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Hormone Research in Pædiatrics

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https://www.readbyqxmd.com/read/28334719/meta-analysis-of-paediatric-patients-with-central-precocious-puberty-treated-with-intramuscular-triptorelin-11-25-mg-3-month-prolonged-release-formulation%C3%A2
#1
Adélaïde Durand, Maithé Tauber, Bharat Patel, Pascale Dutailly
BACKGROUND/AIMS: A meta-analysis was undertaken to assess the effect of triptorelin 11.25 mg 3-month prolonged-release formulation in central precocious puberty (CPP). METHODS: All available clinical studies with triptorelin 11.25 mg were included. The primary outcome was the proportion of children with suppressed luteinising hormone (LH) response (peak LH ≤3 IU/L) to the gonadotrophin-releasing hormone (GnRH) test 3 months after triptorelin 11.25 mg injection...
March 23, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28334714/new-genetic-diagnoses-of-short-stature-provide-insights-into-local-regulation-of-childhood-growth%C3%A2
#2
Anenisia C Andrade, Youn Hee Jee, Ola Nilsson
Idiopathic short stature is a common condition with a heterogeneous etiology. Advances in genetic methods, including genome sequencing techniques and bioinformatics approaches, have emerged as important tools to identify the genetic defects in families with monogenic short stature. These findings have contributed to the understanding of growth regulation and indicate that growth plate chondrogenesis, and therefore linear growth, is governed by a large number of genes important for different signaling pathways and cellular functions, including genetic defects in hormonal regulation, paracrine signaling, cartilage matrix, and fundamental cellular processes...
March 23, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28334704/combining-real-time-cold-and-mama-pcr-taqman-techniques-to-detect-and-quantify-r201-gnas-mutations-in-the-mccune-albright-syndrome%C3%A2
#3
Luisa de Sanctis, Ilaria Galliano, Paola Montanari, Patrizia Matarazzo, Daniele Tessaris, Massimiliano Bergallo
BACKGROUND/AIM: The McCune-Albright syndrome (MAS) is a potentially severe disorder hallmarked by fibrous bone dysplasia, café-au-lait skin spots, and endocrine hyperfunction. It is caused by postzygotic activating mutations at the R201 codon of the GNAS gene, leading to a state of somatic mosaicism. Our aim was to improve the mutation detection rate and to quantify the presence of R201 GNAS mutations in different DNA samples from MAS patients. METHODS: Real-time COLD- and MAMA-PCR TaqMan techniques were combined to search for R201 mutations in the DNA of blood or affected tissues from 16 previously molecularly characterized MAS patients, from a further 84 subjects with MAS signs who were R201 negative at RFLP analysis, and from 36 controls...
March 23, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28285312/world-perspective-on-the-epidemiology-of-stunting-between-1990-and-2015%C3%A2
#4
Susan C Campisi, Arlin Mary Cherian, Zulfiqar A Bhutta
BACKGROUND: Stunting is the most prevalent form of child undernutrition with current worldwide estimates at 156 million. The majority of these children reside in low- and middle-income countries. When stunting prevails to adulthood, optimal growth potential and economic productivity are minimized. RESULTS: In 2015 there were 98.5 million fewer stunted children under 5 years of age than in 1990. In East Asia and Pacific and South Asia, the stunting prevalence decreased by 24...
March 10, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28259872/characterization-of-thyroid-abnormalities-in-a-large-cohort-of-children-with-down-syndrome%C3%A2
#5
Melinda J Pierce, Stephen H LaFranchi, Joseph D Pinter
BACKGROUND/AIMS: Thyroid disease is a common comorbidity in individuals with Down syndrome (DS), but historical studies have multiple limitations. We assessed thyroid abnormalities in a large cohort of children with DS. METHODS: Retrospective records review from a single institution. Calculated prevalence of common thyroid abnormalities and associations with common comorbidities. RESULTS: Among 508 patients, 120 (24%) had a thyroid-related diagnosis, the majority having elevated thyrotropin treated with levothyroxine...
March 3, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28253515/growth-promotion-ethics-and-the-challenge-to-resist-cosmetic-endocrinology%C3%A2
#6
David B Allen
The advancement of "human growth hormone (hGH)-for-height" - increasing height attainment in children short for reasons other than GH deficiency - arose from intuitive, deep-seated assumptions about the disability of short stature, its improvement with hGH-mediated height gain, and the safety of escalating dosages of hGH in healthy children. Evidence challenging these assumptions now strengthens criticism of hGH-for-height as cosmetic endocrinology. To counter this characterization, collective acceptance of guidelines is needed that advise nontreatment of the vast majority of short children, support strategies that minimize treatment duration and dosage, and restrain enhancement of normal adult stature...
March 2, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28253507/hormonal-and-metabolic-responses-to-a-single-bout-of-resistance-exercise-in-prader-willi-syndrome%C3%A2
#7
Daniela A Rubin, Susan J Clark, Andrea M Haqq, Diobel M Castner, Jason Ng, Daniel A Judelson
BACKGROUND: Prader-Willi syndrome (PWS) is characterized by excessive adiposity. Excess adiposity negatively affects hormonal and metabolic responses to aerobic exercise. This study determined whether PWS and/or adiposity affected hormonal and metabolic responses to resistance exercise. METHODS: Eleven children with PWS (11.4 ± 3.1 years, 43.9 ± 7.5% body fat), 12 lean children (9.3 ± 1.4 years, 18.3 ± 4.9% body fat), and 13 obese children (9.6 ± 1.3 years, 40...
