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Pediatric endocrine

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https://www.readbyqxmd.com/read/27911611/the-influence-of-gh-treatment-on-glucose-homeostasis-in-girls-with-turner-syndrome-a-7-years-study
#1
Federico Baronio, Laura Mazzanti, Ylenia Girtler, Federica Tamburrino, Fiorenzo Lupi, Silvia Longhi, Antonio Fanolla, Giorgio Radetti
CONTEXT: Growth hormone (GH) influences glucose homeostasis mainly by negatively affecting insulin sensitivity. OBJECTIVE: to longitudinally study the insulin sensitivity (HOMA-S), insulin secretion (insulinogenic index - IGI) and capacity of beta cells to adapt to changes in insulin sensitivity (oral disposition index-ODI) in girls affected by Turner syndrome (TS) undergoing GH treatment. DESIGN: a longitudinal retrospective seven years study Setting: a tertiary pediatric endocrine unit and a University pediatric Clinic...
December 2, 2016: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/27903117/risk-factors-associated-with-the-surgical-management-of-craniopharyngiomas-in-pediatric-patients-analysis-of-1961-patients-from-a-national-registry-database
#2
Joshua Bakhsheshian, Diana L Jin, Ki-Eun Chang, Ben A Strickland, Dan A Donoho, Steven Cen, William J Mack, Frank Attenello, Eisha A Christian, Gabriel Zada
OBJECTIVE Patient demographic characteristics, hospital volume, and admission status have been shown to impact surgical outcomes of sellar region tumors in adults; however, the data available following the resection of craniopharyngiomas in the pediatric population remain limited. The authors sought to identify potential risk factors associated with outcomes following surgical management of pediatric craniopharyngiomas. METHODS The Nationwide Inpatient Sample database and Kids' Inpatient Database were analyzed to include admissions for pediatric patients (≤ 18 years) who underwent a transcranial or transsphenoidal craniotomy for resection of a craniopharyngioma...
December 2016: Neurosurgical Focus
https://www.readbyqxmd.com/read/27884013/guidelines-for-growth-hormone-and-insulin-like-growth-factor-i-treatment-in-children-and-adolescents-growth-hormone-deficiency-idiopathic-short-stature-and-primary-insulin-like-growth-factor-i-deficiency
#3
Adda Grimberg, Sara A DiVall, Constantin Polychronakos, David B Allen, Laurie E Cohen, Jose Bernardo Quintos, Wilma C Rossi, Chris Feudtner, Mohammad Hassan Murad
BACKGROUND/AIMS: On behalf of the Drug and Therapeutics, and Ethics Committees of the Pediatric Endocrine Society, we sought to update the guidelines published in 2003 on the use of growth hormone (GH). Because idiopathic short stature (ISS) remains a controversial indication, and diagnostic challenges often blur the distinction between ISS, GH deficiency (GHD), and primary IGF-I deficiency (PIGFD), we focused on these three diagnoses, thereby adding recombinant IGF-I therapy to the GH guidelines for the first time...
November 25, 2016: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/27876089/rapid-onset-obesity-hypoventilation-hypothalamic-dysfunction-autonomic-dysregulation-and-neuroendocrine-tumor-syndrome-with-a-homogenous-enlargement-of-the-pituitary-gland-a-case-report
#4
Lama Aljabban, Lina Kassab, Nour Alhuda Bakoura, Mohammad Fayez Alsalka, Ismaeil Maksoud
BACKGROUND: Rapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation syndrome is a rare pediatric disorder with a variable sequence of clinical presentations, undefined etiology, and high risk of mortality. Our patient presented an unusual course of the disease accompanied by a homogenous mild enlargement of her pituitary gland with an intact pituitary-endocrine axis which, to the best of our knowledge, represents a new finding in rapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation syndrome...
November 22, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27871293/genetic-and-epigenetic-alterations-in-the-gnas-locus-and-clinical-consequences-in-pseudohypoparathyroidism-italian-common-healthcare-pathways-adoption
#5
L de Sanctis, F Giachero, G Mantovani, G Weber, M Salerno, G I Baroncelli, M F Elli, P Matarazzo, M Wasniewska, L Mazzanti, G Scirè, D Tessaris
BACKGROUND: Genetic and epigenetic alterations in the GNAS locus are responsible for the Gsα protein dysfunctions causing Pseudohypoparathyroidism (PHP) type Ia/c and Ib, respectively. For these heterogeneous diseases characterized by multiple hormone resistances and Albright's Hereditary Osteodystrophy (AHO) the current classification results inadequate because of the clinical overlap between molecular subtypes and a standard clinical approach is still missing. In the present paper several members of the Study Group Endocrine diseases due to altered function of Gsα protein of the Italian Society of Pediatric Endocrinology and Diabetology (ISPED) have reviewed and updated the clinical-molecular data of the largest case series of (epi)/genetically characterized AHO/PHP patients; they then produced a common healthcare pathway for patients with these disorders...
