-
CMAJ : Canadian Medical Association... Apr 2023
Topics: Female; Humans; Postmenopause; Virilism
PubMed: 37094875
DOI: 10.1503/cmaj.220685-f -
Frontiers in Endocrinology 2023Adult pure androgen-secreting adrenal tumors (PASATs) are extremely rare, and their characteristics are largely unknown.
BACKGROUND
Adult pure androgen-secreting adrenal tumors (PASATs) are extremely rare, and their characteristics are largely unknown.
METHODS
A rare case of adult bilateral PASATs was reported, and a systematic literature review of adult PASATs was conducted to summarize the characteristics of PASATs.
RESULTS
In total, 48 studies, including 40 case reports and 8 articles, were identified in this review. Analysis based on data of 42 patients (including current case and 41 patients from 40 case reports) showed that average age was 40.48 ± 15.80 years (range of 18-76). The incidence of adult PASAT peaked at 21-30 years old, while that of malignant PASAT peaked at 41-50 years old. Most PASAT patients were female (40/42, 95.23%), and hirsutism was the most common symptom (37/39, 94.87%). Testosterone (T) was the most commonly elevated androgen (36/42, 85.71%), and 26 of 32 tested patients presented increased dehydroepiandrosterone sulfate (DS) levels. In malignancy cases, disease duration was significantly decreased (1.96 vs. 4.51 years, P=0.025), and tumor diameter was significantly increased (8.9 vs. 4.9 cm, p=0.011). Moreover, the androgen levels, namely, T/upper normal range limit (UNRL) (11.94 vs. 4.943, P=0.770) and DS/UNRL (16.5 vs. 5.28, P=0.625), were higher in patients with malignancy. In total, 5 out of 7 patients showed an increase in DS or T in the human chorionic gonadotropin (HCG) stimulation test. Overall, 41 out of 42 patients (including current case) underwent adrenal surgery, and recurrence, metastasis, or death was reported in 5 out of 11 malignant patients even with adjuvant or rescue mitotane chemotherapy.
CONCLUSION
Adult PASAT, which is predominant in women, is characterized by virilism and menstrual dysfunction, especially hirsutism. Elevated T and DS may contribute to the diagnosis of adult PASAT, and HCG stimulation test might also be of help in diagnosis. Patients with malignant PASAT have a shorter disease duration, larger tumor sizes and relatively higher androgen levels. Surgery is recommended for all local PASATs, and Malignancy of PASAT should be fully considered due to the high risk of malignancy, poor prognosis and limited effective approaches.
Topics: Adult; Humans; Female; Adolescent; Young Adult; Middle Aged; Aged; Male; Androgens; Hirsutism; Adrenal Gland Neoplasms; Testosterone; Virilism
PubMed: 37033242
DOI: 10.3389/fendo.2023.1138114 -
Trends in Molecular Medicine May 2023Adolescent polycystic ovary syndrome (PCOS) is a highly prevalent, reversible, endocrine-metabolic mode essentially driven by ectopic fat, which, in turn, often results... (Review)
Review
Adolescent polycystic ovary syndrome (PCOS) is a highly prevalent, reversible, endocrine-metabolic mode essentially driven by ectopic fat, which, in turn, often results from a mismatch between early adipogenesis and later lipogenesis, or between prenatal and postnatal weight gain. The key features of adolescent PCOS are menstrual irregularity and androgen excess (hirsutism, acne, and/or high testosterone). Adolescent PCOS is frequently preceded by rapid maturation (early variants of adrenarche/pubarche and puberty/menarche, also accelerated by ectopic fat) and is diagnosed between 2 and 8 years after menarche, thus during late adolescence or early adulthood. Treatment of adolescent PCOS should not only focus on symptoms, but also reduce the amount of ectopic fat, thereby aiming for an overall state of preconception health.
Topics: Female; Adolescent; Humans; Adult; Polycystic Ovary Syndrome; Obesity, Abdominal; Hirsutism; Obesity; Menstruation Disturbances
PubMed: 36964058
DOI: 10.1016/j.molmed.2023.02.006 -
Clinical Endocrinology Jul 2023As part of the update of the International Evidence-Based Guidelines for the Assessment and Management of polycystic ovary syndrome (PCOS), a systematic review was...
OBJECTIVE
As part of the update of the International Evidence-Based Guidelines for the Assessment and Management of polycystic ovary syndrome (PCOS), a systematic review was performed to inform evidence-based recommendations.
DESIGN
Systematic review. Only randomised controlled trial were included.
PATIENTS
Women with PCOS; the use of combined oral contraceptive pills (COCP) was compared with no medical treatment.
