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Growth hormone deficiency

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https://www.readbyqxmd.com/read/29353275/endocrine-and-metabolic-disturbances-in-survivors-of-hematopoietic-stem-cell-transplantation-in-childhood-and-adolescence
#1
Shlomit Shalitin, Lihi Pertman, Michal Yackobovitch-Gavan, Isaac Yaniv, Yael Lebenthal, Moshe Phillip, Jerry Stein
BACKGROUND/AIMS: The objective was to evaluate endocrine complications in survivors of hematopoietic stem cell transplantation (HSCT) performed during childhood. METHODS: Endocrine dysfunction and metabolic syndrome parameters were assessed by chart review of 178 childhood HSCT survivors (median age at evaluation, 15.5 [range: 3.8-29.8] years; median follow-up, 8.5 [range: 2-23.4] years). RESULTS: The following statistically significant associations were identified (p < 0...
January 19, 2018: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/29353264/response-to-growth-hormone-treatment-in-very-young-patients-with-growth-hormone-deficiencies-and-mini-puberty
#2
Semra Çetinkaya, Şükran Poyrazoğlu, Firdevs Baş, Oya Ercan, Metin Yıldız, Erdal Adal, Abdullah Bereket, Saygın Abalı, Zehra Aycan, Şenay Savaş Erdeve, Merih Berberoğlu, Zeynep Şıklar, Meltem Tayfun, Şükran Darcan, Eda Mengen, İffet Bircan, Filiz Mine Çizmecioğlu Jones, Enver Şimşek, Esra Deniz Papatya, Mehmet Nuri Özbek, Semih Bolu, Ayhan Abacı, Muammer Büyükinan, Feyza Darendeliler
BACKGROUND: The aim of the study was to assess the response to growth hormone (GH) treatment in very young patients with GH deficiency (GHD) through a national, multi-center study. Possible factors affecting growth response were assessed (especially mini-puberty). METHODS: Medical reports of GHD patients in whom treatment was initiated between 0 and 3 years of age were retrospectively evaluated. RESULTS: The cohort numbered 67. The diagnosis age was 12...
January 20, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29352447/long-term-endocrine-effects-and-trends-in-body-mass-index-changes-in-patients-with-childhood-onset-brain-tumors
#3
Go Hun Seo, Jin-Ho Choi, Yoon-Myung Kim, Kyung-Nam Koh, Ho Joon Im, Young Shin Ra, Han-Wook Yoo
As survival rates have improved owing to advances in management strategies for pediatric brain tumors, long-term complications such as endocrine dysfunction, have emerged as a major issue. This study investigated the long-term endocrine effects of childhood-onset brain tumors in a large number of patients. This study included 151 patients with brain tumors diagnosed between January 1995 and December 2016. The following data were retrospectively reviewed: tumor location, tumor histology, endocrine abnormalities, hypothalamic involvement on brain imaging, treatment modalities, and trends in body mass index...
January 19, 2018: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/29343946/a-new-pen-device-for-injection-of-recombinant-human-growth-hormone-a-convenience-functionality-and-usability-evaluation-study
#4
Maritta Sauer, Carole Abbotts
Purpose: Adherence to recombinant human growth hormone (r-hGH) is critical to growth and other outcomes in patients with growth disorders, but the requirement for daily injections means that ease of use is an important factor. This study assessed the perceived ease of use and functionality of the prototype of a reusable pen injector (pen device) for r-hGH that incorporates several advanced features. Participants and methods: Semi-structured 60-minute qualitative interviews were conducted in 5 countries with 57 health care professionals (HCPs) and 30 patients with GH deficiency/caregivers administering r-hGH to patients, including children...
2018: Patient Preference and Adherence
https://www.readbyqxmd.com/read/29343614/suppressor-of-cytokine-signaling-2-socs2-deletion-protects-bone-health-of-mice-with-dss-induced-inflammatory-bowel-disease
#5
Ross Dobie, Vicky E MacRae, Chloe Pass, Elspeth M Milne, S Faisal Ahmed, Colin Farquharson
Individuals with inflammatory bowel disease (IBD) often present with poor bone health. The development of targeted therapies for this bone loss requires a fuller understanding of the underlying cellular mechanisms. Although bone loss in IBD is multifactorial, the altered sensitivity and secretion of growth hormone (GH) and insulin-like growth factor-1 (IGF-1) in IBD is understood to be a critical contributing mechanism. The expression of suppressor of cytokine signaling 2 (SOCS2), a well-established negative regulator of GH signaling, is stimulated by proinflammatory cytokines...