November 21, 2016: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/27834132/surgical-management-of-medullary-thyroid-carcinoma-in-pediatric-age
#6
Claudio Spinelli, Leonardo Rossi, Silvia Strambi, Jessica Piscioneri, Rossella Elisei, Maura Massimino, Paolo Miccoli
Medullary thyroid carcinoma (MTC) is a rare, accounting for 5% of thyroid malignancies. It is a neuroendocrine tumor wich origins from thyroid parafollicular cells. It may be sporadic, mostly in adult patients, or inherited as autosomal dominant pattern, mostly in pediatric patients. As familial cancer, MTC may presented isolated as familial medullary thyroid carcinoma (FMTC) - 10% of cases - or, most often, as part of multiple endocrine neoplasm type 2 (MEN 2A or MEN 2B) syndromes - 90% of cases. The therapy for sporadic or hereditary MTC is surgical resection and consists in total thyroidectomy associated with central compartment lymph nodal dissection; the radicality of this intervention is fundamental to obtain a definitive cure...
November 11, 2016: Current Pediatric Reviews
https://www.readbyqxmd.com/read/27819155/drugs-for-the-treatment-of-pediatric-type-2-diabetes-mellitus-and-related-co-morbidities
#7
Ozra Tabatabaei-Malazy, Shekoufeh Nikfar, Bagher Larijani, Mohammad Abdollahi
The continuing global epidemic of obesity in adolescents has raised the prevalence of type 2 diabetes mellitus (T2DM). Despite the wealth of information concerning T2DM in adults, rare data are available targeting treatment of T2DM in pediatric. Areas covered: This article has reviewed clinical practice guidelines, particularly the American Diabetes Association and the Pediatric Endocrine Society consensus, jointly with clinical trial data available in databases with respect to the use of available pharmacological options to treat T2DM and its complications in youth...
November 21, 2016: Expert Opinion on Pharmacotherapy
https://www.readbyqxmd.com/read/27807915/barriers-to-participation-in-industry-sponsored-clinical-trials-in-pediatric-type-2-diabetes
#8
Ryan Farrell, Kathleen Bethin, Georgeanna Klingensmith, William V Tamborlane, Rose Gubitosi-Klug
BACKGROUND: The rapid emergence of type 2 diabetes (T2D) in the pediatric population has left pediatric endocrinologists with limited artillery in terms of management. While multiple medications are available for adults, Food and Drug Administration (FDA)-approved medications in children are limited to only metformin and insulin. Additional treatment options require randomized controlled trials, yet heretofore several barriers at the participant and institutional level have impeded these studies from proceeding in children and adolescents...
November 3, 2016: Pediatric Diabetes
https://www.readbyqxmd.com/read/27804856/a-papillary-thyroid-carcinoma-in-pediatric-age-an-example-of-a-rare-tumour-managed-within-a-cooperative-comprehensive-project
#9
Claudio Spinelli, Maura Massimino, Giovanna Sironi, Andrea Ferrari, Marta Podda, Stefano Chiaravalli
Though rare in childhood, thyroid cancers represent the most frequent tumours of endocrine glands in childhood and adolescence. Papillary thyroid carcinoma is a non-medullary, follicular-derived differentiated tumour of the thyroid. There is still controversy concerning the therapeutic approach of PTCs in the paediatric population; regarding which should be the extent of the surgical approach, and the need for radioactive iodine (RAI) therapy. The cooperative TREP project (Tumori Rari in Età Pediatrica [Rare Tumours in Paediatric Age]) was launched in Italy in 2000 (under the patronage of AIEOP - Associazione Italiana Ematologia Oncologia Pediatrica) with a view to improving both research the clinical management on the less common paediatric cancers...
