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Hormone Research in Pædiatrics

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https://www.readbyqxmd.com/read/28618411/prevalence-and-physical-distribution-of-sry-in-the-gonads-of-a-woman-with-turner-syndrome-phenotypic-presentation-tubal-formation-and-malignancy-risk
#1
Tamar G Baer, Christopher E Freeman, Claudia Cujar, Mahesh Mansukhani, Bahadur Singh, Xiaowei Chen, Rosanna Abellar, Sharon E Oberfield, Brynn Levy
Although monosomy X is the most common karyotype in patients with Turner syndrome, the presence of Y chromosome material has been observed in about 10% of patients. Y chromosome material in patients with Turner syndrome poses an increased risk of gonadoblastoma and malignant transformation. We report a woman with a diagnosis of Turner syndrome at 12 years of age, without signs of virilization, and karyotype reported as 46,X,del(X)(q13). At 26 years, cytogenetic studies indicated the patient to be mosaic for monosomy X and a cell line that contained a du-plicated Yq chromosome...
June 15, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28614818/thyroid-stimulating-hormone-degree-of-obesity-and-metabolic-risk-markers-in-a-cohort-of-obese-swedish-children
#2
Veroniqa Lundbäck, Kerstin Ekbom, Emilia Hagman, Ingrid Dahlman, Claude Marcus
BACKGROUND/AIMS: Thyroid-stimulating hormone (TSH) is affected in obesity and might influence metabolic risk. It is unclear what mechanisms cause elevated TSH in obesity. We aimed to investigate TSH status within the normal range and the association of TSH with degree of obesity and metabolic parameters in children with obesity. METHODS: A total of 3,459 children, aged 3.0-17.9 years, were identified in the Swedish Childhood Obesity Treatment Registry, BORIS. Age, gender, TSH, free triiodothyronine (fT3), free thyroxine (fT4), body mass index standard deviation scores (BMI SDS), as well as variables of lipid and glucose metabolism were examined...
June 14, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28605746/pls3-mutations-in-x-linked-osteoporosis-clinical-and-bone-characteristics-of-two-novel-mutations
#3
Peter Kannu, Areej Mahjoub, Riyana Babul-Hirji, Melissa T Carter, Jennifer Harrington
BACKGROUND AND OBJECTIVES: Plastin 3 (PLS3) mutations are associated with an X-linked osteoporosis. Here we describe two new families with novel mutations, including one with a whole gene PLS3 deletion, and review the literature on 9 previously reported cases. RESULTS: Hemizygous male carriers presented with multiple peripheral bone fractures, low bone mineral density (BMD), and vertebral compression fractures. Heterozygous female carriers did not have a history of fragility fractures, although 1 individual presented with low BMD...
June 12, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28571015/insulin-like-growth-factors-and-metabolic-syndrome-in-obese-children
#4
Elena Inzaghi, Barbara Baldini Ferroli, Danilo Fintini, Armando Grossi, Valerio Nobili, Stefano Cianfarani
BACKGROUND/AIMS: Insulin-like growth factor (IGF)-I is related to cardiometabolic risk in adults, whereas the metabolic role of IGF-II is unclear. The aim of this study was to assess IGFs in obese children and correlate them with metabolic syndrome (MetS) components. METHODS: This is a retrospective study including 574 obese children (11.34 ± 3.16 years). All subjects underwent complete anthropometry and biochemical assessment. In a subgroup of 136 subjects, body composition was evaluated...
June 1, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28554178/vitamin-d-and-albuminuria-in-youth-with-and-without-type-1-diabetes
#5
Debika Nandi-Munshi, Maryam Afkarian, Kathryn B Whitlock, Jamie L Crandell, Ronny A Bell, Ralph D'Agostino, Sharon Saydah, Amy K Mottl, Dana Dabelea, Mary Helen Black, Elizabeth J Mayer-Davis, Catherine Pihoker
BACKGROUND/AIMS: In adults, lower vitamin D has been associated with increased albuminuria. This association has not been extensively studied in youth with or without type 1 diabetes. METHODS: We examined the cross-sectional association between vitamin D and albuminuria (urine albumin to creatinine ratio ≥30 mg/g) in 8,789 participants of the National Health and Nutrition Survey 2001-2006 (NHANES), who were 6-19 years old. Further, we examined the association between vitamin D and albuminuria in 938 participants from the SEARCH Nutritional Ancillary Study (SNAS), a longitudinal cohort of youth with type 1 diabetes...
May 29, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28554168/juan-jorge-heinrich-md-phd-1937-2016
#6
Ignacio Bergadá, Ana Keselman, Rodolfo Rey
No abstract text is available yet for this article.
May 29, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28528334/hormones-and-human-and-nonhuman-primate-growth
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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