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Hormone Research in Pædiatrics

Paul Saenger, Luis Zamora-Siliezar
No abstract text is available yet for this article.
July 12, 2018: Hormone Research in Pædiatrics
Christian L Roth, Julia von Schnurbein, Clinton Elfers, Anja Moss, Martin Wabitsch
BACKGROUND: We tested whether leptin treatment affects secretion of satiety-related gut peptides and brain-derived neurotrophic factor (BDNF), which is a regulator of energy homeostasis downstream of hypothalamic leptin signaling. METHODS: We report the case of a morbidly obese 14.7-year-old girl with a novel previously reported homozygous leptin gene mutation, in whom hormone secretion was evaluated in 30-min intervals for 10 h (07.30-17.30) to assess BDNF, insulin, glucagon-like peptide-1 (GLP-1), ghrelin, and peptide YY (PYY) secretion before as well as 11 and 46 weeks after start of metreleptin treatment...
July 11, 2018: Hormone Research in Pædiatrics
Saila Laakso, Heli Viljakainen, Marita Lipsanen-Nyman, Ursula Turpeinen, Kaisa K Ivaska, Ravinder Anand-Ivell, Richard Ivell, Outi Mäkitie
BACKGROUND: Previous studies suggest increased risk for hypoandrogenism and fractures in men with obesity. We aimed to describe the effects of severe childhood-onset obesity on the cross talk between metabolic state, testes, and skeleton at late puberty. METHODS: A cohort of adolescent and young adult males with severe childhood-onset obesity (n = 21, mean age 18.5 years) and an age-matched control group were assessed for testicular hormones and X-ray absorptiometry-derived bone mass...
June 29, 2018: Hormone Research in Pædiatrics
Anni Larnkjær, Ken K Ong, Emma M Carlsen, Katrine T Ejlerskov, Christian Mølgaard, Kim F Michaelsen
BACKGROUND/AIMS: Exposure to obesity during pregnancy may lead to adverse changes in the offspring's metabolic profile. We compared appetite- and growth-related hormones in a cohort of infants born to obese mothers (SKOT-II) with infants born mainly to nonobese mothers (SKOT-I). METHODS: Infants from SKOT-I (n = 273) and SKOT-II (n = 132) were examined including anthropometric measurements and blood samples analyzed for glucose, insulin, insulin-like growth factor-I (IGF-I), adiponectin, and leptin...
June 29, 2018: Hormone Research in Pædiatrics
Kriti Joshi, Adrian F Daly, Albert Beckers, Margaret Zacharin
BACKGROUND: Somatotropinomas are rare in childhood and frequently associated with genetic mutations. AIP mutations are found in 20-25% cases and cause aggressive somatotropinomas, often resistant to somatostatin analogues. AIMS: To assess responses to multimodal therapy including pegvisomant in 2 children with sporadic somatotropinomas due to AIP mutations. CASE DESCRIPTION: We report 2 children, a boy aged 13 and a girl aged 10, with rapid growth, visual impairment, and growth hormone hypersecretion...
June 28, 2018: Hormone Research in Pædiatrics
Francis de Zegher, Nele Reynaert, Lien De Somer, Carine Wouters, Mathieu Roelants
BACKGROUND: Biologicals targeting the interleukin (IL)-1β or IL-6 pathway are becoming prime choices for the treatment of children with systemic juvenile idiopathic arthritis (sJIA). Up to 1 in 3 sJIA children receiving such treatment continues to have inflammatory activity and to require supra-physiological glucocorticoid doses which may reduce growth velocity for years and may lead to an extremely short stature for age, if not for life. Currently, there is no long-term proposal to normalize the adult height of these children with sJIA...
June 25, 2018: Hormone Research in Pædiatrics
Jin Soon Hwang, Hae Sang Lee, Kee-Hyoung Lee, Han-Wook Yoo, Dae-Yeol Lee, Byung-Kyu Suh, Cheol Woo Ko, Woo Yeong Chung, Dong-Kyu Jin, Choong Ho Shin, Heon-Seok Han, Song Han, Ho-Seong Kim
BACKGROUND/AIMS: To determine the optimal dose of LB03002, a sustained-release, once-weekly formulation of recombinant human growth hormone (rhGH), and to compare its efficacy and safety with daily rhGH in children with idiopathic short stature (ISS). METHODS: This multicenter, randomized, open-label, phase II study included GH-naïve, prepubertal children with ISS, randomized to receive daily rhGH 0.37 mg/kg/week (control, n = 16), LB03002 0.5 mg/kg/week (n = 14), or LB03002 0...
