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Pediatric endocrine

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https://www.readbyqxmd.com/read/28820733/pubertal-assessment-targeted-educational-intervention-for-pediatric-trainees
#1
Aditi Khokhar, Sairaman Nagarajan, Yagnaram Ravichandran, Sheila Perez-Colon
Background Timely and periodic pubertal assessment in children is vital to identify puberty related disorders. Pediatricians need to have working knowledge of puberty time and tempo. Pediatric residency is an important platform to acquire physical examination skills including pubertal assessment. Objective An educational intervention for teaching pubertal assessment was piloted on pediatric residents at our institution. Methods The intervention comprised of interactive lecture series, ID badge size Tanner stage cards and Tanner posters placed in residents' continuity clinics...
August 18, 2017: International Journal of Adolescent Medicine and Health
https://www.readbyqxmd.com/read/28757413/restoration-of-mir-30a-expression-inhibits-growth-tumorigenicity-of-medulloblastoma-cells-accompanied-by-autophagy-inhibition
#2
Satishkumar Vishram Singh, Aditi Nigam Dakhole, Akash Deogharkar, Sadaf Kazi, Rohan Kshirsagar, Atul Goel, Aliasgar Moiyadi, Rakesh Jalali, Epari Sridhar, Tejpal Gupta, Prakash Shetty, Nikhil Gadewal, Neelam Vishwanath Shirsat
Medulloblastoma is a highly malignant pediatric brain tumor. About 30% patients have metastasis at diagnosis and respond poorly to treatment. Those that survive, suffer long term neurocognitive, endocrine and developmental defects due to the cytotoxic treatment to developing child brain. It is therefore necessary to develop targeted treatment strategies based on underlying biology for effective treatment of medulloblastoma with minimal side effects. Medulloblastomas are believed to be the result of deregulated nervous system development as evident from the role of WNT and SHH developmental signaling pathways in pathogenesis of medulloblastomas...
July 27, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28755589/children-s-environmental-health-based-on-birth-cohort-studies-of-asia
#3
Meng-Shan Tsai, Mei-Huei Chen, Ching-Chun Lin, Sharon Ng, Chia-Jung Hsieh, Chen-Yu Liu, Wu-Shiun Hsieh, Pau-Chung Chen
Numerous studies have explored the associations between environmental pollutants and pediatric health. Recent studies have investigated the issue in Asia, but no systematic review has been published to date. This study aims to elucidate the issue by summarizing relevant epidemiologic evidence for cohorts in Asia, using information from the Birth Cohort Consortium of Asia (BiCCA). Environmental pollutants include mercury, environmental tobacco smoke (ETS), polychlorinated biphenyls (PCB), perfluoroalkyl substances (PFAS) and phthalates...
July 26, 2017: Science of the Total Environment
https://www.readbyqxmd.com/read/28752085/review-of-pediatric-pheochromocytoma-and-paraganglioma
#4
REVIEW
Reshma Bholah, Timothy Edward Bunchman
Pheochromocytoma (PCC) and paraganglioma (PGL) are rare chromaffin cell tumors which secrete catecholamines and form part of the family of neuroendocrine tumors. Although a rare cause of secondary hypertension in pediatrics, the presentation of hypertension in these patients is characteristic, and treatment is definitive. The gold standard for diagnosis is via measurement of plasma free metanephrines, with imaging studies performed for localization, identification of metastatic lesions and for surgical resection...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28729012/evidence-that-children-born-at-early-term-37-38-6-7-weeks-are-at-increased-risk-for-diabetes-and-obesity-related-disorders
#5
Dorit Paz Levy, Eyal Sheiner, Tamar Wainstock, Ruslan Sergienko, Daniella Landau, Asnat Walfisch
BACKGROUND: Prematurity is known to be associated with high rates of endocrine and metabolic complications in the offspring. Offspring born early term (37-38 6/7 weeks' gestation) were also shown to exhibit long-term morbidity resembling that of late preterm, in several health categories. OBJECTIVE: We aimed to determine whether early term delivery impacts on the long-term endocrine and metabolic health of the offspring. STUDY DESIGN: A population-based cohort analysis was performed, including all term singleton deliveries occurring from 1991 through 2013 at a single regional tertiary medical center...
