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Inhibin A puberty

Hao Wu, Stephanie A Pangas, Karen W Eldin, Kalyani R Patel, John Hicks, Jennifer E Dietrich, Rajkumar Venkatramani
STUDY OBJECTIVE: To report on the clinical characteristics and outcome of pediatric patients with juvenile granulosa cell tumor (JGCT) of the ovary. DESIGN, SETTING, AND PARTICIPANTS: Patients with histopathologically confirmed ovarian JGCT diagnosed between 1990 and 2016 were identified. Data on the clinical presentation, surgical management, oncologic management, laboratory investigation, follow-up, and outcome were collected. Tumor was staged according to the International Federation of Gynecology and Obstetrics (FIGO) criteria...
October 1, 2016: Journal of Pediatric and Adolescent Gynecology
Gilvydas Verkauskas, Dalius Malcius, Audrone Eidukaite, Juozas Vilimas, Darius Dasevicius, Vytautas Bilius, Faruk Hadziselimovic
INTRODUCTION: A transient increase in gonadotropins and testosterone during mini-puberty causes gonocytes to differentiate into Ad spermatogonia, which establish male germ cell memory and male-specific DNA methylation pathways. Over half of patients with unilateral cryptorchidism and the majority of patients with bilateral cryptorchidism display an abnormal spermiogram, which indicates that unilateral cryptorchidism is a bilateral disease; therefore, it represents a serious andrological problem...
August 2016: Journal of Pediatric Urology
Tero Varimo, Leo Dunkel, Kirsi Vaaralahti, Päivi J Miettinen, Matti Hero, Taneli Raivio
OBJECTIVE: Makorin ring finger protein 3 (MKRN3) gene restrains the hypothalamic-pituitary-gonadal axis. In girls, peripheral levels of MKRN3 decline prior to the onset of puberty. We described longitudinal changes in serum MKRN3 levels in boys before and during puberty and assessed the effect of inhibition of estrogen biosynthesis on MKRN3 levels. DESIGN: Longitudinal serum samples from a double-blind, randomized controlled study in 30 boys (age range: 9.1-14.2years) with idiopathic short stature who received placebo (Pl; n=14) or aromatase inhibitor letrozole (Lz; 2...
June 2016: European Journal of Endocrinology
Stefano Stagi, Mariarosaria di Tommaso, Perla Scalini, Elisabetta Lapi, Stefania Losi, Erica Bencini, Fabrizio Masoni, Laura Dosa, Sabrina Becciani, Maurizio de Martino
OBJECTIVE: To evaluate the hypothalamus-hypophysis-gonad axis in a cohort of children and adolescents with nonmosaic triple X syndrome. DESIGN: Cross-sectional study with retrospective analysis. SETTING: University pediatric hospital. PATIENT(S): Fifteen prepubertal subjects (median age 9.0 years, range 6.9-11.9 years) with nonmosaic triple X syndrome and age- and pubertal-matched control group (30 girls, median age 9...
June 2016: Fertility and Sterility
Alexander S Busch, Casper P Hagen, Kristian Almstrup, Katharina M Main, Anders Juul
STUDY QUESTION: Do variants of the genes encoding follicle stimulating hormone (FSH) beta subunit (B) and FSH receptor (R) impact circulating reproductive hormone levels and ovarian follicle maturation in healthy peripubertal girls? SUMMARY ANSWER: FSHB and FSHR genetic variants exert, alone or their combination, distinct effects on reproductive hormone levels as well as ovarian follicle maturation in healthy peripubertal girls. WHAT IS KNOWN ALREADY: FSHB and FSHR genetic variants impact reproductive hormone levels as well as associated pathologies in women...
April 2016: Human Reproduction
Ralf Werner, Isabel Mönig, Julia August, Clemens Freiberg, Ralf Lünstedt, Benedikt Reiz, Lutz Wünsch, Paul-Martin Holterhus, Alexandra Kulle, Ulla Döhnert, Stefan A Wudy, Annette Richter-Unruh, Christoph Thorns, Olaf Hiort
The differential diagnosis of 46,XY disorders of sex development (DSD) is based on the distinction between forms of gonadal dysgenesis and disorders of androgen biosynthesis and action. However, clinical and endocrine evaluations are often not conclusive. Here, we describe an adolescent female with hirsutism and hyperandrogenization at puberty. Her karyotype was 46,XY, and clinical investigation demonstrated clitoromegaly, but no uterine remnants were detected. Histology of the gonads revealed a testicular structure with a Sertoli-cell-only pattern...
