705,091 results match your criteria Pediatric Nursing[Journal]


Lennox-Gastaut Syndrome in Mitochondrial Disease.

Yonsei Med J 2019 Jan;60(1):106-114

Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea.

Purpose: Previous studies have shown that neurologic symptoms are dominant in patients with mitochondrial diseases, and most of these patients have seizure-related disorders. The epileptic classification of these patients as Lennox-Gastaut syndrome (LGS) is as high as 25%. This study aimed to investigate the clinical manifestations, diagnoses, treatments, and epilepsy in LGS, which is associated with mitochondrial disease. Read More

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January 2019

The Usefulness of Muscle Biopsy in Initial Diagnostic Evaluation of Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-Like Episodes.

Yonsei Med J 2019 Jan;60(1):98-105

Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea.

Purpose: The disease entity mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) is characterized by an early onset of stroke-like episodes. MELAS is the most dominant subtype of mitochondrial disease. Molecular genetic testing is important in the diagnosis of MELAS. Read More

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January 2019

Characterization of Flow Efficiency, Pulsatility, and Respiratory Variability in Different Types of Fontan Circuits Using Quantitative Parameters.

Yonsei Med J 2019 Jan;60(1):56-64

Division of Cardiovascular Surgery, Severance Cardiovascular Hospital, Yonsei University College of Medicine, Seoul, Korea.

Purpose: Details on the hemodynamic differences among Fontan operations remain unclear according to respiratory and cardiac cycles. This study was undertaken to investigate hemodynamic characteristics in different types of Fontan circulation by quantification of blood flow with the combined influence of cardiac and respiratory cycles.

Materials And Methods: Thirty-five patients [10 atriopulmonary connections (APC), 13 lateral tunnels (LT), and 12 extracardiac conduits (ECC)] were evaluated, and parameters were measured in the superior vena cava, inferior vena cava (IVC), hepatic vein (HV), baffles, conduits, and left and right pulmonary artery. Read More

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January 2019

Validation of the Rotterdam Elderly Pain Observation Scale (REPOS) in the hospital setting.

Pain Pract 2018 Dec 16. Epub 2018 Dec 16.

Intensive Care and Department of Pediatric surgery, Erasmus MC-Sophia Children's Hospital, Rotterdam, the Netherlands.

Background: The Rotterdam Elderly Pain Observation Scale (REPOS) has been proven useful to assess pain in non-communicative and cognitively impaired nursing home residents. We evaluated whether the REPOS is also reliable and valid for pain assessment in the hospital setting.

Methods: In this prospective multicenter observational study, surgical patients were observed perioperatively at bedside and internal medicine patients were filmed during a possible painful moment and in rest. Read More

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December 2018

Fecal volatile organic compounds for early detection of colorectal cancer: where are we now?

J Cancer Res Clin Oncol 2018 Dec 15. Epub 2018 Dec 15.

Department of Gastroenterology and Hepatology, Amsterdam UMC, Vrije Universiteit Amsterdam, AG&M Research Institute, Amsterdam, The Netherlands.

Introduction: The fecal volatolome, which is composed of fecal volatile organic compounds (VOCs), seems to hold potential as non-invasive biomarker for the detection of colorectal cancer (CRC) and its precursor lesions advanced adenomas (AA). The potential of the fecal volatolome has been subject of various studies using either chemical analytical or pattern-recognition techniques. The available literature on the potential of the fecal volatolome as CRC and AA biomarker was reviewed. Read More

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December 2018

Intraoperative stent placement for the treatment of acute portal vein complications in pediatric living donor liver transplantation.

Langenbecks Arch Surg 2018 Dec 15. Epub 2018 Dec 15.

Department of General Surgery, Division of HPB Surgery and Liver Transplant Unit, Hospital Italiano de Buenos Aires, Juan D. Peron 4190, C1181ACH, Buenos Aires, Argentina.

Purpose: Pediatric living donor liver transplantation (LDLT) in low weight recipients remains one of the most complex surgical procedures, with portal vein (PV) complications occurring in up to 19% of cases. When decreased PV flow is diagnosed intra- or perioperatively, intraoperative stent placement is a good substitute for surgical adjustment. Still, at the present moment, little is known about the technical feasibility, safety, efficacy, and long-term outcome of intraoperative stenting in LDLT. Read More

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December 2018

Epidemiology of gastrostomy insertion for children and adolescents with intellectual disability.