March 2, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28253506/the-effect-of-the-testis-on-the-ovary-structure-function-relationships-in-a-neonate-with-a-unilateral-ovotestis-ovotesticular-disorder-of-sex-development-%C3%A2
#8
Siri Atma W Greeley, Elizabeth Littlejohn, Aliya N Husain, Darrel Waggoner, Mohan Gundeti, Robert L Rosenfield
AIMS: To evaluate gonadal function in a newborn with suspected ovotesticular disorder of sex development (DSD). METHODS: Gonadal function was evaluated at baseline and after gonadotropin-releasing hormone agonist (GnRHag) stimulation testing. RESULTS: A full-term 46,XX neonate with genital ambiguity produced serum testosterone and anti-Müllerian hormone (AMH) levels appropriate for males within days, while serum estradiol remained prepubertal, both spontaneously and in response to GnRHag stimulation testing...
March 2, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28253502/short-stature-in-a-boy-with-multiple-early-onset-autoimmune-conditions-due-to-a-stat3-activating-mutation-could-intracellular-growth-hormone-signalling-be-compromised-%C3%A2
#9
Hana Sediva, Petra Dusatkova, Veronika Kanderova, Barbora Obermannova, Jana Kayserova, Lucie Sramkova, Dana Zemkova, Lenka Elblova, Michal Svaton, Radana Zachova, Stanislava Kolouskova, Eva Fronkova, Zdenek Sumnik, Anna Sediva, Jan Lebl, Stepanka Pruhova
BACKGROUND: Germline STAT3 gain-of-function (GOF) mutations cause multiple endocrine and haematologic autoimmune disorders, lymphoproliferation, and growth impairment. As the JAK-STAT pathway is known to transduce the growth hormone (GH) signalling, and STAT3 interacts with STAT5 in growth regulation, we hypothesised that short stature in STAT3 GOF mutations results mostly from GH insensitivity via involving activation of STAT5. CASE REPORT: A boy with a novel STAT3 c...
March 2, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28253494/how-milk-and-its-proteins-affect-growth-bone-health-and-weight%C3%A2
#10
Michal Yackobovitch-Gavan, Moshe Phillip, Galia Gat-Yablonski
Milk has long been recognized to constitute a complete, well-balanced source of the nutrients and energy required to ensure the proper postnatal growth and development of infants. A growing body of evidence suggests the positive effects of dairy products and particularly of milk proteins on linear growth also in older children, both healthy or during recovery from malnutrition. This evidence led the way to the performance of extensive research aimed to delineate the components of milk and the mechanisms acting to make milk so effective...
March 2, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28196370/in-memoriam-solomon-a-kaplan-1924-2017-%C3%A2
#11
Stephen H LaFranchi
No abstract text is available yet for this article.
February 14, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28196362/practical-application-of-linear-growth-measurements-in-clinical-research-in-low-and-middle-income-countries%C3%A2
#12
Jan M Wit, John H Himes, Stef van Buuren, Donna M Denno, Parminder S Suchdev
BACKGROUND/AIMS: Childhood stunting is a prevalent problem in low- and middle-income countries and is associated with long-term adverse neurodevelopment and health outcomes. In this review, we define indicators of growth, discuss key challenges in their analysis and application, and offer suggestions for indicator selection in clinical research contexts. METHODS: Critical review of the literature. RESULTS: Linear growth is commonly expressed as length-for-age or height-for-age z-score (HAZ) in comparison to normative growth standards...
February 14, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28183093/putative-effects-of-obesity-on-linear-growth-and-puberty%C3%A2
#13
Shlomit Shalitin, Wieland Kiess
Childhood obesity is a major public health problem that has grown to epidemic proportions throughout the world. Obesity is influenced by genetic and environmental factors. The nutritional status plays an important role in growth and body weight regulation. Excess adiposity during childhood can affect the process of growth and puberty. Obese children are frequently tall for their age, with accelerated epiphyseal growth plate maturation despite low growth hormone levels. Several regulatory hormones may affect the process of linear growth in the constellation of obesity, as high levels of insulin and leptin are observed in obese children...
February 9, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28166531/challenges-in-the-cross-sectional-assessment-of-cardiovascular-risk-in-children-with-premature-adrenarche-due-to-the-multifaceted-nature-of-atherosclerosis%C3%A2
#14
Ahmet Uçar
No abstract text is available yet for this article.
