135,795 results match your criteria Pediatric Blood & Cancer[Journal]


The hemostasis system in children with hereditary spherocytosis.

Thromb Res 2019 Feb 5;176:11-17. Epub 2019 Feb 5.

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia; Pirogov Russian National Research Medical University, Moscow, Russia.

Introduction: Patients with hereditary spherocytosis (HS) are characterized by having an increased risk for thrombosis. An early manifestation of thrombotic complications can occur even in childhood, especially after surgery. Hypercoagulability can be associated with hemolytic crises. Read More

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http://dx.doi.org/10.1016/j.thromres.2019.02.004DOI Listing
February 2019

Association betweenPNPLA3rs738409, polymorphism, decreased kidney function in postmenopausal type 2, diabetic women with or without non-alcoholic fatty liver disease.

Diabetes Metab 2019 Feb 11. Epub 2019 Feb 11.

Division of Endocrinology, Diabetes and Metabolism, Department of Medicine, University Hospital of Verona, Verona, Italy. Electronic address:

Aim: - Evidence is emerging that PNPLA3 rs738409 polymorphism (the major genetic variant associated with susceptibility to non-alcoholic fatty liver disease [NAFLD]) is associated with chronic kidney disease (CKD) in non-diabetic individuals. Currently, little is known about this association in type 2 diabetic (T2DM) patients with and without NAFLD.

Methods: - We studied 101 Caucasian post-menopausal women with T2DM, consecutively attending our diabetes outpatient service during a 3-month period. Read More

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http://dx.doi.org/10.1016/j.diabet.2019.01.011DOI Listing
February 2019

Non-inflammatory emphysema induced by NO chronic exposure and intervention with demethylation 5-Azacytidine.

Life Sci 2019 Feb 11. Epub 2019 Feb 11.

State Key Laboratory of Respiratory Disease, Guangdong Key Laboratory of Vascular Disease, Guangzhou Institute of Respiratory Disease, The First Affiliated Hospital, Guangzhou Medical University, Guangzhou, Guangdong 510180, PR China; Division of Translational and Regenerative Medicine, Department of Medicine, The University of Arizona, Tucson, AZ 85721-0202, USA; Department of Laboratory Medicine, The First Affiliated Hospital, Guangzhou Medical University, Guangzhou, Guangdong 510180, PR China. Electronic address:

Aims: A rat model of emphysema was established that mimics the features of the human emphysema subtype and explores the effects of demethylation on lung function and blood tests.

Materials And Methods: Rats were randomly assigned to NO, NO + 5-Azacytidine, and normal air groups based on a emphysema rat model induced by chronic NO exposure. This study estimates the characteristics of emphysema by conducting an analysis for IL-6 and TNF-α levels in bronchoalveolar lavage fluids (BALF) and plasma. Read More

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http://dx.doi.org/10.1016/j.lfs.2019.02.022DOI Listing
February 2019

Pharmacokinetics of morphine in encephalopathic neonates treated with therapeutic hypothermia.

PLoS One 2019 14;14(2):e0211910. Epub 2019 Feb 14.

Department of Clinical Pharmacy, University Medical Center Utrecht, Utrecht, the Netherlands.

Objective: Morphine is a commonly used drug in encephalopathic neonates treated with therapeutic hypothermia after perinatal asphyxia. Pharmacokinetics and optimal dosing of morphine in this population are largely unknown. The objective of this study was to describe pharmacokinetics of morphine and its metabolites morphine-3-glucuronide and morphine-6-glucuronide in encephalopathic neonates treated with therapeutic hypothermia and to develop pharmacokinetics based dosing guidelines for this population. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0211910PLOS
February 2019

Reproductive characteristics modify the association between global DNA methylation and breast cancer risk in a population-based sample of women.

PLoS One 2019 14;14(2):e0210884. Epub 2019 Feb 14.

Department of Epidemiology, University of North Carolina, Chapel Hill, NC, United States of America.

DNA methylation has been implicated in breast cancer aetiology, but little is known about whether reproductive history and DNA methylation interact to influence carcinogenesis. This study examined modification of the association between global DNA methylation and breast cancer risk by reproductive characteristics. A population-based case-control study assessed reproductive history in an interviewer-administered questionnaire. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0210884PLOS
February 2019

Coagulation Changes induced by Lower Body Negative Pressure in Men and Women.

J Appl Physiol (1985) 2019 Feb 14. Epub 2019 Feb 14.

Physiology, Medical University of Graz, Austria.

We investigated whether lower body negative pressure (LBNP) application leads to coagulation activation in whole blood (WB) samples in healthy men and women. Twenty-four women and twenty-one men, all healthy young participants, with no histories of thrombotic disorders and not on medications, were included. LBNP was commenced at -10 mmHg and increased by -10 mmHg every-five minutes until a maximum of -40 mmHg. Read More

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http://dx.doi.org/10.1152/japplphysiol.00940.2018DOI Listing
February 2019

Recommendations for ultrasonographic assessment of renal arteries.

J Ultrason 2018 ;18(75):338-343

Department of General Surgery and Endocrinology, Medical University of Warsaw , Poland.

