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At least 667 records · Page 37Linked to original sources
PubMed1971

Genital ducts and external genitalia: development and anomalies.

Human wolffian ducts are considered to be of ectodermal origin. Developing müllerian ducts in the area of the mesonephric ducts grow in contact with wolffian ducts. Vaginal differentiation occurs only if müllerian ducts contact the urogenital sinus, inducing proliferation of the entodermal epithelium and if the caudal ends of the müllerian ducts do not regress. Development of the external genitalia and the role of fetal androgens are discussed. Malformations of the external genitalia are classified pathogenetically as steroid-independent and steroid-dependent. Definitions for steroid-dependent malformations such as ambiguous genitalia, hypospadic genitalia, phallus, hypertrophic and peniform clitoris are presented.

Androgens↗
PubMed1971

Androgen-insensitive male pseudohermaphroditism.

Androgen insensitivity is a mendelian trait transmitted through phenotypically normal females. The affected males exhibit normal fetal testicular development and regression of the müllerian ducts. A luminized uterus is never present. External genitalia are feminine or malformed. The vagina, found in most cases, ends blindly. Female breasts are present in adults. The following three syndromes can be distinguished which differ in the character of the external genitalia: 1) Complete testicular feminization with normal female external genitalia: 2) Incomplete testicular feminization with ambiguous external genitalia showing an enlarged clitoris or phallus; 3) Testicular feminization with hypospadias (androgen-insensitive Reifenstein syndrome). External genitalia are hypospadic, exhibiting scrotal development. The presence of immature Sertoli cells in testicular tubules, in spite of hyperplastic and hypertrophic Leydig cells, is considered to be a pathognomonic histopathologic feature for androgen-insensitivity syndromes. The Leydig cells are able to produce androgens, but androgen-insensitive Sertoli cells are unable to mature.

Adult↗
PubMed1979

Silver-Russell syndrome. Observations in 20 patients.

The growth and development data of 20 patients with the Silver-Russell syndrome (14 boys, 6 girls) were analyzed. Family history, pregnancy and delivery did not reveal any significant anomalies. Birth length was 44.0 +/- 3.0 cm (boys) and 43.8 +/- 2.1 cm (girls), birth weight 2.0 +/- 0.4 kg and 2.05 +/- 0.3 kg, respectively. At the time of diagnosis (mean age 4.1 +/- 2.2 years), height was -4.4, bone age -1.9, weight -3.7, and head circumference -1.5 standard deviations below the normal mean for age. Calculated or reached adult height corresponded to 82--94% of target height. Intelligence was normal in most patients. 8 had asymmetrical extremities, 3 an asymmetrical face. 7 of 14 boys had cryptorchidism (3 uni-, 4 bilateral), 2 incomplete masculinization, and 2 of 6 girls hypertrophy of the clitoris. Development of secondary sex characters was appropriate for bone age with exception of one boy, whose puberty was early. In 3 boys with completed pubertal development, testicular volume was small and gonadotropins (before and after LHRH) high. It is concluded that 1. the growth pattern in Silver-Russell syndrome is quite homogeneous, and rather accurate predictions are possible; 2. Intersexual genitalia do not seem to be related to endocrine factors, and 3. hypergonadotropic hypogonadism appears to be frequent in males.

Abnormalities, Multiple↗
PubMedDate not supplied

[Late diagnosis hyperandrogenism due to adrenal enzyme deficiency (author's transl)].

