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Growth hormone syndrome

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https://www.readbyqxmd.com/read/28208663/endoplasmic-reticulum-er-stress-and-endocrine-disorders
#1
REVIEW
Daisuke Ariyasu, Hiderou Yoshida, Yukihiro Hasegawa
The endoplasmic reticulum (ER) is the organelle where secretory and membrane proteins are synthesized and folded. Unfolded proteins that are retained within the ER can cause ER stress. Eukaryotic cells have a defense system called the "unfolded protein response" (UPR), which protects cells from ER stress. Cells undergo apoptosis when ER stress exceeds the capacity of the UPR, which has been revealed to cause human diseases. Although neurodegenerative diseases are well-known ER stress-related diseases, it has been discovered that endocrine diseases are also related to ER stress...
February 11, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28191538/lipodystrophy-hiv-related-and-fgf21-a-new-marker-to-follow-the-progression-of-lipodystrophy
#2
Stefano Benedini, Livio Luzi
Recently new evidence about fibroblast growth factor 21 (FGF21) highlights the opportunities to use this molecule in new pharmaceutical formulations to combat type 2 diabetes and metabolic syndrome. It is well known that HIV is per se a condition of insulin resistance and in particular the patient with HIV-related lipodystrophy has a condition strictly related to metabolic syndrome. Lipodystrophy is associated with severe metabolic side effects, including dyslipidemia, hepatic insulin resistance, and lipid oxidation impairment...
December 1, 2016: Journal of Translational Internal Medicine
https://www.readbyqxmd.com/read/28178857/dietary-2-oxoglutarate-prevents-bone-loss-caused-by-neonatal-treatment-with-maximal-dexamethasone-dose
#3
Piotr Dobrowolski, Ewa Tomaszewska, Siemowit Muszyński, Tomasz Blicharski, Stefan G Pierzynowski
Synthetic glucocorticoids (GCs) are widely used in the variety of dosages for treatment of premature infants with chronic lung disease, respiratory distress syndrome, allergies, asthma, and other inflammatory and autoimmune conditions. Yet, adverse effects such as glucocorticoid-induced osteoporosis and growth retardation are recognized. Conversely, 2-oxoglutarate (2-Ox), a precursor of glutamine, glutamate, and collagen amino acids, exerts protective effects on bone development. Our aim was to elucidate the effect of dietary administered 2-Ox on bone loss caused by neonatal treatment with clinically relevant maximal therapeutic dexamethasone (Dex) dose...
January 1, 2017: Experimental Biology and Medicine
https://www.readbyqxmd.com/read/28178326/quantitative-liver-proteomics-identifies-fgf19-targets-that-couple-metabolism-and-proliferation
#4
Vittoria Massafra, Alexandra Milona, Harmjan R Vos, Boudewijn M T Burgering, Saskia W C van Mil
Fibroblast growth factor 19 (FGF19) is a gut-derived peptide hormone that is produced following activation of Farnesoid X Receptor (FXR). FGF19 is secreted and signals to the liver, where it contributes to the homeostasis of bile acid (BA), lipid and carbohydrate metabolism. FGF19 is a promising therapeutic target for the metabolic syndrome and cholestatic diseases, but enthusiasm for its use has been tempered by FGF19-mediated induction of proliferation and hepatocellular carcinoma. To inform future rational design of FGF19-variants, we have conducted temporal quantitative proteomic and gene expression analyses to identify FGF19-targets related to metabolism and proliferation...
2017: PloS One
https://www.readbyqxmd.com/read/28173651/-rapidly-progressive-puberty-in-a-patient-with-mosaic-turner-syndrome-a-case-report-and-literature-review
#5
Y Liang, H Wei, X Yu, W Huang, X P Luo
Objective: To explore the clinical characteristics of diagnosis and treatment in patients with Turner syndrome and rapidly progressive puberty. Method: A rare case of rapidly progressive puberty in Turner syndrome with a mosaic karyotype of 45, X/46, X, del(X)(p21)(80%/20%)was diagnosed at Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology in January. 2015. Clinical characteristics and the related literature were reviewed. Original papers on precocious puberty or rapidly progressive puberty in Turner syndrome, published until Apr...
