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https://www.readbyqxmd.com/read/29139538/limitations-of-the-pax7-creer-t2-transgene-for-driving-deletion-of-nf1-in-adult-mouse-muscle
#1
Matthew A Summers, Kathy Mikulec, Lauren Peacock, David G Little, Aaron Schindeler
Neurofibromatosis Type 1 (NF1) is an autosomal dominant genetic disorder that results in a variety of characteristic manifestations. Prior studies have shown reduced muscle size and global skeletal muscle weakness in children with NF1. This associated weakness can lead to significant challenges impacting on quality of life. Pre-clinical studies using a muscle-specific NF1 knockout mouse have linked this weakness to an underlying primary metabolic deficiency in the muscle. However, the neonatal lethality of this strain prevents analysis of the role of NF1 in adult muscle...
2017: International Journal of Developmental Biology
https://www.readbyqxmd.com/read/29138640/prevalence-of-soil-transmitted-helminthiases-and-schistosomiasis-in-preschool-age-children-in-mwea-division-kirinyaga-south-district-kirinyaga-county-and-their-potential-effect-on-physical-growth
#2
Stephen Sifuna Wefwafwa Sakari, Amos K Mbugua, Gerald M Mkoji
Intestinal parasitic infections can significantly contribute to the burden of disease, may cause nutritional and energetic stress, and negatively impact the quality of life in low income countries of the world. This cross-sectional study done in Mwea irrigation scheme, in Kirinyaga, central Kenya, assessed the public health significance of soil-transmitted helminthiases (STH), schistosomiasis, and other intestinal parasitic infections, among 361 preschool age children (PSAC) through fecal examination, by measuring anthropometric indices, and through their parents/guardians, by obtaining sociodemographic information...
2017: Journal of Tropical Medicine
https://www.readbyqxmd.com/read/29135879/cochlear-implantation-in-children-with-congenital-unilateral-deafness-a-case-series
#3
Rainer Linus Beck, Antje Aschendorff, Frederike Hassepaß, Thomas Wesarg, Stefanie Kröger, Till F Jakob, Susan Arndt
OBJECTIVES: Cochlear implant is regarded as a treatment option for hearing rehabilitation of adults with unilateral sensorineural hearing loss. A clear benefit has been experienced in regard to speech comprehension in noise, localization, and quality of life. The aim of this study was to investigate the benefit of cochlear implantation for children with congenital unilateral hearing loss. STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral center; cochlear implant program...
December 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/29135446/-cross-informant-agreement-between-parent-reported-and-adolescent-self-reported-quality-of-life-in-children-with-externalising-symptoms
#4
Dóra Szentiványi, József Halász, Lili O Horváth, Gergely Mészáros, Martina Miklós, Mónika Miklósi, Szabina Velo, Péter Vida, Judit Balázs
BACKGROUND: It is well-known phenomena both in clinical practice and research, that there is a discrepancy between the parents' and children's report about children's Quality of Life (QoL). The purpose of our research was to examine the differences in self and caregiver's proxy report of child's QoL. We also compared mothers', fathers' and other caregivers' reports of children's QoL. METHODS: Patients with externalizing symptoms in their history, aged 13-18 years were selected from the Vadaskert Child and Adolescent Psychiatry Hospital and Outpatient Clinic...
2017: Psychiatria Hungarica: A Magyar Pszichiátriai Társaság Tudományos Folyóirata
https://www.readbyqxmd.com/read/29133326/risks-for-comorbidity-in-children-with-atopic-disorders-an-observational-study-in-dutch-general-practices
#5
David H J Pols, Arthur M Bohnen, Mark M J Nielen, Joke C Korevaar, Patrick J E Bindels
OBJECTIVE: This study aimed to investigate both atopic and non-atopic comorbid symptoms and diseases in children with physician-diagnosed atopic disorders (atopic eczema, asthma and allergic rhinitis). METHODS: All children aged 0-18 years listed in a nationwide primary care database (the Netherlands Institute for Health Services Research-Primary Care Database) with routinely collected healthcare data in 2014 were selected. Children with atopic disorders were matched on age and gender with non-atopic controls within the same general practice...
