1/11. Polycystic ovaries, precocious puberty and acquired hypothyroidism: The Van Wyk and Grumbach syndrome.Long-standing acquired hypothyroidism can rarely be associated with precocious puberty and polycystic ovaries. The authors report such a case, which responded to the simple replacement of thyroid hormone. It is important to look for hypothyroidism in girls with ovarian masses and precocious puberty to avoid surgery on the ovaries.- - - - - - - - - - ranking = 1keywords = puberty (Clic here for more details about this article) |
2/11. 4: polycystic ovary syndrome.polycystic ovary syndrome (PCOS) is a common condition characterised by menstrual abnormalities and clinical or biochemical features of hyperandrogenism. Features of PCOS may manifest at any age, ranging from childhood (premature puberty), teenage years (hirsutism, menstrual abnormalities), early adulthood and middle life (infertility, glucose intolerance) to later life (diabetes mellitus and cardiovascular disease). While pelvic ultrasound examination is useful, many women without PCOS have polycystic ovaries; ultrasound evidence is not necessary for the diagnosis. Testing for glucose intolerance and hyperlipidaemia is wise, especially in obese women, as diabetes mellitus is common in PCOS. Lifestyle changes as recommended in diabetes are fundamental for treatment; addition of insulin-sensitising agents (eg, metformin) may be valuable in circumstances such as anovulatory infertility. infertility can be treated successfully in most women by diet and exercise, clomiphene citrate with or without metformin, ovarian drilling, or ovulation induction with gonadotrophins; in-vitro fertilisation should be avoided unless there are other indications.- - - - - - - - - - ranking = 0.16666666666667keywords = puberty (Clic here for more details about this article) |
3/11. Follow-up study of two sisters with type A syndrome of severe insulin resistance gives a new insight into PCOS pathogenesis in relation to puberty and pregnancy outcome: a case report.We report two sisters with profound insulin resistance associated with a novel heterozygous missense mutation in exon 19 (His1130Arg) of the insulin receptor gene. The eldest was seen after puberty at age 15 and she presented a severe form of polycystic ovary syndrome (PCOS) with biological hyperandrogenism (HA) mimicking a virilizing tumour. However, she has been able to ovulate under clomiphene citrate (CC) and to achieve two uneventful pregnancies. The patient had no glucose tolerance abnormality during pregnancies. The outcome of pregnancy was good except for a low birthweight. The youngest sister was seen earlier in life (at age 11) before puberty. First, she developed polycystic ovaries (PCO), seen under ultrasound scan, and later also developed full PCOS. This second finding gave us the opportunity to observe that PCO developed before and at the beginning of puberty despite low LH levels. We postulate that the development of PCO was the consequence of an LH-independent intra-ovarian HA likely induced by the severe hyperinsulinism in the context of genetic abnormalities.- - - - - - - - - - ranking = 1.1666666666667keywords = puberty (Clic here for more details about this article) |
4/11. Evolution of polycystic ovaries in a girl with delayed menarche. A case report.A 17-year-old girl had delayed growth secondary to low birth weight. Although she entered puberty at 12.5 years, she had delayed menarche. She underwent serial ovarian ultrasound scans, and a change in ovarian morphology was observed, from a normal multicystic appearance to one typical of polycystic ovaries (PCOs). We hypothesize that normal ovarian morphology may change to PCOs during the peripubertal period because of a change in the endocrine milieu.- - - - - - - - - - ranking = 0.16666666666667keywords = puberty (Clic here for more details about this article) |
