1/11. prolactinoma causing secondary amenorrhea in a woman with Ullrich-turner syndrome.This is the case report of a girl who was diagnosed as having Ullrich-Turner mosaic at the age of 12 years. She had normal pubertal development and menarche at the age of 15 years. The patient had regular menstrual cycles for 12 months before developing secondary amenorrhea. She was started on estrogen/gestagen replacement therapy by her gynecologist. Several months later a prolactinoma was diagnosed by laboratory and imaging techiques. A second-generation dopamine agonist led to almost regular cycles. Therefore, even in patients with susceptibility to ovarian failure secondary amenorrhea necessitates thorough diagnostic investigation. Copyrightz1999S.KargerAG,Basel- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
2/11. 21-hydroxylase deficiency and Turner's syndrome: a reason for diminished endometrial receptivity.OBJECTIVE: To report a case of cryptic 21-hydroxylase deficiency identified at the time of ovum donation in a patient with Turner's syndrome. DESIGN: Case report. SETTING: University IVF practice. PATIENT(S): A 28-year-old woman with Turner's syndrome who presented for ovum donation. INTERVENTION(S): Four cycles of donor IVF. MAIN OUTCOME MEASURE(S): pregnancy, endometrial appearance, progesterone, and 17-hydroxyprogesterone values. RESULT(S): The patient failed two fresh and two frozen ET cycles with donated oocytes. The appearance of the endometrium suggested elevated progesterone before progesterone supplementation. An elevated progesterone was detected but not suppressed by leuprolide acetate. progesterone was suppressed by adding dexamethasone. The diagnosis of cryptic 21-hydroxylase deficiency was confirmed biochemically. CONCLUSION(S): patients with Turner's syndrome reportedly have poorer outcomes with donor IVF than other women. They also have an increased incidence of carrying a defective 21-hydroxylase gene. We suggest that some of the poorer outcomes may be explained by the presence of elevated progesterone and recommend evaluation of possible congenital adrenal hyperplasia in patients with Turner's syndrome who want oocyte donation.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
3/11. A case of 45,X turner syndrome with spontaneous ovulation proven by ultrasonography.A well-documented case of non-mosaic turner syndrome, with spontaneous pubertal development and ovulatory cycles is reported. mosaicism could be excluded both by karyotyping of 172 metaphases of blood lymphocytes and fibroblasts, and by fluorescence in situ hybridization, using an X-centromeric probe, in 200 blood lymphocyte nuclei. This turner syndrome patient underwent normal pubertal development, with spontaneous menarche at 14 years, followed by regular monthly periods. Hormonal measurements performed during puberty were consistent with the patient's pubertal development. At the age of 26 years the patient was referred for complete fertility evaluation. Detailed hormonal analyses were performed in a given cycle. They showed midluteal phase estradiol and progesterone values within the range corresponding to normal ovulation and corpus luteum function. In the same cycle, pelvic ultrasonography was also performed at days 13, 15 and 18. It demonstrated a spontaneous ovulation, with follicular rupture that occurred between days 15 and 18. This is the first report of a spontaneous ovulation in turner syndrome evidenced, not only by hormonal analysis, but also by ultrasonographic demonstration of follicular rupture.- - - - - - - - - - ranking = 1.5keywords = cycle (Clic here for more details about this article) |
4/11. Turner's syndrome mosaicism 45X/47XXX: an interesting natural history.mosaicism 45X/47XXX is a sporadic form of ovarian dysgenesis. Many of the cases previously described were characterized by a variable phenotype expression. We here report the case of a 33-yr-old woman with recent secondary amenorrhea, weight loss and breast regression. Her menarche had occurred at the age of 11 yr and 6 months and her menstrual cycles had been regular until the age of 28; then, oligomenorrhea and hypertricosis developed. A pelvic ultrasound showed enlarged polycystic-like ovaries and normal uterus. She was treated with ethynil-estradiol and cyproterone acetate for one year. At the age of 31 yr, she underwent a pelvic ultrasound--which revealed normal volume of the ovaries--and hormonal assays including FSH (69 UI/l), LH (113 UI/l), 17beta-estradiol (88 pg/ml), plasma androgens