An Update on Pediatric Cardiomyopathy

AbstractPurpose of reviewThis review summarizes the clinical characteristics and updated outcomes of primary pediatric cardiomyopathies including dilated (DCM), hypertrophic (HCM), and restrictive cardiomyopathy (RCM), and briefly discusses left ventricular non-compaction (LVNC) and arrhythmogenic cardiomyopathy (ACM), primarily arrythmogenic right ventricular cardiomyopathy (ARVC).Recent findingsPediatric cardiomyopathies are diseases of the heart muscle with an estimated annual incidence of 1.1 –1.5 cases per 100,000. They are progressive in nature and are frequently caused by a genetic mutation causing a structural abnormality in the myocyte. Dilated cardiomyopathy, characterized by left ventricular dilation and systolic dysfunction with normal left ventricular wall thickness, accounts for about 50–60% of all pediatric cardiomyopathy cases. This is followed by hypertrophic cardiomyopathy accounting for about 40%, characterized by abnormally thickened myocardium in the absence of another cause of hypertrophy with non-dilated left ventricle. Left ventricular non-compaction and res trictive cardiomyopathy each account for about 5% of the cases. Genetic mutations play a dominant role in the development of pediatric cardiomyopathies. While treatment for congestive heart failure and arrhythmias alleviates symptoms, it has not been shown to reduce the risk of sudden death. The 5-y ear transplant-free survival of DCM, HCM, RCM, and LVNC are 50%, 90%, 30%, and 60% respe...
Source: Current Treatment Options in Cardiovascular Medicine - Category: Cardiology Source Type: research

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Conclusion: The results of our clinical and simulation experiments demonstrate that localization - f left-ventricular endocardial activation by means of the Bayesian approach, based on sparse representation of sources by EDL, is feasible and accurate. Significance: The proposed approach to localizing endocardial sources may have important applications in pre-procedure assessment of arrhythmias and in guiding their ablation treatment.
Source: IEEE Transactions on Biomedical Engineering - Category: Biomedical Engineering Source Type: research
Authors: Catho G, Huttner BD Abstract Introduction: Among the multidrug resistant pathogens, extended-spectrum beta-lactamase (ESBL-E) or carbapenemase-producing Enterobacteriaceae (CPE) are currently considered the main threat due to the scarcity of therapeutic options and their rapid spread around the globe. In addition to developing new antibiotics and stopping transmission, recent research has focused on "decolonization" strategies to eradicate the carriage of ESBL-E/CPE before infection occurs. Areas covered: In this narrative review, we aim to describe the current evidence of decolonization strategi...
Source: Expert Review of Anti-Infective Therapy - Category: Infectious Diseases Tags: Expert Rev Anti Infect Ther Source Type: research
Abstract Experimental animal tumor models have been broadly used to evaluate anticancer drugs in the preclinical setting. They have also been widely applied for drug target discovery and validation, which usually follows four experimental strategies: first, assess the roles of putative drug targets using in vivo tumorigenicity and tumor growth kinetics assays of transplanted tumors, engineered through gain-of-function (GOF) by overexpressing transgene or knock-in (KI) or loss-of-function by gene silencing using knockdown (KD) or knockout (KO) or mutation via mutagenesis procedures; second, similarly genetically en...
Source: Mol Biol Cell - Category: Molecular Biology Authors: Tags: Methods Mol Biol Source Type: research
Authors: Pierri F, Dufour C Abstract Introduction: About 60% of aplastic anemia (AA) patients are in need of further treatment after front line standard immunosuppressive therapy (IST). This along with the prolonged survival of AA subjects who do not respond to or relapse after this treatment makes management of these patients a rising and very challenging issue. Areas Covered: Literature research, carried out from the most commonly used data bases, included the following key words: aplastic anemia, immunosuppressive treatment, Anti Thymocyte Globuline, Ciclosporine A, refractory aplastic anemia, relapsing aplastic...
