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Paediatric endocrine

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https://www.readbyqxmd.com/read/29769501/thyroid-status-in-children-with-transfusion-dependent-hb-e-%C3%AE-thalassaemia
#1
T Sharmin, A H Mollah, A A Morshed, M K Chowdhury
Despite improved haematological care, multi-endocrine dysfunction is a common complication in thalassemia. Iron overload is thought to be the most likely mechanism in thyroid dysfunction in these patients. Moreover, chronic tissue hypoxia might havedirect toxic effect on thyroid gland resulting in hypothyroidism. This study was designed to evaluate the thyroid status of children with Hb-E β-thalassemia. This cross sectional analytic study was conducted among thepatients with Hb-E β-thalassemia attending both in-patient & out-patient department of Paediatrics, Dhaka Medical College & Hospital, Dhaka, Bangladesh from April 2012 to March 2013...
April 2018: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/29643102/research-priority-setting-in-childhood-chronic-disease-a-systematic-review
#2
Harrison Lindsay Odgers, Allison Tong, Pamela Lopez-Vargas, Andrew Davidson, Adam Jaffe, Anne McKenzie, Ross Pinkerton, Melissa Wake, Peter Richmond, Sally Crowe, Patrina Ha Yuen Caldwell, Sophie Hill, Jennifer Couper, Suzy Haddad, Behrouz Kassai, Jonathan C Craig
OBJECTIVE: To evaluate research priority setting approaches in childhood chronic diseases and to describe the priorities of stakeholders including patients, caregivers/families and health professionals. DESIGN: We conducted a systematic review of MEDLINE, Embase, PsycINFO and CINAHL from inception to 16 October 2016. Studies that elicited stakeholder priorities for paediatric chronic disease research were eligible for inclusion. Data on the prioritisation process were extracted using an appraisal checklist...
April 11, 2018: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/29575874/influence-of-growth-hormone-therapy-on-selected-dental-and-skeletal-system-parameters
#3
Małgorzata Partyka, Renata Chałas, Izabella Dunin-Wilczyńska, Myroslava Drohomyretska, Maria Klatka
INTRODUCTION: Growth hormone deficiency (GHD) is one of the main indications for growth hormone therapy. One characteristic of this disease is bone age delay in relation to the chronological age. Pituitary dysfunction negatively affects the growth and development of the jaws and teeth of the child. The secretion of endocrine glands regulates growth, development, and gender differentiation. It also controls the growth of bones and teeth, regulates metabolism of calcium and phosphate, proteins, lipids and carbohydrates...
March 14, 2018: Annals of Agricultural and Environmental Medicine: AAEM
https://www.readbyqxmd.com/read/29512963/adrenal-insufficiency-steroid-sick-day-rules-and-the-paediatric-endocrine-nurse
#4
Taffy Makaya, Jennifer Gilbert, Fiona Ryan, Wendy Watts
Clinical governance processes are important for improving patient care. Patients with adrenal insufficiency are at significant risk if they have an adrenal crisis and require steroid therapy. Families should receive education on managing illness or stress, that is, steroid sick day rules. Most of this education is delivered by children's nurses. Two local cases of mortality related to adrenal insufficiency were reviewed and a questionnaire audit was undertaken to compare the steroid sick day rules education provided to patients and their families with published standards...
March 7, 2018: Nursing Children and Young People
https://www.readbyqxmd.com/read/29504223/consensus-guidelines-on-the-use-of-bisphosphonate-therapy-in-children-and-adolescents
#5
Peter J Simm, Andrew Biggin, Margaret R Zacharin, Christine P Rodda, Elaine Tham, Aris Siafarikas, Craig Jefferies, Paul L Hofman, Diane E Jensen, Helen Woodhead, Justin Brown, Benjamin J Wheeler, Denise Brookes, Antony Lafferty, Craig F Munns
Bisphosphonate therapy is the mainstay of pharmacological intervention in young people with skeletal fragility. The evidence of its use in a variety of conditions remains limited despite over three decades of clinical experience. On behalf of the Australasian Paediatric Endocrine Group, this evidence-based consensus guideline presents recommendations and discusses the graded evidence (using the GRADE system) for these recommendations. Primary bone fragility disorders such as osteogenesis imperfecta are considered separately from osteoporosis secondary to other clinical conditions (such as cerebral palsy, Duchenne muscular dystrophy)...