2015: Sexual Development: Genetics, Molecular Biology, Evolution, Endocrinology, Embryology, and Pathology of Sex Determination and Differentiation
Dina Cortes, Erik Clasen-Linde, John M Hutson, Ruili Li, Jorgen Thorup
OBJECTIVES AND HYPOTHESES: The Sertoli-cells produce inhibin-B and Anti-Müllerian-Hormone (AMH). Much is still unknown about these hormones in prepubertal cryptorchids. The Sertoli-cells are mandatory for germ cell development. The aim of the study was to investigate if there are differences in secretion pattern of Sertoli-cell hormones and their gonadotropin feed-back mechanisms. METHODS: Included were 94 prepubertal cryptorchid boys 0.5-13.1years with measurements of serum-inhibin-B, Anti-Müllerian-Hormone (AMH), Luteinizing Hormone (LH) and Follicle Stimulation Hormone (FSH)...
March 2016: Journal of Pediatric Surgery
K Uma Devi, N Purushotham, N Jayashree
BACKGROUND: Ovarian Cancer is a broad spectrum of diseases comprising several subtypes. Three major categories in younger women are germ cell tumor, sex cord stromal tumor and epithelial ovarian neoplasia. OBJECTIVE: literature search was for an update on management of ovarian cancer in young women. CONTEXT: Germ cell tumor is suspected in young girls presenting with solid ovarian neoplasm as abdominal mass, discomfort, dyspnea or pain abdomen...
2015: Reviews on Recent Clinical Trials
Christina Wei, Elizabeth Clare Crowne
Delayed puberty, especially in boys, is a common presentation in paediatrics. Recent advances have improved our understanding of the neuroendocrine, genetic and environmental factors controlling pubertal development, and hence inform the pathophysiology of delayed puberty. The discovery of kisspeptin signalling through its receptor identified neuroendocrine mechanisms controlling the gonadotrophin-releasing hormone (GnRH) pulse generator at the onset of puberty. Genetic mechanisms from single gene mutations to single nucleotide polymorphism associated with delayed puberty are being identified...
May 2016: Archives of Disease in Childhood
Mikkel G Mieritz, Lars L Rakêt, Casper P Hagen, John E Nielsen, Maj-Lis M Talman, Jørgen H Petersen, Stefan H Sommer, Katharina M Main, Niels Jørgensen, Anders Juul
CONTEXT: Physiological gynecomastia is common and affects a large proportion of otherwise healthy adolescent boys. It is thought to be caused by an imbalance between estrogen and testosterone, although this is rarely evident in analyses of serum. OBJECTIVE: This study aimed to describe the frequency of physiological gynecomastia and to determine possible etiological factors (eg, auxology and serum hormone levels) in a longitudinal setup. DESIGN, SETTINGS, AND PARTICIPANTS: A prospective cohort study of 106 healthy Danish boys (5...
October 2015: Journal of Clinical Endocrinology and Metabolism
J Rohayem, E Nieschlag, S Kliesch, M Zitzmann
In pre-pubertal boys ≥ 14 years, the differentiation between constitutional delay of growth and puberty (CDGP) and hypogonadotropic hypogonadism (HH) is challenging, as current diagnostic tools have limitations in sensitivity and specificity. The aim of this study was to assess the usefulness of markers of gonadal activity, growth axis activation and adrenarche in differentiation between pre-pubertal CDGP and HH. This retrospective study was carried out between 2006 and 2015 in an academic out-patient referral centre...
September 2015: Andrology
Xing-Fa Han, Jun-Li Li, Yu-Qin Zhou, Xiao-Hua Ren, Gong-Cheng Liu, Xiao-Han Cao, Xiao-Gang Du, Xian-Yin Zeng
GnRH sterilization vaccines have been developed for various practical and clinical reasons. However, conjugation of GnRH peptide to carrier protein has many drawbacks, hampering the further commercialization of GnRH vaccines. In this study, a new nonconjugated GnRH vaccine, D-Lys6-GnRH-tandem-dimer peptide (TDK), emulsified in Specol adjuvant was investigated for its immunocastration efficacy in young male rats. Prepubertal male rats were randomly allocated into three groups (n = 12): control (no treatment), surgically castrated or immunized against 100 μg TDK in Specol adjuvant at 6 weeks of age (with a booster 8 weeks later)...
May 2016: Asian Journal of Andrology
Gerhard Binder, Roland Schweizer, Peter Haber, Gunnar Blumenstock, Regina Braun
OBJECTIVE: To assess the accuracy of inhibin B and the gonadotropin releasing hormone agonist test for the diagnosis of hypogonadotropic hypogonadism (HH). STUDY DESIGN: We performed a retrospective analysis of data collected 2009-2014 using a strict clinical protocol. All prepubertal nonunderweight girls, aged 13-17.5 years with Tanner breast stage B1/B2 and low estradiol levels, were tested and re-examined at 6-month intervals (n = 21). Constitutional delay of growth and puberty was defined by spontaneous menarche; HH was identified by association with specific causes of HH or no spontaneous progress of puberty during follow-up...