Eur J Pediatr 2018 Dec 15. Epub 2018 Dec 15.

Telethon Kids Institute, Centre for Child Health Research, The University of Western Australia, PO Box 855, West Perth, Western Australia, 6872, Australia.

The largest group of recipients of pediatric gastrostomy have neurological impairment with intellectual disability (ID). This study investigated trends in first gastrostomy insertion according to markers of disadvantage and ID etiology. Linked administrative and health data collected over a 32-year study period (1983-2014) for children with ID born between 1983 and 2009 in Western Australia were examined. Read More

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December 2018

Neurocognitive and functional outcomes at 5 years of age after renal transplant in early childhood.

Pediatr Nephrol 2018 Dec 15. Epub 2018 Dec 15.

Department of Pediatrics, Edmonton Clinic Health Academy, University of Alberta, 11405 - 87 Ave, Edmonton, AB, T6G 1C9, Canada.

Background: Clinicians often use information about developmental outcomes in decision-making around offering complex, life-saving interventions in children such as dialysis and renal transplant. This information in children with end-stage renal disease (ESRD) is limited, particularly when ESRD onset is in infancy or early childhood.

Methods: Using data from an ongoing prospective, longitudinal, inception cohort study of children with renal transplant before 5 years of age, we evaluated (1) the risk of adverse neurocognitive and functional outcomes at 5 years of age and (2) predictors of developmental outcomes. Read More

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December 2018

Metabolic requirements of the nephron.

Pediatr Nephrol 2018 Dec 15. Epub 2018 Dec 15.

Rangos Research Center, UPMC Children's Hospital of Pittsburgh, Pittsburgh, USA.

The mammalian kidney is a complex organ that has several metabolically active cell types to aid in waste filtration, salt-water balance, and electrolyte homeostasis in the body. These functions are done primarily through the nephron, which relies on strict regulation of various metabolic pathways. Any deviations in the metabolic profile of nephrons or their precursor cells called nephron progenitors can lead to renal pathologies and abnormal development. Read More

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December 2018
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Efficacy of allergen-blocker mechanical barrier gel on symptoms and quality of life in patients with allergic rhinitis.

Eur Arch Otorhinolaryngol 2018 Dec 15. Epub 2018 Dec 15.

Division of Pediatric Immunology and Allergy, Dokuz Eylul University, Izmir, Turkey.

Propose: Allergic rhinitis (AR) is a very common, chronic and global health problem. In the last two decades, the efficiency of barrier-enforcing measures in AR has been investigated. In this study, we aimed to evaluate the effect of allergen-blocker mechanical barrier gel (MBG) (AlerjiSTOP) treatment on symptoms and quality of life score (QoLS) in patients with seasonal and perennial allergic rhinitis. Read More

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December 2018
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Unhealthy lifestyle and oxidative damage in childhood obesity.

Eat Weight Disord 2018 Dec 15. Epub 2018 Dec 15.

Dietetics and Clinical Nutrition Laboratory, Department of Public Health, Experimental and Forensic Medicine, University of Pavia, Pavia, Italy.

Purpose: Oxidized LDL cholesterol (oxLDL) has been considered as a sensor of oxidative stress (OS) in childhood obesity. We integrated and related our oxLDL existing results previously assessed in overweight/obese children to lifestyle variables to investigate OS-related lifestyle variables.

Methods: 178 Caucasian children/adolescents have been evaluated and according to BMI percentiles have been classified as normal weight (BMI < 75th); overweight (BMI 75-97th) and obese (BMI > 97th). Read More

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December 2018

Treatment failure of syringomyelia associated with Chiari I malformation following foramen magnum decompression: how should we proceed?

Neurosurg Rev 2018 Dec 15. Epub 2018 Dec 15.

Departments of Neurosurgery and Pediatric Neurosurgery, Tel-Aviv Medical Center and Dana Children's Hospital Tel Aviv, Tel Aviv University, 6 Weizmann Street, Tel Aviv, Israel.