July 18, 2017: American Journal of Obstetrics and Gynecology
https://www.readbyqxmd.com/read/28723610/pediatric-reference-intervals-for-29-ortho-vitros-5600-immunoassays-using-the-caliper-cohort-of-healthy-children-and-adolescents
#6
Victoria Higgins, Angela W S Fung, Man Khun Chan, Joseph Macri, Khosrow Adeli
BACKGROUND: Accurate reference intervals (RIs) based on a healthy pediatric population are essential for pediatric test result interpretation. The CALIPER project has recruited a large healthy cohort and completed a series of a priori studies to address gaps in pediatric RIs. As immunoassays from different manufacturers for endocrine and special chemistry markers are not standardized and show marked intermethod differences, direct RI studies are needed for each major analytical platform...
July 19, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28720594/impact-of-transition-on-quality-of-life-in-patients-with-congenital-adrenal-hyperplasia-diagnosed-during-childhood
#7
Anne Bachelot, Magaly Vialon, Amandine Baptiste, Isabelle Tejedor, Caroline Elie, Michel Polak, Philippe Touraine
BACKGROUND: Health-related quality of life (QoL) in adult patients with congenital adrenal hyperplasia (CAH) has been variously reported. However, there is no study evaluating the impact of transition on quality of life. METHODS: Adult patients with classic or non-classic CAH diagnosed during childhood CAH, born between 1970 and 1990, were recruited from the registers of Pediatric departments belonging to the French reference center for endocrine rare disease. Primary end point was the quality of life (WHOQoL -BREF)...
July 18, 2017: Endocrine Connections
https://www.readbyqxmd.com/read/28719390/total-pancreatectomy-with-islet-autotransplantation-recent-updates-and-outcomes
#8
Yoshitaro Shindo, Mazhar A Kanak
PURPOSE OF REVIEW: Total pancreatectomy with islet autotransplantation (TPIAT) is a reliable therapy to retain endocrine function, to alleviate pain and improve quality of life in adult and pediatric patients suffering from refractory chronic pancreatitis and recurrent acute pancreatitis. Recently, an expansion of indications to include benign and malignant pancreatic disease has been suggested. Improved methods for evaluating the functional quality of islets and predicting transplant outcome have been discussed...
July 14, 2017: Current Opinion in Organ Transplantation
https://www.readbyqxmd.com/read/28705803/clinical-practice-guidelines-for-the-care-of-girls-and-women-with-turner-syndrome-proceedings-from-the-2016-cincinnati-international-turner-syndrome-meeting
#9
Claus H Gravholt, Niels H Andersen, Gerard S Conway, Olaf M Dekkers, Mitchell E Geffner, Karen O Klein, Angela E Lin, Nelly Mauras, Charmian A Quigley, Karen Rubin, David E Sandberg, Theo C J Sas, Michael Silberbach, Viveca Söderström-Anttila, Kirstine Stochholm, Janielle A van Alfen-van derVelden, Joachim Woelfle, Philippe F Backeljauw
Turner syndrome affects 25-50 per 100,000 females and can involve multiple organs through all stages of life, necessitating multidisciplinary approach to care. Previous guidelines have highlighted this, but numerous important advances have been noted recently. These advances cover all specialty fields involved in the care of girls and women with TS. This paper is based on an international effort that started with exploratory meetings in 2014 in both Europe and the USA, and culminated with a Consensus Meeting held in Cincinnati, Ohio, USA in July 2016...
