Introduction: Reduced brain energy metabolism, mammalian target of rapamycin (mTOR) dysregulation, and extracellular amyloid beta (Aβ) oligomer (xcAβO) buildup are some well-known Alzheimer's disease (AD) features; how they promote neurodegeneration is poorly understood. We previously reported that xcAβOs inhibit nutrient-induced mitochondrial activity (NiMA) in cultured neurons. We now report NiMA disruption in vivo.
View Article and Find Full Text PDFWe present a case of a child with congenital thrombotic thrombocytopenic purpura found to have a compound heterozygous variant in the gene with a novel variant resulting in a large duplication of exons 9-11 of This variant was identified through additional molecular testing via a chromosomal microarray analysis. To our knowledge, this assay had not previously been utilised to identify an variant and the additional testing was possible through the involvement of a genetic counsellor.
View Article and Find Full Text PDFBackground: Distal femur fractures are known to have challenging nonunion rates. Despite various available treatment methods aimed to improve union, optimal interventions are yet to be determined. Importantly, there remains no standard agreement on what defines radiographic union.
View Article and Find Full Text PDFIntroduction: Reduced brain energy metabolism, mTOR dysregulation, and extracellular amyloid-β oligomer (xcAβO) buildup characterize AD; how they collectively promote neurodegeneration is poorly understood. We previously reported that xcAβOs inhibit N utrient-induced M itochondrial A ctivity (NiMA) in cultured neurons. We now report NiMA disruption .
View Article and Find Full Text PDFMature oil fields potentially contain multiple fluid migration pathways toward protected groundwater (total dissolved solids, TDS, in nonexempted aquifer <10,000 mg/L) because of their extensive development histories. Time-series data for water use, fluid pressures, oil-well construction, and geochemistry from the South Belridge and Lost Hills mature oil fields in California are used to explore the roles of injection/production of oil-field water and well-integrity issues in fluid migration. Injection/production of oil-field water modified hydraulic gradients in both oil fields, resulting in chemical transport from deeper groundwater and hydrocarbon-reservoir systems to aquifers in the oil fields.
View Article and Find Full Text PDFBackground: Despite the disproportionate morbidity and mortality experienced by American Indian and Alaska Native (AI/AN) persons during the coronavirus disease 2019 (COVID-19) pandemic, few studies have reported vaccine effectiveness (VE) estimates among these communities.
Methods: We conducted a test-negative case-control analysis among AI/AN persons aged ≥12 years presenting for care from January 1, 2021, through November 30, 2021, to evaluate the effectiveness of mRNA COVID-19 vaccines against COVID-19-associated outpatient visits and hospitalizations. Cases and controls were patients with ≥1 symptom consistent with COVID-19-like illness; cases were defined as those test-positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), and controls were defined as those test-negative for SARS-CoV-2.
Pickering emulsions stabilized by TEMPO-oxidized chitin nanocrystals (T-ChNCs) were developed for quercetin delivery. T-ChNCs were synthesized by TEMPO oxidation chitin and systematically characterized in terms of their physicochemical properties. T-ChNCs were rod-like with a length of 279.
View Article and Find Full Text PDFAutoimmune myelofibrosis (AIMF) is a rare disorder characterized by cytopenias and autoimmunity, with characteristic bone marrow findings that include lymphocytic infiltration and fibrosis. AIMF is described predominantly in adult populations who have systemic lupus erythematosis (SLE), with scant pediatric cases described mainly in older adolescents with SLE. Here, we described the largest single-center pediatric experience of pediatric autoimmune myelofibrosis (PAIMF) series, demonstrating both similarities and distinctions from the adult experience.
View Article and Find Full Text PDFThe outcomes and characteristics of acquired thrombotic thrombocytopenic purpura (TTP) in adolescents is poorly understood due to an absence of studies focused on this population. To better understand the life-threatening disorder in this age, we performed an analysis of adolescent patients (ages 10-21) with TTP in the Pediatric Health Information Systems database from 2009 to 2020. The primary outcomes evaluated were in-hospital mortality and rate of TTP relapse.