The preferred treatment of patients with persistent, recurrent, or progressive syringomyelia after foramen magnum decompression (FMD) for Chiari I (CMI)-associated syringomyelia is controversial, and may include redo FMD, stabilization, or shunting procedures (such as syringopleural or syringo-subarachnoid shunts). We describe our experience in treating these patients and discuss the treatment modalities for these patients. We retrospectively collected data of CMI patients with persistent, recurrent, or progressive syringomyelia after FMD. Read More

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December 2018

Prognostic models for predicting overall survival in metastatic castration-resistant prostate cancer: a systematic review.

World J Urol 2018 Dec 15. Epub 2018 Dec 15.

UroEvidence@Deutsche Gesellschaft für Urologie, Berlin, Germany.

Purpose: Prognostic models are developed to estimate the probability of the occurrence of future outcomes incorporating multiple variables. We aimed to identify and summarize existing multivariable prognostic models developed for predicting overall survival in patients with metastatic castration-resistant prostate cancer (mCRPC).

Methods: The protocol was prospectively registered (CRD42017064448). Read More

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December 2018

Cranioplasties following craniectomies in children-a multicenter, retrospective cohort study.

Childs Nerv Syst 2018 Dec 15. Epub 2018 Dec 15.

Department of Neurosurgery, Division of Neuroscience, Brain Center Rudolf Magnus, University Medical Center Utrecht, Heidelberglaan 100, room G03.124, 3484 CX, Utrecht, The Netherlands.

Objective: Complications following pediatric cranioplasty after craniectomy with either autologous bone flaps or cranial implants are reported to be common, particularly bone flap resorption. However, only sparse data are available regarding cranioplasty strategies, complications, and outcomes. This manuscript describes a Canadian-Dutch multicenter pediatric cohort study with autografts and cranial implant cranioplasties following craniectomies for a variety of indications. Read More

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December 2018

Strategies to control antibiotic resistance: results from a survey in Italian children's hospitals.

Ann Ig 2019 Jan-Feb;31(1):3-12

Medical Direction, Bambino Gesù Children's Hospital, Rome, Italy.

Background: Antimicrobial stewardship programs and comprehensive infection control programs represent the main strategies to limit the emergence and transmission of multi-drug resistant bacteria in hospital settings. The purpose of this study was to describe strategies implemented in Italian children's hospitals for controlling antibiotic resistance.

Study Design: Cross sectional multicenter study. Read More

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December 2018

Molecular Basis of Cystinosis: Geographic Distribution, Functional Consequences of Mutations in the CTNS Gene, and Potential for Repair.

Nephron 2018 Dec 14;141(2):1-14. Epub 2018 Dec 14.

Department of Development and Regeneration, Organ Systems Cluster, KU Leuven, Leuven,

Mutations in the CTNS gene encoding the lysosomal membrane cystine transporter cystinosin are the cause of cystinosis, an autosomal recessive lysosomal storage disease. More than 140 CTNS mutations have been reported worldwide. Recent studies have discovered that cystinosin exerts other key cellular functions beyond cystine transport such as regulation of oxidative state, lysosomal dynamics and autophagy. Read More

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December 2018

Screening and surgery for foramen magnum stenosis in children with achondroplasia: a large, national database analysis.

Authors:

J Neurosurg Pediatr 2018 Dec 14:1-7. Epub 2018 Dec 14.

OBJECTIVEThe goal of this study was to determine the rates of screening and surgery for foramen magnum stenosis in children with achondroplasia in a large, privately insured healthcare network.METHODSRates of screening and surgery for foramen magnum stenosis in children with achondroplasia were determined using de-identified insurance claims data from a large, privately insured healthcare network of over 58 million beneficiaries across the United States between 2001 and 2014. Cases of achondroplasia and screening and surgery claims were identified using a combination of International Classification of Diseases diagnosis codes and Current Procedural Terminology codes. Read More

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December 2018

Childbearing and maternity leave in residency: determinants and well-being outcomes.

Postgrad Med J 2018 Dec 15. Epub 2018 Dec 15.

Department of Medicine, University of Washington School of Medicine, Seattle, Washington, USA.

Purpose: To characterise determinants of resident maternity leave and their effect on maternal and infant well-being. Among non-parents, to identify factors that influence the decision to delay childbearing STUDY DESIGN: In 2016, a survey was sent to female residents at a large academic medical centre on their experiences with maternity leave, the impact of personal and programme factors on length of leave, reasons for delaying childbearing and measures of well-being.

