18,171 results match your criteria Childrens Geographies[Journal]


Clinical characteristics and outcome of SARS-CoV-2 infection in Italian pediatric oncology patients: a study from the Infectious Diseases Working Group of the AIEOP.

J Pediatric Infect Dis Soc 2020 Jul 11. Epub 2020 Jul 11.

Pediatric Hematology Oncology, Department of Mother and Child, Azienda Ospedaliera Universitaria Integrata Verona, Verona, Italy.

Background: Little is known as yet about the outcome of SARS-CoV-2 infection in children being treated for cancer.

Methods: We collected information on the clinical characteristics and outcomes of a cohort of 29 children (16 females and 13 males, median age 7 years, range [0-16]) diagnosed with SARS-CoV-2 infection while on chemotherapy/immunotherapy (N=26), or after stem cell transplantation (N=3) during the peak of the epidemic in Italy. These patients suffered from leukemia (N=16), lymphoma (N=3), solid tumors (N=10), and Langerhans cell histiocytosis (N=1). Read More

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http://dx.doi.org/10.1093/jpids/piaa088DOI Listing

Corpus callosotomy for refractory epilepsy in Aicardi syndrome: a case report and focused review of the literature.

World Neurosurg 2020 Jul 8. Epub 2020 Jul 8.

Department of Neurosurgery, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts. Electronic address:

Aicardi syndrome is a severe neurodevelopmental disorder that occurs primarily in females and is characterized by seizures, agenesis of the corpus callosum, and chorioretinal lacunae, which occur together in the majority of affected individuals. Seizures begin in infancy and tend to progress in intensity and are often refractory to standard multimodal medication treatments. We present here a unique case of a 12-year old girl with partial agenesis of the corpus callosum who underwent a corpus callosotomy for treatment of medically refractory epilepsy. Read More

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http://dx.doi.org/10.1016/j.wneu.2020.06.230DOI Listing

A de novo BRPF1 variant in a case of Sudden Unexplained Death in Childhood.

Eur J Med Genet 2020 Jul 8:104002. Epub 2020 Jul 8.

Robert's Program for Sudden Unexpected Death in Pediatrics, Boston Children's Hospital, USA; Department of Pediatrics, Harvard Medical School, USA; Division of General Pediatrics, Department of Pediatrics, Boston Children's Hospital, Boston, MA, USA. Electronic address:

Sudden Unexplained Death in Childhood (SUDC), the death of a child that remains unexplained after a complete autopsy and investigation, is a rare and poorly understood entity. This case report describes a 3-year-old boy with history of language delay and ptosis, who died suddenly in his sleep without known cause. A pathogenic de novo frameshift mutation in BRPF1, a gene which has been associated with the syndrome of Intellectual Developmental Disorder with Dysmorphic Facies and Ptosis (IDDDFP), was identified during a post-mortem evaluation. Read More

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http://dx.doi.org/10.1016/j.ejmg.2020.104002DOI Listing

Explanatory style in youth self-harm: an Indian qualitative study to inform intervention design.

Evid Based Ment Health 2020 Jul 10. Epub 2020 Jul 10.

Department of Global Health and Social Medicine, Harvard University T H Chan School of Public Health, Boston, Massachusetts, USA.

Background: There are very few studies that have examined the effectiveness of psychological interventions (PIs) that have been developed and tested in high-income countries to reduce self-harm in low and middle-income countries.

Objective: To evaluate the perspectives and explanatory styles of youth with self-harm and their caregivers to inform the design of an evidence based PI in a non-Western cultural setting. An additional objective was to suggest ways of integrating local practices and traditions to enhance its acceptability. Read More

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http://dx.doi.org/10.1136/ebmental-2020-300159DOI Listing

Human Milk Oligosaccharide profiles and allergic disease up to 18 years.

J Allergy Clin Immunol 2020 Jul 7. Epub 2020 Jul 7.

Department of Pediatrics and Larsson-Rosenquist Foundation Mother-Milk-Infant Center of Research Excellence, University of California San Diego, La Jolla, US.

Background: Human Milk Oligosaccharides (HMO) are a diverse range of sugars secreted in breast milk that have direct and indirect effects on immunity. The profiles of HMOs produced differ between mothers.

Objective: To determine the relationship between maternal HMO profiles and offspring allergic diseases up to 18 years METHODS: Colostrum and early lactation milk samples were collected from 285 mothers enrolled in a high allergy risk birth cohort, the Melbourne Atopy Cohort Study. Read More

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http://dx.doi.org/10.1016/j.jaci.2020.06.027DOI Listing

Suffering Children and Handcuffed Doctors: US Immigration Policy and a Call for Advocacy.

