Single Ultra-High-Dose Cholecalciferol to Prevent Vitamin D Deficiency in Pediatric Hematopoietic Stem Cell Transplantation.

Vitamin D deficiency is prevalent among childhood hematopoietic stem cell transplantation (HSCT) recipients and associated with inferior survival at 100 days after transplantation. Achieving and maintaining therapeutic vitamin D levels in HSCT recipients is extremely challenging in the first 3 to 6 months after transplantation due to poor compliance in the setting of mucositis and the concomitant use of critical transplantation drugs that interfere with vitamin D absorption. We sought to evaluate the safety and efficacy of a single, ultra-high-dose of vitamin D given before childhood HSCT to maintain levels in a therapeutic range during the peritransplantation period.

Cluster of differentiation 4+ T-cell counts and human immunodeficiency virus-1 viral load in patients coinfected with hepatitis B virus and hepatitis C virus.

Background: Coinfections of human immunodeficiency virus (HIV) with hepatitis viruses may affect the progress of disease and response to therapy.

Objectives: To study the incidence of hepatitis B virus (HBV) and hepatitis C virus (HCV) coinfections in HIV-positive patients and their influence on HIV-1 viral load and cluster of differentiation 4+ (CD4+) T-cell counts.

Materials And Methods: This pilot study was done on 179 HIV-positive patients attending antiretroviral therapy (ART) centre.

Mutations in () gene in Indian children with hemolytic uremic syndrome.

Background: Mutations in the gene account for an important proportion of patients with atypical hemolytic uremic syndrome (aHUS) who characteristically show multiple relapses, no response to plasma exchange and low recurrence risk in allograft. We screened for mutations in in patients with and without circulating anti-factor H (FH) antibodies-associated aHUS.

Methods: We estimated surface expression by flow cytometry and sequenced the gene in 23 and 56 patients with and without circulating anti-FH antibodies, respectively.

Post-Transplant CD34 Selected Stem Cell "Boost" for Mixed Chimerism after Reduced-Intensity Conditioning Hematopoietic Stem Cell Transplantation in Children and Young Adults with Primary Immune Deficiencies.

Mixed chimerism and eventual graft loss occurs in a proportion of children with primary immune deficiencies receiving alemtuzumab, fludarabine, and melphalan reduced-intensity conditioning (RIC) regimens before allogeneic hematopoietic stem cell transplantation (HSCT). We investigated the usefulness of a CD34 selected stem cell "boost" without conditioning to treat mixed chimerism in children and young adults who received predominantly an alemtuzumab, fludarabine, and melphalan RIC regimen for primary immune deficiencies and reported the outcomes. Patients with a primary immune deficiency disorder who were either enrolled on a prospective CD34 boost study for treatment of mixed chimerism from 2011 to 2014 (n = 9) or treated with a CD34 boost on a clinical basis from 2014 to 2016 (n = 3) were included in this analysis.

(teak) - A review on its phytochemical and therapeutic potential.

Linn (Teak), is locally known as Sagwan, belongs to Lamiaceae family. It is one of the most valuable timber in the world, due to its beautiful surface and its resistance to termite and fungal damage. The main active ingredient compounds that are responsible for these action are tectoquinone, lapachol and deoxylapachol.

Characterization of genetic predisposition and autoantibody profile in atypical haemolytic-uraemic syndrome.

We previously reported that Indian paediatric patients with atypical haemolytic-uraemic syndrome (aHUS) showed high frequencies of anti-complement factor H (FH) autoantibodies that are correlated with homozygous deletion of the genes for FH-related proteins 1 and 3 (FHR1 and FHR3) (FHR1/3 ). We now report that Indian paediatric aHUS patients without anti-FH autoantibodies also showed modestly higher frequencies of the FHR1/3 genotype. Further, when we characterized epitope specificities and binding avidities of anti-FH autoantibodies in aHUS patients, most anti-FH autoantibodies were directed towards the FH cell-surface anchoring polyanionic binding site-containing C-terminal short conservative regions (SCRs) 17-20 with higher binding avidities than for native FH.

Transradial cerebral angiography: techniques and outcomes.

Background: Despite several retrospective studies analyzing the safety and efficacy of transradial access (TRA) versus transfemoral access (TFA) for cerebral angiography, this transition for neurointerventional procedures has been gradual. Nonetheless, based on our positive initial institutional experience with TRA for mechanical thrombectomy in acute ischemic stroke patients, we have started transitioning more of our cerebral angiography cases to TRA. Here we present our single institution experience.

