137,906 results match your criteria Pediatric Blood & Cancer[Journal]


Regulation of Neprilysin Activity and Cognitive Functions in Rats After Prenatal Hypoxia.

Neurochem Res 2019 Apr 20. Epub 2019 Apr 20.

I.M. Sechenov Institute of Evolutionary Physiology and Biochemistry, Russian Academy of Sciences, 44 Thorez av, St. Petersburg, Russia, 194223.

The amyloid-degrading enzyme neprilysin (NEP) is one of the therapeutic targets in prevention and treatment of Alzheimer's disease (AD). As we have shown previously NEP expression in rat parietal cortex (Cx) and hippocampus (Hip) decreases with age and is also significantly reduced after prenatal hypoxia. Following the paradigms for enhancement of NEP expression and activity developed in cell culture, we analysed the efficacy of various compounds able to upregulate NEP using our model of prenatal hypoxia in rats. Read More

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http://link.springer.com/10.1007/s11064-019-02796-3
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http://dx.doi.org/10.1007/s11064-019-02796-3DOI Listing
April 2019
1 Read

Sunitinib in pediatric patients with advanced gastrointestinal stromal tumor: results from a phase I/II trial.

Cancer Chemother Pharmacol 2019 Apr 20. Epub 2019 Apr 20.

Pediatrics, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, MA, USA.

Background: Sunitinib is approved for treatment of adults with imatinib-resistant gastrointestinal stromal tumor (GIST) or imatinib intolerance.

Methods: This single-arm, multicenter, multinational phase I/II clinical trial (NCT01396148) enrolled eligible patients aged 6 to < 18 years with advanced, unresectable GIST with non-mutant KIT, or who demonstrated disease progression or intolerance to imatinib. Patients received sunitinib 15 mg/m per day, 4-weeks-on/2-weeks-off (schedule 4/2), for ≤ 18 cycles over 24 months. Read More

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http://link.springer.com/10.1007/s00280-019-03814-5
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http://dx.doi.org/10.1007/s00280-019-03814-5DOI Listing
April 2019
1 Read

Next-generation reference intervals for pediatric hematology.

Clin Chem Lab Med 2019 Apr 22. Epub 2019 Apr 22.

Department of Pediatrics and Adolescent Medicine, University Hospital Erlangen, Erlangen, Germany.

Background Interpreting hematology analytes in children is challenging due to the extensive changes in hematopoiesis that accompany physiological development and lead to pronounced sex- and age-specific dynamics. Continuous percentile charts from birth to adulthood allow accurate consideration of these dynamics. However, the ethical and practical challenges unique to pediatric reference intervals have restricted the creation of such percentile charts, and limitations in current approaches to laboratory test result displays restrict their use when guiding clinical decisions. Read More

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http://www.degruyter.com/view/j/cclm.ahead-of-print/cclm-201
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http://dx.doi.org/10.1515/cclm-2018-1236DOI Listing
April 2019
1 Read

Screening umbilical cord blood for congenital Iron deficiency.

Blood Cells Mol Dis 2019 Apr 8;77:95-100. Epub 2019 Apr 8.

The Statistical Data Center, Intermountain Healthcare, Salt Lake City, UT, USA.

Objectives: Small for gestational age infants (SGA), infants of diabetic mothers (IDM), and very low birth weight infants (VLBW) are at risk for congenital iron deficiency. We evaluated the iron status of SGA, IDM, and VLBW neonates at birth and sought mechanistic explanations in those with iron deficiency.

Methods: This was a prospective study. Read More

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http://dx.doi.org/10.1016/j.bcmd.2019.04.005DOI Listing

A retrospective study on clinical manifestations of neonates with FXIII-A deficiency.

Blood Cells Mol Dis 2019 Apr 11;77:78-81. Epub 2019 Apr 11.

Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran. Electronic address:

We assessed clinical presentations and the rate of central nervous system (CNS) bleeding in neonates with FXIIID who exhibited bleeding diathesis in the early days of their lives. A total of 27 neonates presented bleeding or abnormal clinical symptoms, diagnosed with FXIII deficiency were evaluated. Factor XIII concentrate was initiated as the first-line of treatment, and prophylactic therapy was given to all patients. Read More

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http://dx.doi.org/10.1016/j.bcmd.2019.04.006DOI Listing

In vitro assessment of edoxaban anticoagulant effect in pediatric plasma.

Thromb Res 2019 Apr 13;178:112-118. Epub 2019 Apr 13.

CHU Clermont-Ferrand, Service d'Hématologie Biologique, Clermont-Ferrand, France; Université Clermont Auvergne, INRA, UNH, Unité de Nutrition Humaine, CRNH Auvergne, Clermont-Ferrand, France.

Introduction: Anticoagulant therapy in pediatric patients remains an issue and safer therapies, such as direct oral anticoagulants could overcome the limitations of conventional anticoagulant treatments in this population. Edoxaban, a factor Xa inhibitor, is used for the prevention and treatment of venous thromboembolism. Due to its pharmacokinetic characteristics, edoxaban is a promising candidate molecule for children. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00493848193020
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http://dx.doi.org/10.1016/j.thromres.2019.04.014DOI Listing
April 2019
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Influence of nutritional factors and the PON1 C(-107)T polymorphism on paraoxonase-1 activity in childhood.

