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Growth hormon in children

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https://www.readbyqxmd.com/read/29769040/novel-aggrecan-variant-p-gln2364pro-causes-severe-familial-nonsyndromic-adult-short-stature-and-poor-growth-hormone-response-in-chinese-children
#1
Dandan Xu, Chengjun Sun, Zeyi Zhou, Bingbing Wu, Lin Yang, Zhuo Chang, Miaoying Zhang, Li Xi, Ruoqian Cheng, Jinwen Ni, Feihong Luo
BACKGROUND: Mutations in the aggrecan (ACAN) gene can cause short stature (with heterogeneous clinical phenotypes), impaired bone maturation, and large variations in response to growth hormone (GH) treatment. For such cases, long-term longitudinal therapy data from China are still scarce. We report that a previously unknown ACAN gene variant reduces adult height and we analyze the GH response in children from an affected large Chinese family. METHODS: Two children initially diagnosed with idiopathic short stature (ISS) and a third mildly short child from a large Chinese family presented with poor GH response...
May 16, 2018: BMC Medical Genetics
https://www.readbyqxmd.com/read/29768519/influence-of-growth-hormone-replacement-on-neurological-and-psychomotor-development-case-report
#2
Felipe Motta, Adriana Pasmanik Eisencraft, Lindiane Gomes Crisostomo
The height response to the use of growth hormone in short height cases has already been confirmed in the literature. The influence of the insulin-like growth factor 1 (GH-IGF1) axis components on development, function, regeneration, neuroprotection, cognition, and motor functions has been evaluated in experimental studies and in adults with central nervous system lesions. However, there is still little research on the clinical impact of hormone replacement on neurological and psychomotor development. This report presents the case of a patient with excellent weight-height recovery and, even more surprisingly, neurological and psychomotor development in response to use of growth hormone...
May 14, 2018: Einstein
https://www.readbyqxmd.com/read/29767691/thyroid-hormones-and-female-reproduction
#3
Juneo F Silva, Natália M Ocarino, Rogéria Serakides
Thyroid hormones are vital for the proper functioning of the female reproductive system, since they modulate the metabolism and development of ovarian, uterine and placental tissues. Therefore, hypo- and hyperthyroidism may result in subfertility or infertility in both women and animals. Other well-documented sequelae of maternal thyroid dysfunctions include menstrual/estral irregularity, anovulation, abortion, preterm delivery, preeclampsia, intrauterine growth restriction, postpartum thyroiditis, and mental retardation in children...
May 14, 2018: Biology of Reproduction
https://www.readbyqxmd.com/read/29750651/predictors-of-bone-maturation-growth-rate-and-adult-height-in-children-with-central-precocious-puberty-treated-with-depot-leuprolide-acetate
#4
Karen O Klein, Sanja Dragnic, Ahmed M Soliman, Peter Bacher
BACKGROUND: Children with central precocious puberty (CPP) are treated with gonadotropin-releasing hormone agonists (GnRHa) to suppress puberty. Optimizing treatment outcomes continues to be studied. The relationships between growth, rate of bone maturation (bone age/chronological age [ΔBA/ΔCA]), luteinizing hormone (LH), predicted adult stature (PAS), as well as variables influencing these outcomes, were studied in children treated with depot leuprolide (LA Depot) Methods: Subjects (64 girls, seven boys) with CPP received LA Depot every 3 months for up to 42 months...
May 11, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29750649/cardiac-examination-in-children-with-laron-syndrome-undergoing-mecasermin-therapy
#5
Nurdan Erol, Metin Yıldız, Ayla Güven, Ayse Yıldırım
BACKGROUND: Laron syndrome (LS), which can be defined as primary growth hormone resistance or insensitivity, is a rare genetic disease inherited by an autosomal recessive trait. Although it is undistinguishable from growth hormone deficiency, LS has high levels of growth hormone, but insulin-like growth factor (IGF-1) cannot be synthesized. Mecasermin treatment is the only option for the patients who suffer from LS. This study aims to research cardiac findings of children with LS, who receive treatment with mecasermin...
