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Diana MacKay, Anna Nordenström, Henrik Falhammar
Context: Management of congenital adrenal hyperplasia (CAH) involves suppression of the hypothalamic-pituitary-adrenal (HPA) axis using supraphysiological doses of exogenous glucocorticoids. This can pose a challenge, with Cushing's syndrome being a frequent complication of adequate suppression. Bilateral adrenalectomy, with subsequent replacement of glucocorticoids and mineralocorticoids at physiological doses, has been proposed as an alternative therapeutic strategy. Objective: To review the outcomes following bilateral adrenalectomy for CAH...
March 15, 2018: Journal of Clinical Endocrinology and Metabolism
Xi Song, Qiyang Shen, Liting Fan, Qiuxiao Yu, Xiao Jia, Yu Sun, Wenpei Bai, Jihong Kang
Polycystic ovary syndrome (PCOS) is the most common endocrinopathy in women of reproductive age and also an important metabolic disorder associated with insulin resistance (IR). Hyperandrogenism is a key feature of PCOS. However, whether hyperandrogenism can cause IR in PCOS remains largely unknown. The mammalian target of rapamycin complex 1 (mTORC1) and its regulated autophagy are closely associated with IR. In the present study, we investigated the role of mTORC1-autophagy pathway in skeletal muscle IR in a dehydroepiandrosterone (DHEA)-induced PCOS mouse model...
February 23, 2018: Oncotarget
Priyadarshini Tripathy, Asutosh Sahu, Mahija Sahu, Attila Nagy
OBJECTIVES: Though polycystic ovarian syndrome (PCOS) is associated with multiple metabolic abnormalities, the metabolic risk profile of various PCOS phenotypes is still debated. Here we sought to compare the clinical, biochemical and metabolic parameters among the different PCOS phenotypes and controls. STUDY DESIGN: A total of 394 newly diagnosed PCOS women and 108 controls were enrolled consecutively. PCOS women were divided into four phenotypes based on the presence of two of the following Rotterdam criteria: oligo/anovulation (O), hyperandrogenism (H), and polycystic ovaries (P): A (O + H + P), B (O + H), C (H + P), D (O + P)...
March 1, 2018: European Journal of Obstetrics, Gynecology, and Reproductive Biology
Xiao Wang, Huarong Wang, Wei Liu, Zhiyuan Zhang, Yanhao Zhang, Wenqiang Zhang, Zijiang Chen, Guoliang Xia, Chao Wang
Polycystic ovary syndrome (PCOS), which is characterized by hyperandrogenism, is a complex endocrinopathy that affects the fertility of 9%-18% of reproductive-age women. However, the exact mechanism of PCOS, especially hyperandrogen-induced anovulation, is largely unknown to date. Physiologically, the natriuretic peptide type C/natriuretic peptide receptor 2 (CNP/NPR2) system is essential for sustaining oocyte meiotic arrest until the preovulatory luteinizing hormone (LH) surge. We therefore hypothesized that the CNP/NPR2 system is also involved in PCOS and contributes to arresting oocyte meiosis and ovulation...
March 13, 2018: Clinical Science (1979-)
Yanglu Li, Xiangyan Ruan, Husheng Wang, Xue Li, Guiju Cai, Juan Du, Lijuan Wang, Yue Zhao, Alfred O Mueck
OBJECTIVE: To evaluate the prevalence of adverse pregnancy outcomes in healthy Chinese women and to investigate whether these outcomes could be decreased in patients with polycystic ovary syndrome (PCOS) by ethinylestradiol/cyproterone acetate (EE/CPA) pretreatment. DESIGN: Retrospective study. SETTING: Medical university. PATIENT(S): Six thousand healthy women (group A) were selected from 24,566 pregnant women by randomized sampling...
