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Hormone Research in Pædiatrics

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https://www.readbyqxmd.com/read/28437788/high-prevalence-of-polycystic-ovary-syndrome-in-type-1-diabetes-mellitus-adolescents-is-there-a-difference-depending-on-the-nih-and-rotterdam-criteria
#1
Kanetee Busiah, Ana Colmenares, Maud Bidet, Nadia Tubiana-Rufi, Claire Levy-Marchal, Christine Delcroix, Paul Jacquin, Delphine Martin, Lila Benadjaoud, Evelyne Jacqz-Aigrain, Kathleen Laborde, Jean-Jacques Robert, Dinane Samara-Boustani, Michel Polak
BACKGROUND: Polycystic ovary syndrome (PCOS) is more frequently observed in type 1 diabetes mellitus (T1DM) adult women than in nondiabetic women. No such prevalence has yet been studied in adolescent girls with T1DM. AIM: The aim of this study was to evaluate the prevalence of PCOS in adolescent girls with T1DM and to determine the clinical and hormonal features associated with the disorder. METHODS: A cross-sectional study of 53 adolescent girls (gynecological age >2 years) referred for routine evaluation for T1DM was conducted...
April 24, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28437784/the-physiology-of-childhood-growth-hormonal-regulation
#2
Emelie Benyi, Lars Sävendahl
The growth patterns of a child changes from uterine life until the end of puberty. Height velocity is highest in utero and declines after birth until puberty when it rises again. Important hormonal regulators of childhood growth are growth hormone, insulin-like growth factor 1, sex steroids, and thyroid hormone. This review gives an overview of these hormonal regulators of growth and their interplay with nutrition and other key players such as inflammatory cytokines.
April 24, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28433999/circadian-plasma-cortisol-measurements-reflect-severity-of-hypercortisolemia-in-children-with-different-etiologies-of-endogenous-cushing-syndrome
#3
Amit Tirosh, Maya B Lodish, Charalampos Lyssikatos, Elena Belyavskaya, Georgios Z Papadakis, Constantine A Stratakis
BACKGROUND: The utility of circadian cortisol variation in estimating the degree of hypercortisolemia in different forms of endogenous Cushing syndrome (CS) has not been evaluated in children yet. METHODS: A retrospective cohort study, including children who underwent surgery due to CS (n = 115), was divided into children with a pituitary adenoma (Cushing disease) (n = 88), primary adrenal CS (n = 21), or ectopic adrenocorticotropin- or corticotropin-releasing hormone (ACTH-/CRH)-secreting tumors (n = 6)...
April 21, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28399539/pigment-epithelium-derived-factor-declines-in-response-to-an-oral-glucose-load-and-is-correlated-with-vitamin-d-and-bmi-but-not-diabetes-status-in-children-and-young-adults
#4
Allison C Sylvetsky, Najy T Issa, Avinash Chandran, Rebecca J Brown, Hussam J Alamri, Gabriella Aitcheson, Mary Walter, Kristina I Rother
BACKGROUND: Pigment epithelium-derived factor (PEDF) is associated with obesity and diabetes complications in adults, yet little is known about PEDF in younger individuals. We investigated the relationship between PEDF and various metabolic biomarkers in young healthy volunteers (HV) and similar-aged patients with diabetes (type 1 and type 2). METHODS: A fasting blood sample was collected in 48 HV, 11 patients with type 1 diabetes (T1D), and 11 patients with type 2 diabetes (T2D) 12-25 years of age...
April 11, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28399535/17%C3%AE-estradiol-and-gonadotropin-levels-for-the-diagnosis-of-the-benign-form-of-breast-development-in-girls-aged-up-to-4-years-in-real-life-clinical-practice
#5
Martin Österbrand, Hans Fors, Kerstin Allvin, Ensio Norjavaara
BACKGROUND: Early onset of breast development in a young girl is usually a benign and isolated prepubertal condition, i.e., premature thelarche (PT), but can sometimes be progressive and the first sign of pubertal precocity (PP). Serum 17β-estradiol (17β-E2) level is a possible marker to differentiate between benign and pathological forms of breast development. We defined an upper serum 17β-E2 level for benign, "classic" PT for girls aged 9-48 months. METHODS: Serum 17β-E2 was analysed with a highly sensitive extraction radioimmunoassay (RIA)...
April 11, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28399519/pharmacokinetic-and-pharmacodynamic-modeling-of-mod-4023-a-long-acting-human-growth-hormone-in-growth-hormone-deficiency-children
#6
Dennis M Fisher, Ron G Rosenfeld, Michal Jaron-Mendelson, Leanne Amitzi, Ronit Koren, Gili Hart
BACKGROUND/AIMS: MOD-4023 is a long-acting human growth hormone (hGH) in clinical trials for the treatment of growth hormone deficiency (GHD). A key goal is maintenance of serum concentrations of insulin-like growth factor (IGF) 1 within normal range throughout GH dosing. The study aimed to develop a pharmacokinetic model for MOD-4023 and a pharmacodynamic model for the effect of MOD-4023 on IGF-1 to allow estimation of peak and mean IGF-1 and to identify the optimal IGF-1 sampling day...