June 20, 2018: Hormone Research in Pædiatrics
Lyne N Chiniara, Herbert J Bonifacio, Mark R Palmert
BACKGROUND/AIMS: To examine characteristics, including mental health comorbidities, among adolescents presenting to a transgender clinic and to compare these data to previous reports. METHODS: Retrospective chart review among youth seen at The Hospital for Sick Children between January 2014 and June 2016. Demographic data, clinical characteristics, and mental health comorbidities were assessed. Baseline and repeat blood work were also examined. RESULTS: Charts from 203 adolescents aged 12-18 years were reviewed (156 assigned female at birth [AFAB] (77%) aged 16...
June 19, 2018: Hormone Research in Pædiatrics
Constanza Navarro Moreno, Amaury Delestienne, Etienne Marbaix, Selda Aydin, Konstanze Hörtnagel, Sarah Lechner, Yves Sznajer, Véronique Beauloye, Dominique Maiter, Philippe A Lysy
Cushing syndrome (CS) is a rare disease in children, frequently associated with subtle or periodic symptoms that may delay its diagnosis. Weight gain and growth failure, the hallmarks of hypercortisolism in pediatrics, may be inconsistent, especially in ACTH-independent forms of CS. Primary pigmented nodular adrenocortical disease (PPNAD) is the rarest form of ACTH-independent CS, and can be associated with endocrine and nonendocrine tumors, forming the Carney complex (CNC). Recently, phenotype/genotype correlations have been described with particular forms of CNC where PPNAD is isolated or associated only with skin lesions...
June 15, 2018: Hormone Research in Pædiatrics
Christopher E Gibson, Kara E Boodhansingh, Changhong Li, Laura Conlin, Pan Chen, Susan A Becker, Tricia Bhatti, Vaneeta Bamba, N Scott Adzick, Diva D De Leon, Arupa Ganguly, Charles A Stanley
BACKGROUND: Previous case reports have suggested a possible association of congenital hyperinsulinism with Turner syndrome. OBJECTIVE: We examined the clinical and molecular features in girls with both congenital hyperinsulinism and Turner syndrome seen at The Children's Hospital of Philadelphia (CHOP) between 1974 and 2017. METHODS: Records of girls with hyperinsulinism and Turner syndrome were reviewed. Insulin secretion was studied in pancreatic islets and in mouse islets treated with an inhibitor of KDM6A, an X chromosome gene associated with hyperinsulinism in Kabuki syndrome...
June 14, 2018: Hormone Research in Pædiatrics
Lauren K Wood Heickman, Ladan Davallow Ghajar, Mark Conaway, Alan D Rogol
BACKGROUND/AIMS: A meta-analysis was performed to determine the likelihood of hypothalamic-pituitary-adrenal (HPA) axis suppression following short-term cutaneous treatment of atopic dermatitis with topical corticosteroids (TCS) in pediatric patients. METHODS: All published pediatric clinical trials evaluating TCS use with pre- and post-treatment HPA axis assessment by cosyntropin stimulation testing were included. RESULTS: Of 128 eligible trials, 12 were selected for meta-analysis with a total of 522 participants...
June 13, 2018: Hormone Research in Pædiatrics
Rajdee de Randamie, Gabriel Ángel Martos-Moreno, César Lumbreras, Maria Chueca, Sergio Donnay, Manuel Luque, Rita María Regojo, Marta Mendiola, David Hardisson, Jesús Argente, José C Moreno
BACKGROUND/AIMS: A genomic HABP2 variant was proposed to be responsible for familial nonmedullary thyroid carcinoma (FNMTC). However, its involvement has been questioned in subsequent studies. We aimed to identify genetic HABP2 mutations in a series of FNMTC patients and investigate their involvement in the disease. METHODS: HABP2 was sequenced from 6 index patients. Presence of the variants was investigated in all members of one family. Somatic BRAF and RAS "hotspot" mutations were investigated by the IdyllaTM BRAF Mutation Test and/or Sanger sequencing...
June 12, 2018: Hormone Research in Pædiatrics
Brittany K Wise-Oringer, Marina Goldis, Molly O Regelmann, Michelle Klein, Josef Machac, Henrietta Kotlus Rosenberg, Robert Rapaport
BACKGROUND: Papillary thyroid cancer (PTC) is an uncommon pediatric disease with an excellent prognosis. In follow-up surveillance, neck ultrasound (US), basal and thyroid-stimulating hormone-stimulated serum thyroglobulin (Tg) levels, and diagnostic whole-body radioactive iodine scans (DxWBS) have been traditionally used in both adults and children for the detection of recurrence or metastases of PTC. METHODS: Two pediatric patients with metastatic PTC were followed after standard ablative treatment with routine neck US and serum Tg levels, as well as periodic DxWBS...