MEASUREMENTS
Outcomes were designed in collaboration with clinical experts, researchers, and consumers. Critical outcomes included hirsutism, irregular cycles, quality of life, body mass index (BMI), and weight.
RESULTS
1660 publications were identified, but only four studies were included. No studies could be combined for meta-analysis. COCP treatment improved cycle regularity compared with no medical treatment (100% vs. 0%, with low certainty of evidence). COCP showed no difference in improvement of hirsutism or BMI compared with placebo or lifestyle; a lower weight after COCP compared with no treatment (mean difference [MD] -8.0 (95% confidence interval, CI -11.67); -4.33 kg); and improvement in quality of life (MD 1.2 [95% CI 0.96]; 1.44), but these results were all very low certainty of evidence.
CONCLUSION
Results show that COCP benefit cycle regulation, but other benefits or potential adverse effects were only identified with very low certainty of evidence. The COCP is frontline medical treatment in PCOS, but this is still based on established efficacy in the broader general population. Our results show that research in PCOS is seriously lacking and should be prioritised to capture core reproductive, metabolic and psychological outcomes important in PCOS.
Topics: Female; Humans; Contraceptives, Oral, Combined; Hirsutism; Polycystic Ovary Syndrome; Quality of Life
PubMed: 36939017
DOI: 10.1111/cen.14913 -
Frontiers in Endocrinology 2023Most children with non-classic congenital adrenal hyperplasia (NC-CAH) due to 21-hydroxylase deficiency are asymptomatic and do not require cortisol replacement therapy...
Most children with non-classic congenital adrenal hyperplasia (NC-CAH) due to 21-hydroxylase deficiency are asymptomatic and do not require cortisol replacement therapy unless they develop symptoms of hyperandrogenemia. The current practice is to treat symptomatic children with hydrocortisone aimed at suppressing excess adrenal androgen production irrespective of the child's level of endogenous cortisol production. Once on hydrocortisone therapy, even children with normal cortisol production require stress dosing. Some children with NC-CAH may present with premature adrenarche, growth acceleration, and advanced bone age, but with no signs of genital virilization and normal endogenous cortisol production. In these cases, an alternative therapy to hydrocortisone treatment that does not impact the hypothalamic-pituitary-adrenal axis, but targets increased estrogen production and its effects on bone maturation, could be considered. Aromatase inhibitors (AIs), which block the aromatization of androgen to estrogen, have been used off-label in men with short stature to delay bone maturation and as an adjunct therapy in children with classic CAH. The use of AI as a monotherapy for children with NC-CAH has never been reported. We present three pre-pubertal female children with a diagnosis of NC-CAH treated with anastrozole monotherapy after presenting with advanced bone age, early adrenarche, no signs of genital virilization, and normal peak cortisol in response to ACTH stimulation testing. Bone age z-scores normalized, and all three reached or exceeded their target heights. Monotherapy with anastrozole can be an effective alternative in slowing down bone maturation and improving height outcomes in children with NC-CAH and normal adrenal cortisol production.
Topics: Child; Female; Humans; Adrenal Hyperplasia, Congenital; Anastrozole; Androgens; Estrogens; Hydrocortisone; Hypothalamo-Hypophyseal System; Pituitary-Adrenal System; Virilism
PubMed: 36936152
DOI: 10.3389/fendo.2023.1101843 -
Gynecological Endocrinology : the... Dec 2023Steroid cell tumors not otherwise specified are rare sex cord-stromal tumors of the ovary that may produce various steroids and are associated with hirsutism and... (Review)
Review
Steroid cell tumors not otherwise specified are rare sex cord-stromal tumors of the ovary that may produce various steroids and are associated with hirsutism and virilization. We report a rare case of ovarian steroid cell tumor with subsequent spontaneous pregnancy after tumor removal. A 31-year-old woman presented with secondary amenorrhea, hirsutism, and inability to conceive. Clinical and diagnostic evaluations revealed a left adnexal mass and elevated serum total testosterone and 17α-hydroxyprogesterone levels. She underwent a left salpingo-oophorectomy, and histopathological examination confirmed the diagnosis of a steroid cell tumor not otherwise specified. Her serum total testosterone and 17α-hydroxyprogesterone normalized one month after surgery. Her menses resumed spontaneously one month after the operation. She spontaneously conceived 12 months after the surgery. The patient had an uncomplicated pregnancy and delivered a healthy male infant. In addition, we reviewed the literature on steroid cell tumors not otherwise specified with subsequent spontaneous pregnancies after surgery and data regarding pregnancy outcomes.