The present clinical practice recommendations are addressed to physicians of all specialties, who perform Doppler ultrasound examinations of the kidneys on a daily basis, and in particular to specialists in radiology and imaging diagnostics. The recommendations were based on the Ultrasonography Standards of the Polish Ultrasound Society and current scientific reports consistent with Evidence Based Medicine. The paper discusses patient preparation protocol, examinat ion technique with particular emphasis on patient's position allowing to obtain proper Doppler angle of insonation, as well as diagnostic limitations of the technique. Read More

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http://dx.doi.org/10.15557/JoU.2018.0049DOI Listing
January 2018

The role of CNS macrophages in streptococcal meningoencephalitis.

J Leukoc Biol 2019 Feb 14. Epub 2019 Feb 14.

Institute for Immunodeficiency, Center for Chronic Immunodeficiency (CCI), Medical Center and Faculty of Medicine, University of Freiburg, Freiburg, Germany.

In the healthy brain, microglia and other CNS macrophages are the most abundant immune cell type. Thus, they form the natural immune cell interface with streptococci, which are the leading cause of bacterial meningitis and encephalitis in infants and young children. In homeostasis, the blood-brain barrier allows for very limited access of immune cells circulating in the periphery. Read More

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http://dx.doi.org/10.1002/JLB.4MR1118-419RDOI Listing
February 2019

Allogeneic blood transfusion in 163 children with acute lymphocytic leukemia (a STROBE-compliant article).

Medicine (Baltimore) 2019 Feb;98(7):e14518

Department of Pediatrics, the First Affiliated Hospital of Zhengzhou University.

Little research has been done about the effects of allogeneic blood transfusion (ABT) on the recurrence and prognosis in the cases with childhood acute lymphocytic leukemia (cALL). In order to provide a basis for clinical safe blood transfusion, the data of 163 cases with cALL were retrospectively analyzed to explore the issue.The data of 163 cases with cALL between 2006 and 2011 were retrospectively analyzed. Read More

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http://dx.doi.org/10.1097/MD.0000000000014518DOI Listing
February 2019

Neuroimaging Abnormalities in Patients With Nontransfusion-dependent Thalassemia.

J Pediatr Hematol Oncol 2019 Feb 12. Epub 2019 Feb 12.

Pediatric Hematology Oncology Unit, Advanced Pediatric Centre.

Independence from regular transfusions is the hallmark of nontransfusion-dependent thalassemia (NTDT). However, the associated complications need anticipation and screening. One such complication is a hypercoagulable state predisposing to development of thrombosis. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001432DOI Listing
February 2019

Metabolic Monitoring of Child and Adolescent Patients on Atypical Antipsychotics by Psychiatrists and Primary Care Providers.

Am J Ther 2019 Feb 7. Epub 2019 Feb 7.

Department of Psychiatry, McLeod Medical Center, Florence, SC.

Background: Antipsychotic drug use in children has doubled from 2001 to 2007 with concomitant increase in obesity. Second-generation antipsychotic (SGA) medication use is associated with weight gain, metabolic derangements, and blood sugar and lipid abnormalities in children. The American Psychiatric Association and the American Diabetes Association have recommended metabolic monitoring guidelines for patients using SGA. Read More

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http://dx.doi.org/10.1097/MJT.0000000000000853DOI Listing
February 2019

Experimental pediatric stroke shows age-specific recovery of cognition and role of hippocampal Nogo-A receptor signaling.

J Cereb Blood Flow Metab 2019 Feb 14:271678X19828581. Epub 2019 Feb 14.

1 Department of Anesthesiology, University of Colorado School of Medicine, Aurora, CO, USA.

Ischemic stroke is a leading cause of death worldwide and clinical data suggest that children may recover from stroke better than adults; however, supporting experimental data are lacking. We used our novel mouse model of experimental juvenile ischemic stroke (MCAO) to characterize age-specific cognitive dysfunction following ischemia. Juvenile and adult mice subjected to 45-min MCAO, and extracellular field recordings of CA1 neurons were performed to assess hippocampal synaptic plasticity changes after MCAO, and contextual fear conditioning was performed to evaluate memory and biochemistry used to analyze Nogo-A expression. Read More

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http://dx.doi.org/10.1177/0271678X19828581DOI Listing
February 2019

Aspirin in childhood acute ischemic stroke: The evidence for treatment and efficacy testing.

Pediatr Blood Cancer 2019 Feb 14:e27665. Epub 2019 Feb 14.

Department of Pediatrics, Cancer and Blood Diseases Institute, Cincinnati Children's Hospital, Cincinnati, Ohio.

Aspirin is the most commonly prescribed antiplatelet agent worldwide, but evidence supporting its use varies by age and disease process. Despite its frequent use in childhood acute ischemic stroke prevention and management, major knowledge gaps exist about optimal pediatric aspirin use, particularly in this setting, where high-quality clinical trials are urgently needed. This review focuses upon the evidence for aspirin use in childhood acute ischemic stroke, includes a summary of aspirin pharmacology to highlight misconceptions and common clinical situations which may limit its efficacy, and discusses the techniques and potential role of laboratory monitoring of aspirin efficacy in children. Read More

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http://dx.doi.org/10.1002/pbc.27665DOI Listing
February 2019

Rhabdomyosarcoma diagnosed in the first year of life: Localized, metastatic, and relapsed disease. Outcome data from five trials and one registry of the Cooperative Weichteilsarkom Studiengruppe (CWS).

