Search results for "MYOPATHY"
showing 10 items of 352 documents
Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: a severe fatty acid oxidation disorder
1994
3-Hydroxyacyl-CoA dehydrogenase deficiency is a newly recognised fatty acid oxidation disorder with a usually fatal outcome. We present a further patient who presented with hypoketotic hypoglycaemia, hepatopathy, secondary carnitine deficiency and increased plasma long-chain acylcarnitines. 3-Hydroxydicarboxylic aciduria was present and the diagnosis confirmed in cultured skin fibroblasts. Our patient is compared with those reported in the literature with respect to clinical symptoms, differential diagnosis and possible therapeutic regimens.
Climatic variables in Takotsubo cardiomyopathy: role of temperature
2017
Background Recent studies documented a seasonal (summer) and circadian (morning) temporal distribution of takotsubo cardiomyopathy (TTC). Aim The aim of our study was to investigate whether there is a relationship among season, temperature and the occurrence of TTC. A second aim of our study was the comparison of climatic variables in Takotsubo cardiomyopathy versus acute myocardial infarction (AMI). Methods We enrolled consecutive patients with TTC in three Italian centres and, for comparison consecutive patients with AMI. The frequency of TTC and AMI patients according to month, season and quartiles of temperature (I quartile: 9.8–15-, II quartile: 15–19-, III quartile: 19–25- and IV quar…
Independent Impact of RV Involvement on In-Hospital Outcome of Patients With Takotsubo Syndrome
2016
Takotsubo syndrome (TTS) is an acute clinical condition characterized by transient left ventricular dysfunction and reversible heart failure, the pathogenetic mechanism of which remains unclear. Although left ventricular apical ballooning is the most frequent morphological pattern, other variant forms have been described (1). In addition, right ventricular involvement (RVi), characterized by the presence of right ventricular (RV) apical dysfunction (biventricular ballooning), has been documented using echocardiography or cardiac magnetic resonance imaging. However, the prevalence, clinical profile, and in-hospital course of TTS patients with RVi are still not well defined. To date, although…
Right ventricular strain assessment by cardiovascular magnetic resonance myocardial feature tracking allows optimized risk stratification in Takotsub…
2018
Background A substantial number of patients with Takotsubo syndrome (TTS) exhibit right ventricular (RV) dysfunction which has been associated with adverse outcome. The aim of this study was to assess the clinical and prognostic value of RV myocardial strain in TTS using cardiovascular magnetic resonance myocardial feature tracking (CMR-FT). Methods CMR-FT was performed in a core laboratory to determine RV longitudinal strain in 134 TTS patients undergoing CMR in median 2 days after admission to 2 experienced centers. For comparison, RV involvement was evaluated by sole visual assessment concerning RV contraction abnormalities. Both approaches were analyzed regarding their long-term prognos…
Chronic pharmacological treatment in takotsubo cardiomyopathy.
2008
Abstract Background Takotsubo cardiomyopathy is a disorder that has been appreciated only recently. In most of reported cases, this syndrome mimes an acute myocardial infarction. Till this moment no data are available from literature about the treatment in the acute phase of this disease. Aim of the study In our multicentric experience we have retrospectively looked at the benefits of a treatment with ACE-inhibitors, beta-blockers, Aspirin and calcium channels blockers, started until the early phases of the disease and continued for 30 days, in 36 patients affected by Takotsubo cardiomyopathy. We chose as endpoint of the study the efficacy of the used drug in improving left ventricular myoc…
Troponin I/ejection fraction ratio: A new index to differentiate Takotsubo cardiomyopathy from myocardial infarction
2015
– Transient hypokinesis, akinesis, or dyskinesis of the left ventricular mid-segments with or without apical involvement; the regional wall motion abnormalities extend beyond a single epicardial vascular distribution; a stressful trigger is often, but not always, present; – Absence of obstructive coronary disease or angiographic evidence of acute plaque rupture; – New ECG abnormalities (either ST-segment elevation and/or T wave inversion) or modest elevation in cardiac troponin; and – Absence of pheochromocytoma or myocarditis.
Does increased serum creatine kinase activity reflect exercise-induced muscle damage in rats?
1995
To test a hypothesis that exercise-induced increase in serum creatine kinase activity and the concomitant necrotic muscle damage in unaccustomed rats may be interrelated phenomena, and that the first might largely be caused by changes in lymph flow, groups of rats were separately exposed to a swimming, combination of swimming and running, and running protocol. Their serum was then repeatedly analysed over a period of 72 h for creatine kinase activity, and their soleus and the red parts of quadriceps femoris muscles for beta-glucuronidase activity (damage marker) 72 h after the commencement of the experiment, i.e. at a moment when muscle damage is in the necrotic phase. The results clearly s…
Food deprivation decreases the exertion-induced acid hydrolase response in mouse skeletal muscle
1988
Strenuous prolonged running causes muscle fibre necrosis in skeletal muscles. The muscle injury is associated with inflammation and a strong increase in the total activities of certain acid hydrolases a few days after exertion. The activity changes of acid hydrolases quantitatively well reflect the severity of histopathological changes during the myopathy (for review see Salminen, Acta Physiol Scand [Suppl 539] 1985). In this study male NMRI-mice were exposed to a protocol of fasting and refeeding together with or without a 6 h run on a treadmill at 13.5 m.min-1. The animals were killed 4 days after the exercise and samples from the red part of quadriceps femoris were analyzed for arylsulfa…
Assessment of the German and Italian Stress Cardiomyopathy Score for Risk Stratification for In-hospital Complications in Patients With Takotsubo Syn…
2019
IMPORTANCE Takotsubo syndrome (TTS) is an acute, reversible heart failure syndrome featured by significant rates of in-hospital complications. There is a lack of data for risk stratification during hospitalization. OBJECTIVE To derive a simple clinical score for risk prediction of in-hospital complications among patients with TTS. DESIGN, SETTING, AND PARTICIPANTS In this prognostic study, 1007 consecutive patients were enrolled in the German and Italian Stress Cardiomyopathy (GEIST) registry from July 1, 2007, through December 31, 2017, and identified as the derivation cohort; 946 patients were enrolled in the Spanish Registry for Takotsubo Cardiomyopathy (RETAKO) as the external score val…
Anomalous origin of all three coronary arteries from right sinus of Valsalva
2016
AbstractCongenital anomalies of the coronary arteries, although uncommon, have the potential to cause serious myocardial damage, ischemic cardiomyopathy, and sudden cardiac death. We report a rare case of origin of all three coronaries from the right sinus of Valsalva.