Therapeutic and diagnostic efficacy of adenosine in childhood tachycardia

Adenosine is an endogenous nucleoside that has been used in the diagnosis and treatment of tachycardias, including those with normal QRS durations and those with wide QRS complexes in both children and adults. The electrophysiologic basis for the use of adenosine is primarily its depressant effect on conduction in the atrioventricular node and its antiadrenergic effect an the ventricular myocardium. In our study, adenosine was administered intravenously via bolus dose to 43 patients with supraventricular and ventricular tachycardias as well as ventricular extrasystoles. Adenosine successfully terminated 11 of 12 tachycardias using the atrioventricular node as reentrant circuit. It induced transient complete atrioventricular nodal block, unmasking the typical appearance of the flutter and fibrillation waves on the surface electrogram. It was also useful in terminating and therefore diagnostic in ventricular ectopic activity in two patients and ventricular tachycardias in five patients, suggesting that the mechanism is triggered activity. Adenosine's rapid metabolism ensures that a bolus injection is rapidly removed from the circulation; consequently no serious side effects were seen in our study

Initial Experience with Dual-Sensor Rate-Responsive Pacemakers in Children

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Calpain-3 deficiency causes a mild muscular dystrophy in childhood

Among our 20 families with LGMD2, 10 were documented to have muscle-specific calcium-activated neutral protease 3 (calpain-3) deficiency. Consanguinity was present in all. The current ages of the index cases were between 12 and 23 years, and there were additional nine members affected. Clinically, the patients showed mild courses; none of the cases below age 30 lost autonomy so far. The dystrophy is mainly proximal and atrophic with calf enlargement and scapular wasting in some. In three cases walking was delayed. Creatine kinase levels were at least 10 times elevated. All obligate carriers had normal creatine kinase levels. Five families shared the same 551 delA frameshift mutation. In four of these families there was the same core haplotype, whereas one was distinct suggesting an independent origin. Calpain-3 deficiency in general is a mild muscular dystrophy during childhood

A cross section of autosomal recessive limb-girdle muscular dystrophies in 38 families

Limb-girdle muscular dystrophies constitute a broad range of clinical and genetic entities. We have evaluated 38 autosomal recessive limb-girdle muscular dystrophy (LGMD2) families by linkage analysis for the known loci of LGMD2A-F and protein studies using immunofluorescence and western blotting of the sarcoglycan complex. One index case in each family was investigated thoroughly. The age of onset and the current ages were between 11/2 and 15 years and 6 and 36 years, respectively. The classification of families was as follows: calpainopathy 7, dysferlinopathy 3, α sarcoglycan deficiency 2, β sarcoglycan deficiency 7, γ sarcoglycan deficiency 5, δ sarcoglycan deficiency 1, and merosinopathy 2. There were two families showing an Emery-Dreifuss phenotype and nine showing no linkage to the LGMD2A-F loci, and they had preserved sarcoglycans.
γ sarcoglycan deficiency seems to be the most severe group as a whole, whereas dysferlinopathy is the mildest. Interfamilial variation was not uncommon. Cardiomyopathy was not present in any of the families. In sarcoglycan deficiencies, sarcoglycans other than the primary ones may also be considerably reduced; however, this may not be reflected in the phenotype. Many cases of primary γ sarcoglycan deficiency showed normal or only mildly abnormal δ sarcoglycan staining.


Keywords: limb-girdle muscular dystrophy; genetic linkage analysis; sarcolemmal complex protein

Interventional treatment in diabetics in the era of drugeluting stents and compliance to the ESC guidelines: Lessons learned from the Euro Heart Survey Programme

Aims: The objective of the study is to determine the demographics and the in-hospital outcome of diabetic and non-diabetic patients treated with percutaneous coronary interventions (PCI) in Europe, to report the type of equipment and technology used for PCI procedures in diabetics and to clarify whether the treatment of diabetic patients complies with current European Society of Cardiology (ESC) guidelines. Methods and results: A total of 14,458 patients treated with PCI were enrolled from 29 member countries of the ESC between June 2005 and January 2006. Data were collected on patient characteristics and treatment, using new Cardiology Audit and Registration Data standards. In total, 3,603 patients (24.9%) were diabetic. Diabetics were older, more often female and had a higher body mass index than non-diabetics. Diabetics had higher rates of hypercholesterolaemia and hypertension, while current smokers were more frequent in the non-diabetics. Diabetics also had significantly higher rates of previous cardiovascular events. Clopidogrel was administered only in 48.1% of diabetic patients before PCI, while IIb/IIIa inhibitors were 22.9% during PCI. At discharge, there was a major adjustment of treatment with increases in the use of Beta-blocker (80.4%), angiotensin converting enzyme inhibitor (ACEI, 71.3%) and statins (89.8%) compared with on admission (Beta-blocker 60.9%, ACEI 55.0%, statin 63.1%). Inhospital mortality was higher in diabetics (1.8% vs 1.2%) although the in-hospital MACCE rate was not significantly different (3.6% vs 3.0%, p=0.09). Conclusions: Diabetic patients treated with PCI were older with more comorbidity. According to ESC guideline, the under-usage of clopidogrel, GP IIb/IIIa inhibitors should be improved. PCI is now taken as a good opportunity to adjust the use of appropriate medication. © Europa Edition. All rights reserved