Results: Forty-four percent (214/481) of residents responded. Read More

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December 2018

Health-related quality of life in surgical children and adolescents with congenital heart disease compared with their age-matched healthy sibling: a cross-sectional study from a lower middle-income country, Pakistan.

Arch Dis Child 2018 Dec 15. Epub 2018 Dec 15.

Charles Perkins Centre and Susan Wakil School of Nursing and Midwifery, Sydney Nursing School, University of Sydney, Camperdown, New South Wales, Australia.

Objective: Inconsistencies have been reported in health-related quality of life (HRQOL) in postoperative congenital heart disease (CHD). Despite the need for lifelong care due for residual symptoms, only a few studies have explored cardiac-related HRQOL but none in lower middle-income countries (LMIC). This study therefore addresses the gap by exploring HRQOL and its associated predictors in postoperative CHD in Pakistan. Read More

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December 2018

Lumbar erector spinae plane catheter via a thoracic approach for iliac crest autograft in a pediatric patient.

J Clin Anesth 2018 Dec 13;54:164-165. Epub 2018 Dec 13.

Department of Anesthesiology, Perioperative, and Pain Medicine, Lucile Packard Children's Hospital Stanford, Palo Alto, CA, United States. Electronic address:

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December 2018

Association between prenatal opioid exposure, neonatal opioid withdrawal syndrome, and neurodevelopmental and behavioral outcomes at 5-8 months of age.

Early Hum Dev 2018 Dec 13;128:69-76. Epub 2018 Dec 13.

Department of Family and Community Medicine, School of Medicine, University of New Mexico, Albuquerque, NM, United States of America.

Background: While use of prescription opioids and medication assisted therapy (MAT) for opioid use disorder in pregnancy, as well as the incidence of neonatal opioid withdrawal syndrome (NOWS) continue to rise, little is known about outcomes for children with NOWS beyond the newborn period.

Methods: We examined 1) prenatal MAT exposure vs. unexposed healthy controls [HC]; and 2) treatment for NOWS and NOWS severity on infant neurodevelopmental and behavioral outcomes at 5-8 months of age in 78 maternal-infant pairs from the ENRICH prospective cohort study. Read More

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December 2018

Clinical Exome Sequencing unravels new disease-causing mutations in the myeloproliferative neoplasms: A pilot study in patients from the state of Qatar.

Gene 2018 Dec 13. Epub 2018 Dec 13.

National Center for Cancer Care and Research (NCCCR), HMC, Doha, Qatar.

Clinical Exome Sequencing (CES) has increasingly become a popular diagnostic tool in patients suffering from genetic disorders that are clinically and genetically complicated. Myeloproliferative Neoplasms (MPNs) is an example of a heterogeneous disorder. In Qatar, familial cases of MPNs are more frequently seen than described in the literature. Read More

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December 2018

3D Printing and Heart Failure: The Present and the Future.

JACC Heart Fail 2018 Dec 10. Epub 2018 Dec 10.

Department of Internal Medicine, Division of Cardiology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, New York.

Advanced imaging modalities provide essential anatomic and spatial information in patients with complex heart disease. Two-dimensional imaging can be limited in the extent to which true 3-dimensional (3D) relationships are represented. The application of 3D printing technology has increased the creation of physical models that overcomes the limitations of a 2D screen. Read More

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December 2018
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Home food availability and child intake among rural families identified to be at-risk for health disparities.

Appetite 2018 Dec 13. Epub 2018 Dec 13.

Food Science & Human Nutrition, Colorado State University, United States. Electronic address:

Background: Home food environments (HFE) of children impact dietary intake, though relatively few studies have focused on young children from backgrounds with socioeconomic and racial-ethnic diversity. The objective of the present study was to examine the relationship between the HFE and child dietary intake of preschool-aged children from rural and low-income, culturally diverse families.

Methods: Children (aged 2-5 years) and their primary caregivers (n = 164 parent-child dyads) participated in this study using a cross-sectional design. Read More

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December 2018

Accuracy of IP-10 for diagnosing latent tuberculosis infection: a systematic review and meta-analysis.

Clin Microbiol Infect 2018 Dec 13. Epub 2018 Dec 13.