February 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28165551/thyroid-disorders-in-polycystic-ovary-syndrome
#6
K Kowalczyk, G Franik, D Kowalczyk, D Pluta, Ł Blukacz, P Madej
OBJECTIVE: Thyroid disorders, especially Hashimoto's thyroiditis (HT), are observed significantly more often in patients with polycystic ovary syndrome (PCOS) than in the general population - approximately 27% and 8%, respectively. This is extremely important in young women, because both disorders are connected with fertility problems. As HT and PCOS occur together, fertility problems may become a serious clinical issue in these patients. MATERIALS AND METHODS: A systematic literature review in PubMed of PCOS- and HT-related articles in English, published until December 2015 was conducted...
January 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/28164505/association-study-between-ghrelin-gene-polymorphism-and-metabolic-syndrome-in-a-han-chinese-population
#7
Yueyue You, Yaqin Yu, Yanhua Wu, Wenwang Rao, Yangyu Zhang, Yingyu Liu, Guang Yang, Yingli Fu, Jieping Shi, Changgui Kou
BACKGROUND: Ghrelin, in humans, is a hormone secreted from the stomach with an orexigenic effect, which is good for digestion and absorption, as well as regulating physical growth, metabolism, and energy balance. It is also involved in the development of metabolic syndrome (MetS) and type 2 diabetes mellitus (T2DM). This study assessed the association between single nucleotide variants of the GHRL gene and the risk of metabolic syndrome in a Han Chinese population. METHODS: A case-control study was performed on 3780 Han Chinese comprising 1813 MetS cases and 1967 controls...
January 1, 2017: Clinical Laboratory
https://www.readbyqxmd.com/read/28164079/short-stature-and-growth-hormone-deficiency-in-a-girl-with-encephalocraniocutaneous-lipomatosis-and-jaffe-campanacci-syndrome-a-case-report
#8
Eun Mi Choi, Nani Jung, Ye Jee Shim, Hee Joung Choi, Joon Sik Kim, Heung Sik Kim, Kwang Soon Song, Hee Jung Lee, Sang Pyo Kim
A 9-year-old Tajikistani girl presented to Keimyung University Dongsan Medical Center for evaluation of a skin lesion on her left eyelid, focal alopecia, unilateral ventricular dilatation, and aortic coarctation. She was diagnosed with encephalocraniocutaneous lipomatosis (ECCL) according to Moog's diagnostic criteria. Café-au-lait spots were found on the left side of her trunk. Multiple nonossifying fibromas were found on her left proximal humerus, left distal femur, both proximal tibias, and left proximal fibula, suggesting Jaffe-Campanacci syndrome (JCS), following imaging of the extremities...
December 2016: Annals of Pediatric Endocrinology & Metabolism
https://www.readbyqxmd.com/read/28150161/diabetes-in-patients-with-acromegaly
#9
REVIEW
A M Hannon, C J Thompson, M Sherlock
Acromegaly is a clinical syndrome which results from growth hormone excess. Uncontrolled acromegaly is associated with cardiovascular mortality, due to an excess of risk factors including diabetes mellitus, hypertension and cardiomegaly. Diabetes mellitus is a frequent complication of acromegaly with a prevalence of 12-37%. This review will provide an overview of a number of aspects of diabetes mellitus and glucose intolerance in acromegaly including the following: 1. Epidemiology and pathophysiology of abnormalities of glucose homeostasis 2...
February 2017: Current Diabetes Reports
https://www.readbyqxmd.com/read/28144395/new-therapeutic-approaches-to-metastatic-gastroenteropancreatic-neuroendocrine-tumors-a-glimpse-into-the-future
#10
REVIEW
Esther Una Cidon
Neuroendocrine (NE) gastroenteropancreatic tumors are a heterogeneous group of neoplasias arising from neuroendocrine cells of the embryological gut. Their incidence have increased significantly over the past 3 decades probably due to the improvements in imaging and diagnosis. The recent advances in molecular biology have translated into an expansion of therapeutic approaches to these patients. Somatostatin analogs, which initially were approved for control of hormonal syndromes, have recently been proven to inhibit tumor growth...
