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https://www.readbyqxmd.com/read/28433098/wilson-disease-in-children
#1
Eve A Roberts, Piotr Socha
Wilson disease (WD) is an inherited disorder mainly of hepatocellular copper disposition, due to dysfunction of the Wilson ATPase, a P1B-ATPase encoded by the gene ATP7B. In children, as in older age brackets, clinical disease is highly diverse. Although hepatic disease is the common presentation in children/adolescents, neurologic, psychiatric, and hematologic clinical presentations do occur. Very young children may have clinically evident liver disease due to WD. Early diagnosis, preferably when the child/adolescent is asymptomatic, is most likely to result in near-normal longevity with generally good health so long as the patient tolerates effective medication, is adherent to the lifelong treatment regimen, and has consistent access to the medication...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28433088/parental-age-and-offspring-psychopathology-in-the-philadelphia-neurodevelopmental-cohort
#2
Alison K Merikangas, Monica E Calkins, Warren B Bilker, Tyler M Moore, Ruben C Gur, Raquel E Gur
OBJECTIVE: Increasing evidence implicates advanced paternal age at offspring birth in neuropsychiatric disorders. Advanced maternal age has also been associated with schizophrenia and other neurodevelopmental disorders, whereas younger maternal age has been linked with behavioral disorders. Few studies have considered the specificity of the associations with respect to comorbidity. In addition, most prior studies have been conducted in clinical samples or registries that may reflect more severe forms of psychopathology...
May 2017: Journal of the American Academy of Child and Adolescent Psychiatry
https://www.readbyqxmd.com/read/28432019/feasibility-of-mini-tablets-as-a-flexible-drug-delivery-tool
#3
Biplob Mitra, Jessica Chang, Sy-Juen Wu, Chad N Wolfe, Robert L Ternik, Thomas Z Gunter, Michael C Victor
Mini-tablets have potential applications as a flexible drug delivery tool in addition to their generally perceived use as multi-particulates. That is, mini-tablets could provide flexibility in dose finding studies and/or allow for combination therapies in the clinic. Moreover, mini-tablets with well controlled quality attributes could be a prudent choice for administering solid dosage forms as a single unit or composite of multiple mini-tablets in patient populations with swallowing difficulties (e.g., pediatric and geriatric populations)...
April 18, 2017: International Journal of Pharmaceutics
https://www.readbyqxmd.com/read/28430913/prediction-of-adverse-events-after-catheter-based-procedures-in-adolescents-and-adults-with-congenital-heart-disease-in-the-impact-registry
#4
Ada C Stefanescu Schmidt, Aimee Armstrong, Kevin F Kennedy, David Nykanen, Jamil Aboulhosn, Ami B Bhatt
Aims: We sought to identify factors associated with major adverse events (MAE) after cardiac catheterization in adolescents and adults with congenital heart disease (CHD), and create the first model to individualize risk discussions in this growing population. Methods and results: Improving Pediatric and Adult Congenital Treatment (IMPACT), a National Cardiovascular Data Registry, contains congenital catheterization data from over 87 hospitals in the United States...
April 18, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28429488/interdisciplinary-periodontics-the-multidisciplinary-approach-to-the-planning-and-treatment-of-complex-cases
#5
REVIEW
Karl M Lyons, Ivan Darby
Periodontics cannot be practiced in isolation as frequently many patients have multiple dental needs or medical health issues requiring management. In addition, pathology may manifest in the periodontal tissues, and the onset and progression of periodontitis can be affected by systemic conditions, such as diabetes, and vice versa. The focus of this volume of Periodontology 2000 is interdisciplinary periodontics, and the articles included discuss the interactions and the interrelationshipbetween periodontal tissues/periodontal diseases and endodontics, fixed prosthodontics, implant dentistry, esthetics, gerodontology, radiology, orthodontics, pediatric dentistry, oral and maxillofacial surgery, oral pathology, special needs dentistry and general medicine...