Authors:
Bonnie Arzuaga

Pediatrics 2020 Jul 9. Epub 2020 Jul 9.

Division of Newborn Medicine, Boston Children's Hospital; and Department of Pediatrics, Harvard Medical School, Harvard University, Boston, Massachusetts

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http://dx.doi.org/10.1542/peds.2020-0495DOI Listing

Association of Isolated Congenital Heart Disease with Fetal Brain Maturation.

AJNR Am J Neuroradiol 2020 Jul 9. Epub 2020 Jul 9.

Neurology (C.K.R.), Fetal-Neonatal Neuroimaging

Background And Purpose: Brain MRI of newborns with congenital heart disease show signs of immaturity relative to healthy controls. Our aim was to determine whether the semiquantitative fetal total maturation score can detect abnormalities in brain maturation in fetuses with congenital heart disease in the second and third trimesters.

Materials And Methods: We analyzed data from a prospective study of fetuses with and without congenital heart disease who underwent fetal MR imaging at 25-35 weeks' gestation. Read More

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http://dx.doi.org/10.3174/ajnr.A6635DOI Listing

Cerebrospinal Fluid Magnetic Resonance Imaging: Improving Early Diagnosis of Autism and Other Neurodevelopmental Conditions.

Biol Psychiatry Cogn Neurosci Neuroimaging 2020 Jul;5(7):635-637

Department of Pathology, Boston Children's Hospital, Boston, Massachusetts. Electronic address:

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http://dx.doi.org/10.1016/j.bpsc.2020.05.007DOI Listing

Prediction of mortality in very low birth weight neonates in Spain.

PLoS One 2020 9;15(7):e0235794. Epub 2020 Jul 9.

Neonatology Department, Hospital Clínic, BCNatal, Hospital Clínic- Hospital Sant Joan de Déu, Barcelona University, Barcelona, Spain.

Objective: Predictive models for preterm infant mortality have been developed internationally, albeit not valid for all populations. This study aimed to develop and validate different mortality predictive models, using Spanish data, to be applicable to centers with similar morbidity and mortality.

Methods: Infants born alive, admitted to NICU (BW<1500 g or GA<30 w), and registered in the SEN1500 database, were included. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0235794PLOS

Everything Illuminated-Clostridium perfringens β-toxin.

Authors:
Wayne I Lencer

Cell Host Microbe 2020 Jul;28(1):5-6

Boston Children's Hospital, Boston, MA 02115, USA; Harvard Medical School, Boston, MA 02115, USA; Harvard Digestive Disease Center, Boston, MA 02115, USA. Electronic address:

In this issue of Cell Host & Microbe, Bruggisser et al. show that Clostridium perfringens β-toxin (CPB) binds platelet endothelial cell adhesion molecule-1 (PECAM-1) (also known as CD31) to induce membrane pores. The discovery explains the cell type specificity for CPB and, in large part, the basic pathophysiology of disease. Read More

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http://dx.doi.org/10.1016/j.chom.2020.06.006DOI Listing

p190A RhoGAP induces CDH1 expression and cooperates with E-cadherin to activate LATS kinases and suppress tumor cell growth.

Oncogene 2020 Jul 8. Epub 2020 Jul 8.

GI Cell Biology Laboratory, Boston Children's Hospital and Harvard Medical School, Boston, MA, 02115, USA.

The ARHGAP35 gene encoding p190A RhoGAP (p190A) is significantly altered by both mutation and allelic deletion in human cancer, but the functional implications of such alterations are not known. Here, we demonstrate for the first time that p190A is a tumor suppressor using a xenograft mouse model with carcinoma cells harboring defined ARHGAP35 alterations. In vitro, restoration of p190A expression in carcinoma cells promotes contact inhibition of proliferation (CIP) through activation of LATS kinases and phosphorylation of the proto-oncogenic transcriptional co-activator YAP. Read More

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http://dx.doi.org/10.1038/s41388-020-1385-2DOI Listing

MYSM1 maintains ribosomal protein gene expression in hematopoietic stem cells to prevent hematopoietic dysfunction.

JCI Insight 2020 Jul 9;5(13). Epub 2020 Jul 9.

Department of Physiology.