Pediatr Blood Cancer 2019 Feb 14:e27652. Epub 2019 Feb 14.

Department for Children and Adolescents, University Hospital of Frankfurt, Frankfurt/M., Germany.

Background: Rhabdomyosarcoma (RMS) diagnosed during the first year of life is reported to have poor outcome. Little is known about treatment and outcome data of relapsed disease (RD).

Methods: Characteristics, treatment, and outcome of 155 patients ≤ 12 months registered within the Cooperative Weichteilsarkom Studiengruppe (CWS) between 1981 and 2016 were evaluated. Read More

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http://doi.wiley.com/10.1002/pbc.27652
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http://dx.doi.org/10.1002/pbc.27652DOI Listing
February 2019
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Morphometric demonstration of portal vein stenosis and hepatic arterial medial hypertrophy in patients with biliary atresia.

Pediatr Surg Int 2019 Feb 14. Epub 2019 Feb 14.

Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University, 8-35-1, Sakuragaoka, Kagoshima, 890-8520, Japan.

Purpose: Portal hypertension in patients with biliary atresia (BA) is generally thought to result from portal vein (PV) narrowing secondary to hepatic fibrosis. To test the hypothesis, we morphometrically analyzed the PVs and hepatic arteries (HAs).

Methods: Morphometrical analyses of 25 BA and 26 non-BA liver biopsy specimens from patients treated from 2000 to 2014. Read More

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http://dx.doi.org/10.1007/s00383-019-04459-4DOI Listing
February 2019

Levels of Melatonin in Continuous Spikes and Waves During Sleep.

J Child Neurol 2019 Feb 14:883073819828580. Epub 2019 Feb 14.

1 Department of Pediatric Neurology, School of Medicine, Celal Bayar University, Manisa, Turkey.

Levels of melatonin have been reported before in children with epilepsy, but such has not been reported to date in those with continuous spikes and waves during sleep. The aim of the present study was to assess serum melatonin levels and melatonin circadian rhythm in patients with continuous spikes and waves during sleep and epilepsy. Serum melatonin was measured in 39 children stratified into 3 groups. Read More

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http://dx.doi.org/10.1177/0883073819828580DOI Listing
February 2019

Prevalence of chronic hepatitis C virus infection among childhood cancer survivors in Stockholm, Sweden.

Acta Oncol 2019 Feb 14:1-6. Epub 2019 Feb 14.

a Department of Clinical Science, Intervention and Technology (CLINTEC) , Karolinska Institutet , Stockholm , Sweden.

Background: Childhood cancer survivors treated before 1992, when blood donor screening for hepatitis C virus (HCV) infection was introduced, are at risk of transfusion-transmitted HCV infection. A national HCV screening campaign targeting blood transfusion recipients was launched in Sweden in 2007-2010. The aims of this study were to, among adult childhood cancer survivors in Stockholm County, investigate the prevalence of HCV infection, the natural course of infection, treatment outcome and anti-HCV testing frequency before, during and after the screening campaign and finally to actively screen the untested ones. Read More

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https://www.tandfonline.com/doi/full/10.1080/0284186X.2019.1
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http://dx.doi.org/10.1080/0284186X.2019.1574105DOI Listing
February 2019
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The diagnostic accuracy and clinical utility of pediatric renal tumor biopsy: Report of the UK experience in the SIOP UK WT 2001 trial.

Pediatr Blood Cancer 2019 Feb 13:e27627. Epub 2019 Feb 13.

Department of Cellular Pathology, University Hospital of Wales, Cardiff, UK.

Introduction: The International Society of Paediatric Oncology (SIOP) protocols recommend preoperative chemotherapy appropriate for Wilms tumors (WTs) in children with renal tumors aged ≥6 months, reserving biopsy for "atypical" cases. The Children's Cancer and Leukaemia Group (CCLG) joined the SIOP-WT-2001 study but continued the national practice of biopsy at presentation.

Method: Retrospective study of concordance between locally reported renal tumor biopsies and central pathology review nephrectomy diagnoses of children enrolled by CCLG centers in the SIOP-WT-2001 study. Read More

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http://dx.doi.org/10.1002/pbc.27627DOI Listing
February 2019

Review of Leipzig protocol for intravenous insulin infusion in pediatric patients with type 1 diabetes during intercurrent illness and surgery.

Pediatr Diabetes 2019 Feb 13. Epub 2019 Feb 13.

Department of Endocrinology and Diabetology, University of Leipzig, Hospital for Children and Adolescents, 04103 Leipzig, Sachsen, Germany.