May 25, 2018: Hormone Research in Pædiatrics
Nidhi Gupta, Michael Rivera, Paul Novotny, Vilmarie Rodriguez, Irina Bancos, Aida Lteif
BACKGROUND/AIMS: Adrenocortical carcinoma (ACC) is an aggressive childhood cancer. Limited evidence exists on a definite histopathological criterion to differentiate ACC from adrenocortical adenoma. The aim of this study was to investigate the clinicopathological data of children with ACC, identify prognostic factors, and validate a histopathological criterion to differentiate ACC from adrenocortical adenoma. METHODS: This retrospective cohort included 41 children, followed at the Mayo Clinic from 1950 to 2017 (onset of symptoms ≤21 years)...
May 25, 2018: Hormone Research in Pædiatrics
Macy T Hardley, Andrew H Chon, Jorge Mestman, Caroline T Nguyen, Mitchell E Geffner, Ramen H Chmait
BACKGROUND: Iodine is necessary for fetal thyroid development. Excess maternal intake of iodine can cause fetal hypothyroidism due to the inability to escape from the Wolff-Chaikoff effect in utero. CASE REPORT: We report a case of fetal hypothyroid goiter secondary to inadvertent excess maternal iodine ingestion from infertility supplements. The fetus was successfully treated with intra-amniotic levothyroxine injections. Serial fetal blood sampling confirmed fetal escape from the Wolff-Chaikoff effect in the mid third trimester...
May 23, 2018: Hormone Research in Pædiatrics
Anna Kasielska-Trojan, Marian Danilewicz, Bogusław Antoszewski
BACKGROUND/AIMS: The aim of this research was to analyze digit ratio in relation to estrogen receptor (ER) and progesterone receptor (PR) expression and to verify digit ratio (2D: 4D) as a marker of ER and PR overexpression in the male breast. METHODS: This study included 35 patients who underwent breast reduction due to the idiopathic form of gynecomastia. The average age of the studied individuals was 25.7 years (SD = 7.8). ER and PR expression was detected in breasts, and digit ratios were calculated in patients with idiopathic gynecomastia...
May 17, 2018: Hormone Research in Pædiatrics
Ryan A Heksch, Rohan K Henry
BACKGROUND: Hashimoto thyroiditis (HT) is uncommon in infancy, and myxedema coma (MC) is even less common. While prior reports have documented these entities separately, to our knowledge, MC in combination with HT has not been reported before in this age group. METHODS/RESULTS: A 10-month-old female presented with ptosis, lethargy, dysphagia, and failure to thrive (FTT). She developed hypotension, bradycardia, hypothermia, and apnea requiring intubation. Initial thyroid-stimulating hormone was 422 μIU/mL, and free thyroxine was < 0...
May 4, 2018: Hormone Research in Pædiatrics
Thomas Reinehr, Juliane Rothermel, Andreas Wegener-Panzer, Michaela F Hartmann, Stefan A Wudy, Paul-Martin Holterhus
We present a boy with a genetically proven congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. While massively elevated 17-hydroxyprogesterone (17-OHP) concentrations after birth led to the diagnosis, 17-OHP concentrations became immeasurable starting with the second year of life even though the dose of hydrocortisone was continuously decreased to ∼7 mg/m2/day. Furthermore, 17-OHP levels were immeasurable during the ACTH test and after withdrawing hydrocortisone medication. In contrast, ACTH levels increased after cessation of hydrocortisone treatment suggesting complete primary adrenal cortex failure...
April 25, 2018: Hormone Research in Pædiatrics
Philippe Klee, Mirjam Dirlewanger, Vanessa Lavallard, Valerie A McLin, Anne Mornand, Nadine Pernin, Laetitia-Marie Petit, Paola M Soccal, Barbara E Wildhaber, Urs Zumsteg, Jean-Louis Blouin, Thierry Berney, Valerie M Schwitzgebel
BACKGROUND: Cystic fibrosis-related diabetes (CFRD) is the most frequent extrapulmonary complication of cystic fibrosis (CF). METHODS: We report the first combined pancreatic islet-lung-liver transplantation in a 14-year-old adolescent. CFTR was analyzed by Sanger sequencing. Further genes were analyzed by high-throughput sequencing. RESULTS: The patient was diagnosed with CF at the age of 14 months. Nine years later, after diagnosis of CFRD, the patient's BMI and lung function began to decline...
April 18, 2018: Hormone Research in Pædiatrics
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2018: Hormone Research in Pædiatrics
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