J Pediatr (Rio J) 2019 Apr 18. Epub 2019 Apr 18.

Universidade Federal de Pelotas (UFPel), Faculdade de Nutrição, Pelotas, RS, Brazil. Electronic address:

Objective: The cardioprotective enzyme paraoxonase-1 (PON1) suffers an important influence from genetic polymorphisms and nutritional factors. The aim of this study was to investigate the influence of diet, nutritional status, and the C(-107)T polymorphism on PON1 arylesterase activity in children.

Methods: This was a cross-sectional study with 97 children, aged between 5 and 8 years, of both genders, from a pediatric outpatient clinic in southern Brazil. Read More

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http://dx.doi.org/10.1016/j.jped.2019.02.004DOI Listing

High blood pressure in schoolchildren: Associated sociodemographic and biochemical factors.

Rev Port Cardiol 2019 Apr 17. Epub 2019 Apr 17.

Universidade de Santa Cruz do Sul (UNISC), Santa Cruz do Sul, Brasil.

Introduction And Objective: Pediatric hypertension has increased in the last decade, and it is thus crucial to identify the factors associated with the development of high blood pressure (BP) and other cardiovascular disorders. The aim of this study was to determine whether there is an association between high BP and sociodemographic and biochemical factors in schoolchildren.

Methods: This cross-sectional study included 1201 children and adolescents, between seven and 17 years old, of both sexes. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S08702551173043
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http://dx.doi.org/10.1016/j.repc.2018.06.009DOI Listing
April 2019
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Growth, health, and motor development of 5-year-old children born after preimplantation genetic diagnosis.

Fertil Steril 2019 Apr 17. Epub 2019 Apr 17.

Department of Clinical Genetics, Maastricht University Medical Center+, Maastricht, The Netherlands; School for Oncology and Developmental Biology (GROW), Maastricht University, Maastricht, The Netherlands.

Objective: To evaluate the growth, health, and motor development of children born after preimplantation genetic diagnosis (PGD).

Design: Observational cohort study and comparison of 5-year-old children born after PGD to similar aged children born after IVF/intracytoplasmic sperm injection (ICSI) and children from families with a genetic disorder born after natural conception (NC).

Setting: University hospital. Read More

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http://dx.doi.org/10.1016/j.fertnstert.2019.01.035DOI Listing

Low levels of urinary epidermal growth factor predict chronic kidney disease progression in children.

Kidney Int 2019 Mar 20. Epub 2019 Mar 20.

Division of Pediatric Nephrology, Center for Pediatrics and Adolescent Medicine, Heidelberg University Hospital, Heidelberg, Germany. Electronic address:

Urinary epidermal growth factor (uEGF) has recently been identified as a promising biomarker of chronic kidney disease (CKD) progression in adults with glomerular disease. Low levels of uEGF predict CKD progression and appear to reflect the extent of tubulointerstitial damage. We investigated the relevance of uEGF in pediatric CKD. Read More

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http://dx.doi.org/10.1016/j.kint.2019.01.035DOI Listing

Human Erythroblasts with c-Kit Activating Mutations Have Reduced Cell Culture Costs and Remain Capable of Terminal Maturation.

Exp Hematol 2019 Apr 17. Epub 2019 Apr 17.

Center for Pediatric Biomedical Research, Department of Pediatrics, University of Rochester, Rochester, NY. Electronic address:

A major barrier to the in vitro production of red blood cells for transfusion therapy is the cost of culture components, with cytokines making up greater than half of the culture costs. Cell culture cytokines also represent a major expense for in vitro studies of human erythropoiesis. HUDEP2 cells are an E6/E7 immortalized erythroblast line used for the in vitro study of human erythropoiesis. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S0301472X193013
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http://dx.doi.org/10.1016/j.exphem.2019.04.001DOI Listing
April 2019
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Do Third Molars Contribute to Systemic Inflammation? Results From a Population-Based Study From Northeast Germany.

J Oral Maxillofac Surg 2019 Mar 27. Epub 2019 Mar 27.

Associate Professor, Institute for Community Medicine, University Medicine Greifswald, Greifswald, Germany.

Purpose: Erupted and impacted third molars have been reported to contribute to systemic inflammation. This study investigated the systemic effect of third molars on serum levels of inflammatory parameters and on inflammatory messenger peptide hormones in a general population sample.

Materials And Methods: Data of 2,151 participants from the Study of Health in Pomerania were included in this study. Read More

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http://dx.doi.org/10.1016/j.joms.2019.03.025DOI Listing

Wheezing independently predicts viral infection in children with community-acquired pneumonia.

Pediatr Pulmonol 2019 Apr 19. Epub 2019 Apr 19.

Department of Pediatrics, Federal University of Bahia School of Medicine, Salvador, Brazil.

Aim: To assess whether there was a difference in the frequency of symptoms and signs among children with community-acquired pneumonia (CAP) with viral or bacterial infection.

Methods: A prospective cross-sectional study was conducted in Salvador, Brazil. Children less than 5-years-old hospitalized with CAP were recruited. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1002/ppul.24339
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http://dx.doi.org/10.1002/ppul.24339DOI Listing
April 2019
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Metabolic Risk Factors Associated with Visceral and Subcutaneous Adipose Tissue in a Sex-Specific Manner in Seven-Year-Olds.