Ribosomopathies are congenital disorders caused by mutations in the genes encoding ribosomal and other functionally related proteins. They are characterized by anemia, other hematopoietic and developmental abnormalities, and p53 activation. Ribosome assembly requires coordinated expression of many ribosomal protein (RP) genes; however, the regulation of RP gene expression, especially in hematopoietic stem cells (HSCs), remains poorly understood. Read More

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http://dx.doi.org/10.1172/jci.insight.125690DOI Listing

Prevalence, Patterns, and Correlates of Pain in Medically Hospitalized Pediatric Patients With Somatic Symptom and Related Disorders.

Psychosomatics 2020 May 22. Epub 2020 May 22.

Department of Psychiatry, Boston Children's Hospital and Harvard Medical School, Boston, MA.

Background: Somatic symptom and related disorders (SSRDs) and pain are highly comorbid in the pediatric population. Little is known about the prevalence of pain and factors that may predispose and perpetuate pain in hospitalized youth with SSRD.

Objective: To describe the prevalence of pain and widespread pain symptoms in hospitalized youth with SSRD and describe differences between patients who endorsed limited (1-4 sites) versus widespread (5-8 sites) pain. Read More

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http://dx.doi.org/10.1016/j.psym.2020.05.008DOI Listing

Transcatheter balloon atrial septostomy in thoraco-omphalopagus conjoined twins.

Postepy Kardiol Interwencyjnej 2020 Jun 23;16(2):209-212. Epub 2020 Jun 23.

Department of Pediatrics, Division of Pediatric Cardiology, University of Minnesota, Masonic Children's Hospital, Minneapolis, MN, USA.

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http://dx.doi.org/10.5114/aic.2020.96067DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7333194PMC

Author Correction: Clinical Validation of Targeted and Untargeted Metabolomics Testing for Genetic Disorders: A 3 Year Comparative Study.

Sci Rep 2020 Jul 7;10(1):11160. Epub 2020 Jul 7.

Department of Laboratory Medicine, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.

An amendment to this paper has been published and can be accessed via a link at the top of the paper. Read More

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http://dx.doi.org/10.1038/s41598-020-68532-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7341886PMC

An evolving problem-Mental health symptoms among health care workers during COVID-19 pandemic.

Asian J Psychiatr 2020 Jun 23;54:102257. Epub 2020 Jun 23.

Department of Medicine, Reading Hospital -Tower Health System, Reading, PA, United States.

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http://dx.doi.org/10.1016/j.ajp.2020.102257DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7309875PMC

EEG features of brain injury during extracorporeal membrane oxygenation in children.

Neurology 2020 Jul 6. Epub 2020 Jul 6.

Division of Epilepsy and Neurophysiology (AJS, MLD, MHL, TL, PLP).

Objective: To examine EEG features of major pathophysiology in children undergoing extracorporeal membrane oxygenation (ECMO).

Methods: Single-center, retrospective study of 201 pediatric ECMO patients, using the first 24 hours of continuous EEG (cEEG) monitoring, collating background activity and electrographic seizures (ES) with imaging, ECMO type and outcome.

Results: Severely abnormal cEEG background occurred in 12% (25/201), and was associated with death (sensitivity 0. Read More

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http://dx.doi.org/10.1212/WNL.0000000000010188DOI Listing

Dental Caries in Children Under Five Years of Age in Mongolia.

Int J Environ Res Public Health 2020 Jul 1;17(13). Epub 2020 Jul 1.

Faculty of Human Life Sciences, Tokushima Bunri University, Tokushima-shi, Tokushima 770-8514, Japan.

During the Japan International Cooperation Agency (JICA) partnership program in Mongolia, six times between October 2017 and October 2019, education for caries prevention, a questionnaire of daily oral health behavior, and an oral examination for parents and children aged 0-5 years old were done. The target parent population were middle socio-economic class families. In total, there were 2223 participants. Read More

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http://dx.doi.org/10.3390/ijerph17134741DOI Listing

ITK deficiency presenting as Autoimmune Lymphoproliferative Syndrome.

J Allergy Clin Immunol 2020 Jul 3. Epub 2020 Jul 3.

Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA. Electronic address:

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http://dx.doi.org/10.1016/j.jaci.2020.06.019DOI Listing

A Single Cell Dissociation Approach for Molecular Analysis of Urinary Bladder in the Mouse Following Spinal Cord Injury.

J Vis Exp 2020 Jun 17(160). Epub 2020 Jun 17.