Objective: Continuous intravenous (IV) insulin infusion therapy minimizes blood glucose (BG) fluctuations and prevents metabolic deterioration in pediatric patients with type 1 diabetes (T1D) during intercurrent illness and surgery. However, data on the adequate fluid and insulin substitution in this situation is rare. We evaluated the effectiveness and safety of IV insulin therapy according to our local protocol. Read More

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http://dx.doi.org/10.1111/pedi.12830DOI Listing
February 2019

Post-extubation continuous positive airway pressure improves oxygenation after pediatric laparoscopic surgery: A randomized controlled trial.

Acta Anaesthesiol Scand 2019 Feb 14. Epub 2019 Feb 14.

Anesthesia and Intensive Care Department, Faculty of Medicine, Assiut University, Assiut, Egypt.

Background: Effects of intraoperative recruitment maneuvers (RMs) on oxygenation and pulmonary compliance are lost during recovery if high inspired oxygen and airway suctioning are used. We investigated the effect of post-extubation noninvasive CPAP mask application on the alveolar arterial oxygen difference [(A-a) DO ] after pediatric laparoscopic surgery.

Methods: Sixty patients (1-6 years) were randomly allocated to three groups of 20 patients, to receive zero end-expiratory pressure (ZEEP group), RM with decremental PEEP titration only (RM group), or followed with post-extubation CPAP for 5 minutes (RM-CPAP group). Read More

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http://dx.doi.org/10.1111/aas.13324DOI Listing
February 2019

Twenty-four-hour urine NE level as a predictor of the therapeutic response to metoprolol in children with recurrent vasovagal syncope.

Ir J Med Sci 2019 Feb 13. Epub 2019 Feb 13.

Department of Pediatrics, Qilu Hospital, Shandong University, No. 107 Wenhuaxi Road, Jinan, 250012, Shandong Province, China.

Background: Vasovagal syncope (VVS) is a heterogeneous disorder that creates challenges for treatment. Metoprolol is an important therapeutic option for children with VVS.

Aims: The study examined the predictive value of 24-h urine norepinephrine (NE) levels in the assessment of the therapeutic efficacy of metoprolol for recurrent VVS in children. Read More

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http://dx.doi.org/10.1007/s11845-019-01979-9DOI Listing
February 2019

Volumetric Assessment of Pediatric Vascular Malformations Using a Rapid, Hand-Held Three-Dimensional Imaging System.

J Digit Imaging 2019 Jan 31. Epub 2019 Jan 31.

George W. Woodruff School of Mechanical Engineering, Georgia Institute of Technology, Atlanta, GA, USA.

The effect of percutaneous, surgical, and medical therapies for vascular malformations (VMs) is often difficult to quantify volumetrically using cross-sectional imaging. Volumetric measurement is often estimated with serial, expensive MRI examinations which may require sedation or anesthesia. We aim to explore whether a portable 3D scanning device is capable of rapid, accurate volumetric analysis of pediatric VMs. Read More

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http://dx.doi.org/10.1007/s10278-019-00183-6DOI Listing
January 2019

Preclinical evaluation of a novel anterior non-fusion fixation device for atlantoaxial instability: an in vivo comparison study in a canine model.

Eur Spine J 2019 Feb 13. Epub 2019 Feb 13.

Department of Orthopaedic Surgery, Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, People's Republic of China.

Purpose: The Anterior Atlantoaxial Non-Fusion Fixation System (AANFS) was a novel motion preservation device for atlantoaxial instability to replace traditional fusion techniques. The purpose of this in vivo study was to evaluate the clinical features and biomechanical properties of this new device in a canine model by comparing it with a conventional method.

Methods: Eighteen adult male canines were randomly divided into group 1, which received the AANFS replacement, group 2 which received the Harms rigid fixation procedures, and group 3, which served as the control group. Read More

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http://dx.doi.org/10.1007/s00586-019-05916-3DOI Listing
February 2019

Potential and Limitations of Atrial Natriuretic Peptide as Biomarker in Pediatric Heart Failure-A Comparative Review.

Front Pediatr 2018 29;6:420. Epub 2019 Jan 29.

Institute of Clinical Pharmacy and Pharmacotherapy, Heinrich-Heine-University, Düsseldorf, Germany.

Although B-type Natriuretic Peptide (BNP), N-terminal-proBNP (NT-proBNP), and mid-regional-proANP (MR-proANP) are included in current guidelines on heart failure in adults, no guideline considering these biomarkers in pediatric heart failure is available. A new drug class of neprilysin inhibitors as fixed-dose combination (Sacubitril/valsartan) has been introduced and is currently being investigated in children suffering from heart failure. Atrial Natriuretic Peptide (ANP) is discussed as a more useful alternative to BNP because it may grants better insights into the effects of this treatment. Read More

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http://dx.doi.org/10.3389/fped.2018.00420DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6361796PMC
January 2019

Coarctation of Aorta in Children.

Cureus 2018 Dec 5;10(12):e3690. Epub 2018 Dec 5.

Pediatric Cardiology, Seattle Children's Hospital, University of Washington School of Medicine, Seattle, USA.

Coarctation of aorta (CoA) is a discrete narrowing in aorta causing obstruction to the flow of blood. It accounts for 6-8% of all congenital heart diseases. With advances in fetal echocardiography rate of prenatal diagnosis of coarctation of aorta has improved but it still remains a challenging diagnosis to make prenatally. Read More

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http://dx.doi.org/10.7759/cureus.3690DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6368362PMC
December 2018

Acquired and Innate Immunity Impairment and Severe Disseminated Infection in a Patient With a NF-κB1 Deficiency.

