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"Growth Hormone Deficiency"

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https://www.readbyqxmd.com/read/27884013/guidelines-for-growth-hormone-and-insulin-like-growth-factor-i-treatment-in-children-and-adolescents-growth-hormone-deficiency-idiopathic-short-stature-and-primary-insulin-like-growth-factor-i-deficiency
#1
Adda Grimberg, Sara A DiVall, Constantin Polychronakos, David B Allen, Laurie E Cohen, Jose Bernardo Quintos, Wilma C Rossi, Chris Feudtner, Mohammad Hassan Murad
BACKGROUND/AIMS: On behalf of the Drug and Therapeutics, and Ethics Committees of the Pediatric Endocrine Society, we sought to update the guidelines published in 2003 on the use of growth hormone (GH). Because idiopathic short stature (ISS) remains a controversial indication, and diagnostic challenges often blur the distinction between ISS, GH deficiency (GHD), and primary IGF-I deficiency (PIGFD), we focused on these three diagnoses, thereby adding recombinant IGF-I therapy to the GH guidelines for the first time...
November 25, 2016: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/27881164/administration-of-anti-receptor-activator-of-nuclear-factor-kappa-b-ligand-rankl-antibody-for-the-treatment-of-osteoporosis-was-associated-with-amelioration-of-hepatitis-in-a-female-patient-with-growth-hormone-deficiency-a-case-report
#2
Ayumu Takeno, Masahiro Yamamoto, Masakazu Notsu, Toshitsugu Sugimoto
BACKGROUND: Growth hormone deficiency (GHD) is associated with non-alcoholic fatty liver disease (NAFLD). A recent animal study showed that hepatocyte-specific receptor activator of nuclear factor-kappa B (RANK) knockout mice had significantly lower liver fat content compared with control mice concomitant with a decrease in production of inflammatory cytokines such as tumor necrosis factor-α (TNF-α) from hepatocytes and kupffer cells. The role of anti-RANK ligand (RANKL) antibody for osteoporosis on hepatitis in patients with aGHD is still unknown...
November 24, 2016: BMC Endocrine Disorders
https://www.readbyqxmd.com/read/27878771/growth-hormone-deficiency-and-hypopituitarism-in-adults-after-complicated-mild-traumatic-brain-injury
#3
Stefania Giuliano, Serafina Talarico, Lucia Bruno, Francesco Beniamino Nicoletti, Claudio Ceccotti, Antonino Belfiore
PURPOSE: Traumatic brain injury is considered the main cause of hypopituitarism in adults, and GH deficiency appears to be the most frequent pituitary deficit. Most of the available studies have included all degrees of severity of trauma. We aimed to assess pituitary function and GH deficiency in adult patients at different time lengths after complicated mild TBI according to Glasgow Coma Scale. We also aimed to evaluate whether mild TBI patients with GH deficiency had developed alterations in the glycolipid profile...
November 23, 2016: Endocrine
https://www.readbyqxmd.com/read/27867878/prevalence-of-hypothalamo-pituitary-dysfunction-in-patients-of-traumatic-brain-injury
#4
K V S Hari Kumar, M N Swamy, M A Khan
BACKGROUND: Traumatic brain injury (TBI) is common in young soldiers of armed forces leading to significant morbidity and mortality. We studied the prevalence of hypopituitarism following TBI and its association with trauma severity. MATERIALS AND METHODS: We conducted a 12-month prospective study of 56 TBI patients for the presence of hormonal dysfunction. Hormonal parameters were estimated during the early phase (0-10 days posttraumatically) and after 6 and 12 months...
November 2016: Indian Journal of Endocrinology and Metabolism
https://www.readbyqxmd.com/read/27867396/challenges-in-the-diagnosis-and-management-of-growth-hormone-deficiency-in-india
#5
REVIEW
Mathew John, Ekaterina Koledova, Kanakatte Mylariah Prasanna Kumar, Harshal Chaudhari
In clinical practice, every year approximately 150,000 children are referred with short stature (SS) based on a cut-off of fifth percentile. The most important endocrine and treatable cause of SS is growth hormone deficiency (GHD). The lack of reliable data on the prevalence of GHD in India limits estimation of the magnitude of this problem. The diagnosis and treatment of GHD are hurdled with various challenges, restricting the availability of growth hormone (GH) therapy to only a very limited segment of the children in India...