October 31, 2016: Current Pediatric Reviews
https://www.readbyqxmd.com/read/27786545/vitamin-d-levels-in-children-affected-by-vernal-keratoconjuctivitis
#10
Anna Maria Zicari, Arianna Cafarotti, Francesca Occasi, Valeria Lollobrigida, Marcella Nebbioso, Irene Pecorella, Giovanna De Castro, Alberto Spalice, Lorenzo Loffredo, Maria Pia Villa, Marzia Duse
BACKGROUND: Vernal keratoconjunctivitis (VKC) is a chronic and often severe bilateral conjunctivitis. VKC etiology remains still unclear although endocrine, genetic, neurogenic and environmental factors have been implied. Vitamin D is a fat-soluble prohormone whose main function is the regulation of calcium and phosphate metabolism. The aim of this study was to evaluate serum vitamin D in children affected by VKC compared to the healthy children and investigate the relationship between its levels and disease severity...
October 27, 2016: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/27784201/iodine-supplementation-in-women-during-preconception-pregnancy-and-lactation-current-clinical-practice-by-u-s-obstetricians-and-midwives
#11
Simone De Leo, Elizabeth N Pearce, Lewis E Braverman
BACKGROUND: Iodine deficiency is a major public health problem throughout the world, especially for pregnant women, and it is considered the most common cause of preventable intellectual impairment. In the United States, iodine status in pregnant women is considered mildly deficient. Therefore, the Endocrine Society, the American Thyroid Association, the Teratology Society, and the American Academy of Pediatrics recommend that women receive prenatal vitamins containing 150 µg of iodine daily during preconception, pregnancy, and lactation...
October 26, 2016: Thyroid: Official Journal of the American Thyroid Association
https://www.readbyqxmd.com/read/27777794/molecular-analysis-of-cyp21a2-gene-mutations-among-iraqi-patients-with-congenital-adrenal-hyperplasia
#12
Ruqayah G Y Al-Obaidi, Bassam M S Al-Musawi, Munib Ahmed K Al-Zubaidi, Christian Oberkanins, Stefan Németh, Yusra G Y Al-Obaidi
Congenital adrenal hyperplasia is a group of autosomal recessive disorders. The most frequent one is 21-hydroxylase deficiency. Analyzing CYP21A2 gene mutations was so far not reported in Iraq. This work aims to analyze the spectrum and frequency of CYP21A2 mutations among Iraqi CAH patients. Sixty-two children were recruited from the Pediatric Endocrine Consultation Clinic, Children Welfare Teaching Hospital, Baghdad, Iraq, from September 2014 till June 2015. Their ages ranged between one day and 15 years...
2016: Enzyme Research
https://www.readbyqxmd.com/read/27775306/-endocrine-disrupting-chemicals-exposure-and-other-parental-factors-in-hypospadias-and-cryptorchidism-etiology
#13
B Estors Sastre, P Bragagnini Rodríguez, R Fernández Atuan, R Delgado Alvira, M A Rihuete Heras, J Gracia Romero
AIM OF THE STUDY: To investigate the association between endocrine disrupting chemicals (EDC) exposure and other paternal factors in the etiology of hipospadias and cryptorchidism. METHODS: A case-control study. Cases were infants between 0 and 6 years of age diagnosed with hypospadias or cryptorchidism in our pediatric urology and general pediatric surgery services during a period of 6 months, and controls were infants with the same range of age attending the same services without any urological problem...
July 20, 2015: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/27761562/diverse-etiology-of-hyperlipidemia-among-hospitalized-children-in-western-region-of-saudi-arabia
#14
Abdulmoein E Al-Agha, Abrar M Alnawab, Tala M Hejazi
To determine the various etiologies of primary and secondary hyperlipidemia among children visiting the pediatric endocrine clinic. Methods: This is a retrospective, cross-sectional, cohort study conducted at King Abdulaziz University Hospital (KAUH), Jeddah, Kingdom of Saudi Arabia from January 2010 to 2015 that included 253 children aged from birth to 12 years old. Data were obtained by reviewing medical reports of patients who presented with hyperlipidemia to the clinic, and their laboratory investigation results using KAUH electronic "Phoenix" system...
November 2016: Saudi Medical Journal
https://www.readbyqxmd.com/read/27752263/the-prevalence-of-metabolic-syndrome-and-cardiovascular-risk-factors-in-obese-children-and-adolescents-in-dalmatia-a-hospital-based-study
#15
Marko Šimunović, Joško Božić, Lukrecija Milić, Ivana Unić, Veselin Škrabić
Obesity and metabolic syndrome (MS) are one of the biggest public health issues in child and adolescent population. To the best of the authors' knowledge, this hospital based study is the first report on the prevalence of MS in obese children and adolescents in Dalmatia, the Mediterranean part of Croatia. The objectives of this study were to determine the prevalence of individual cardiovascular risk factors and MS. Between January 2009 and June 2014, 201 obese subjects aged 6 to 18 were analyzed retrospectively from our Pediatric Endocrine Unit database...