January 15, 2017: World Journal of Gastrointestinal Oncology
https://www.readbyqxmd.com/read/28138333/short-stature-and-hypoparathyroidism-in-a-child-with-kenny-caffey-syndrome-type-2-due-to-a-novel-mutation-in-fam111a-gene
#11
Mary B Abraham, Dong Li, Dave Tang, Susan M O'Connell, Fiona McKenzie, Ee Mun Lim, Hakon Hakonarson, Michael A Levine, Catherine S Choong
BACKGROUND: Hypoparathyroidism in children is a heterogeneous group with diverse genetic etiologies. To aid clinicians in the investigation and management of children with hypoparathyroidism, we describe the phenotype of a 6-year-old child with hypoparathyroidism and short stature diagnosed with Kenny-Caffey syndrome (KCS) Type 2 and the subsequent response to growth hormone (GH) treatment. CASE PRESENTATION: The proband presented in the neonatal period with hypocalcemic seizures secondary to hypoparathyroidism...
2017: International Journal of Pediatric Endocrinology
https://www.readbyqxmd.com/read/28127864/continuing-role-for-classical-cytogenetics-case-report-of-a-boy-with-ring-syndrome-caused-by-complete-ring-chromosome-4-and-review-of-literature
#12
Anna Lena Burgemeister, Eva Daumiller, Ilona Dietze-Armana, Christine Klett, Clemens Freiberg, Wiebke Stark, Michael Lingen, Izabela Centonze, Günther Rettenberger, Karl Mehnert, Birgit Zirn
Constitutional ring chromosomes can be found for all human chromosomes and are very rare chromosomal abnormalities. A complete ring chromosome without loss of genetic material results from fusion of subtelomeric regions or telomere-telomere fusion. In cases of complete ring chromosome, an increased incidence of severe growth failure with no or only minor anomalies has been observed and attributed to ring syndrome. Ring syndrome is thought to be caused by "dynamic mosaicism" due to ring instability. We report a 6-year-old boy with de novo ring chromosome 4 and typical characteristics of the ring syndrome, namely, proportionate severe growth failure, microcephaly, and minor anomalies...
January 27, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28124993/growth-hormone-gh-and-rehabilitation-promoted-distal-innervation-in-a-child-affected-by-caudal-regression-syndrome
#13
Jesús Devesa, Alba Alonso, Natalia López, José García, Carlos I Puell, Tamara Pablos, Pablo Devesa
Caudal regression syndrome (CRS) is a malformation occurring during the fetal period and mainly characterized by an incomplete development of the spinal cord (SC), which is often accompanied by other developmental anomalies. We studied a 9-month old child with CRS who presented interruption of the SC at the L2-L3 level, sacral agenesis, a lack of innervation of the inferior limbs (flaccid paraplegia), and neurogenic bladder and bowel. Given the known positive effects of growth hormone (GH) on neural stem cells (NSCs), we treated him with GH and rehabilitation, trying to induce recovery from the aforementioned sequelae...
January 23, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28120721/progranulin-a-new-adipokine-at-the-crossroads-of-metabolic-syndrome-diabetes-dyslipidemia-and-hypertension
#14
Agnieszka Korolczuk, Jerzy Bełtowski
BACKGROUND: Progranulin is a multifunctional regulatory protein with growth-promoting, neuroprotective and antiinflammatory activities. Recent studies indicate that progranulin is one of the adipose tissue hormones (adipokines). Progranulin expression in visceral adipose tissue and circulating progranulin concentration are increased in obesity and hyperprogranulinemia is involved in the pathogenesis of obesity-associated insulin resistance. Progranulin impairs insulin signaling and reduces insulin-induced glucose uptake both in vitro and in vivo whereas progranulin deficiency protects from high fat diet-induced insulin resistance...
January 24, 2017: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/28104732/iatrogenic-cushing-s-syndrome-due-to-topical-ocular-glucocorticoid-treatment
#15
Daisuke Fukuhara, Toshihiko Takiura, Hiroshi Keino, Annabelle A Okada, Kunimasa Yan
Iatrogenic Cushing's syndrome (CS) is a severe adverse effect of systemic glucocorticoid (GC) therapy in children, but is extremely rare in the setting of topical ocular GC therapy. In this article, we report the case of a 9-year-old girl suffering from idiopathic uveitis who developed CS due to topical ocular GC treatment. She was referred to the ophthalmology department with a complaint of painful eyes, at which time she was diagnosed with bilateral iridocyclitis and started on a treatment of betamethasone sodium phosphate eye drops...