Obesity (Silver Spring) 2019 Apr 19. Epub 2019 Apr 19.

Department of Pediatrics, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.

Objective: This study aimed to investigate how visceral adipose tissue (VAT) and subcutaneous adipose tissue (SAT) volumes were associated with metabolic risk factors in 7-year-old children.

Methods: A total of 81 children (52% girls) from a Swedish birth cohort were studied. At 6 years of age, anthropometric data, fasting insulin, glucose, cholesterol, and blood pressure were collected on 53 children with normal weight and 28 children with overweight or obesity, and insulin resistance was estimated. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1002/oby.22453
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http://dx.doi.org/10.1002/oby.22453DOI Listing
April 2019
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The impact of maternal stress on infant alpha-amylase is buffered by high infant regulation and low infant negative reactivity.

Dev Psychobiol 2019 Apr 19. Epub 2019 Apr 19.

Department of Psychology, University of Florida, Gainesville, Florida.

This study examined the main and interactive effects of maternal perceived stress and infant temperament-surgency, negative affectivity, and orienting/regulation-on infant salivary alpha-amylase (sAA) responses to stress. Saliva samples were collected prior to and following two naturalistic stressors: maternal separation conducted at 9 months and blood draw/immunizations conducted at 12 months. sAA area under the curve (AUC) was computed to determine response of the sympathetic nervous system to each stressor. Read More

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http://dx.doi.org/10.1002/dev.21858DOI Listing

Efficacy, safety, and pharmacokinetics of emicizumab prophylaxis given every 4 weeks in people with haemophilia A (HAVEN 4): a multicentre, open-label, non-randomised phase 3 study.

Lancet Haematol 2019 Apr 16. Epub 2019 Apr 16.

Hospital Universitario La Paz, Autónoma University, Madrid, Spain.

Background: Emicizumab, a subcutaneously administered, humanised, bispecific, monoclonal antibody, is approved to treat people with haemophilia A of all ages with and without coagulation factor VIII (FVIII) inhibitors. HAVEN 4 assessed emicizumab prophylaxis administered as one dose every 4 weeks in adults and adolescents with haemophilia A, regardless of FVIII inhibitor status.

Methods: In this phase 3, multicentre, open-label, two-stage study, patients aged 12 years and older with severe congenital haemophilia A (<1% of normal FVIII activity in blood) or haemophilia A with FVIII inhibitors, undergoing treatment with either FVIII concentrates or bypassing agents were recruited from three sites in Japan and Spain for a run-in cohort, and from 17 sites in Australia, Belgium, Japan, Poland, Spain, and the USA for a subsequent expansion cohort. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S23523026193005
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http://dx.doi.org/10.1016/S2352-3026(19)30054-7DOI Listing
April 2019
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Lower platelet count with increased density of platelet antigens in granulocyte colony-stimulating factor mobilized peripheral blood stem cell donors.

J Formos Med Assoc 2019 Apr 16. Epub 2019 Apr 16.

Department of Hematology and Oncology, Buddhist Hualien Tzu Chi Hospital, Hualien, Taiwan; Department of Medicine, College of Medicine, Tzu Chi University, Hualien, Taiwan. Electronic address:

Background/purpose: Granulocyte colony-stimulating factor (G-CSF) is widely used for prophylaxis and treatment of neutropenia in cancer patients and also for peripheral blood stem cells (PBSC) mobilization. The aim of this study is to evaluate the possible changes of platelet surface antigens after G-CSF injection in PBSC donors compared with healthy control.

Methods: Between January 1st and December 31st, 2014, 48 healthy voluntary PBSC donors were eligible for this study. Read More

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http://dx.doi.org/10.1016/j.jfma.2019.04.003DOI Listing

Abdominal Lymphoma Presenting as Terminal Ileitis: A Case Report.

J Emerg Med 2019 Apr 16. Epub 2019 Apr 16.

Department of Emergency Medicine, Cincinnati Children's Hospital and Medical Center, Cincinnati, Ohio; Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio.

Background: Most pediatric patients with lymphoma do not have classic symptoms of fever, night sweats, and weight loss. Lymphoma can present as vague symptoms and may mimic common pediatric abdominal emergencies. In this case report, we present a child who presented with abdominal pain and who was initially misdiagnosed as having a surgical emergency. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S07364679193013
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http://dx.doi.org/10.1016/j.jemermed.2019.03.004DOI Listing
April 2019
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Thrombin Preconditioning of Extracellular Vesicles Derived from Mesenchymal Stem Cells Accelerates Cutaneous Wound Healing by Boosting Their Biogenesis and Enriching Cargo Content.

J Clin Med 2019 Apr 18;8(4). Epub 2019 Apr 18.

Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 06351, Korea.

The aim of this study was to determine the optimal preconditioning regimen for the wound healing therapeutic efficacy of mesenchymal stem cell (MSC)-derived extracellular vesicles (EVs). To this end, we compared various preconditioning regimens for both the quantitative and qualitative production of MSC-derived EVs, and their therapeutic efficacy for proangiogenic activity in vitro and cutaneous wound healing in vivo. After preconditioning with thrombin (40 U), HO (50 μM), lipopolysaccharide (1 μg/mL), or hypoxia (10% O), EV secretion was assessed quantitatively by measuring production per cell and protein quantification, and qualitatively by measuring a proteome profiler and an enzyme-linked immunosorbent assay (ELISA) contained within EVs. Read More

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http://dx.doi.org/10.3390/jcm8040533DOI Listing

Observed and calculated cerebral critical closing pressure are highly correlated in preterm infants.