May 11, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29746309/the-current-state-of-long-acting-growth-hormone-preparations-for-growth-hormone-therapy
#6
Kevin C J Yuen, Bradley S Miller, Beverly M K Biller
PURPOSE OF REVIEW: To discuss the rationale of developing long-acting growth hormone (LAGH) preparations, to describe the technologies designed to prolong GH action, and to address key issues regarding efficacy, safety, and monitoring while on treatment. REVIEW FINDINGS: Recombinant human GH is currently approved for daily use and has been shown to restore longitudinal growth, and improve body composition with relatively few side-effects in children and adults with GH deficiency, respectively...
May 4, 2018: Current Opinion in Endocrinology, Diabetes, and Obesity
https://www.readbyqxmd.com/read/29744522/-impact-of-thyroid-diseases-on-bone
#7
REVIEW
E Tsourdi, F Lademann, H Siggelkow
Thyroid hormones are key regulators of skeletal development in childhood and bone homeostasis in adulthood, and thyroid diseases have been associated with increased osteoporotic fractures. Hypothyroidism in children leads to an impaired skeletal maturation and mineralization, but an adequate and timely substitution with thyroid hormones stimulates bone growth. Conversely, hyperthyroidism at a young age accelerates skeletal development, but may also cause short stature because of a premature fusion of the growth plates...
May 9, 2018: Der Internist
https://www.readbyqxmd.com/read/29740400/the-role-of-kcnq1-mutations-and-maternal-beta-blocker-use-during-pregnancy-in-the-growth-of-children-with-long-qt-syndrome
#8
Heta Huttunen, Matti Hero, Mitja Lääperi, Johanna Känsäkoski, Heikki Swan, Joel A Hirsch, Päivi J Miettinen, Taneli Raivio
Objective: Two missense mutations in KCNQ1 , an imprinted gene that encodes the alpha subunit of the voltage-gated potassium channel Kv7.1, cause autosomal dominant growth hormone deficiency and maternally inherited gingival fibromatosis. We evaluated endocrine features, birth size, and subsequent somatic growth of patients with long QT syndrome 1 (LQT1) due to loss-of-function mutations in KCNQ1 . Design: Medical records of 104 patients with LQT1 in a single tertiary care center between 1995 and 2015 were retrospectively reviewed...
2018: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/29739044/diagnostic-usefulness-of-insulin-like-growth-factor-1-and-insulin-like-growth-factor-binding-protein-3-in-children-with-suspected-pituitary-dwarfism
#9
Beata Zelazowska-Rutkowska, Marta Trusiak, Artur Bossowski, Bogdan Cylwik
BACKGROUND: Pituitary dwarfism (also known as short stature) is a medical condition in which the pituitary gland does not produce enough growth hormone (GH). To confirm the diagnosis of growth hormone deficiency the overnight profile of GH secretion and GH provocative tests are usually performed; however, due to wide GH fluctuations throughout the day and night and the invasiveness of stimulation tests, their clinical utility is limited. Therefore, screening for IGF-1 (insulin-like growth factor 1) and IGFBP-3 (insulin-like growth factor binding protein type 3) is proposed, suggesting that these tests provide a more accurate reflection of the mean plasma GH level, although the results of these tests are still problematic...
May 1, 2018: Clinical Laboratory
https://www.readbyqxmd.com/read/29737268/evaluation-and-management-of-the-child-with-hypothyroidism
#10
Alexander A C Leung, Alexander K C Leung
BACKGROUND: Thyroid hormones are critical for early neurocognitive development as well as growth and development throughout childhood. Prompt recognition and treatment of hypothyroidism is therefore of utmost importance to optimize physical and neurodevelopmental outcomes. OBJECTIVE: To review in depth the evaluation, diagnosis, and treatment of hypothyroidism in children. METHODS: A PubMed search was completed in Clinical Queries using the key term "hypothyroidism"...