February 2017: Pediatrics
https://www.readbyqxmd.com/read/28102548/the-shade-avoidance-syndrome-multiple-signals-and-ecological-consequences
#16
REVIEW
Carlos L Ballaré, Ronald Pierik
Plants use photoreceptor proteins to detect the proximity of other plants and to activate adaptive responses. Of these photoreceptors, phytochrome B (phyB), which is sensitive to changes in the red (R) to far-red (FR) ratio of sunlight, is the one that has been studied in greatest detail. The molecular connections between the proximity signal (low R:FR) and a model physiological response (increased elongation growth) have now been mapped in considerable detail in Arabidopsis seedlings. We briefly review our current understanding of these connections, and discuss recent progress in establishing the roles of other photoreceptors in regulating growth-related pathways in response to competition cues...
January 19, 2017: Plant, Cell & Environment
https://www.readbyqxmd.com/read/28099131/turner-syndrome-and-pituitary-adenomas-a-case-report-and-review-of-literature
#17
Tiffany Yeh, Angela Ganan Soto, Jose Bernardo Quintos, Lisa Swartz Topor
BACKGROUND: Turner syndrome (TS) is the most common sex chromosome abnormality in females, typically associated with primary amenorrhea and premature ovarian failure due to gonadal dysgenesis. The association of TS with hypopituitarism is an uncommon finding. The objective of the study was to describe an adolescent with TS with hypergonadotropic hypogonadism and subsequent hypogonadotropic hypogonadism. CASE PRESENTATION: A 16-year-old female with primary amenorrhea was diagnosed with TS based on karyotype 45,XO...
February 1, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28095041/oral-versus-transdermal-estrogen-in-turner-syndrome-a-systematic-review-and-meta-analysis
#18
Feras Zaiem, Fares Alahdab, Alaa Al Nofal, Mohammad Hassan Murad, Asma Javed
OBJECTIVE: To conduct a systematic review and meta-analysis comparing transdermal estrogens (TDE) versus oral estrogens (OE) in Turner syndrome (TS). METHOD: Randomized trials and observational comparative studies with a minimal follow up of 6 months for skeletal and metabolic outcomes and serum hormone changes. Outcomes were pooled with a random effects model and were reported as mean difference between OE and TDE groups and 95% confidence interval (CI). RESULT: Of 845 candidate references, 4 studies were included...
January 17, 2017: Endocrine Practice
https://www.readbyqxmd.com/read/28090459/anti-m%C3%A3-llerian-hormone-as-a-predictor-of-polycystic-ovary-syndrome-treated-with-clomiphene-citrate
#19
Andon Hestiantoro, Yuwono Sri Negoro, Yohana Afrita, Budi Wiweko, Kanadi Sumapradja, Muharam Natadisastra
OBJECTIVE: This study aimed to determine the threshold of anti-Müllerian hormone (AMH) as predictor of follicular growth failure in polycystic ovary syndrome (PCOS) patients treated with clomiphene citrate (CC). METHODS: Fifty female subjects with PCOS were recruited and divided into two groups based on successful and unsuccessful follicular growth. Related variables such as age, infertility duration, cigarette smoking, use of Moslem hijab, sunlight exposure, fiber intake, body mass index, waist circumference, AMH level, 25-hydroxy vitamin D level, and growth of dominant follicles were obtained, assessed, and statistically analyzed...
December 2016: Clinical and Experimental Reproductive Medicine
https://www.readbyqxmd.com/read/28089836/delayed-csf-rhinorrhea-after-gamma-knife-radiosurgery-with-or-without-preceding-transsphenoidal-resection-for-pituitary-pathology
#20
Avital Perry, Christopher S Graffeo, William R Copeland, Kathryn M Van Abel, Matthew L Carlson, Bruce E Pollock, Michael J Link
BACKGROUND: Skull base cerebrospinal fluid (CSF) leak after gamma knife radiosurgery (GKRS) is a very rare complication. In patients who were treated with both GKRS and transsphenoidal resection (TSR) for pituitary lesions, early CSF leak occurs at a comparable rate to the general TSR population (4%). Delayed CSF leak occurring more than a year after TSR, GKRS, or dual therapy, is exceedingly rare. METHODS: Retrospective chart review and review of the literature RESULTS: We present two cases of delayed CSF leak following GKRS to treat pituitary adenoma...
January 9, 2017: World Neurosurgery
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