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Adult Growth Hormone Deficiency

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https://www.readbyqxmd.com/read/30338487/gender-dependent-growth-and-insulin-like-growth-factor-1-responses-to-growth-hormone-therapy-in-prepubertal-growth-hormone-deficient-children
#1
Ewelina Witkowska-Sędek, Małgorzata Rumińska, Anna Majcher, Beata Pyrżak
Gender seems to be an important factor influencing the response to recombinant human growth hormone (rhGH) therapy in GH-deficient adolescents and adults. The results of studies evaluating gender-specific response to rhGH therapy in prepubertal GH-deficient children are divergent. The aim of this study was to determine the effect of gender on the growth and insulin-like growth factor-1 (IGF-1) responses in 75 prepubertal GH-deficient children during the first 2 years of rhGH therapy. There were no baseline gender differences in age, bone age, anthropometrical parameters, and IGF-1 SDS for bone age...
October 19, 2018: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/30289596/preliminary-observations-of-mitochondrial-dysfunction-in-prader-willi-syndrome
#2
Merlin G Butler, Waheeda A Hossain, Robert Tessman, Partha C Krishnamurthy
Prader-Willi syndrome (PWS) is a complex multisystem disorder because of errors in genomic imprinting with severe hypotonia, decreased muscle mass, poor suckling, feeding problems and failure to thrive during infancy, growth and other hormone deficiency, childhood-onset hyperphagia, and subsequent obesity. Decreased energy expenditure in PWS is thought to contribute to reduced muscle mass and physical activity but may also relate to cellular metabolism and disturbances in mitochondrial function. We established fibroblast cell lines from six children and adults with PWS and six healthy controls for mitochondrial assays...
October 5, 2018: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/30251164/adult-individuals-with-congenital-untreated-severe-isolated-growth-hormone-deficiency-have-satisfactory-muscular-function
#3
Alana L Andrade-Guimarães, Manuel H Aguiar-Oliveira, Roberto Salvatori, Vitor O Carvalho, Fabiano Alvim-Pereira, Carlos R Araújo Daniel, Giulliani A Moreira Brasileiro, Ananda A Santana-Ribeiro, Hugo A Santos-Carvalho, Carla R P Oliveira, Edgar R Vieira, Miburge B Gois-Junior
PURPOSE: While growth hormone (GH) and the insulin-like growth factor type I (IGF-I) are known to exert synergistic actions on muscle anabolism, the consequences of prolonged GH deficiency (GHD) on muscle function have not been well defined. We have previously described a large cohort of subjects with isolated GHD (IGHD) caused by a mutation in the GH-releasing hormone receptor gene, with low serum levels of GH and IGF-I. The aim of this study was to assess muscular function in these IGHD subjects...
September 24, 2018: Endocrine
https://www.readbyqxmd.com/read/30199857/growth-hormone-treatment-for-short-stature-in-the-usa-germany-and-france-15-years-of-surveillance-in-the-genetics-and-neuroendocrinology-of-short-stature-international-study-genesis
#4
Roland Pfäffle, Christof Land, Eckhard Schönau, Paul-Martin Holterhus, Judith L Ross, Carolina Piras de Oliveira, Christopher J Child, Imane Benabbad, Nan Jia, Heike Jung, Werner F Blum
BACKGROUND/AIMS: To describe characteristics, auxological outcomes and safety in paediatric patients with growth disorders treated with growth hormone (GH), for cohorts from the USA, Germany and France enrolled in GeNeSIS, a post-authorisation surveillance programme. METHODS: Diagnosis and biochemical measurement data were based on reporting from, and GH treatment was initiated at the discretion of, treating physicians. Auxological outcomes during the first 4 years of GH treatment and at near-adult height (NAH) were analysed...
September 10, 2018: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/30175865/plasmatic-free-light-chains-as-inflammatory-marker-in-insulin-resistance-comparison-of-metabolic-syndrome-with-adult-growth-hormone-deficiency
#5
Umberto Basile, Carmine Bruno, Cecilia Napodano, Edoardo Vergani, Krizia Pocino, Alessandro Brunetti, Francesca Gulli, Stefano Angelo Santini, Antonio Mancini
Biological functions of immunoglobulin-free light chains (FLCs), other than in chronic inflammatory diseases, are still poorly defined; the field of insulin resistance (IR) has not been investigated, despite the strict relationships with oxidative stress (OS) and inflammation. Therefore, we evaluated FLCs levels and their relationships with metabolic parameters in adult growth hormone deficiency (GHD) and metabolic syndrome (MetS), both characterized by IR. One hundred subjects were enrolled: group A, patients with GHD [n =31, 24-69 years, mean ± SEM body mass index (BMI) 26...