View Article and Find Full Text PDFCold-reactive autoimmune hemolytic anemia (AIHA) is rare among the hemolytic anemias. It results when 1 of a variety of processes causes the generation of immunoglobulin M (IgM) autoantibodies against endogenous erythrocytes, resulting in complement activation and predominantly intravascular hemolysis. Cold AIHA is typically a primary lymphoproliferative disorder with marrow B-cell clones producing pathogenic IgM.
View Article and Find Full Text PDFObjectives: To establish the incidence, risk factors and correlation with survival of thrombocytopenia and thrombocytosis (T/T) among children with HIV infection (CWH).
Design: A retrospective nested case control study of patients 0-18 years in five Baylor International Pediatric AIDS Initiative (BIPAI) centers in sub-Sahara Africa, 2004-2014.
Methods: Clinical and laboratory variables including complete blood counts (CBC) were extracted from the BIPAI electronic medical record system.
Objectives: To establish the incidence, risk factors and prognostic effect of anemia in children living with HIV (CLWH).
Design: Retrospective nested case-control study of patients 0-18 years in five centers in sub-Saharan Africa, 2004-2014.
Methods: Incident cases of anemia were identified from electronic records and matched with CLWH without anemia.
Plast Reconstr Surg Glob Open
May 2022
Background: Learning curves can reflect a surgical trainee's rate of progress and competence in acquiring new skills. The INSORB subdermal staple device has been well established to facilitate decreased closure time across various surgical procedures, with similar cosmesis, healing, and consistency as those of standard subdermal sutures. This study is unique, as it demonstrated an observable learning curve with this device when used by a junior surgeon.
View Article and Find Full Text PDFRetinoic acid receptor responder 1 (RARRES1) is among the most commonly methylated loci in multiple cancers. RARRES1 regulates mitochondrial and fatty acid metabolism, stem cell differentiation, and survival of immortalized cell lines . Here, we created constitutive knockout () mouse models to study RARRES1 function .
View Article and Find Full Text PDFImmune thrombocytopenia (ITP) is a platelet disorder. Pediatric and adult ITP have been associated with sialic acid alterations, but the pathophysiology of ITP remains elusive, and ITP is often a diagnosis of exclusion. Our analysis of pediatric ITP plasma samples showed increased anti-Thomsen-Friedenreich antigen (TF antigen) antibody representation, suggesting increased exposure of the typically sialylated and cryptic TF antigen in these patients.
View Article and Find Full Text PDFPediatr Hematol Oncol
November 2021
An understanding of the behavior of SARS-CoV-2 in pediatric hematology-oncology patients is essential to the optimal management of these patients during the COVID-19 pandemic. This study describes the characteristics and outcomes of COVID-19 disease in children with cancer or hematologic disorders treated at a large children's hospital. A retrospective cohort study was conducted at Texas Children's Cancer and Hematology Center from January 1, 2020 to September 30, 2020.
View Article and Find Full Text PDFEvans syndrome is a rare but challenging disorder in children; and despite rapidly growing evidence for targetable systemic immune dysregulation driving these "idiopathic" autoimmune cytopenias, precision diagnosis and management remains sub-optimal among these patients. We analyzed retrospective clinical data for 60 pediatric ES patients followed at 3 large tertiary referral centers in the United States over a recent 6-year period and found that definable underlying systemic immune dysregulation was identified in only 42% of these patients throughout the course of clinical care. Median time from ES diagnosis to identification of the underlying systemic immune dysregulation disorder was 1.
View Article and Find Full Text PDFImmune thrombocytopenia (ITP) is an immune-mediated disorder resulting in platelet destruction and subsequent thrombocytopenia. Bleeding symptoms range from mild cutaneous bleeding to life-threatening hemorrhage. Romiplostim, a peptide-antibody fusion product, is a thrombopoietin receptor agonist (TPO-RA) indicated for use in patients with ITP.
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