Pediatr Res 2019 Apr 19. Epub 2019 Apr 19.

Department of Pediatrics, Neonatology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, USA.

Introduction: Cerebrovascular critical closing pressure (CrCP) is the arterial blood pressure (ABP) at which cerebral blood flow ceases. Preterm ABP is low and close to CrCP. The diastolic closing margin (diastolic ABP minus CrCP) has been associated with intraventricular hemorrhage in preterm infants. Read More

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http://dx.doi.org/10.1038/s41390-019-0403-yDOI Listing

Population Pharmacokinetics of Clofarabine as Part of Pre-Transplant Conditioning in Pediatric Subjects Prior to Hematopoietic Cell Transplantation (HCT).

Biol Blood Marrow Transplant 2019 Apr 16. Epub 2019 Apr 16.

Department of Pediatrics, Division of Allergy, Immunology, and Bone Marrow Transplantation, University of California San Francisco, San Francisco, CA; Department of Clinical Pharmacy, University of California San Francisco, San Francisco, CA.

The primary objective of this work was to characterize the pharmacokinetics (PK) of systemic clofarabine (clo-fara) in pediatric allogeneic hematopoietic cell transplantation (HCT) recipients receiving either nucleoside monotherapy or a dual nucleoside analogue preparative regimen. Fifty-one children (median age 4.9 years, range 0. Read More

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http://dx.doi.org/10.1016/j.bbmt.2019.04.017DOI Listing

Allogeneic stem cell transplantation for acute lymphoblastic leukemia in adolescents and young adults.

Biol Blood Marrow Transplant 2019 Apr 16. Epub 2019 Apr 16.

Children's Cancer Center, National Center for Child Health and Development, Tokyo, Japan; Department of Pediatric Hematology and Oncology Research, National Center for Child Health and Development, Tokyo, Japan.

Hematologic stem cell transplantation (HSCT) is the most potent consolidation therapy for high-risk acute lymphoblastic leukemia (ALL), but their outcomes and complications in adolescent and young adult (AYA) patients are still unclear. We compared outcomes after HSCT for ALL among children (1 to 9 years old, n = 607), adolescents (10 to 19 years old, n= 783) and young adults (20 to 29 years old, n = 603), based on nationwide registry data in Japan. The overall survival rate among AYA patients was worse than that of children; survival rate at 5 years among AYA patients was 64% (95% confidence intervals [CI], 60% to 68%). Read More

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http://dx.doi.org/10.1016/j.bbmt.2019.04.014DOI Listing
April 2019
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Maternal hyperuricemia superimposed on maternal hypertension aggravates the risk of small-for-gestational-age fetus.

Life Sci 2019 Apr 16. Epub 2019 Apr 16.

Department of Endocrinology, Shandong Provincial Hospital affiliated to Shandong University, Institute of Endocrinology and Metabolism, Shandong Academy of Clinical Medicine, Shandong Clinical Medical Center of Endocrinology and Metabolism, Jinan, Shandong 250021, China. Electronic address:

Aims: Small-for-gestational-age (SGA) fetus is an important public health issue because of its high mortality and long-term effects on health. Maternal hyperuricemia is associated with diverse adverse pregnant outcomes and neonatal disturbance. We aimed to evaluate whether maternal hyper-uric acid (HUA) is associated with the risk of SGA fetus and to explore whether it can modify the association between maternal hyper-blood pressure (HBP) and SGA fetus. Read More

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http://dx.doi.org/10.1016/j.lfs.2019.04.033DOI Listing

Chemerin and Dermcidin in Human Milk and Their Alteration in Gestational Diabetes.

J Hum Lact 2019 Apr 19:890334419837523. Epub 2019 Apr 19.

5 Department of Medical Biochemistry and Clinical Biochemistry, Firat Hormones Research Group, Medical School, Firat University, Elazig, Turkey.

Background: Chemerin and dermcidin, which have antimicrobial properties, are molecules that are also related to insulin resistance and inflammation.

Research Aims: The aims were to determine the amounts of chemerin and dermcidin in the milk and blood of mothers with gestational diabetes, and to compare the amounts of chemerin and dermcidin in the milk and blood of mothers with and without diabetes.

Methods: This was a two-group nonrandomized longitudinal study with a convenience sampling of mothers without gestational diabetes ( n = 27) and mothers with gestational diabetes ( n = 26). Read More

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http://dx.doi.org/10.1177/0890334419837523DOI Listing

Cardiometabolic risk factors in children born with marginally low birth weight: A longitudinal cohort study up to 7 years-of-age.

PLoS One 2019 19;14(4):e0215866. Epub 2019 Apr 19.

Department of Clinical Sciences, Pediatrics, Umeå University, Umeå, Sweden.

Introduction: Low birth weight (LBW, <2500 g) may predict an increased risk of an adverse cardiometabolic profile later in life, but long-term effects in different populations and birth weight strata are still unclear. We explored laboratory markers of cardiometabolic risk in children born with marginally LBW (2000-2500 g).