March 7, 2018: Fertility and Sterility
C Berthin, P Sibilia, J Martins-Hericher, A Donzeau, L Martin
BACKGROUND: Non-classical congenital adrenal hyperplasia (NC-CAH) is a recessive autosomal disease caused by a deficiency of adrenal steroidogenesis enzymes. It must be distinguished from classical CAH, either simple virilising or salt-wasting, diagnosed during the neonatal period and responsible for potentially lethal disorders of sexual differentiation. NC-CAH presents a simpler and less specific clinical picture. Herein, we present two cases comprising twin girls consulting for diffuse hypertrichosis...
March 7, 2018: Annales de Dermatologie et de Vénéréologie
Marco Mezzullo, Flaminia Fanelli, Guido Di Dalmazi, Alessia Fazzini, Daniela Ibarra-Gasparini, Marianna Mastroroberto, Jenny Guidi, Antonio Maria Morselli-Labate, Renato Pasquali, Uberto Pagotto, Alessandra Gambineri
Hyperandrogenic disorders have been associated with psychological distress, reduced quality of life, anxiety and depression. The hypothalamic-pituitary-adrenal (HPA) axis plays a pivotal role in the adaptive response to stressor events. Salivary cortisol (SalF) and cortisone (SalE) testing have been proven to be useful in the evaluation of HPA-axis activity. This study investigated whether SalF and SalE responses to two putative stressor levels differed between the hyperandrogenic states in late adolescent and young women, thus measuring the HPA-axis adaptive response to acute stress events...
February 24, 2018: Psychoneuroendocrinology
Min Chen, Weibin Zhou, Zhe Zhang, Yuting Zou, Chengjiang Li
RATIONALE: The incidence of severe hyperandrogenism associated with masculinity in women is very low. While rare and difficult to diagnose, androgen secreting tumors should be suspected in women with hyperandrogenism and hirsutism, especially in the postmenopausal population. Herein we present one case of ovarian Leydig cell tumor (LCT) with markedly elevated serum testosterone levels and frank hirsutism. PATIENT CONCERNS: A 60-year-old woman, presented with increased hair growth and androgenic alopecia and the hormonal laboratory examination showed that she had elevated serum testosterone level and normal dehydroepiandrosterone sulfate (DHEAS), androstenedione, 17- hydroxyprogesterone, cortisol and thyroid stimulating hormone (TSH)...
March 2018: Medicine (Baltimore)
Urszula Ambroziak, Alina Kuryłowicz, Anna Kępczyńska-Nyk, Agnieszka Kondracka, Sylvia Gajda, Damian Sieńko
AIM: The diagnosis of biochemical hyperandrogenism is still challenging because a set of appropriate, recommended diagnostic tests has not been established. In our study, we aimed to answer the question of whether salivary testosterone is a reliable test to establish the diagnosis of biochemical hyperandrogenism as compared to serum total testosterone (TT) measured either by liquid chromatography-tandem mass spectrometry (LC-MS/MS) or immunoassay and to assess which set of biochemical tests would be the most appropriate for the identification of biochemical hyperandrogenism...
March 8, 2018: Journal of Obstetrics and Gynaecology Research
Shayesteh Mehdinejadiani, Fardin Amidi, Mehdi Mehdizadeh, Mahmood Barati, Leili Safdarian, Reza Aflatoonian, Ashraf Alyasin, Marzieh Aghahosseini, Azar Pazhohan, Parisa Hayat, Farzaneh Mohammadzadeh Kazorgah, Aligholi Sobhani
Polycystic ovarian syndrome (PCOS) is a common endocrinologic disorder in women of reproductive age characterized by polycystic ovaries, oligo/anovulation, and hyperandrogenism. Not only anovulation but also endometrial dysfunction can reduce fertility in PCOS patients. Wnt pathway is responsible for endometrial proliferation which be strongly regulated by estradiol. To determine the effects of clomiphene citrate (CC) and letrozole, we measured the expression of some main ligands of Wnt/β-catenin signaling including Wnt7a, Wnt3, and Wnt8b in the endometrial samples taken from PCOS women on day 12 of the menses who received 100 mg CC or 5 mg letrozole as well as from women without treatment...