September 3, 2018: BioFactors
https://www.readbyqxmd.com/read/30148090/serum-lipid-and-leptin-concentrations-in-patients-with-sheehan-syndrome
#6
Shahnaz A Mir, Tabinda Shah, Hardeep Singh, Iram Shabir, Bashir A Laway
Background: Sheehan syndrome (SS) refers to the occurrence of hypopituitarism after parturition. Hypopituitary adults with growth hormone (GH) deficiency have abnormal body composition with increased fat mass. As leptin is secreted almost exclusively by fat cells and the circulating leptin level is proportional to total fat mass, it is expected that abnormal elevations of leptin concentrations are found in GH deficient hypopituitary patients. The present study was undertaken to evaluate the anthropometric, lipid and leptin levels in patients with SS...
July 2018: Indian Journal of Endocrinology and Metabolism
https://www.readbyqxmd.com/read/30133980/a-will-and-a-way-to-fund-medicines-for-rare-diseases-the-story-of-human-growth-hormone-replacement-for-adults-with-growth-hormone-deficiency
#7
Wendy Lipworth, Geoffrey Ambler, Morton G Burt, Jan Fairchild, Warrick J Inder, George Werther, Ken Ho
Growth hormone (GH) replacement therapy was recently recommended by the Pharmaceutical Benefits Advisory Committee (PBAC) for listing on the Pharmaceutical Benefits Scheme for adults with severe GH deficiency and impaired quality of life. This approval was significant for two reasons. First, the application was initiated and coordinated by a health professional working group, who prepared a 'public interest' submission to PBAC. Second, it resulted in a recommendation to subsidise therapy for a rare disease after two prior rejections on the basis of uncertainty about efficacy and cost effectiveness...
August 2018: Internal Medicine Journal
https://www.readbyqxmd.com/read/30130150/igf1-suppresses-cholesterol-accumulation-in-the-liver-of-growth-hormone-deficient-mice-via-the-activation-of-abca1
#8
Kensaku Fukunaga, Hitomi Imachi, Jingya Lyu, Tao Dong, Seisuke Sato, Tomohiro Ibata, Toshihiro Kobayashi, Takuo Yoshimoto, Kazuko Yonezaki, Toru Matsunaga, Koji Murao
Recently, several clinical studies have suggested that adult growth hormone deficiency that also has low concentration of IGF1 is associated with an increased prevalence of fatty liver (FL). ABCA1 is a pivotal regulator of lipid efflux from cells to apolipoproteins and play an important role on formation of FL. In this study, we determined the effects of IGF1 on ABCA1 expression in GH deficient mice to clarify its effects on FL. Western blotting, real-time PCR and a luciferase assay were employed to examine the effect of IGF1...
August 21, 2018: American Journal of Physiology. Endocrinology and Metabolism
https://www.readbyqxmd.com/read/30118397/endocrine-deficiency-as-a-function-of-radiation-dose-to-the-hypothalamus-and-pituitary-in-pediatric-and-young-adult-patients-with-brain-tumors
#9
Ralph E Vatner, Andrzej Niemierko, Madhusmita Misra, Elizabeth A Weyman, Claire P Goebel, David H Ebb, Robin M Jones, Mary S Huang, Anita Mahajan, David R Grosshans, Arnold C Paulino, Takara Stanley, Shannon M MacDonald, Nancy J Tarbell, Torunn I Yock
PURPOSE: There are sparse data defining the dose response of radiation therapy (RT) to the hypothalamus and pituitary in pediatric and young adult patients with brain tumors. We examined the correlation between RT dose to these structures and development of endocrine dysfunction in this population. MATERIALS AND METHODS: Dosimetric and clinical data were collected from children and young adults (< 26 years of age) with brain tumors treated with proton RT on three prospective studies (2003 to 2016)...
October 1, 2018: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/30117531/national-program-of-severe-growth-hormone-deficiency-treatment-in-adults-and-adolescents-after-completion-of-growth-promoting-therapy
#10
Andrzej Lewiński, Joanna Smyczyńska, Renata Stawerska, Maciej Hilczer, Magdalena Stasiak, Tomasz Bednarczuk, Marek Bolanowski, Roman Junik, Marek Ruchała, Anhelli Syrenicz, Mieczysław Walczak, Wojciech Zgliczyński, Małgorzata Karbownik-Lewińska
Growth hormone (GH) has been used in the treatment of short stature in children with GH deficiency (GHD) for 60 years, and for about 30 years also in the treatment of adults with GHD, in whom such treatment is carried out due to metabolic indications. In Poland, GH treatment is reimbursed only in children with GHD, while so far it has not been refunded in adults with GHD. There are two groups of adults (or adolescents after growth completion) with GHD, who require GH therapy: patients with GHD that occurred in childhood (childhood-onset GHD - CO-GHD) and patients with GHD acquired in adulthood (adulthood-onset GHD - AO-GHD)...
