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Hormone Research in Pædiatrics

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https://www.readbyqxmd.com/read/28528334/hormones-and-human-and-nonhuman-primate-growth
#1
Robin Miriam Bernstein
The aim of this paper was to review information pertaining to the hormonal regulation of nonhuman primate growth, with specific focus on the growth hormone (GH)-insulin-like growth factor (IGF) axis and adrenal androgens. Hormones of the GH-IGF axis are consistently associated with measures of growth - linear, weight, or both - during the growth period; in adulthood, concentrations of IGF-I, IGF-binding protein-3, and GH-binding protein are not associated with any measures of size. Comparing patterns of dehydroepiandrosterone (DHEA) and DHEA sulfate (DHEAS) may be especially relevant for understanding whether the childhood stage of growth and development is unique to humans and perhaps other apes...
May 19, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28528327/three-quarters-adrenalectomy-for-infantile-onset-cushing-syndrome-due-to-bilateral-adrenal-hyperplasia-in-mccune-albright-syndrome
#2
Tomoyo Itonaga, Hironori Goto, Manabu Toujigamori, Yasuharu Ohno, Seigo Korematsu, Tatsuro Izumi, Satoshi Narumi, Tomonobu Hasegawa, Kenji Ihara
BACKGROUND: Bilateral adrenalectomy is performed in cases with infantile-onset Cushing syndrome due to bilateral adrenal hyperplasia in McCune-Albright syndrome (MAS) because severe Cushing syndrome with heart failure and liver dysfunction can have a lethal outcome. This procedure can completely ameliorate hypercortisolism, although lifetime steroid replacement therapy and steps to prevent adrenal crisis are necessary. Recently, the efficacy of unilateral adrenalectomy has been reported in adult cases of bilateral macronodular adrenal hyperplasia, but there is no consensus regarding the appropriate surgical treatment for bilateral adrenal hyperplasia in MAS...
May 19, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28521312/the-influence-of-the-manner-of-performing-the-thyroid-ultrasound-examination-on-the-reliability-of-the-assessment-of-the-thyroid-size-in-school-aged-children
#3
Arkadiusz Zygmunt, Zbigniew Adamczewski, Agnieszka Zygmunt, Malgorzata Karbownik-Lewinska, Andrzej Lewinski
BACKGROUND/AIMS: Goitre incidence in school-aged children evaluated using ultrasonography is one of the essential indicators of iodine intake in a given area. The aim of the study was to examine what the difference is between the volume of the thyroid gland measured in the supine and sitting position and to determine the intra-observer, inter-observer, and inter-position variations. METHODS: The survey was conducted among 87 children (56 girls and 31 boys aged 7-13 years, mean age 10...
May 18, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28505613/to-prime-or-not-to-prime-is-that-still-a-question-a-comment-on-the-us-guidelines-on-growth-hormone-and-insulin-like-growth-factor-i-treatment-in-children-and-adolescents
#4
Maria Lodefalk, Ola Nilsson
No abstract text is available yet for this article.
May 15, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28505610/predictors-of-loss-to-follow-up-among-children-with-type-2-diabetes
#5
Ashley Shoemaker, Peiyao Cheng, Robin L Gal, Craig Kollman, William V Tamborlane, Georgeanna J Klingensmith, Mark A Clements, Tamara S Hannon, Rubina Heptulla, Joane Less, Jamie Wood
BACKGROUND/AIMS: Youth with type 2 diabetes (T2D) have poor compliance with medical care. This study aimed to determine which demographic and clinical factors differ between youth with T2D who receive care in a pediatric diabetes center versus youth lost to follow-up for >18 months. METHODS: Data were analyzed from 496 subjects in the Pe-diatric Diabetes Consortium registry. Enrollment variables were selected a priori and analyzed with univariable and multivariable logistic regression models...
May 15, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28486235/outcome-of-doctors-attending-the-european-society-for-paediatric-endocrinology-winter-schools-2008-2014
#6
Malcolm D C Donaldson
BACKGROUND/AIMS: Since 1995, the European Society for Paediatric Endocrinology (ESPE) has hosted Winter School (WS), a 5-day interactive meeting of 25 trainees and 7 teachers designed to cover all main endocrine topics. The aim of the present study was to determine subsequent outcome in ex-WS "students." METHODS: A questionnaire was sent in August 2016 to all ESPE WS participants between 2008 and 2014. Information was requested on job/training status at WS attendance and in 2016, and subsequent involvement with ESPE...
May 9, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28486222/biomarkers-to-stratify-risk-groups-among-children-with-malnutrition-in-resource-limited-settings-and-to-monitor-response-to-intervention
#7
Christine J McGrath, Michael B Arndt, Judd L Walson
Despite global efforts to reduce childhood undernutrition, current interventions have had little impact on stunting and wasting, and the mechanisms underlying growth faltering are poorly understood. There is a clear need to distinguish populations of children most likely to benefit from any given intervention and to develop tools to monitor response to therapy prior to the development of morbid sequelae. In resource-limited settings, environmental enteric dysfunction (EED) is common among children, contributing to malnutrition and increasing childhood morbidity and mortality risk...
May 9, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28478437/alterations-in-glucose-effectiveness-and-insulin-dynamics-polycystic-ovary-syndrome-or-body-mass-index
#8
Patricia Vuguin, Aviva B Sopher, Hailey Roumimper, Vivian Chin, Miriam Silfen, Donald J McMahon, Ilene Fennoy, Sharon E Oberfield
BACKGROUND/AIMS: To delineate the relationship of polycystic ovary syndrome (PCOS), obesity, and hyperandrogenism (HA) with glucose and insulin dynamics in adolescents across a broad body mass index (BMI). METHODS: Seventy-four PCOS subjects (aged 16 years) and 82 controls (aged 16 years) were evaluated by an oral glucose tolerance test. Subjects were categorized by BMI: normal weight (21 ± 0.4), overweight/obesity (OO; 33 ± 1.0), and severe obesity (SO; 48 ± 1...
May 5, 2017: Hormone Research in Pædiatrics
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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