Front Immunol 2018 29;9:3148. Epub 2019 Jan 29.

Research Institute Hospital 12 Octubre (I+12), Madrid, Spain.

NF-κB1 is a master regulator of both acquired and innate responses. loss-of-function mutations elicit a wide clinical phenotype with asymptomatic individuals at one end of the spectrum and patients with common variable immunodeficiency, combined immunodeficiency or autoinflammation at the other. Impairment of acquired and innate immunity and disseminated infection expands the clinical and immunological phenotype of NF-κB1 deficiency. Read More

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http://dx.doi.org/10.3389/fimmu.2018.03148DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6362422PMC
January 2019

Evidence for Innate and Adaptive Immune Responses in a Cohort of Intractable Pediatric Epilepsy Surgery Patients.

Front Immunol 2019 29;10:121. Epub 2019 Jan 29.

Department of Neurosurgery, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, United States.

Brain-infiltrating lymphocytes (BILs) were isolated from resected brain tissue from 10 pediatric epilepsy patients who had undergone surgery for Hemimegalencephaly (HME) ( = 1), Tuberous sclerosis complex (TSC) ( = 2), Focal cortical dysplasia (FCD) ( = 4), and Rasmussen encephalitis (RE) ( = 3). Peripheral blood mononuclear cells (PBMCs) were also isolated from blood collected at the time of the surgery. Cells were immunostained with a panel of 20 antibody markers, and analyzed by mass cytometry. Read More

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http://dx.doi.org/10.3389/fimmu.2019.00121DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6362260PMC
January 2019

Diabetes relief in mice by glucose-sensing insulin-secreting human α-cells.

Nature 2019 Feb 13. Epub 2019 Feb 13.

Department of Genetic Medicine and Development, iGE3 and Centre Facultaire du Diabète, Faculty of Medicine, University of Geneva, Geneva, Switzerland.

Cell-identity switches, in which terminally differentiated cells are converted into different cell types when stressed, represent a widespread regenerative strategy in animals, yet they are poorly documented in mammals. In mice, some glucagon-producing pancreatic α-cells and somatostatin-producing δ-cells become insulin-expressing cells after the ablation of insulin-secreting β-cells, thus promoting diabetes recovery. Whether human islets also display this plasticity, especially in diabetic conditions, remains unknown. Read More

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http://dx.doi.org/10.1038/s41586-019-0942-8DOI Listing
February 2019

High frequency of shared clonotypes in human B cell receptor repertoires.

Nature 2019 Feb 13. Epub 2019 Feb 13.

The Vanderbilt Vaccine Center, Vanderbilt University Medical Center, Nashville, TN, USA.

The human genome contains approximately 20 thousand protein-coding genes, but the size of the collection of antigen receptors of the adaptive immune system that is generated by the recombination of gene segments with non-templated junctional additions (on B cells) is unknown-although it is certainly orders of magnitude larger. It has not been established whether individuals possess unique (or private) repertoires or substantial components of shared (or public) repertoires. Here we sequence recombined and expressed B cell receptor genes in several individuals to determine the size of their B cell receptor repertoires, and the extent to which these are shared between individuals. Read More

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http://dx.doi.org/10.1038/s41586-019-0934-8DOI Listing
February 2019

Genetic mechanisms of primary chemotherapy resistance in pediatric acute myeloid leukemia.

Leukemia 2019 Feb 13. Epub 2019 Feb 13.

Department of Pediatrics, Memorial Sloan Kettering Cancer Center, New York, NY, USA.

Acute myeloid leukemias (AML) are characterized by mutations of tumor suppressor and oncogenes, involving distinct genes in adults and children. While certain mutations have been associated with the increased risk of AML relapse, the genomic landscape of primary chemotherapy-resistant AML is not well defined. As part of the TARGET initiative, we performed whole-genome DNA and transcriptome RNA and miRNA sequencing analysis of pediatric AML with failure of induction chemotherapy. Read More

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http://dx.doi.org/10.1038/s41375-019-0402-3DOI Listing
February 2019

IDH1-R132H acts as a tumor suppressor in glioma via epigenetic up-regulation of the DNA damage response.

Sci Transl Med 2019 Feb;11(479)

Department of Neurosurgery, University of Michigan Medical School, Ann Arbor, MI 48109, USA.

Patients with glioma whose tumors carry a mutation in isocitrate dehydrogenase 1 (IDH1) are younger at diagnosis and live longer. mutations co-occur with other molecular lesions, such as 1p/19q codeletion, inactivating mutations in the tumor suppressor protein 53 ) gene, and loss-of-function mutations in alpha thalassemia/mental retardation syndrome X-linked gene (). All adult low-grade gliomas (LGGs) harboring ATRX loss also express the IDH1 mutation. Read More

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http://dx.doi.org/10.1126/scitranslmed.aaq1427DOI Listing
February 2019

Enterovirus as trigger of coeliac disease: nested case-control study within prospective birth cohort.

