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Adult Growth Hormone Deficiency

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). Deficiencies of growth hormone (GH), thyroid hormone, adrenocorticotropic hormone, and gonadotropins were determined clinically and serologically...
August 17, 2018: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
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
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
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...
July 16, 2018: Pituitary
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...
June 27, 2018: Journal of Diabetes and its Complications
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
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
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
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
Cheol Ryong Ku, Thierry Brue, Katharina Schilbach, Stanislav Ignatenko, Sándor 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...
July 4, 2018: European Journal of Endocrinology
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.)
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
Francis de Zegher, Nele Reynaert, Lien De Somer, Carine Wouters, Mathieu Roelants
BACKGROUND: Biologicals targeting the interleukin (IL)-1β or IL-6 pathway are becoming prime choices for the treatment of children with systemic juvenile idiopathic arthritis (sJIA). Up to 1 in 3 sJIA children receiving such treatment continues to have inflammatory activity and to require supra-physiological glucocorticoid doses which may reduce growth velocity for years and may lead to an extremely short stature for age, if not for life. Currently, there is no long-term proposal to normalize the adult height of these children with sJIA...
June 25, 2018: Hormone Research in Pædiatrics
David M Siebert, Ashwin L Rao
CONTEXT: Recombinant human growth hormone (rHGH) has become a target of abuse in the sporting world. Conversely, sports medicine clinicians may encounter athletes using rHGH to achieve normalcy in the context of growth hormone (GH) deficiency. EVIDENCE ACQUISITION: Medline and PubMed databases were queried using the following keywords: GH, GH physiology, GH deficiency, acromegaly, GH athlete, GH sports, GH athletic performance, and GH deficiency concussion. Articles focusing on GH physiology, deficiency, excess, and its effects in both deficient and healthy patients were included...
June 1, 2018: Sports Health
Amélie Poidvin, Jean-Claude Carel, Emmanuel Ecosse, Dominique Levy, Jean Michon, Joël Coste
The association between growth hormone (GH) treatment and cancer risk has not been thoroughly evaluated and there are questions about any increased risk of bone tumors. We examined cancer risk and especially bone tumor risk in a population-based cohort study of 6874 patients treated with recombinant GH in France for isolated GH deficiency, short stature associated with low birth weight or length or idiopathic short stature. Adult mortality and morbidity data obtained from national databases and from questionnaires...
June 14, 2018: Cancer Medicine
Atif Nawaz, Muhammad Azeemuddin, Jehanzeb Shahid
Pituitary stalk interruption syndrome (PSIS) is a distinct and rare clinical entity responsible for congenital hypopituitarism resulting in deficiency of pituitary hormones with deficiency of the growth hormone (100%) and gonadotropins (97.2%) being its most common presentation at the time of hospital encounter (Wang et al., 2015). Isolated sparing of thyroid-stimulating hormone (TSH) with deficiency of the remaining anterior pituitary hormones may be present in PSIS, as is true in our case. Therefore, it should be kept in mind at the time of examination in suspected cases of PSIS...
April 2018: Radiology Case Reports
Sandro Loche, Natascia Di Iorgi, Giuseppa Patti, Serena Noli, Marta Giaccardi, Irene Olivieri, Anastasia Ibba, Mohamad Maghnie
Growth hormone (GH) is essential not only for normal growth during childhood, but also for the acquisition of bone mass and muscle strength in both sexes. This process is completed after the achievement of adult height in the phase of transition from adolescence to adulthood. Adolescents with childhood onset GH deficiency (GHD) show reduction of bone mineral density, decrease in lean body mass, increase in fat mass, and deterioration of the lipid profile. For this reason, continuation of GH replacement therapy in the transition age is recommended in all patients with a confirmed diagnosis of GHD...
2018: Endocrine Development
Sarah Thilmany, Leila Mchirgui, Chloé Brunelle, Véronique Beauloye, Dominique Maiter, Orsalia Alexopoulou
Our aim was to analyze a cohort of patients with childhood-onset growth hormone deficiency (GHD) to evaluate if there is some correlation between the response to GH treatment during childhood and adulthood, respectively. This was an observational retrospective monocentric cohort study of 47 patients treated with GH during childhood and adulthood. Changes in growth parameters during childhood were compared with the increase of IGF-I z-score and other indexes of GH response (body composition, lipid profile) after 1 year of treatment in adulthood...
June 2018: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
Jose M Garcia, Beverly M K Biller, Márta Korbonits, Vera Popovic, Anton Luger, Christian J Strasburger, Philippe Chanson, Milica Medic-Stojanoska, Jochen Schopohl, Anna Zakrzewska, Sandra Pekic-Djurdjevic, Marek Bolanowski, Ronald Swerdloff, Christina Wang, Thomas Blevins, Marco Marcelli, Nicola Ammer, Richard Sachse, Kevin C J Yuen
PURPOSE: Diagnosis of adult growth hormone deficiency (AGHD) is challenging and often requires confirmation with a GH stimulation test (GHST). The insulin tolerance test (ITT) is considered the gold standard GHST but is labor-intensive, may cause severe hypoglycemia, and is contraindicated in certain patients. Macimorelin, an orally-active GH secretagogue, could be used to diagnose AGHD by measuring stimulated GH levels after an oral dose. METHODS: This multicenter, open-label, randomized, 2-way crossover trial was designed to validate the efficacy and safety of a single-dose oral macimorelin for AGHD diagnosis compared to the ITT...
May 31, 2018: Journal of Clinical Endocrinology and Metabolism
Shirin Hasani-Ranjbar, Hanieh Sadat Ejtahed, Mahsa M Amoli, Fatemeh Bitarafan, Mostafa Qorbani, Akbar Soltani, Bahareh Yarjoo
OBJECTIVE: Hereditary Hypophosphatemic Rickets with Hypercalciuria (HHRH) is a very rare inheritable hypophosphatemic rickets/osteomalacia characterized by decreased renal phosphate reabsorption, hypophosphatemia, vitamin D refractory rickets, hyperphosphaturia, hypercalciuria, elevated circulating 1, 25-dihydroxy vitamin D levels and low serum parathyroid hormone (PTH) levels, leading to growth retardation, limb deformities, bone pain, muscle weakness, rickets and osteomalacia. Biallelic mutations in SLC34A3/NPT2c gene are responsible for the occurrence of the disease...
May 29, 2018: Journal of Clinical Research in Pediatric Endocrinology
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