and cortisol levels within normal ranges. No organ-specific autoantibodies toward ovaries, steroid-producing cells or adrenals were found. At the age of 33 yr, there was ultrasound evidence of streak-like ovaries. The patient's height was 145 cm and her weight 45 kg. She had normal female external genitalia, abnormal upper-to-lower body segment ratio, webbed neck, low posterior hair line, cubitus valgus, short and asymmetrical 4th metacarpi, hallux with lateral deviation and moderate scoliosis. No increase in ovarian steroids were found after GnRH-analogue triptorelin (0,1 mg sc) administration. The karyotype analysis on peripheral blood lymphocytes showed a mosaic 45X (90% cells) and 47XXX (10% cells). Diagnostic pelviscopy confirmed streak gonads. Chronic lymphocytic thyroiditis was diagnosed but no cardiovascular or kidney abnormalities were found. A neuro-psychological evaluation revealed emotional and social immaturity, disorders in motorial coordination, visual-spatial organization, as well as reading difficulties and impaired complex phrase construction. The presence of several somatic features of Turner's syndrome, neuro-psychological disorders and an interesting natural history probably depended on the quantitative proportion of 45X to 47XXX cell-lines in different tissues and organs. Estrogen and progestin replacement therapy led to weight gain, re-appearance of secondary sexual characteristics and a mild improvement in mental equilibrium.- - - - - - - - - - ranking = 0.5keywords = cycle (Clic here for more details about this article) |
5/11. Girls with Turner's syndrome with spontaneous menarche have an increased risk of endometrial carcinoma: a case report and review from the literature.BACKGROUND: patients with Turner's syndrome receiving unopposed estrogens for the induction of feminization have an increased risk of endometrial carcinoma. Only seven patients who were not treated with estrogen replacement therapy have been reported to have developed endometrial carcinoma at different age levels. CASE: A young girl with Turner's syndrome phenotype, spontaneous puberty, and karyotype 45,X0/47,XXX from peripheral blood, after irregular menstrual cycles of 9 years, at the age of 21, was diagnosed with a non-invasive well-differentiated endometrial carcinoma confined to a hyperplastic endometrial polyp. Analysis of the ovarian tissue by FISH confirmed mosaicism: 45,X0/46,XX/47,XXX. CONCLUSION(S): The endogenous estrogen secretion from the ovaries might have caused malignancy in this case. patients with Turner's syndrome with spontaneous menarche might carry a higher risk of endometrial carcinoma.- - - - - - - - - - ranking = 0.5keywords = cycle (Clic here for more details about this article) |
6/11. Hypothalamic-pituitary-ovarian function in menstruating women with turner syndrome (45,X).The hypothalamic-pituitary-ovarian hormone secretion patterns were evaluated in two women with 45,X turner syndrome, spontaneous sexual development, and monthly menstrual periods. Each women had serum gonadotropin and sex steroid determinations during two or more menstrual cycles. During the follicular phase of a menstrual cycle, both women received 100 micrograms gonadotropin-releasing hormone (GnRH) s.c., and serum LH and FSH responses were determined. In addition, one woman collected daily overnight urine specimens for 40 consecutive days, spanning two menstrual periods, for the measurement of LH, FSH, estriol, and free progesterone. The randomly measured hormone results showed low serum progesterone concentrations during luteal phases, consistent with the interpretation of anovulation or inadequate corpus luteum function. At the time of the GnRH stimulation tests, baseline serum FSH concentrations and FSH responses to GnRH were within normal limits, whereas baseline LH levels and LH responses to GnRH were low. The pituitary gonadotropin secretion patterns were more consistent with patterns seen during early puberty than in the perimenopausal state. This interpretation was further confirmed by the urinary excretion patterns of gonadotropins, which were not significantly elevated. Furthermore, the urinary hormone profiles revealed that, although the intermenstrual period was of normal length, the follicular phase was prolonged, with normal levels of LH, FSH, and estriol excreted. The menstrual cycle studied was ovulatory but had a short luteal phase. The hormone results indicated that the dysgenetic ovary of women with 45,X turner syndrome is capable of producing sufficient quantities of sex steroids and other regulatory factors to maintain gonadotropin secretion patterns that are reminiscent of early puberty.(ABSTRACT TRUNCATED AT 250 WORDS)- - - - - - - - - - ranking = 1.5keywords = cycle (Clic here for more details about this article) |