Department of Urology, Boston Children's Hospital; Department of Surgery, Harvard Medical School;

We describe the implementation of spinal cord injury in mice to elicit detrusor-sphincter dyssynergia, a functional bladder outlet obstruction, and subsequent bladder wall remodeling. To facilitate assessment of the cellular composition of the bladder wall in non-injured control and spinal cord injured mice, we developed an optimized dissociation protocol that supports high cell viability and enables the detection of discrete subpopulations by flow cytometry. Spinal cord injury is created by complete transection of the thoracic spinal cord. Read More

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http://dx.doi.org/10.3791/61455DOI Listing

Post-operative complications following feminizing genitoplasty in moderate to severe genital atypia: Results from a multicenter, observational prospective cohort study.

J Pediatr Urol 2020 Jun 1. Epub 2020 Jun 1.

University of California San Francisco Medical Center, United States. Electronic address:

Disorders/differences of sex development (DSD) are congenital conditions in which there is atypical chromosomal, gonadal and/or phenotypic sex. While there remains controversy around the traditionally binary concept of sex, most patients with DSD are reared either male or female depending on their genetic sex, gonadal sex, genital phenotype and status of their internal genital tract. This study uses prospective data from 12 institutions across the United States that specialize in DSD care. Read More

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http://dx.doi.org/10.1016/j.jpurol.2020.05.166DOI Listing

Introduction to the Hearing Research special issue on inner ear gene therapy.

Hear Res 2020 Jun 18:108010. Epub 2020 Jun 18.

Kresge Hearing Research Institute, Department of Otolaryngology, Head and Neck Surgery, Michigan Medicine, Ann Arbor, MI, USA. Electronic address:

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http://dx.doi.org/10.1016/j.heares.2020.108010DOI Listing

Review of Transcranial Magnetic Stimulation in Epilepsy.

Clin Ther 2020 Jul 2. Epub 2020 Jul 2.

Neuromodulation Program, Department of Neurology, Division of Epilepsy and Clinical Neurophysiology, Boston Children's Hospital, Boston, MA, USA; FM Kirby Neurobiology Center, Department of Neurology, Boston Children's Hospital, Boston, MA, USA; Berenson-Allen Center for Noninvasive Brain Stimulation, Beth Israel Deaconess Medical Center, Boston, MA, USA. Electronic address:

Purpose: Despite the availability of numerous pharmacologic and nonpharmacologic antiseizure therapies, a fraction of patients with epilepsy remain refractory to current treatment options, underscoring the need for novel drugs and neuromodulatory therapies. Transcranial magnetic stimulation (TMS), coupled with either electromyography or electroencephalography, enables rapid measurement of the cortical excitation/inhibition ratio, which is pathologically shifted toward excess excitability in patients with epilepsy. In this review, we summarize: (1) TMS protocols that have been deployed to identify promising compounds in the antiepilepsy drug (AED)-development pipeline, and (2) the therapeutic potential of TMS in the treatment of drug-resistant seizures. Read More

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http://dx.doi.org/10.1016/j.clinthera.2020.05.016DOI Listing

Variation in management of pediatric post-traumatic urine leaks.

Eur J Trauma Emerg Surg 2020 Jul 4. Epub 2020 Jul 4.

Department of Surgery, Boston Children's Hospital, Fegan 3, 300 Longwood Avenue, Boston, MA, 02115, USA.

Purpose: High-grade pediatric renal trauma may be associated with a urine leak and appropriate management remains unclear.

Method: Data on patients with a traumatic renal injury were retrieved from the trauma registry and data warehouse of a pediatric level 1 trauma center over a 15-year period. Demographics, diagnoses, imaging, interventions performed, and follow-up information on patients with a urine leak were analyzed. Read More

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http://dx.doi.org/10.1007/s00068-020-01430-0DOI Listing

Deep learning with noisy labels: Exploring techniques and remedies in medical image analysis.

Med Image Anal 2020 Jun 20;65:101759. Epub 2020 Jun 20.

Department of Radiology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.

Supervised training of deep learning models requires large labeled datasets. There is a growing interest in obtaining such datasets for medical image analysis applications. However, the impact of label noise has not received sufficient attention. Read More

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http://dx.doi.org/10.1016/j.media.2020.101759DOI Listing

Congenital Disseminated Pyogenic Granuloma: Characterization of an Aggressive Multisystemic Disorder.

J Pediatr 2020 Jul 2. Epub 2020 Jul 2.