BMJ 2019 Feb 13;364:l231. Epub 2019 Feb 13.

Department of Pediatrics, Østfold Hospital Trust, Grålum, Norway.

Objective: To determine whether infection with human enterovirus or adenovirus, both common intestinal viruses, predicts development of coeliac disease.

Design: Case-control study nested within Norwegian birth cohort recruited between 2001 and 2007 and followed to September 2016.

Setting: Norwegian population. Read More

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http://www.bmj.com/lookup/doi/10.1136/bmj.l231
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http://dx.doi.org/10.1136/bmj.l231DOI Listing
February 2019
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Epigenetic findings in periodontitis in UK twins: a cross-sectional study.

Clin Epigenetics 2019 Feb 13;11(1):27. Epub 2019 Feb 13.

Department of Twin Research and Genetic Epidemiology, School of Life Course Sciences, King's College London, London, UK.

Background: Genetic and environmental risk factors contribute to periodontal disease, but the underlying susceptibility pathways are not fully understood. Epigenetic mechanisms are malleable regulators of gene function that can change in response to genetic and environmental stimuli, thereby providing a potential mechanism for mediating risk effects in periodontitis. The aim of this study is to identify epigenetic changes across tissues that are associated with periodontal disease. Read More

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http://dx.doi.org/10.1186/s13148-019-0614-4DOI Listing
February 2019

Focal Chorangiosis: Does It Have Clinical and Pathologic Significance?

Pediatr Dev Pathol 2019 Feb 13:1093526619830866. Epub 2019 Feb 13.

1 Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, New York.

Introduction: Chorangiosis is a proliferation of capillaries in terminal chorionic villi and is considered to be a marker for hypoxia and poor clinical outcome. Not all cases with hypervascular villi meet the generally accepted diagnostic criteria as reported by Altshuler. Our aim was to evaluate cases with villous hypervascularity that do not meet the diagnosis of chorangiosis, in which increased vascularity was present in a significant portion of the villous tissue but was not a diffuse process, which we call focal chorangiosis, to ascertain whether there were clinical or pathologic associations. Read More

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http://dx.doi.org/10.1177/1093526619830866DOI Listing
February 2019

Changing trend of microbiologic profile and antibiotic susceptibility of the microorganisms isolated in the neonatal nosocomial sepsis: a 14 years analysis.

J Matern Fetal Neonatal Med 2019 Feb 13:1-181. Epub 2019 Feb 13.

f Department of Infectious Diseases and Clinical Microbiology , Karadeniz Technical University , Trabzon , Turkey.

Objective: Neonatal sepsis , especially nosocomial sepsis (NS) is one of the main causes of mortality and morbidity  in neonates. Our aim was to investigate microorganisms responsible of NS and antimicrobial susceptibility patterns and to compare them in the different period.

Methods: Blood culture registers from the Microbiology Laboratory were reviewed for the study population. Read More

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http://dx.doi.org/10.1080/14767058.2019.1582633DOI Listing
February 2019

Associations Between Serum Uric Acid Levels and Cardiometabolic Risk, Renal İnjury in Obese and Overweight Children

J Clin Res Pediatr Endocrinol 2019 Feb 14. Epub 2019 Feb 14.

Manisa Celal Bayar University, School of Medicine, Department of Pediatrics, Division of Pediatric Endocrinology and Metabolism, Manisa, Turkey

Objective: In this study we aimed to assess the association between serum uric acid (SUA) concentration and metabolic syndrome (MetS) parameters and insulin resistance (IR). Our second aim was evaluate whether hyperuricemia is related with renal injury and cardiovascular risk in obese (OB) and overweight(OW) children.

Methods: This study was conducted on 128 OB/OW children and adolescents (ages: 8-18 years), of whom 52 (40%) with SUA elevation (SUA persantile>75), 76 with (60%) normal SUA level (SUAL). Read More

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http://dx.doi.org/10.4274/jcrpe.galenos.2019.2018.0241DOI Listing
February 2019

Modifiable Clinical Correlates of Vascular Health in Children and Adolescents with Dyslipidemia.

Pediatr Cardiol 2019 Feb 13. Epub 2019 Feb 13.

The Ward Family Heart Center, Children's Mercy Hospital, 2401 Gillham Rd, Kansas City, MO, 64108, USA.

Atherosclerosis promoting cardiovascular disease risk factors (CVDrf) are highly prevalent among youth in the U.S. Determining which standard modifiable clinical measures (SMCMs) has the greatest impact on vascular structure and function is valuable for the health care provider to help identify children at highest risk. Read More

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http://dx.doi.org/10.1007/s00246-019-02071-wDOI Listing
February 2019

Abnormal effector and regulatory T cell subsets in paediatric-onset multiple sclerosis.

Brain 2019 Feb 12. Epub 2019 Feb 12.

Neuroimmunology Unit, Department of Neurology and Neurosurgery, Montreal Neurological Institute, McGill University, 3801 University Street, Suite # 111, Montreal, Quebec, Canada.