March 2018: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/29397377/a-genetic-approach-to-evaluation-of-short-stature-of-undetermined-cause
#6
REVIEW
Philip G Murray, Peter E Clayton, Steven D Chernausek
Short stature is a common presentation to paediatric endocrinologists. After exclusion of major endocrine or systemic disease, most children with short stature are diagnosed based on a description of their growth pattern and the height of their parents (eg, familial short stature). Height is a polygenic trait and genome-wide association studies have identified many of the associated genetic loci. Here we review the application of genetic studies, including copy number variant analysis, targeted gene panels, and whole-exome sequencing in children with idiopathic short stature...
January 31, 2018: Lancet Diabetes & Endocrinology
https://www.readbyqxmd.com/read/29392744/international-survey-on-high-and-low-dose-synacthen-test-and-assessment-of-accuracy-in-preparing-low-dose-synacthen
#7
Alexandra S Cross, E Helen Kemp, Anne White, Leanne Walker, Suzanne Meredith, Pooja Sachdev, Nils P Krone, Richard J Ross, Neil P Wright, Charlotte J Elder
OBJECTIVE: The short synacthen test (SST) is widely used to assess patients for adrenal insufficiency, but the frequency and protocols used across different centres for the low-dose test (LDT) are unknown. This study aimed to survey centres and test the accuracy of ten different synacthen preparation strategies used for the LDT. METHODS: Members of 6 international endocrine societies were surveyed regarding diagnostic tests used for adrenal insufficiency, and in particular the SST...
May 2018: Clinical Endocrinology
https://www.readbyqxmd.com/read/29383523/a-simple-method-for-bone-age-assessment-the-capitohamate-planimetry
#8
Jung-Ah Choi, Young Chul Kim, Seon Jeong Min, Eun Kyung Khil
OBJECTIVES: To determine if the capitohamate (CH) planimetry could be a reliable indicator of bone age, and to compare it with Greulich-Pyle (GP) method. METHODS: This retrospective study included 391 children (age, 1-180 months). Two reviewers manually measured the areas of the capitate and hamate on plain radiographs. CH planimetry was defined as the measurement of the sum of areas of the capitate and hamate. Two reviewers independently applied the CH planimetry and GP methods in 109 children whose heights were at the 50th percentile of the growth chart...
January 30, 2018: European Radiology
https://www.readbyqxmd.com/read/29358036/descriptive-epidemiology-of-metabolic-syndrome-among-obese-adolescent-population
#9
Sharmin Mahbuba, Fauzia Mohsin, Farhana Rahat, Jebun Nahar, Tahmina Begum, Nazmun Nahar
AIMS: The study was done to assess the magnitude of problems of metabolic syndrome among obese adolescents. MATERIALS AND METHOD: It was a cross-sectional study done from January 2013 to June 2014 in paediatric endocrine outpatient department in BIRDEM General Hospital, Dhaka, Bangladesh. Total 172 adolescents having exogenous obesity aged 10-18 years were included. Impaired fasting glucose (IFG), impaired glucose tolerance (IGT) and type 2 diabetes mellitus (DM) were defined as per WHO criteria...
January 4, 2018: Diabetes & Metabolic Syndrome
https://www.readbyqxmd.com/read/29353717/medical-neglect-at-a-tertiary-paediatric-hospital
#10
Julia Parmeter, Dimitra Tzioumi, Susan Woolfenden
Medical neglect is under-researched and the extent of the problem in Australia is unknown. We conducted a review of the referrals for medical neglect to the Child Protection Unit (CPU) at a tertiary children's hospital in Sydney over a 5 years period, from 2011 to 2016, to determine what medical conditions are being referred, the reason for the medical neglect concern and whether cases are managed in line with American Academy of Pediatrics (AAP) guideline on medical neglect. 61 cases of medical neglect were identified, constituting 4...
March 2018: Child Abuse & Neglect
https://www.readbyqxmd.com/read/29302507/blunt-trauma-pancreas-in-children-is-non-operative-management-appropriate-for-all-grades
#11
Ravi Kumar Garg, Jai Kumar Mahajan
Purpose: Blunt trauma of pancreas in children is uncommon and its management varies from observational to early operative intervention. We analysed the feasibility and outcome of non-operative management in all grades of paediatric pancreatic injuries. Methods: A total of 15 patients of pancreatic trauma seen in a Paediatric Surgery Unit were retrospectively analyzed. Results: Age of the patients ranged from 3-11 years (mean, 7.7 years). The mode of injury was local trauma in 9 children...
