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https://www.readbyqxmd.com/read/28525352/evaluation-of-endocrine-and-metabolic-dysfunctions-after-hematopoietic-stem-cell-transplantation-in-children-a-study-from-turkey
#1
Dilek Gurlek Gokcebay, Fatih Azik, Cengiz Bayram, Arzu Yazal Erdem, Ali Fettah, Pamir Isik, Nese Yarali, Fatma Demirel, Bahattin Tunc, Namik Ozbek
BACKGROUND: Endocrine organs are highly susceptible to effects of high-dose chemotherapy. The objective of the study was to evaluate endocrine and metabolic complications after hematopoietic stem cell transplantation (HSCT) in children. METHODS: The patients who underwent HSCT in our center from April 2010 to October 2014 with at least 1 year follow-up were analyzed retrospectively. RESULTS: One-hundred children (M/F:59/41; mean age 8.9±4...
May 18, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28523940/delay-in-estrogen-commencement-is-associated-with-lower-bone-mineral-density-in-turner-syndrome
#2
H H Nguyen, P Wong, B J Strauss, G Jones, P R Ebeling, F Milat, A Vincent
OBJECTIVE: Turner syndrome (TS) is associated with hypogonadism, osteoporosis and fractures. We investigated the prevalence and risk factors for low bone density and fractures in a TS cohort. METHODS: We included 76 TS patients (median age 28.5 years) attending a tertiary hospital between 1998 and 2015 who underwent dual-energy X-ray absorptiometry. Spine and femoral neck (FN) areal bone mineral density (aBMD) were compared with those of a control group. To adjust for smaller bone size, bone mineral apparent density (BMAD) was calculated...
May 19, 2017: Climacteric: the Journal of the International Menopause Society
https://www.readbyqxmd.com/read/28511423/bardet-biedl-syndrome-a-report-of-two-cases-with-otolaryngologic-symptoms
#3
Mahendra K Singh, Shrinkhal, Sidharth Pradhan, Priyanko Chakraborty
Bardet-Biedl Syndrome (BBS) is a rare autosomal recessive disorder characterized primarily by rod-cone dystrophy, postaxial polydactyly, central obesity, mental retardation, hypogonadism, and renal dysfunction. We present two cases of this syndrome, both female, who presented with complaints of nyctalopia and mental retardation, and additionally one of them had sensorineural hearing loss while the other had serous otitis media. Hearing loss being a rare presentation is worth reporting. Both the patients were given a course of vitamin A and the parents were counseled regarding the prognosis and additional complications associated with the syndrome...
March 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28508601/effects-of-anorexia-nervosa-on-the-endocrine-system
#4
Charumathi Baskaran, Madhusmita Misra, Anne Klibanski
Anorexia nervosa (AN) is characterized by severe undernutrition associated with alterations in multiple endocrine axes, which are primarily adaptive to the state of caloric deprivation. Hormonal changes include growth hormone (GH) resistance with low insulin like growth factor-1 (IGF-1) levels, hypothalamic hypogonadism, relative hypercortisolemia and changes in appetite regulating hormones, including leptin, ghrelin, and peptide YY. These alterations contribute to abnormalities in bone metabolism leading to low bone mass, impaired bone microarchitecture, and increased risk for fracture, and may also negatively impact cognition, emotions and mood...
March 2017: Pediatric Endocrinology Reviews: PER
https://www.readbyqxmd.com/read/28505621/gnrha-treatment-of-cryptorchid-boys-affects-genes-involved-in-hormonal-control-of-the-hpg-axis-and-fertility
#5
Faruk Hadziselimovic, Katharina Gegenschatz-Schmid, Gilvydas Verkauskas, Philippe Demougin, Vytautas Bilius, Darius Dasevicius, Michael B Stadler
The gonadotropin-releasing hormone agonist (GnRHa; Buserelin) rescues fertility during adulthood in the majority of high infertility risk cryptorchid boys presenting with defective mini-puberty. However, the molecular events governing this effect are not understood. We report the outcome of an RNA profiling analysis of testicular biopsies from 4 operated patients who were treated with GnRHa for 6 months versus 3 operated controls who were not treated. GnRHa induces a significant transcriptional response, including protein-coding genes involved in pituitary development, the hypothalamic-pituitary-gonadal axis, and testosterone synthesis...