February 6, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28152538/two-cases-of-mistaken-polyuria-and-nephrocalcinosis-in-infants-with-glucose-galactose-malabsorption-a-possible-role-of-1-25-oh-2d3%C3%A2
#15
Melissa Fiscaletti, Marie-Jeanne Lebel, Nathalie Alos, Geneviève Benoit, Prévost Jantchou
BACKGROUND/AIMS: Glucose-galactose malabsorption (GGM) is a rare and potentially fatal disorder. The autosomal recessive mutation of the SGLT1 gene interferes with the active glucose transport in the gut resulting in osmotic diarrhea and failure to thrive (FTT). Two nonrelated infants with GGM are presented as well as a novel mutation in SGLT1. CASE PRESENTATION: The first case consulted for FTT and presented with hypercalcemia and hypercalciuria. His mother had self-medicated with high doses of vitamin D...
February 2, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28114141/lung-function-in-women-with-idiopathic-central-precocious-puberty-a-pilot-study%C3%A2
#16
Virginia Murri, Franco Antoniazzi, Michele Piazza, Paolo Cavarzere, Claudia Banzato, Attilio Boner, Rossella Gaudino
BACKGROUND: Studies have reported that women with early menarche (≤10 years) have lower lung function. AIM: To investigate lung function in women with a history of idio pathic central precocious puberty (ICPP) treated during childhood with gonadotropin-releasing hormone agonist (GnRHa). METHODS: ICPP women (n = 23) were compared with healthy age-matched controls (n = 23). Subjects were clinically evaluated by means of a questionnaire, baseline and post-β<Sub>2</Sub> agonist spirometry, impulse oscillometry (a measure of airway resistance), and measurement of fractional exhaled nitric oxide (FeNO)...
January 23, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28114130/identifying-cardiovascular-risk-in-survivors-of-childhood-leukaemia-treated-with-haematopoietic-stem-cell-transplantation-and-total-body-irradiation%C3%A2
#17
Christina Wei, Linda Hunt, Rachel Cox, Karin Bradley, Ruth Elson, Julian Shield, Michael Stevens, Elizabeth Crowne
BACKGROUND: Survivors of childhood with haematopoietic stem cell transplantation and total body irradiation (HSCT/TBI) have an increased cardiometabolic risk without overt obesity. AIM: To describe cardiometabolic risk in HSCT/TBI survivors and identify anthropometric measurements of adiposity representative of cardiometabolic risks in HSCT/TBI survivors. METHOD: Childhood leukaemia survivors treated with HSCT/TBI (n = 21, 11 males) were compared with chemotherapy-only (n = 31) and obese non-leukaemic controls (n = 30)...
January 23, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28081535/efficacy-and-safety-of-continuous-subcutaneous-infusion-of-recombinant-human-gonadotropins-for-congenital-micropenis-during-early-infancy%C3%A2
#18
Athanasia Stoupa, Dinane Samara-Boustani, Isabelle Flechtner, Graziella Pinto, Isabelle Jourdon, Laura González-Briceño, Maud Bidet, Kathleen Laborde, Didier Chevenne, Anne-Elodie Millischer, Henri Lottmann, Thomas Blanc, Yves Aigrain, Michel Polak, Jacques Beltrand
BACKGROUND: Early postnatal administration of gonadotropins to infants with congenital hypogonadotropic hypogonadism (CHH) can mimic minipuberty, thereby increasing penile growth. We assessed the effects of gonadotropin infusion on stretched penile length (SPL) and hormone levels in infants with congenital micropenis. METHODS: Single-center study including 6 males with micropenis in case of isolated CHH (n = 4), panhypopituitarism (n = 1), and partial androgen insensitivity syndrome (PAIS; n = 1)...
January 12, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28073108/salivary-testosterone-during-the-minipuberty-of-infancy%C3%A2
#19
Maria Contreras, Manish Raisingani, Donald Walt Chandler, William D Curtin, Julia Barillas, Preneet Cheema Brar, Kris Prasad, Bina Shah, Raphael David
BACKGROUND: The hypothalamic-pituitary-gonadal axis is transiently activated during the postnatal months in boys, a phenomenon termed "minipuberty" of infancy, when serum testosterone (T) increases to pubertal levels. Despite high circulating T there are no signs of virilization. We hypothesize that free T as measured in saliva is low, which would explain the absence of virilization. METHODS: We measured serum total T and free T in saliva using liquid chromatography-tandem mass spectrometry (LC-MS/MS) in 30 infant boys, aged 1-6 months, and in 12 adolescents, aged 11-17 years...
January 10, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28068655/carotid-intima-media-thickness-in-children-treated-with-growth-hormone-a-follow-up-study-over-three-years
#20
Juliane Rothermel, Caroline Knop, Nina Lass, Christina Toschke, Rainer Wunsch, Thomas Reinehr
BACKGROUND: There is an ongoing discussion whether high doses of growth hormone (GH) may lead to cardiovascular diseases. Therefore, we studied the longitudinal relationships between GH treatment and carotid intima-media thickness (cIMT), which is predictive of the development of atherosclerosis. METHODS: We measured blood pressure, lipids, hemoglobin HbA1c, IGF-1, IGFBP-3, and cIMT in 28 children treated with supraphysiological doses of GH (mean age 9.8 ± 2.2 years, 39% males) and 36 children suffering from GH deficiency (GHD) and treated with physiological doses of GH (mean age 9...
January 10, 2017: Hormone Research in Pædiatrics
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