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    13233 results match your criteria Growth Hormone Deficiency

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    Efficient Non-Invasive Plasmid-DNA Administration into Tibialis Cranialis Muscle of "Little" Mice.
    Curr Mol Med 2017 Jul 21. Epub 2017 Jul 21.
    Biotechnology Center, Instituto de Pesquisas Energéticas e Nucleares (IPEN-CNEN), Cidade Universitária, São Paulo, SP, Brazil.
    An alternative treatment for growth hormone deficiency based on hGH-DNA administration, followed by electro gene transfer, was investigated by injecting the plasmid into surgically exposed or non-exposed quadriceps or tibialis muscle of immunodeficient "little" mice. Previously optimized electrotransfer conditions were also improved via a new combination of high/low voltage pulses: after 3 days, serum hGH was determined. Both groups exhibited similar results: 5. Read More

    Growth hormone, prolactin, and sexuality.
    J Endocrinol Invest 2012 Sep 22;35(8):782-794. Epub 2014 Mar 22.
    Department of Molecular & Clinical Endocrinology and Oncology, "Federico II" University of Naples, Via S. Pansini 5, 80131, Naples, Italy.
    GH and PRL, although not considered as 'classi cal' sexual hormones, could play a role in the endocrine control of sexual function both in men and women. Physiologically, PRL seems to be involved in the central control of sexual behavior and activity, by modulating mainly the effects of dopaminergic and serotoninergic systems on sexual function. Indeed, circulating PRL levels increase after orgasm and may potentially play a role in the acute regulation of further sexual arousal following orgasm both in men and women. Read More

    PREPL deficiency: delineation of the phenotype and development of a functional blood assay.
    Genet Med 2017 Jul 20. Epub 2017 Jul 20.
    Laboratory of Biochemical Neuroendocrinology, Department of Human Genetics, University of Leuven, Leuven, Belgium.
    PurposePREPL deficiency causes neonatal hypotonia, ptosis, neonatal feeding difficulties, childhood obesity, xerostomia, and growth hormone deficiency. Different recessive contiguous gene deletion syndromes involving PREPL and a variable combination of SLC3A1 (hypotonia-cystinuria syndrome), CAMKMT (atypical hypotonia-cystinuria syndrome), and PPM1B (2p21 deletion syndrome) have been described. In isolated PREPL deficiency, previously described only once, the absence of cystinuria complicates the diagnosis. Read More

    Hepatic JAK2 protects against atherosclerosis through circulating IGF-1.
    JCI Insight 2017 Jul 20;2(14). Epub 2017 Jul 20.
    Toronto General Hospital Research Institute, University Health Network, Toronto, Ontario, Canada.
    Atherosclerosis is considered both a metabolic and inflammatory disease; however, the specific tissue and signaling molecules that instigate and propagate this disease remain unclear. The liver is a central site of inflammation and lipid metabolism that is critical for atherosclerosis, and JAK2 is a key mediator of inflammation and, more recently, of hepatic lipid metabolism. However, precise effects of hepatic Jak2 on atherosclerosis remain unknown. Read More

    Childhood-Onset Adult Growth Hormone Deficiency: Clinical, Hormonal, and Radiological Assessment in a Single Center in China.
    Horm Res Paediatr 2017 Jul 18. Epub 2017 Jul 18.
    Background: Although growth hormone deficiency (GHD) is an important issue in pediatric patients, adult GHD (AGHD) is a neglected field of endocrinology in China. The aim of this study is to characterize the clinical, hormonal, and radiological features in childhood-onset AGHD (CO AGHD) in a single center in China and to compare them with counterparts from Japan.

    Methods: The medical records of 78 Chinese patients with CO AGHD were reviewed and compared with data from the HypoCCS database study from Japan (N = 69). Read More

    Vitamin D levels in critically ill patients with acute kidney injury: a protocol for a prospective cohort study (VID-AKI).
    BMJ Open 2017 Jul 12;7(7):e016486. Epub 2017 Jul 12.
    Department of Critical Care, Guy's and St Thomas' NHS Foundation Trust, London, UK.
    Introduction: Acute kidney injury (AKI) affects more than 50% of critically ill patients. The formation of calcitriol, the active vitamin D metabolite, from the main inactive circulating form, 25-hydroxyvitamin D (25(OH)D), occurs primarily in the proximal renal tubules. This results in a theoretical basis for reduction in levels of calcitriol over the course of an AKI. Read More