Topics: Humans; Pregnancy; Female; Male; Adult; Hirsutism; Ovarian Neoplasms; Sex Cord-Gonadal Stromal Tumors; Steroids; 17-alpha-Hydroxyprogesterone; Testosterone
PubMed: 36878245
DOI: 10.1080/09513590.2023.2186138 -
Frontiers in Endocrinology 2023Polycystic ovary syndrome (PCOS) is the most common infertility disorder worldwide, typically characterised by high circulating androgen levels, oligo- or anovulation,...
INTRODUCTION
Polycystic ovary syndrome (PCOS) is the most common infertility disorder worldwide, typically characterised by high circulating androgen levels, oligo- or anovulation, and polycystic ovarian morphology. Sexual dysfunction, including decreased sexual desire and increased sexual dissatisfaction, is also reported by women with PCOS. The origins of these sexual difficulties remain largely unidentified. To investigate potential biological origins of sexual dysfunction in PCOS patients, we asked whether the well-characterized, prenatally androgenized (PNA) mouse model of PCOS exhibits modified sex behaviours and whether central brain circuits associated with female sex behaviour are differentially regulated. As a male equivalent of PCOS is reported in the brothers of women with PCOS, we also investigated the impact of maternal androgen excess on the sex behaviour of male siblings.
METHODS
Adult male and female offspring of dams exposed to dihydrotestosterone (PNAM/PNAF) or an oil vehicle (VEH) from gestational days 16 to 18 were tested for a suite of sex-specific behaviours.
RESULTS
PNAM showed a reduction in their mounting capabilities, however, most of PNAM where able to reach ejaculation by the end of the test similar to the VEH control males. In contrast, PNAF exhibited a significant impairment in the female-typical sexual behaviour, lordosis. Interestingly, while neuronal activation was largely similar between PNAF and VEH females, impaired lordosis behaviour in PNAF was unexpectedly associated with decreased neuronal activation in the dorsomedial hypothalamic nucleus (DMH).
CONCLUSION
Taken together, these data link prenatal androgen exposure that drives a PCOS-like phenotype with altered sexual behaviours in both sexes.
Topics: Female; Male; Pregnancy; Humans; Animals; Mice; Polycystic Ovary Syndrome; Androgens; Lordosis; Siblings; Virilism
PubMed: 36875467
DOI: 10.3389/fendo.2023.1116482 -
BMC Endocrine Disorders Feb 2023Considering the high prevalence of polycystic ovary syndrome (PCOS) in women of reproductive age and the metabolic disorders associated with it, this study was conducted... (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
Considering the high prevalence of polycystic ovary syndrome (PCOS) in women of reproductive age and the metabolic disorders associated with it, this study was conducted to determine the effects of curcumin on metabolic indices and androgen level (primary outcomes), and menstruation characteristics, and hirsutism (secondary outcomes) in women with PCOS.
METHODS
This triple-blind randomized controlled trial was conducted on women with PCOS who visited the health centers at Eslamshahr County (Tehran Province-Iran) from 2020 to 2022. The participants were allocated into two groups (curcumin and placebo) using block randomization method. The treatment group received two 500 mg edible curcumin tablets together at the same time per day for twelve weeks while the control group received placebo tablets similar to curcumin. Biochemical parameters such as Fasting Blood Insulin (FBI), Fasting Blood Sugar (FBS), triglyceride, total cholesterol, Low Density Lipoprotein- cholesterol (LDL-C), High Density Lipoprotein- cholesterol (HDL-C) were measured before intervention and then 3 months after the intervention. Sex Hormone Binding Globulin (SHBG) and testosterone serum levels were measured 3 months after the intervention. Questionnaires regarding the menstrual cycle characteristics and the Ferriman-Gallwey score were also filled for evaluating hirsutism before the intervention as well as 3 months after the intervention. The independent t-test, Mann-Whitney U test, and ANCOVA were used to analyze the data.
RESULTS
There was no statistically significant difference between the two groups in terms of socio-demographic and the baseline levels of measured outcomes. After 12 weeks of intervention, the mean serum FBS levels in the curcumin group were significantly lower than in the placebo group (mean difference: 6.24; 95%confidence interval: -11.73 to -0.76; P = 0.027) but there was no significant difference between the two groups in terms of triglyceride (P = 0.351), cholesterol (P = 0.528), LDL (P = 0.064), HDL (P = 0.306), FBI (p = 0.929), SHBG (p = 0.682), and testosterone (p = 0.133) serum levels. After the intervention, amenorrhea and oligomenorrhea frequency in the curcumin group was significantly lower than in the placebo group (13% vs. 22%, P = 0.038). There was no significant difference in terms of duration of menstruation (P = 0.286) and hirsutism (P = 0.630) between the two groups.