November 12, 2017: BMJ Open
https://www.readbyqxmd.com/read/29132970/at-home-and-in-group-delivery-of-constraint-induced-movement-therapy-in-children-with-hemiparesis-a-systematic-review
#6
REVIEW
Emilie Durand, Pascale Plante, Andrey-Anne Pelletier, Johanie Rondeau, Frédérique Simard, Julien Voisin
BACKGROUND: Constraint-induced movement therapy (CIMT) is increasingly recognized as an effective therapy for children with hemiparesis. However, the effectiveness of CIMT outside the standard rehabilitation protocol in clinical settings is less known. The aim of this systematic review was to investigate the effectiveness of CIMT conducted at home or in a group. METHODS: We searched CINAHL, PubMed and ScienceDirect in August 2017 to select articles of studies investigating the impact of CIMT performed at home and in a group on affected upper-limb ability, occupational performance, and quality of life of children...
November 10, 2017: Annals of Physical and Rehabilitation Medicine
https://www.readbyqxmd.com/read/29132675/the-role-of-baked-egg-and-milk-in-the-diets-of-allergic-children
#7
REVIEW
Melissa L Robinson, Bruce J Lanser
Baked egg and baked milk are tolerated by most children who are allergic to hen's egg and cow's milk. Incorporating baked goods into the diets of allergic children may help them outgrow their primary allergy more quickly, with changes observed akin to immunotherapy. Benefits may also include increased quality of life and improved nutritional status. The search for a reliable biomarker to predict tolerance to baked goods is ongoing. Most children with a milk or egg allergy who are not previously tolerating egg or milk in baked goods should be offered an observed oral food challenge...
February 2018: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/29130289/development-and-pilot-testing-of-a-parent-reported-health-related-quality-of-life-measure-for-children-on-the-ketogenic-diet-the-ketoqol
#8
Katie Barwick, Tamara Parker, Nicole Murphy, Alwyn Todd, Michael Leveritt, Shelley A Wilkinson
AIM: The aim of the present study was to develop a parent-reported tool that will measure health-related quality of life (HRQoL) in children following ketogenic diet (KD) therapies for refractory epilepsy once it has been pilot tested and analysed. METHODS: Parents of children following KD therapies for epilepsy were recruited through a public hospital in Queensland, Australia, in 2012 and 2014. Qualitative semistructured interviews were conducted in 2012 with 13 parents who described changes seen in their child's HRQoL while on the KD...
November 2017: Nutrition & Dietetics: the Journal of the Dietitians Association of Australia
https://www.readbyqxmd.com/read/29129379/children-with-epilepsy-against-their-healthy-peers-and-those-with-headaches-differences-similarities
#9
Dorota Talarska, Michał Michalak, Patrycja Talarska, Barbara Steinborn
INTRODUCTION: Epilepsy, like most chronic diseases, affects bio-psycho-social functioning of children and adolescents. The aim of this work was to assess functioning of children with epilepsy compared with the group of healthy children and those with headaches carried out by children themselves and their mothers. MATERIAL AND METHODS: The study included 209 children with epilepsy and 173 children with headaches and 182 healthy students and their mothers. The research tool was Pediatric Quality of Life Inventory 4...
October 27, 2017: Neurologia i Neurochirurgia Polska
https://www.readbyqxmd.com/read/29129152/attention-deficit-hyperactivity-disorder-adhd-in-epilepsy-and-primary-adhd-differences-in-symptom-dimensions-and-quality-of-life
#10
Ozalp Ekinci, Çetin Okuyaz, Semra Erdoğan, Serkan Gunes, Nuran Ekinci, Merve Kalınlı, Halenur Teke, Meltem Çobanoğulları Direk
PURPOSE: We aimed to (1) compare quality of life (QOL) among children with epilepsy, epilepsy and attention-deficit hyperactivity disorder (ADHD), and primary ADHD and (2) compare ADHD symptom dimensions and subtypes between children with epilepsy-ADHD and primary ADHD. METHODS: A total of 140 children; 53 with epilepsy, 35 with epilepsy-ADHD, and 52 with primary ADHD were included. KINDL-R (quality of life measure), Turgay DSM-IV Disruptive Behavior Disorders Rating Scale (T-DSM-IV-S), and Conners' Parent Rating Scale (CPRS) were completed...
