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Growth hormone syndrome

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https://www.readbyqxmd.com/read/28930716/antinociceptive-effect-of-ghrelin-in-a-rat-model-of-irritable-bowel-syndrome-involves-trpv1-opioid-systems
#1
Yuqing Mao, Zhengyang Li, Kan Chen, Huafang Yu, Shaoren Zhang, Miao Jiang, Yuanhua Ma, Chunli Liang, Hongyan Liu, Huanqing Li, Qian Hua, Hao Zhou, Yonghong Sun, Xiaoming Fan
BACKGROUND/AIMS: Irritable bowel syndrome (IBS), defined as recurrent abdominal pain and changes in bowel habits, seriously affects quality of life and ability to work. Ghrelin is a brain-gut hormone, which has been reported to show antinociceptive effects in peripheral pain. We investigated the effect of ghrelin on visceral hypersensitivity and pain in a rat model of IBS. METHODS: Maternal deprivation (MD) was used to provide a stress-induced model of IBS in Wistar rats...
September 20, 2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/28928761/hormonal-regulation-in-shade-avoidance
#2
REVIEW
Chuanwei Yang, Lin Li
At high vegetation density, shade-intolerant plants sense a reduction in the red (660 nm) to far-red (730 nm) light ratio (R/FR) in addition to a general reduction in light intensity. These light signals trigger a spectrum of morphological changes manifested by growth of stem-like tissue (hypocotyl, petiole, etc.) instead of harvestable organs (leaves, fruits, seeds, etc.)-namely, shade avoidance syndrome (SAS). Common phenotypical changes related to SAS are changes in leaf hyponasty, an increase in hypocotyl and internode elongation and extended petioles...
2017: Frontiers in Plant Science
https://www.readbyqxmd.com/read/28928245/evi-and-mds-evi-are-required-for-adult-intestinal-stem-cell-formation-during-postembryonic-vertebrate-development
#3
Morihiro Okada, Yun-Bo Shi
The gene ectopic viral integration site 1 (EVI) and its variant myelodysplastic syndrome 1 (MDS)/EVI encode zinc-finger proteins that have been recognized as important oncogenes in various types of cancer. In contrast to the established role of EVI and MDS/EVI in cancer development, their potential function during vertebrate postembryonic development, especially in organ-specific adult stem cells, is unclear. Amphibian metamorphosis is strikingly similar to postembryonic development around birth in mammals, with both processes taking place when plasma thyroid hormone (T3) levels are high...
September 19, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/28926833/circulating-igf-i-igfbp-3-and-the-igf-i-igfbp-3-molar-ratio-concentration-and-height-outcome-in-prepubertal-short-children-on-rhgh-treatment-over-two-years-of-therapy
#4
María Gabriela Ballerini, Débora Braslavsky, Paula Alejandra Scaglia, Ana Keselman, María Eugenia Rodríguez, Alicia Martínez, Analía Verónica Freire, Horacio Mario Domené, Héctor Guillermo Jasper, Ignacio Bergadá, María Gabriela Ropelato
OBJECTIVE: To investigate the occurrence of abnormally elevated values of biomarkers of growth hormone (GH) action in short children on recombinant human GH (rhGH) therapy. METHODS: Sixty-three prepubertal short children were examined: 31 with GH deficiency (GHD), 25 small for gestational age (SGA), and 9 with Turner syndrome (TS). The main outcomes were the following: standard deviation score (SDS) values of IGF-I, IGFBP-3, and IGF-I/IGFBP-3 molar ratio before, at the 1st and at the 2nd year on rhGH and Δheight (Ht)-SDS to evaluate GH treatment efficacy (adequate 1st-year ΔHt SDS: >0...
September 19, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28925408/liver-volume-and-hepatic-adiposity-in-childhood-relations-to-body-growth-and-visceral-fat
#5
R Malpique, J Bassols, A López-Bermejo, M Diaz, F Villarroya, J Pavia, A Congo, F de Zegher, L Ibáñez
BACKGROUND AND OBJECTIVE: The sequence of prenatal growth restraint and postnatal catch-up growth may lead to hepato-visceral adiposity, insulin resistance and low-grade inflammation before the onset of puberty. In prepubertal children born appropriate for gestational age (AGA) or small for gestational age (SGA), we assessed potential relationships between the aforementioned sequence and liver volume. SUBJECTS/METHODS: The study population consisted of 86 children (41 AGA and 45 SGA with catch-up growth; age (mean±s...