May 8, 2018: Recent Patents on Endocrine, Metabolic & Immune Drug Discovery
https://www.readbyqxmd.com/read/29736148/the-pre-treatment-characteristics-and-evaluation-of-the-effects-of-recombinant-human-growth-hormone-therapy-in-children-with-growth-hormone-deficiency-and-celiac-disease-or-inflammatory-bowel-disease
#11
Ewelina Witkowska-Sędek, Dominika Labochka, Anna Majcher, Beata Pyrżak
The aim of the study: was to investigate the coincidence of growth hormone deficiency (GHD) and celiac disease (CD) or inflammatory bowel disease (IBD) in patients referred for short stature, and to evaluate the baseline anthropometric parameters and the effectiveness of recombinant human growth hormone (rhGH) therapy in the first year in those patients (GHD+CD/IBD subgroup) in comparison to patients with GHD without CD or IBD (GHD-CD/IBD subgroup). Material and methods: The study was retrospective and included 2196 short patients (height SDS [Standard Deviation Score] ≤ -1...
2018: Central-European Journal of Immunology
https://www.readbyqxmd.com/read/29724795/the-growth-hormone-insulin-like-growth-factor-i-axis-in-the-diagnosis-and-treatment-of-growth-disorders
#12
Werner Blum, Abdullah Alherbish, Afaf Alsagheir, Ahmed El Awwa, Walid Kaplan, Ekaterina Koledova, Martin O Savage
The growth hormone (GH)-insulin-like growth factor (IGF)-I axis is a key endocrine mechanism regulating linear growth in children. While paediatricians have a good knowledge of GH secretion and assessment, understanding and use of measurements of the components of the IGF system are less current in clinical practice. The physiological function of this axis is to increase the anabolic cellular processes of protein synthesis and mitosis, and reduction of apoptosis, with each being regulated in the appropriate target tissue...
May 3, 2018: Endocrine Connections
https://www.readbyqxmd.com/read/29704908/latent-trajectories-of-internalizing-symptoms-from-preschool-to-school-age-a-multi-informant-study-in-a-high-risk-sample
#13
Annette M Klein, Andrea Schlesier-Michel, Yvonne Otto, Lars O White, Anna Andreas, Susan Sierau, Sarah Bergmann, Sonja Perren, Kai von Klitzing
Recent proposals suggest early adversity sets in motion particularly chronic and neurobiologically distinct trajectories of internalizing symptoms. However, few prospective studies in high-risk samples delineate distinct trajectories of internalizing symptoms from preschool age onward. We examined trajectories in a high-risk cohort, oversampled for internalizing symptoms, several preschool risk/maintenance factors, and school-age outcomes. Parents of 325 children completed the Strengths and Difficulties Questionnaire on up to four waves of data collection from preschool (3-5 years) to school age (8-9 years) and Preschool Age Psychiatric Assessment interviews at both ages...
April 29, 2018: Development and Psychopathology
https://www.readbyqxmd.com/read/29702359/combined-simultaneous-arginine-clonidine-stimulation-test-timing-of-peak-growth-hormone-gh-concentration-and-correlation-with-clinical-indices-of-gh-status
#14
Nandini Bhat, Eric Dulmovits, Andrew Lane, Catherine Messina, Thomas Wilson
BACKGROUND: The aims of this study were to determine if it is possible to truncate a combined simultaneous arginine clonidine stimulation test, and to correlate the outcome of the test with clinical indices of GH status. METHODS: Charts of subjects who underwent a combined simultaneous arginine clonidine stimulation test between January 1, 2007 and August 31, 2016 were reviewed. RESULTS: 131/203 (64.5%) tests performed in children with growth failure demonstrated a peak GH ≥ 10 ng/ml...
April 7, 2018: Growth Hormone & IGF Research
https://www.readbyqxmd.com/read/29688887/hematuria-as-an-adverse-outcome-following-provocative-growth-hormone-stimulation-testing-in-children
#15
Rajeev Thirunagari, Alexandra Marrone, Hannah Elsinghorst, Lucy D Mastrandrea
BACKGROUND: Provocative growth hormone (GH) stimulation testing is used to evaluate short stature and growth failure in children. Agents commonly used for testing include clonidine, arginine and glucagon. While stimulation testing is generally considered safe, gross hematuria has been described as a rare idiopathic complication of GH stimulation testing. This study was designed to estimate the incidence of both microscopic and macroscopic hematuria following GH testing with different provocative agents...
