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Julia Quitmann, Anja Rohenkohl, Rachel Sommer, Monika Bullinger, Neuza Silva
BACKGROUND: In the context of health-related quality of life (HrQoL) assessment in pediatric short stature, the present study aimed to examine the levels of agreement/disagreement between parents' and children's reports of generic and condition-specific HrQoL, and to identify socio-demographic, clinical and psychosocial variables associated with the extent and direction of parent-child discrepancies. METHODS: This study was part of the retest phase of the QoLISSY project, which was a multicenter study conducted simultaneously in France, Germany, Spain, Sweden and UK...
October 21, 2016: Health and Quality of Life Outcomes
S Birla, R Khadgawat, V P Jyotsna, V Jain, M K Garg, A S Bhalla, A Sharma
Growth hormone deficiency (GHD) results from variations affecting the production and release of growth hormone (GH) and is of 2 types: isolated growth hormone deficiency (IGHD) and combined pituitary hormone deficiency (CPHD). IGHD results from mutations in GH1 and GHRHR while CPHD is associated with defects in transcription factor genes PROP1, POU1F1, and HESX1. The present study reports on screening of POU1F1, PROP1, and HESX1 in CPHD patients and the novel variations identified. Fifty-one CPHD patients from 49 unrelated families clinically diagnosed on the basis of biochemical and imaging investigations along with 100 controls were enrolled...
October 18, 2016: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
Matthias M Weber, Beverly Mk Biller, Birgitte Tønnes Pedersen, Effie Pournara, Jens Sandahl Christiansen, Charlotte Höybye
OBJECTIVE: To assess the effect of 4 years' GH replacement on glucose homeostasis and evaluate factors affecting glycosylated haemoglobin (HbA1c ) in adults with GH deficiency (GHD). DESIGN: NordiNet(®) International Outcome Study, a non-interventional study, monitors long-term effectiveness and safety of GH-replacement (Norditropin(®) [somatropin], Novo Nordisk A/S) in real-life clinical practice. PATIENTS: Non-diabetic patients (n=245) with adult-onset GHD (age ≥20 years at GH start), ≥4 years' GH replacement and HbA1c values at baseline and 4 years were included in the analysis...
October 13, 2016: Clinical Endocrinology
Tami Rubinek, Shiri Shahmoon, Ayelet Shabtay-Orbach, Michal Ben Ami, Yael Levy-Shraga, Kineret Mazor-Aronovitch, Yonatan Yeshayahu, Ram Doolman, Rina Hemi, Hannah Kanety, Ido Wolf, Dalit Modan-Moses
CONTEXT: Klotho is an aging-modulating protein expressed mainly in the kidneys, which can be cleaved and shed to act as a circulating hormone. Several lines of evidence suggest a tight interaction between klotho and the GH-IGF-I axis. We showed previously that klotho levels are decreased in pediatric patients with growth hormone deficiency (GHD). Our aim now is to investigate the effect of GH therapy on klotho levels in these patients and to elucidate the role of IGF-1 in mediating secretion of klotho...
November 2016: Metabolism: Clinical and Experimental
Chiara Diazzi, Giulia Brigante, Giulia Ferrannini, Anna Ansaloni, Lucia Zirilli, Maria Cristina De Santis, Stefano Zona, Giovanni Guaraldi, Vincenzo Rochira
Biochemical growth hormone deficiency is prevalent among human immunodeficiency virus-infected patients, but if this condition is clinically relevant remains challenging. The aim is to prospectively compare the growth hormone deficiency/insulin-like growth factor-1 status of 71 human immunodeficiency virus-infected patients with impaired growth hormone response to growth hormone releasing hormone + Arginine with that of 65 hypopituitary patients affected by a true growth hormone deficiency secondary to pituitary disease...
October 11, 2016: Endocrine
Marion Kessler, Michael Tenner, Michael Frey, Richard Noto
BACKGROUND: The objective of the study was to describe the pituitary volume (PV) in pediatric patients with isolated growth hormone deficiency (IGHD), idiopathic short stature (ISS) and normal controls. METHODS: Sixty-nine patients (57 male, 12 female), with a mean age of 11.9 (±2.0), were determined to have IGHD. ISS was identified in 29 patients (20 male, 9 female), with a mean age of 12.7 (±3.7). Sixty-six controls (28 female, 38 male), mean age 9.8 (±4.7) were also included...
October 1, 2016: Journal of Pediatric Endocrinology & Metabolism: JPEM
D Lanssens, A Van Moerebeke, A van den Hoogen, N Geusens, L Grieten, W Gyselaers
BACKGROUND: Gestational hypertensive disorders (GHD) are major contributors to maternal mortality worldwide. In Ziekenhuis Oost- Limburg (Genk, Belgium), a remote monitoring (RM) program for pregnant women at risk for developing GHD was initiated in January 2015 to evaluate the relevance of a RM program. RM is a relatively new approach that broadly can be defined as the application of telecommunication technologies in interchanging medical data, information and services between healthcare providers and patients...