May 16, 2017: Sexual Development: Genetics, Molecular Biology, Evolution, Endocrinology, Embryology, and Pathology of Sex Determination and Differentiation
https://www.readbyqxmd.com/read/28497912/testosterone-replacement-therapy-long-term-safety-and-efficacy
#6
REVIEW
Giovanni Corona, Alessandra Sforza, Mario Maggi
Recent position statements and guidelines have raised the distinction between a true and false, age-related hypogonadism (HG) or late-onset hypogonadism (LOH). The former is the consequence of congenital or acquired "organic" damage of the brain centers or of the testis. The latter is mainly secondary to age-related comorbidities and does not require testosterone (T) therapy (TTh). In addition, concerns related to cardiovascular (CV) safety have further increased the scepticism related to TTh. In this paper, we reviewed the available evidence supporting the efficacy of TTh in non-organic HG and its long term safety...
April 30, 2017: World Journal of Men's Health
https://www.readbyqxmd.com/read/28497534/effects-of-testosterone-replacement-therapy-on-metabolic-syndrome-among-japanese-hypogonadal-men-a-subanalysis-of-a-prospective-randomised-controlled-trial-earth-study
#7
K Shigehara, H Konaka, T Nohara, K Izumi, Y Kitagawa, Y Kadono, T Iwamoto, E Koh, A Mizokami, M Namiki
We investigated the effects of testosterone replacement therapy (TRT) on metabolic factors among hypogonadal men with a metabolic syndrome. From the study population of the EARTH study, which was a randomised controlled study in Japan, 65 hypogonadal patients with a metabolic syndrome, comprising the TRT group (n = 32) and controls (n = 33), were included in this study analysis. The TRT group was administered 250 mg of testosterone enanthate as an intramuscular injection every 4 weeks for 12 months...
May 12, 2017: Andrologia
https://www.readbyqxmd.com/read/28495861/testosterone-treatment-and-cardiovascular-and-venous-thromboembolism-risk-what-is-new
#8
G Corona, M Dicuio, G Rastrelli, E Maseroli, F Lotti, A Sforza, M Maggi
In men, testosterone (T) production declines as a function of ageing. Late-onset hypogonadism (LOH) is the most commonly used term to indicate this age-related condition. In LOH, the relative clinical significance and the potential benefit of testosterone treatment (TTh) are still the subject of strong criticisms in the scientific community. The debate is further complicated by the recent position statement of the US Food and Drug Administration (FDA) emphasizing that, in LOH, the benefits and safety of TTh have not been fully established...
May 10, 2017: Journal of Investigative Medicine: the Official Publication of the American Federation for Clinical Research
https://www.readbyqxmd.com/read/28493439/very-late-presentation-of-a-disorder-of-sex-development
#9
J M Martins, M Fraga, J Miguens, F Tortosa, B Marques, A D Sousa
Disorders of sex development generally present in the neonatal period with ambiguity of external genitalia. We report a very old male patient presenting at 75 years because of panhypopituitarism and a large nonsecreting pituitary macroadenoma secondary to long-standing primary hypogonadism due to 46,XX sex reversal disorder now first diagnosed. Sex development disorders may go unrecognised for the entire life span, despite infertility and long-standing primary gonadic failure may lead to uncommon complications...
May 11, 2017: Andrologia
https://www.readbyqxmd.com/read/28490722/response-to-comment-on-hypogonadotrophic-hypogonadism-due-to-a-mutation-in-the-luteinizing-hormone-%C3%AE-subunit-gene
#10
Sung-Hee Ihm
No abstract text is available yet for this article.
May 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/28490716/comment-on-hypogonadotrophic-hypogonadism-due-to-a-mutation-in-the-luteinizing-hormone-%C3%AE-subunit-gene
#11
Hernan Valdes-Socin, Adrian F Daly, Albert Beckers
No abstract text is available yet for this article.
