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Growth hormone deficiency

Susana Gonzalez, Thozhukat Sathyapalan, Zeeshan Javed, Stephen L Atkin
Objective: The aim of this study is to evaluate the effect of growth hormone therapy (rGH) on mitochondrial function on peripheral muscle and to correlate with exercise capacity in subjects with severe adult growth hormone deficiency (GHD). Design: Six months, double-blind, randomized, crossover, placebo-controlled trial of subcutaneous rGH in 17 patients with GHD. Measurements: Quadriceps muscle biopsies were obtained at baseline, 3 months, and 6 months to measure succinate dehydrogenase (SDH) to assess mitochondrial activity...
2018: Frontiers in Endocrinology
Greisa Vila, Anton Luger
Growth hormone (GH) is not approved for use during conception and pregnancy. Nevertheless, data from the clinical care practice reveal that most women concieve on GH replacement therapy (GHRT), and more than half continue on GHRT also during pregnancy. GH stimulates the hypothalamic-pituitary-gonadal axis at all levels, and there is evidence that GH deficiency impacts the morphology of reproductive organs, onset of puberty, ovarian function and fertility. Patients with hypopituitarism often conceive using assisted reproductive techniques and several studies support the benefit of GH supplementation for achieving fertility in women with growth hormone deficiency...
March 8, 2018: Minerva Endocrinologica
J P Zhang, Q H Guo, Y M Mu, Z H Lyu, W J Gu, G Q Yang, J Du, J M Ba, J M Lu
Objective: To evaluate the clinical characteristics and etiologies of central diabetes insipidus (CDI). Methods: The clinical data of 230 patients with CDI in the Department of Endocrinology of Chinese PLA General Hospital from 2008 June to 2014 December were collected and analyzed retrospectively. Results: The three most common causes of CDI were idiopathic CDI, lymphocytic hypophysitis and intracranial germ cell tumors. Among all the CDI, the idiopathic CDI accounted for 37.48%. There were significant differences in age onset and gender distribution among the different causes of CDI...
March 1, 2018: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
Arundhati Undurti, Elizabeth A Colasurdo, Carl L Sikkema, Jaclyn S Schultz, Elaine R Peskind, Kathleen F Pagulayan, Charles W Wilkinson
The most frequent injury sustained by US service members deployed to Iraq or Afghanistan is mild traumatic brain injuries (mTBI), or concussion, by far most often caused by blast waves from improvised explosive devices or other explosive ordnance. TBI from all causes gives rise to chronic neuroendocrine disorders with an estimated prevalence of 25-50%. The current study expands upon our earlier finding that chronic pituitary gland dysfunction occurs with a similarly high frequency after blast-related concussions...
2018: Frontiers in Neurology
Hiroki Goto, Miki Nishio, Yoko To, Tatsuya Oishi, Yosuke Miyachi, Tomohiko Maehama, Hiroshi Nishina, Haruhiko Akiyama, Tak Wah Mak, Yuma Makii, Taku Saito, Akihiro Yasoda, Noriyuki Tsumaki, Akira Suzuki
Hippo signaling is modulated in response to cell density, external mechanical forces, or rigidity of the extracellular matrix (ECM). The Mps one binder kinase activator (MOB) adaptor proteins are core components of Hippo signaling and have important effects on Yes-associated protein-1 (YAP1) and transcriptional co-activator with PDZ-binding motif (TAZ), which are potent transcriptional regulators. YAP1/TAZ are key contributors to cartilage and bone development but the molecular mechanisms by which the Hippo pathway controls chondrogenesis are largely unknown...
March 6, 2018: Development
Eugènia Moix Gil, Olga Giménez-Palop, Assumpta Caixàs
INTRODUCTION: The Prader-Willi syndrome (PWS) is a rare genetic disorder caused by absence of expression of the paternal alleles in región 15q11.2-q13. Obesity and hormonal deficiencies, especially of growth hormone (GH), are the most important signs from the therapeutic viewpoint. Recombinant GH (rGH) is effective in children and represents the mainstay in treatment; by contrast, little evidence in available in adult patients. OBJECTIVE: To review the reported evidence on the beneficial and adverse effects of treatment with rGH in children and adults...
March 3, 2018: Endocrinología, Diabetes y Nutrición
Marie-José Lecomte, Chloé Bertolus, Nélina Ramanantsoa, Françoise Saurini, Jacques Callebert, Catherine Sénamaud-Beaufort, Maud Ringot, Thomas Bourgeois, Boris Matrot, Corinne Collet, Jeannette Nardelli, Jacques Mallet, Guilan Vodjdani, Jorge Gallego, Jean-Marie Launay, Sylvie Berrard
Pituitary growth hormone (GH) and insulin-like growth factor-1 (IGF-1) are anabolic hormones whose physiological roles are particularly important during development. The activity of the GH/IGF-1 axis is controlled by complex neuroendocrine systems including two hypothalamic neuropeptides, GH-releasing hormone (GHRH) and somatostatin (SRIF), and a gastrointestinal hormone, ghrelin. The neurotransmitter acetylcholine (ACh) is involved in tuning GH secretion, and its GH-stimulatory action has mainly been shown in adults but is not clearly documented during development...