    Arid1b haploinsufficient mice reveal neuropsychiatric phenotypes and reversible causes of growth impairment.
    Elife 2017 Jul 11;6. Epub 2017 Jul 11.
    Children's Research Institute, University of Texas Southwestern Medical Center, Dallas, United States.
    Sequencing studies have implicated haploinsufficiency of ARID1B, a SWI/SNF chromatin-remodeling subunit, in short stature (Yu et al., 2015), autism spectrum disorder (O'Roak et al., 2012), intellectual disability (Deciphering Developmental Disorders Study, 2015), and corpus callosum agenesis (Halgren et al. Read More

    Impact of discontinuation of growth hormone treatment on lipids and weight status in adolescents.
    J Pediatr Endocrinol Metab 2017 Jun 24. Epub 2017 Jun 24.
    Background: While the main role of growth hormone (GH) replacement therapy in children is to promote linear growth, GH has also an effect on lipids and body composition. There is an ongoing discussion whether discontinuation of GH treatment is associated with deterioration of lipids.

    Methods: We analyzed weight status [as body mass index-standard deviation score (BMI-SDS)], insulin like growth factor (IGF)-1, triglycerides, total, low-density liporptoein (LDL)- and high-density lipoprotein (HDL)-cholesterol at the end of GH treatment and in mean 6 months later in 90 adolescents (53 with GH deficiency, 16 with Turner syndrome [TS] and 21 born small-for-gestational age [SGA]). Read More

    2q37 Deletion syndrome confirmed by high-resolution cytogenetic analysis.
    Ann Pediatr Endocrinol Metab 2017 Jun 28;22(2):129-132. Epub 2017 Jun 28.
    Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
    Chromosome 2q37 deletion syndrome is a rare chromosomal disorder characterized by mild to moderate developmental delay, brachydactyly of the third to fifth digits or toes, short stature, obesity, hypotonia, a characteristic facial appearance, and autism spectrum disorder. Here, we report on a patient with 2q37 deletion presenting with dilated cardiomyopathy (DCMP). Congenital heart malformations have been noted in up to 20% of patients with 2q37 deletions. Read More

    Effect of growth hormone treatment on children with idiopathic short stature and idiopathic growth hormone deficiency.
    Ann Pediatr Endocrinol Metab 2017 Jun 28;22(2):119-124. Epub 2017 Jun 28.
    Department of Pediatrics, Chungbuk National University Hospital, Chungbuk National University College of Medicine, Cheongju, Korea.
    Purpose: There are inconsistencies in the results reported in a small number of previous studies into growth hormone (GH) treatment in Korean children with idiopathic short stature (ISS) and idiopathic growth hormone deficiency (IGHD). Thus, the authors retrospectively compared the effects of GH in ISS and IGHD.

    Methods: From the medical records of 26 ISS and 30 IGHD children, auxological and biochemical changes including chronologic age (CA), bone age (BA), height standard deviation score (HT-SDS), predicted adult height (PAH), midparental height (MPH), insulin-like growth factor-1 (IGF-1), and insulin-like growth factor binding protein-3 (IGFBP-3) were compared. Read More

    Atypical skeletal manifestations of rickets in a familial hypocalciuric hypercalcemia patient.
    Bone Res 2017 27;5:17001. Epub 2017 Jun 27.
    Department of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.
    Familial hypocalciuric hypercalcemia (FHH) is caused by inactivating mutations in the calcium-sensing receptor (CaSR) gene. The loss of function of CaSR presents with rickets as the predominant skeletal abnormality in mice, but is rarely reported in humans. Here we report a case of a 16-year-old boy with FHH who presented with skeletal manifestations of rickets. Read More

    Survey on the levels of 25-hydroxy vitamin D and bone metabolic markers and evaluation of their correlations with osteoporosis in perimenopausal woman in Xi'an region.
    PLoS One 2017 7;12(7):e0180366. Epub 2017 Jul 7.
    The Laboratory Department of Xi'an Jiaotong University Medical College First Affiliated Hospital, Xi'an, Shaanxi, China.
    It has been accepted that vitamin D (VD) plays an important role in bone metabolism. However, the levels of VD in people of different regions are quite different and there is still no final conclusion on the significant correlation between VD and osteoporosis. 245 cases of peri-menopausal women were collected to study the relationship between VD and osteoporosis in western China. Read More

    Growth impairment and gonadal axis abnormalities are common in survivors of paediatric brain tumours.
    Acta Paediatr 2017 Jul 6. Epub 2017 Jul 6.
    Department of Pediatrics, Tampere University Hospital, Tampere, Finland.
    Aim: Childhood brain tumour survivors have a high risk of endocrine morbidity. This study evaluated the growth, pubertal development and gonadal function in survivors of childhood brain tumours and identified factors associated with the problems we observed.

