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"Growth Hormone Deficiency"

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https://www.readbyqxmd.com/read/29765966/further-clinical-evidence-for-the-effect-of-igf-1-on-hair-growth-and-alopecia
#1
Ralph M Trüeb
Observations on the Laron syndrome originally offered the opportunity to explore the effect of insulin-like growth factor 1 (IGF-1) deficiency on human hair growth and differentiation. According to its expression in the dermal hair papilla, IGF-1 is likely involved in reciprocal signaling. It has been shown to affect follicular proliferation, tissue remodeling, and the hair growth cycle, as well as follicular differentiation, identifying IGF-1 signaling as an important mitogenic and morphogenetic regulator in hair follicle biology...
April 2018: Skin Appendage Disorders
https://www.readbyqxmd.com/read/29750649/cardiac-examination-in-children-with-laron-syndrome-undergoing-mecasermin-therapy
#2
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/29748174/pituitary-dysfunction-and-association-with-fatigue-in-stroke-and-other-acute-brain-injury
#3
Heleen A Booij, Wietse D C Gaykema, Kamiel A J Kuijpers, Marie-José M Pouwels, Heleen M den Hertog
BACKGROUND: Poststroke fatigue (PSF) is a highly prevalent and debilitating condition. However, the etiology remains incompletely understood. Literature suggests the co-prevalence of pituitary dysfunction (PD) with stroke, and the question raises whether this could be a contributing factor to the development of PSF. This study reviews the prevalence of PD after stroke and other acquired brain injuries and its association with fatigue. SUMMARY: We performed a bibliographic literature search of MEDLINE and Embase databases for English language studies on PD in adult patients with stroke, traumatic brain injury (TBI) or aneurysmatic subarachnoid hemorrhage (aSAH)...
May 10, 2018: Endocrine Connections
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
#4
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
#5
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/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
#6
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/29703730/screening-for-germline-kcnq1-and-kcne2-mutations-in-a-set-of-somatotropinoma-patients
#7
Anna-Pauliina Iivonen, Johanna Känsäkoski, Atte Karppinen, Leena Kivipelto, Camilla Schalin-Jäntti, Auli Karhu, Taneli Raivio
OBJECTIVE: Recently, mutations in KCNQ1 , a potassium channel gene usually linked to long QT syndrome, were reported to cause maternally inherited gingival fibromatosis and growth hormone deficiency (GHD). Expression of the mutated KCNQ1 with the auxiliary potassium channel subunit KCNE2 was shown to reduce pituitary hormone secretion in functional experiments. Here, we investigated if germline mutations in KCNQ1 and KCNE2 were present in patients with somatotropinomas, which represent a model of growth hormone excess...
May 2018: Endocrine Connections
https://www.readbyqxmd.com/read/29699852/relative-bioavailability-of-a-single-4-mg-dose-of-somatropin-administered-by-subcutaneous-injection-or-by-needle-free-device-and-coadministered-with-the-growth-hormone-inhibitor-octreotide-acetate-in-healthy-adult-subjects
#8
Darin B Brimhall, Niclas Petri, Pina D'Angelo
PURPOSE: Somatropin, used to treat growth hormone deficiency, has been traditionally administered by subcutaneous (SC) injection with needle and syringe. Needle-free devices offer ease of administration and may improve adherence and outcomes. This study evaluated the relative bioavailability of somatropin delivered with a needle-free device compared with traditional SC injection. METHODS: In this randomized, single-dose, crossover study, healthy adults aged 18 to 35 years received single 4-mg doses of somatropin via a needle-free device or SC injection, along with octreotide to suppress endogenous growth hormone production...
April 23, 2018: Clinical Therapeutics
https://www.readbyqxmd.com/read/29688888/body-composition-and-metabolic-health-of-young-male-adults-with-childhood-onset-multiple-pituitary-hormone-deficiency-after-cessation-of-growth-hormone-treatment
#9
Hongbo Yang, Linjie Wang, Xiaonan Qiu, Kemin Yan, Fengying Gong, Huijuan Zhu, Hui Pan
BACKGROUND: Recombinant human growth hormone (rhGH) replacement therapy is usually stopped after linear growth completion in patients with growth hormone deficiency. In patients with multiple pituitary hormone deficiency (MPHD), the long-term effects of discontinuation of rhGH replacement are unknown. METHODS: In this study, the anthropometric and metabolic parameters of 24 male patients with adult growth hormone deficiency (AGHD) due to MPHD in childhood after cessation of rhGH therapy for a mean of 7...