December 2017: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/29238946/papp-a2-a-new-key-regulator-of-growth
#12
REVIEW
Magdalena Banaszak-Ziemska, Marek Niedziela
Short stature is the main problem that paediatric endocrinologists have to grapple with. Endocrine disorders account for only 5% of patients with short stature, but this is still one of the most common causes of reports to the endocrine clinic and hospitalisation in the endocrine department. A properly functioning growth hormone/insulin-like growth factor (GH/IGF) axis is one of the most important factors in proper growth. A lot of genetic defects in this axis lead to syndromes marked by impaired growth, like Laron syndrome, muta-tions in the STAT5B, insulin-like growth factor 1 (IGF1), and insulin-like growth factor 1 receptor (IGF1R) and mutations in the acid labile subunit (ALS)...
2017: Endokrynologia Polska
https://www.readbyqxmd.com/read/29199112/-management-of-patients-with-major-beta-thalassemia-in-a-paediatric-department-in-the-south-of-tunisia-about-26-cases
#13
I Maaloul, O Laaroussi, I Jedidi, L Sfaihi, S Kmiha, T Kamoun, H Aloulou, M Hachicha
AIM: Our objectives were to assess the management of patients with major thalassemia and identify the various complications and monitoring means. PATIENTS AND METHODS: A retrospective study was conducted on 26 β-thalassemic patients in the department of paediatrics, Hédi Chaker hospital, Sfax, Tunisia during a period of 25 years (from 1 January 1990 to 31 December 2014). RESULTS: The mean age of the beginning of transfusion was 11.5 months...
February 2018: Transfusion Clinique et Biologique: Journal de la Société Française de Transfusion Sanguine
https://www.readbyqxmd.com/read/29156452/an-international-consortium-update-pathophysiology-diagnosis-and-treatment-of-polycystic-ovarian-syndrome-in-adolescence
#14
Lourdes Ibáñez, Sharon E Oberfield, Selma Witchel, Richard J Auchus, R Jeffrey Chang, Ethel Codner, Preeti Dabadghao, Feyza Darendeliler, Nancy Samir Elbarbary, Alessandra Gambineri, Cecilia Garcia Rudaz, Kathleen M Hoeger, Abel López-Bermejo, Ken Ong, Alexia S Peña, Thomas Reinehr, Nicola Santoro, Manuel Tena-Sempere, Rachel Tao, Bulent O Yildiz, Haya Alkhayyat, Asma Deeb, Dipesalema Joel, Reiko Horikawa, Francis de Zegher, Peter A Lee
This paper represents an international collaboration of paediatric endocrine and other societies (listed in the Appendix) under the International Consortium of Paediatric Endocrinology (ICPE) aiming to improve worldwide care of adolescent girls with polycystic ovary syndrome (PCOS)1. The manuscript examines pathophysiology and guidelines for the diagnosis and management of PCOS during adolescence. The complex pathophysiology of PCOS involves the interaction of genetic and epigenetic changes, primary ovarian abnormalities, neuroendocrine alterations, and endocrine and metabolic modifiers such as anti-Müllerian hormone, hyperinsulinemia, insulin resistance, adiposity, and adiponectin levels...
2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/29151082/survey-on-paediatric-differentiated-thyroid-cancer-care-in-europe
#15
Bernadette L Dekker, Kate L Newbold, Dagmar Führer, Steven G Waguespack, Daria Handkiewicz-Junak, Thera P Links
BACKGROUND/AIMS: Thyroid cancer among children is a very rare disease. Although survival is favourable, morbidity caused by the treatment remains considerable, so there is a great need to optimize management by international cooperation. For this reason, the 2016 European Thyroid Association-Cancer Research Network (ETA-CRN) meeting in Copenhagen, Denmark, paid considerable attention to this topic and aimed to give an overview of the care for this paediatric patient group in different European countries...