January 17, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29339473/identification-of-thioredoxin-interacting-protein-txnip-as-a-downstream-target-for-igf1-action
#6
Karthik Nagaraj, Lena Lapkina-Gendler, Rive Sarfstein, David Gurwitz, Metsada Pasmanik-Chor, Zvi Laron, Shoshana Yakar, Haim Werner
Laron syndrome (LS), or primary growth hormone (GH) insensitivity, is the best-characterized entity among the congenital insulin-like growth factor 1 (IGF1) deficiencies. Life-long exposure to minute endogenous IGF1 levels is linked to low stature as well as a number of endocrine and metabolic abnormalities. While elevated IGF1 is correlated with increased cancer incidence, epidemiological studies revealed that patients with LS do not develop tumors. The mechanisms associated with cancer protection in LS are yet to be discovered...
January 16, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29330548/shox-haploinsufficiency-presenting-with-isolated-short-long-bones-in-the-second-and-third-trimester
#7
Shwetha Ramachandrappa, Abhijit Kulkarni, Hina Gandhi, Cheryl Ellis, Renata Hutt, Lesley Roberts, Rosol Hamid, Aris Papageorghiou, Sahar Mansour
Haploinsufficiency of the transcription factor short stature homeobox (SHOX) manifests as a spectrum of clinical phenotypes, ranging from disproportionate short stature and Madelung deformity to isolated short stature. Here, we describe five infants with molecularly confirmed diagnoses of SHOX haploinsufficiency who presented in utero with short long bones during routine antenatal scanning from as early as 19 weeks gestation. Other foetal growth parameters were normal. The molecular basis of SHOX haploinsufficiency was distinct in each case...
January 12, 2018: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/29329447/congenital-hyperinsulinism-and-hypopituitarism-attributable-to-a-novel-mutation-in-foxa2
#8
Mary Ellen Vajravelu, Jinghua Chai, Bryan Krock, Samuel Baker, David Langdon, Craig Alter, Diva D De León
Context: Persistent hypoglycemia in the newborn period most commonly occurs due to hyperinsulinism. The phenotype of hypoketotic hypoglycemia can also result from pituitary hormone deficiencies, including growth hormone and adrenocorticotropic hormone deficiency. Forkhead box A2 (Foxa2) is a transcription factor shown in mouse models to influence insulin secretion by pancreatic beta cells. In addition, Foxa2 is involved in regulation of pituitary development, and deletions of FOXA2 have been linked to panhypopituitarism...
January 10, 2018: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29329424/brassinosteroids-regulate-pavement-cell-growth-by-mediating-bin2-induced-microtubule-stabilization
#9
Xiaolei Liu, Qin Yang, Yuan Wang, Linhai Wang, Ying Fu, Xuelu Wang
Brassinosteroids (BRs), a group of plant steroid hormones, play important roles in regulating plant development. The cytoskeleton also affects key developmental processes and a deficiency in BR biosynthesis or signaling leads to abnormal phenotypes similar to those of microtubule-defective mutants. However, how BRs regulate microtubule and cell morphology remains unknown. Here, using liquid chromatography-tandem mass spectrometry, we identified tubulin proteins that interact with Arabidopsis BRASSINOSTEROID INSENSITIVE2 (BIN2), a negative regulator of BR responses in plants...
January 10, 2018: Journal of Experimental Botany
https://www.readbyqxmd.com/read/29328027/the-impact-of-12-month-growth-hormone-replacement-therapy-on-lipid-metabolism-and-adipose-tissue-distribution-in-georgian-patients-with-adult-growth-hormone-deficiency
#10
N Bostoganashvili, T Zerekidze, Sh Janjgava, M Lomidze
Growth hormone deficiency (GHD) is one of the reasons of significant metabolic morbidities inchildren and adults. The aim of our study was to evaluate the impact of growth hormone (GH) replacement therapy on lipid profile and adipose tissue distribution in adults with GHD. Twenty hypopituitary adults, aged 40.75±2.2 years (mean ± SE, range 20.5-60), with adult onset GHD (aGHD) were enrolled in a randomized, double blind, placebo-controlled study. 10 patients received recombinant growth hormone injection once weekly for 12 months, and the rest 10 patients (as control group) received placebo...