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
#10
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
#11
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/29658834/alterations-in-thyroid-hormone-levels-following-growth-hormone-replacement-exert-complex-biological-effects
#12
Nigel Glynn, Helena Kenny, Tarik Salim, David J Halsall, Diarmuid Smith, Tommy Kyaw Tun, John H McDermott, William Tormey, Christopher J Thompson, Brendan McAdam, Donal O' Gorman, Amar Agha
OBJECTIVE: Alterations in the thyroid axis are frequently observed following growth hormone (GH) replacement, but uncertainty exists regarding their clinical significance. We aimed to compare fluctuations in circulating thyroid hormone levels, induced by GH, to changes in sensitive biological markers of thyroid hormone action. METHODS: This was a prospective observational clinical study. Twenty hypopituitary men were studied before and after GH replacement. Serum thyroid-stimulating hormone (TSH), thyroid hormones, and insulin-like growth factor 1 were measured...
April 2018: Endocrine Practice
https://www.readbyqxmd.com/read/29656861/efficacy-of-growth-hormone-treatment-in-children-with-type-1-diabetes-mellitus-and-growth-hormone-deficiency-an-analysis-of-kigs-data
#13
Walter Bonfig, Anders Lindberg, Martin Carlsson, Wayne Cutfield, David Dunger, Cecilia Camacho-Hübner, Reinhard W Holl
OBJECTIVE: To analyze first-year treatment growth response and growth hormone (GH) dosage in prepubertal patients with the combination of type 1 diabetes mellitus (T1DM) and growth hormone deficiency (GHD). STUDY DESIGN: A total of 69 patients with T1DM and GHD treated with GH have been enrolled in KIGS (Pfizer International Growth Database). Of these, 24 prepubertal patients had developed T1DM before GHD and were included in this analysis. Of 30 570 patients with GHD without T1DM, 15 024 were prepubertal and served as controls...
April 12, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29649340/the-relationship-between-alkaline-phosphatase-and-bone-alkaline-phosphatase-activity-and-the-growth-hormone-insulin-like-growth-factor-1-axis-and-vitamin-d-status-in-children-with-growth-hormone-deficiency
#14
Ewelina Witkowska-Sędek, Anna Stelmaszczyk-Emmel, Anna Majcher, Urszula Demkow, Beata Pyrżak
The relationships between bone turnover, the growth hormone/insulin-like growth factor-1 (GH/IGF-1) axis and vitamin D are complex, but still not fully explained. The GH/IGF-1 axis and vitamin D can mutually modulate each other's metabolism and influence the activation of cell proliferation, maturation, and mineralization as well as bone resorption. The aim of this study was to evaluate the reciprocal associations between bone formation markers [alkaline phosphatase (ALP), bone alkaline phosphatase (BALP)], the GH/IGF-1 axis and 25-hydroxyvitamin D [25(OH)D] in children with growth hormone deficiency at baseline and during recombinant human growth hormone (rhGH) therapy...
April 13, 2018: Acta Biochimica Polonica
https://www.readbyqxmd.com/read/29618660/transcriptomics-and-machine-learning-predict-diagnosis-and-severity-of-growth-hormone-deficiency
#15
Philip G Murray, Adam Stevens, Chiara De Leonibus, Ekaterina Koledova, Pierre Chatelain, Peter E Clayton
BACKGROUND: The effect of gene expression data on diagnosis remains limited. Here, we show how diagnosis and classification of growth hormone deficiency (GHD) can be achieved from a single blood sample using a combination of transcriptomics and random forest analysis. METHODS: Prepubertal treatment-naive children with GHD (n = 98) were enrolled from the PREDICT study, and controls (n = 26) were acquired from online data sets. Whole blood gene expression was correlated with peak growth hormone (GH) using rank regression and a random forest algorithm tested for prediction of the presence of GHD and in classification of GHD as severe (peak GH <4 μg/l) and nonsevere (peak ≥4 μg/l)...
April 5, 2018: JCI Insight
https://www.readbyqxmd.com/read/29615058/effects-of-recombinant-human-growth-hormone-administration-on-cardiovascular-risk-factors-in-obese-children-with-relative-growth-hormone-deficiency
#16
Shuang Liang, Jiang Xue, Guimei Li
BACKGROUND: Based on the sample of obese children with relative growth hormone deficiency (GHD), the objective of our study was to determine the effects of rhGH treatment on cardiovascular risk factors, including body mass index (BMI), lipid levels and glucose metabolism index. METHODS: A total of 43 obese children with relative GHD were included in our final analysis. The obese subjects were divided into two groups: recombinant human growth hormone (rhGH) treatment group and untreated control group...