Department of Pediatrics, West China Second University Hospital, Sichuan University, Chengdu, China; Key Laboratory of Obstetric & Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, Sichuan University, Chengdu, China. Electronic address:

Background: Effective diagnostic methods for detecting latent tuberculosis infection (LTBI) are important for its eradication. A number of studies have evaluated the use of interferon gamma-induced protein 10 (IP-10), which is elevated after tuberculosis infection, as a biomarker for LTBI, but conclusive results regarding its effectiveness have not been reported.

Objectives: Our objective was to assess the diagnostic value of IP-10 for LTBI. Read More

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December 2018

Palatal augmentation prosthesis (PAP) can improve swallowing function for the patients in rehabilitation hospital.

J Prosthodont Res 2018 Dec 12. Epub 2018 Dec 12.

Department of Advanced Prosthodontics, Hiroshima University Graduate School of Biomedical & Health Sciences, Hiroshima, Japan.

Purpose: The aim of this study was to clarify the effects of fitting palatal augmentation prosthesis (PAP) on the swallowing function for the patients in rehabilitation hospital.

Methods: The subjects included 18 elderly hospitalized patients whose body mass index was <18.5kg/m. Read More

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December 2018

TTC7A: Steward of Intestinal Health.

Cell Mol Gastroenterol Hepatol 2018 Dec 13. Epub 2018 Dec 13.

SickKids Inflammatory Bowel Disease Center and Cell Biology Program, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada; Division of Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Institute for Medical Science and Biochemistry, University of Toronto, Hospital for Sick Children, Toronto, Ontario, Canada. Electronic address:

The rising incidence of paediatric inflammatory bowel disease (IBD), coupled with the efficiency of whole exome sequencing, has led to the identification of Tetratricopeptide Repeat Domain 7A (TTC7A) as a steward of intestinal health. TTC7A-deficiency is an autosomal recessively inherited disease. In five years since the original description more than 50 patients with >20 distinct disease-causing TTC7A mutations have been identified. Read More

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December 2018
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Efficacy and safety of lipoprotein apheresis in children with homozygous familial hypercholesterolemia: A systematic review.

J Clin Lipidol 2018 Nov 3. Epub 2018 Nov 3.

Department of Paediatric Medicine, Amsterdam UMC, University of Amsterdam, the Netherlands.

Background: Homozygous familial hypercholesterolemia (HoFH) is a rare genetic disorder that may cause life-threatening cardiovascular disease (CVD) at childhood. Marginal effectiveness of statins in reducing low-density lipoprotein cholesterol (LDL-C) is the reason why extracorporeal removal of LDL-C by lipoprotein apheresis (LA) is recommended at the earliest possible age.

Objective: It is, however, unknown to what extent LA effectively reduces the burden of CVD in children with HoFH. Read More

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November 2018

Comparison of multiplex real-time polymerase chain reaction with serological tests and culture for diagnosing human brucellosis.

J Infect Public Health 2018 Dec 12. Epub 2018 Dec 12.

Ankara Yıldırım Beyazıt University, Faculty of Medicine, Department of Clinical Microbiology, Ankara, Turkey. Electronic address:

Objective: Brucellosis is a zoonotic disease with various clinical presentations and early diagnosis is crucial to avoid severe complications. Due to limitations of conventional diagnostic methods, polymerase chain reaction (PCR) based approaches have gained importance in diagnosis.We aimed to evaluate diagnostic value of multiplex real time-PCR (mRT-PCR) in serum samples collected from brucellosis suspected patients by comparision sensitivity of mRT-PCR with those of conventional diagnostic methods. Read More

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December 2018

A New Concern: Persistent Coronary Artery Compression After Balloon Testing During Transcatheter Pulmonary Valve Replacement.

JACC Cardiovasc Interv 2018 Dec 7. Epub 2018 Dec 7.

The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio; Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio. Electronic address:

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December 2018

Sinus Venosus Defects: Anatomic Variants and Transcatheter Closure Feasibility Using Virtual Reality Planning.

JACC Cardiovasc Imaging 2018 Dec 6. Epub 2018 Dec 6.

Department of Pediatrics (Cardiology), University of Texas Southwestern Medical Center, Children's Medical Center Dallas, Dallas, Texas; Department of Radiology, University of Texas Southwestern Medical Center, Children's Medical Center Dallas, Dallas, Texas.