    Methods: The 52 subjects (52% male) were diagnosed in 1983-1997 and treated for brain tumours at Tampere University Hospital, Finland. Read More

    The response to growth hormone treatment in prepubertal children with growth hormone deficiency in Japan: Comparing three consecutive years of treatment data of The Foundation for Growth Science in Japan between the 1990s and 2000s.
    Endocr J 2017 Jul 4. Epub 2017 Jul 4.
    Growth Hormone (GH) and its related Factors Study Committee and GH Treatment Study Committee, The Foundation for Growth Science in Japan, Tokyo, Japan.
    Growth hormone (GH) treatment for children with GH deficiency (GHD) is effective in improving adult height. To achieve favorable effects, GH treatment before puberty is very important, because prepubertal height gain is highly correlated with total height gain. However, no report has studied the effects by analyzing a nationwide data from recent GHD patients in Japan. Read More

    The Role of Growth Hormone and Insulin-Like Growth Factor-I in the Liver.
    Int J Mol Sci 2017 Jul 5;18(7). Epub 2017 Jul 5.
    Division of Diabetes and Endocrinology, Department of Internal Medicine, Kobe University Graduate School of Medicine, 7-5-2, Kusunoki-cho, Chuo-ku, Kobe 650-0017, Japan.
    Adult growth hormone deficiency (GHD) is characterized by metabolic abnormalities associated with visceral obesity, impaired quality of life, and increased mortality. Patients with adult GHD show increased prevalence of non-alcoholic fatty liver disease (NAFLD)/non-alcoholic steatohepatitis (NASH), and growth hormone (GH) replacement therapy has been shown to improve these conditions. It has also been demonstrated that a decrease in the GH insulin-like growth factor-I (IGF-I) axis is closely associated with the progression of general NAFLD, suggesting a physiological role of these hormones for the maintenance of the liver. Read More

    Experiencing health-related quality of life in paediatric short stature - a cross-cultural analysis of statements from patients and parents.
    Clin Psychol Psychother 2017 Jul 4. Epub 2017 Jul 4.
    Department of Medical Psychology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
    Objectives: Direct assessment of the patient perspective is necessary to thoroughly understand patients' experiences of disease. We aimed to examine information from children with short stature on their perceived HrQoL within 5 European countries.

    Methods: Patients, identified through clinical databases, were approached by their clinicians according to the inclusion criteria regarding a diagnosis of growth hormone deficiency or idiopathic short stature and age requirements. Read More

    Impact of discontinuation of growth hormone treatment on lipids and weight status in adolescents.
    J Pediatr Endocrinol Metab 2017 Jul;30(7):749-757
    Background: While the main role of growth hormone (GH) replacement therapy in children is to promote linear growth, GH has also an effect on lipids and body composition. There is an ongoing discussion whether discontinuation of GH treatment is associated with deterioration of lipids.

    Methods: We analyzed weight status [as body mass index-standard deviation score (BMI-SDS)], insulin like growth factor (IGF)-1, triglycerides, total, low-density liporptoein (LDL)- and high-density lipoprotein (HDL)-cholesterol at the end of GH treatment and in mean 6 months later in 90 adolescents (53 with GH deficiency, 16 with Turner syndrome [TS] and 21 born small-for-gestational age [SGA]). Read More

    The impact of growth hormone on proteomic profiles: a review of mouse and adult human studies.
    Clin Proteomics 2017 29;14:24. Epub 2017 Jun 29.
    Edison Biotechnology Institute, Ohio University, Athens, OH USA.
    Growth hormone (GH) is a protein that is known to stimulate postnatal growth, counter regulate insulin's action and induce expression of insulin-like growth factor-1. GH exerts anabolic or catabolic effects depending upon on the targeted tissue. For instance, GH increases skeletal muscle and decreases adipose tissue mass. Read More

