It is synonymous with the H-Y antigen.
It is synonymous with Sry in humans.
It is a zinc finger gene on the Y chromosome known as ZFY.
The expressed SRY protein has a characteristic highmobility group (HMG), DNA-binding domain.
It was genetically mapped by the study of patients with Klinefelter and Turner syndromes.
It acts systemically to produce müllerian regression.
It functions normally in patients with hernia uteri inguinale.
It is secreted by the fetal testis at 10 weeks of gestation, after testosterone production has begun.
It is secreted at 7 to 8 weeks of gestation, representing the initial endocrine function of the fetal testis.
It is secreted by the fetal Leydig cells.
Mutations in WT1 can result in either Denys-Drash or Frasier syndrome.
The gene was originally isolated in cloning experiments and localized to the X chromosome.
Duplication of WT1 has been associated with dosagesensitive sex reversal.
Loss of WT1 function has not been associated with genitourinary anomalies.
Mutations in WT1 are associated with adrenocortical carcinoma.
It enters target tissue by active diffusion.
It results in regression of the müllerian ducts.
It is produced primarily by the adrenal gland.
It acts locally to virilize the internal wolffian duct structures.
It acts locally to virilize the urogenital sinus and genital tubercle.
It produces virilization of wolffian duct structures.
It is converted by 5α-reductase to testosterone in target tissues.
It acts locally to produce regression of müllerian structures.
It is secreted in large quantities by the fetal testis.
It produces virilization of the urogenital sinus.
They are at increased risk for development of adenocarcinoma of the breast.
They undergo replacement of Leydig cells with hyaline.
They have at least one X and two Y chromosomes.
They are characteristically fertile.
They bear little resemblance to XX males.
has a reduced number of oocytes.
in the presence of a Y chromosome results in risk for development of gonadoblastoma.
can descend to the scrotum.
in the presence of a Y chromosome results in increased risk for development of seminoma.
is located in the round ligament.
They derive similar benefit from synthetic growth hormone as do patients with Turner syndrome.
They frequently have chromosomal anomalies.
They lack the somatic defects associated with Turner syndrome.
They have gonadal histology different from that of patients with Turner syndrome.
They are at lesser risk for gonadal tumors than are patients with Turner syndrome.
Progressive renal failure
Nephropathy with early-onset proteinuria
They have castrate testosterone and normal gonadotropin levels.
They have normal follicle-stimulating hormone but decreased luteinizing hormone levels.
They have castrate testosterone and elevated gonadotropin levels.
They have normal testosterone but decreased DHT levels.
They have normal testosterone and elevated estradiol levels.
It can be unilateral or bilateral.
It cannot descend from the retroperitoneum.
It has testicular and ovarian elements randomly distributed.
It is found in the minority of patients.
It is impossible to cleave surgically.
the impossibility of precisely dividing an ovotestis surgically.
that malignant degeneration of gonads does not occur.
the familial pattern of inheritance of the disorder.
the potential for fertility.
the unresponsiveness of the external genitalia to testosterone.
It occurs with a predictable phenotype.
It accounts for 99% of CAH cases.
It occurs as a result of gene inactivation in the majority of cases.
It occurs with simple virilization in 75% of cases and salt wasting in 25% of cases.
It is transmitted in an autosomal dominant pattern.
is initiated after a diagnosis of CAH is confirmed.
is of no risk to the fetus.
is appropriate therapy in seven of eight at-risk fetuses.
is demonstrated to be effective.
acts by suppressing maternal corticotropin.
They may be associated with fertility.
They appear clinically with a predictable phenotype.
They are associated with persistent müllerian structures.
They are transmitted in an autosomal dominant pattern.
They may involve impaired glucocorticoid and mineralocorticoid synthesis.
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