Division of Vascular and Interventional Radiology, Boston Children's Hospital, Boston, Massachusetts; Harvard Medical School, Boston, Massachusetts. Electronic address:

Objective: To describe the clinical, radiological and histopathological features of "congenital disseminated pyogenic granuloma" (CDPG) involving various organs with high morbidity related to cerebral hemorrhagic involvement.

Study Design: We searched the database of the Vascular Anomalies Center at Boston Children's Hospital from 1999 to 2019 for patients diagnosed as having multiple vascular lesions, visceral vascular tumors, congenital hemangiomatosis, multiple pyogenic granulomas or multiple vascular lesions without a definite diagnosis. A retrospective review of the medical records, photographs, histopathological and imaging studies was performed. Read More

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http://dx.doi.org/10.1016/j.jpeds.2020.06.079DOI Listing

Invited Expert Opinion: Guidelines for improving the use and presentation of P values.

J Thorac Cardiovasc Surg 2020 Apr 30. Epub 2020 Apr 30.

Department of Surgery, Boston Children's Hospital, Harvard Medical School, Boston, Mass; Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Harvard Medical School, Boston, Mass. Electronic address:

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http://dx.doi.org/10.1016/j.jtcvs.2020.03.156DOI Listing

Severe bilateral ankyloblepharon filiforme adnatum.

J AAPOS 2020 Jul 1. Epub 2020 Jul 1.

Department of Ophthalmology, Research Institute of Ophthalmology, Cairo University, Egypt.

We present a case of bilateral ankyloblepharon filiforme adnatum in 1-day-old girl and describe our surgical approach. The bands connecting the upper and lower eyelids of both eyes were severed using blunt scissors. Point bleeding at the cut bands stopped in 1-2 minutes, without the need for cauterization or compression. Read More

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http://dx.doi.org/10.1016/j.jaapos.2020.04.002DOI Listing

Neck CT angiography examinations for pediatric oropharyngeal trauma: diagnostic yield and proposal of a new targeted technique.

Pediatr Radiol 2020 Jul 3. Epub 2020 Jul 3.

Department of Radiology, Boston Children's Hospital, 300 Longwood Ave., Boston, MA, 02115, USA.

Background: Neck computed tomography (CT) angiography is commonly ordered for pediatric patients with soft palate trauma to exclude vascular injury. Debate exists regarding what type of imaging is indicated in this setting, particularly amid growing concern that standard neck CT angiography results in considerable radiation exposure.

Objective: To assess the diagnostic yield and estimated dose reduction of a novel targeted protocol extending from the skull base to the hyoid bone to evaluate pediatric oropharyngeal trauma. Read More

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http://dx.doi.org/10.1007/s00247-020-04737-7DOI Listing

Predictors of Increased Lactate in Neonatal Cardiac Surgery: The Impact of Cardiopulmonary Bypass.

J Cardiothorac Vasc Anesth 2020 Jun 10. Epub 2020 Jun 10.

Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Harvard Medical School, Boston, MA.

Objective: Hyperlactatemia develops intraoperatively during cardiac surgery and is associated with postoperative mortality. This study aimed to determine the factors that lead to an increase in lactate during cardiopulmonary bypass (CPB) in neonates undergoing cardiac surgery.

Design: Retrospective study from July 2015 to December 2018. Read More

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http://dx.doi.org/10.1053/j.jvca.2020.06.009DOI Listing

Direct Activation of Cortical Neurons in the Primary Somatosensory Cortex of the Rat in Vivo Using Focused Ultrasound.

Ultrasound Med Biol 2020 Jun 30. Epub 2020 Jun 30.

Division of Newborn Medicine, Dept. Pediatrics, Boston Children's Hospital/Harvard Medical School, Boston, Massachusetts, USA. Electronic address:

We address the recent controversy over whether focused ultrasound (FUS) activates cortical neurons directly or indirectly by initially activating auditory pathways. We obtained two types of evidence that FUS can directly activate cortical neurons. The depth profile of the local field potential (LFP) in the barrel cortex of the rat in vivo indicated a generator was located within the cortical gray matter. Read More

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http://dx.doi.org/10.1016/j.ultrasmedbio.2020.06.003DOI Listing

High-dose versus low-dose tranexamic acid for paediatric craniosynostosis surgery: a double-blind randomised controlled non-inferiority trial.

Br J Anaesth 2020 Jun 30. Epub 2020 Jun 30.

Department of Anaesthesia, IRCCS Istituto Giannina Gaslini, Genoa, Italy.