September 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28693116/long-term-follow-up-of-a-child-with-klinefelter-syndrome-and-achondroplasia-from-infancy-to-16-years
#10
Jessica D Arditi, Loretta Thomaidis, Helen Frysira, Artemis Doulgeraki, George P Chrousos, Christina Kanaka-Gantenbein
BACKGROUND: Achondroplasia (ACH), an autosomal dominant skeletal dysplasia, occurs in approximately 1:20,000 births. On the other hand, 47,XXY aneuploidy (Klinefelter syndrome [KS]) is the most common sex chromosome disorder, with a prevalence of approximately 1:600 males. To the best of our knowledge, only five cases of patients presenting both ACH and KS have been reported to date in the international literature. However, none of these cases has been longitudinally followed during the entire childhood...
June 23, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28690986/next-generation-sequencing-and-array-based-comparative-genomic-hybridization-for-molecular-diagnosis-of-pediatric-endocrine-disorders
#11
REVIEW
Maki Fukami, Mami Miyado
Next-generation sequencing (NGS) and array-based comparative genomic hybridization (array CGH) have enabled us to perform high-throughput mutation screening and genome-wide copy number analysis, respectively. These methods can be used for molecular diagnosis of pediatric endocrine disorders. NGS has determined the frequency and phenotypic variation of mutations in several disease-associated genes. Furthermore, whole exome analysis using NGS has successfully identified several novel causative genes for endocrine disorders...
June 2017: Annals of Pediatric Endocrinology & Metabolism
https://www.readbyqxmd.com/read/28674121/multiple-endocrine-neoplasia-and-hyperparathyroid-jaw-tumor-syndromes-clinical-features-genetics-and-surveillance-recommendations-in-childhood
#12
REVIEW
Jonathan D Wasserman, Gail E Tomlinson, Harriet Druker, Junne Kamihara, Wendy K Kohlmann, Christian P Kratz, Katherine L Nathanson, Kristian W Pajtler, Andreu Parareda, Surya P Rednam, Lisa J States, Anita Villani, Michael F Walsh, Kristin Zelley, Joshua D Schiffman
Children and adolescents who present with neuroendocrine tumors are at extremely high likelihood of having an underlying germline predisposition for the multiple endocrine neoplasia (MEN) syndromes, including MEN1, MEN2A and MEN2B, MEN4, and hyperparathyroid-jaw tumor (HPT-JT) syndromes. Each of these autosomal dominant syndromes results from a specific germline mutation in unique genes: MEN1 is due to pathogenic MEN1 variants (11q13), MEN2A and MEN2B are due to pathogenic RET variants (10q11.21), MEN4 is due to pathogenic CDKN1B variants (12p13...
July 1, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28672740/long-term-follow-up-of-a-child-with-klinefelter-syndrome-and-achondroplasia-from-infancy-to-16-years
#13
Jessica D Arditi, Loretta Thomaidis, Helen Frysira, Artemis Doulgeraki, George P Chrousos, Christina Kanaka-Gantenbein
BACKGROUND: Achondroplasia (ACH), an autosomal dominant skeletal dysplasia, occurs in approximately 1:20,000 births. On the other hand, 47,XXY aneuploidy (Klinefelter syndrome [KS]) is the most common sex chromosome disorder, with a prevalence of approximately 1:600 males. To the best of our knowledge, only five cases of patients presenting both ACH and KS have been reported to date in the international literature. However, none of these cases has been longitudinally followed during the entire childhood...
July 26, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28658184/improving-compliance-in-obtaining-daily-weights-in-a-large-academic-children-s-hospital
#14
Jacqueline E Crawford, Colleen Garvin Coyne, Katherine Calder
Noncompliance in obtaining daily weights leads to delays in establishing treatment and discharge plans in pediatric populations. An inpatient gastrointestinal and endocrine nursing unit aimed to increase compliance of obtaining daily weights from baseline of 38% to 80%. The time daily weights were obtained was changed from 8 AM to an interval of time 4 PM to 6 PM. The compliance rate increased to 96% and has been sustained at an average of 94% over 24 months. This report describes a low-risk intervention that took minimal effort to implement but yielded high results to exceed the goal...