Protected Chloroethyl and Chloropropyl Amines as Conformationally Unrestricted Annulating Reagents.

The purpose of this letter is to document the use of protected chloroethyl and chloropropyl amines as conformationally unrestricted ambiphilic reagents that undergo annulation reactions with Michael acceptors. This reaction is wide in scope and utilizes reagents that are commercially available, inexpensive, and stable. Furthermore, this reaction is easy to execute and proceeds rapidly.

Fluorescent metal quantum clusters: an updated overview of the synthesis, properties, and biological applications.

Metal quantum clusters are evolving as excellent systems for a wide range of biological applications due to their small size (∼2 nm), tunable optical properties, including optical absorption, photoluminescence (UV to NIR), nonlinear optical properties (two-photon absorption, two-photon fluorescence, and second/third harmonic generation), ultrafast dynamics (relaxation kinetics, electron-phonon coupling, and radiative emission), and magnetism. These excellent properties have resulted in their use in a broad range of applications, including the sensing of ions (heavy metal ions, anions), biomolecules (proteins, DNA, miRNA, and enzymes), biological cells, diagnosis, and therapy. This article presents an introduction to metal quantum clusters, including a brief history of research in this system and an overview of the existing theories to understand their properties.

National Patterns of Carotid Revascularization Before and After the Carotid Revascularization Endarterectomy vs Stenting Trial (CREST).

Importance: The Carotid Revascularization Endarterectomy vs Stenting Trial (CREST) showed greater safety of carotid artery stenting (CAS) in patients younger than 70 years and carotid endarterectomy (CEA) in those older than 70 years. It is unknown how the result of CREST has influenced carotid revascularization choices in the United States.

Objective: To evaluate national patterns in CAS performance in patients older than 70 years in the post-CREST (2011-2014) compared with the pre-CREST (2007-2010) era.

RAB27B requirement for stretch-induced exocytosis in bladder umbrella cells.

Umbrella cells, which must maintain a tight barrier, modulate their apical surface area during bladder filling by exocytosis of an abundant, subapical pool of discoidal- and/or fusiform-shaped vesicles (DFVs). Despite the importance of this trafficking event for bladder function, the pathways that promote DFV exocytosis remain to be identified. We previously showed that DFV exocytosis depends in part on a RAB11A-RAB8A-MYO5B network, but RAB27B is also reported to be associated with DFVs, and knockout mice lacking RAB27B have fewer DFVs.

Endovascular Renal Artery Stent Retriever Embolectomy in a Young Patient With Cardiac Myxoma: Case Report and Review of the Literature.

Purpose: Cardiac myxomas are common tumors of the heart with disproportionate impact on young patients, occasionally with dramatic systemic dissemination of tumor emboli with catastrophic multiorgan system ischemia. The coincident comorbidities can increase the risk of traditional treatments for tumor embolus in each region.

Case Report: A young patient with previously unknown cardiac myxoma presented with seizure and was found to have stress cardiomyopathy, multiple cerebral large vessel occlusions with acute ischemic stroke, bilateral lower extremity tumor emboli and rhabdomyolysis, and renal tumor embolus with acute tubular necrosis.

Utilizing CT with Maximum Intensity Projection Reconstruction Bypassing CTA Improves Time to Groin Puncture in Large Vessel Occlusion Stroke Thrombectomy.

Background And Purpose: Prior to thrombectomy for proximal anterior circulation large vessel occlusion (LVO) stroke, recent trials have utilized CT angiography (CTA) for vascular imaging immediately following noncontrast CT (NCCT) for decision-making, but thin-section NCCT with automated maximum intensity projection (MIP) reconstruction also has high accuracy in demonstrating the site of an occluding thrombus. We hypothesized that performing thin-section NCCT with MIP alone prior to thrombectomy improves the time to groin puncture (GP) compared to performing CTA after NCCT.

Materials And Methods: We performed a retrospective cohort study of anterior circulation LVO thrombectomy at our tertiary care academic medical center.

Fanconi syndrome and neonatal diabetes: phenotypic heterogeneity in patients with GLUT2 defects.

