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Autoantibodies against β1‐Adrenergic Receptors: Response to Cardiac Resynchronization Therapy and Renal Function 下载免费PDF全文
ANTONIO MICHELUCCI M.D. MARIO MILCO D'ELIOS M.D. ELENA STICCHI Ph.D. PAOLO PIERAGNOLI M.D. GIUSEPPE RICCIARDI M.D. CINZIA FATINI Ph.D. MARISA BENAGIANO Ph.D. ELENA NICCOLAI Ph.D. ALESSIA GRASSI M.Sc. PAOLA ATTANÀ M.D. MARTINA NESTI M.D. GINO GRIFONI M.D. LUIGI PADELETTI M.D. ROSANNA ABBATE M.D. DOMENICO PRISCO M.D. 《Pacing and clinical electrophysiology : PACE》2016,39(1):65-72
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MEGAN SPENCER-SMITH BPSYCSC PHD ; RICHARD LEVENTER MBBS BMEDSCI PHD FRACP ; RANI JACOBS BSC PHD ; CINZIA DE LUCA BA/BSC MPSYCH ; VICKI ANDERSON PHD 《Developmental medicine and child neurology》2009,51(11):909-916
Aim Subcortical band heterotopia (SBH) or 'double cortex' is a malformation of cortical development resulting from impaired neuronal migration. So far, research has focused on the neurological, neuroimaging, and genetic correlates of SBH. More recently, clinical reports and small sample studies have documented neuropsychological dysfunction in patients with this malformation. This study aimed to characterize further the phenotype of patients with SBH by describing the neuropsychological profiles of children.
Method Seven children (six females) aged 4 to 15 years were assessed for cognitive functioning (intellectual ability, processing speed, attention, working memory) and academic achievement (reading, spelling, arithmetic). Parents completed questionnaires examining their child's social skills and problem behaviours. Magnetic resonance images (MRI) conducted for routine clinical follow-up were coded by a paediatric neurologist. Genetic and seizure history were obtained from medical records.
Results There was variation in the neurological, neuroimaging, and genetic presentation of children in the sample. Impairments were observed in all areas of neuropsychological functioning examined. Intellectual ability was generally within the 'extremely low' range (full-scale IQ 44–74; performance IQ 45–72; verbal IQ 57–80). Generalized impairments in cognitive skills were typical, with severe impairments (scores greater than 2SD below the test mean) reported in processing speed, working memory, and arithmetic. Impairments in academic, social, and behavioural functioning were less generalized. No clear relationship between neuroimaging and neuropsychological impairments was found.
Interpretation Children with SBH demonstrate cognitive, academic, social, and behavioural problems, with the greatest difficulties in processing speed and complex cognitive skills. 相似文献
Method Seven children (six females) aged 4 to 15 years were assessed for cognitive functioning (intellectual ability, processing speed, attention, working memory) and academic achievement (reading, spelling, arithmetic). Parents completed questionnaires examining their child's social skills and problem behaviours. Magnetic resonance images (MRI) conducted for routine clinical follow-up were coded by a paediatric neurologist. Genetic and seizure history were obtained from medical records.
Results There was variation in the neurological, neuroimaging, and genetic presentation of children in the sample. Impairments were observed in all areas of neuropsychological functioning examined. Intellectual ability was generally within the 'extremely low' range (full-scale IQ 44–74; performance IQ 45–72; verbal IQ 57–80). Generalized impairments in cognitive skills were typical, with severe impairments (scores greater than 2SD below the test mean) reported in processing speed, working memory, and arithmetic. Impairments in academic, social, and behavioural functioning were less generalized. No clear relationship between neuroimaging and neuropsychological impairments was found.
Interpretation Children with SBH demonstrate cognitive, academic, social, and behavioural problems, with the greatest difficulties in processing speed and complex cognitive skills. 相似文献
3.