    [Ten years experience with the first approved biosimilar recombinant human growth hormone drug in normal clinical practice].
    An Pediatr (Barc) 2017 Jun 28. Epub 2017 Jun 28.
    Facultad de Medicina, Universidad Santiago de Compostela, Santiago de Compostela, A Coruña, España.
    Introduction: Recombinant human growth hormone (rhGH) is the first biosimilar drug approved by the European Medicines Agency in 2006, using the biosimilar registration process. It was authorised for the treatment of growth hormone deficiency, and growth disorders associated with Turner's syndrome, chronic renal failure, Prader-Willi syndrome, and growth disorders in children/adolescents born small for gestational age, and replacement therapy in adults with pronounced growth hormone deficiency.

    Materials And Methods: This review is focused on the scientific evidence published about this drug in the last ten years, including the clinical trials on which the approval of the regulatory authority is based, and the most relevant studies evaluating the clinical impact of the drug in clinical practice. Read More

    Long-term and late treatment consequences: endocrine and metabolic effects.
    Curr Opin Support Palliat Care 2017 Jun 28. Epub 2017 Jun 28.
    aInstitute of Cardiovascular Research, Royal Holloway, University of London & Ashford and St Peter's Hospitals NHS Foundation Trust, Egham bUniversity Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
    Purpose Of Review: Cancer therapies often result in the 'late effect of cancer treatment' whereby secondary health complications emerge years after radiotherapy and chemotherapy. This review focuses on endocrine and metabolic consequences in adult cancer survivors as late treatment effects.

    Recent Findings: Endocrine and metabolic disorders are among the most common late effects. Read More

    Insulin resistance in endocrine disorders - treatment options.
    Endokrynol Pol 2017 ;68(3):334-351
    Department of Internal Medicine and Diabetology, Poznan University of Medical Science, Poland.
    Changes in sensitivity to insulin occur in the course of a number of endocrine disorders. Most of the hormones through their antagonistic action to insulin lead to increased hepatic glucose output and its decreased utilisation in peripheral tissues. Carbohydrate disorders observed in endocrine diseases result from the phenomenon of insulin resistance, and in some cases also a reduction in insulin secretion is present. Read More

    Effect of fetal hypothyroidism on MyomiR network and its target gene expression profiles in heart of offspring rats.
    Mol Cell Biochem 2017 Jun 28. Epub 2017 Jun 28.
    Drug Applied Research Center, Tabriz University of Medical Sciences, Tabriz, Iran.
    Thyroid hormone deficiency during fetal life (fetal hypothyroidism) causes intrauterine growth restriction (IUGR). Fetal hypothyroidism (FH) could attenuate normal cardiac functions in the later life of the offspring rats. The aim of this study was to evaluate the contribution of myomiR network and its target gene expression in cardiac dysfunction in fetal hypothyroid rats. Read More

    Clinical and Molecular Characterization of Prader-Willi Syndrome.
    Indian J Pediatr 2017 Jun 29. Epub 2017 Jun 29.
    Department of Pediatric Genetics, Indira Gandhi Institute of Child Health, South Hospital Complex, Dharmaram College Post, Bangalore, Karnataka, 560 029, India.
    Objectives: To describe the clinical presentations and molecular diagnosis to aid the clinicians in early diagnosis and appropriate management of Prader-Willi syndrome (PWS).

    Methods: Thirty-four clinically diagnosed PWS cases were enrolled after obtaining informed consent/assent. Demographic details, clinical data and anthropometry were recorded using structured proforma. Read More

    Somapacitan, a once-weekly reversible albumin-binding GH derivative, in children with GH deficiency: a randomized dose-escalation trial.
    Clin Endocrinol (Oxf) 2017 Jun 28. Epub 2017 Jun 28.
    Department of Women's and Children's Health, Department of Women's and Children's Health, Karolinska Institutet, and Pediatric Endocrinology Unit, Karolinska University Hospital, Stockholm, Sweden.
    Objective: To evaluate the safety, local tolerability, pharmacodynamics and pharmacokinetics of escalating single doses of once-weekly somapacitan, a reversible, albumin-binding GH derivative, versus once-daily GH in children with GH deficiency (GHD).

    Design: Phase 1, randomized, open-label, active-controlled, dose-escalation trial (NCT01973244).