Six women aged from 17 to 30 years (mean: 21 years) were referred on account disorders which had begun at puberty and had been present for 3 to 15 years. The reasons for consulting were hirsutism in 5 cases and sterility in one. The patients' height (1.61 to 1.70 m; mean: 1.64 m) and weight (54 to 70 kg; mean: 59 kg) were normal. Three women menstruated regularly and 3 had anovular spaniomenorrhaea. Hirsutism with enlargement of the clitoris (Prader's stage I) was apparent in all six cases. Three patients had permanent, though moderate hypertension. The biochemical changes essential to the diagnosis were as follows: 1) in 2 women with 21-hydroxalyse deficiency there was a rise in cortisol precursors (plasma 17-OH progesterone and its urinary metabolite, pregnanetriol). In 4 women with 11-hydroxylase deficiency urinary 17-OH corticosteroids were increased; two had high desoxycorticol levels. A rise in plasma desoxycortisol/cortisol ration under tetracosactrin stimulation is of considerable diagnostic value; 2) plasma androgens (testosterone, delta 4 androstenedione) and their urinary metabolites (17-ketosteroids) were increased; 3) all abnormalities disappeared when the adrenal function was suppressed. Under dexamethasone treatment hirsutism became stabilized or even regressed, blood pressure values rapidly returned to normal, menstrual disorders disappeared and ovular cycles were established. Three women became pregnant and delivered on term.

Adolescent↗
PubMed1981

[Kidney and osseous responsiveness to PTH in a case of pseudohypoparathyroidism (author's transl)].

A case of pseudohypoparathyroidism is described, finding in addition to the typical clinical data described in the literature an all almost total atrophy of the minor lips and clitoris, a perforated hymen and a "paradoxical" increase of convulsions with anticomicial treatment. Study of response of urinary phosphates and AMPc to PTH, was negative (Drezner type I). Osseous response to PTH was negative (Frame type I pseudohypoparathyroidism). Pathogenic possibilities are discussed.

Anticonvulsants↗
PubMed1984

[Intersexuality in the horse. Morphologic, hormone analytic and cytogenetic studies in an Arabohaflinger horse].

A case of intersexuality in an arabo-haflinger horse is described. The external genitalia consisted of mammary gland, vulva and hypertrophic clitoris; the inner genitalia of rudimental testes. The sexual behaviour was typically male-like. The chromosome analysis showed the female karyotype 64,XX. The presence of testes in a genetically female animal is explained by translocation of an Y-chromosome fragment bearing the gene for the H-Y-antigen.

Animals↗
PubMed1983

Testicular feminization in the Finnish racoon dog (Nyctereutes procyonoides).

The clinical features of testicular feminization in the racoon dog (Nyctereutes procyonoides) are reported. The condition is characterized by a normal male karyotype, but a mixed phenotype consisting of vulva, enlarged clitoris and scrotal testes. Partial spermatogenesis with a relative arrest at the first meiotic division was observed. The likely underlying genetic defect and mode of inheritance are discussed, together with implications for breeding programmes.

Androgen-Insensitivity Syndrome↗
PubMed1980

Congenital adrenal hyperplasia diagnosed in a middle-aged woman.

Short stature, severe hirsutism, an enlarged clitoris and lack of vaginal introitus were manifest in a 55-year-old female karyotype, male phenotype patient brough up as a female. The hormone profile suggested congenital adrenal hyperplasia due to 21-hydroxylase adrenal enzyme deficiency. Follicle-stimulating hormone and luteinizing hormone levels were 122.5 and 100.3 mIU/ml, respectively, within the normal range for a postmenopausal woman. Computerized axial tomography revealed a large cystic tumor connected to the right adrenal gland. Both the right adrenal gland and the cyst were removed. The adrenal cortex showed diffuse hyperplasia and contained a well-defined nodule. The case was interesting because we had the opportunity of examining a patient of advanced age with untreated congenital adrenal hyperplasia and because of the very rare combination of adrenogenital syndrome with nodular hyperplasia and a cystic tumor, which was most probably a degenerated adenoma.

Adrenal Glands↗
PubMed1981

Ring chromosome 11 [46,XX,r(11) (p15q25)] associated with clinical features of the 11q- syndrome.