March 6, 2018: Gynecological Endocrinology
Jan Ulrich, Julia Goerges, Christoph Keck, Dirk Muller-Wieland, Sven Diederich, Onno Eilard Janssen
BACKGROUND: Autoimmune thyroiditis (AIT) has been found to be associated with polycystic ovary syndrome (PCOS). The aim of this retrospective cohort study using data from a fertility clinic, with patients recruited from 2009 to 2010, was to confirm the higher prevalence of AIT in PCOS and to evaluate the impact of AIT on reproductive and metabolic parameters of PCOS patients. METHODS: Patients comprised 827 PCOS subjects seen for reproductive or metabolic complaints...
March 5, 2018: Experimental and Clinical Endocrinology & Diabetes
Jacob P Christ, Tommaso Falcone
OBJECTIVE: To characterize the impact of bariatric surgery on reproductive and metabolic features common to polycystic ovary syndrome (PCOS) and to assess the relevance of preoperative evaluations in predicting likelihood of benefit from surgery. METHODS: A retrospective chart review of records from 930 women who had undergone bariatric surgery at the Cleveland Clinic Foundation from 2009 to 2014 was completed. Cases of PCOS were identified from ICD coding and healthy women with pelvic ultrasound evaluations were identified using Healthcare Common Procedure Coding System coding...
March 2, 2018: Obesity Surgery
Anna Eichler, Hartmut Heinrich, Gunther H Moll, Matthias W Beckmann, Tamme W Goecke, Peter A Fasching, Marcel-René Muschler, Polyxeni Bouna-Pyrrou, Bernd Lenz, Johannes Kornhuber
The second-to-forth digit length ratio (2D:4D) is considered to be a biomarker for intrauterine androgen levels. It is associated with adult and child mental health problems, primarily with behavioral symptoms and predominantly in males. Using a cross-sectional design, we examined whether 2D:4D was associated with conduct disorder (CD) symptoms in 138 primary-school aged children (54% boys, Mage  = 7.70 years) and considered child sex as a moderating factor. Children's digit lengths were measured from hand scans and mothers rated the behavioral/emotional symptoms of their child...
February 24, 2018: Early Human Development
Birgül Kirel, Özkan Bozdağ, Pelin Köşger, Sultan Durmuş Aydoğdu, Eylem Alıncak, Neslihan Tekin
Donohue syndrome (Leprechaunism) is characterized by severe insulin resistance, hyperinsulinemia, postprandial hyperglycemia, preprandial hypoglycemia, intrauterine and postnatal growth retardation, dysmorphic findings, and clinical and laboratory findings of hyperandrogenemia due to homozygous or compound heterozygous inactivating mutations in the insulin receptor gene. A female newborn presented with lack of subcutaneous fat tissue, bilateral simian creases, hypertrichosis, especially on her face, gingival hypertrophy, cliteromegaly, and prominent nipples...
December 2017: Türk Pediatri Arşivi
Jian Li, Q Wu, Xiao-Ke Wu, Zhong-Ming Zhou, Ping Fu, Xiu-Hua Chen, Ying Yan, Xin Wang, Zheng-Wang Yang, Wei-Li Li, Elisabet Stener-Victorin, Richard S Legro, Ernest Hung-Yu Ng, Heping Zhang, Ben Willem J Mol, Chi Chiu Wang
STUDY QUESTION: Does second-hand smoke (SHS) exposure from husbands have adverse effects on sex hormones, metabolic profiles, clinical phenotypes and fertility outcomes in women with polycystic ovary syndrome (PCOS) undergoing ovulation induction? SUMMARY ANSWER: SHS exposure is associated with worsened biochemical hyperandrogenism, higher incidence of metabolic syndrome and reduced conception rates in women with PCOS. WHAT IS KNOWN ALREADY: Smoking in women impairs fecundity at some stages of the reproductive process including folliculogenesis, embryo transport, endometrial angiogenesis and uterine blood flow...