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December 2018

Cough and airway clearance in Duchenne muscular dystrophy.

Paediatr Respir Rev 2018 Nov 24. Epub 2018 Nov 24.

Department of Pediatrics, S. Orsola-Malpighi Hospital, University of Bologna, Via Massarenti 11, 40138 Bologna, Italy. Electronic address:

People with Duchenne muscular dystrophy (DMD), develop a respiratory muscle weakness that results in weakened cough, airway clearance impairment and over time respiratory failure and death. Assessment of cough effectiveness through vital capacity, peak cough flow and maximal inspiratory and expiratory pressures has been used to identify the optimal timing of cough augmentation techniques initiation. The choice of therapies depends on physician knowledge, and patient/care giver abilities. Read More

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November 2018
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Prospective Validation of an mRNA-based Urine Test for Surveillance of Patients with Bladder Cancer.

Eur Urol 2018 Dec 12. Epub 2018 Dec 12.

Radboudumc, Nijmegen, The Netherlands.

Background: A fast, noninvasive test with high sensitivity (SN) and a negative predictive value (NPV), which is able to detect recurrences in bladder cancer (BC) patients, is needed. A newly developed urine assay, Xpert Bladder Cancer Monitor (Xpert), measures five mRNA targets (ABL1, CRH, IGF2, UPK1B, and ANXA10) that are frequently overexpressed in BC.

Objective: To validate Xpert characteristics in patients previously diagnosed with non-muscle-invasive BC. Read More

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December 2018

Anesthetic Considerations for Pediatric Patients With Congenital Long QT Syndrome.

J Cardiothorac Vasc Anesth 2018 Nov 12. Epub 2018 Nov 12.

Department of Anesthesiology and Critical Care Medicine, Johns Hopkins University School of Medicine, Johns Hopkins All Children's Hospital, St. Petersburg, FL.

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November 2018

AVIATOR: An open international registry to evaluate medical and surgical outcomes of aortic valve insufficiency and ascending aorta aneurysm.

J Thorac Cardiovasc Surg 2018 Oct 26. Epub 2018 Oct 26.

Department of Cardiac Surgery, Institut Mutualiste Montsouris, Paris, France.

Objectives: Current national registries are lacking detailed pathology-driven analysis and long-term patients outcomes. The Heart Valve Society (HVS) aortic valve (AV) repair research network started the Aortic Valve Insufficiency and ascending aorta Aneurysm InternATiOnal Registry (AVIATOR) to evaluate long-term patient outcomes of AV repair and replacement. The purpose of the current report is to describe the AVIATOR initiative and report in a descriptive manner the patients included. Read More

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October 2018

Singling out single ventricles after Fontan.

J Thorac Cardiovasc Surg 2018 Nov 15. Epub 2018 Nov 15.

Department of Pediatrics and Duke Clinical Research Institute, Duke University School of Medicine, Durham, NC. Electronic address:

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November 2018

Maternal reading fluency is associated with functional connectivity between the child's future reading network and regions related to executive functions and language processing in preschool-age children.

Brain Cogn 2018 Dec 12. Epub 2018 Dec 12.

Reading and Literacy Discovery Center, Pediatric Neuroimaging Research Consortium, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA; Educational Neuroimaging Center, Faculty of Education in Science and Technology, Technion, Haifa, Israel. Electronic address:

Reading is an acquired skill that relies on cognitive-control and language abilities. Home reading environment has been positively correlated with activation in parietal-temporal-occipital association cortex supporting mental imagery and narrative comprehension during a story-listening task in preschool-age children. However, the degree to which maternal reading ability influences early brain development, specifically neural circuits involved with language and reading, is not well understood. Read More

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December 2018

Surgical validation of functional magnetic resonance urography in the study of ureteropelvic junction obstruction in a pediatric cohort.

J Pediatr Urol 2018 Nov 22. Epub 2018 Nov 22.

Pediatric Surgery Department, IRCCS, Istituto Giannina Gaslini, Largo Gaslini 5, 16147, Genoa, Italy; DINOGMI, University of Genoa, Genoa, Italy. Electronic address:

Introduction: Ureteropelvic junction obstruction (UPJO) is one of the most common urological diseases in children. The etiology can be intrinsic, extrinsic (crossing vessel [CV] or adhesions), or mixed. To date, ultrasonography and scintigraphy are considered gold-standard imaging techniques for the study of UPJO. Read More

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November 2018

Involuntary Eye Movements Accompanied by Head Thrusting to View Objects.