Methods: This was a prospective longitudinal cohort study including 285 Swedish marginally LBW children and 95 normal birth weight (NBW, 2501-4500 g) controls. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0215866PLOS

Human genotyping and an experimental model reveal NPR-C as a possible contributor to morbidity in coarctation of the aorta.

Physiol Genomics 2019 Apr 19. Epub 2019 Apr 19.

Department of Surgery, Division of Cardiothoracic Surgery, Medical College of Wisconsin.

Coarctation of the aorta (CoA) is a common congenital cardiovascular (CV) defect characterized by a stenosis of the descending thoracic aorta. Treatment exists, but many patients develop hypertension (HTN). Identifying the cause of HTN is challenging because of patient variability (e. Read More

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http://dx.doi.org/10.1152/physiolgenomics.00049.2018DOI Listing

Endotipsitis as an indication for pediatric liver transplantation.

Transpl Infect Dis 2019 Apr 19:e13097. Epub 2019 Apr 19.

Hepatobiliary Surgery and Transplant Unit, Vall d'Hebron University Hospital, Barcelona, Spain.

Endotipsitis is a rare but severe complication of transjugular intrahepatic portosystemic shunt (TIPS), a device widely used to treat portal hypertension in adults, but sparsely used in children. We report a case of endotipsitis in a 3-year-old child affected of biliary atresia. She underwent a Kasai procedure at 3 months of age but, although the bile flow was restored, she presented upper gastrointestinal bleeding due to portal hypertension 1. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1111/tid.13097
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http://dx.doi.org/10.1111/tid.13097DOI Listing
April 2019
2 Reads

ARPC2 promotes breast cancer proliferation and metastasis.

Oncol Rep 2019 Apr 12. Epub 2019 Apr 12.

Department of Pathology, Anhui Medical University, Hefei, Anhui 230032, P.R. China.

Actin-related protein 2/3 complex (ARPC2) is an actin‑binding component involved in the regulation of actin polymerization. It mediates the formation of branched actin networks and contacts the mother actin filament. Migration and invasion are key processes which enable tumor cells to infiltrate blood vessels or lymphatic vessels, and the actin pathway plays a very important role. Read More

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http://dx.doi.org/10.3892/or.2019.7113DOI Listing
April 2019
4 Reads

Relationship between systolic hypertension assessed by 24-h ambulatory blood pressure monitoring and aortic diameters in young women with Turner syndrome.

Clin Endocrinol (Oxf) 2019 Apr 18. Epub 2019 Apr 18.

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

Objective: Patients with Turner syndrome (TS) are at high risk for cardiovascular morbidity and mortality due to aortic dilation. We evaluated the prevalence of hypertension and its risk factors, and investigated the relationship between systolic hypertension and aortic diameter in young TS patients.

Design: Observational, cross-sectional study. Read More

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http://dx.doi.org/10.1111/cen.13995DOI Listing

Biochemical Changes in Blood of Patients with Duchenne Muscular Dystrophy Treated with Granulocyte-Colony Stimulating Factor.

Biomed Res Int 2019 13;2019:4789101. Epub 2019 Mar 13.

Department of Pediatric Radiology, Medical University of Bialystok, Bialystok, Poland.

Introduction: In addition to the "gold standard" of therapy-steroids and gene therapy-there are experimental trials using granulocyte-colony stimulating factor (G-CSF) for patients with Duchenne muscular dystrophy (DMD). The aim of this study was to present the biochemical changes in blood after repeating cycles of granulocyte-colony stimulating factor G-CSF therapy in children with DMD.

Materials And Methods: Nineteen patients, aged 5 to 15 years, with diagnosed DMD confirmed by genetic tests, participated; nine were in wheelchairs, and ten were mobile and independent. Read More

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http://dx.doi.org/10.1155/2019/4789101DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6436375PMC

Alpha-Thalassemia in North Morocco: Prevalence and Molecular Spectrum.

Biomed Res Int 2019 13;2019:2080352. Epub 2019 Mar 13.

Biomedical Genomics and Oncogenetics Research Laboratory, Faculty of Sciences and Techniques of Tangier, University Abdelmalek Essaâdi, Tangier 90000, Morocco.

Unlike the other hemoglobinopathies, few researches have been published concerning -thalassemia in Morocco. The epidemiological features and the mutation spectrum of this disease are still unknown. This regional newborn screening is the first to study -thalassemia in the north of Morocco. Read More

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https://www.hindawi.com/journals/bmri/2019/2080352/
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http://dx.doi.org/10.1155/2019/2080352DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6436373PMC
March 2019
1 Read

Pediatric Patients With Steroid-Sensitive Nephrotic Syndrome Have Higher Expression of T Regulatory Lymphocytes in Comparison to Steroid-Resistant Disease.

Front Pediatr 2019 2;7:114. Epub 2019 Apr 2.

Unidade de Nefrologia Pediátrica, Departamento de Pediatria, Faculdade de Medicina, Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, Brazil.