August 2016: Journal of Maternal-fetal & Neonatal Medicine
S Vonck, J Oben, A S Staelens, D Lanssens, K Tomsin, W Gyselaers
INTRODUCTION: Subclinical hypertension has been reported in the first trimester of future hypertensive patients compared to normotensive patients. However, in clinical practice today, a cut off of 140/90 mmHg is still used to discriminate normotension from hypertension during pregnancy. We aim to investigate the most appropriate threshold for early gestational blood pressure values, which allow discriminating pregnant women between those at low or at high risk for gestational hypertensive diseases...
August 2016: Journal of Maternal-fetal & Neonatal Medicine
Galina Yordanova, Lee Martin, Farhad Afshar, Ian Sabin, Ghassan Alusi, Nicholas P Plowman, Fiona Riddoch, Jane Evanson, Matthew Matson, Ashley B Grossman, Scott A Akker, John P Monson, William M Drake, Martin O Savage, Helen L Storr
PURPOSE: Pediatric Cushing's disease (CD) is rare and there are limited data on the long-term outcomes. We assessed CD recurrence, body composition, pituitary function and psychiatric comorbidity in a cohort of pediatric CD patients. METHODS: Retrospective review of 21 CD patients, mean age at diagnosis 12.1 years (5.7-17.8), managed in our center between 1986 and 2010. Mean follow-up from definitive treatment was 10.6 years (2.9-27.2). RESULTS: Fifteen patients were in remission following transsphenoidal surgery (TSS) and 5 were in remission following TSS + external pituitary radiotherapy (RT)...
September 27, 2016: Pituitary
Joanna Oświęcimska, Wojciech Roczniak, Agata Mikołajczak, Agnieszka Szymlak
Growth hormone (GH) is a naturally occurring polypeptide hormone produced by somatotropic cells in the anterior pituitary. The main function of somatotropin is stimulation of linear growth, but it also affects carbohydrate metabolism, increases bone mass and has potent lipolytic, antinatriuretic and antidiuretic effects. Growth hormone deficiency (GHD) may occur both in children and in adults. At the moment there is no gold standard for the diagnosis of GHD, and the diagnosis should take into account clinical, auxological, biochemical and radiological changes and, if necessary, genetic testing...
2016: Postȩpy Higieny i Medycyny Doświadczalnej
Adam Stevens, Philip George Murray, Jerome Wojcik, John Raelson, Ekaterina Koledova, Pierre Chatelain, Peter E Clayton
OBJECTIVE: Single nucleotide polymorphisms (SNPs) associated with the response to r-hGH have previously been identified in GHD and TS children in the PREDICT long-term follow-up (LTFU) study (NCT00699855). Here we describe the PREDICT validation (VAL) study (NCT01419249) which aimed to confirm these genetic associations. DESIGN AND METHODS: Children with GHD (n=293) or TS (n=132) were recruited retrospectively from 29 sites in nine countries. All children had completed one year of r-hGH therapy...
September 20, 2016: European Journal of Endocrinology
J-S He, C-W Lian, H-W Zhou, X-F Lin, H-C Yang, X-L Ye, S-B Zhu
OBJECTIVE: Growth hormone deficiency (GHD) is the most common cause for childhood dwarfism. Currently, the significance of insulin-like growth factor-1 (IGF-1) in diagnosis of GHD is still debatable. Due to the possible correlation between leptin (LEP) and GHD pathogenesis, this study investigated the gene polymorphism of LEP and its receptor (LEPR) genes, along with serum IGF-1 and LEP levels in GHD patients. This study attempted to illustrate the correlation between gene polymorphism and GHD pathogenesis...
September 2016: European Review for Medical and Pharmacological Sciences
Federico Baronio, Laura Mazzanti, Ylenia Girtler, Federica Tamburrino, Anisia Fazzi, Fiorenzo Lupi, Silvia Longhi, Giorgio Radetti
BACKGROUND: Growth hormone (GH) influences glucose homeostasis by negatively affecting insulin sensitivity, leading to a compensatory increase in insulin secretion. It has recently been reported, in animals and humans, that GH might also stimulate insulin secretion by directly affecting the growth and function of pancreatic β-cells. The aim of this work was to longitudinally study the insulin sensitivity (HOMA-S), insulin secretion [insulinogenic index (IGI)] and capacity of β-cells to adapt to changes in insulin sensitivity [oral disposition index (ODI)] in GH-deficient (GHD) children under GH treatment...