May 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/28487784/respiratory-failure-due-to-severe-obesity-and-kyphoscoliosis-in-a-24-year-old-male-with-molecularly-confirmed-prader-willi-syndrome-in-tertiary-hospital-in-northern-tanzania
#12
Elichilia R Shao, Lucas F Kiyegi, Amos O Mwasamwaja, Kajiru Kilonzo, Ben C J Hamel
Obesity, mild intellectual disability, hypotonia, poor sucking, cryptorchidism in males, hypogonadism, and kyphoscoliosis are common features of Prader-Willi syndrome (PWS). We report a case who had severe respiratory complications due to extreme obesity and kyphoscoliosis, which are important causes of morbidity and mortality, and discuss management. Furthermore, this is the first molecularly confirmed PWS case in Sub-Saharan Africa outside South Africa.
2017: Case Reports in Genetics
https://www.readbyqxmd.com/read/28486593/prevalence-of-obesity-associated-gonadal-dysfunction-in-severely-obese-men-and-women-and-its-resolution-after-bariatric-surgery-a-systematic-review-and-meta-analysis
#13
Hector F Escobar-Morreale, Elisa Santacruz, Manuel Luque-Ramírez, José I Botella Carretero
BACKGROUND: Sexual dimorphism manifests noticeably in obesity-associated gonadal dysfunction. In women, obesity is associated with androgen excess disorders, mostly the polycystic ovary syndrome (PCOS), whereas androgen deficiency is frequently present in obese men in what has been termed as male obesity-associated secondary hypogonadism (MOSH). Obesity-associated gonadal dysfunction, consisting of PCOS in women and MOSH in men, is a frequent finding in patients with severe obesity and it may be ameliorated or even resolve with marked weight loss, especially after bariatric surgery...
May 9, 2017: Human Reproduction Update
https://www.readbyqxmd.com/read/28486441/bone-mineral-density-in-patients-with-inherited-bone-marrow-failure-syndromes
#14
Roopa Kanakatti Shankar, Neelam Giri, Maya B Lodish, Ninet Sinaii, James C Reynolds, Sharon A Savage, Constantine A Stratakis, Blanche P Alter
BACKGROUNDPatients with inherited bone marrow failure syndromes (IBMFS) may have several risk factors for low bone mineral density (BMD). We aimed to evaluate the prevalence of low BMD in IBMFS and determine associated risk factors.METHODSPatients with IBMFS with at least one Dual Energy X-ray absorptiometry (DXA) scan were evaluated. Diagnosis of each IBMFS, Fanconi anemia (FA), dyskeratosis congenita, Diamond-Blackfan anemia, and Shwachman-Diamond syndrome, was confirmed by syndrome-specific tests. Data were gathered on age, height and clinical history...
May 9, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28485664/controlled-ovarian-stimulation-and-ivf-pregnancy-in-a-trisomy-x-carrier-with-associated-hypogonadotropic-hypogonadism
#15
Claudia Massarotti, Patrizia Fiorio, Roberto Gastaldi, Lucia Rosaia De Santis, Daniela Pastorino, Valentino Remorgida, Paola Anserini
We describe successful controlled ovarian stimulation (COS) and the first known IVF pregnancy in a trisomy X carrier with associated hypogonadotropic hypogonadism (HH) linked to a chromosome 4 double mutation in the allele of the Gonadotropins Releasing Hormone receptor (GnRHr) gene. Previous administration of low dose of gonadotropins, as recommended in patients with HH, led to poor follicular recruitment. Since trisomy X is a risk factor for diminished ovarian reserve (DOR) and premature ovarian insufficiency (POI), higher doses of gonadotropins led to better ovarian response...