2016: International Journal of Endocrinology
https://www.readbyqxmd.com/read/27857044/the-impact-of-the-d3-growth-hormone-receptor-d3-ghr-polymorphism-on-the-therapeutic-effect-of-growth-hormone-replacement-in-children-with-idiopathic-growth-hormone-deficiency-in-poland
#6
(no author information available yet)
OBJECTIVES: The human growth hormone receptor (GHR) exon 3 deletion (d3) polymorphism has been reported to be associated with the responsiveness to growth hormone (GH) therapy. This study aimed to: (a) assess the frequency of this polymorphism in a group of Polish children with idiopathic growth hormone deficiency (IGHD) and (b) analyze their response to GH therapy. METHODS: The study group consisted of 67 prepubertal children with IGHD. The control group was composed of 150 Caucasian newborns from whom umbilical cord blood samples were drawn...
September 18, 2016: Neuro Endocrinology Letters
https://www.readbyqxmd.com/read/27835910/growth-hormone-replacement-therapy-reduces-risk-of-cancer-in-adult-with-growth-hormone-deficiency-a-meta-analysis
#7
Zhanzhan Li, Qin Zhou, Yanyan Li, Jun Fu, Xinqiong Huang, Liangfang Shen
The risk of growth hormone on cancer in adult with growth hormone deficiency remains unclear. We carried out a meta-analysis to evaluate the risk of cancer in adult with and without growth hormone replacement therapy. We searched PubMed, Web of Science, China National Knowledge Infrastructure, and WanFang databases up to 31 July 2016 for eligible studies. Pooled risk ratio (RR) with 95% confidence interval (CI) was calculated using fixed-or random-effects models if appropriate. The Newcastle-Ottawa Scale was used to assess the study quality...
November 9, 2016: Oncotarget
https://www.readbyqxmd.com/read/27820671/contribution-of-lhx4-mutations-to-pituitary-deficits-in-a-cohort-of-417-unrelated-patients
#8
Enzo Cohen, Mohamad Maghnie, Nathalie Collot, Juliane Leger, Florence Dastot, Michel Polak, Sophie Rose, Philippe Touraine, Philippe Duquesnoy, Maïté Tauber, Bruno Copin, Anne-Marie Bertrand, Frederic Brioude, Daniela Larizza, Thomas Edouard, Laura González Briceño, Irène Netchine, Isabelle Oliver-Petit, Marie-Laure Sobrier, Serge Amselem, Marie Legendre
CONTEXT: LHX4 encodes a LIM-homeodomain transcription factor implicated in early pituitary development. In humans, only 13 heterozygous LHX4 mutations have been associated with congenital hypopituitarism. OBJECTIVE: To evaluate the prevalence of LHX4 mutations in patients with hypopituitarism, and to precise the associated phenotypes. To characterize the functional impact of the identified variants and the respective role of the two LIM domains of LHX4. PATIENTS AND DESIGN: We screened 417 unrelated patients with isolated growth hormone deficiency or combined pituitary hormone deficiency associated with ectopic posterior pituitary and/or sella turcica anomalies for LHX4 mutations (Sanger sequencing)...
November 7, 2016: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/27812793/uk-iatrogenic-creutzfeldt-jakob-disease-investigating-human-prion-transmission-across-genotypic-barriers-using-human-tissue-based-and-molecular-approaches
#9
Diane L Ritchie, Marcelo A Barria, Alexander H Peden, Helen M Yull, James Kirkpatrick, Peter Adlard, James W Ironside, Mark W Head
Creutzfeldt-Jakob disease (CJD) is the prototypic human prion disease that occurs most commonly in sporadic and genetic forms, but it is also transmissible and can be acquired through medical procedures, resulting in iatrogenic CJD (iCJD). The largest numbers of iCJD cases that have occurred worldwide have resulted from contaminated cadaveric pituitary-derived human growth hormone (hGH) and its use to treat primary and secondary growth hormone deficiency. We report a comprehensive, tissue-based and molecular genetic analysis of the largest series of UK hGH-iCJD cases reported to date, including in vitro kinetic molecular modelling of genotypic factors influencing prion transmission...