    Patients: Thirty-two pre-pubertal GH-treated children with GHD were sequentially randomized 3:1 within each of four cohorts to a single dose of somapacitan (0. Read More

    Serum adiponectin levels in adolescents and young adults with growth hormone deficiency.
    • Authors:
    Neuro Endocrinol Lett 2017 May 26;38(2):107-116. Epub 2017 May 26.
    Introduction: Adiponectin (APN) is adipose tissue-derived hormone influencing energy metabolism. Growth hormone deficiency (GHD) may contribute to the development of disturbances in the hormonal function of adipose tissue (AT), and many disorders observed in untreated patients with GHD coincides with these contributed to low serum APN levels.

    Objectives: The assessment of serum adiponectin levels in adolescents and young adults with severe or partial GHD and analysis of relationships between serum APN and GH/IGF-1 axis function impairment as well as cardiometabolic risk factors. Read More

    Fibroblast growth factor 19 regulates skeletal muscle mass and ameliorates muscle wasting in mice.
    Nat Med 2017 Jun 26. Epub 2017 Jun 26.
    Department of Biology, University of Bergen, Bergen, Norway.
    The endocrine-derived hormone fibroblast growth factor (FGF) 19 has recently emerged as a potential target for treating metabolic disease. Given that skeletal muscle is a key metabolic organ, we explored the role of FGF19 in that tissue. Here we report a novel function of FGF19 in regulating skeletal muscle mass through enlargement of muscle fiber size, and in protecting muscle from atrophy. Read More

    Body Mass Disorders in Healthy Short Children and in Children with Growth Hormone Deficiency.
    Adv Exp Med Biol 2017 Jun 23. Epub 2017 Jun 23.
    Department of Health Sciences, CUNY Lehman College, New York, USA.
    The aim of the study was to determine the degree of adiposity and the incidence of body mass disorders, including abdominal obesity, in healthy short children and children with growth hormone deficiency. The study included 134 short children (height < 10th percentile) aged 7-15. In this cohort there were 63 (31 boys and 32 girls) children without diagnosed hormonal disorders and 71 patients (35 boys and 36 girls) with growth hormone deficiency. Read More

    Severe Short Stature in an Adolescent Male with Prader-Willi Syndrome and Congenital Adrenal Hyperplasia: A Therapeutic Conundrum.
    Case Rep Endocrinol 2017 30;2017:4271978. Epub 2017 May 30.
    Division of Pediatric Endocrinology, Rhode Island Hospital and Hasbro Children's Hospital, The Warren Alpert Medical School, Brown University, Providence, RI, USA.
    Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency results in excess androgen production which can lead to early epiphyseal fusion and short stature. Prader-Willi syndrome (PWS) is a genetic disorder resulting from a defect on chromosome 15 due to paternal deletion, maternal uniparental disomy, or imprinting defect. Ninety percent of patients with PWS have short stature. Read More

    Etiological factors of short stature in children and adolescents: experience at a tertiary care hospital in Egypt.
    Ther Adv Endocrinol Metab 2017 May 3;8(5):75-80. Epub 2017 May 3.
    Department of Clinical Pathology, Faculty of Medicine, Aswan University, Aswan, Egypt.
    Background: Accurate anthropometric measurements and critical analysis of growth data allow the clinician to promptly recognize children with short stature. The aim of this study was to determine the frequency of etiological factors causing short stature among children referred to the pediatric endocrinology clinic of Assiut University Children's Hospital, the main tertiary care center in Upper Egypt.

    Methods: We conducted this descriptive observational study from May 2012 to December 2015, to analyze 637 children (boys 354, girls 283) with short stature. Read More

    More Favorable Metabolic Impact of Three-Times-Weekly versus Daily Growth Hormone Treatment in Naïve GH-Deficient Children.
    Int J Endocrinol 2017 28;2017:8469680. Epub 2017 May 28.
    Section of Endocrinology, Biomedical Department of Internal and Specialist Medicine (DIBIMIS), University of Palermo, Palermo, Italy.
    Objective: To evaluate whether two different regimens of weekly injections could lead to similar auxological and metabolic effects in children with growth hormone deficiency (GHD).

    Design: 32 GHD children (25 males, mean age 10.5 ± 2. Read More

    Isolated growth hormone deficiency due to the R183H mutation in GH1: Clinical analysis of a four-generation family.
    Clin Endocrinol (Oxf) 2017 Jun 19. Epub 2017 Jun 19.
    Division of Endocrinology, Cincinnati Center for Growth Disorders, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.

