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https://www.readbyqxmd.com/read/29907114/amelogenesis-imperfecta-therapeutic-strategy-from-primary-to-permanent-dentition-across-case-reports
#1
Steve Toupenay, Benjamin Philippe Fournier, Marie-Cécile Manière, Chantal Ifi-Naulin, Ariane Berdal, Muriel de La Dure-Molla
BACKGROUND: Hereditary enamel defect diseases are regrouped under the name "Amelogenesis Imperfecta" (AIH). Both dentitions are affected. Clinical expression is heterogeneous and varies between patients. Mutations responsible for this multigene disease may alter various genes and the inheritance can be either autosomal dominant or recessive, or X-linked. Until now, no therapeutic consensus has emerged for this rare disease. CASE PRESENTATION: The purpose of this article was to report treatments of AIH patients from childhood to early adulthood...
June 15, 2018: BMC Oral Health
https://www.readbyqxmd.com/read/29885824/myocardial-inflammation-detected-by-cardiac-mri-in-arrhythmogenic-right-ventricular-cardiomyopathy-a-paediatric-case-series
#2
Duarte Martins, Caroline Ovaert, Diala Khraiche, Nathalie Boddaert, Damien Bonnet, Francesca Raimondi
INTRODUCTION: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited myocardial disease with an elusive association with myocardial inflammation. A myocarditis phenotype has been well established without systematic evidence of a viral trigger. We intend to study the relationship between myocardial inflammation detected by Cardiac magnetic resonance (CMR) and ARVC in a paediatric population. METHODS: Retrospective case series of all patients <18 years admitted to two CMR units for clinical suspicion of myocarditis from March 2012 to June 2017 who had genetic testing for inherited cardiomyopathies including analysis for known ARVC genes...
May 31, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29875345/spondylodiscitis-in-paediatric-patients-the-importance-of-early-diagnosis-and-prolonged-therapy
#3
Sonia Bianchini, Andrea Esposito, Nicola Principi, Susanna Esposito
Background: Spondylodiscitis (SD), the concurrent infection of a vertebral disc and the adjacent vertebral bodies, is a very severe disease that can lead to death or cause spinal deformities, segmental instabilities, and chronic pain, which significantly reduces the quality of life for affected patients. Early diagnosis and treatment are essential in order to reduce the risk of negative outcomes. The two cases of SD that are described in this paper might be useful for informing paediatric approaches to children with SD...
June 7, 2018: International Journal of Environmental Research and Public Health
https://www.readbyqxmd.com/read/29862079/the-burden-and-outcomes-of-abdominal-pain-among-children-presenting-to-an-emergency-department-of-a-tertiary-hospital-in-tanzania-a-descriptive-cohort-study
#4
Francis M Sakita, Hendry R Sawe, Victor Mwafongo, Juma A Mfinanga, Michael S Runyon, Brittany L Murray
Background: Abdominal pain in children can represent benign conditions or life-threatening emergencies. Aetiologies of paediatric abdominal pain vary geographically and have not been studied in acute care settings in East Africa. This study describes the clinical profiles and outcomes of children presenting with undifferentiated abdominal pain to the Emergency Department of Muhimbili National Hospital (ED-MNH). Methods: This was a prospective cohort study of children below 18 years of age presenting to the ED-MNH with abdominal pain...
2018: Emergency Medicine International
https://www.readbyqxmd.com/read/29859017/intramuscular-versus-oral-corticosteroids-to-reduce-relapses-following-discharge-from-the-emergency-department-for-acute-asthma
#5
REVIEW
Scott W Kirkland, Elfriede Cross, Sandra Campbell, Cristina Villa-Roel, Brian H Rowe
BACKGROUND: Acute asthma is a common cause of presentations to acute care centres, such as the emergency department (ED), and while the majority of patients can be discharged, relapse requiring additional medical care is common. Systemic corticosteroids are a major part in the treatment of moderate to severe acute asthma; however, there is no clear evidence regarding the most effective route of administration for improving outcomes in patients discharged from acute care. OBJECTIVES: To examine the effectiveness and safety of a single dose of intramuscular (IM) corticosteroids provided prior to discharge compared to a short course of oral corticosteroids in the treatment of acute asthma patients discharged from an ED or equivalent acute care setting...