November 3, 2016: Acta Neuropathologica
https://www.readbyqxmd.com/read/27809913/long-term-safety-and-efficacy-of-omnitrope%C3%A2-a-somatropin-biosimilar-in-children-requiring-growth-hormone-treatment-italian-interim-analysis-of-the-patro-children-study
#10
Lorenzo Iughetti, Gianluca Tornese, Maria Elisabeth Street, Flavia Napoli, Claudia Giavoli, Franco Antoniazzi, Stefano Stagi, Caterina Luongo, Sara Azzolini, Letizia Ragusa, Gianni Bona, Clara Zecchino, Tommaso Aversa, Luca Persani, Laura Guazzarotti, Emiliano Zecchi, Alberto Pietropoli, Stefano Zucchini
BACKGROUND: PATRO Children is an ongoing observational, longitudinal, non-interventional, global post-marketing surveillance study, which is investigating the long-term safety and effectiveness of Omnitrope®, a somatropin biosimilar to Genotropin®, in children with growth disturbances. The primary endpoint of PATRO Children is long-term safety and the secondary endpoint is effectiveness, which is assessed by analysing auxological data such as height (HSDS) and height velocity (HVSDS) standard deviation scores...
November 3, 2016: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/27809640/loss-of-functional-osteoprotegerin-more-than-a-skeletal-problem
#11
Corinna Grasemannn, Nicole Unger, Matthias Hövel, Diana Arweiler-Harbeck, Ralf Herrmann, Michael M Schündeln, Oliver Müller, Bernd Schweiger, Ekkehart Lausch, Thomas Meissner, Cordula Kiewert, Berthold P Hauffa, Nick J Shaw
INTRODUCTION: Juvenile Pagets disease (JPD), an ultra-rare, debilitating bone disease stemming from unopposed RANKL action due to loss of functional osteoprotegerin (OPG) is caused by recessive mutations in TNFRSF11B. A genotype-phenotype correlation spanning from mild to very severe forms is described. AIM: To describe the complexity of the human phenotype of OPG deficiency in more detail and to investigate heterozygous mutation carriers for clinical signs of JPD...
November 3, 2016: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/27803155/bridging-the-gap-metabolic-and-endocrine-care-of-patients-during-transition
#12
REVIEW
Anita Hokken-Koelega, Aart-Jan van der Lely, Berthold Hauffa, Gabriele Häusler, Gudmundur Johannsson, Mohamad Maghnie, Jesús Argente, Jean DeSchepper, Helena Gleeson, John W Gregory, Charlotte Höybye, Fahrettin Keleştimur, Anton Luger, Hermann L Müller, Sebastian Neggers, Vera Popovic-Brkic, Eleonora Porcu, Lars Sävendahl, Stephen Shalet, Bessie Spiliotis, Maithé Tauber
OBJECTIVE: Seamless transition of endocrine patients from the paediatric to adult setting is still suboptimal, especially in patients with complex disorders, i.e., small for gestational age, Turner or Prader-Willi syndromes; Childhood Cancer Survivors, and those with childhood-onset growth hormone deficiency. METHODS: An expert panel meeting comprised of European paediatric and adult endocrinologists was convened to explore the current gaps in managing the healthcare of patients with endocrine diseases during transition from paediatric to adult care settings...
November 2016: Endocrine Connections
https://www.readbyqxmd.com/read/27803031/seven-years-of-growth-hormone-gh-replacement-improves-quality-of-life-in-hypopituitary-patients-with-adult-onset-gh-deficiency
#13
Mariam Elbornsson, Alexandra Horvath, Galina Götherström, Bengt-Åke Bengtsson, Gudmundur Johannsson, Johan Svensson
OBJECTIVE: Few studies have determined the effects of long-term growth hormone (GH) replacement on quality of life (QoL). This study investigated the effects of 7 years of GH replacement on QoL. DESIGN: A prospective, single-center, open-label study of 95 adults (mean age 52.8 years; 46 men) with adult onset GH deficiency (GHD). METHODS: QoL was measured using Quality of Life-Assessment for Growth Hormone Deficiency in Adults (QoL-AGHDA) and Psychological General Well-Being (PGWB) scores...
