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Quantitative assessment of left ventricular myocardial involvement in patients with connective tissue disease: a 3.0T contrast-enhanced cardiovascular magnetic resonance study
This study indicated that CTD patients showed impaired LV global myocardial deformation and m icrovascular perfusion, and presence of LGE. Cardiac involvement might be more severe in non-IIM patients than in IIM patients. Impaired microvascular perfusion and the presence of LGE were independently associated with LV global deformation.
Source: The International Journal of Cardiovascular Imaging - March 13, 2022 Category: Radiology Source Type: research

Muscle-Skeletal Abnormalities and Muscle Oxygenation during Isokinetic Strength Exercise in Heart Failure with Preserved Ejection Fraction Phenotype: A Cross-Sectional Study
Int J Environ Res Public Health. 2022 Jan 9;19(2):709. doi: 10.3390/ijerph19020709.ABSTRACTExercise intolerance, a hallmark of patients with heart failure (HF), is associated with muscle weakness. However, its causative microcirculatory and muscle characteristics among those with preserved or reduced ejection fraction (HFpEF or HFrEF) phenotype is unclear. The musculoskeletal abnormalities that could result in impaired peripheral microcirculation are sarcopenia and muscle strength reduction in HF, implying lowered oxidative capacity and perfusion affect transport and oxygen utilization during exercise, an essential task fr...
Source: Biomed Res - January 21, 2022 Category: Research Authors: Amanda Vale-Lira Nat ália Turri-Silva Kenneth Verboven Jo ão Luiz Quagliotti Durigan Alexandra Corr êa G B de Lima Martim Bottaro Gaspar R Chiappa Dominique Hansen Gerson Cipriano Source Type: research

A Swine Hind Limb Ischemia Model Useful for Testing Peripheral Artery Disease Therapeutics
AbstractCurrently, there is no large animal model of sustained limb ischemia suitable for testing novel angiogenic therapeutics for peripheral artery disease (PAD) such as drugs, genes, materials, or cells. We created a large animal model suitable for efficacy assessment of these therapies by testing 3 swine hind limb ischemia (HLI) variations and quantifying vascular perfusion, muscle histology, and limb function. Ligation of the ipsilateral external and bilateral internal iliac arteries produced sustained gait dysfunction compared to isolated external iliac or unilateral external and internal iliac artery ligations. Hype...
Source: Journal of Cardiovascular Translational Research - December 1, 2021 Category: Cardiology Source Type: research

Mechanism of Hydrogen Sulfide Preconditioning-Associated Protection Against Ischemia-Reperfusion Injury Differs in Diabetic Heart That Develops Myopathy.
Abstract Hydrogen sulfide (H2S) is reported to be effective in the management of the myocardial ischemia-reperfusion (I/R) injury via PI3K/GSK3β pathway in normal rats. However, its efficacy against I/R in the presence of diabetic cardiomyopathy is relatively obscure. Thus, the present work aimed to find out H2S-mediated cardioprotection against I/R in diabetic cardiomyopathy and to evaluate its mode of action using Langendorff isolated heart perfusion system. The present work includes three groups of rat, viz. (i) normal, (ii) diabetes mellitus (DM: streptozotocin: 35 mg/kg; normal diet), and (iii) diabetes +...
Source: Cardiovascular Toxicology - July 16, 2019 Category: Cardiology Authors: Ansari M, Kurian GA Tags: Cardiovasc Toxicol Source Type: research

Acute heart failure and rhabdomyolysis: a clue for the diagnosis of polymyositis with cardiac involvement.
We report the case of a 51-year-old male, with history of complete heart block, which required pacemaker implantation, and subsequently heart failure, presenting to the emergency department with worsening of dyspnea and peripheral edema. He was admitted to the Internal Medicine ward with acute heart failure and started on diuretic therapy. During hospitalization, he was discovered to have marked rhabdomyolysis. Examination revealed proximal symmetrical muscle weakness and arthralgia. The immunological study, electromyography and muscle biopsy confirmed polymyositis. The patient was started on prednisolone with clinical imp...
Source: Reumatismo - August 3, 2017 Category: Rheumatology Authors: Pinto-Lopes P, Carneiro-Leão L, Morais R, Pinheiro J, Vieira Lopes A, Bettencourt P Tags: Reumatismo Source Type: research

