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"Growth Hormone Deficiency"

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https://www.readbyqxmd.com/read/28643234/body-mass-disorders-in-healthy-short-children-and-in-children-with-growth-hormone-deficiency
#1
Paweł Tomaszewski, Katarzyna Milde, Anna Majcher, Beata Pyrżak, Gul Tiryaki-Sonmez, Brad J Schoenfeld
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. Basic somatic features were assessed and the study participants were categorized according to the percentage of body fat (%FAT), body mass index (BMI), and waist-to-height ratio (WHtR)...
June 23, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28634534/etiological-factors-of-short-stature-in-children-and-adolescents-experience-at-a-tertiary-care-hospital-in-egypt
#2
Almontaser Hussein, Hekma Farghaly, Eman Askar, Kotb Metwalley, Khaled Saad, Asmaa Zahran, Hisham A Othman
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...
May 2017: Therapeutic Advances in Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28634491/more-favorable-metabolic-impact-of-three-times-weekly-versus-daily-growth-hormone-treatment-in-na%C3%A3-ve-gh-deficient-children
#3
Alessandro Ciresi, Floriana Cicciò, Stefano Radellini, Valentina Guarnotta, Anna Maria Calcaterra, Carla Giordano
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 ± 2.2 yr) were randomly assigned to receive daily (group A, 16 patients) or TIW (group B, 16 patients) GHT for 12 months. METHODS: Auxological parameters, insulin-like growth factor-I (IGF-I), glucose and insulin during OGTT, glycosylated hemoglobin (HbA1c), lipid profile, the oral disposition index (DIo), the homeostasis model assessment estimate of insulin resistance (Homa-IR), and the insulin sensitivity index (ISI)...
2017: International Journal of Endocrinology
https://www.readbyqxmd.com/read/28626954/isolated-growth-hormone-deficiency-due-to-the-r183h-mutation-in-gh1-clinical-analysis-of-a-four-generation-family
#4
Catalina Cabrera-Salcedo, Amy S Shah, Melissa Andrew, Leah Tyzinski, Vivian Hwa, Iris Gutmark-Little, Philippe Backeljauw, Andrew Dauber
The R183H mutation in the growth hormone gene (GH1) is a well-described genetic variant that causes autosomal dominant isolated growth hormone deficiency (IGHD) type II. Previous studies have demonstrated that individuals with this mutation have releasable growth hormone (GH) stores, but such release is severely impaired. Hess et al. reported variable height deficits (-4.5 to -1.0 SDS), variable IGF-I concentrations (-2.9 to -0.8 SDS), and low but detectable, or even normal stimulated peak GH in several patients with the R183H mutation...
June 19, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28596789/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
#5
Renata Stawerska, Joanna Smyczyńska, Maciej Hilczer, Andrzej Lewiński
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. METHODS: The analysis comprised 26 children with GHD, aged 5...
2017: International Journal of Endocrinology
https://www.readbyqxmd.com/read/28593555/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
#6
Eline Starink, Anita C S Hokken-Koelega, Theo J Visser, Janneke Baan, Robin P Peeters, Laura C G de Graaff
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. After whole exome sequencing revealed a new mutation in IRF6 in a family with Idiopathic Growth Hormone Deficiency (IGHD), we screened the remainder of our IGHD cohort for mutations in this gene and related their genotypes to pituitary and craniofacial morphology...
June 7, 2017: Pituitary
https://www.readbyqxmd.com/read/28591479/hypothalamic-hamartoma-with-epilepsy-review-of-endocrine-comorbidity
#7
Victor S Harrison, Oliver Oatman, John F Kerrigan
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...
June 2017: Epilepsia
https://www.readbyqxmd.com/read/28587427/mri-features-of-growth-hormone-deficiency-in-children-with-short-stature-caused-by-pituitary-lesions
#8
Chao Xu, Xinxian Zhang, Lina Dong, Bin Zhu, Tao Xin
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 (61.3%) with anterior pituitary dysplasia; 45 cases (7.8%) of pituitary stalk interruption syndrome (PSIS); 15 cases (2.6%) of pituitary hyperplasia due to primary hypothyroidism; 38 cases (6...
June 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28586151/a-heterozygous-microdeletion-of-20p12-2-3-encompassing-prokr2-and-bmp2-in-a-patient-with-congenital-hypopituitarism-and-growth-hormone-deficiency
#9
Samuel J H Parsons, Neville B Wright, Emma Burkitt-Wright, Mars S Skae, Phillip G Murray
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. The proband is a 5-year-old girl who presented with neonatal hypoglycaemia and prolonged jaundice...