August 17, 2018: Endokrynologia Polska
https://www.readbyqxmd.com/read/30052519/resistance-exercise-alone-improves-muscle-strength-in-growth-hormone-deficient-males-in-the-transition-phase
#11
Nadia Mohamad Amer, Marilza J Modesto, Cláudia Duarte Dos Santos, Oscar Erichsen, Luis P G Mascarenhas, Suzana Nesi-França, Rosana Marques-Pereira, Luiz De Lacerda
Background During the transition phase (TP), patients with growth hormone deficiency (GHD) exhibit decreased muscle strength. Studies assessing the effects of resistance exercise alone on muscle strength in these individuals are scarce. The objective of this study was to evaluate the effects of a program of resistance exercise (PRE) on parameters of muscle strength in subjects in the TP and with childhood-onset GHD treated with recombinant GH (rGH). Methods Sixteen male patients were enrolled and divided into two groups: GHD (n=9) and GH sufficiency (GHS, n=7)...
August 28, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/30014342/pituitary-tumour-apoplexy-within-prolactinomas-in-children-a-more-aggressive-condition
#12
Elizabeth Culpin, Matthew Crank, Mark Igra, Daniel J A Connolly, Paul Dimitri, Showkat Mirza, Saurabh Sinha
OBJECTIVES: To evaluate clinical presentations, diagnosis and management of paediatric patients presenting with pituitary apoplexy. METHODS: A retrospective case series describing a cohort of paediatric patients presenting with this condition from 2010-2016 to a tertiary referral children's hospital in the United Kingdom. RESULTS: Pituitary apoplexy is a rare condition that seems to have a higher relative incidence in children than adults...
October 2018: Pituitary
https://www.readbyqxmd.com/read/29996975/the-interrelation-between-fgf23-and-glucose-metabolism-in-humans
#13
Stan R Ursem, Marc G Vervloet, Rahel M Büttler, Mariëtte T Ackermans, Mirjam M Oosterwerff, Elisabeth M V Eekhoff, Paul Lips, Mireille J Serlie, Susanne E la Fleur, Annemieke C Heijboer
AIMS: Different studies point to a link between glucose metabolism and Fibroblast Growth Factor 23 (FGF23), an osteocyte-derived phosphaturic hormone. We aimed to investigate in humans the effect of (I) a glucose load and (II) a hyperinsulinemic-euglycemic clamp on FGF23 concentrations and conversely (III) the effect of a diet-induced increase in FGF23 concentration on glucose and insulin concentrations. METHODS: Plasma cFGF23 concentrations were measured during: I...
September 2018: Journal of Diabetes and its Complications
https://www.readbyqxmd.com/read/29989677/dysregulated-growth-hormone-insulin-like-growth-factor-1-axis-in-adult-type-1-diabetes-with-long-duration
#14
Kerstin Gutefeldt, Christina A Hedman, Ingrid S M Thyberg, Margareta Bachrach-Lindström, Anna Spångeus, Hans J Arnqvist
CONTEXT: In type 1 diabetes (T1D), dysregulation of the GH-IGF-1 axis has been reported. Whether this is related to upper extremity impairments (UEI) is unknown. OBJECTIVE: Examine differences in GH-IGF-1 axis between T1D on subcutaneous insulin treatment and matched controls without diabetes and possible associations between GH-IGF-1 axis and UEI. DESIGN: Cross-sectional population-based study. Patients with T1D, onset <35 years, duration ≥ 20 years, <67 years old and controls were invited to answer questionnaires and take blood samples...
July 10, 2018: Clinical Endocrinology
https://www.readbyqxmd.com/read/29982555/gh-therapy-in-childhood-cancer-survivors-a-systematic-review-and-meta-analysis
#15
Shrikant Tamhane, Jad G Sfeir, Nana Esi N Kittah, Sina Jasim, Wassim Chemaitilly, Laurie E Cohen, M Hassan Murad
Background: GH deficiency (GHD) is common among childhood cancer survivors (CCSs) with history of tumors, surgery, and/or radiotherapy involving the hypothalamus-pituitary region. We aimed to evaluate the effects of GH therapy (GHT) in CCSs on adult height, risk of diabetes mellitus, abnormal lipids, metabolic syndrome, quality of life, secondary tumors, and disease recurrence. Methods: We searched multiple databases for randomized and observational studies. Pairs of reviewers independently selected studies and collected data...