August 14, 2017: International Journal of Obesity: Journal of the International Association for the Study of Obesity
https://www.readbyqxmd.com/read/28913408/hepatocyte-specific-deletion-of-mouse-lamin-a-c-leads-to-male-selective-steatohepatitis
#6
Raymond Kwan, Graham F Brady, Maria Brzozowski, Sujith V Weerasinghe, Hope Martin, Min-Jung Park, Makayla J Brunt, Ram K Menon, Xin Tong, Lei Yin, Colin L Stewart, M Bishr Omary
BACKGROUND & AIMS: Lamins are nuclear intermediate filament proteins that comprise the major components of the nuclear lamina. Mutations in LMNA, which encodes lamins A/C, cause laminopathies, including lipodystrophy, cardiomyopathy, and premature aging syndromes. However, the role of lamins in the liver is unknown, and it is unclear whether laminopathy-associated liver disease is caused by primary hepatocyte defects or systemic alterations. METHODS: To address these questions, we generated mice carrying a hepatocyte-specific deletion of Lmna (knockout [KO] mice) and characterized the KO liver and primary hepatocyte phenotypes by immunoblotting, immunohistochemistry, microarray analysis, quantitative real-time polymerase chain reaction, and Oil Red O and Picrosirius red staining...
November 2017: Cellular and Molecular Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28902629/individualised-growth-response-optimisation-igro-tool-an-accessible-and-easy-to-use-growth-prediction-system-to-enable-treatment-optimisation-for-children-treated-with-growth-hormone
#7
REVIEW
Jane Loftus, Anders Lindberg, Ferah Aydin, Roy Gomez, Mohamad Maghnie, Raoul Rooman, Heinz Steinkamp, Helmuth Doerr, Michael Ranke, Cecilia Camacho-Hubner
BACKGROUND: Growth prediction models (GPMs) exist to support clinical management of children treated with growth hormone (GH) for growth hormone deficiency (GHD), Turner syndrome (TS) and for short children born small for gestational age (SGA). Currently, no prediction system has been widely adopted. CONTENT: The objective was to develop a stand-alone web-based system to enable the widespread use of an 'individualised growth response optimisation' (iGRO) tool across European endocrinology clinics...
September 13, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28898905/taguchi-experimental-design-for-optimization-of-recombinant-human-growth-hormone-production-in-cho-cell-lines-and-comparing-its-biological-activity-with-prokaryotic-growth-hormone
#8
Zahra Sadat Aghili, Sayyed Hamid Zarkesh-Esfahani
Growth hormone deficiency results in growth retardation in children and the GH deficiency syndrome in adults and they need to receive recombinant-GH in order to rectify the GH deficiency symptoms. Mammalian cells have become the favorite system for production of recombinant proteins for clinical application compared to prokaryotic systems because of their capability for appropriate protein folding, assembly, post-translational modification and proper signal. However, production level in mammalian cells is generally low compared to prokaryotic hosts...
September 12, 2017: Drug Research
https://www.readbyqxmd.com/read/28872989/orthodontic-treatment-in-conjunction-with-twin-bock-treatment-and-growth-hormone-therapy-in-silver-russell-syndrome
#9
Su-Jin Ko, Ji Young Seo, Yong-Dae Kwon, Kyounga Cheon, Jae Hyun Park
Silver-Russell syndrome (SRS) is a very rare genetic disorder characterized by intrauterine growth retardation, short stature, and typical craniofacial abnormalities including micrognathia. While growth hormone (GH) therapy in children with SRS significantly improves somatic growth, functional orthopedic treatment can also be effective in adolescents with mandibular deficiency. We report the effects of Phase 1 functional orthopedic treatment of a twin-block appliance in conjunction with GH administration in a 9-year-old boy with GH deficiency and SRS, and the result of the subsequent Phase 2 orthodontic treatment...
2017: Journal of Clinical Pediatric Dentistry
https://www.readbyqxmd.com/read/28872123/ileectomy-induced-bile-overaccumulation-in-mouse-intestine
#10
Rongli Zhang, Jonathan W Ray, Mukesh K Jain, Shuxin Han
Intestinal resection is a common therapeutic approach for human diseases such as obesity, inflammatory bowel disease, Crohn's disease, and colon cancer that often results in severe short bowel syndrome-like adverse effects including bile acid diarrhea, dehydration, electrolyte disturbances, and nutrient malabsorption. Here we introduce a murine ileal resection model, termed ileectomy, to evaluate tissue communication and the maintenance of systemic homeostasis. After ileal resection, circulating blood is permanently devoid of the ileum-specific endocrine hormone fibroblast growth factor 15 (FGF15), which releases its endocrinal inhibition of bile acid synthesis in the liver...