Pediatr Neurol 2018 Oct 23. Epub 2018 Oct 23.

Department of Pediatrics, Jichi Medical University, Shimotsuke, Japan.

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October 2018

A Pilot Study of Soluble Form of LOX-1 as a Novel Biomarker for Neonatal Hypoxic-Ischemic Encephalopathy.

J Pediatr 2018 Dec 11. Epub 2018 Dec 11.

Department of Mental Retardation and Birth Defect Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Tokyo, Japan. Electronic address:

Objective: To evaluate the soluble form of lectin-like oxidized low-density lipoprotein receptor-1 (sLOX-1) as a biomarker of severity staging and prognosis in neonatal hypoxic-ischemic encephalopathy (HIE).

Study Design: We performed an observational study enrolling 27 infants with HIE and 45 control infants of gestational age ≥36 weeks and birth weight ≥1800 g. The HIE criteria were pH ≤7. Read More

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December 2018

The Medical Home for Children with Medical Complexity: Back to Basics.

Authors:
Dennis Z Kuo

J Pediatr 2018 Dec 13. Epub 2018 Dec 13.

Department of Pediatrics University at Buffalo Buffalo, New York. Electronic address:

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December 2018

Primary aneurysmal bone cyst with a novel SPARC-USP6 translocation identified by next-generation sequencing.

Cancer Genet 2018 Dec 20;228-229:12-16. Epub 2018 Jul 20.

Faculty of Medicine, Institute of Pathology, University of Ljubljana, Korytkova 2, 1000 Ljubljana, Slovenia.

Aneurysmal bone cyst (ABC) is a benign but locally aggressive, mostly pediatric neoplasm, with characteristic USP6 gene rearrangement that distinguishes it from a secondary ABC and other primary bone tumors. With the advent of next-generation sequencing (NGS) technology, several hitherto unknown USP6 fusion partners have been identified in ABC. Accordingly, we present a case of an 18-year-old male with a solid sub-periosteal primary ABC in the diaphysis of the left femur. Read More

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December 2018

A new complex rearrangement in infant ALL: t(X;11;17)(p11.2;q23;q12).

Cancer Genet 2018 Dec 28;228-229:110-114. Epub 2018 Oct 28.

Department of Pediatrics, Ribeirão Preto School of Medicine, University of São Paulo, Av. Bandeirantes 3900, Bairro Monte Alegre, Ribeirão Preto, SP CEP 14040-900, Brazil.

We present a case of an infant who developed pro-B acute lymphoblastic leukemia with a rare and complex MLL-translocation. Cytogenetic analysis of bone marrow cells at diagnosis showed a 46,XY,t(X;11)(p11.2;q23)[13]/46,XY[7] karyotype. Read More

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December 2018

B23/Nucleophosmin promotes reconstitution of synaptic path in hippocampus after injury.

Biochem Biophys Res Commun 2018 Dec 12. Epub 2018 Dec 12.

Department of Molecular Cell Biology, Sungkyunkwan University School of Medicine, Suwon, 16419, South Korea; Single Cell Network Research Center, Sungkyunkwan University School of Medicine, Suwon, 16419, South Korea; Samsung Biomedical Research Institute, Samsung Medical Center, Seoul, 06351, South Korea. Electronic address:

B23, also known as nucleophosmin (NPM), is multifunctional protein directly implicated in cell proliferation, cell cycle progression, and cell survival. In the current study, in addition to confirming its anti-apoptotic function in neuronal survival, we demonstrated that the spatial-temporal expression profile of B23 during development of hippocampal neurons is high in the embryonic stage, down-regulated after birth, and preferentially localized at the tips of growing neuritis and branching points. Overexpression of B23 promotes axon growth with abundant branching points in growing hippocampal neurons, but depletion of B23 impairs axon growth, leading to neuronal death. Read More

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December 2018

IL-17A modulates osteoclast precursors' apoptosis through autophagy-TRAF3 signaling during osteoclastogenesis.

Biochem Biophys Res Commun 2018 Dec 12. Epub 2018 Dec 12.