March 2, 2018: Endocrinology
Netsiri Dumrongpisutikul, Ammarut Chuajak, Sukalaya Lerdlum
BACKGROUND: Magnetic resonance imaging (MRI) is used for neuroradiologic evaluation of patients with idiopathic growth hormone deficiency (IGHD). OBJECTIVES: To compare pituitary height and morphology at MRI between patients with IGHD and controls. MATERIALS AND METHODS: This retrospective study was conducted in pediatric patients, 3 years-15 years old, who had had brain MRI with non-contrast-enhanced midsagittal T1-weighted images. These images were measured for pituitary height and morphology of the pituitary gland including shape, stalk and posterior pituitary bright spot was evaluated...
March 6, 2018: Pediatric Radiology
Tzu-Chun Tsai, Chun-Che Shih, Hsin-Ping Chien, An-Hang Yang, Jenn-Kan Lu, Jen-Her Lu
BACKGROUND: Tbx5 deficiency in zebrafish causes several abnormal phenotypes of the heart and pectoral fins. It has been reported that exogenous human growth hormone can enhance expression of downstream mediators in the growth hormone and insulin-like growth factor I (IGF-I) pathway and partially restore dysmorphogenesis in tbx5 morphants. This study aimed to further evaluate the effects of IGF-I on cell apoptosis and dysmorphogenesis in zebrafish embryos deficient for tbx5. RESULTS: Among the five studied groups of zebrafish embryos (wild-type embryos [WT], tbx5 morphants [MO], mismatched tbx5 morpholino-treated wild-type embryos [MIS], IGF-I-treated wild-type embryos [WTIGF1], and IGF-I-treated tbx5 morphants [MOIGF1]), the expression levels of the ifg1, igf1-ra, ifg-rb, erk1, and akt2 genes as well as the ERK and AKT proteins were significantly reduced in the MO group, but were partially restored in the MOIGF1 group...
March 5, 2018: BMC Developmental Biology
Philippe Hernigou, Jean Charles Auregan, Arnaud Dubory
The vitamin D history started early in the evolution of life (billion years ago) as a photochemical reaction producing an inert molecule. During the early evolution of vertebrates, this molecule became essential for calcium and bone homeostasis of terrestrial animals and arrived to the status of hormone. Phytoplankton, zooplankton, and most plants and animals that are exposed to sunlight have the capacity to make vitamin D. Vitamin D is critically important for the development, growth, and maintenance of a healthy skeleton from birth until death...
March 5, 2018: International Orthopaedics
Gudmundur Johannsson, Ulla Feldt-Rasmussen, Ida Holme Håkonsson, Henrik Biering, Patrice Rodien, Shigeyuki Tahara, Andrew A Toogood, Michael Højby Rasmussen
OBJECTIVE: Somapacitan is a reversible albumin-binding growth hormone (GH) derivative, developed for once-weekly administration. This study aimed to evaluate the safety of once-weekly somapacitan vs once-daily Norditropin® Local tolerability and treatment satisfaction were also assessed. DESIGN: 26-week randomized, controlled phase 3 safety and tolerability trial in six countries (NCT02382939). METHODS: Male or female patients aged 18-79 years with adult GH deficiency (AGHD), treated with once-daily GH for ≥6 months, were randomized to once-weekly somapacitan (n = 61) or once-daily Norditropin® (n = 31) administered subcutaneously by pen...
March 2, 2018: European Journal of Endocrinology
Sumana Chatterjee, Lucy Shapiro, Stephen J Rose, Talat Mushtaq, Peter E Clayton, Svetlana Ten, Amrit Bhangoo, Uma Kumbattae, Renuka Dias, Martin O Savage, Louise A Metherell, Helen L Storr
BACKGROUND: Patients with homozygous intronic pseudoexon GH receptor(GHR) mutations(6Ψ) have growth hormone Insensitivity(GHI) (growth failure, IGF1 deficiency and normal/elevated serum GH). We report 9 patients in addition to previously described 11 GHR 6Ψ patients and their responses to rhIGF1 therapy. METHODS: 20 patients (12 males, 11 families, mean age 4.0±2.2yrs) were diagnosed genetically in our centre. Phenotypic data and responses to rhIGF1 treatment were provided by referring clinicians...
March 2, 2018: European Journal of Endocrinology
Raluca Maria Furnica, Julie Lelotte, Thierry Duprez, Dominique Maiter, Orsalia Alexopoulou
A 26-year-old woman presented with severe postpartum headaches. Magnetic resonance imaging (MRI) revealed a symmetric, heterogeneous enlargement of the pituitary gland. Three months later, she developed central diabetes insipidus. A diagnosis of postpartum hypophysitis was suspected and corticosteroids were prescribed. Six months later, the pituitary mass showed further enlargement and characteristics of a necrotic abscess with a peripheral shell and infiltration of the hypothalamus. Transsphenoidal surgery was performed, disclosing a pus-filled cavity which was drained...
