Prevalence of Testicular Feminisation

Jagiello, Georgiana; Atwell, J. D.

doi:10.1016/s0140-6736(62)91289-8

Cited by 84 publications

(21 citation statements)

References 6 publications

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“…Androgen insensitivity syndrome [AIS; testicular fem inization (TF)] is thought to be the most common form of male pseudothermaphroditism [1] and is caused by a defect in the androgen receptor system [2,3]. Testicular histology in adults with TF is comparable to long-standing cryptorchidism [4],…”

Section: Introductionmentioning

confidence: 99%

Gonadotropin Regulation during Puberty in Complete Androgen Insensitivity Syndrome with Testicles in situ

Schmitt¹,

Knorr²,

Schwarz³

et al. 1994

Horm Res

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This paper describes the time course of luteinizing hormone (LH) and follicle-stimulating hormone (FSH) levels in two patients with the complete androgen insensitivity syndrome (AIS) during puberty and adolescence prior to gonadectomy. In early puberty, LH values were comparable to normal male as well as female standards. At the age of 17 and 18.7 years, respectively, a sudden rise in LH occurred just after the start of estradiol supplementation. These high LH concentrations in the late puberty of our patients are comparable to the values found during early puberty in castrated individuals and children with gonadal dysgenesis. In contrast, FSH concentrations continuously showed levels in the normal male or female range with no rise during adolescence. The sudden increase of LH in late puberty is most probably due to defective testosterone receptors in the pituitary/hypothalamus which apparently can no longer be suppressed by rising testosterone levels and an escape from the negative estradiol feedback. The role of estradiol supplementation might be incidental and further investigation into its role is needed. In contrast to the elevated levels of FSH in patients with defective spermatogenesis, FSH is normal in our patients with AIS and testes in situ. This indicates that FSH secretion is regulated by a combined action of estradiol and gonadal hormones like inhibin.

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Section: Introductionmentioning

confidence: 99%

Gonadotropin Regulation during Puberty in Complete Androgen Insensitivity Syndrome with Testicles in situ

Schmitt¹,

Knorr²,

Schwarz³

et al. 1994

Horm Res

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“…At present it is generally agreed that the testis should be removed shortly after puberty, when feminization is complete. 13 Gans,11 however, advocates its removal at the time of the first surgery.…”

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confidence: 99%

Inguinal Hernias in Females and the Testicular Feminization Syndrome

Kaplan¹,

Snyder²,

Little³

1969

Arch Pediatr Adolesc Med

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The purpose of this seminar is to discuss a problem which has aroused considerably difference of opinion among the staff members of this hospital. The problem is: Should every female with inguinal hernias be studied to determine if she has the testicular feminization syndrome? Recently, I received a complaint from a member of the staff about such a patient. The patient had undergone surgery and, on the day of discharge, the intern made a buccal smear for sex chromatin determination in the presence of the parents. When the parents asked about the purpose of the test, they learned that it was to find out if the gonads were normal. This question had not been raised previously and the parents were quite upset about the possibility of such an abnormality. Moreover they were distressed to learn about the possibility of a gonadal disorder in this way. They had been informed by their pediatrician and surgeon that surgical correction of inguinal hernias was a simple routine pro¬ cedure and were unaware that anything

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“…Although an abnormality of sexual differentiation, androgen insensitivity syndrome is identi fi ed in as many as 5 % of all patients presenting with primary amenorrhea [ 7 ] . Patients with androgen insensitivity commonly present with distinctive physical characteristics, including a blind vagina, eunuchoid habitus, breasts that have matured only to Tanner stage 3, and small nipples with pale areolae.…”

Section: Androgen Insensitivity Syndromementioning

confidence: 99%