LORENZO MAGGI FRANCO SALERNO CINZIA BRAGATO SIMONA SAREDI FLAVIA BLASEVICH ELIO MACCAGNANO BARBARA PASANISI CESARE DANESINO MARINA MORA LUCIA MORANDI 《Acta myologica》2013,32(2):85-90
The adult-onset form of Pompe disease had a wide clinical spectrum, ranging from asymptomatic patients with increased CK to muscle cramps and pain syndrome or rigid-spine syndrome. In addition clinical severity and disease progression are greatly variable. We report on a family with 3 siblings characterized by an unusual adult-onset Pompe disease including dysphagia and weakness of tongue, axial and limb-girdle muscles, in association with atypical globular inclusions in muscle fibres. Our study confirms the great clinical and histological variability of adult-onset Pompe disease and further supports the need of careful evaluation of bulbar function in patients affected by this pathology.Key words: Pompe disease, globular inclusions, bulbar symptomsGlycogen storage disease type II (Pompe disease or acid maltase deficiency) is a rare autosomal recessive muscular disorder characterized by deficiency of acidalfa glucosidase (GAA), determining accumulation of glycogen in the lysosomes, mainly in cardiac and skeletal muscle cells. Typical phenotypes of glycogenosis type II include the severe classic infantile form, characterized by severe muscle weakness and hypertrophic cardiomyopathy, almost invariably fatal by 12 months, a "non-classic" form presenting between 1 and 2 years of age and the lateonset form, presenting at any time after the age of 1 year, including juvenile and adult-onset subtypes, which are considered as part of a continuous clinical spectrum (1). In particular the adult-onset form presents with slowly progressive proximal lower limb and/or paraspinal muscle weakness, often followed by restrictive respiratory failure, which could be life-threatening, as it is in infants and children (2). However the clinical spectrum of adultonset form is wide, ranging from asymptomatic patients with increased CK to muscle cramps and pain syndrome or rigid-spine syndrome (2, 3). Furthermore clinical severity and disease progression is greatly variable.We report on a family with 3 siblings with an unusual adult-onset Pompe disease clinically characterized by weakness of bulbar, axial and limb-girdle muscles in association with atypical histopathological changes. 相似文献
4.
Angiotensin-Converting Enzyme and Endothelial Nitric Oxide Synthase Polymorphisms in Patients with Atrial Fibrillation 总被引:8,自引:0,他引:8
FRANCESCA GENSINI LUIGI PADELETTI† CINZIA FATINI‡ ELENA STICCHI GIAN FRANCO GENSINI† ANTONIO MICHELUCCI† 《Pacing and clinical electrophysiology : PACE》2003,26(1P2):295-298
GENSINI, F., et al. ; Angiotensin-Converting Enzyme and Endothelial Nitric Oxide Synthase Polymorphisms in Patients with Atrial Fibrillation. Experimental studies have shown a significant increase in angiotensin-converting enzyme (ACE) expression in atrial tissue of AF patients. ACE regulates the synthesis of endothelial nitric oxide (NO), which modulates autonomic nervous activity involved in the development of AF. The aim of the study was to evaluate the prevalence of ACE insertion/deletion and endothelial NO synthase (eNOS) T-786C, G894T, and 4a/4b polymorphisms in 148 patients with persistent AF, compared with 210 control subjects. ACE insertion/deletion polymorphism genotype distribution and allele frequency were significantly different between patients and controls ( P < 0.0001 and P < 0.0001 , respectively). ACE DD genotype was significantly associated with the risk of AF (OR DD/ID + II = 3.24, P < 0.0001) . Analysis of eNOS polymorphisms showed no significant difference in genotype distribution and allele frequency between patients and controls. The results suggest a possible role of ACE DD genotype as a predisposing factor to AF and a pathophysiological mechanism of ACE inhibition in reducing the incidence of AF in patients with left ventricular dysfunction. (PACE 2003; 26[Pt. II]:295–298) 相似文献
5.
FULVIO MAGNI ISABELLA FERMO CINZIA ARCELLONI LOLITA ARNOLDI MARINA DEL PUPPO RITA PARONI 《Chemical biology & drug design》1997,49(3):191-194
In this work we report data on the hydrolysis of CsA, allowing interpretation on the formation of previously identified open chain oligopeptides from CsA. © Munksgaard 1997. 相似文献
6.