September 2015: Journal of Pediatrics
E M van der Plas, J van Brakel, A Meij-de Vries, S M P F de Muinck Keizer-Schrama, F W J Hazebroek, W W M Hack, G R Dohle
The aim of this study was to evaluate testicular function in men with previous acquired undescended testis (UDT) in whom orchiopexy was performed at diagnosis compared with a similar group of men in whom spontaneous descent was awaited until puberty. Secondly, we examined the influence of age at orchiopexy on fertility parameters in adult life. A total of 169 men of the 'orchiopexy at diagnosis' group and 207 men of the 'wait and see' protocol group were invited for participation. All participants underwent an andrological evaluation, including medical history, physical examination, scrotal ultrasound, determination of reproductive hormones, and semen analysis...
July 2015: Andrology
Ishwari Kadariya, Jiaxing Wang, Zia ur Rehman, Hamid Ali, Hasan Riaz, JiuYa He, Dinesh Bhattarai, Jia Jia Liu, Shu Jun Zhang
Inhibin α (INHα), a member of TGFβ superfamily, is an important modulator of reproductive function that plays a vital role in follicular changes, cell differentiation, oocyte development, and ultimately in mammalian reproduction. However, the role of inhibin α in female fertility and ovarian function remains largely unknown. To define its role in reproduction, transgenic mice of RNAi-INHα that knock down the INHα expression by shRNAi were used. Inhibin α subunit gene was knocked down successfully at both transcriptional and translational levels by RNAi PiggyBac transposon (Pbi) mediated recombinant pshRNA vectors and purified DNA fragments were microinjected into mouse zygotes...
August 2015: Journal of Steroid Biochemistry and Molecular Biology
Stine Aa Lunding, Lise Aksglaede, Richard A Anderson, Katharina M Main, Anders Juul, Casper P Hagen, Anette T Pedersen
CONTEXT: The majority of Turner syndrome (TS) patients suffer from accelerated loss of primordial follicles. Low circulating levels of anti-Müllerian hormone (AMH) may predict the lack of spontaneous puberty in prepubertal girls and imminent premature ovarian insufficiency (POI) in TS women with preserved ovarian function. OBJECTIVES: To evaluate the association between circulating AMH and ovarian status in TS patients. DESIGN: Longitudinal observational cohort study...
July 2015: Journal of Clinical Endocrinology and Metabolism
Catherine Itman, Amanda Bielanowicz, Hoey Goh, Queenie Lee, Alex J Fulcher, Sarah C Moody, James C G Doery, Jacinta Martin, Sarah Eyre, Mark P Hedger, Kate L Loveland
Activin production and signaling must be strictly regulated for normal testis development and function. Inhibins are potent activin inhibitors; mice lacking the inhibin-α gene (Inha-/- mice) cannot make inhibin and consequently have highly elevated activin and FSH serum concentrations and excessive activin signaling, resulting in somatic gonadal tumors and infertility. Dose-dependent effects of activin in testicular biology have been widely reported; hence, we hypothesized that male mice lacking one copy of the Inha gene would produce less inhibin and have an abnormal reproductive phenotype...
June 2015: Endocrinology
Audra L Gollenberg, O Yaw Addo, Zhiwei Zhang, Mary L Hediger, John H Himes, Peter A Lee
BACKGROUND/AIMS: Evidence is unclear whether prenatal smoking affects age at menarche and pubertal development, and its impact upon hormones has not been well studied. We aim to identify potential pathways through which prenatal smoking and environmental tobacco smoke (ETS) affect reproductive hormones in girls approaching puberty. METHODS: We examined the association between prenatal smoking, current ETS and luteinizing hormone (LH) and inhibin B (InB) in 6- to 11-year-old girls in the 3rd National Health and Nutrition Examination Survey, 1988-1994...
2015: Hormone Research in Pædiatrics
Marie-Anne Burckhardt, Verena Obmann, Rainer Wolf, Marco Janner, Christa E Flück, Primus E Mullis
BACKGROUND: Aromatase deficiency may result in a complete block of estrogen synthesis because of the failure to convert androgens to estrogens. In females, this results in virilisation at birth, ovarian cysts in prepuberty and lack of pubertal development but virilisation, thereafter. OBJECTIVE AND METHODS: We studied the impact of oral 17β-estradiol treatment on ovarian and uterine development, and on LH/FSH and inhibin B during the long-term follow-up of a girl harboring compound heterozygote point mutations in the CYP19A1 gene...
May 2015: Gynecological Endocrinology
Monica Interdonato, Gabriele Pizzino, Alessandra Bitto, Federica Galfo, Natasha Irrera, Anna Mecchio, Giovanni Pallio, Vincenzo Ramistella, Filippo De Luca, Angelo Santamaria, Letteria Minutoli, Herbert Marini, Francesco Squadrito, Domenica Altavilla
OBJECTIVE: Cadmium (Cd) has been shown to impair pubertal development in experimental animals. However, no data are available for male adolescents with increased urinary cadmium levels. DESIGN: The aim of this cross-sectional study was to evaluate pubertal onset and pituitary-gonadal axis hormones in male adolescents with increased urinary levels of Cd. SUBJECTS: We studied 111 males, aged 12-14 years living in the Milazzo-Valle del Mela area...
September 2015: Clinical Endocrinology
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