    Transcriptome Analysis of Differentially Expressed Genes Induced by Low and High Potassium Levels Provides Insight into Fruit Sugar Metabolism of Pear.
    Front Plant Sci 2017 31;8:938. Epub 2017 May 31.
    Key Laboratory of Plant Nutrition and Fertilization in Low-Middle Reaches of the Yangtze River, Ministry of Agriculture, Jiangsu Key Laboratory of Solid Organic Waste Utilization, Jiangsu Collaborative Innovation Center for Solid Organic Waste Resource Utilization, College of Resources and Environmental Science, Nanjing Agricultural UniversityNanjing, China.
    Potassium (K) deficiency is a common abiotic stress that can inhibit the growth of fruit and thus reduce crop yields. Little research has been conducted on pear transcriptional changes under low and high K conditions. Here, we performed an experiment with 7-year-old pot-grown "Huangguan" pear trees treated with low, Control or high K levels (0, 0. Read More

    Vitamin D levels in newborns and association with neonatal hypocalcemia.
    J Matern Fetal Neonatal Med 2017 Jun 14:1-5. Epub 2017 Jun 14.
    b Büyük Anadolu Hastanesi , Samsun , Turkey.
    Objective: Vitamin D has many important functions in our body. Especially in intrauterine and early infancy periods, Vitamin D plays a major role in bone development, growth, and the maturation of tissues such as lung and brain. Fetus is dependent on the mother in terms of Vitamin D and maternal Vitamin D deficiency results in a Vitamin D deficient newborn. Read More

    Oral glucose-stimulated growth hormone (GH) test in adult GH deficiency patients and controls: Potential utility of a novel test.
    Eur J Intern Med 2017 Jun 9. Epub 2017 Jun 9.
    Department of Medicine, Faculty of Health Sciences, University of A Coruña, A Coruña, Spain; Instituto de Investigación Biomedica (INIBIC), University Hospital A Coruña, A Coruña, Spain; Department of Endocrinology, University Hospital A Coruña, A Coruña, Spain. Electronic address:
    Context: The diagnosis of adult GH deficiency requires confirmation with a GH stimulation test. Oral glucose (OG) administration affects GH secretion, initially decreasing and subsequently stimulating GH secretion.

    Objective: The aim of this study was to investigate the diagnostic efficacy and safety of a long OG test (LOGT) as a stimulus of GH secretion for the diagnosis of adult GH deficiency (AGHD). Read More

    Comparison of Bone Mineral Density in Common variable immunodeficiency and X-Linked Agammaglobulinaemia Patients.
    Endocr Metab Immune Disord Drug Targets 2017 Jun 11. Epub 2017 Jun 11.
    Pediatric Research Center, Qom University of Medical Sciences, Qom. Iran.
    Background: Primary antibody deficiency (PAD) is the most common group of primary immunodeficiency disorders, resulting from different defects in development and function of B cell lineage. Common variable immunodeficiency (CVID) and X-linked agammaglobulinemia (XLA) are two of the major types of PADs. Optimal growth and subsequently bone health could potentially compromise due to the interference of several factors in PAD with childhood onset. Read More

    Klotho Lacks an FGF23-Independent Role in Mineral Homeostasis.
    J Bone Miner Res 2017 Jun 10. Epub 2017 Jun 10.
    Department of Biomedical Sciences, University of Veterinary Medicine Vienna, Vienna, Austria.
    Fibroblast growth factor-23 (FGF23) is a bone-derived hormone regulating vitamin D hormone production and renal handling of minerals by signaling through an FGF receptor/αKlotho (Klotho) receptor complex. Whether Klotho has FGF23-independent effects on mineral homeostasis is a controversial issue. Here, we aimed to shed more light on this controversy by comparing male and female triple knockout mice with simultaneous deficiency in Fgf23 and Klotho and a nonfunctioning vitamin D receptor (VDR) (Fgf23/Klotho/VDR) with double (Fgf23/VDR, Klotho/VDR, and Fgf23/Klotho) and single Fgf23, Klotho, and VDR mutants. Read More

    Relationship between IGF-I Concentration and Metabolic Profile in Children with Growth Hormone Deficiency: The Influence of Children's Nutritional State as well as the Ghrelin, Leptin, Adiponectin, and Resistin Serum Concentrations.
    Int J Endocrinol 2017 15;2017:5713249. Epub 2017 May 15.
    Department of Endocrinology and Metabolic Diseases, Polish Mother's Memorial Hospital-Research Institute, Rzgowska Street 281/289, 93-338 Lodz, Poland.
    Background: Some, however not all, children with growth hormone deficiency (GHD) reveal a tendency towards metabolic disorders. Insulin-like growth factor I (IGF-I) is the main mediator of GH anabolic effects.