April 11, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28395282/expanding-genetic-and-functional-diagnoses-of-igf1r-haploinsufficiencies
#7
Paula Ocaranza, Marjorie C Golekoh, Shayne F Andrew, Michael H Guo, Paul Kaplowitz, Howard Saal, Ron G Rosenfeld, Andrew Dauber, Fernando Cassorla, Philippe F Backeljauw, Vivian Hwa
BACKGROUND: The growth-promoting effects of IGF-I is mediated through the IGF-I receptor (IGF1R), a widely expressed cell-surface tyrosine kinase receptor. IGF1R copy number variants (CNV) can cause pre- and postnatal growth restriction or overgrowth. METHODS: Whole exome sequence (WES), chromosomal microarray, and targeted IGF1R gene analyses were performed on 3 unrelated children who share features of small for gestational age, short stature, and elevated serum IGF-I, but otherwise had clinical heterogeneity...
April 10, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28395280/triple-a-syndrome-preliminary-response-to-the-antioxidant-n-acetylcysteine-treatment-in-a-child
#8
Maria Candida Barisson Villares Fragoso, Edoarda Vasco de Albuquerque Albuquerque, Ana Luiza de Almeida Cardoso, Paula Waki Lopes da Rosa, Rodrigo Bomeny de Paulo, Maria Heloisa Massola Schimizu, Antonio Carlos Seguro, Marisa Passarelli, Katrin Koehler, Angela Huebner, Madson Q Almeida, Ana Claudia Latronico, Ivo Jorge Prado Arnhold, Berenice Bilharinho Mendonca
INTRODUCTION: Triple A syndrome (AAAS) is a rare autosomal recessive disorder characterized by alacrima, achalasia, ACTH-resistant adrenal insufficiency, autonomic dysfunction, and progressive neurodegeneration. Increased oxidative stress, demonstrated in patients' fibroblasts in vitro, may be a central disease mechanism. N-acetylcysteine protects renal function in patients with kidney injuries associated with increased oxidative stress and improves viability of AAAS-knockdown adrenal cells in vitro...
April 10, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28391281/inside-out-the-ragbag-of-glucose-intolerance-in-obese-adolescents
#9
Claudia Brufani, Andrea Tura, Giorgio Bedogni, Rosa Luciano, Stefano Sbrignadello, Danilo Fintini, Marco Cappa, Ram Weiss, Melania Manco
BACKGROUND/AIMS: The prevalence of impaired glucose tolerance (IGT) is rising among obese adolescents in parallel with epidemic obesity. In some cases, IGT reverts to normal glucose tolerance (NGT) by the end of puberty. The aims of the present study were to investigate metabolic factors determining changes over time of glucose at 120 min (Glu120) following an oral glucose tolerance test (OGTT), and to verify whether preserved β-cell glucose sensitivity (βCGS) protects against persistent IGT...
April 7, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28376481/use-of-hormone-replacement-in-females-with-endocrine-disorders
#10
Sophie Christin-Maitre
Hormone replacement therapy (HRT) is necessary in adolescents with primary ovarian insufficiency (POI) in order to avoid estrogen deficiency. The goal of this minirewiew is to present the different types of estrogens (17β-estradiol, estradiol valerate, ethinyl estradiol, and combined equine estrogens) as well as the different types of progestins available. In order to choose among the different types of HRTs, the features of each regimen are being discussed as well as their risks and their respective benefits...
April 4, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28376506/the-spectrum-of-ovotesticular-disorders-of-sex-development-in-south-africa-a-single-centre-experience
#11
Yasmeen Ganie, Colleen Aldous, Yusentha Balakrishna, Rinus Wiersma
OBJECTIVE: To describe the clinical characteristics, biochemistry, histopathology, and long-term outcomes in subjects with ovotesticular (OT) disorder of sex development (DSD). STUDY DESIGN: This is a retrospective subset analysis of 64 cases of histologically confirmed OT DSD. RESULTS: All subjects were South African; 97% (n = 62) were African and 92% (n = 59) were of Zulu ethnicity. The most common karyotype was 46,XX (88%; n = 56), followed by 46,XY (8%), 46,XY/45,X (3%), and 46,XX/46,XY (1%)...
April 3, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28376474/magnetic-resonance-imaging-features-as-surrogate-markers-of-x-linked-hypophosphatemic-rickets-activity
#12
Marta Lempicki, Anya Rothenbuhler, Valérie Merzoug, Stéphanie Franchi-Abella, Catherine Chaussain, Catherine Adamsbaum, Agnès Linglart
OBJECTIVE: X-linked hypophosphatemic rickets (XLH) is the most common form of inheritable rickets. Rickets treatment is monitored by assessing alkaline phosphatase (ALP) levels, clinical features, and radiographs. Our objectives were to describe the magnetic resonance imaging (MRI) features of XLH and to assess correlations with disease activity. STUDY DESIGN: Twenty-seven XLH patients (median age 9.2 years) were included in this prospective single-center observational study...