Peripheral vein injection of AAV8-MTM1 leads to long-term survival and correction of severe muscle pathology in a canine model of X-linked myotubular myopathy: Results from a dose escalation study
Mutations in the myotubularin gene (MTM1) result in X-linked myotubular myopathy (XLMTM), a fatal pediatric disease of the skeletal muscle characterized by small centrally nucleated myofibers containing abnormal mitochondrial accumulations. Patients typically present with severe hypotonia and respiratory failure. Previous studies in Mtm1 mutant mice demonstrated efficacy of gene therapy to treat the disease. We also previously reported that administration of an adeno-associated virus serotype 8 (AAV8) vector expressing myotubularin under the muscle-specific desmin promoter via high pressure regional hind limb perfusion pro...
Source: Neuromuscular Disorders - September 11, 2015 Category: Neurology Authors: D. Mack, K. Poulard, M. Goddard, J. Snyder, R. Grange, J. Doering, J. Strande, V. Latournerie, P. Veron, L. Yang, L. Buscara, C. Le Bec, S. Martin, M. O'Callaghan, F. Mingozzi, A. Beggs, M. Lawlor, F. Mavilio, M. Childers, A. Buj-Bello Source Type: research

MELAS syndrome: Clinical manifestations, pathogenesis, and treatment options
Publication date: Available online 15 June 2015 Source:Molecular Genetics and Metabolism Author(s): Ayman W. El-Hattab , Adekunle M. Adesina , Jeremy Jones , Fernando Scaglia Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is one of the most frequent maternally inherited mitochondrial disorders. MELAS syndrome is a multi-organ disease with broad manifestations including stroke-like episodes, dementia, epilepsy, lactic acidemia, myopathy, recurrent headaches, hearing impairment, diabetes, and short stature. The most common mutation associated with MELAS syndrome is the m.3243A&a...
Source: Molecular Genetics and Metabolism - June 27, 2015 Category: Genetics & Stem Cells Source Type: research

Pulmonary arterial hypertension-related myopathy: An overview of current data and future perspectives
Conclusions Skeletal muscles of PAH patients show a wide spectrum of cellular abnormalities that finally culminate in muscle atrophy and reduced contractility. Exercise training improves muscle function and bears a positive impact on clinical outcomes of PAH patients.
Source: Nutrition, Metabolism and Cardiovascular Diseases - November 6, 2014 Category: Nutrition Source Type: research

G.p.41
Mutations in the myotubularin gene (MTM1) result in X-linked myotubular myopathy (XLMTM), a fatal pediatric disease of skeletal muscle characterized by small centrally nucleated myofibers containing abnormal mitochondrial accumulations. Patients typically present with severe hypotonia and respiratory failure. Previous local studies in Mtm1-mutant mice demonstrated potential efficacy of gene therapy to treat the disease. We recently reported that administration of adeno-associated virus serotype 8 (AAV8) vector expressing myotubularin under the muscle-specific desmin promoter, delivered systemically in myotubularin deficien...
Source: Neuromuscular Disorders - October 1, 2014 Category: Neurology Authors: D.L. Mack, M. Goddard, J.M. Snyder, J. Doering, M.W. Lawlor, P. Moullier, M. O’Callaghan, A.H. Beggs, F. Mavilio, K. Poulard, V. Latournerie, A. Buj-Bello, Source Type: research

G.P.41: Canine X-linked myotubular myopathy: A dose-finding study of systemic AAV8-MTM1 effects on muscle strength, gait, respiration, neuromuscular function and survival
Mutations in the myotubularin gene (MTM1) result in X-linked myotubular myopathy (XLMTM), a fatal pediatric disease of skeletal muscle characterized by small centrally nucleated myofibers containing abnormal mitochondrial accumulations. Patients typically present with severe hypotonia and respiratory failure. Previous local studies in Mtm1-mutant mice demonstrated potential efficacy of gene therapy to treat the disease. We recently reported that administration of adeno-associated virus serotype 8 (AAV8) vector expressing myotubularin under the muscle-specific desmin promoter, delivered systemically in myotubularin deficien...
Source: Neuromuscular Disorders - September 4, 2014 Category: Neurology Authors: D.L. Mack, M. Goddard, J.M. Snyder, J. Doering, M.W. Lawlor, P. Moullier, M. O’Callaghan, A.H. Beggs, F. Mavilio, K. Poulard, V. Latournerie, A. Buj-Bello, M. Childers Source Type: research

Mitochondria: Role of citrulline and arginine supplementation in MELAS syndrome.
Abstract Mitochondria are found in all nucleated human cells and generate most of the cellular energy. Mitochondrial disorders result from dysfunctional mitochondria that are unable to generate sufficient ATP to meet the energy needs of various organs. Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a frequent maternally inherited mitochondrial disorder. There is growing evidence that nitric oxide (NO) deficiency occurs in MELAS syndrome and results in impaired blood perfusion that contributes significantly to several complications including stroke-like episodes, myop...
Source: The International Journal of Biochemistry and Cell Biology - January 8, 2014 Category: Biochemistry Authors: El-Hattab AW, Emrick LT, Chanprasert S, Craigen WJ, Scaglia F Tags: Int J Biochem Cell Biol Source Type: research