June 2, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29851190/choosing-wisely-in-pediatric-anesthesia-an-interpretation-from-the-german-scientific-working-group-of-paediatric-anaesthesia-wakka
#6
Karin Becke, Christoph Eich, Claudia Höhne, Martin Jöhr, Andreas Machotta, Markus Schreiber, Robert Sümpelmann
Inspired by the Choosing Wisely initiative, a group of pediatric anesthesiologists representing the German Working Group on Paediatric Anaesthesia (WAKKA) coined and agreed upon 10 concise positive ("dos") or negative ("don'ts") evidence-based recommendations. (i) In infants and children with robust indications for surgical, interventional, or diagnostic procedures, anesthesia or sedation should not be avoided or delayed due to the potential neurotoxicity associated with the exposure to anesthetics...
May 30, 2018: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/29848517/unusual-presentation-of-hen%C3%A3-ch-schonlein-purpura
#7
Andrew Brodie, Natasha G, Rynda Nitiahpapand, Liaqat Chowoo
We present a rare case of a 4-year-old boy with newly diagnosed Henöch-Schonlein purpura (HSP) affecting the scrotum and penis. The patient presented to the emergency department with palpable purpura symmetrically distributed over the lower limbs. This was associated with arthritis of the right knee, abdominal pain and scrotal swelling. These symptoms were preceded by an upper respiratory tract infection (URTI). The patient was initially treated with empirical oral antibiotics for epididymitis and was discharged...
May 30, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29797060/correlates-and-predictors-of-paediatric-leg-pain-a-case-control-study
#8
Angela Margaret Evans, Trupti Berde, Leila Karimi, Prajakta Ranade, Nehal Shah, Raju Khubchandani
Paediatric leg pains, long described as 'growing pains', frequently present to clinicians, are prevalent in early childhood, disrupt sleep, and distress affected children and parents. There are many cited associations, but no defined leg pain sub-types, nor revealed predictive factors. We explored the implicated factors (viz. foot arches, foot strength, joint mobility, vitamin D, iron) in children with leg pain versus a control group. Leg pain sub-groups-growing pains (GP), restless legs (RLS), both (mixed)-are defined for the first time...
May 24, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29785937/the-effect-of-enzyme-replacement-therapy-on-clinical-outcomes-in-paediatric-patients-with-fabry-disease-a-systematic-literature-review-by-a-european-panel-of-experts
#9
REVIEW
Marco Spada, Ralf Baron, Perry M Elliott, Bruno Falissard, Max J Hilz, Lorenzo Monserrat, Camilla Tøndel, Anna Tylki-Szymańska, Christoph Wanner, Dominique P Germain
BACKGROUND: Fabry disease is caused by a deficiency of the lysosomal enzyme α-galactosidase, resulting in progressive accumulation of globotriaosylceramide (GL-3). The disease can manifest early during childhood and adolescence. Enzyme replacement therapy (ERT) with recombinant human α-galactosidase is the first specific treatment for Fabry disease and has been available in Europe since 2001. This paper presents the findings of a systematic literature review of clinical outcomes with ERT in paediatric patients with Fabry disease...
April 26, 2018: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29779912/how-often-do-we-perform-painful-and-stressful-procedures-in-the-paediatric-intensive-care-unit-a-prospective-observational-study
#10
Manuel A Baarslag, Sharan Jhingoer, Erwin Ista, Karel Allegaert, Dick Tibboel, Monique van Dijk
BACKGROUND: Adequate analgesia and sedation is crucial in critical care. There is little knowledge on the extent of painful and stressful procedures on children admitted to a paediatric intensive care unit (PICU) and its analgesic and/or sedative management. OBJECTIVE: The primary objective was to determine the number of painful and stressful procedures per patient per day in our PICU patients, including the numbers of attempts. A secondary objective was to map PICU nurses' perceptions of the painfulness of the included procedures...