November 1, 2016: European Journal of Endocrinology
https://www.readbyqxmd.com/read/27789461/hypopituitarism-in-children-with-cerebral-palsy
#14
REVIEW
Suma Uday, Nick Shaw, Ruth Krone, Jeremy Kirk
Poor growth and delayed puberty in children with cerebral palsy is frequently felt to be related to malnutrition. Although growth hormone deficiency is commonly described in these children, multiple pituitary hormone deficiency (MPHD) has not been previously reported. We present a series of four children with cerebral palsy who were born before 29 weeks gestation who were referred to the regional endocrinology service, three for delayed puberty and one for short stature, in whom investigations identified MPHD...
October 27, 2016: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/27789066/novel-aicda-mutation-in-a-case-of-autosomal-recessive-hyper-igm-syndrome-growth-hormone-deficiency-and-autoimmunity
#15
A Fazel, S Kashef, S Aleyasin, S Harsini, Z Karamizadeh, S Zoghi, S K Flores, K Boztug, N Rezaei
BACKGROUND: The Hyper-immunoglobulin M syndromes (HIGM) are a heterogeneous group of genetic disorders, which have been rarely reported to be associated with growth hormone deficiency (GHD). METHODS AND RESULTS: A nine-year-old girl with recurrent urinary tract infections, diarrhoea, sinopulmonary infections, and failure to thrive since the age of six months had normal CD3+, CD4+, CD8+T lymphocytes, and CD19+B lymphocytes and natural killer (NK) cells, but extremely elevated IgM and significantly decreased IgG and IgA...
October 24, 2016: Allergologia et Immunopathologia
https://www.readbyqxmd.com/read/27775790/effect-of-single-nucleotide-polymorphisms-on-cdk4-and-zn-supplementation-in-children-with-growth-hormone-deficiency
#16
Y Liu, Z-X Zhang
OBJECTIVE: Precision medicine is a personalized disease prevention and treatment program combining modern genetic technology, molecular imaging techniques, and biological information with patients' living environment and clinical data, for accurate classification and diagnosis of diseases. CASE REPORT: Our study presents the case of a 7-year-old female patient with clinical manifestations of growth hormone (GH) deficiency. After treatment with recombinant human GH for 2 years, the patient showed a reduced growth rate...
October 2016: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/27769307/effects-of-financial-support-on-treatment-of-adolescents-with-growth-hormone-deficiency-a-retrospective-study-in-japan
#17
Eri Maeda, Takahiro Higashi, Tomonobu Hasegawa, Susumu Yokoya, Takahiro Mochizuki, Tomohiro Ishii, Junko Ito, Susumu Kanzaki, Akira Shimatsu, Koji Takano, Toshihiro Tajima, Hiroyuki Tanaka, Yusuke Tanahashi, Akira Teramoto, Toshiro Nagai, Kunihiko Hanew, Reiko Horikawa, Toru Yorifuji, Naohiro Wada, Toshiaki Tanaka
BACKGROUND: Treatment costs for children with growth hormone (GH) deficiency are subsidized by the government in Japan if the children meet clinical criteria, including height limits (boys: 156.4 cm; girls: 145.4 cm). However, several funding programs, such as a subsidy provided by local governments, can be used by those who exceed the height limits. In this study, we explored the impacts of financial support on GH treatment using this natural allocation. METHODS: A retrospective analysis of 696 adolescent patients (451 boys and 245 girls) who reached the height limits was conducted...
October 21, 2016: BMC Health Services Research
https://www.readbyqxmd.com/read/27769269/explaining-parent-child-dis-agreement-in-generic-and-short-stature-specific-health-related-quality-of-life-reports-do-family-and-social-relationships-matter
#18
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
https://www.readbyqxmd.com/read/27757412/successful-combined-treatment-for-atrophic-thyroiditis-with-growth-hormone-deficiency
#19
Taketo Otsuka, Naoya Tajima, Keisuke Nagasaki, Minoru Okazaki
No abstract text is available yet for this article.
2016: Global Pediatric Health
https://www.readbyqxmd.com/read/27756091/identification-of-novel-prop1-and-pou1f1-mutations-in-patients-with-combined-pituitary-hormone-deficiency
#20
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
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