5/11. The evolution of polycystic ovaries in a girl with hypogonadotropic hypogonadism before puberty and during puberty induced with pulsatile gonadotropin-releasing hormone.We describe a girl of 12.7 years who had GnRH deficiency after surgery and radiotherapy for a craniopharyngioma at age 5 years. She had large cystic ovaries with a small uterus in the absence of spontaneous gonadotropin pulsatility. puberty was induced by low-dose pulsatile GnRH treatment over a 1.5-year period. In the perimenarcheal period, ovarian morphology became typical of PCO with abnormal diurnal pulsatile gonadotropin secretion. These data suggest that PCO is primarily a disorder of the ovary and that the observed gonadotropin abnormalities are secondary to the ovarian lesion.- - - - - - - - - - ranking = 1.3333333333333keywords = puberty (Clic here for more details about this article) |
6/11. Pseudo-precocious puberty associated with mediastinal teratoma and polycystic ovary.A 10-month-old girl with sexual precocity of recent onset was found to have elevated levels of estrogens, progesterone and androgen precursors, associated with a polycystic left ovary. After ovariectomy, estrogen and androgen levels were normal, and the clinical symptoms began to regress. A few months later, a huge mediastinal mass was unexpectedly discovered. Removal of the mass, identified later as a benign teratoma, was followed by the total normalization of the clinical and hormonal findings. The patient's pseudo-precocious puberty might have been due to an ovarian overproduction of estroprogestins and androgens, associated with a paraneoplastic production of progesterone, or in theory, it might have been due to a gonadotropin-like stimulation of the ovary by the teratoma.- - - - - - - - - - ranking = 0.83333333333333keywords = puberty (Clic here for more details about this article) |
7/11. Evolution of the hyperandrogenism-polycystic ovary syndrome from isosexual precocious puberty: report of two cases.Two girls who presented initially with isosexual precocity later developed the hyperandrogenism-polycystic ovary syndrome. We propose that the pathogenesis of the hyperandrogenism-polycystic ovary syndrome in these two children is related to an abnormal release of hypothalamic gonadotropin-releasing hormone, a subsequent sustained secretion of luteinizing hormone, and a consequent excessive secretion of ovarian androgens that results in hyperandrogenism. The clinical findings in these patients suggest that the dysfunction of the hypothalamic-pituitary regulation of luteinizing hormone secretion that leads to the hyperandrogenism-polycystic ovary syndrome may occur prior to puberty.- - - - - - - - - - ranking = 0.83333333333333keywords = puberty (Clic here for more details about this article) |
8/11. A syndrome of female pseudohermaphrodism, hypergonadotropic hypogonadism, and multicystic ovaries associated with missense mutations in the gene encoding aromatase (P450arom).We report the features of a new syndrome of aromatase deficiency due to molecular defects in the CYP19 (P450arom) gene in a 46,XX female. At birth, the patient presented with a nonadrenal form of female pseudohermaphrodism. At 17 months of age, laparotomy revealed normal female internal genital structures; the histological appearance of the ovaries was normal. FSH concentrations were markedly elevated at 9.4 ng/mL LER 869, and estrone and estradiol levels were undetectable (< 37 pmol/L). By 14 yr of age, she had failed to exhibit breast development. The clitoris had enlarged to 4 x 2 cm, and pubic hair was Tanner stage IV. The plasma concentration of testosterone was elevated at 3294 pmol/L, as was androstenedione at 9951 pmol/L. plasma estradiol levels were below 37 pmol/L. ACTH and dexamethasone tests indicated a nonadrenal source of testosterone and androstenedione. plasma gonadotropin levels were in the castrate range. Pelvic sonography and magnetic resonance imaging showed multiple 4- to 6-cm ovarian cysts bilaterally. Despite increased circulating androgens and clitoral growth, the bone age was 10 yr at chronologic age 14 2/12 yr. estrogen replacement therapy resulted in a growth spurt, breast development, menarche, suppression of gonadotropin levels, and resolution of the cysts. The clinical findings suggested the diagnosis of P450arom deficiency. Analyses of genomic dna from ovarian fibroblasts demonstrated two single base changes in the coding region of the P450arom gene, one at 1303 basepairs (C-T), R435C, and the other at 1310 basepairs (G-A), C437Y, in exon 10. The molecular genetic studies indicate that the patient is a