7/11. serum PP14 levels in a patient with Turner's syndrome pregnant after frozen embryo transfer.Data are presented on serum levels of the pregnancy-associated placental protein (PP14) in an XO mother, pregnant after frozen embryo transfer. Steroid replacement before and during early pregnancy was provided with transdermal oestradiol and vaginal micronized progesterone. Characterization of a pre-treatment cycle indicated a physiological response by the endometrium. The pregnancy was associated with normal maternal blood levels of all hormones considered responsible for maintenance of early gestation but with consistently subnormal serum levels of PP14. Hitherto, PP14 has been believed to be an endometrial protein arising from the decidua during pregnancy, under the influence of ovarian steroids. The findings in this case suggest that other factors are involved in its production, possibly under the control of, or originating from, the maternal ovary.- - - - - - - - - - ranking = 0.5keywords = cycle (Clic here for more details about this article) |
8/11. Ovarian function in the non-mosaic turner syndrome; a case report.A 19-year-old girl, with the 45,XO non-mosaic turner syndrome, presented having spontaneous regular menstrual activity. Hormonal analysis showed normal ovulatory cycles; histology of the ovaries demonstrated primary follicles. Nevertheless, the karyotype of the ovary and of the peripheral lymphocytes was pure 45,XO. comment on ovarian function in the 45,XO karyotype is given.- - - - - - - - - - ranking = 0.5keywords = cycle (Clic here for more details about this article) |
9/11. Cytogenetic and endocrine studies in a 45,X female subject with spontaneous sexual development.Cytogenetic and endocrine evaluation of a postpubertal 45,X female subject with Turner's stigmas and spontaneous sexual development was performed. A 45,X chromosomal complement was found in the peripheral blood lymphocytes, bone marrow, and fibroblasts derived from skin and ovaries. menarche, pubarche, and thelarche occurred at age 12; at age 16 she developed menstrual irregularities, with endometrial bleeding occurring every 60 to 90 days. The ovaries were normal in size, and histologic examination revealed a marked paucity of primordial follicles, increased collagenization, and absence of corpora albicans. Anovulatory cycles with moderately elevated levels of luteinizing hormone (LH) and low follicle-stimulating hormone (FSH) levels were observed. LH-releasing hormone pituitary stimulation induced a normal LH release with a very slight FSH increase. Administration of clomiphene citrate successfully induced a normal-length ovulatory cycle. Similarities in the hormonal situation of this patient and that seen in the Stein-Leventhal syndrome are pointed out.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
10/11. Turner phenotype in mother and daughter.Two females are described, mother and daughter, who had the Turner phenotype and spontaneous sexual development. The mother is short and had ovulatory menstrual cycles, normal breast development, X-chromatin negative buccal smear, 45,X chromosomal pattern in her peripheral blood lymphocytes, and 45,X/46,X,r(X) mosaicism in her skin, with the majority of the cells (85%) showing X monosomy. She had a successful uncomplicated pregnancy at the age of 25 years. The daughter is short and had spontaneous sexual development, including menstruation at the age of 15 years. Her buccal smear was X-chromatin negative and karyotypes from peripheral blood lymphocytes and skin fibroblasts showed a 45,x chromosome constitution. Her menstrual cycles are irregular and, most probably, anovulatory. She has a horseshoe kidney. Six women with a 45, x chromosome complement are known to have delivered normal infants with no chromosomal abnormality. Five children with 45,X mosaicism have been born to mothers with 45,X mosaicism; all had a 46,XX cell line as well. This is the first report of a 45,X female born to a mother with mosaicism composed of 2 abnormal cell lines, 1 with X monosomy and 1 with a ring x chromosome.- - - - - - - - - - ranking = 1keywords = cycle (Clic here for more details about this article) |
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