Fanconi-Bickel syndrome, caused by mutations in SLC2A2 encoding the glucose transporter 2 (GLUT2), is characterized by generalized proximal renal tubular dysfunction manifesting in late infancy. We describe phenotypic heterogeneity of Fanconi-Bickel syndrome in three siblings, including early and atypical presentation with transient neonatal diabetes mellitus in one. The second-born of a non-consanguineous couple, evaluated for polyuria and growth retardation, had rickets, hepatomegaly and proximal tubular dysfunction from 4 to 6  months of age.

Increasing prevalence of vascular risk factors in patients with stroke: A call to action.

Objective: To evaluate trends in prevalence of cardiovascular risk factors (hypertension, diabetes, dyslipidemia, smoking, and drug abuse) and cardiovascular diseases (carotid stenosis, chronic renal failure [CRF], and coronary artery disease [CAD]) in acute ischemic stroke (AIS) in the United States.

Methods: We used the 2004-2014 National Inpatient Sample to compute weighted prevalence of each risk factor in hospitalized patients with AIS and used joinpoint regression to evaluate change in prevalence over time.

Results: Across the 2004-2014 period, 92.

Efficacy of Atorvastatin in Prevention of Contrast-induced Nephropathy in High-risk Patients Undergoing Angiography: A Double-blind Randomized Controlled Trial.

Objective: To evaluate the efficacy and safety of atorvastatin (ATN) 80 mg in the prevention of contrast medium- induced nephropathy (CIN) in high risk patients undergoing angiograph.

Materials And Methods: This was a prospective, double-blind, two-arm, parallel group RCT. A total of 216 patients undergoing coronary angiography were screened, and 188 eligible patients were randomized to two treatment arms.

Circulating dsDNA, endothelial injury, and complement activation in thrombotic microangiopathy and GVHD.

Transplant-associated thrombotic microangiopathy (TA-TMA) is a common and poorly recognized complication of hematopoietic stem cell transplantation (HSCT) associated with excessive complement activation, likely triggered by endothelial injury. An important missing piece is the link between endothelial injury and complement activation. We hypothesized that neutrophil extracellular traps (NETs) mechanistically link endothelial damage with complement activation and subsequent TA-TMA.

Hematopoietic Stem Cell Transplantation Activity in Pediatric Cancer between 2008 and 2014 in the United States: A Center for International Blood and Marrow Transplant Research Report.

This Center for International Blood and Marrow Transplant Research report describes the use of hematopoietic stem cell transplantation (HSCT) in pediatric patients with cancer, 4408 undergoing allogeneic (allo) and3076 undergoing autologous (auto) HSCT in the United States between 2008 and 2014. In both settings, there was a greater proportion of boys (n = 4327; 57%), children < 10 years of age (n = 4412; 59%), whites (n = 5787; 77%), and children with a performance score ≥ 90% at HSCT (n = 6187; 83%). Leukemia was the most common indication for an allo-transplant (n = 4170; 94%), and among these, acute lymphoblastic leukemia in second complete remission (n = 829; 20%) and acute myeloid leukemia in first complete remission (n = 800; 19%) werethe most common.

Conjugate Eye Deviation on CT Associated With Worse Outcomes Despite IV Thrombolysis.

Introduction: Rapid stroke management has significant implications in patient outcomes. Ipsilateral computed tomography conjugate eye deviation (CT-CED) has been associated with worse outcomes but has never been evaluated as predictive of vascular occlusion. To test the hypothesis that CT-CED is a marker for vascular occlusion, we evaluated patients treated with intravenous tissue plasminogen activator (IV tPA).

Transradial approach for mechanical thrombectomy in anterior circulation large-vessel occlusion.

OBJECTIVE The goals of this study were to describe the authors' recent institutional experience with the transradial approach to anterior circulation large-vessel occlusions (LVOs) in acute ischemic stroke patients and to report its technical feasibility. METHODS The authors reviewed their institutional database to identify patients who underwent mechanical thrombectomy via a transradial approach over the 2 previous years, encompassing their experience using modern techniques including stent retrievers. RESULTS Eleven patients were identified.

Ruxolitinib as Salvage Therapy in Steroid-Refractory Acute Graft-versus-Host Disease in Pediatric Hematopoietic Stem Cell Transplant Patients.

We describe our retrospective clinical experience with ruxolitinib for steroid-refractory acute graft-versus-host disease (GVHD) in pediatric allogeneic hematopoietic stem cell transplant (HSCT) patients. Ruxolitinib was administered orally at 5 mg twice daily for children ≥ 25 kg or 2.5 mg twice daily if <25 kg.