Friedreich's ataxia (FA) is a hereditary spinocerebellar degenerativedisease characterised clinically by ataxia, dysarthria, skeletaldeformities, and progressive dystrophia of the skeletal muscles.The disease is frequently associated with concentric and, insome cases, eccentric hypertrophic cardiomyopathy. Presentationof a dilated cardiomyopathy with global dysfunction of the myocardiumis rare and commonly supposed to represent an end-stage of aprogressive transition from the hypertrophic cardiomyopathy[1].We report the echocardiographic and tissue-Doppler findingsin a case with FA who presented with dilated cardiomyopathyshortly after debut of cardiac symptoms.    相似文献   
992.
Vasoactive intestinal polypeptidergic nerves and Brunner's gland secretion in the rat   总被引:1,自引:0,他引:1  
P Kirkegaard  J M Lundberg  S S Poulsen  P S Olsen  J Fahrenkrug  T H?kfelt  J Christiansen 《Gastroenterology》1981,81(5):872-878
Vasoactive intestinal polypeptide is known to have powerful effect on the secretions from endocrine and exocrine glands. By immunohistochemical studies on the rat, both a dense network of vasoactive intestinal polypeptide-immunoreactive nerve fibers around the acini of Brunner's glands, and small ganglia with vasoactive intestinal polypeptide-immunoreactive nerve-cell bodies close to the glands were demonstrated. Intravenous infusions of vasoactive intestinal polypeptide in doses of 10, 100, and 1000 ng/kg.h significantly increased flow rate, as well as bicarbonate and protein output from Brunner's glands in the rat. After infusion of vasoactive intestinal polypeptide the secretory cells, which in the control group were rich in PAS-positive mucin, became almost completely PAS-negative. It is suggested that physiologic secretion from Brunner's glands may be stimulated by the vasoactive intestinal polypeptidergic nerves.  相似文献   
993.
Ejection fraction in left bundle branch block is disproportionately reduced in relation to amount of myocardial scar     
Jimmy Axelsson  Björn Wieslander  Robert Jablonowski  Igor Klem  Robin Nijveldt  Erik B. Schelbert  Peder Sörensson  Andreas Sigfridsson  Uzma Chaudhry  Pyotr G. Platonov  Rasmus Borgquist  Henrik Engblom  David G. Strauss  Håkan Arheden  Brett D. Atwater  Martin Ugander 《Journal of electrocardiology》2018,51(6):1071-1076

Introduction

The relationship between left ventricular (LV) ejection fraction (EF) and LV myocardial scar can identify potentially reversible causes of LV dysfunction. Left bundle branch block (LBBB) alters the electrical and mechanical activation of the LV. We hypothesized that the relationship between LVEF and scar extent is different in LBBB compared to controls.

Methods

We compared the relationship between LVEF and scar burden between patients with LBBB and scar (n?=?83), and patients with chronic ischemic heart disease and scar but no electrocardiographic conduction abnormality (controls, n?=?90), who had undergone cardiovascular magnetic resonance (CMR) imaging at one of three centers. LVEF (%) was measured in CMR cine images. Scar burden was quantified by CMR late gadolinium enhancement (LGE) and expressed as % of LV mass (%LVM). Maximum possible LVEF (LVEFmax) was defined as the function describing the hypotenuse in the LVEF versus myocardial scar extent scatter plot. Dysfunction index was defined as LVEFmax derived from the control cohort minus the measured LVEF.

Results

Compared to controls with scar, LBBB with scar had a lower LVEF (median [interquartile range] 27 [19–38] vs 36 [25–50] %, p?<?0.001), smaller scar (4 [1–9] vs 11 [6–20] %LVM, p?<?0.001), and greater dysfunction index (39 [30–52] vs 21 [12–35] % points, p?<?0.001).