Background: Tranexamic acid (TXA) reduces blood loss and transfusion in paediatric craniosynostosis surgery. The hypothesis is that low-dose TXA, determined by pharmacokinetic modelling, is non-inferior to high-dose TXA in decreasing blood loss and transfusion in children.

Methods: Children undergoing craniosynostosis surgery were enrolled in a two-centre, prospective, double-blind, randomised, non-inferiority controlled trial to receive high TXA (50 mg kg followed by 5 mg kg h) or low TXA (10 mg kg followed by 5 mg kg h). Read More

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http://dx.doi.org/10.1016/j.bja.2020.05.054DOI Listing

A 23-Year Follow-Up of a Patient with Gain-of-Function IkB-Alpha Mutation and Stable Full Chimerism After Hematopoietic Stem Cell Transplantation.

J Clin Immunol 2020 Jul 2. Epub 2020 Jul 2.

University Department of Pediatrics, Unit of Immune and Infectious Diseases, Childrens' Hospital Bambino Gesù, Piazza S. Onofrio 4, Rome, Italy.

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http://dx.doi.org/10.1007/s10875-020-00780-zDOI Listing

The Gluten-Free Family Ripple Effect: The Tides that Bond and the Tides that Divide.

Dig Dis Sci 2020 Jul 2. Epub 2020 Jul 2.

Harvard Celiac Research Program, Boston Children's Hospital, Boston, MA, 0211, USA.

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http://dx.doi.org/10.1007/s10620-020-06428-7DOI Listing

Surveillance Practice for Sonographic Detection of Intracranial Abnormalities in Premature Neonates: A Snapshot of Current Neonatal Cranial Ultrasound Practice in Australia.

Ultrasound Med Biol 2020 Jun 29. Epub 2020 Jun 29.

Department of Medical Imaging and Radiation Sciences, Faculty of medicine, Nursing and Health Sciences, Monash University, Clayton, Victoria, Australia. Electronic address:

There are no publications reporting on scan duration and Doppler use during neonatal cranial ultrasound scans. We investigated current practice of neonatal cranial ultrasound at four large tertiary neonatal intensive care units in Australia. Cranial scans were prospectively recorded between March 2015 and November 2016. Read More

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http://dx.doi.org/10.1016/j.ultrasmedbio.2020.06.002DOI Listing

Did the rising tide float all boats?

Authors:
Alyna T Chien

J Pediatr 2020 Jun 29. Epub 2020 Jun 29.

Division of General Pediatrics, Harvard Medical School, Boston Massachusetts; Department of Pediatrics, Harvard Medical School, Boston Massachusetts. Electronic address:

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http://dx.doi.org/10.1016/j.jpeds.2020.04.071DOI Listing

Haematopoietic Cell Transplantation in Chronic Granulomatous Disease: a Study on 712 Children and Adults.

Blood 2020 Jul 2. Epub 2020 Jul 2.

Newcastle University, United Kingdom.

Chronic Granulomatous Disease (CGD) is a primary immunodeficiency resulting in life-threatening infections and inflammatory complications. Allogeneic hematopoietic cell transplantation (allo-HCT) can cure patients, but indication to transplant remains controversial. We performed a retrospective multicentre study on 712 patients with CGD undergoing allo-HCT transplanted in EBMT centres between 1993 and 2018. Read More

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http://dx.doi.org/10.1182/blood.2020005590DOI Listing

The Effectiveness of Nurses Implemented Music Add-on Therapy in Children with Behavioral Problems.

Indian J Psychol Med 2020 May-Jun;42(3):274-280. Epub 2020 Apr 25.

Department of Biostatistics, National Institute of Mental Health and Neuro Sciences, Bengaluru, Karnataka, India.

Background: Increasing rates of behavioral problems among children in India necessitates newer ways of managing them with medical and nonmedical approaches. Music add-on therapy is a method for treating mental disturbances. This study examines the effectiveness of music add-on therapy in managing children with behavioral problems. Read More

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http://dx.doi.org/10.4103/IJPSYM.IJPSYM_240_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7320726PMC

Protocol for a multinational risk-stratified randomised controlled trial in paediatric Crohn's disease: methotrexate versus azathioprine or adalimumab for maintaining remission in patients at low or high risk for aggressive disease course.

BMJ Open 2020 Jul 1;10(7):e034892. Epub 2020 Jul 1.

Department of Paediatric Gastroenterology, Royal Hospital for Children Glasgow, Glasgow, UK.