December 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/29127678/allogeneic-matched-related-transplantation-for-%C3%AE-thalassemia-and-sickle-cell-anemia
#11
Françoise Bernaudin, Corinne Pondarré, Claire Galambrun, Isabelle Thuret
Allogeneic hematopoietic stem cell transplantation (HSCT) can cure single gene disorders such as thalassemia and sickle cell anemia (SCA). These non-malignant diseases have in common severe hemolytic anemia and high proliferative bone marrow, requiring frequent transfusions. The risk of rejection is high and graft-vs-host disease is not desirable. Important progress has been made in the management of these diseases, including leukocyte depletion of blood products, and chelation therapy, for both diseases, and erythrocytapheresis and hydroxycarbamide for SCA...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29127595/neurological-manifestations-of-congenital-zika-virus-infection
#12
Tania Saad, Alessandra Augusta PennaeCosta, Fernanda Veiga de Góes, Marcela de Freitas, Julia Valeriano de Almeida, Lúcio José de Santa Ignêz, Ana Paula Amancio, Renata Joviano Alvim, Ludmilla Athayde Antunes Kramberger
INTRODUCTION: In 2015, it was observed a rise in the number of microcephalic newborns associated with a history of non-specific febrile sickness and rash during pregnancy in Brazil. Since then, microcephaly has emerged as a public health concern. A few months after, the causal relation between congenital microcephaly and the Zika virus was discovered. Zika virus, an arbovirus, is a new TORCH member that leads to congenital infection through vertical transmission and harms the developing brain, disrupting synaptogenesis, and causing other central nervous system lesions...
November 10, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29127480/quality-assessment-of-atopic-dermatitis-clinical-practice-guidelines-in-%C3%A2-%C3%A2-18-years
#13
Luz de María Díaz Granados, María Adelaida Quijano, Paola Andrea Ramírez, Natalia Aguirre, Gloria Sanclemente
Atopic dermatitis (AD) is a chronic inflammatory skin disease that affects the patients' quality of life greatly often from a very young age. Its worldwide incidence in children and adults varies, but it is usually among the first ten causes of dermatological consultation worldwide. There is a wide variety of treatment options for this condition including topical and systemic regimes. The decision to choose a treatment option in dermatological diseases is greatly influenced by the personal experience of each specialist, which increases variability in the selection of available therapies...
November 10, 2017: Archives of Dermatological Research
https://www.readbyqxmd.com/read/29127099/individualised-advance-care-planning-in-children-with-life-limiting-conditions
#14
Erik A H Loeffen, Wim J E Tissing, Meggi A Schuiling-Otten, Chris C de Kruiff, Leontien C M Kremer, A A Eduard Verhagen
INTRODUCTION: In 2013, the Pediatric Association of the Netherlands launched an evidence-based guideline 'Palliative care for children'. To promote implementation in daily practice and hereby improve quality of paediatric palliative care, we aimed to develop a functional individualised paediatric palliative care plan (IPPCP) that covers physical, psychological, spiritual and social functioning, with great emphasis on the guideline's recommendations, advance care planning and patients' and parents' preferences and desires...
November 10, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/29126508/complications-in-children-with-ulcerative-colitis-undergoing-ileal-pouch-anal-anastomosis
#15
Dani O Gonzalez, Benedict C Nwomeh
Total colectomy with ileal pouch-anal anastomosis (IPAA) is considered the standard procedure for the surgical management of ulcerative colitis. Despite the widespread utility of the procedure, as many as 75% of patients who undergo IPAA, experience at least 1 complication. This review highlights difficult intraoperative scenarios and complications of pouch surgery in children, including intraoperative, postoperative, and functional complications. Intraoperative scenarios include insufficient mesenteric length and positive leak tests...