Conclusions

Among LBBB patients referred for CMR, LVEF is disproportionately reduced in relation to the amount of scar. Dyssynchrony in LBBB may thus impair compensation for loss of contractile myocardium.  相似文献   
994.
Impact of early changes in left ventricular filling pattern on long-term outcome after acute myocardial infarction     
Møller JE  Poulsen SH  Søndergaard E  Seward JB  Appleton CP  Egstrup K 《International journal of cardiology》2003,89(2-3):207-215
BACKGROUND: In patients with heart failure due to chronic ischemic heart disease improvement of diastolic function indicates improved survival and a reduced morbidity, but whether this is also the case after acute myocardial infarction is not known. METHODS: To assess the prognostic importance of changes in left ventricular filling pattern, assessed with mitral deceleration time and colour M-mode flow propagation velocity, on cardiac death and readmission due to heart failure serial Doppler echocardiography was carried out in 103 patients with a first myocardial infarction. Based on echocardiography on hospital admission and after 1 month, patients were divided into three groups: group A (n=29) comprised patients with normal filling at either examination, group B (n=29) comprised patients with improvement of initially abnormal filling, and group C (n=45) patients with deterioration or no change of an abnormal filling pattern. RESULTS: One-year survival free of cardiac death or hospitalisation for heart failure was 97% in group A, 86% in group B and 64% in group C (P<0.0001). In Cox analysis persistence of abnormal filling or deterioration of left ventricular filling was still a predictor of the combined endpoint (risk ratio 4.4, 95% CI 1.8-12.0, P=0.003) after adjustment of LV filling on admission, left ventricular systolic function and clinical variables. Serial analyses of left ventricular systolic function demonstrated a significant improvement after 1 year in ejection fraction in groups A and B, whereas ejection fraction remained unchanged in group C. CONCLUSION: Patients with a persistently abnormal or a deterioration of left ventricular filling pattern as opposed to improved or normal filling are at increased risk of cardiac death and readmission due to heart failure after acute myocardial infarction.  相似文献   
995.
Usefulness of the exercise electrocardiogram in diagnosing ischemic or coronary heart disease in patients with chest pain   总被引:1,自引:0,他引:1  
Høilund-Carlsen PF  Johansen A  Christensen HW  Pedersen LT  Jøhnk IK  Vach W  Haghfelt T 《The American journal of cardiology》2005,95(1):96-99
We compared exercise electrocardiograms with myocardial perfusion images and coronary angiograms in 186 patients who had been referred to coronary angiography for stable angina pectoris. All had normal electrocardiographic findings at rest, and none had undergone coronary revascularization. Sensitivity, specificity, and positive and negative predictive values with the exercise electrocardiogram for prediction of reversible and/or irreversible hypoperfusion were 58%, 89%, 76%, and 77% and 70%, 88%, 72%, and 87%, respectively, for prediction of reversibility only. Corresponding values for comparison with coronary angiography were 65%, 89%, 74%, and 83%, respectively. Sensitivities were considerably lower in women than in men. Exercise electrocardiographic findings were normal in 50% of women and 25% of men who had reversible perfusion defects.  相似文献   
996.
Congestive heart failure with preserved left ventricular systolic function after acute myocardial infarction: clinical and prognostic implications     
Møller JE  Brendorp B  Ottesen M  Køber L  Egstrup K  Poulsen SH  Torp-Pedersen C;Bucindolol Evaluation in Acute Myocardial Infarction Trail Group 《European journal of heart failure》2003,5(6):811-819
AIMS: To characterise the prevalence, in-hospital complications, management, and long-term outcome of patients with congestive heart failure but preserved left ventricular systolic function after acute myocardial infarction. METHODS: 3166 consecutive patients screened for entry in the Bucindolol Evaluation in Acute Myocardial Infarction Trial with definite acute myocardial infarction and echocardiographic assessment of left ventricular systolic function were included between 1998 and 1999 in this prospective observational study. Main outcome measures were occurrences of in-hospital complications and all cause mortality. RESULTS: Congestive heart failure was seen during hospitalisation in 1464 patients (46%), 717 patients had preserved left ventricular systolic function (wall motion index > or =1.3 corresponding to ejection