A 2-year-old girl with a ring of chromosome 11[46,XX,r(11)(p15q25)] was reported. Her clinical features included growth and psychomotor retardation, microbrachycephaly, hypertelorism, strabismus externus, short nose, low nasal bridge, low-set ears, microretrognathism, short neck, small opening of vagina with large clitoris, deformity of nails, café-au-lait spot of the skin, general hirsutism, congenital heart disease, generalized convulsions, and pancytopenia. Most of these features are those characteristic of the 11q- syndrome. The fact that the patients with the deletion distal from q22 (reported cases) to q25 (our case) had a common phenotype suggests that the loss of the q25 leads to qter segment is mainly responsible for the characteristic clinical features of the 11q- syndrome.

Child, Preschool↗
PubMed1982

[Virilization caused by methandrostenolone-containing cream in 2 prepubertal girls].

Two prepubertal girls were treated with Dianabol cream by their family physicians during 6 and 8 months because of an anal exzema. In both of them, growth velocity and bone maturation were accelerated, and there was hypertrophy of the clitoris and deepening of the voice. In one girl, all symptoms with the exception of the deep voice had disappeared six years after the discontinuation of treatment. In the other girl, final evaluation is not yet possible. The two observations show that androgens and anabolic steroids may have a marked systemic action if applied percutaneously. Treatment with these compounds is indicated very rarely in children and should be restricted to pediatric endocrinologists.

Body Height↗
PubMed1981

[Prevention of oestrus and/or pregnancy in dogs by methods other than ovariohysterectomy (author's transl)].

The present paper is based on findings reported in the literature and concerned with various possible methods of preventing heat and/or pregnancy without resorting to ovariohysterectomy. The drugs used to suppress and prevent oestrus, such as progestagens, testosterone and 19-nortestosterone derivates, are reviewed, the mechanism of action, mode of administration and advantages and disadvantages being discussed. Progestagens are particularly found to affect the uterus (CEH) and mammary glands (increased incidence of mammary tumours), and they may also induce diabetes mellitus and acromegaly. On the other hand, the untoward side-effects of 19-nortestosterone derivatives are found to be mainly due to their androgenic action, resulting in enlargement of the clitoris, vaginitis, changes of behaviour and masculinization of puppies when the drug is administered during pregnancy. Subsequently, those mechanical and surgical procedures which are less commonly employed, are discussed. The pros and cons of the various methods and drugs as well as the possible causes of the difference in gestagenic effect of a number of progestational agents are discussed in greater detail.

Animals↗
PubMed1981

[Female epispadias (author's transl)].

The authors report a rare malformation : a minor form of epispadias responsible for urinary stress incontinence. This incontinence was present during childhood, disappeared during adolescence and recurred following several pregnancies. The diagnosis was made on the basis of separation of the labia minora combined with apparent absence of the clitoris. The authors report a perfect result with a two year follow-up, obtained by V-Y plasty of the bladder neck associated with aponeurotic suspension using the technique of Gobbel-Stoeckel. The risk of increasing existing reflux led to the performance at the same time of a bilateral anti-reflux operation by advancement. This case is of interest by virtue of its rarity.

Epispadias↗
PubMed1980

[Androgen-producing hilus cell tumor (Leydig cell tumor) of the ovary: case contribution to the differential diagnosis of virilization in the adult woman].

Severe hirsutism, deepening of the voice and enlargement of the clitoris developed in the course of two years in a 55-year old hysterectomized woman. Laboratory examinations showed markedly elevated plasma concentrations of testosterone and androstenedione and a moderately increased urinary excretion of 17-ketosteroids. Gaschromatographic analysis of the urinary steroids, revealed that she mainly excreted etiocholanolone, androsterone and pregnanetriol. Plasma androgens and urinary 17-ketosteroids were not suppressed by dexamethasone treatment. During catheterization of the vena cava blood samples were selectively taken from the ovarian veins. Androgen concentrations were the highest in the plasma from the right ovarian vein. However, phlebography as well as adrenal scanning with 131I-adosterol indicated a tumor of the right adrenal gland. During laparoscopy the right ovary was found to be enlarged, and it was subsequently removed. It contained a tumor 2.5 cm in diameter which had the histological features of a hilus cell tumor (Leydig cell adenoma). Two years later, the patient underwent surgery for suspected tumor of the left kidney. A functionless atrophic left kidney was removed. There was a complete thrombosis of the left renal and ovarian veins. Possibly, this severe lesion of the two vessels had been caused by their previous catheterization.