May 17, 2018: Australian Critical Care: Official Journal of the Confederation of Australian Critical Care Nurses
https://www.readbyqxmd.com/read/29779292/paediatric-constipation-an-approach-and-evidence-based-treatment-regimen
#11
Harveen Singh, Frances Connor
BACKGROUND: Constipation affects 5-30% of children and is responsible for 3% of primary care visits. General practitioners (GPs) are frequently the first medical encounter for concerned parents regarding their child's bowel habit. OBJECTIVE: The aim of this article is to review the assessment and management of children with constipation to empower GPs to initiate treatment and know when to refer to a paediatrician. DISCUSSION: In the absence of organic aetiology, childhood constipation is almost always functional and often due to painful bowel movements that prompt the child to withhold stool...
May 2018: Australian journal of general practice
https://www.readbyqxmd.com/read/29776346/a-systematic-review-of-the-risk-factors-for-clinical-response-to-opioids-for-all-age-patients-with-cancer-related-pain-and-presentation-of-the-paediatric-stop-pain-study
#12
Ersilia Lucenteforte, Laura Vagnoli, Alessandra Pugi, Giada Crescioli, Niccolò Lombardi, Roberto Bonaiuti, Maurizio Aricò, Sabrina Giglio, Andrea Messeri, Alessandro Mugelli, Alfredo Vannacci, Valentina Maggini
BACKGROUND: Inter-patient variability in response to opioids is well known but a comprehensive definition of its pathophysiological mechanism is still lacking and, more importantly, no studies have focused on children. The STOP Pain project aimed to evaluate the risk factors that contribute to clinical response and adverse drug reactions to opioids by means of a systematic review and a clinical investigation on paediatric oncological patients. METHODS: We conducted a systematic literature search in EMBASE and PubMed up to the 24th of November 2016 following Cochrane Handbook and PRISMA guidelines...
May 18, 2018: BMC Cancer
https://www.readbyqxmd.com/read/29771901/achilles-tenotomy-as-an-office-procedure-and-current-practising-trends-among-new-zealand-orthopaedic-surgeons
#13
Lewis Agius, Angus Wickham, Cameron Walker, Joshua Knudsen
BACKGROUND: Percutaneous Achilles tenotomy (PAT) is performed during the final phase of casting with Ponseti method. Several settings have been proposed as venues for this procedure, however it is increasingly being performed in theatre under a general anaesthetic (GA). General anaesthesia, however, is expensive and not without risks. The purpose of the present study was to compare results of outpatient releases to theatre releases, and assess current practising trends among orthopaedic surgeons...
May 18, 2018: New Zealand Medical Journal
https://www.readbyqxmd.com/read/29764491/does-a-low-fodmaps-diet-reduce-symptoms-of-functional-abdominal-pain-disorders-a-systematic-review-in-adult-and-paediatric-population-on-behalf-of-italian-society-of-pediatrics
#14
REVIEW
Rossella Turco, Silvia Salvatore, Erasmo Miele, Claudio Romano, Gian Luigi Marseglia, Annamaria Staiano
BACKGROUND: Despite the rising of the Functional Gastrointestinal Disorders (FGIDs)' incidence in the last years, the etio-pathogenesis of FGIDs remains unclear. The diet seems to play an important role in these disorders. Indeed, at least two thirds of adult patients with Irritable Bowel Syndrome (IBS) and of children with FGIDs perceive their GI symptoms to be food-related. In particular, in the last years, more interest has been focused in the low Fermentable Oligosaccharides, Disaccharides, Monosaccharides, and Polyol (FODMAPs) diet...
May 15, 2018: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/29755909/malrotation-of-the-gut-in-adults-an-often-forgotten-entity
#15
Sushant Bhatia, Sudhir Jain, Chandra B Singh, Lovenish Bains, Rohit Kaushik, Nishant S Gowda
Malrotation of the gut is a common paediatric condition that usually presents in the first month of life. However, presentation in adults is rare, and as a diagnostic dilemma quite often surprises the surgeon intraoperatively. If this condition is not timely recognized, it may result in disastrous consequences, such as gangrene of the small gut. We present the case of a 21-year-old male who presented to the emergency room with recurrent episodes of colicky abdominal pain and bilious vomiting. Contrast-enhanced computerized tomography (CT) revealed malrotation of the gut...