September 6, 2016: Hormone Research in Pædiatrics
Kyu Hong Lee, Hee Jin Park, So Yeon Lee, In Young Youn, Eugene Kim, Jai Hyung Park, Se Jin Park
OBJECTIVE: To evaluate the usefulness of glenohumeral distance (GHD) on axial images of magnetic resonance (MR) arthrography for diagnosis of adhesive capsulitis and to compare this finding with previously reported classic MR arthrographic findings of adhesive capsulitis. MATERIALS AND METHODS: Our study was approved by the institutional ethical review board of our institute. We evaluated 41 patients (M-F, 35:6; mean age, 46 years; adhesive capsulitis, 21; no adhesive capsulitis, 20) who underwent MR arthrography...
August 25, 2016: Journal of Computer Assisted Tomography
Nurit Ashkenazi, Moti Rosenstock, Hussein Hallak, Merav Bassan, Michele Rasamoelisolo, Jost Leuschner, Doron Shinar
PURPOSE: TV-1106 is a recombinant human albumin genetically fused to growth hormone which is intended to reduce the frequency of injections for GH therapy users. We report the safety, tolerability, pharmacokinetics and pharmacodynamics of repeated subcutaneous injections of TV-1106 in Cynomolgus monkeys. METHOD: Cynomolgus monkeys received four weekly subcutaneous injections of 0, 5, 10 or 20mg/kg TV-1106 and were monitored for safety signals throughout the study...
August 3, 2016: Growth Hormone & IGF Research
Candace A Robledo, Edwina H Yeung, Pauline Mendola, Rajeshwari Sundaram, Nansi S Boghossian, Erin M Bell, Charlotte Druschel
Objectives We sought to examine whether there are systematic differences in ascertainment of preexisting maternal medical conditions and pregnancy complications from three common data sources used in epidemiologic research. Methods Diabetes mellitus, chronic hypertension, gestational diabetes mellitus (GDM), gestational hypertensive disorders (GHD), placental abruption and premature rupture of membranes (PROM) among 4821 pregnancies were identified via birth certificates, maternal self-report at approximately 4 months postpartum and by discharge codes from the Statewide Planning and Research Cooperative System (SPARCS), a mandatory New York State hospital reporting system...
August 22, 2016: Maternal and Child Health Journal
Julia D J Thomas, Abhishek Dattani, Filip Zemrak, Thomas Burchell, Scott A Akker, Mark Gurnell, Ashley B Grossman, L Ceri Davies, Márta Korbonits
Growth hormone (GH) can profoundly influence cardiac function. While GH excess causes well-defined cardiac pathology, fewer data are available regarding the more subtle cardiac changes seen in GH deficiency (GHD). This preliminary study uses cardiac magnetic resonance imaging (CMR) to assess myocardial structure and function in GHD. Ten adult-onset GHD patients underwent CMR, before and after 6 and 12 months of GH replacement. They were compared to 10 age-matched healthy controls and sex-matched healthy controls...
August 17, 2016: Endocrine
Juliane Rothermel, Nina Lass, Christina Toschke, Thomas Reinehr
BACKGROUND: Familial short stature (FSS) and constitutional delay of growth (CDG) are the most frequent norm variants in children presenting with short stature. Knowing the growth patterns of these entities in the first years of life might be helpful to distinguish them from growth hormone deficiency (GHD) or other chronic diseases. METHODS: We studied the height in the first 5 years of life in 26 children with FSS, in 38 children with CDG and in 14 children with idiopathic GHD...
2016: Hormone Research in Pædiatrics
Louise Cheryl Gregory, Kyriaki Sandy Alatzoglou, Mark James McCabe, Peter Christopher Hindmarsh, Jose William Saldanha, Nicola Romano, Paul Le Tissier, Mehul Tulsidas Dattani
OBJECTIVE: Recessive mutations in GHRHR are associated with severe isolated growth hormone deficiency (IGHD), with a final height in untreated patients of 130 cm ± 10 cm (-7.2 ± 1.6 SDS; males) and 114 ± 0.7 cm (-8.3 ± 0.1 SDS; females). DESIGN: We hypothesized that a consanguineous Pakistani family with IGHD in three siblings (two males, one female) would have mutations in GH1 or GHRHR. RESULTS: Two novel homozygous missense variants [c...
October 2016: Journal of Clinical Endocrinology and Metabolism
Yuko Goto, Satoru Oshino, Akio Nishino, Toshiyuki Fujinaka, Hajime Nakamura, Takamichi Yuguchi, Shintaro Mori, Toshiki Yoshimine, Youichi Saitoh
To elucidate the pituitary function of Japanese patients after aneurysmal subarachnoid hemorrhage (aSAH) and implicative factors related to growth hormone deficiency (GHD) after aSAH. We evaluated basal pituitary hormone levels among 59 consecutive aSAH patients with a modified Rankin Scale (mRS) ⩽4 at 3months after aSAH onset. Patients with low insulin-like growth factor 1 (IGF-1) SD score (SDS) or who seemed to develop pituitary dysfunction underwent provocative endocrine testing during a period of 3-36months after SAH onset...
August 1, 2016: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
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