G. SCANO R. DURANTI CINZIA LO CONTE A. SPINELLI F. GIGLIOTTI LOREDANA STENDARDI L. EMMI R. SERGYSELS† P. ARCANGELI 《Clinical and experimental allergy》1987,17(3):169-180
In animals, histamine inhalation is known to increase either respiratory frequency or respiratory drive by stimulation of airway vagal sensitive endings. However, it is not well known whether these changes are concomitant in man. In order to elucidate this point, we carried out the present investigation in thirty-five asthmatic patients who underwent bronchial provocation test by progressively doubling the dose of inhaled histamine. Bronchial reactivity to histamine allowed two populations of patients to be defined: group I with moderate and group II with mild, increased reactivity. In the twenty-three group I patients, neuromuscular inspiratory drive, assessed by mouth occlusion pressure (P0.1 ), was found to be significantly increased while no significant changes in breathing pattern were noted. In the twelve group II patients histamine did not modify P0.1 or breathing pattern. However, we were able to separate in group I a sub-group of ten patients, as with atopic asthma, in which histamine-induced increase in P0.1 was paralleled by rapid and shallow breathing (RSB). Changes in P0.1 and breathing pattern did not depend on baseline airway calibre. In group I, after bronchoconstriction had been reversed by inhaling a β2 -agonist bronchodilator agent (fenoterol), P0.1 decreased significantly and RSB was found to be reversed; however, these changes were not interrelated. We concluded that: (i) in asthmatics, histamine-induced increase in P0.1 is not necessarily paralleled by, nor related with, change in breathing pattern and (ii) in atopics a 'sensitization' of vagal receptors could account for the concomitance of enhanced P0.1 with RSB. 相似文献
7.
Three Years of Cardiac Resynchronization Therapy: Could Superior Benefits be Obtained in Patients with Heart Failure and Narrow QRS? 总被引:1,自引:0,他引:1
MAURIZIO GASPARINI M.D. FRANÇOIS REGOLI M.D. PAOLA GALIMBERTI M.D. CARLO CERIOTTI M.D. MARIKA BONADIES M.D. MAURIZIO MANGIAVACCHI BRUNO ANDREUZZI M.D. RENATO BRAGATO M.D. DANIELA PINI M.D. CATHERINE KLERSY M.D. M.Sc. † EDOARDO GRONDA M.D. 《Pacing and clinical electrophysiology : PACE》2007,30(S1):S34-S39
Aim of the study: To examine the long-term effects of cardiac resynchronization therapy (CRT) in patients presenting with heart failure (HF) and QRS ≤120 ms.
Methods: This was a prospective, longitudinal study of 376 patients [mean age = 65 years, mean left ventricular (LV) ejection fraction (EF) = 29%, mean QRS duration =165 ms, mean distance covered during a 6-minute hall walk (6-MHW) = 325 m], who underwent successful implantation of CRT systems. The QRS duration at baseline was ≤120 ms in 45 patients (12%) who were not pre-selected by echocardiographic criteria of dyssynchrony, and >120 ms in the remaining 331 patients. The baseline characteristics of the 2 groups were similar. We evaluated indices of cardiac function, percentage of responders, and survival rates over a mean 28-month follow-up.
Results: Both groups experienced similar long-term increases in 6-MHW, and decreases in New York Heart Association functional class and LV end-systolic volume (all comparisons P < 0.0001 in both groups). Time interaction of changes in LVEF and percentage of responders were significantly different (P = 0.03 and P = 0.004, respectively), in favor of the narrow QRS group, where the changes were sustained and persisted at 2 and 3 years. The long-term death rate from HF was lower in the group with narrow than in the group with wide QRS complex (P = 0.04; log-rank test).
Conclusions: CRT confers considerable long-term clinical, functional, and survival benefits in patients presenting with HF and narrow QRS, not preselected by echocardiographic criteria of dyssynchrony. Caution is advised before denying CRT to these patients on the basis of QRS width only. 相似文献
Methods: This was a prospective, longitudinal study of 376 patients [mean age = 65 years, mean left ventricular (LV) ejection fraction (EF) = 29%, mean QRS duration =165 ms, mean distance covered during a 6-minute hall walk (6-MHW) = 325 m], who underwent successful implantation of CRT systems. The QRS duration at baseline was ≤120 ms in 45 patients (12%) who were not pre-selected by echocardiographic criteria of dyssynchrony, and >120 ms in the remaining 331 patients. The baseline characteristics of the 2 groups were similar. We evaluated indices of cardiac function, percentage of responders, and survival rates over a mean 28-month follow-up.