CONCLUSION
Curcumin decreased FBS levels and improved menstruation characteristics (amenorrhea, oligomenorrhea, and menstrual irregularities) in women with PCOS but did not affect other metabolic, hormonal, and hirsutism indices. More studies using a larger sample size are required for a definitive conclusion.
TRIAL REGISTRATION
Iranian Registry of Clinical Trials (IRCT): IRCT20120718010324N51 Date of registration: 30/11/2019. URL: https://en.irct.ir/user/trial/40597/view ; Date of first registration: 30/11/2020.
Topics: Female; Humans; Polycystic Ovary Syndrome; Hirsutism; Androgens; Curcumin; Amenorrhea; Oligomenorrhea; Iran; Testosterone; Triglycerides; Cholesterol; Cholesterol, LDL
PubMed: 36788534
DOI: 10.1186/s12902-023-01295-5 -
Endocrinology Jan 2023Androgens are steroid hormones crucial for sexual differentiation of the brain and reproductive function. In excess, however, androgens may decrease fertility as...
Androgens are steroid hormones crucial for sexual differentiation of the brain and reproductive function. In excess, however, androgens may decrease fertility as observed in polycystic ovary syndrome, a common endocrine disorder characterized by oligo/anovulation and/or polycystic ovaries. Hyperandrogenism may also disrupt energy homeostasis, inducing higher central adiposity, insulin resistance, and glucose intolerance, which may exacerbate reproductive dysfunction. Androgens bind to androgen receptors (ARs), which are expressed in many reproductive and metabolic tissues, including brain sites that regulate the hypothalamo-pituitary-gonadal axis and energy homeostasis. The neuronal populations affected by androgen excess, however, have not been defined. We and others have shown that, in mice, AR is highly expressed in leptin receptor (LepRb) neurons, particularly in the arcuate (ARH) and the ventral premammillary nuclei (PMv). Here, we assessed if LepRb neurons, which are critical in the central regulation of energy homeostasis and exert permissive actions on puberty and fertility, have a role in the pathogenesis of female hyperandrogenism. Prenatally androgenized (PNA) mice lacking AR in LepRb cells (LepRbΔAR) show no changes in body mass, body composition, glucose homeostasis, or sexual maturation. They do show, however, a remarkable improvement of estrous cycles combined with normalization of ovary morphology compared to PNA controls. Our findings indicate that the prenatal androgenization effects on adult reproductive physiology (ie, anestrus and anovulation) are mediated by a subpopulation of LepRb neurons directly sensitive to androgens. They also suggest that the effects of hyperandrogenism on sexual maturation and reproductive function in adult females are controlled by distinct neural circuits.
Topics: Pregnancy; Humans; Mice; Female; Animals; Receptors, Androgen; Hyperandrogenism; Receptors, Leptin; Anovulation; Sexual Maturation; Androgens; Polycystic Ovary Syndrome; Virilism; Estrous Cycle
PubMed: 36683455
DOI: 10.1210/endocr/bqad015 -
Nutrients Jan 2023Obesity is an established risk factor for the development of polycystic ovary syndrome (PCOS), especially phenotype A. PCOS is an important cause of fertility disorders... (Review)
Review
Obesity is an established risk factor for the development of polycystic ovary syndrome (PCOS), especially phenotype A. PCOS is an important cause of fertility disorders in a large group of women of reproductive age. For many years, effective methods of treating hormonal disorders associated with PCOS have been sought in order to restore ovulation with regular menstrual cycles. Numerous studies support obesity treatment as an effective therapeutic method for many women. A seemingly simple method of treatment may prove to be particularly difficult in this group of women. The reason for this may be the lack of recognition the primary cause of obesity development or the occurrence of a vicious circle of disease. Primary causes of developing obesity may be emotional eating (EE) and eating disorders (EDs), such as binge eating disorder (BED) and its extreme form, addictive eating, as well as night eating syndrome (NES). All of these are caused by impaired function of the reward system. Consequently, these disorders can develop or be exacerbated in women with obesity and PCOS as a result of depression and anxiety related to hirsutism and fertility disturbances. Therefore, for the effective treatment of obesity, it is very important to recognize and treat EE, BED, and NES, including the appropriate selection of pharmacotherapy and psychotherapy. Therefore, the aim of our manuscript is to analyze the available data on the relationships between EE, BED, NES, obesity, and PCOS and their impact on the treatment of obesity in women with PCOS.
Topics: Humans; Female; Polycystic Ovary Syndrome; Binge-Eating Disorder; Night Eating Syndrome; Hirsutism; Obesity
PubMed: 36678165
DOI: 10.3390/nu15020295