Elucidation of distinct T-cell subsets involved in multiple sclerosis immune-pathophysiology continues to be of considerable interest since an ultimate goal is to more selectively target the aberrant immune response operating in individual patients. While abnormalities of both effector (Teff) and regulatory (Treg) T cells have been reported in patients with multiple sclerosis, prior studies have mostly assessed average abnormalities in either limb of the immune response, rather than both at the same time, which limits the ability to evaluate the balance between effectors and regulators operating in the same patient. Assessing both phenotypic and functional responses of Teffs and Tregs has also proven important. Read More

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http://dx.doi.org/10.1093/brain/awz017DOI Listing
February 2019

A rare case of accessory spleen torsion in a child diagnosed by ultrasound (US) and contrast-enhanced ultrasound (CEUS).

J Ultrasound 2019 Feb 13. Epub 2019 Feb 13.

Department of Radiology, Careggi University Hospital, L.go Giovanni Alessandro Brambilla, 3, 50134, Florence, Italy.

Introduction: Accessory spleen, also known as supernumerary spleen or splenunculum, is a congenital anomaly of the spleen due to a fusion defect during the embryogenesis. Usually it is detected casually during an ultrasound (US) examination of the abdomen and it is asymptomatic.

Case Report: RESULTS: We present a case of a 12 years old male patient, with 2-days history of left abdominal pain, without fever, gastrointestinal or genitourinary symptoms. Read More

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http://dx.doi.org/10.1007/s40477-019-00359-4DOI Listing
February 2019

Optical Redox Imaging Detects the Effects of DEK Oncogene Knockdown on the Redox State of MDA-MB-231 Breast Cancer Cells.

Mol Imaging Biol 2019 Feb 13. Epub 2019 Feb 13.

Department of Radiology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.

Purpose: Optical redox imaging (ORI), based on collecting the endogenous fluorescence of reduced nicotinamide adenine dinucleotide (NADH) and oxidized flavoproteins (Fp) containing a redox cofactor flavin adenine dinucleotide (FAD), provides sensitive indicators of cellular metabolism and redox status. ORI indices (such as NADH, FAD, and their ratio) have been under investigation as potential progression/prognosis biomarkers for cancer. Higher FAD redox ratio (i. Read More

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http://dx.doi.org/10.1007/s11307-019-01321-wDOI Listing
February 2019

Understanding, measuring, and addressing the financial impact of cancer on adolescents and young adults.

Pediatr Blood Cancer 2019 Feb 12:e27660. Epub 2019 Feb 12.

Children's Center for Cancer and Blood Diseases, Children's Hospital Los Angeles, Los Angeles, California.

The financial impact of cancer treatment among adolescents and young adults (AYAs, 15-39 years) is deep and long lasting. Compared with other age groups, because of their life stage, AYAs are particularly vulnerable to the adverse economic effects of cancer treatment, also known as financial toxicity. Clinical manifestations of cancer-related financial toxicity include interrupted work and income loss, accumulated debt, treatment nonadherence, avoidance of medical care, and social isolation. Read More

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http://dx.doi.org/10.1002/pbc.27660DOI Listing
February 2019

The T-helper cells 17 instead of Tregs play the key role in acute rejection after pediatric liver transplantation.

Pediatr Transplant 2019 Feb 12:e13363. Epub 2019 Feb 12.

Department of Transplant Surgery, Tianjin First Center Hospital, Tianjin, China.

Th17 and imbalance of Treg/Th17 might be one of the mechanisms of acute rejection. We aim to explore the role of Th17s in the balance of Treg/Th17 in acute rejection after LT in children diagnosed with BA. The ratios of Treg and Th17 in peripheral blood were detected by flow cytometry pre-LT, post-LT, and when rejection occurred. Read More

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http://dx.doi.org/10.1111/petr.13363DOI Listing
February 2019

miR-939-5p decreases the enrichment of RNA polymerase II in the promoter region of CD2AP involved in nephrotic syndrome.

J Cell Biochem 2019 Feb 12. Epub 2019 Feb 12.

Department of Pediatrics, the First Affiliated Hospital, Nanjing Medical University, Nanjing, Jiangsu Province, China.

The expression changes of CD2-associated protein (CD2AP) can lead to kidney diseases with proteinuria, including nephrotic syndrome (NS). A recent study reported that miRNAs may be important transcriptional regulators. In this study, we found increased expression of miR-939-5p and decreased expression of CD2AP in the peripheral blood of patients with NS. Read More

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http://dx.doi.org/10.1002/jcb.28413DOI Listing
February 2019
3.263 Impact Factor

Potential role of activated factor VIII (FVIIIa) in FVIIa/tissue factor-dependent FXa generation in initiation phase of blood coagulation.

Int J Hematol 2019 Feb 13. Epub 2019 Feb 13.

Department of Pediatrics, Nara Medical University, 840 Shijo-cho, 634-8522, Kashihara, Nara, Japan.