Introduction: Immunomodulators such as thiopurines (azathioprine (AZA)/6-mercaptopurine (6MP)), methotrexate (MTX) and biologics such as adalimumab (ADA) are well established for maintenance of remission within paediatric Crohn's disease (CD). It remains unclear, however, which maintenance medication should be used first line in specific patient groups.

Aims: To compare the efficacy of maintenance therapies in newly diagnosed CD based on stratification into high and low-risk groups for severe CD evolution; MTX versus AZA/6MP in low-risk and MTX versus ADA in high-risk patients. Read More

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http://dx.doi.org/10.1136/bmjopen-2019-034892DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7332179PMC

Association of guideline publication and delays to treatment in pediatric status epilepticus.

Neurology 2020 Jul 1. Epub 2020 Jul 1.

Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, MA

Objective: To determine whether publication of evidence on delays in time to treatment shortens time to treatment in pediatric refractory convulsive status epilepticus (rSE), we compared time to treatment before (2011-2014) and after (2015-2019) publication of evidence of delays in treatment of rSE in the pediatric status epilepticus research group (pSERG) as assessed by patient interviews and record review.

Methods: We performed a retrospective analysis of a prospectively collected dataset from June 2011 to September 2019 on pediatric patients (1 month to 21 years of age) with rSE.

Results: We studied 328 patients (56% males) with median (p-p) age of 3. Read More

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http://dx.doi.org/10.1212/WNL.0000000000010174DOI Listing

Pure-tone audiometry without bone-conduction thresholds: using the digits-in-noise test to detect conductive hearing loss.

Int J Audiol 2020 Jul 1:1-8. Epub 2020 Jul 1.

Department of Speech-Language Pathology and Audiology, University of Pretoria, Gauteng, South Africa.

COVID-19 has been prohibitive to traditional audiological services. No- or low-touch audiological assessment outside a sound-booth precludes test batteries including bone conduction audiometry. This study investigated whether conductive hearing loss (CHL) can be differentiated from sensorineural hearing loss (SNHL) using pure-tone air conduction audiometry and a digits-in-noise (DIN) test. Read More

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http://dx.doi.org/10.1080/14992027.2020.1783585DOI Listing

Working with families: A systematic scoping review of family-centred care in universal, community-based maternal, child, and family health services.

J Child Health Care 2020 Jun 30:1367493520930172. Epub 2020 Jun 30.

Judith Lumley Centre , La Trobe University.

Children's long- and short-term health and developmental outcomes can be improved when families are engaged and supported, and inform care planning. Family-centred care (FCC) underpins policy directions for universal, community-based, child and family health services in the early years, although its implementation in this context is poorly understood. This systematic scoping review of the current literature aimed to improve understanding of FCC implementation in maternal, child, and family health universal services. Read More

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http://dx.doi.org/10.1177/1367493520930172DOI Listing

Single-molecule dynamics of Dishevelled at the plasma membrane and Wnt pathway activation.

Proc Natl Acad Sci U S A 2020 Jun 29. Epub 2020 Jun 29.

Department of Systems Biology, Harvard Medical School, Boston, MA 02115;

Dvl (Dishevelled) is one of several essential nonenzymatic components of the Wnt signaling pathway. In most current models, Dvl forms complexes with Wnt ligand receptors, Fzd and LRP5/6 at the plasma membrane, which then recruits the destruction complex, eventually leading to inactivation of β-catenin degradation. Although this model is widespread, direct evidence for the individual steps is lacking. Read More

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http://dx.doi.org/10.1073/pnas.1910547117DOI Listing

LIN28B regulates transcription and potentiates MYCN-induced neuroblastoma through binding to ZNF143 at target gene promotors.

Proc Natl Acad Sci U S A 2020 Jun 29. Epub 2020 Jun 29.

Department of Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA 02215;

LIN28B is highly expressed in neuroblastoma and promotes tumorigenesis, at least, in part, through inhibition of microRNA biogenesis. Here, we report that overexpression of either wild-type (WT) LIN28B or a LIN28B mutant that is unable to inhibit processing increases the penetrance of MYCN-induced neuroblastoma, potentiates the invasion and migration of transformed sympathetic neuroblasts, and drives distant metastases in vivo. Genome-wide chromatin immunoprecipitation coupled with massively parallel DNA sequencing (ChIP-seq) and coimmunoprecipitation experiments show that LIN28B binds active gene promoters in neuroblastoma cells through protein-protein interaction with the sequence-specific zinc-finger transcription factor ZNF143 and activates the expression of downstream targets, including transcription factors forming the adrenergic core regulatory circuitry that controls the malignant cell state in neuroblastoma as well as and that are involved in neuronal cell adhesion and migration. Read More

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http://dx.doi.org/10.1073/pnas.1922692117DOI Listing

Electronic Documentation in Pediatrics: The Rationale and Functionality Requirements.