March 12, 2018: Curēus
https://www.readbyqxmd.com/read/29752769/systematic-review-on-ureteral-access-sheaths-facts-and-myths
#16
REVIEW
Vincent De Coninck, Etienne Xavier Keller, María Rodríguez-Monsalve, Marie Audouin, Steeve Doizi, Olivier Traxer
OBJECTIVES: To review literature on all available ureteral access sheaths (UASs) with their indications, limitations, risks, advantages and disadvantages in current modern endourological practice. PATIENTS AND METHODS: Two authors searched Medline, Scopus, Embase and Web of Science databases to identify studies on UASs published in English. No time period restriction was applied. All original articles reporting about outcomes or innovations were included. Additional articles identified through references lists were also included...
May 11, 2018: BJU International
https://www.readbyqxmd.com/read/29751600/inflamed-phylloides-tumour-in-a-girl-a-challenging-diagnosis-in-paediatric-breast-lesions
#17
Ilaria Testa, Cristina Salvatori, Marco Prestipino, Maria Elena Laurenti, Paolo Gerli, Giuseppe Di Cara, Nicola Principi, Susanna Esposito, Mirko Bertozzi
Introduction : Phylloides tumours (PTs) are rare fibroepithelial neoplasms that account for 0.3⁻0.9% of all breast tumours. These tumours typically occur in women aged 30⁻70 years. The occurrence of these tumours in older children and adolescents poses particular diagnostic and therapeutic problems. However, early diagnosis is mandatory because although most of the cases of PTs in children are benign, the borderline and malignant cases with potential negative outcomes cannot be excluded. Case presentation : A 12-year-old girl presented at the Paediatric Emergency Department for hyperaemia and warmth of the left breast that occurred a few days prior without fever...
May 11, 2018: International Journal of Environmental Research and Public Health
https://www.readbyqxmd.com/read/29750665/interactive-versus-passive-distraction-and-parent-psychoeducation-as-pain-management-techniques-during-paediatric-venepuncture-a-randomized-controlled-trial
#18
Anna Newell, Jennifer Keane, Brian E McGuire, Caroline Heary, Vincent McDarby, Bernie Dudley, Josh Moran, Kady Francis, Line Caes
OBJECTIVES: The aim of the current research was to compare the relative efficacy of interactive and passive distraction, with or without parental psychoeducation, on child pain and distress during a venepuncture. We also compared the effect of parental psychoeducation on parental distress, knowledge of distraction strategies and engagement in distraction. METHODS: This cross-sectional study included 213 children scheduled for a venepuncture, and one of their parents, who were randomly allocated to one of four conditions; interactive distraction, passive distraction, interactive distraction with parent psychoeducation and passive distraction with parent psychoeducation...
May 10, 2018: Clinical Journal of Pain
https://www.readbyqxmd.com/read/29737317/first-robotic-pylorus-preserving-pancreaticoduodenectomy-for-frantz-s-tumour-in-an-adolescent-girl
#19
Palanivelu Chinnusamy, Sumanta Dey, Bhushan Chittawadagi, Srivatsan Gurumurthy, Sandeep Sabnis, Senthilnathan Palanisamy
Solid pseudopapillary tumour (SPT) is one of the uncommon benign cystic neoplasms of pancreas occurring predominantly in young females. Being benign in nature, surgical resection is the treatment of choice with excellent 5-year survival. A 14-year-old girl presented with pain abdomen for 1 week. On evaluation, she was found to have a large SPT involving head and uncinate process of Pancreas She underwent robotic pylorus preserving pancreaticoduodenectomy (R-PPPD) with da Vinci® Si Robotic System (Intuitive Surgical, Sunnyvale, CA, USA)...
May 4, 2018: Journal of Minimal Access Surgery
https://www.readbyqxmd.com/read/29707047/flexible-intramedullary-nailing-versus-nonoperative-treatment-for-paediatric-displaced-midshaft-clavicle-fractures
#20
I A Mukhtar, K M Yaghmour, A F Ahmed, T Ibrahim
Purpose: The treatment of displaced midshaft clavicle fractures in children remains controversial. The purpose of our study was to compare the outcome of displaced midshaft clavicle fractures in children who were managed operatively by flexible intramedullary nailing (FIN) with nonoperative treatment. Methods: A prospective review of 31 children (mean age 10.5 years) with displaced midshaft clavicle fractures treated either by FIN or nonoperatively and with at least a six-month follow-up was undertaken...
April 1, 2018: Journal of Children's Orthopaedics
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