February 20, 2018: Human Reproduction
Genevieve E Ryan, Shaddy Malik, Pamela L Mellon
Polycystic ovary syndrome (PCOS), the most common endocrinopathy in women of reproductive age, is characterized by hyperandrogenism, anovulation, and polycystic ovaries. Although its etiology is unknown, excess androgens are thought to be a critical factor driving the pathology of PCOS. We previously demonstrated that continuous exposure to the aromatase inhibitor letrozole (LET) in mice produces many hallmarks of PCOS, including elevated testosterone (T) and luteinizing hormone, anovulation, and obesity. In the current study, we sought to determine whether androgen receptor (AR) actions are responsible for any of the phenotypes observed in LET mice...
April 1, 2018: Endocrinology
Leila Hadjadj, Szabolcs Várbíró, Eszter Mária Horváth, Anna Monori-Kiss, Éva Pál, Gellért Balázs Karvaly, Andrea Heinzlmann, Attila Magyar, István Szabó, Réka Eszter Sziva, Zoltán Benyó, Mariann Buday, György László Nádasy
Hyperandrogenic state in females is accompanied with metabolic syndrome, insulin resistance and vascular pathologies. A total of 67%-85% of hyperandrogenic women suffer also from vitamin D deficiency. We aimed to check a potential interplay between hyperandrogenism and vitamin D deficiency in producing insulin resistance and effects on coronary resistance arteries. Adolescent female rats were divided into four groups, 11-12 animals in each. Transdermal testosterone-treated and vehicle-treated animals were kept either on vitamin D-deficient or on vitamin D-supplemented diet for 8 weeks...
February 1, 2018: Diabetes & Vascular Disease Research
Pınar Kocaay, Zeynep Siklar, Sema Buyukfirat, Merih Berberoglu
STUDY OBJECTIVE: Polycystic ovarian syndrome (PCOS) is a common endocrine disorder characterized by hyperandrogenism and chronic anovulation, which affects 5-10% of reproductive age females. Diagnosis of adult patients with PCOS is made easily with clinical and laboratory methods and the antimullerian hormone (AMH) level are accepted as a good indicator. However, there is still no complete consensus on the diagnosis of PCOS in adolescents. DESIGN: and Settings: Prospective cohort study, December 2013 to November 2014 PARTICIPANTS: The study was conducted on adolescent girls with oligomenorrhea, with at least two years since menarche...
February 17, 2018: Journal of Pediatric and Adolescent Gynecology
Mina Amiri, Ali Kabir, Fatemeh Nahidi, Maryam Shekofteh, Fahimeh Ramezani Tehrani
INTRODUCTION: Nowadays, selection of COCs with maximum antiandrogenic effects is one the main issues in treatment of women with polycystic ovary syndrome (PCOS). OBJECTIVE: This systematic review and meta-analysis aimed to compare the effects of COCs on the clinical and biochemical parameters of hyperandrogenism (HA) in patients with PCOS. METHODS: Electronic databases (PubMed, Scopus, ScienceDirect and web of science) were searched from 1987 to November 2015 to identify clinical trials investigating effect of the various COCs on the clinical and biochemical parameters of HA in patients...
February 19, 2018: European Journal of Contraception & Reproductive Health Care
Michel Pugeat, Ingrid Plotton, Aude Brac de la Perrière, Gerald Raverot, Henri Déchaud, Véronique Raverot
Measuring total testosterone level is the first-line approach in assessing androgen excess in women. The main pitfalls in measuring testosterone relate to its low concentration and to the structural similarity between circulating androgens and testosterone, requiring accurate techniques with high specificity and sensitivity. These goals can be achieved by immunoassay using a specific anti-testosterone monoclonal antibody, ideally after an extraction step. Liquid chromatography coupled to tandem mass spectrometry (LC-MS/MS) will be commonly used for measuring testosterone, providing optimal accuracy with a low limit of detection...
February 16, 2018: European Journal of Endocrinology
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