June 6, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28574152/serum-igf-i-levels-are-associated-with-improved-white-matter-recovery-after-tbi
#10
Claire Feeney, David J Sharp, Peter J Hellyer, Amy E Jolly, James H Cole, Gregory Scott, David Baxter, Sagar Jilka, Ewan Ross, Timothy E Ham, Peter O Jenkins, Lucia M Li, Nikos Gorgoraptis, Mark Midwinter, Anthony P Goldstone
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. We conducted a longitudinal study to determine the effects of baseline serum IGF-I concentrations on WM tract and neuropsychological recovery after TBI...
June 2, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28566441/long-acting-pegylated-recombinant-human-growth-hormone-jintrolong%C3%A2-for-children-with-growth-hormone-deficiency-phase-ii-and-phase-iii-multicenter-randomized-studies
#11
Xiaoping Luo, Ling Hou, Li Liang, Guanping Dong, Shuixian Shen, Zhuhui Zhao, Chun Xiu Gong, Yuchuan Li, Min-Lian Du, Zhe Su, Hongwei Du, Chaoying Yan
OBJECTIVE: We assessed the efficacy and safety of a weekly PEGylated human growth hormone (PEG-rhGH) (Jintrolong®) vs. daily rhGH for children with growth hormone deficiency (GHD). DESIGN: Phase II and III, multicenter, open-label, randomized controlled trials. METHODS: 108 and 343 children with treatment-naive GHD from 6 hospitals in China were enrolled in the phase II and III studies, respectively. Patients in the phase II study were randomized 1:1:1 to weekly Jintrolong® (0...
May 31, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28543706/using-a-spontaneous-profile-rather-than-stimulation-test-makes-the-kigs-idiopathic-growth-hormone-deficiency-model-more-accessible-for-clinicians
#12
Karel Duchén, Anders Lindberg, Kaire Kiplok, Berit Kriström
AIM: Children treated with a growth hormone (GH) for idiopathic growth hormone deficiency (IGHD) may be monitored with the first-year prediction model from the Pfizer International Growth Database (KIGS) using auxology, age, GH dose and the maximum GH concentration from a stimulation test (GHmax stim). We tested the hypothesis that using a 12-hour spontaneous profile (GHmax 12h) would be as accurate. METHODS: We studied 98 prepubertal Swedish children (78 boys) aged 2-12 years enrolled in KIGS...
May 22, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/28529275/safety-and-effectiveness-of-long-term-growth-hormone-therapy-in-japanese-patients-with-adult-growth-hormone-deficiency-a-postmarketing-multicenter-observational-study
#13
Akira Shimatsu, Hitoshi Ishii, Hiromi Nishinaga, Osamu Murai, Kazuo Chihara
We aimed to evaluate the long-term safety and effectiveness of growth hormone (GH) therapy in Japanese patients with adult growth hormone deficiency (AGHD). In this observational, multicenter study, Norditropin(®) (Novo Nordisk A/S, Bagsvaerd, Denmark) was administered as injections of 0.021 mg/kg/week as a starting dose divided into 6-7 doses/week. The dose was increased according to clinical response. Patients' data were obtained from medical records. Measurements (lipids, glucose metabolism, and body composition) taken at baseline; 3, 6, and 12 months; and yearly until the end of the study were collected...
May 19, 2017: Endocrine Journal
https://www.readbyqxmd.com/read/28525404/growth-hormone-treatment-for-growth-hormone-deficiency-and-idiopathic-short-stature-new-guidelines-shaped-by-the-presence-and-absence-of-evidence
#14
Adda Grimberg, David B Allen
PURPOSE OF REVIEW: The Pediatric Endocrine Society recently published new guidelines for the use of human growth hormone (hGH) and human insulin-like growth factor-I (hIGF-I) treatment for growth hormone deficiency, idiopathic short stature, and primary IGF-I deficiency in children and adolescents. This review places the new guidelines in historical contexts of the life cycle of hGH and the evolution of US health care, and highlights their future implications. RECENT FINDINGS: The new hGH guidelines, the first to be created by the Grading of Recommendations Assessment, Development and Evaluation approach, are more conservative than their predecessors...
May 18, 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28525353/molecular-genetics-of-growth-hormone-deficient-children-correlation-with-auxology-and-response-to-first-year-of-growth-hormone-therapy
#15
Vaman Khadilkar, Nikhil Phadke, Kavita Khatod, Veena Ekbote, Supriya Phanse Gupte, Ruchi Nadar, Anuradha Khadilkar
BACKGROUND: With the paucity of available literature correlating genetic mutation and response to treatment, we aimed to study the genetic makeup of children with growth hormone (GH) deficiency in Western India and correlate the mutation with auxology and response to GH treatment at end of 1 year. METHODS: Fifty-three (31 boys and 22 girls) children with severe short stature (height for age z-score <-3) and failed GH stimulation test were studied. Those having concomitant thyroid hormone or cortisol deficiencies were appropriately replaced prior to starting GH treatment...