2018: Endocrinology, Diabetes & Metabolism Case Reports
John M McCarthy, Bonnie M McCann-Crosby, Megan E Rech, Jiani Yin, Chun-An Chen, May A Ali, HaiThuy N Nguyen, Jennifer L Miller, Christian P Schaaf
BACKGROUND: Nonsense and frameshift mutations in the maternally imprinted, paternally expressed gene MAGEL2, located in the Prader-Willi critical region 15q11-15q13, have been reported to cause Schaaf-Yang syndrome (SYS), a genetic disorder that manifests as developmental delay/intellectual disability, hypotonia, feeding difficulties and autism spectrum disorder. Prader-Willi syndrome (PWS) is a genetic disorder characterised by severe infantile hypotonia, hypogonadotrophic hypogonadism, early childhood onset obesity/hyperphagia, developmental delay/intellectual disability and short stature...
March 1, 2018: Journal of Medical Genetics
Agathocles Tsatsoulis
Thyroid follicular cells, as well as adrenocortical cells, are endowed by an intrinsic heterogeneity regarding their growth potential, in response to various stimuli. This heterogeneity appears to constitute the underlying cause for the focal cell hyperplasia and eventually the formation of thyroid and adrenal nodules, under the influence of growth stimulatory factors. Among the main stimulatory factors are the pituitary tropic hormones, thyroid-stimulating hormone (TSH) or thyrotropin and adrenocorticotropic hormone (ACTH), which regulate the growth and function of their respective target cells, and the insulin/insulin-like growth factor system, that, through its mitogenic effects, can stimulate the proliferation of these cells...
February 26, 2018: Journal of Clinical Medicine
Mitchell E Geffner
Although metabolic outcomes may be aided by dual therapy with GI and IGF-I, the one published study of the combination approach to treat children with non-GH-deficient short stature showed only a meager additional height response compared to that achieved with GH alone.
March 2018: Pediatric Endocrinology Reviews: PER
G Johannsson, K Nespithal, U Plöckinger, V Alam, M McLean
PURPOSE: To investigate whether a new liquid formulation of recombinant human growth hormone (r-hGH) induces the production of binding antibodies (BAbs) in adults with congenital or adult-onset growth hormone deficiency (GHD). METHODS: Men or women aged 19-65 years with adult growth hormone deficiency who were r-hGH-naïve or had stopped treatment ≥ 1 month before screening were treated with between 0.15 and 0.30 mg/day r-hGH liquid formulation for 39 weeks...
February 27, 2018: Journal of Endocrinological Investigation
Fatemeh Bagheripuor, Mahboubeh Ghanbari, Abass Piryaei, Asghar Ghasemi
NEW FINDINGS: What is the central question of this study? Does fetal hypothyroidism in rats alter uterine contractions and structure in the adult offspring? What is the main finding and its importance? Our study indicated that maternal hypothyroidism during pregnancy increased gestational length and decreased litter size as well as delayed puberty onset; in addition, maternal hypothyroidism caused irregular uterine contractions and histological changes in the uterus in the female offspring...
February 27, 2018: Experimental Physiology
Sebastian Silva, Noriko Miyake, Carolina Tapia, Naomichi Matsumoto
Prolyl endopeptidase-like (PREPL) deficiency (MIM# 616224) is a rare autosomal recessive inherited congenital myasthenic syndrome characterized by neonatal hypotonia, feeding problems, mild dysmorphism, and neuromuscular symptoms, followed by hyperphagia and obesity in later childhood. Some patients also exhibit growth deficits, sexual hormone deficiency, and cognitive impairments. This syndrome is caused by biallelic mutations in PREPL. To date, only one nucleotide deletion and seven small microdeletions in PREPL have been reported...
February 26, 2018: Journal of Human Genetics
Gudmundur Johannsson, Martin Bidlingmaier, Beverly Biller, Margaret Boguszewski, Felipe F Casanueva, Philippe Chanson, Catherine S Choong, Peter E Clayton, David Clemmons, Mehul Dattani, Jan Frystyk, Ken K Y Ho, Andrew Hoffman, Reiko Horikawa, Anders Juul, John Kopchick, Xiaoping Luo, Sebastian Neggers, Irène Netchine, Daniel S Olsson, Sally Radovick, Ron G Rosenfeld, Richard J Ross, Katharina Schilbach, Paulo Ferrez Collett-Solberg, Christian J Strasburger, Peter J Trainer, Kerstin Wickstrom, Kevin Cj Yuen, Jens Otto Lunde Jørgensen
OBJECTIVE: The Growth Hormone Research Society (GRS) convened a Workshop in 2017 to evaluate clinical endpoints, surrogate endpoints and biomarkers during GH treatment of children and adults, and in patients with acromegaly. PARTICIPANTS: GRS invited 34 international experts including clinicians, basic scientists, a regulatory scientist and physicians from the pharmaceutical industry. EVIDENCE: Current literature was reviewed and expert opinion was utilized to establish the state of the art, and identify current gaps and unmet needs...
February 26, 2018: Endocrine Connections
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