April 3, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28365712/determinants-of-advanced-bone-age-in-childhood-obesity%C3%A2
#13
Cornelis J de Groot, Adriaan van den Berg, Bart E P B Ballieux, Herman M Kroon, Edmond H H M Rings, Jan M Wit, Erica L T van den Akker
BACKGROUND: Childhood obesity is associated with advanced bone age (BA). Previous studies suggest that androgens, oestrogens, sex hormone-binding globulin, and insulin are responsible for this phenomenon, but results are contradictory and might be biased by confounders. We aim to elucidate this matter by applying a multivariate approach. METHOD: We performed a correlation analysis of BA standard deviation score (SDS) with age- and sex-specific SDS for androgens, oestrogens, and with indicators of insulin secretion derived from oral glucose tolerance testing, in a group of obese children...
March 31, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28365711/melvin-m-grumbach-md-1925-2016%C3%A2
#14
Walter L Miller
No abstract text is available yet for this article.
March 31, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28365689/effect-of-nutrition-on-statural-growth%C3%A2
#15
Galia Gat-Yablonski, Francesco De Luca
In children, proper growth and development are often regarded as a surrogate marker for good health. A complex system controls the initiation, rate, and cessation of growth, and thus gives a wonderful example of the interactions between genetics, epigenetics, and environmental factors (especially stress and nutrition). Malnutrition is considered a leading cause of growth attenuation in children. This review summarizes our current knowledge regarding the mechanisms linking nutrition and skeletal growth, including systemic factors, such as insulin, growth hormone, insulin-like growth factor-1, fibroblast growth factor-21, etc...
March 31, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28359065/evaluating-weight-status-and-sex-as-moderators-of-the-association-of-serum-leptin-with-bone-mineral-density-in-children-and-adolescents%C3%A2
#16
Sara A Armaiz-Flores, Nichole R Kelly, Ovidiu A Galescu, Andrew P Demidowich, Anne M Altschul, Sheila M Brady, Van S Hubbard, Courtney K Pickworth, Marian Tanofsky-Kraff, Lauren B Shomaker, James C Reynolds, Jack A Yanovski
BACKGROUND/AIMS: Animal studies suggest that leptin may adversely affect bone mineral density (BMD). Clinical studies have yielded conflicting results. We therefore investigated associations between leptin and bone parameters in children. METHODS: 830 healthy children (age = 11.4 ± 3.1 years; 75% female; BMI standard deviation score [BMIz] = 1.5 ± 1.1) had fasting serum leptin measured with ELISA and body composition by dual-energy X-ray absorptiometry. The main effects for leptin and BMIz plus leptin's interactions with sex and BMIz were examined using hierarchical linear regressions for appendicular, pelvis, and lumbar spine BMD as well as bone mineral content (BMC), and bone area (BA)...
March 30, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28359061/intrafamilial-phenotypic-variability-and-consequences-of-non-compliance-with-treatment-in-congenital-adrenal-hyperplasia-and-congenital-hypothyroidism-within-a-single-family%C3%A2
#17
Nicola Improda, Caroline Ponmani, Nadia Schoenmakers, Senthil Senniappan, Abigail Atterbury, Angela Barnicoat, Krishna Chatterjee, Mehul T Dattani
BACKGROUND: Coexistence of congenital adrenal hyperplasia (CAH) and congenital hypothyroidism (CH) due to TG mutation in the same non-consanguineous family is rare. CASE SERIES: We report 4 siblings born to unrelated parents, the father being an asymptomatic carrier of homozygous p.V281L and heterozygous p.I172N CYP21A2 mutations. Sibling 1 had salt-wasting CAH (CYP21A2 genotype Intron 2 splice/p.I172N and p.V281L). She also had CH (TG genotype p.R296/ p.T1416Rfs*30) and learning difficulties...
March 30, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28359060/growth-of-children-with-food-allergy%C3%A2
#18
Ivan Pavić, Sanja Kolaček
BACKGROUND: The prevalence of food allergy in children is increasing worldwide. Strict avoidance of identified allergens from the diet is still the cornerstone of the management of food allergies. There are widespread concerns that food allergy and elimination diet may predispose children to nutrient deficiencies and growth failure. AIM: The aim of this paper was to review the current evidence on growth of children suffering from food allergy. Summarised is literature on the effects of the number and type of offending allergens, the child's age and affected organ system, as well as the importance of supervision by a dietician/nutritionist...
March 30, 2017: Hormone Research in Pædiatrics
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
#19
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
#20
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
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