December 2017: Georgian Medical News
https://www.readbyqxmd.com/read/29328024/endometrial-polyps-in-women-of-reproductive-age-clinical-and-pathogene-tic-variations
#11
N Kosei, N Zakharenko, D Herman
The aim of the study was to study the relationship between the morphofunctional characteristics of the endometrium, hormonal homeostasis and microbiocenosis of the reproductive system in patients with endometrial polyps. The study involved 130 patients aged 18-35 years: 34 patients with endometrial polyps, 30 patients with micropolyps, 36 patients with endometrial polyps and micropolyps, 30 healthy women of the control group. Hysteroscopy was performed for women who had been suspected for endometrial polyps and who had infertility or repeated recurrent miscarriages...
December 2017: Georgian Medical News
https://www.readbyqxmd.com/read/29327380/effects-of-deficiency-and-surplus-dietary-threonine-on-reproductive-performance-of-primiparous-pregnant-gilts
#12
M Shi, T Liu, T Li, H Wang, T Yuan, D Li, J Wang
Dietary threonine imbalance is known to impair reproductive performances of gestating sows, but the underlying mechanisms are largely unknown. In this study, effects of deficiency and surplus dietary threonine during gestation on reproductive performance, serum metabolites and hormones concentration, and colostral nutrient and immunoglobulin contents of primiparous sows were investigated. Ninety primiparous pregnant gilts were assigned to one of the three dietary treatments with different standardized ileal digestible threonine/lysine ratios at 0...
January 11, 2018: Journal of Animal Physiology and Animal Nutrition
https://www.readbyqxmd.com/read/29324800/hepcidin-deficiency-and-iron-deficiency-do-not-alter-tuberculosis-susceptibility-in-a-murine-m-tb-infection-model
#13
Rachel Harrington-Kandt, Elena Stylianou, Lucy A Eddowes, Pei Jin Lim, Lisa Stockdale, Nawamin Pinpathomrat, Naomi Bull, Janet Pasricha, Marta Ulaszewska, Yulia Beglov, Sophie Vaulont, Hal Drakesmith, Helen McShane
Tuberculosis (TB), caused by the macrophage-tropic pathogen Mycobacterium tuberculosis (M.tb) is a highly prevalent infectious disease. Since an immune correlate of protection or effective vaccine have yet to be found, continued research into host-pathogen interactions is important. Previous literature reports links between host iron status and disease outcome for many infections, including TB. For some extracellular bacteria, the iron regulatory hormone hepcidin is essential for protection against infection...
2018: PloS One
https://www.readbyqxmd.com/read/29318530/a-novel-heterozygous-gli2-mutation-in-a-patient-with-congenital-urethral-stricture-and-renal-hypoplasia-dysplasia-leading-to-end-stage-renal-failure
#14
Toshihiko Shirakawa, Yumiko Nakashima, Satoshi Watanabe, Sadatomo Harada, Mariko Kinoshita, Toshiharu Kihara, Yuko Hamasaki, Seiichiro Shishido, Koh-Ichiro Yoshiura, Hiroyuki Moriuchi, Sumito Dateki
Glioblastoma 2 (GLI2) is a mediator of Sonic hedgehog signaling pathway that plays an important role in development of the central nervous system and limbs. Heterozygous GLI2 mutations have been associated with postaxial polydactyly, various pituitary dysfunction, and holoprosencephaly-like phenotype. Herein, we report a Japanese boy who presented with isolated growth hormone deficiency with ectopic posterior pituitary, postaxial polydactyly, atrioventricular septal defect, intellectual disability and dysmorphic facial features including mid-facial hypoplasia...
January 9, 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29318462/long-term-response-to-recombinant-human-growth-hormone-treatment-a-new-predictive-mathematical-method
#15
G Migliaretti, S Ditaranto, C Guiot, S Vannelli, P Matarazzo, N Cappello, I Stura, F Cavallo
INTRODUCTION: Recombinant GH has been offered to GH-deficient (GHD) subjects for more than 30 years, in order to improve height and growth velocity in children and to enhance metabolic effects in adults. AIM: The aim of our work is to describe the long-term effect of rhGH treatment in GHD pediatric patients, suggesting a growth prediction model. MATERIAL AND METHODS: A homogeneous database is defined for diagnosis and treatment modalities, based on GHD patients afferent to Hospital Regina Margherita in Turin (Italy)...
January 9, 2018: Journal of Endocrinological Investigation
https://www.readbyqxmd.com/read/29317355/identification-of-candidate-serum-biomarkers-of-childhood-onset-growth-hormone-deficiency-using-swath-ms-and-feature-selection
#16
Ignacio Ortea, Isabel Ruíz, Ramón Cañete, Javier Caballero-Villarraso, María Dolores Cañete
A typical clinical manifestation of growth hormone deficiency (GHD) is a short stature resulting from delayed growth, but GHD affects bone health, cardiovascular function and metabolic profile and therefore quality of life. Although early GH treatment during childhood has been shown to improve outcomes, no single biochemical parameter is currently available for the accurate diagnosis of GHD in children. There is hence a need for non-invasive biomarkers. In this study, the relative abundance of serum proteins from GHD children and healthy controls was measured by next-generation proteomics SWATH-MS technology...