Idiopathic nephrotic syndrome (INS) is classified according to the response to drug therapy in steroid-sensitive (SS), steroid-dependent (SD), and steroid-resistant (SR) categories. Previous studies showed changes in inflammatory activity of subpopulations of lymphocytes in INS. This study aimed to compare SS and SR patients in regard to subpopulations of leukocytes, profile of regulatory lymphocytes, and migratory activity of lymphocyte subpopulations. Read More

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https://www.frontiersin.org/article/10.3389/fped.2019.00114/
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http://dx.doi.org/10.3389/fped.2019.00114DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6455073PMC
April 2019
1 Read

Peritoneal Dialysis Vintage and Glucose Exposure but Not Peritonitis Episodes Drive Peritoneal Membrane Transformation During the First Years of PD.

Front Physiol 2019 2;10:356. Epub 2019 Apr 2.

Center for Pediatric and Adolescent Medicine, University Hospital Heidelberg, Heidelberg, Germany.

The impact of peritoneal dialysis (PD) associated peritonitis on peritoneal membrane integrity is incompletely understood. Children are particularly suited to address this question, since they are largely devoid of preexisting tissue damage and life-style related alterations. Within the International Peritoneal Biobank, 85 standardized parietal peritoneal tissue samples were obtained from 82 children on neutral pH PD fluids with low glucose degradation product (GDP) content. Read More

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http://dx.doi.org/10.3389/fphys.2019.00356DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6455046PMC

Health-related quality of life in sickle cell disease.

Nat Rev Dis Primers 2019 Apr 18;5(1):27. Epub 2019 Apr 18.

Division of General Internal Medicine, Department of Medicine, Virginia Commonwealth University, Richmond, VA, USA.

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http://www.nature.com/articles/s41572-019-0080-5
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http://dx.doi.org/10.1038/s41572-019-0080-5DOI Listing
April 2019
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Major Adverse Kidney Events in Pediatric Sepsis.

Clin J Am Soc Nephrol 2019 Apr 18. Epub 2019 Apr 18.

Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania.

Background And Objectives: Major adverse kidney events, a composite of death, new kidney replacement therapy, or persistent kidney dysfunction, is a potential patient-centered outcome for clinical trials in sepsis-associated kidney injury. We sought to determine the incidence of major adverse kidney events within 30 days and validate this end point in pediatric sepsis.

Design, Setting, Participants, & Measurements: We conducted a retrospective observational study using the Pediatric Health Information Systems Plus database of patients >6 months to <18 years old with a diagnosis of severe sepsis/septic shock; orders for bacterial blood culture, antibiotics, and at least one fluid bolus on hospital day 0/1; and known hospital disposition between January 2007 and December 2011. Read More

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http://dx.doi.org/10.2215/CJN.12201018DOI Listing

Findings of amplitude-integrated electroencephalogram recordings and serum vitamin B6 metabolites in perinatal lethal hypophosphatasia during enzyme replacement therapy.

Brain Dev 2019 Apr 15. Epub 2019 Apr 15.

Division of Neonatology, Center for Maternal-Neonatal Care, Nagoya University Hospital, Nagoya, Japan.

Hypophosphatasia (HPP) is a rare disorder caused by low serum tissue non-specific alkaline phosphatase (ALP) activity due to hypomorphic mutations in the ALPL gene. HPP is characterized by defective bone mineralization. It frequently accompanies pyridoxine-responsive seizures. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S03877604183059
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http://dx.doi.org/10.1016/j.braindev.2019.03.015DOI Listing
April 2019
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Evaluation of hematologic profile may be needed for patients treated with oxcarbazepine.

Korean J Pediatr 2019 Apr 11. Epub 2019 Apr 11.

Department of Pediatrics, Inje University Sanggye Paik Hospital, Seoul, Korea.

Purpose: The major side effects of treatment with oxcarbazepine (OXC) are skin rash and hyponatremia. Hematologic side effects are reported to be rare. The aim of this study was to investigate the rate and types of hematologic side effects of OXC. Read More

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http://dx.doi.org/10.3345/kjp.2019.00017DOI Listing

Encephalopathy in neonates with subgaleal hemorrhage is a key predictor of outcome.

Pediatr Res 2019 Apr 18. Epub 2019 Apr 18.

Department of Pediatric Newborn Medicine, Brigham and Women's Hospital, Boston, MA, USA.

Objective: Subgaleal hemorrhage (SGH) is reported to be associated with severe hemodynamic instability, coagulopathy, and even mortality. The importance of the presence or absence of neonatal encephalopathy in predicting SGH outcomes has not been explored. The aim of this study was to determine the relationship of clinical encephalopathy to short-term outcomes in neonates with SGH. Read More

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http://www.nature.com/articles/s41390-019-0400-1
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http://dx.doi.org/10.1038/s41390-019-0400-1DOI Listing
April 2019
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Functional Connectivity and Metabolic Alterations in Medial Prefrontal Cortex in a Rat Model of Fetal Alcohol Spectrum Disorder: A Resting-State Functional Magnetic Resonance Imaging and in vivo Proton Magnetic Resonance Spectroscopy Study.

Dev Neurosci 2019 Apr 18:1-12. Epub 2019 Apr 18.

