An Interesting Case of Infant Sudden Death: Severe Hypertrophic Cardiomyopathy in Pompe's Disease

Glycogen storage disease type II (Pompe's disease) is a rare inherited metabolic disorder, which often leads to infantile death from severe cardiomyopathy. This case of sudden death illustrates the features of the cardiac findings in the disorder, resulting from massive lysosomal accumulation of glycogen in the heart and other tissues. Pompe's disease should be considered in cases of unexplained infantile cardiomyopathy.     

A randomized study of sodium hypochlorite versus formocresol pulpotomy in primary molar teeth

International Journal of Paediatric Dentistry 2013; 23: 145–152 Background. Alternatives to vital pulpotomy treatment in primary teeth are being sought because of the high formaldehyde content of traditional formocresol (FC) pulpotomy medicaments. Aim. The aim was to compare the clinical and radiographic success of vital pulpotomy treatment in primary molars using 3% sodium hypochlorite (NaOCl) versus a 1 : 5 dilution of Buckley’s FC. Design. Pulpotomies were performed in primary molars of healthy children between 3 and 10 years old. Sixty‐five primary teeth were randomized into two groups that were evaluated for treatment outcomes. Following treatment, the pulp chamber was filled with zinc oxide eugenol (ZnOE) and restored with a stainless steel crown cemented with glass ionomer cement. Clinical and radiographic outcomes were recorded at 6 and 12 months. Results. The control (FC) and experimental (NaOCl) groups demonstrated 100% clinical success at 6 and 12 months. The NaOCl group had 86% (19/22) radiographic success at 6 months and 80% (12/15) at 12 months. The FC group had 84% (21/25) radiographic success at 6 months and 90% (9/10) at 12 months. No significant differences were found in the radiographic outcomes between the two groups at 6 and 12 months (Fisher’s exact test; P = 0.574 and P = 0.468, respectively). Conclusion. NaOCl demonstrated clinical and radiographic success comparable to FC.     

A Survey of the Practice of Lead Extraction in the United States

Background: Endovascular lead extraction is an important component of the management of patients with chronically implanted arrhythmia control devices. Although it is associated with the potential for significant morbidity and mortality, there is little information about its scope and practice. Methods: We surveyed 1,000 physician members of the Heart Rhythm Society via e‐mail solicitation. Results: Of the 252 respondents (25%), 221 (88%) reported either performing extractions themselves (63%), or having privileges at a hospital where extractions are performed (25%). Electrophysiologists perform extractions at most sites (83%) but cardiac surgeons perform endovascular lead extraction at a significant minority of sites (20%). Most respondents report low annual volumes of extractions at their site: 15% reported <10 procedures/year, 42% 10–25 procedures/year, 23% 26–50 procedure/year, and only 19% reported >50 procedures/year. Thirty‐six percent of respondents reported that extractions were done in the operating room (OR) with surgeon present or immediately available, 39% in the electrophysiology (EP) lab with surgeon and OR identified and available, and 25% in EP lab without a surgeon or OR identified. The overall risks of lead extraction were felt to be 1–5% of major complication and 0.5–1% of mortality, roughly in line with published data. Conclusions: While there is agreement as to the risk of major complication and death from lead extraction, the degree of surgical availability varies considerably. The new guidelines document recommends the ability to promptly initiate an emergent surgical procedure, and this should be an important goal for all extractionists. (PACE 2010; 33:721–726)     

Job stress, absenteeism and coronary heart disease European cooperative study (the JACE study): Design of a multicentre prospective study

Background: The motives, objectives and design of a multicentreprospective study on job stress, absenteeism and coronary heartdisease in Europe (the JACE study) is presented in this paper.Some specific gaps in the reviewed literature are explicitlytapped into by the JACE study. Its objectives are i) to comparethe distributions of the Karasek job stress scales for the samebroad categories of occupations in different European countries(in males and females), ii) to study the predictive power ofthe job stress scales and the job strain model for one yearof sickness absence (in males and females) and iii) to studythe predictive power of the job stress scales and the job strainmodel for a three year incidence of coronary heart disease (Inmales only). Methods: In answering these questions, relationsare studied controlling for gender, age, level of education,company size, physical work risks and shift work, as well astraditional risk factors for CHD (i.e serum cholesterol, serumHDL cholesterol, smoking habits and blood pressure). The JACEstudy is a Biomed 1 concerted action. The JACE group consistsof eight participating centres from six countries, i.e. fromBelgium and Sweden (two centres), France, Italy, Spain, Swedenand The Netherlands (each one centre). The coordination of thegroup is in Brussels. The participating centres brought in over15, 000 European workers to test the hypotheses.     

The impact of 1992 on food law

The essential purpose of food law is consumer protection while quality judgement is a matter for the individual, based on informative labelling. Although the Council has agreed major frameworks directives, much detailed work is still required including; an agreed common list of food additives; the problems of the constituents of packaging materials and their possible migration into food; the assessment of quality of dietary products for their declared aims; limits for environmental contaminants; the approach to be adopted for biotechnology products and the labelling of foods containing ingredients which have been irradiated.     

Computerized Pacemaker Patient Analysis

BYRD, C.B., ET AL.: Computerized Pacemaker Patient Analysis. We have developed computer hardware and software that imports analog waveforms and other measured data from a patient into the PaceBase database system supported by any IBM PC/AT compatible. The programmable AID converter has the capacity to acquire the pacemaker artifact from the surface ECG leads. Analysis of the pacemaker artifact permits confirmation of pulse width as well as programmability and facilitates discovery of pacemaker hardware failures otherwise undetectable. Continuous recording of real-time surface ECG can be made as other measurements or storing functions are being performed. In this way, sporadic or infrequent intrinsic events are automatically recorded and can be selected to be played back and reviewed or stored on the permanent record. Pacemaker spike detection enhances identification of paced and intrinsic complexes by emphasizing the paced artifact. Even bipolar atrial spikes with pulse widths as short as 0.05 ms are easily identified; myopotential muscle noise is rejected. Enhancement of pacemaker spikes takes the guesswork out of interpretation of ECGs, especially for bipolar systems and when testing or troubleshooting for myopotential tracking or inhibition.     

Overview: The treatment of drug dependence

Addictive disorders resemble other chronic medical disorders in that they are characterized by relapses and remissions. This central feature of the addictions is addressed by many of the contributors in their individual chapters. There are also several specific points raised in the chapters which merit further discussion. For instance, any assumption that long–term anti–anxiety treatment with benzodiazepines implies a form of dependence that requires treatment could be set against an alternative view that there may be appropriate medical/psychiatric indications for such prolonged treatment. There are also interesting questions which deserve discussion surrounding the debate over treatment setting for opiate dependence treatments. The paper by Johns presents a case for inpatient treatment whereas in the United States long–term methadone maintenance is seen as one of the most effective treatments. Given the complexity of the bio–psycho–social mechanisms involved in Addiction a combination of therapeutic approaches tailored to individual needs should probably continue to be the guiding principle.