June 2017: Periodontology 2000
https://www.readbyqxmd.com/read/28429483/management-of-periodontal-health-in-children-pediatric-dentistry-and-periodontology-interface
#6
REVIEW
Bernadette K Drummond, Michael G Brosnan, Jonathan W Leichter
The oral health of children and adolescents mirrors their general health. Because oral health care is often delivered in isolation from general health care, oral signs and symptoms do not always alert practitioners to their significance beyond the mouth. An important example of this is the association of a higher risk of dental caries and periodontal disease in children and adolescents with overweight, obesity and prediabetic conditions. Oral-health practitioners need to consider the health conditions that their patients may have...
June 2017: Periodontology 2000
https://www.readbyqxmd.com/read/28429117/a-comparison-of-mcgrath-mac%C3%A2-and-standard-direct-laryngoscopy-in-simulated-immobilized-cervical-spine-pediatric-intubation-a-manikin-study
#7
Marcin Madziala, Jacek Smereka, Marek Dabrowski, Steve Leung, Kurt Ruetzler, Lukasz Szarpak
Emergency airway management in children is generally considered to be challenging, and endotracheal intubation requires a high level of personal skills and experience. Immobilization of the cervical spine is indicated in all patients with the risk of any cervical spine injury but significantly aggravates endotracheal intubation. The best airway device in this setting has not been established yet, although the use of videolaryngoscopes is generally promising. Seventy-five moderately experienced paramedics of the Emergency Medical Service of Poland performed endotracheal intubations in a pediatric manikin in three airway scenarios: (A) normal airway, (B) manual in-line cervical immobilization, and (C) cervical immobilization using a Patriot cervical extrication collar and using two airway techniques: (1) McGrath videolaryngoscope and (2) Macintosh blade in a randomized sequence...
April 21, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28428716/transition-after-pediatric-liver-transplantation-perceptions-of-adults-adolescents-and-parents
#8
Norman Junge, Katarina Migal, Imeke Goldschmidt, Ulrich Baumann
AIM: To develop a locally adapted, patient-focused transition-program, we evaluated the perceptions of adult and adolescent patients and parents regarding transition-programs and transfer. METHODS: We evaluated these perceptions by analyzing the responses of pre-transfer adolescents (n = 57), their parents (n = 57) and post-transfer adults (n = 138) from a cohort of pediatric-liver-transplant-patients using a self-designed questionnaire. Furthermore, we compared a responder group with a non-responder group as well as the provided answers with baseline characteristics and clinical outcomes to exclude selection bias, characterize high-risk patients for non-adherence and test for gender differences...
April 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28428029/relationship-between-high-white-blood-cell-count-and-insulin-resistance-homa-ir-in-korean-children-and-adolescents-korean-national-health-and-nutrition-examination-survey-2008-2010
#9
J-M Park, D-C Lee, Y-J Lee
BACKGROUND AND AIMS: Increasing evidence has indicated that insulin resistance is associated with inflammation. However, few studies have investigated the association between white blood cell (WBC) count and insulin resistance, as measured by a homeostasis model assessment of insulin resistance (HOMA-IR) in a general pediatric population. This study aimed to examine the association between WBC count and insulin resistance as measured by HOMA-IR in a nationally representative sample of children and adolescents...
March 10, 2017: Nutrition, Metabolism, and Cardiovascular Diseases: NMCD
https://www.readbyqxmd.com/read/28427924/long-term-deterioration-of-perceived-health-and-functioning-in-adults-with-cerebral-palsy
#10
Joyce L Benner, Sander R Hilberink, Thessa Veenis, Henk J Stam, Wilma M A van der Slot, Marij E Roebroeck
OBJECTIVE: To describe longitudinal change in perceived health, presence of health issues and functional level in adults with cerebral palsy (CP). DESIGN: Prospective cohort study. SETTING: Participants who completed baseline assessments in 1996 or 2000 were invited for a long-term follow-up in 2010. PARTICIPANTS: Forty-nine Dutch adults with CP (age 35-45 years, 55% male, 75% spastic) formerly known in pediatric rehabilitation care...