April 3, 2018: Lipids in Health and Disease
https://www.readbyqxmd.com/read/29609574/utility-of-serum-igf-1-for-diagnosis-of-growth-hormone-deficiency-following-traumatic-brain-injury-and-sport-related-concussion
#17
Kirstie Lithgow, Alex Chin, Chantel T Debert, Gregory A Kline
BACKGROUND: Growth hormone deficiency (GHD) is a potential consequence of traumatic brain injury (TBI), including sport-related concussion (SRC). GH stimulation testing is required for definitive diagnosis; however, this is resource intensive and can be associated with adverse symptoms or risks. Measurement of serum IGF-1 is more practical and accessible, and pituitary tumour patients with hypopituitarism and low serum IGF-1 have been shown to have a high probability of GHD. We aimed to evaluate IGF-1 measurement for diagnosing GHD in our local TBI population...
April 2, 2018: BMC Endocrine Disorders
https://www.readbyqxmd.com/read/29609449/design-of-the-long-term-observational-cohort-study-with-recombinant-human-growth-hormone-in-korean-children-lg-growth-study
#18
Sochung Chung, Jae-Ho Yoo, Jin Ho Choi, Young-Jun Rhie, Hyun-Wook Chae, Jae Hyun Kim, Il Tae Hwang, Choong Ho Shin, Eun Young Kim, Kee-Hyoung Lee
PURPOSE: Regarding recombinant human growth hormone (rhGH) use in the pediatric population, no long-term follow-up data are available for Korean patients. To fill in the gap of knowledge, a registry study (LG Growth Study) was initiated to assess the safety and effectiveness of four types of rhGH products in real-life settings. METHODS: A total of 4,000 children will be registered and prospectively followed up at 6-month intervals until 2 years after epiphyseal closure to collect data on treatment and adverse events, with primary interest in malignancies and growth outcomes...
March 2018: Annals of Pediatric Endocrinology & Metabolism
https://www.readbyqxmd.com/read/29605215/direct-and-indirect-effects-of-growth-hormone-deficiency-ghd-on-lung-function-in-children-a-mediation-analysis
#19
Giovanna Cilluffo, Giuliana Ferrante, Salvatore Fasola, Alessandro Ciresi, Irene Cardillo, Giancarlo Tancredi, Giovanni Viegi, Carla Giordano, Nicola Scichilone, Stefania La Grutta
BACKGROUND: Studies on pulmonary function tests (PFTs) in Growth Hormone Deficiency (GHD) children are lacking. The aims of this study were: (i) to investigate PFTs in GHD pre-pubertal children with respect to Controls, before starting Growth Hormone Therapy (GHT) (T0); (ii) to evaluate changes of PFTs in GHD vs Controls, after 1-year GHT (T1). For both aims the mediation analysis (MA) was applied to evaluate the extent to which the relationship between GHD and PFTs could be ascribed to a height-mediated (indirect) or a GH direct effect...
April 2018: Respiratory Medicine
https://www.readbyqxmd.com/read/29599419/chitayat-hall-and-schaaf-yang-syndromes-a-common-aetiology-expanding-the-phenotype-of-magel2-related-disorders
#20
Rebekah Jobling, Dimitri James Stavropoulos, Christian R Marshall, Cheryl Cytrynbaum, Michelle M Axford, Vanessa Londero, Sharon Moalem, Jennifer Orr, Francis Rossignol, Fatima Daniela Lopes, Julie Gauthier, Nathalie Alos, Rosemarie Rupps, Margaret McKinnon, Shelin Adam, Malgorzata J M Nowaczyk, Susan Walker, Stephen W Scherer, Christina Nassif, Fadi F Hamdan, Cheri L Deal, Jean-François Soucy, Rosanna Weksberg, Patrick Macleod, Jacques L Michaud, David Chitayat
BACKGROUND: Chitayat-Hall syndrome, initially described in 1990, is a rare condition characterised by distal arthrogryposis, intellectual disability, dysmorphic features and hypopituitarism, in particular growth hormone deficiency. The genetic aetiology has not been identified. METHODS AND RESULTS: We identified three unrelated families with a total of six affected patients with the clinical manifestations of Chitayat-Hall syndrome. Through whole exome or whole genome sequencing, pathogenic variants in the MAGEL2 gene were identified in all affected patients...
March 29, 2018: Journal of Medical Genetics
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