December 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/29126507/surgical-options-in-the-treatment-of-ulcerative-colitis
#16
Daniel P Ryan, Daniel P Doody
Children and young adults with ulcerative colitis tend to present with more extensive colonic disease than an adult population. The need for surgical intervention in the pediatric population with ulcerative colitis occurs earlier after diagnosis and has a greater incidence than a comparably matched adult population with an estimated need for colectomy at 5 years following diagnosis of 14-20%. Perhaps, even more than the adult population, there is a desire to restore intestinal continuity for the pediatric patient to achieve as healthy and normal quality of life as possible...
December 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/29125526/bowel-management-and-continence-in-adults-with-spina-bifida-results-from-the-national-spina-bifida-patient-registry-2009-15
#17
John S Wiener, Kristina D Suson, Jonathan Castillo, Jonathan C Routh, Stacy Tanaka, Tiebin Liu, Elisabeth Ward, Judy Thibadeau, David Joseph, National Spina Bifida Patient Registry
INTRODUCTION: Advances in care have allowed most children with spina bifida (SB) to live to adulthood. The majority have neuropathic bowel dysfunction (NBD), resulting in constipation, incontinence, and diminished quality of life. We sought to 1) describe contemporary NBD management and continence outcomes of adults with SB; 2) describe differences from younger patients; and 3) assess for association with socio-economic factors. METHODS: We analyzed data on NBD management and continence from the National Spina Bifida Patient Registry (NSBPR)...
October 20, 2017: Journal of Pediatric Rehabilitation Medicine
https://www.readbyqxmd.com/read/29123651/recent-advances-in-managing-and-understanding-enuresis
#18
REVIEW
Charlotte Van Herzeele, Johan Vande Walle, Karlien Dhondt, Kristian Vinter Juul
Enuresis, particularly in children during sleep, can be a debilitating condition, affecting the quality of life of the child and his or her family. The pathophysiology of nocturnal enuresis, though not clear, revolves around the inter-related mechanisms of overactive bladder, excessive nocturnal urine production, and sleep fragmentation. The first mechanism is more related to isolated nocturnal voiding, whereas the latter two are more related to nocturnal enuresis, in which circadian variations in arginine vasopressin hormone play a key role...
2017: F1000Research
https://www.readbyqxmd.com/read/29123343/multiple-nutritional-deficiencies-in-cerebral-palsy-compounding-physical-and-functional-impairments
#19
P G Hariprasad, K E Elizabeth, Mathew J Valamparampil, D Kalpana, T S Anish
Introduction: Cerebral palsy (CP) refers to a spectrum of disorders causing physical and intellectual morbidity. Macro and micro nutrient deficiencies often contribute to the subnormal physical and mental capabilities of them. Objectives: To assess the growth, nutritional status, physical and functional ability and quality of life in cerebral palsy children and to determine any relation with their gross motor and functional capabilities. Method: The study was conducted at a Tertiary Care Centre, with the participants in the age group 1-16 years...
October 2017: Indian Journal of Palliative Care
https://www.readbyqxmd.com/read/29122268/comparison-of-pediatric-self-reports-and-parent-proxy-reports-utilizing-promis-results-from-a-chiropractic-practice-based-research-network
#20
Joel Alcantara, Jeanne Ohm, Junjoe Alcantara
OBJECTIVE: To measure the cross-informant variant of pediatric quality of life (QoL) based on self-reports and parent proxy measures. METHODS: A secondary analysis of baseline data obtained from two independent studies measuring the QoL based on the pediatric PROMIS-25 self-report and the PROMIS parent-proxy items banks. A scoring manual associated raw scores to a T score metric (mean = 50; SD = 10). Reliability of QoL ratings utilized the ICC while comparison of mean T Scores utilized the unpaired t-test...
November 2017: Complementary Therapies in Clinical Practice
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