Academy of Orthopedics, The Third Affiliated Hospital, Southern Medical University, Guangzhou, 510630, China. Electronic address:

Osteoclasts play an important role in bone remodeling. The inflammatory cytokine IL-17A could modulate the RANKL-induced osteoclastogenesis by regulating the autophagic activity. It is well accepted that protective autophagy has an anti-apoptotic effect. Read More

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December 2018

Novel mTORC1 Mechanism Suggests Therapeutic Targets for COMPopathies.

Am J Pathol 2018 Dec 7. Epub 2018 Dec 7.

Department of Pediatrics, McGovern Medical School, University of Texas Health Science Center, Houston, Texas; School of Dentistry, University of Texas Health Science Center, Houston, Texas.

Cartilage oligomeric matrix protein (COMP) is a large, multifunctional extracellular protein that, when mutated, is retained in the rough endoplasmic reticulum (ER). This retention elicits ER stress, inflammation, and oxidative stress, resulting in dysfunction and death of growth plate chondrocytes. While identifying the cellular pathologic mechanisms underlying the murine mutant (MT)-COMP model of pseudoachondroplasia, increased midline-1 (MID1) expression and mammalian target of rapamycin complex 1 (mTORC1) signaling was found. Read More

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December 2018

Seeking to improve care for young patients: Development of tools to support the implementation of the CDC Pediatric mTBI Guideline.

J Safety Res 2018 Dec 1;67:203-209. Epub 2018 Oct 1.

Centers for Disease Control and Prevention, National Center for Injury Prevention and Control, Division of Unintentional Injury Prevention, 1600 Clifton Road, Atlanta, GA 30329-4027, United States.

Introduction: The Centers for Disease Control and Prevention (CDC) Pediatric Mild Traumatic Brain Injury (mTBI) Guideline was created to help standardize diagnosis, prognosis, and management and treatment of pediatric mTBI. This paper describes the process CDC used to develop educational tools, and a dissemination and implementation strategy, in support of the CDC Pediatric mTBI Guideline.

Methods: Two qualitative data collection projects with healthcare providers who care for pediatric patients were conducted. Read More

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December 2018

LARGE expression in different types of muscular dystrophies other than dystroglycanopathy.

BMC Neurol 2018 Dec 15;18(1):207. Epub 2018 Dec 15.

Department of Medical Biology, Hacettepe University Faculty of Medicine, 06100 Sihhiye, Ankara, Turkey.

Background: Alpha-dystroglycan (αDG) is an extracellular peripheral glycoprotein that acts as a receptor for both extracellular matrix proteins containing laminin globular domains and certain arenaviruses. An important enzyme, known as Like-acetylglucosaminyltransferase (LARGE), has been shown to transfer repeating units of -glucuronic acid-β1,3-xylose-α1,3- (matriglycan) to αDG that is required for functional receptor as an extracellular matrix protein scaffold. The reduction in the amount of LARGE-dependent matriglycan result in heterogeneous forms of dystroglycanopathy that is associated with hypoglycosylation of αDG and a consequent lack of ligand-binding activity. Read More

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December 2018

Molecular characterization and antimicrobial susceptibility of Staphylococcus aureus isolated from children with acute otitis media in Liuzhou, China.

BMC Pediatr 2018 Dec 15;18(1):388. Epub 2018 Dec 15.

Children's Hospital of Michigan, Detroit, MI, USA.

Background: There have been few studies focused on the prevalence, bacterial etiology, antibiotic resistance, and genetic background of Staphylococcus aureus (S. aureus) in children with acute otitis media (AOM) in China.

Methods: A retrospective study was conducted in Liuzhou Maternity and Child Healthcare Hospital. Read More

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December 2018

Preterm Prelabor Rupture of Membranes: Outcomes with Expectant Management until 34 versus 35 Weeks.

Am J Perinatol 2018 Dec 15. Epub 2018 Dec 15.

Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, University of Rochester Medical Center, Rochester, New York.

Objective:  To evaluate outcomes with expectant management of preterm prelabor rupture of membranes (PROM) until 35 weeks versus immediate delivery at ≥34 weeks.

Study Design:  This was a multicenter retrospective cohort study of singletons with preterm PROM at >20 weeks from 2011 through 2017. Groups were defined as expectant management until 35 weeks versus immediate delivery at ≥34 weeks. Read More

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December 2018
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