2016: International Journal of Endocrinology
https://www.readbyqxmd.com/read/27741188/disorders-of-sexual-development-in-adult-women
#16
Veronica Gomez-Lobo, Anne-Marie Amies Oelschlager
Disorders (differences) of sexual development encompass a variety of conditions with atypical development of chromosomal, gonadal, or anatomic sex. Three of the most common differences of sex development conditions include congenital adrenal hyperplasia, complete androgen insensitivity, and Turner syndrome. Obstetrician-gynecologists who care for affected individuals in their practice must be familiar with the genetic, endocrine, and anatomic considerations of the most common conditions to provide optimal care...
November 2016: Obstetrics and Gynecology
https://www.readbyqxmd.com/read/27737391/development-of-height-and-body-mass-index-after-pediatric-kidney-transplantation-experience-of-the-nephrology-pediatric-service-at-hcfmrp-usp-2005-2014
#17
Elaine Hillesheim, Valéria Laguna Salomão Ambrósio, Inalda Facincani
Introduction: Chronic kidney disease in children often determines poor nutritional status. Although renal transplantation (RTx) resolves endocrine and metabolic disorders, growth continues to be suboptimal and excessive weight gain may result in obesity. Objectives: Evaluating the development of height and body mass index in renal transplanted children and adolescents and identifying associated factors with final nutritional status. Methods: We reviewed the medical records of 17 patients with regular follow-ups up to 24 months after RTx...
July 2016: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/27734690/-thyroid-cancer-in-children-and-adolescents-and-its-molecular-genetic-background
#18
Běla Bendlová, Vlasta Sýkorová, Eliška Václavíková, Josef Včelák, Rami Katra, Pavla Sýkorová, Petr Vlček, Šárka Dvořáková
Thyroid cancer is the main endocrine malignancy. Its incidence is steadily growing and what is alarming is its increase in children and adolescent population. Pediatric thyroid carcinomas differ from the adult ones in phenotype as well as in genetics. These carcinomas tend to be clinically more aggressive, with more frequent local and distant metastases. However, their long-term prognosis is better in comparison with the adult thyroid cancers. Due to the rarity of the disease, there is lack of data on genetic changes in this age group...
2016: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/27710244/the-diagnosis-and-management-of-lipodystrophy-syndromes-a-multi-society-practice-guideline
#19
Rebecca J Brown, David Araujo-Vilar, Pik To Cheung, David Dunger, Abhimanyu Garg, Michelle Jack, Lucy Mungai, Elif A Oral, Nivedita Patni, Kristina Rother, Julia von Schnurbein, Ekaterina Sorkina, Takara Stanley, Corinne Vigouroux, Martin Wabitsch, Rachel Williams, Tohru Yorifuji
OBJECTIVE: Lipodystrophy syndromes are extremely rare disorders of deficient body fat associated with potentially serious metabolic complications, including diabetes, hypertriglyceridemia, and steatohepatitis. Due to their rarity, most clinicians are not familiar with their diagnosis and management. This practice guideline summarizes diagnosis and management of lipodystrophy syndromes not associated with HIV or injectable drugs. PARTICIPANTS: Seventeen participants were nominated by worldwide endocrine societies or selected by the committee as content experts...
October 6, 2016: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/27707443/growing-up-with-type-1-narcolepsy-its-anthropometric-and-endocrine-features
#20
Virginia Ponziani, Monia Gennari, Fabio Pizza, Antonio Balsamo, Filippo Bernardi, Giuseppe Plazzi
STUDY OBJECTIVES: To evaluate the effect of type 1 narcolepsy (NT1) on anthropometric and endocrine features in childhood/adolescence, focusing on patterns and correlates of weight, pubertal development, and growth in treated and untreated patients. METHODS: We collected anthropometric (height, weight, body mass index (BMI) z-scores), pubertal, metabolic, and endocrine data from 72 NT1 patients at diagnosis and all available premorbid anthropometric parameters of patients from their pediatric files (n = 30)...
September 29, 2016: Journal of Clinical Sleep Medicine: JCSM: Official Publication of the American Academy of Sleep Medicine
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