Department of Pediatrics, University of Maryland School of Medicine, Baltimore, Maryland, USA,

Prenatal ethanol exposure alters brain structure, functional connectivity, and behavior in humans and rats. Behavioral changes include deficits in executive function, which requires cooperative activity between the frontal cortices and other brain regions. In this study, we analyzed the functional connectivity and neurochemical levels of the prefrontal cortex (PFC) using resting-state functional magnetic resonance imaging (rsfMRI) and proton magnetic resonance spectroscopy (1H-MRS) in ethanol-exposed (Eth) and control (Ctr) rats. Read More

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http://dx.doi.org/10.1159/000499183DOI Listing

Utility of Ophthalmologic Screening for Patients With Candida Bloodstream Infections: A Systematic Review.

JAMA Ophthalmol 2019 Apr 18. Epub 2019 Apr 18.

Vanderbilt University School of Medicine, Nashville, Tennessee.

Importance: The Infectious Diseases Society of America recommends ophthalmologic examinations for everyone with positive Candida blood culture results (candidemia) to screen for endophthalmitis, a practice that remains controversial because of multiple concerns for its limited usefulness and potential for harm.

Objective: To determine guideline efficacy by reconciling discrepancies in the incidence of endophthalmitis and evaluating outcomes of studies assessing ophthalmologic screening for candidemia.

Evidence Review: PubMed literature searches, including the search terms candidemia, fungemia, chorioretinitis, and endophthalmitis, identified longitudinal studies prior to 2018 of patients who underwent ophthalmologic evaluations in the setting of positive fungal blood culture results regardless of symptoms or clinical status. Read More

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http://dx.doi.org/10.1001/jamaophthalmol.2019.0733DOI Listing

Pediatric Blood Lead Levels Within New York City Public Versus Private Housing, 2003-2017.

Am J Public Health 2019 Apr 18:e1-e6. Epub 2019 Apr 18.

The authors are with the Institute for Family Health, New York, NY. Casey Crump and Neil Calman are also with the Department of Family Medicine and Community Health, Icahn School of Medicine at Mount Sinai, New York.

To compare blood lead levels (BLLs) among children residing in public and private housing in New York City and examine the implications for lead identification and remediation policies. We examined electronic medical records for BLLs among 4693 children receiving care at a multisite Federally Qualified Health Center during 2003 to 2017. We plotted home addresses against city housing data to assess BLL differences between children living in public housing and private housing. Read More

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https://ajph.aphapublications.org/doi/10.2105/AJPH.2019.3050
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http://dx.doi.org/10.2105/AJPH.2019.305021DOI Listing
April 2019
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Differences in Cost of Care by Palliation Strategy for Infants With Ductal-Dependent Pulmonary Blood Flow.

Circ Cardiovasc Interv 2019 Apr;12(4):e007232

The Cardiac Center at the Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine (M.L.O., D.D., H.M.G., C.E.M., A.C.G.).

Background: In infants with ductal-dependent pulmonary blood flow, initial palliation with patent ductus arteriosus (PDA) stent or modified Blalock-Taussig (BT) shunt have comparable mortality but discrepant length of stay, procedural complication rates and reintervention burdens, which may influence cost. The relative economic impact of these palliation strategies is unknown.

Methods And Results: Retrospective study of infants with ductal-dependent pulmonary blood flow palliated with PDA stent (n=104) or BT shunt (n=251) from 2008 to 2015 at 4 centers of the Congenital Catheterization Research Collaborative. Read More

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http://dx.doi.org/10.1161/CIRCINTERVENTIONS.118.007232DOI Listing

[Association between IL-10 Gene -592(C→A) (rs1800872) SNP and GVHD after Allogeneic Hematopoietic Stem Cell Transplantation in Children].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2019 Apr;27(2):573-579

Hematology Oncology Center of Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing Key Laboratory of Pediatric Hematology Oncology; National Key Discipline of Pediatrics (Capital Medical University); Key Laboratory of Major Diseases in Children, Ministry of Education; Beijing 100045, ChinaE-mail:

Objective: To study the association between IL-10 gene-592(C→A) (rs1800872) single nucleotide polymorphism (SNP) and the graft versus host disease (GVHD) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in children.

Methods: Ninety-seven childhood patients and seventy-one donors in the Hematology Oncology Center of Beijing Children's Hospital from Jan 2011 to Jul 2017 were enrolled in this study. The genomic DNA was extracted from peripheral blood cells and the SNP genotype was analyzed using TaqMan SNP genotyping assay. Read More

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http://dx.doi.org/10.19746/j.cnki.issn.1009-2137.2019.02.043DOI Listing
April 2019
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[E-cadherin Expression in Children with Acute Leukemia and Its Clinical Significance].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2019 Apr;27(2):339-347

Department of Hematology & Oncology, Childhren's Hospital Affiliated to Chongqing Medical University; Key Laboratory of Child Development and Disorders of Ministry of Education; China International Science and Technology Cooperation Base of Child Development and Critical Disorders; Chongqing Key Laboratory of Pediatrics, Chongqing 400014, China,E-mail:

Objective: To investigate the correlation of E-cadherin expression level with the clinical characterastics in children with acute leukemia (AL), and to explore the possible regulatory mechanism.

Methods: Real-time quantitative RT-PCR was applied to detect the expression level of E-cadherin in bone marrow samples from 135 child patients diagnosed as AL, and its relevance with clinical indicators was statistically analyzed. The expression levels of E-cadherin, β-catenin, and Akt/p-Akt were detected by using Western blot. Read More

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http://dx.doi.org/10.19746/j.cnki.issn.1009-2137.2019.02.005DOI Listing

Nationwide central diagnosis review for childhood solid tumors: From concept to realization of an Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) integrated project.