Pediatrics 2020 Jul 29;146(1). Epub 2020 Jun 29.

Divisions of Health Informatics and Emergency Medicine, Department of Pediatrics, University of Pittsburgh School of Medicine and UPMC Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania.

Clinical documentation is a fundamental component of the practice of medicine. It has significantly evolved over the past decade, largely because of the growth of health information technology and electronic health records. Although government agencies and other professional organizations have published position statements on the structure and use of electronic documentation, few have specifically addressed the documentation needs for the care of children. Read More

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http://dx.doi.org/10.1542/peds.2020-1682DOI Listing

Staphylococcal meningitis therapy with linezolid in a young infant: efficacy, CSF levels and side effects.

Ital J Pediatr 2020 Jun 29;46(1):90. Epub 2020 Jun 29.

Department of Pediatric Medicine, Laboratory of Metabolic Biochemistry Unit, Bambino Gesù Children's Hospital, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

Background: Linezolid is a synthetic antibiotic which is active against most Gram-positive bacteria, especially on Staphylococcus aureus. Its administration can be required when the infection is due to staphylococcus strains, which are resistant to vancomycin. Although mostly well tolerated, some mild to moderate side effects have been reported. Read More

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http://dx.doi.org/10.1186/s13052-020-00854-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7325046PMC

Myofiber organization in the failing systemic right ventricle.

J Cardiovasc Magn Reson 2020 06 29;22(1):49. Epub 2020 Jun 29.

Cardiac Registry, Departments of Cardiology, Pathology and Cardiac Surgery, Boston Children's Hospital, 300 Longwood Ave, Boston, MA 02115, USA. and Department of Pediatrics, Harvard Medical School, Boston, MA, USA.

Background: The right ventricle (RV) often fails when functioning as the systemic ventricle, but the cause is not understood. We tested the hypothesis that myofiber organization is abnormal in the failing systemic right ventricle.

Methods: We used diffusion-weighted cardiovascular magnetic resonance imaging to examine 3 failing hearts explanted from young patients with a systemic RV and one structurally normal heart with postnatally acquired RV hypertrophy for comparison. Read More

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http://dx.doi.org/10.1186/s12968-020-00637-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7322876PMC

Stress ulcer prophylaxis versus placebo-a blinded randomized control trial to evaluate the safety of two strategies in critically ill infants with congenital heart disease (SUPPRESS-CHD).

Trials 2020 Jun 29;21(1):590. Epub 2020 Jun 29.

Harvard Medical School, Boston, MA, USA.

Background: Critically ill infants with congenital heart disease (CHD) are often prescribed stress ulcer prophylaxis (SUP) to prevent upper gastrointestinal bleeding, despite the low incidence of stress ulcers and limited data on the safety and efficacy of SUP in infants. Recently, SUP has been associated with an increased incidence of hospital-acquired infections, community-acquired pneumonia, and necrotizing enterocolitis. The objective of this pilot study is to investigate the feasibility of performing a randomized controlled trial to assess the safety and efficacy of withholding SUP in infants with congenital heart disease admitted to the cardiac intensive care unit. Read More

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http://dx.doi.org/10.1186/s13063-020-04513-wDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7322718PMC

Botulinum Toxin as a Treatment for Feeding Difficulties in Young Children.

J Pediatr 2020 Jun 26. Epub 2020 Jun 26.

Aerodigestive Center, Center for Motility and Functional Gastrointestinal Disorders, Division of Gastroenterology, Hepatology & Nutrition, Boston Children's Hospital, Boston, MA. Electronic address:

Objective: To determine the effectiveness of intrapyloric botulinum toxin injection (IPBI) for treatment of feeding disorders and associated gastrointestinal symptoms in very young children.

Study Design: A single-center retrospective study of patients 2 months to 5 years old who received IPBI at Boston Children's Hospital from May 2007 to June 2019 was performed. Charts were reviewed for demographic data, comorbidities, symptoms leading to IPBI, oral and tube feeding data, symptom improvement after IPBI, and need for repeat injections. Read More

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http://dx.doi.org/10.1016/j.jpeds.2020.06.063DOI Listing