April 24, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29687007/association-between-growth-hormone-insulin-like-growth-factor-1-axis-gene-polymorphisms-and-short-stature-in-chinese-children
#16
Yanhong Zhang, Mei Zhang, Yuntian Chu, Baolan Ji, Qian Shao, Bo Ban
Objective: This study was designed to analyze the association between the growth hormone-insulin-like growth factor-1 (GH-IGF-1) axis gene polymorphisms and short stature in Chinese children. Methods: 181 growth hormone deficiency (GHD) patients and 206 normal stature controls were enrolled to attend this study. Five single-nucleotide polymorphisms in the GH receptor (GHR) and 5 SNPs within the GH-signaling pathway were genotyped by matrix-assisted laser desorption/ionization time of flight mass spectrometry...
2018: BioMed Research International
https://www.readbyqxmd.com/read/29680781/adherence-to-somatotropin-treatment-administered-with-an-electronic-device
#17
María Auxiliadora Arrabal Vela, Carlota Prado García Gijón, María Pascual Martin, Isabel Benet Giménez, Vera Áreas Del Águila, José Ramón Muñoz-Rodríguez, Enrique Palomo Atance
INTRODUCTION: Adherence to somatotropin treatment is associated with increased growth velocity and improved adult height. The purpose of this study is to determine the adherence of patients undergoing treatment with an electronic device and its relationship with different variables (age, gender, duration of treatment, diagnosis, height, and growth rate). MATERIAL AND METHODS: Descriptive, longitudinal and retrospective study of children less than 14 years of age undergoing treatment with somatotropin administered with the Easypod® electronic device in the Paediatric Endocrinology Outpatient Clinic of the General University Hospital of Ciudad Real, Spain...
April 18, 2018: Endocrinología, Diabetes y Nutrición
https://www.readbyqxmd.com/read/29675261/growth-trajectory-and-pubertal-tempo-from-birth-till-final-height-in-a-girl-with-wolf-hirschhorn-syndrome
#18
Jia Xuan Siew, Fabian Yap
Growth anomaly is a prominent feature in Wolf-Hirschhorn syndrome (WHS), a rare congenital disorder caused by variable deletion of chromosome 4p. While growth charts have been developed for WHS patients 0-4 years of age and growth data available for Japanese WHS patients 0-17 years, information on pubertal growth and final height among WHS children remain lacking. Growth hormone (GH) therapy has been reported in two GH-sufficient children with WHS, allowing for pre-puberty catch up growth; however, pubertal growth and final height information was also unavailable...
2018: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/29671202/pharmacokinetics-and-pharmacodynamics-of-once-weekly-somapacitan-in-children-and-adults-supporting-dosing-rationales-with-a-model-based-analysis-of-three-phase-i-trials
#19
Rasmus Vestergaard Juul, Michael Højby Rasmussen, Henrik Agersø, Rune Viig Overgaard
BACKGROUND: Somapacitan, a long-acting growth hormone (GH) derivative, has been well-tolerated in children with GH deficiency (GHD) and adults (healthy and adult GHD), in phase I, single- and multiple-dose trials, respectively, and has pharmacokinetic and pharmacodynamic properties supporting a once-weekly dosing regimen. OBJECTIVE: In the absence of a multiple-dose phase I trial in children with GHD, the aim was to develop a pharmacokinetic/pharmacodynamic model to predict somapacitan exposure and insulin-like growth factor-I (IGF-I) response after once-weekly multiple doses in both children and adults with GHD...
April 18, 2018: Clinical Pharmacokinetics
https://www.readbyqxmd.com/read/29669803/growth-hormone-in-combination-with-leuprorelin-in-pubertal-children-with-idiopathic-short-stature
#20
Imane Benabbad, Myriam Rosilio, Maithe Tauber, Emmanuel Paris, Anne Fjellestad-Paulsen, Lovisa Berggren, Hiren Patel, Jean-Claude Carel
OBJECTIVE: There is a scarcity of data from randomised controlled trials on the association of growth hormone (GH) with gonadotrophin-releasing hormone (GnRH) agonists in idiopathic short stature (ISS), although this off-label use is common. We aimed to test whether delaying pubertal progression could increase near-adult height (NAH) in GH-treated patients with ISS. METHODS: Patients with ISS at puberty onset were randomised to GH with leuprorelin (combination, n=46) or GH alone (n=45)...
April 18, 2018: Endocrine Connections
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