Source: Expert Review of Hematology - Category: Hematology Tags: Expert Rev Hematol Source Type: research
Authors: Schielke A, Meurisse N, Lamproye A, Honoré P, Delwaide J, Hustinx R, Detry O Abstract Ever since the initial description of the Milan criteria, used for selecting patients with hepatocellular carcinoma (HCC) for liver transplantation (LT), there has been a clear need to go further than solely morphological criteria. Tumours exceeding the Milan criteria, but presenting favourable biological behaviour, might still allow for comparable overall- and disease-free survivals after LT. As it is well established that the presence of microvascular invasion is a major factor that influences HCC recurrence afte...
Source: Acta Gastro-Enterologica Belgica - Category: Gastroenterology Tags: Acta Gastroenterol Belg Source Type: research
Authors: Verhelst X, Geerts A, Callewaert N, Van Vlierberghe H Abstract The study of glycomics is a novel and fascinating approach for the development of biomarkers. It has become clear that in the field of liver disease specific glycomic patters are present in specific disease states, which has led to the development of diagnostic biomarkers. In this manuscript, we will describe two new applications of this technology for the development of prognostic biomarkers. The first biomarker is associated with the risk of hepatocellular carcinoma development in patients with compensated cirrhosis. The second biomarker is p...
Source: Acta Gastro-Enterologica Belgica - Category: Gastroenterology Tags: Acta Gastroenterol Belg Source Type: research
71 yo, history of single-lung transplant with T1N1 triple negative breast cancer. immediate surgery (ie no neoadjuvant therapy). s/p full ax dissection (11 nodes) - final path with single positive node and associated ECE. Would anyone treat axilla in this situation? If it matters the transplanted lung is on the right and the cancer was on the left.
Source: Student Doctor Network - Category: Universities & Medical Training Authors: Tags: Radiation Oncology Source Type: forums
Conclusions: Some CHMs seem effective and safe as alternative remedies for patients with IPF, suggesting that further study of CHMs in the treatment of IPF is warranted. Although this systematic review suggests that CHMs may have positive effect on quality of life, 6-minute walk test distance, and lung function (TLC, DLOC%) and seem to be relatively safe during the course of treatment, the results must be treated with great caution because of the methodological flaws of the included trials. Long-term and high-quality trials are needed in the future to provide clear evidence for the use of CHMs. PMID: 31312224 [PubMed]
Source: Evidence-based Complementary and Alternative Medicine - Category: Complementary Medicine Tags: Evid Based Complement Alternat Med Source Type: research
oa JM Abstract Atelectasis and inadequate oxygenation in lung donors is a common problem during the retrieval of these organs. Nevertheless, the use of high positive end-expiratory pressure (PEEP) is not habitual during procedures of lung retrieval. Twenty-one Sprague-Dawley male consanguineous rats were used in the study. The animals were divided into 3 groups according to the level of PEEP used: low (2 cmH2O), moderate (5 cmH2O), and high (10 cmH2O). Animals were ventilated with a tidal volume of 6 mL/kg. Before lung removal, the lungs were inspected for the presence of atelectasis. When atelectasis was detected...
Source: Braz J Med Biol Res - Category: Research Authors: Tags: Braz J Med Biol Res Source Type: research
Abstract Studies regarding the prognostic value of circulating adiponectin level in patients with heart failure are conflicting. The aim of this meta-analysis was to evaluate the association between elevated circulating adiponectin level and adverse outcomes in patients with heart failure. We searched PubMed and Embase databases from their inception to June 2018. Original observational studies that investigated the prognostic value of adiponectin in heart failure patients and reported all-cause mortality or combined endpoints of death/readmission as outcome measure were included. Pooled risk ratio (RR) with 95% co...
Source: Braz J Med Biol Res - Category: Research Authors: Tags: Braz J Med Biol Res Source Type: research
More News: Arrhythmia | Cardiology | Cardiomyopathy | Cardiovascular | Child Development | Children | Congestive Heart Failure | Dilated Cardiomyopathy | Genetics | Heart | Heart Failure | Heart Transplant | Hypertrophic Cardiomyopathy | Pediatrics | Restrictive Cardiomyopathy | Transplant Surgery | Transplants