An update on clinical, pathological, diagnostic, and therapeutic perspectives of childhood leukodystrophies.

An update on clinical, pathological, diagnostic, and therapeutic perspectives of childhood leukodystrophies. Expert Rev Neurother. 2019 Dec 12;:1-20 Authors: Ashrafi MR, Amanat M, Garshasbi M, Kameli R, Nilipour Y, Heidari M, Rezaei Z, Tavasoli AR Abstract Introduction: Leukodystrophies constitute heterogenous group of rare heritable disorders primarily affecting the white matter of central nervous system. These conditions are often under-appreciated among physicians. The first clinical manifestations of leukodystrophies are often nonspecific and can occur in different ages from neonatal to late adulthood periods. The diagnosis is, therefore, challenging in most cases.Area covered: Herein, the authors discuss different aspects of leukodystrophies. The authors used MEDLINE, EMBASE, and GOOGLE SCHOLAR to provide an extensive update about epidemiology, classifications, pathology, clinical findings, diagnostic tools, and treatments of leukodystrophies. Comprehensive evaluation of clinical findings, brain magnetic resonance imaging, and genetic studies play the key roles in the early diagnosis of individuals with leukodystrophies. No cure is available for most heritable white matter disorders but symptomatic treatments can significantly decrease the burden of events. New genetic methods and stem cell transplantation are also under investigation to further increase the quality and duration of life in affected population.Expert opinion: The improvements in molecular diag...
Source: Expert Review of Neurotherapeutics - Category: Neurology Tags: Expert Rev Neurother Source Type: research

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This study investigated the cardioprotective effect of vitexin against MIRI and its possible mechanism. Isolated SD rat hearts were subjected to MIRI in a Langendorff perfusion system, and H9c2 cells were subjected to hypoxia/reoxygenation (H/R) in vitro. Ex vivo experiments showed improved left ventricular function and reduced infarct size in the vitexin group. Transmission electron microscopy showed that I/R caused outer mitochondrial membrane rupture, cristae disappearance and vacuolation, while vitexin reduced mitochondrial damage and ultimately reduced cardiomyocyte apoptosis. In vitro, vitexin protected H9c2 cells fr...
Source: Biomedicine and Pharmacotherapy - Category: Drugs & Pharmacology Source Type: research
Source: Core Evidence - Category: Drugs & Pharmacology Tags: Core Evidence Source Type: research
Publication date: Available online 20 January 2020Source: Clinica Chimica ActaAuthor(s): Hanna AntushevichAbstractFecal microbiota transplantation (FMT) is the introduction (transplantation) of gut microbiota obtained from the faeces of a healthy donor into the patient’s gastrointestinal tract. Most often, such therapy is used the treatment of gastrointestinal diseases caused by the activity of pathogenic or conditionally pathogenic microorganisms, however, recently an increasing number of studies have reported the use of fecal microbiota transplantation for the treatment of diseases such as metabolic syndrome, diabe...
Source: Clinica Chimica Acta - Category: Laboratory Medicine Source Type: research
Authors: Harrington P, Radia D, de Lavallade H Abstract INTRODUCTION: The outlook for patients with chronic myeloid leukaemia (CML) has changed dramatically with the development of tyrosine kinase inhibitors (TKIs) with the current treatment goal for many patients being to obtain a durable deep molecular remission, discontinue TKI therapy and remain treatment-free.Areas covered: In this article the authors review the data from the major TKI discontinuation studies, explore potential predictors of discontinuation outcome and look at possible mechanisms to explain the variable outcomes following TKI discontinuation i...
Source: Expert Review of Hematology - Category: Hematology Tags: Expert Rev Hematol Source Type: research
Authors: Tönshoff B Abstract Introduction: The goal of immunosuppressive therapy post-transplantation in pediatric renal transplant recipients is to prevent acute and chronic rejection while minimizing drug side effects. Most therapies alter immune response mechanisms but are not immunologically specific, and a careful balance is required to find the dose that prevents rejection of the graft, while minimizing the risks of over-immunosuppression leading to infection and cancer.Areas covered: This review article focuses on immunosuppressive therapy in pediatric renal transplant recipients, but many aspects can b...
Source: Expert Review of Clinical Immunology - Category: Allergy & Immunology Tags: Expert Rev Clin Immunol Source Type: research
ConclusionBM-MSCs delivered miR-222-3p via exosomes to inhibit cell proliferation and promote cell apoptosis by targeting IRF2 and negatively regulating IRF2/INPP4B signaling in THP-1 cells.
Source: Molecular and Cellular Probes - Category: Molecular Biology Source Type: research
Those who consume up to 20 drinks per week are less likely to develop chronic kidney disease than never drinkers, but authors warn"potential benefits" of alcohol must be weighed against"harms."Medscape Medical News
Source: Medscape Transplantation Headlines - Category: Transplant Surgery Tags: Nephrology News Source Type: news
A series of functionalized 1,1,1-trihalo-4-methoxy-3-alken-2-ones [CX3C(O)CR1=CROMe, where X = F or Cl; R = n-heptyl, n-octyl, n-nonyl, n-decyl, n-undecyl, n-tridecyl, (CH2)2CH=C(Me)2, (CH2)2Ph, (CH2)2-(4-HOC6H4), (CH2)2-(4-MeOC6H4), (CH2)2CO2Me, (CH2)3CO2Me, CH(SMe)CH3, CH2(2-MeOC6H4), and R1 = H, and R = H and R1 = n-decyl] were synthesized from respective alkyl methyl ketones or aldehyde via acetal acylation using trifluoroacetic anhydride and trichloroacetyl chloride. 1,1,1-Trihalo-4-methoxy-3-alken-2-ones with acid-compatible substituents were easily hydrolyzed to respective trihalomethyl-1,3-diketones. The 1,1,1-trih...
Source: Journal of the Brazilian Chemical Society - Category: Chemistry Source Type: research
Chem. Commun., 2020, Accepted Manuscript DOI: 10.1039/C9CC09362A, CommunicationYiqian Ren, Yao Qiang, Xinrui Duan, Zhengping Li In our report, we found a distinct difference in azido sugar metabolic rate between neural stem cells and fibroblasts, which can be used for selective removal of fibroblasts from neural... The content of this RSS Feed (c) The Royal Society of Chemistry
Source: RSC - Chem. Commun. latest articles - Category: Chemistry Authors: Source Type: research
RESUMO O programa de transplante de f ígado teve início em nosso centro em 1992, e pacientes em pós-operatório de transplante hepático ainda são admitidos à unidade de terapia intensiva. Uma curva de aprendizado do médico intensivista teve então início, com aquisição de habilidades e estabelecimento de uma prática específi ca. Contudo, muitos dos conceitos se modificaram com o tempo, o que conduziu a uma melhora nos cuidados proporcionados a esses pacientes. A abordagem prática varia entre diferentes centros de transpla...
Source: Revista Brasileira de Terapia Intensiva - Category: Intensive Care Source Type: research
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