April 17, 2017: Archives of Physical Medicine and Rehabilitation
https://www.readbyqxmd.com/read/28426647/association-of-prenatal-urinary-phthalate-metabolite-concentrations-and-childhood-bmi-and-obesity
#11
Kim G Harley, Kimberly Berger, Stephen Rauch, Katherine Kogut, Birgit Claus Henn, Antonia M Calafat, Karen Huen, Brenda Eskenazi, Nina Holland
BACKGROUND: Although experiments in animals suggest phthalates may have obesogenic effects, studies of prenatal exposure in children show inconsistent results. METHODS: We measured urinary concentrations of 11 phthalate metabolites collected twice during pregnancy from mothers in the Center for the Health Assessment of Mothers and Children of Salinas (CHAMACOS) cohort study (N=345). Height, weight, waist circumference, and percent body fat were assessed in their children between 5 and 12 years of age...
April 20, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28425922/detailed-follow-up-study-of-pediatric-orofacial-granulomatosis-patients
#12
Anu Haaramo, Heikki Alapulli, Liisa Aine, Ulla Saarnisto, Jetta Tuokkola, Tarja Ruuska, Taina Sipponen, Anne Pitkäranta, Kaija-Leena Kolho
OBJECTIVES: Orofacial granulomatosis (OFG) is a chronic inflammatory condition affecting the orofacial area. Its connection to Crohn's disease (CD) is debated. Our aim was to describe a cohort of pediatric OFG patients in detail, study the long-term behavior of OFG and evaluate factors predicting CD in OFG patients. METHODS: We invited patients diagnosed with OFG at two university hospitals, Finland for a follow-up appointment. Patients (n = 29) were examined by a dentist and an otorhinolaryngologist using a structural schema...
February 22, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28425688/pediatric-epilepsy-and-psychiatric-comorbidity-preliminary-observational-data-from-a-prospective-study
#13
Michela Gatta, Alessia Raffagnato, Stefania Mannarini, Laura Balottin, Irene Toldo, Marilena Vecchi, Clementina Boniver
BACKGROUND: Several studies have confirmed psychiatric comorbidity and a worse quality of life in children with epilepsy, but the clinical assessment and monitoring of these patients often pays insufficient attention to their psychological aspects alongside their neurological issues. The present study aims to describe the distribution of psychopathologies and their clinical evolution over 18 months in a sample of children followed up since the onset of their epilepsy. METHODS: After being diagnosed with epilepsy, 49 subjects (age 4-18y) were followed up with psychiatric and psychological assessments based on the use of dimensional and categorical psychometric tools...
April 20, 2017: Minerva Pediatrica
https://www.readbyqxmd.com/read/28425236/predictors-of-health-related-quality-of-life-in-childhood-epilepsy-and-comparison-with-healthy-children-findings-from-an-indian-study
#14
Dipika Bansal, Chandrika Azad, Kapil Gudala, Anil Dasari
BACKGROUND/AIM: Children with epilepsy have reduced health-related quality of life (HRQOL) due to disease and medications. We aimed to assess child-reported HRQOL in Indian children with epilepsy and compare it with that in healthy children. MATERIALS AND METHODS: A cross-sectional study of 256 children with epilepsy aged between 5 and 18 years on antiepileptic drug (AED) treatment for at least 3 months was performed and 125 age and sex matched healthy children were included...
April 18, 2017: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/28424766/the-need-for-hematology-nurse-education-in-low-and-middle-income-countries-a-community-case-study-in-tanzania
#15
Julie M Buser
Hematology-related diseases, such as anemia, malaria, sickle cell disease (SCD), and blood cancers, have differing rates of survival between high-income and low- and middle-income countries (LMICs). Nurses in LMICs have an unmet need for specialty training and education to address hematology and hemato-oncology disorders. A gap in the literature exists about hematology nurse education and clinical service demands in LMICs. This community case study documents a collaborative hematology and basic hemato-oncology education program to sustainably strengthen nurse capacity at a national referral hospital and university in Tanzania...