Pediatr Blood Cancer 2019 Apr 17:e27749. Epub 2019 Apr 17.

Pediatric Onco-Haematology, Stem Cell Transplantation and Cellular Therapy Division, Regina Margherita Children's Hospital, Azienda Ospedaliera-Universitaria Città della Salute e della Scienza, Torino, Italy.

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http://dx.doi.org/10.1002/pbc.27749DOI Listing

Cytokine Profiles Associated With Angiotensin II Type 1 Receptor Antibodies.

Kidney Int Rep 2019 Apr 21;4(4):541-550. Epub 2018 Dec 21.

Department of Pediatrics, Division of Pediatric Nephrology, Duke University, Durham, North Carolina, USA.

Introduction: Angiotensin II type 1 receptor antibody (AT1R-Ab), is a non-human leukocyte antigen (HLA) antibody implicated in poor renal allograft outcomes, although its actions may be mediated through a different pathway than HLA donor-specific antibodies (DSAs). Our aim was to examine serum cytokine profiles associated with AT1R-Ab and distinguish them from those associated with HLA DSA in serially collected blood samples from a cohort of pediatric renal transplant recipients.

Methods: Blood samples from 65 pediatric renal transplant recipients drawn during the first 3 months posttransplant, at 6, 12, and 24 months posttransplant, and during suspected episodes of kidney transplant rejection were tested for AT1R-Ab, HLA DSA, and a panel of 6 cytokines (tumor necrosis factor [TNF]-α, interferon [IFN]-γ, interleukin [IL]-8, IL-1β, IL-6, and IL-17). Read More

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https://linkinghub.elsevier.com/retrieve/pii/S24680249183035
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http://dx.doi.org/10.1016/j.ekir.2018.12.011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6451195PMC
April 2019
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Oncolytic Virus-Based Cytokine Expression to Improve Immune Activity in Brain and Solid Tumors.

Mol Ther Oncolytics 2019 Jun 20;13:14-21. Epub 2019 Mar 20.

The Research Institute at Nationwide Children's Hospital Center for Childhood Cancer and Blood Diseases, Columbus, OH 43205, USA.

Oncolytic viral therapy has gained significant traction as cancer therapy over the past 2 decades. Oncolytic viruses are uniquely designed both to lyse tumor cells through their replication and to recruit immune responses against virally infected cells. Increasingly, investigators are leveraging this immune response to target the immunosuppressive tumor microenvironment and improve immune effector response against bystander tumor cells. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S23727705193001
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http://dx.doi.org/10.1016/j.omto.2019.03.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6453942PMC
June 2019
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Importance of Studying the Levels of Hepcidin and Vitamin D in Egyptian Children with Chronic Hepatitis C.

J Transl Int Med 2019 Mar 29;7(1):15-21. Epub 2019 Mar 29.

Department of Microbial Biotechnology, Genetic Engineering and Biotechnology Division, National Research Centre, Cairo, Egypt.

Background And Objective: Hepcidin is the key regulator of iron metabolism and is a significant biomarker for systemic inflammatory states. Vitamin D is a powerful immunomodulator and plays a significant role in the inflammatory responses and fibrosis occurring due to hepatitis C virus (HCV) infection. This study assessed the level of vitamin D and serum hepcidin and its expression in peripheral blood of children with chronic hepatitis C (CHC) and correlated them with other serum markers to reflect iron metabolism and liver disease severity. Read More

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http://dx.doi.org/10.2478/jtim-2019-0004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6463826PMC

Direct detection of bacteremia by exploiting host-pathogen interactions of lipoteichoic acid and lipopolysaccharide.

Sci Rep 2019 Apr 17;9(1):6203. Epub 2019 Apr 17.

Physical Chemistry and Applied Spectroscopy, Chemistry Division, Los Alamos National Laboratory, Los Alamos, New Mexico, United States.

Bacteremia is a leading cause of death in sub-Saharan Africa where childhood mortality rates are the highest in the world. The early diagnosis of bacteremia and initiation of treatment saves lives, especially in high-disease burden areas. However, diagnosing bacteremia is challenging for clinicians, especially in children presenting with co-infections such as malaria and HIV. Read More

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http://dx.doi.org/10.1038/s41598-019-42502-5DOI Listing

Optimized cultivation of porcine choroid plexus epithelial cells, a blood-cerebrospinal fluid barrier model, for studying granulocyte transmigration.

Lab Invest 2019 Apr 17. Epub 2019 Apr 17.

Department of Pediatrics, Pediatric Infectious Diseases, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany.

The blood-cerebrospinal fluid barrier (BCSFB) plays important roles during the transport of substances into the brain, the pathogenesis of central nervous system (CNS) diseases, and neuro-immunological processes. Along these lines, transmigration of granulocytes across the blood-cerebrospinal fluid (CSF) barrier (BCSFB) is a hallmark of inflammatory events in the CNS. Choroid plexus (CP) epithelial cells are an important tool to generate in vitro models of the BCSFB. Read More

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http://dx.doi.org/10.1038/s41374-019-0250-9DOI Listing