas 24 cases (8.7%),urethral strictures 6 (2.2%), diverticulums 3 (1.1%), chordees 2 (0.7%).The rate of urethral fistulas was the first and urethral stricture was the second, they were higher than those of other complications (P

Cryptorchidism is one of the most common congenital malformations in the urogenital system of boys.It can be an isolated disease,and may also be combined with other congenital malformations.It is a risk factor for testicular cancer and male infertility.Orchidopexy is the main treatment method.Although undergo early orchidopexy is becoming more common,there are still some infertility after adulthood.In this review,long-term appearance of the scrotum,fertility and malignancy after operation are reviewed.