May 9, 2017: Gynecological Endocrinology
https://www.readbyqxmd.com/read/28479477/practice-patterns-in-the-diagnosis-and-management-of-hypogonadism-a-survey-of-smsna-members
#16
Faysal A Yafi, Nora M Haney, James Anaissie, Kenneth J DeLay, Landon Trost, Mohit Khera, Wayne J G Hellstrom
OBJECTIVE: To describe practice patterns in the diagnosis and treatment of hypogonadism, as the optimal approaches are controversial. Multiple therapeutic options are currently available for hypogonadal men and treatment patterns vary considerably. The safety of testosterone therapy (TTh) remains understudied. METHODS: A 23-question survey regarding diagnosis and treatment of hypogonadism was sent to all members of the Sexual Medicine Society of North America (SMSNA)...
May 4, 2017: Urology
https://www.readbyqxmd.com/read/28479217/physiological-and-psychological-effects-of-testosterone-during-severe-energy-deficit-and-recovery-a-study-protocol-for-a-randomized-placebo-controlled-trial-for-optimizing-performance-for-soldiers-ops
#17
Stefan M Pasiakos, Claire E Berryman, J Philip Karl, Harris R Lieberman, Jeb S Orr, Lee M Margolis, John A Caldwell, Andrew J Young, Monty A Montano, William J Evans, Oshin Vartanian, Owen T Carmichael, Kishore M Gadde, Melissa Harris, Jennifer C Rood
BACKGROUND: The physiological consequences of severe energy deficit include hypogonadism and the loss of fat-free mass. Prolonged energy deficit also impacts physical performance, mood, attentiveness, and decision-making capabilities. This study will determine whether maintaining a eugonadal state during severe, sustained energy deficit attenuates physiological decrements and maintains mental performance. This study will also assess the effects of normalizing testosterone levels during severe energy deficit and recovery on gut health and appetite regulation...
May 4, 2017: Contemporary Clinical Trials
https://www.readbyqxmd.com/read/28476224/molecular-and-genetic-aspects-of-congenital-isolated-hypogonadotropic-hypogonadism
#18
REVIEW
Lorena Guimaraes Lima Amato, Ana Claudia Latronico, Leticia Ferreira Gontijo Silveira
Congenital isolated hypogonadotropic hypogonadism (IHH) is a clinically and genetically heterogenous disorder characterized by abnormal synthesis, secretion, or action of gonadotropin-releasing hormone, a key hypothalamic decapeptide that orchestrates the reproductive axis. Several modes of inheritance have been identified. A growing list of causative genes has been implicated in the molecular pathogenesis of syndromic and nonsyndromic IHH, largely contributing for better understanding the complex neuroendocrine control of reproduction...
June 2017: Endocrinology and Metabolism Clinics of North America
https://www.readbyqxmd.com/read/28472914/low-testosterone-levels-and-metabolic-syndrome-in-aging-male
#19
Rodrigo Blaya, Patrícia Blaya, Luiza Rhoden, Ernani Luis Rhoden
Advances in medicine and progressive improvements in the health of the general population in developed countries have led to considerable increases in life expectancy over recent years. Metabolic syndrome (MetS) affects approximately 25% of the adult population and its prevalence is increasing all over the world. Central obesity plays an important role in emergence of MetS. Some studies have suggested that there may be link between low Total Testosterone levels and emergence of MetS. The objective of this review is to analyze the complex network of interactions between MetS and low testosterone levels in elderly men, considering etiologic, diagnostic, and therapeutic aspects...
May 3, 2017: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/28470687/correlates-and-prevalence-of-hypogonadism-in-patients-with-early-and-late-onset-type-2-diabetes
#20
Y Li, M Zhang, X Liu, W Cui, S Rampersad, F Li, Z Lin, P Yang, H Li, C Sheng, X Cheng, S Qu
This study aims to compare the prevalence of hypogonadism between male patients with early-onset type 2 diabetes mellitus (T2DM) and late-onset type 2 diabetes. A total of 122 male patients with early-onset T2DM (diagnosis age ≤40 years) and 100 male patients with late-onset T2DM (diagnosis age >40 years) were recruited from our in-patient department between 1 January 2013 and 28 December 2015. Serum FSH, LH, testosterone, lipid profile, uric acid, HbA1c, and beta-cell function were determined in blood samples...
May 3, 2017: Andrology
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