Androgens↗
PubMed1995

Twenty years of experience in managing gender dysphoric patients: I. Surgical management of male transsexuals.

For the past 20 years, the gender treatment team formed under the auspices of the University of Texas Medical Branch Hospitals has treated 121 male individuals with gender identification disorders. The age of this group ranged between 18 and 71 years, with an average of 32.3 years. While all of them were seen regularly for 6 months after surgery, only 68 were followed for a year or longer. The regimen of surgical treatment that consisted of procedures of bilateral orchiectomy, penectomy with relocation of the urethral meatus, labial reconstruction, and neovaginoplasty was used for all patients. The castration procedure used between 1972 and 1977 included removal of both the testes and the spermatic cord. The corpora cavernosa were transected at the public symphysis. For vaginal reconstruction, a partial-thickness skin graft was used frequently, in conjunction with a skin flap fashioned from the penile skin, to line the neovaginal vault. Modifications and changes in technique were, however, introduced in 1978. Skin flaps mobilized from the inguinoperineal area instead of a partial-thickness skin graft were used to line the vaginal cavity. A proximal segment of the corpora cavernosa was retained to reconstruct a clitoris. In addition, the spermatic cord was incorporated into the reconstruction of labia majora. These technical modifications substantially reduced the incidence of vaginal stenosis and improved the external appearance of the genitalia. The experience accumulated from managing this group of patients forms the basis of this report.

Adolescent↗
PubMed1995

[Transsexuality--standards of care].

After the description of transsexualism as a nosological entity by Harry Benjamin standards of care emerged which are useful and protective for patients and doctors involved in treatment. The diagnosis is established by the finding of an irreversibly transposed gender identity. This has to be confirmed by an expert who is competent in counselling gender identity problems. He will give his written opinion which is the indication for the hormonal treatment to be done at least for half a year prior to operation and which leads to virilization or effeminization and which is the preparation for genital surgery. Standards of female-to-male surgery are: 1. Breast-transformation 2. Colpohysterectomy with adnexectomy, possibly by the vaginal route. Surrogates for testes and phalloplasties are only in the experimental stage and not standard. Standards of male-to-female surgery are: 1. Castration by extirpation of testes. 2. Resection of penis shaft with total resection of the spongy bodies. 3. Neovagina by inverted penis-skin technique. 4. Plasty of labia majora and minora and a clitoris. 5. In case of insufficient gynecomastia breast-augmentation. After the operation, follow-up examinations are necessary by the surgeon until complete healing. A supportive psychological care by the expert who has evaluated the transposition of gender identity would be useful. It could be done also by the gynecologist who makes the follow-up examinations in male to female transsexuals. He is also competent for the life-long hormonal substitution.

Female↗
PubMed1995

[Sexual mutilation in women].

The sexual mutilations, a fairly topical subject, remain very frequent (between 80 and 100 millions). The excisions are in practice mainly in Africa (in at last 20 countries), but also in the Far-East and Indonesia. In some cases, such practices are very mutilative (Pharaonic infibulation). They may drag to the death, through haemorrhage or infection. The short and long term medical complications are fearful, tetanus, vesicovaginal fistulae, vaginal or vulvar sclerosis, forbidding any sexual involvement and hampering delivery. The grounds of these practices are basically customary and not religious. Women shall be subservient to men and any manhood mark, embodied by the clitoris, shall disappear. However these manoeuvres are decreasing, due to the women emancipation, and, particularly, in Africa, some leaders are aware of their graveness and are punishing heavily the excision, taking model of the French Penal Code. The immigrants, who, in France, have these mutilations undertaken, are incurring imprisonment sentences (precedents are frequent). They must comply with the French laws.

Adolescent↗