compound heterozygote for these mutations. Expression of these mutations showed that the R435C mutation had 1.1% the activity of the wild-type P450arom enzyme, whereas the C437Y mutation demonstrated no activity. The cardinal features of this syndrome are a consequence of P450arom deficiency: 1) the fetal masculinization in this syndrome can be ascribed to defective placental conversion of C19 steroids to estrogens, leading to exposure of the female fetus to excessive amounts of testosterone; 2) the pubertal failure, mild virilization, multicystic ovaries, and hyperstimulation of the ovaries by FSH and LH are the result of the inability of the ovary to aromatize testosterone and androstenedione to estrogens; and 3) the striking delay in bone age at 14 2/12 yr supports the notion that estrogens, in contrast to androgens, are the major sex steroid driving skeletal maturation during puberty. Familial P450arom deficiency, although rare, may be more common than previously suspected.(ABSTRACT TRUNCATED AT 400 WORDS)- - - - - - - - - - ranking = 0.16666666666667keywords = puberty (Clic here for more details about this article) |
9/11. Induction of ovulation with the sole use of clomiphene citrate in late-onset 21-hydroxylase deficiency.Late-onset 21-hydroxylase deficiency (21-OHD) is a congenital enzymatic defect in the glucocorticoid and mineralocorticoid steroidogenic pathways. The manifestations, including hirsutism and infertility, usually occur at puberty or young adulthood. In infertile, anovulatory women with late-onset 21-OHD, the usual therapy is glucocorticoids for ovulation induction. In this case, we report the sole use of clomiphene citrate to induce ovulation in a patient with late-onset 21-OHD. A hirsute and oligomenorrheic woman was diagnosed as having polycystic ovary syndrome at age 25. Her hirsutism responded to oral contraceptives. At age 31, she was given clomiphene citrate alone for ovulation induction and conceived in her fourth cycle. At age 36, because of increased hirsutism she was diagnosed with late-onset 21-OHD by an ACTH stimulation test. The induction of ovulation in late-onset 21-OHD patients has been with glucocorticoids. Given the success in inducing ovulation with clomiphene citrate alone in this patient with well-documented late-onset 21-OHD, it may be worthwhile to study the sole use of clomiphene citrate for ovulation induction in these patients.- - - - - - - - - - ranking = 0.16666666666667keywords = puberty (Clic here for more details about this article) |
10/11. Ovarian resistance to luteinizing hormone: a novel cause of amenorrhea and infertility.OBJECTIVE: To report the clinical, hormonal, and histopathological features of a woman with ovarian resistance to LH. DESIGN: Clinical study. SETTING: University hospital. PATIENT(S): A woman with amenorrhea, sister of a patient with male pseudohermaphroditism due to Leydig cell hypoplasia. INTERVENTION(S): blood drawing before and after GnRH stimulation and also after dexamethasone and hCG administration, pelvic ultrasound, and ovarian biopsy. MAIN OUTCOME MEASURE(S): karyotype, gonadotropin and steroid measurements, follicular diameter, ovarian histology, and sequencing of the LH receptor gene. RESULT(S): Patient had normal female external genitalia, normal breast development at puberty, rare episodes of vaginal bleeding, and infertility. The karyotype was 46,XX. She had elevated serum LH levels, whereas E2 and P concentrations were in the range seen in the early follicular phase. Pelvic ultrasound revealed a slightly hypoplastic uterus and enlarged polycystic ovaries. A normal follicular reserve for age, antral follicles, and absence of corpora lutea or albicans were observed on ovarian biopsy. Exon 11 of the LH receptor gene had a normal sequence. CONCLUSION(S): In our patient with ovarian resistance to LH, FSH stimulated follicular development until the preovulatory stage, but E2 levels remained in the early follicular phase range, still sufficient for normal pubertal feminization. Apparently, LH is necessary for ovulation and corpus luteum formation.- - - - - - - - - - ranking = 0.16666666666667keywords = puberty (Clic here for more details about this article) |
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