August 1, 2018: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29980224/effect-of-low-normal-and-high-normal-igf-1-levels-on-memory-and-wellbeing-during-growth-hormone-replacement-therapy-a-randomized-clinical-trial-in-adult-growth-hormone-deficiency
#16
RANDOMIZED CONTROLLED TRIAL
Christa C van Bunderen, Jan Berend Deijen, Madeleine L Drent
BACKGROUND: The aim of the present study was to investigate the effect of low-normal and high-normal levels of IGF-1 in growth hormone (GH) deficient adults on cognition and wellbeing during GH treatment. METHODS: A randomized, open-label, clinical trial including 32 subjects receiving GH therapy for at least 1 year. Subjects were randomized to receive either a decrease (IGF-1 target level of - 2 to - 1 SDS) or an increase of their daily GH dose (IGF-1 target level of 1 to 2 SDS) for a period of 24 weeks...
July 6, 2018: Health and Quality of Life Outcomes
https://www.readbyqxmd.com/read/29974043/malnutrition-in-chronic-kidney-disease
#17
REVIEW
Franca M Iorember
Patients with chronic kidney disease are at substantial risk for malnutrition, characterized by protein energy wasting and micronutrient deficiency. Studies show a high prevalence rate of malnutrition in both children and adults with chronic kidney disease. Apart from abnormalities in growth hormone-insulin like growth factor axis, malnutrition also plays a role in the development of stunted growth, commonly observed in children with chronic kidney disease. The pathogenic mechanisms of malnutrition in chronic kidney disease are complex and involve an interplay of multiple pathophysiologic alterations including decreased appetite and nutrient intake, hormonal derangements, metabolic imbalances, inflammation, increased catabolism, and dialysis related abnormalities...
2018: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29973375/long-acting-fc-fusion-rhgh-gx-h9-shows-potential-for-up-to-twice-monthly-administration-in-gh-deficient-adults
#18
RANDOMIZED CONTROLLED TRIAL
Cheol Ryong Ku, Thierry Brue, Katharina Schilbach, Stanislav Ignatenko, Sandor Magony, Yoon-Sok Chung, Byung-Joon Kim, Kyu Yeon Hur, Ho-Cheol Kang, Jung Hee Kim, Min Seon Kim, Aldona Kowalska, Marek Bolanowski, Marek Ruchala, Svetozar Damjanovic, Juraj Payer, Yun Jung Choi, Su Jin Heo, Tae Kyoung Kim, MinKyu Heo, Joan Lee, Eun Jig Lee
OBJECTIVE: Hybrid Fc-fused rhGH (GX-H9) is a long-acting recombinant human growth hormone (GH) under clinical development for both adults and children with GH deficiency (GHD). We compared the safety, pharmacokinetics and pharmacodynamics of weekly and every other week (EOW) dosages of GX-H9 with those of daily GH administration in adult GHD (AGHD) patients. DESIGN: This was a randomized, open-label, active-controlled and dose-escalation study conducted in 16 endocrinology centers in Europe and Korea...
September 2018: European Journal of Endocrinology
https://www.readbyqxmd.com/read/29960877/-efficacy-and-safety-of-replacement-treatment-in-isolated-growth-hormone-deficiency
#19
Ana Belén Ariza Jiménez, María José Martínez-Aedo Ollero, Juan Pedro López-Siguero
INTRODUCTION: Growth in patients with isolated growth hormone (GH) deficiency is heterogeneous despite treatment due to the low specificity of diagnostic tests, making it necessary to define efficacy variables. AIMS: To evaluate efficacy of hormone replacement therapy in children with isolated GH deficiency. METHODS: Observational-ambispective study of patients treated in our department in the last 14 years for isolated GH deficiency. This was defined as a GH level less than 7...
June 27, 2018: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/29959177/growth-hormone-deficiency-causing-micropenis-lessons-learned-from-a-well-adjusted-adult
#20
Peter A Lee, Tom Mazur, Christopher P Houk, Robert M Blizzard
This report of a 46,XY patient born with a micropenis consistent with etiology from isolated congenital growth hormone deficiency is used to (1) raise the question regarding what degree testicular testosterone exposure to the central nervous system during fetal life and early infancy has on the development of male gender identity, regardless of gender of rearing; (2) suggest the obligatory nature of timely full disclosure of medical history; (3) emphasize that virtually all 46,XY infants with functional testes and a micropenis should be initially boys except some with partial androgen insensitivity syndrome; and (4) highlight the sustaining value of a positive long-term relationship with a trusted physician (R...
July 2018: Pediatrics
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