August 21, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28871709/unusual-presentations-of-carney-complex-in-patient-with-a-novel-prkar1a-mutation
#11
Safak Akin, Senem Noyan, Selcuk Dagdelen, Ilhan Pasaoglu, Volkan Kaynaroglu, Melike Mut Askun, Cenk Yucel Bilen, Hayyam Kiratli, Dilek Ertoy Baydar, Sevgen Onder, Cenk Sokmensuer, Kudret Aytemir, Gul Erkin, Pinar Ozgen Kiratli, Mehmet Alikasifoglu, Tomris Erbas
Carney Complex (CNC) is a multiple neoplasia syndrome characterized by skin tumors and pigmented lesions, myxomas, and various endocrine tumors. The aim of this case report was to describe a case of CNC with a novel PRKAR1A mutation. A man aged 46 years with a medical history of surgery for cardiac myxomas at the age of 39 was admitted to our hospital because of four newly-developed heart masses. The histologic examination confirmed cardiac myxomas. He had many presentations of CNC such as growth hormone (GH) and prolactin (PRL)-secreting mixed pituitary adenoma, benign thyroid nodule, large-cell calcifying Sertoli cell tumor (LCCST), and superficial angiomyxoma...
August 5, 2017: Neuro Endocrinology Letters
https://www.readbyqxmd.com/read/28870985/whole-exome-sequencing-gives-additional-benefits-compared-to-candidate-gene-sequencing-in-the-molecular-diagnosis-of-children-with-growth-hormone-or-igf-1-insensitivity
#12
Lucy Shapiro, Sumana Chatterjee, Dina Ramadan, Kate M Davies, Martin O Savage, Louise A Metherell, Helen L Storr
GH insensitivity (GHI) is characterised by short stature, IGF-1 deficiency and normal/elevated serum GH. IGF-1 insensitivity results in pre- and post-natal growth failure with normal/high IGF-1 levels. The prevalence of genetic defects is unknown. OBJECTIVE: To identify the underlying genetic diagnoses in a paediatric cohort with GH or IGF-1 insensitivity using candidate gene (CGS) and whole exome sequencing (WES) and assess factors associated with the discovery of a genetic defect. METHODS: We undertook a prospective study of 132 patients with short stature and suspected GH or IGF-1 insensitivity referred to our centre for genetic analysis...
September 4, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28869938/mineral-and-bone-disorder-in-chronic-kidney-disease-a-case-report-from-vietnam
#13
Pham Van Bui, Nguyyen Minh Tuan, Huynh Thi Nguyen Nghia, Pham Nguyen Thanh Phuong
We report a case of calcium pyrophosphate dihydrate deposition disease (CPDD) involving a patient on maintenance hemodialysis (MHD). The 32-year-old man presented in August 2016 with a complaint of left shoulder swelling of 8 months' duration with no trauma or fever. He was diagnosed with nephrotic syndrome in 1998, which progressed to ESRD. He commenced MHD in 2012. Examination at our hospital revealed a soft nontender swelling of the left shoulder. Blood biochemistry showed elevated serum urate, phosphate, β2 microglobulin, and parathyroid hormone...
2017: Blood Purification
https://www.readbyqxmd.com/read/28864114/leptin-sirna-promotes-ovarian-granulosa-cell-apoptosis-and-affects-steroidogenesis-by-increasing-npy2-receptor-expression
#14
Xiaomeng Ding, Xinxin Kou, Ye Zhang, Xiaoli Zhang, Guomei Cheng, Tianming Jia
Leptin has been found to be involved in the ovarian granulosa cell apoptosis and steroidogenesis. Loss of neuropeptide Y (NPY) can correct the obesity syndrome of mutant mice lacking of leptin (ob/ob). However, the association of NPY and leptin in ovarian granulosa cells and ovarian steroidogenesis has not been investigated. Here, C57BL/6J ob/ob mice and C57BL/6J (control) mice were intraperitoneally injected with PBS, leptin (0.4μg/g bodyweight) or BIIE0246 (NPY2 receptor [NPY2R] antagonist, 30μg/kg bodyweight) every day for 15days...
August 29, 2017: Gene
https://www.readbyqxmd.com/read/28854950/prevalence-and-risk-factors-for-type-2-diabetes-mellitus-with-prader-willi-syndrome-a-single-center-experience
#15
Aram Yang, Jinsup Kim, Sung Yoon Cho, Dong-Kyu Jin
BACKGROUND: Prader-Willi syndrome (PWS) is often related to severe obesity and type-2 diabetes mellitus (T2DM). However, few studies, and none in Korea, have examined prevalence of T2DM and other variables in PWS. The aim of this study was to identify the prevalence and associated risk factors for T2DM in Korean patients with PWS. METHODS: We performed a retrospective cohort study of the 84 PWS patients aged 10 or over (10.3-35.8 years of age) diagnosed with PWS at Samsung Medical Center from 1994 to 2016...