Factor VIIa/tissue factor (FVIIa/TF) initiates blood coagulation by promoting FXa generation (extrinsic-Xa). Subsequent generation of intrinsic FXa (intrinsic-Xa) amplifies thrombin formation. Previous studies suggested that FVIIa/TF activates FVIII rapidly in immediate coagulation reactions, and FVIIa/TF/FXa activates FVIII prior to thrombin-dependent feedback. Read More

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http://dx.doi.org/10.1007/s12185-019-02611-3DOI Listing
February 2019

Maintaining extraembryonic expression allows generation of mice with severe tissue factor pathway inhibitor deficiency.

Blood Adv 2019 Feb;3(3):489-498

Division of Pediatric Pathology, Department of Pathology.

Tissue factor pathway inhibitor (TFPI) is a serine protease with multiple anticoagulant activities. The Kunitz1 (K1) domain of TFPI binds the active site of factor VIIa and is required for inhibition of tissue factor (TF)/factor VIIa catalytic activity. Mice lacking TFPI K1 domain die in utero. Read More

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http://dx.doi.org/10.1182/bloodadvances.2018018853DOI Listing
February 2019

Baboon envelope LVs efficiently transduced human adult, fetal, and progenitor T cells and corrected SCID-X1 T-cell deficiency.

Blood Adv 2019 Feb;3(3):461-475

Centre International de Recherche en Infectiologie, Université Lyon, Université Claude Bernard Lyon 1, INSERM, U1111, Centre National de la Recherche Scientifique, Unité Mixte de Recherche (UMR) 5308, Ecole Normale Supérieure de Lyon, Lyon, France.

T cells represent a valuable tool for treating cancers and infectious and inherited diseases; however, they are mainly short-lived in vivo. T-cell therapies would strongly benefit from gene transfer into long-lived persisting naive T cells or T-cell progenitors. Here we demonstrate that baboon envelope glycoprotein pseudotyped lentiviral vectors (BaEV-LVs) far outperformed other LV pseudotypes for transduction of naive adult and fetal interleukin-7-stimulated T cells. Read More

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http://dx.doi.org/10.1182/bloodadvances.2018027508DOI Listing
February 2019

The BRISC de-ubiquitinating enzyme complex limits hematopoietic stem cell expansion by regulating JAK2 K63-ubiquitination.

Blood 2019 Feb 12. Epub 2019 Feb 12.

Division of Hematology, Children's Hospital of Philadelphia, Philadelphia, PA, United States;

Hematopoietic stem cell (HSC) homeostasis is controlled by cytokine receptor-mediated JAK2 signaling. We previously found that JAK2 is promptly ubiquitinated upon cytokine stimulation. Whether a competing JAK2 de-ubiquitination activity exists is unknown. Read More

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http://www.bloodjournal.org/lookup/doi/10.1182/blood-2018-10
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http://dx.doi.org/10.1182/blood-2018-10-877563DOI Listing
February 2019
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First experience with Tolvaptan for the treatment of neonates and infants with capillary leak syndrome after cardiac surgery.

BMC Pediatr 2019 Feb 12;19(1):57. Epub 2019 Feb 12.

Department of Pediatric Cardiology, University of Erlangen-Nürnberg, Loschgestrasse 15, 91054, Erlangen, Germany.

Background: Postoperative fluid management in critically ill neonates and infants with capillary leak syndrome (CLS) and extensive volume overload after cardiac surgery on cardiopulmonary bypass is challenging. CLS is often resistant to conventional diuretic therapy, aggravating the course of weaning from invasive ventilation, increasing length of stay on ICU and morbidity and mortality.

Methods: Tolvaptan (TLV, vasopressin type 2 receptor antagonist) was used as an additive diuretic in neonates and infants with CLS after cardiac surgery. Read More

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http://dx.doi.org/10.1186/s12887-019-1418-6DOI Listing
February 2019

Comparing Single-Photon Emission Computed Tomography (SPECT), Electroencephalography (EEG), and Magneto-encephalography (MEG) Seizure Localizations in Pediatric Cases of Laser Ablation.

J Child Neurol 2019 Feb 12:883073818822353. Epub 2019 Feb 12.

3 University of Texas Dell Medical School, Austin, TX, USA.

Intractable epilepsy may have a more severe effect on children in comparison to adults because the motor, sensorial, and cognitive functions of children are still undergoing development. For this same reason, however, children maintain a greater potential for recovery from intractable epilepsy because of the remaining cerebral plasticity. Thus, after 2 unsuccessful antiepileptic medications, surgical intervention is recommended. Read More

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http://dx.doi.org/10.1177/0883073818822353DOI Listing
February 2019

A complex case of aortic arch interruption and hypoplastic descending aorta.

Asian Cardiovasc Thorac Ann 2019 Feb 12:218492318804948. Epub 2019 Feb 12.

1 Department of Pediatric Cardiac Surgery, Vall d'Hebron University Hospital Barcelona, Spain.

The case of a newborn with a post-ductal interrupted aortic arch combined with severe hypoplasia of the thoracic descending aorta and intracardiac defects is described. Extraanatomic bypass surgery was performed in the neonatal period to connect the ascending and descending parts of the aorta. Closure of a ventricular septal defect and excision of hypertrophied bands in the right ventricle with subsequent ventriculotomy were delayed beyond neonatal age. Read More

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http://dx.doi.org/10.1177/0218492318804948DOI Listing
February 2019