Results: Both groups experienced similar long-term increases in 6-MHW, and decreases in New York Heart Association functional class and LV end-systolic volume (all comparisons P < 0.0001 in both groups). Time interaction of changes in LVEF and percentage of responders were significantly different (P = 0.03 and P = 0.004, respectively), in favor of the narrow QRS group, where the changes were sustained and persisted at 2 and 3 years. The long-term death rate from HF was lower in the group with narrow than in the group with wide QRS complex (P = 0.04; log-rank test).
Conclusions: CRT confers considerable long-term clinical, functional, and survival benefits in patients presenting with HF and narrow QRS, not preselected by echocardiographic criteria of dyssynchrony. Caution is advised before denying CRT to these patients on the basis of QRS width only. 相似文献
8.
MARIA VITTORIA PITZALIS FILIPPO MASTROPASQUA FRANCESCO MASSARI CINZIA FORLEO REA PASSANTINO ROBERTO COLOMBO PAOLO TOTARO PAOLO RIZZON 《Pacing and clinical electrophysiology : PACE》1998,21(3):559-567
To evaluate the effect of a hydrophilic and a lipophilic β- blocker on the autonomic nervous system, 20 normal subjects were studied under baseline conditions and 7 days after being randomly assigned to metoprolol (200 mg/day), nadolol (80 mg/day), and placebo. Under each condition, the time-domain parameters were analyzed by means of 24-hour ECG monitoring and the frequency-domain parameters by means of the autoregressive method using 10-minute ECGs during rest, controlled respiration, and after a head-up tilt test. The alpha index (the gain in the relationship between the RR period and systolic arterial pressure variability) was also calculated. Both nadolol and metoprolol significantly increased all of the time-domain parameters except the standard deviation of the RR intervals; they also modified the frequency-domain parameters. Both blunted the significant reduction in the high frequency (HF) component and alpha index during tilt. In normal subjects, hydrophilic and lipophilic β-blockers similarly modify the time- and frequency-domain parameters that are particularly evident when high sympathetic tone is present (during daytime and tilt). The value of the alpha index was increased by both β-blockers in the HF, but not in the low frequency band; this difference might be due to the fact that the former is a measure of the vagal component of the baroreflex control and the latter a measure of the sympathethic component. The effects of hydrophilic and lipophilic β-blockers on the time- and frequency-domain parameters of heart rate variability are similar. 相似文献
9.
PIETRO GUIDA Ph.D. MASSIMO IACOVIELLO M.D. Ph.D. CINZIA FORLEO M.D. Ph.D. SANDRO SORRENTINO Ph.D. AGATA PUZZOVIVO M.D. MARICA RODIO M.D. FRANCESCA DE PASCALIS M.D. CATALDO BALDUCCI R.N. MARGHERITA SARLO R.N. STEFANO FAVALE M.D. 《Pacing and clinical electrophysiology : PACE》2010,33(10):1210-1216
Background: To compare head‐up tilt testing (HUT) outcomes and hemodynamic responses, and the prevalence and correlates of prodromes, in elderly and younger patients with suspected vasovagal syncope (VVS). Methods: Consecutive outpatients with a history of recurrent unexplained syncope underwent HUT by being tilted to 70°; the test was potentiated by the administration of 300 μg of nitroglycerine after 20 minutes. Occurrence of VVS and hemodynamic responses during passive and nitroglycerine phases of HUT were evaluated; symptoms preceding HUT‐induced syncope were recorded, together with heart rate and arterial blood pressure values. Results: Four hundred and sixty of the 743 patients were HUT positive: 156 fainted during the unmedicated phase and 304 after nitroglycerine administration. The patients aged ≥65 years (n = 102) experienced VVS more frequently during the pharmacological stage of HUT; the overall rate of positive results was similar to that observed in the patients aged 36–64 years (n = 329) and only slightly lower than that observed in those aged ≤35 years (n = 312). In the older patients, who experienced fewer and mainly prodrome‐free spontaneous syncopal episodes, HUT increased the number of premonitory symptoms, and there were no significant age‐related differences in symptom prevalence or timing or the patients’ hemodynamic characteristics. Conclusions: The rate of VVS induced by nitroglycerine‐potentiated HUT is similar in elderly and younger patients. In the former, nitroglycerine‐potentiated HUT significantly increases the prevalence of prodromes in comparison with spontaneous episodes, which suggests that it may be useful not only for diagnosis but also for patient counseling. (PACE 2010; 33:1210–1216) 相似文献
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