August 30, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28851248/long-term-testosterone-treatment-during-pregnancy-does-not-alter-insulin-or-glucose-profile-in-a-sheep-model-of-polycystic-ovary-syndrome
#16
Monica Recabarren, Albert Carrasco, Daniel Sandoval, Felipe Diaz, Teresa Sir-Petermann, Sergio E Recabarren
The administration of testosterone to pregnant sheep to resemble fetal programming of the polycystic ovary syndrome could alter other hormones/factors of maternal origin with known effects on fetal growth. Hence, we studied the weekly profile of insulin, progesterone and glucose during a treatment with testosterone propionate given biweekly from weeks 5 to 17 of pregnancy (term at 21 weeks) and checked the outcome of their fetuses at 17 weeks of gestation after C-section. Control dams were only exposed to the vehicle of the hormone...
September 7, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28843412/single-tubule-rna-seq-uncovers-signaling-mechanisms-that-defend-against-hyponatremia-in-siadh
#17
Jae Wook Lee, Mohammad Alsady, Chung-Lin Chou, Theun de Groot, Peter M T Deen, Mark A Knepper, Carolyn M Ecelbarger
In the syndrome of inappropriate antidiuretic hormone secretion (SIADH), hyponatremia is limited by onset of vasopressin-escape caused by loss of the water channel aquaporin-2 in the renal collecting duct despite high circulating vasopressin. Here, we use the methods of systems biology in a well-established rat model of SIADH to identify signaling pathways activated at the onset of vasopressin-escape. Using single-tubule RNA-Seq, full transcriptomes were determined in microdissected cortical collecting ducts of vasopressin-treated rats at 1, 2, and 4 days after initiation of oral water loading in comparison to time-control rats without water loading...
August 23, 2017: Kidney International
https://www.readbyqxmd.com/read/28834083/microstructural-white-matter-tract-alteration-in-prader-willi-syndrome-a-diffusion-tensor-imaging-study
#18
Lauren J Rice, Jim Lagopoulos, Michael Brammer, Stewart L Einfeld
Prader-Willi Syndrome (PWS) is a genetic disorder characterized by infantile hypotonia, hyperphagia, hypogonadism, growth hormone deficiency, intellectual disability, and severe emotional and behavioral problems. The brain mechanisms that underpin these disturbances are unknown. Diffusion tensor imaging (DTI) enables in vivo investigation of the microstructural integrity of white matter pathways. To date, only one study has used DTI to examine white matter alterations in PWS. However, that study used selected regions of interest, rather than a whole brain analysis...
September 2017: American Journal of Medical Genetics. Part C, Seminars in Medical Genetics
https://www.readbyqxmd.com/read/28808787/malignant-transformation-of-a-conservatively-managed-incidental-childhood-cerebral-mass-lesion-controversy-regarding-management-paradigm
#19
Jehuda Soleman, Jonathan Roth, Zvi Ram, Michal Yalon, Shlomi Constantini
BACKGROUND: Incidental findings on neuroimaging in the pediatric population are an emerging treatment challenge. Treatment options for these incidental childhood brain mass lesions, which radiologically may be assumed to be low-grade gliomas (LGG), vary, ranging from careful conservative "wait and scan" treatment to surgical biopsy, gross total resection, and upfront radiation and/or chemotherapy. As malignant transformation of LGG in children is extremely rare, some series advocate careful conservative management of these lesions; however, universal treatment protocols are not totally agreed upon...
August 14, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28806298/effect-of-cyproheptadine-on-weight-and-growth-velocity-in-children-with-silver-russell-syndrome
#20
Anaïs Lemoine, Madeleine D Harbison, Jennifer Salem, Patrick Tounian, Irène Netchine, Béatrice Dubern
OBJECTIVES: Nutritional management of children with Silver-Russell syndrome (SRS) is crucial, especially before initiating growth hormone therapy (GHT). Since cyproheptadine (CYP) has been reported to be orexigenic, we retrospectively investigated the effects of CYP on changes in weight and height in SRS patients. METHODS: Anthropometric parameters (weight [W], length or height [H], relative body weight for height [W/H] and body mass index [BMI]) were recorded for 34 children with SRS receiving CYP...
August 11, 2017: Journal of Pediatric Gastroenterology and Nutrition
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