2017: Frontiers in Public Health
https://www.readbyqxmd.com/read/28424007/anesthetic-related-neurotoxicity-and-neuroimaging-in-children-a-call-for-conversation
#16
Kara A Bjur, Eric T Payne, Michael E Nemergut, Danqing Hu, Randall P Flick
Each year millions of young children undergo procedures requiring sedation or general anesthesia. An increasing proportion of the anesthetics used are provided to optimize diagnostic imaging studies such as magnetic resonance imaging. Concern regarding the neurotoxicity of sedatives and anesthetics has prompted the US Food and Drug Administration to change labeling of anesthetics and sedative agents warning against repeated or prolonged exposure in young children. This review aims to summarize the risk of anesthesia in children with an emphasis on anesthetic-related neurotoxicity, acknowledge the value of pediatric neuroimaging, and address this call for conversation...
May 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/28423225/exercise-interventions-for-patients-with-pediatric-cancer-during-inpatient-acute-care-a-systematic-review-of-literature
#17
REVIEW
Vanessa Rustler, Meaghan Hagerty, Julia Daeggelmann, Stacey Marjerrison, Wilhelm Bloch, Freerk T Baumann
Physical inactivity has been shown to exacerbate negative side effects experienced by pediatric patients undergoing cancer therapy. Exercise interventions are being created in response. This review summarizes current exercise intervention data in the inpatient pediatric oncology setting. Two independent reviewers collected literature from three databases, and analyzed data following the PRISMA statement for systematic reviews and meta-analyses. Ten studies were included, representing 204 patients. Good adherence, positive trends in health status, and no adverse events were noted...
April 19, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28423145/brief-behavioral-therapy-for-pediatric-anxiety-and-depression-in-primary-care-a-randomized-clinical-trial
#18
V Robin Weersing, David A Brent, Michelle S Rozenman, Araceli Gonzalez, Megan Jeffreys, John F Dickerson, Frances L Lynch, Giovanna Porta, Satish Iyengar
Importance: Anxiety and depression affect 30% of youth but are markedly undertreated compared with other mental disorders, especially in Hispanic populations. Objective: To examine whether a pediatrics-based behavioral intervention targeting anxiety and depression improves clinical outcome compared with referral to outpatient community mental health care. Design, Setting, and Participants: This 2-center randomized clinical trial with masked outcome assessment conducted between brief behavioral therapy (BBT) and assisted referral to care (ARC) studied 185 youths (aged 8...
April 19, 2017: JAMA Psychiatry
https://www.readbyqxmd.com/read/28422995/characterization-of-rotavirus-infection-in-children-with-acute-gastroenteritis-in-bengo-province-northwestern-angola-prior-to-vaccine-introduction
#19
Carolina Gasparinho, João Piedade, Maria Clara Mirante, Cristina Mendes, Carlos Mayer, Susana Vaz Nery, Miguel Brito, Claudia Istrate
BACKGROUND: Rotavirus group A (RVA) is considered the leading cause of pediatric diarrhea, responsible for the high burden of diarrheal diseases in sub-Saharan Africa. Despite recent studies, the existent data are scarce for some African countries like Angola, a country with one of the highest RVA-related death estimates. The aim of this study was to determine the RVA detection rate and circulating genotypes in children less than five years of age with acute gastroenteritis attended at the Bengo General Hospital in Caxito, Bengo province, Angola, before vaccine introduction...
2017: PloS One
https://www.readbyqxmd.com/read/28422947/factors-affecting-the-cognitive-profile-of-11-year-old-children-born-very-preterm
#20
Anna Nyman, Tapio Korhonen, Petriina Munck, Riitta Parkkola, Liisa Lehtonen, Leena Haataja
BACKGROUND: Preterm birth is a risk for cognitive development. This study assessed the cognitive profile of children born very preterm at the age of 11 years as well as associated risk factors. METHODS: A total of 128 children born very preterm were included. MRI was performed at term age. Cognitive development was assessed using the full-scale intelligence quotient (IQ) and four domains of the Wechsler Intelligence Scale for Children, fourth edition (WISC-IV), Finnish translation...
April 19, 2017: Pediatric Research
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