fraction > or =0.40), and 732 patients had systolic dysfunction (wall motion index <1.3). One year mortality in patients with no heart failure, heart failure with preserved systolic function, and heart failure with systolic dysfunction were 6, 22 and 35%, P<0.0001. Unadjusted risk of death from all causes associated with heart failure and preserved systolic function was 3.3 (95% CI 2.8-4.0), and after adjustment for baseline characteristics and left ventricular systolic function in multivariate Cox proportional hazards analysis the risk was 2.1 (95% CI 1.7-2.6), P<0.0001. CONCLUSIONS: Congestive heart failure is frequently present in patients with preserved left ventricular systolic function, and is associated with increased risk of in-hospital complications and death following acute myocardial infarction.  相似文献   
997.
998.
Schizophrenia and Autism as Contrasting Minds: Neural Evidence for the Hypo-Hyper-Intentionality Hypothesis     
Angela Ciaramidaro  Sven B?lte  Sabine Schlitt  Daniela Hainz  Fritz Poustka  Bernhard Weber  Bruno G Bara  Christine Freitag  Henrik Walter 《Schizophrenia bulletin》2015,41(1):171-179
Both schizophrenia (SCZ) and autism spectrum disorder (ASD) are characterized by mentalizing problems and associated neural dysfunction of the social brain. However, the deficits in mental state attribution are somehow opposed: Whereas patients with SCZ tend to over-attribute intentions to agents and physical events (“hyper-intentionality”), patients with autism treat people as devoid of intentions (“hypo-intentionality”). Here we aimed to investigate whether this hypo-hyper-intentionality hypothesis can be supported by neural evidence during a mentalizing task. Using functional magnetic resonance imaging (fMRI), we investigated the neural responses and functional connectivity during reading others intention. Scanning was performed in 23 individuals with ASD, 18 with paranoid SCZ and 23 gender and IQ matched control subjects. Both clinical groups showed reduced brain activation compared to controls for the contrast intentional vs physical information processing in left posterior superior temporal sulcus (pSTS) and ventral medial prefrontal cortex (vMPFC) for SCZ, and right pSTS in ASD. As predicted, these effects were caused in a group specific way: Relative increased activation for physical information processing in SCZ that was also correlated with positive PANNS score and relative decreased activation for intentional information processing in ASD. Additionally, we could demonstrate opposed connectivity patterns between the right pSTS and vMPFC in the clinical groups, ie, increased for SCZ, decreased for ASD. These findings represent opposed neural signatures in key regions of the social brain as predicted by the hyper-hypo-intentionality hypothesis.  相似文献   
999.
1000.
Fluorescence in situ hybridization (FISH) in the microbiological diagnostic routine laboratory: a review     
Hagen Frickmann  Annette Moter  Judith Kikhney  Ralf Matthias Hagen  Henrik Stender 《Critical reviews in microbiology》2017,43(3):263-293
Early identification of microbial pathogens is essential for rational and conservative antibiotic use especially in the case of known regional resistance patterns. Here, we describe fluorescence in situ hybridization (FISH) as one of the rapid methods for easy identification of microbial pathogens, and its advantages and disadvantages for the diagnosis of pathogens in human infections in the laboratory diagnostic routine. Binding of short fluorescence-labeled DNA or nucleic acid-mimicking PNA probes to ribosomes of infectious agents with consecutive analysis by fluorescence microscopy allows identification of bacterial and eukaryotic pathogens at genus or species level. FISH analysis leads to immediate differentiation of infectious agents without delay due to the need for microbial culture. As a microscopic technique, FISH has the unique potential to provide information about spatial resolution, morphology and identification of key pathogens in mixed species samples. On-going automation and commercialization of the FISH procedure has led to significant shortening of the time-to-result and increased test reliability. FISH is a useful tool for the rapid initial identification of microbial pathogens, even from primary materials. Among the rapidly developing alternative techniques, FISH serves as a bridging technology between microscopy, microbial culture, biochemical identification and molecular diagnostic procedures.  相似文献   
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991.
   Introduction    Case Report
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