2018: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/29037775/neuroendocrine-late-effects-after-tailored-photon-radiotherapy-for-children-with-low-grade-gliomas-long-term-correlation-with-tumour-and-treatment-parameters
#16
Deborah Aloi, Liliana Belgioia, Salvina Barra, Flavio Giannelli, Francesca Cavagnetto, Fabio Gallo, Claudia Milanaccio, MariaLuisa Garrè, Sonia Di Profio, Natascia Di Iorgi, Renzo Corvò
PURPOSE: To evaluate neuroendocrine late effects in paediatric patients with low grade glioma (LGG) who underwent radiotherapy. METHODS AND MATERIAL: We performed a retrospective evaluation of 40 children with LGG treated from July 2002 to January 2015 with external radiotherapy. Tumour locations were cerebral hemisphere (n=2); posterior fossa (n=15); hypothalamic-pituitary axis (HPA, n=15); spine (n=5). Three patients presented a diffuse disease. We looked for a correlation between endocrine toxicity and tumour and treatment parameters...
November 2017: Radiotherapy and Oncology: Journal of the European Society for Therapeutic Radiology and Oncology
https://www.readbyqxmd.com/read/28989569/clinical-profile-of-diabetes-at-diagnosis-among-children-and-adolescents-at-an-endocrine-clinic-in-ghana
#17
Emmanuel Ameyaw, Serwah B Asafo-Agyei, Sumithira Thavapalan, Angela C Middlehurst, Graham D Ogle
AIM: To determine the clinical features of diabetes in children and adolescents in Ghana. METHODS: Retrospective review of clinical features of all children and adolescents with new-onset diabetes seen at the paediatric endocrinology clinic of Komfo Anokye Teaching Hospital in Kumasi, from February 2012 to Auguest 2016. RESULTS: One hundred and six subjects presented with diabetes. Ninety (84.9%) were diagnosed by clinical features and family history as type 1, and 16 (15...
September 15, 2017: World Journal of Diabetes
https://www.readbyqxmd.com/read/28982953/neuroimaging-and-endocrine-disorders-in-paediatric-optic-nerve-hypoplasia
#18
Xiaoxiao Qian, Samksha Fouzdar Jain, Linda A Morgan, Travis Kruse, Monina Cabrera, Donny W Suh
PURPOSE: Optic nerve hypoplasia (ONH) is one of the leading causes of blindness among children. The purpose of this retrospective study is to determine the risk factors and association between brain MRI findings, pituitary abnormalities and endocrine disorders with the presence of ONH. METHODS: A retrospective review of patients seen at paediatric ophthalmology clinics from January 2006 to December 2016 at Children's Hospital and Medical Center and the University of Nebraska Medical Center was performed...
October 5, 2017: British Journal of Ophthalmology
https://www.readbyqxmd.com/read/28866630/primary-osteoporosis-in-children
#19
Lay Ong Tan, Soo Yen Lim, Rashida Farhad Vasanwala
Osteoporosis in childhood is uncommon, and it may be secondary to a spectrum of diverse conditions. Idiopathic juvenile osteoporosis is a primary osteoporosis of unknown aetiology present in previously well children and is a diagnosis of exclusion. We describe a 10-year-old prepubertal boy who presented with back pain of 1-week duration. His spinal X-ray showed generalised loss of vertebral body heights in keeping with osteoporosis. Endocrine and haematological work-up were normal. He was treated with vitamin D supplement and intravenous pamidronate...
September 1, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28803524/endocrinopathies-in-paediatric-onset-neuromyelitis-optica-spectrum-disorder-with-aquaporin-4-aqp4-antibody
#20
Yael Hacohen, Silvia Messina, Hoong-Wei Gan, Sukhvir Wright, Saleel Chandratre, Maria Isabel Leite, Penny Fallon, Angela Vincent, Olga Ciccarelli, Evangeline Wassmer, Ming Lim, Jacqueline Palace, Cheryl Hemingway
The involvement of the diencephalic regions in neuromyelitis optica spectrum disorder (NMOSD) may lead to endocrinopathies. In this study, we identified the following endocrinopathies in 60% (15/25) of young people with paediatric-onset aquaporin 4-Antibody (AQP4-Ab) NMOSD: morbid obesity ( n = 8), hyperinsulinaemia ( n = 5), hyperandrogenism ( n = 5), amenorrhoea ( n = 5), hyponatraemia ( n = 4), short stature ( n = 3) and central hypothyroidism ( n = 2) irrespective of hypothalamic lesions...
August 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
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