June 23, 2017: Journal of Nursing Care Quality
https://www.readbyqxmd.com/read/28613046/anti-mullerian-hormone-amh-determinations-in-the-pediatric-and-adolescent-endocrine-practice
#15
Amir Weintraub, Talia Eldar-Geva
Anti-Mullerian hormone (AMH), secreted by immature testicular Sertoli-cells, triggers the regression of male fetal Mullerian ducts. During puberty, AMH is downregulated by intratesticular testosterone. In females, AMH is secreted from granulosa cells of immature ovarian follicles from late prenatal life until menopause; serum concentration is 5-20 times lower in females than in males through lifetime. In boys, AMH determination is useful in the clinical setting as a marker of Sertoli cell function. Serum AMH is low in infants with hypogonadotrophic hypogonadism (and increases with FSH treatment), in patients with primary hypogonadism from early postnatal life and in Klinefelter syndrome from midpuberty...
June 2017: Pediatric Endocrinology Reviews: PER
https://www.readbyqxmd.com/read/28602495/effects-of-socioeconomic-status-on-children-with-well-differentiated-thyroid-cancer
#16
Evan F Garner, Ilan I Maizlin, Matthew B Dellinger, Kenneth W Gow, Melanie Goldfarb, Adam B Goldin, John J Doski, Monica Langer, Jed G Nuchtern, Sanjeev A Vasudevan, Mehul V Raval, Elizabeth A Beierle
BACKGROUND: Well-differentiated thyroid cancer is the most common endocrine malignancy in children. Adult literature has demonstrated socioeconomic disparities in patients undergoing thyroidectomy, but the effects of socioeconomic status on the management of pediatric well-differentiated thyroid cancer remains poorly understood. METHODS: Patients ≤21 years of age with well-differentiated thyroid cancer remains were reviewed from the National Cancer Data Base...
September 2017: Surgery
https://www.readbyqxmd.com/read/28586453/response-to-letter-pediatric-obesity-assessment-treatment-and-prevention-an-endocrine-society-clinical-practice-guideline
#17
Dennis M Styne, Silva A Arslanian, Ellen L Connor, Ismaa Sadaf Farooqi, M Hassan Murad, Janet H Silverstein, Jack A Yanovski
No abstract text is available yet for this article.
June 1, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28586452/letter-to-the-editor-pediatric-obesity-assessment-treatment-and-prevention-an-endocrine-society-clinical-practice-guideline
#18
Geoff D C Ball, Arnaldo Perez, James D Nobles, Nicholas D Spence, Joseph A Skelton
No abstract text is available yet for this article.
June 1, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28585354/-real-world-pediatric-endocrine-practice-how-much-is-it-influenced-by-physician-s-gender-and-region-of-practice-results-of-an-international-survey
#19
Keren Smuel, Yonatan Yeshayahu
OBJECTIVE: To determine whether hormonal treatments for frequent clinical cases (short stature, delayed and precocious puberty) are prescribed strictly according to clinical guidelines or based on personal tendencies, and whether the decisions correlate with physician's personal demographics (age, sex, and place of practice). METHODS: Cross-sectional survey, with made-up clinical cases, distributed to pediatric endocrinologists using 2 web-based professional forums, Israeli and an international...
June 6, 2017: Journal of Evaluation in Clinical Practice
https://www.readbyqxmd.com/read/28578454/determinants-of-levothyroxine-dose-required-to-achieve-euthyroidism-in-pediatric-population-a-hospital-based-prospective-follow-up-study
#20
Rekha Singh
We analyzed the relation of pretreatment anthropometric measures and serum thyrotropin (TSH) with the levothyroxine requirement. Children (3-18 years) brought to endocrine clinic with newly diagnosed acquired primary hypothyroidism were enrolled consecutively and prospectively with follow-up (July 2014 to April 2016). Children were started on levothyroxine, and dose was adjusted at regular intervals until achieved euthyroidism (serum TSH 0.4 to 4.5 mIU/L). The relationship of age, gender, height, weight, and serum TSH with levothyroxine dose was analyzed by univariate and multivariate regression analysis...
August 2017: European Journal of Pediatrics
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