    Objective: The aim of the study was to compare ghrelin, adiponectin, leptin, resistin, lipid, glucose, and insulin concentrations in GHD children, depending on the IGF-I bioavailability. Read More

    Nonclinical pharmacokinetic and pharmacodynamic characterisation of somapacitan: A reversible non-covalent albumin-binding growth hormone.
    Growth Horm IGF Res 2017 May 24;35:8-16. Epub 2017 May 24.
    Novo Nordisk A/S, Protein & Peptide Chemistry, DK-2760, Maaloev, Denmark.
    Objective: Somapacitan is an albumin-binding growth hormone derivative intended for once weekly administration, currently in clinical development for treatment of adult as well as juvenile GH deficiency. Nonclinical in vivo pharmacological characterisation of somapacitan was performed to support the clinical trials. Here we present the pharmacokinetic and pharmacodynamic effects of somapacitan in rats, minipigs, and cynomolgus monkeys. Read More

    Genetic analysis of IRF6, a gene involved in craniofacial midline formation, in relation to pituitary and facial morphology of patients with idiopathic growth hormone deficiency.
    Pituitary 2017 Jun 7. Epub 2017 Jun 7.
    Dept. of Internal Medicine, Subdiv. Endocrinology, Erasmus MC, University Medical Center, 's Gravendijkwal 230, 3015 CE, Rotterdam, The Netherlands.
    Introduction: Growth hormone is secreted by the pituitary gland, which forms part of the craniofacial midline. IRF6 encodes a transcription factor involved in the development of the craniofacial midline and mutations in IRF6 are known to disturb craniofacial development. Craniofacial and pituitary development are closely related. Read More

    Hypothalamic hamartoma with epilepsy: Review of endocrine comorbidity.
    Epilepsia 2017 Jun;58 Suppl 2:50-59
    Pediatric Neurology Division and Hypothalamic Hamartoma Program, Barrow Neurological Institute at Phoenix Children's Hospital, Phoenix, Arizona, U.S.A.
    The most common, and usually the only, endocrine disturbance in patients with hypothalamic hamartoma (HH) and epilepsy is central precocious puberty (CPP). The mechanism for CPP associated with HH may relate to ectopic generation and pulsatile release of gonadotropin-releasing hormone (GnRH) from the HH, but this remains an unproven hypothesis. Possible regulators of GnRH release that are intrinsic to HH tissue include the following: (1) glial factors (such as transforming growth factor α[TGFα) and (2) γ-aminobutyric acid (GABA)-mediated excitation. Read More

    Long term follow-up of a case with proprotein convertase1/3 deficiency: Transient diabetes mellitus with intervening diabetic ketoacidosis during growth hormone therapy.
    J Clin Res Pediatr Endocrinol 2017 Jun 7. Epub 2017 Jun 7.
    Proprotein convertase 1/3 deficiency is a very rare disease characterized by severe intractable diarrhea in the first years of life, followed by obesity and several hormonal deficiencies later. Diabetes mellitus requiring insulin treatment and diabetic ketoacidosis have not been reported before in this disorder. We herein present a girl with proprotein convertase 1/3 deficiency who has been followed from birth to 17 years of age. Read More

    MRI features of growth hormone deficiency in children with short stature caused by pituitary lesions.
    Exp Ther Med 2017 Jun 24;13(6):3474-3478. Epub 2017 Apr 24.
    Department of Radiology, Xuzhou Children's Hospital, Xuzhou, Jiangsu 221002, P.R. China.
    We verified the advantages of using magnetic resonance imaging (MRI) for improving the diagnostic quality of growth hormone deficiency (GHD) in children with short stature caused by pituitary lesions. Clinical data obtained from 577 GHD patients with short stature caused by pituitary lesions were retrospectively analyzed. There were 354 cases (61. Read More