January 6, 2018: Journal of Proteomics
https://www.readbyqxmd.com/read/29310723/iatrogenic-creutzfeldt-jakob-disease-with-amyloid-%C3%AE-pathology-an-international-study
#17
Ignazio Cali, Mark L Cohen, Stéphane Haїk, Piero Parchi, Giorgio Giaccone, Steven J Collins, Diane Kofskey, Han Wang, Catriona A McLean, Jean-Philippe Brandel, Nicolas Privat, Véronique Sazdovitch, Charles Duyckaerts, Tetsuyuki Kitamoto, Ermias D Belay, Ryan A Maddox, Fabrizio Tagliavini, Maurizio Pocchiari, Ellen Leschek, Brian S Appleby, Jiri G Safar, Lawrence B Schonberger, Pierluigi Gambetti
The presence of pathology related to the deposition of amyloid-β (Aβ) has been recently reported in iatrogenic Creutzfeldt-Jakob disease (iCJD) acquired from inoculation of growth hormone (GH) extracted from human cadaveric pituitary gland or use of cadaveric dura mater (DM) grafts.To investigate this phenomenon further, a cohort of 27 iCJD cases - 21 with adequate number of histopathological sections - originating from Australia, France, Italy, and the Unites States, were examined by immunohistochemistry, amyloid staining, and Western blot analysis of the scrapie prion protein (PrPSc), and compared with age-group matched cases of sporadic CJD (sCJD), Alzheimer disease (AD) or free of neurodegenerative diseases (non-ND)...
January 8, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29306560/treatment-of-growth-failure-in-the-absence-of-gh-signaling-the-ecuadorian-experience
#18
Jaime Guevara-Aguirre, Alexandra Guevara, Carolina Guevara
Recombinant human insulin-like growth factor-1 (rhIGF-1) treatment studies of growth failure in absence of growth hormone (GH) signaling (GH insensitivity -GHI, Laron syndrome -LS, GH Receptor deficiency -GHRD) have taken place in many locations around the globe. Results from these trials are comparable, and slight differences reported can be attributed to specific circumstances at different research sites. rhIGF-I treatment studies of GHI in Ecuador included various trials performed on children belonging to the largest and only homogeneous cohort of subjects with this condition in the world...
December 20, 2017: Growth Hormone & IGF Research
https://www.readbyqxmd.com/read/29300858/evidence-supporting-a-role-for-constitutive-ghrelin-receptor-signaling-in-fasting-induced-hyperphagia-in-male-mice
#19
Gimena Fernandez, Agustina Cabral, María F Andreoli, Alexandra Labarthe, Céline M'Kadmi, Jorge G Ramos, Jacky Marie, Jean-Alain Fehrentz, Jacques Epelbaum, Virginie Tolle, Mario Perello
Ghrelin is a potent orexigenic peptide hormone that acts through the growth hormone secretagogue receptor (GHSR), a G-protein-coupled receptor highly expressed in the hypothalamus. In vitro studies have shown GHSR displays a high constitutive activity, whose physiological relevance is uncertain. Since GHSR gene expression in the hypothalamus is known to increase in fasting conditions, we tested the hypothesis that constitutive GHSR activity at hypothalamic level drives the fasting-induced hyperphagia. We found refed wild-type (WT) mice displayed a robust hyperphagia that continued for 5 days after refeeding and changed their food intake daily pattern...
December 28, 2017: Endocrinology
https://www.readbyqxmd.com/read/29289483/challenges-and-future-for-the-delivery-of-growth-hormone-therapy
#20
Andrés Caicedo, Ron Rosenfeld
Growth hormone (GH) has multiple roles in sustaining human development and homeostasis. Its pulsatile secretion stimulates growth and contributes to an equilibrium in a process tightly regulated and coordinated by many organs. GH deficiency is a medical condition affecting all ages, with not only significant consequences in the health of the patient but also impact on the quality of life. This review gathers the different strategies used today with a glance at future technologies to treat GH deficiency. We present key aspects for consideration when developing new methods to deliver GH, mimicking or replacing its pulsatile activity...
December 17, 2017: Growth Hormone & IGF Research
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