May 24, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28522645/real-life-gh-dosing-patterns-in-children-with-ghd-ts-or-born-sga-a-report-from-the-nordinet%C3%A2-international-outcome-study
#16
Oliver Blankenstein, Marta Snajderova, Joanne C Blair, Effie Pournara, Birgitte Tønnes Pedersen, Isabelle Oliver Petit
OBJECTIVE: To describe real-life dosing patterns in children with growth hormone deficiency (GHD), born small for gestational age (SGA) or with Turner syndrome (TS) receiving growth hormone (GH) and enrolled in the NordiNet® International Outcome Study (IOS; NCT00960128) between 2006 and 2016. DESIGN: This non-interventional, multicentre study included paediatric patients diagnosed with GHD (isolated [IGHD] or multiple pituitary hormone deficiency [MPHD]), born SGA or with TS and treated according to everyday clinical practice from the Czech Republic (IGHD/MPHD/SGA/TS: n=425/61/316/119), France (n=1404/188/970/206), Germany (n=2603/351/1387/411) and the UK (n=259/60/87/35)...
May 18, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28516749/international-comparison-of-adult-height-in-children-with-growth-hormone-deficiency-and-limitations-of-growth-hormone-treatment-in-japan
#17
Toshiaki Tanaka
The approved therapeutic dose of growth hormone (GH) for growth hormone deficiency (GHD) varies depending on the country. Japan has the lowest therapeutic dose globally, with a single dose of 0.175 mg/kg/week. GH treatment for GHD is considered as a replacement therapy and in fact, a dose of 0.175 mg/kg/week is slightly higher than GH secretion in prepubertal healthy children but nearly the same as that of pubertal children. Although the same growth rate as that of healthy children is expected in response to replacement therapy, the catch-up growth observed for the first 1 to 2 years of GH treatment was misinterpreted as an effect of the GH replacement therapy...
March 2017: Pediatric Endocrinology Reviews: PER
https://www.readbyqxmd.com/read/28516747/the-history-of-growth-hormone-treatment-for-ghd-in-japan
#18
Susumu Yokoya, Toshiaki Tanaka
In Japan, treatment of growth hormone deficiency with pituitary-extracted human growth hormone (phGH) was covered by health insurance for the first time in 1975. However, because of the shortage of phGH, the Foundation for Growth Science (FGS) was founded in 1977 to control the use of the product by its registration system and to collect pituitary glands in Japan. In 1986, recombinant human growth hormone was first approved. Since then, the FGS has been involved in the harmonization of growth hormone measurement, assessment for treatment eligibility according to the diagnostic criteria by the research group of the Ministry of Health and Welfare, and database generation and its utilization...
March 2017: Pediatric Endocrinology Reviews: PER
https://www.readbyqxmd.com/read/28511709/incorporation-of-high-dose-131-i-metaiodobenzylguanidine-treatment-into-tandem-high-dose-chemotherapy-and-autologous-stem-cell-transplantation-for-high-risk-neuroblastoma-results-of-the-smc-nb-2009-study
#19
Ji Won Lee, Sanghoon Lee, Hee Won Cho, Youngeun Ma, Keon Hee Yoo, Ki Woong Sung, Hong Hoe Koo, Eun Joo Cho, Suk-Koo Lee, Do Hoon Lim
BACKGROUND: In our previous SMC NB-2004 study of patients with high-risk neuroblastomas, which incorporated total-body irradiation (TBI) with second high-dose chemotherapy and autologous stem cell transplantation (HDCT/auto-SCT), the survival rate was encouraging; however, short- and long-term toxicities were significant. In the present SMC NB-2009 study, only TBI was replaced with (131)I-meta-iodobenzylguanidine (MIBG) treatment in order to reduce toxicities. METHODS: From January 2009 to December 2013, 54 consecutive patients were assigned to receive tandem HDCT/auto-SCT after nine cycles of induction chemotherapy...
May 16, 2017: Journal of Hematology & Oncology
https://www.readbyqxmd.com/read/28511077/clinical-and-laboratory-parameters-predicting-a-requirement-for-the-reevaluation-of-growth-hormone-status-during-growth-hormone-treatment-retesting-early-in-the-course-of-gh-treatment
#20
Dogus Vuralli, E Nazli Gonc, Z Alev Ozon, Ayfer Alikasifoglu, Nurgun Kandemir
OBJECTIVE: We aimed to define the predictive criteria, in the form of specific clinical, hormonal and radiological parameters, for children with growth hormone deficiency (GHD) who may benefit from the reevaluation of GH status early in the course of growth hormone (GH) treatment. DESIGN AND METHODS: Two hundred sixty-five children with growth hormone deficiency were retested by GH stimulation at the end of the first year of GH treatment. The initial clinical and laboratory characteristics of those with a normal (GH≥10ng/ml) response and those with a subnormal (GH<10ng/ml) response were compared to predict a normal GH status during reassessment...
June 2017: Growth Hormone & IGF Research
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