    A heterozygous microdeletion of 20p12.2-3 encompassing PROKR2 and BMP2 in a patient with congenital hypopituitarism and growth hormone deficiency.
    Am J Med Genet A 2017 Aug 6;173(8):2261-2267. Epub 2017 Jun 6.
    Manchester Academic Health Sciences Centre, Centre for Paediatrics and Child Health, Institute of Human Development, University of Manchester, Manchester, UK.
    Congenital growth hormone deficiency is a rare disorder with an incidence of approximately 1 in 4,000 live births. Pituitary development is under the control of a multitude of spatiotemporally regulated signaling molecules and transcription factors. Mutations in the genes encoding these molecules can result in hypopituitarism but for the majority of children with congenital hypopituitarism, the aetiology of their disease remains unknown. Read More

    Phytohormone ethylene-responsive Arabidopsis organ growth under light is in the fine regulation of Photosystem II deficiency-inducible AKIN10 expression.
    Sci Rep 2017 Jun 5;7(1):2767. Epub 2017 Jun 5.
    Department of Life Sciences, Division of Life Sciences, KOREA University, Seoul, Korea.
    For photoautotrophic plants, light-dependent photosynthesis plays an important role in organismal growth and development. Under light, Arabidopsis hypocotyl growth is promoted by the phytohormone ethylene. Despite well-characterized ethylene signaling pathways, the functions of light in the hormone-inducible growth response still remain elusive. Read More

    Congenital adrenal hyperplasia.
    Lancet 2017 May 30. Epub 2017 May 30.
    National Institutes of Health Clinical Center, Bethesda, MD 20892, USA; The Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, MD, USA. Electronic address:
    Congenital adrenal hyperplasia is a group of autosomal recessive disorders encompassing enzyme deficiencies in the adrenal steroidogenesis pathway that lead to impaired cortisol biosynthesis. Depending on the type and severity of steroid block, patients can have various alterations in glucocorticoid, mineralocorticoid, and sex steroid production that require hormone replacement therapy. Presentations vary from neonatal salt wasting and atypical genitalia, to adult presentation of hirsutism and irregular menses. Read More

    Mortality in Children Receiving Growth Hormone Treatment for Growth Disorders: Data from the GeNeSIS Observational Program.
    J Clin Endocrinol Metab 2017 May 26. Epub 2017 May 26.
    Indiana University School of Medicine (C.A.Q.), Indianapolis, Indiana; Lilly Research Laboratories (C.J.C.), Windlesham, Surrey, United Kingdom; Lilly Research Laboratories (A.G.Z.), Indianapolis, Indiana; Oregon Health Sciences University (R.G.R.), Portland, Oregon; St Jude Children's Research Hospital (L.L.R.), Memphis, Tennessee; University Children's Hospital (W.F.B.), Giessen, Germany.
    Context: Although pediatric GH treatment is generally considered safe for approved indications, there have been long-held concerns regarding potential for increased risk of neoplasia and, more recently, of stroke and mortality in adults treated with GH during childhood.

    Objective: To assess mortality in children receiving GH.

    Design: Prospective, multi-national, observational study. Read More

    Serum IGF-I levels are associated with improved white matter recovery after TBI.
    Ann Neurol 2017 Jun 2. Epub 2017 Jun 2.
    Computational, Cognitive and Clinical Neuroimaging Laboratory, Division of Brain Sciences, Imperial College London, Hammersmith Hospital, London, UK.
    Objective: Traumatic brain injury (TBI) is a common disabling condition with limited treatment options. Diffusion tensor imaging (DTI) measures recovery of axonal injury in white matter (WM) tracts after TBI. Growth hormone deficiency (GHD) after TBI may impair axonal and neuropsychological recovery, and serum IGF-I may mediate this effect. Read More

    Sex-Dependent, Osteoblast Stage-Specific Effects of Progesterone Receptor on Bone Acquisition.
    J Bone Miner Res 2017 Jun 1. Epub 2017 Jun 1.
    Center for Musculoskeletal Health, Department of Internal Medicine, University of California Davis Medical Center, Sacramento, CA, USA.
    The role of the progesterone receptor (PR) in the regulation of sexual dimorphism in bone has yet to be determined. Here we utilized genetic fate mapping and Western blotting to demonstrate age-dependent PR expression in the mouse femoral metaphysis and diaphysis. To define sex-dependent and osteoblast stage-specific effects of PR on bone acquisition, we selectively deleted PR at different stages of osteoblast differentiation. Read More

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