Peutz-Jeghers syndrome and its complications. First case report from Malaysia with review of literature.

We report for the first time from Malaysia a patient with Peutz-Jeghers syndrome complicated by successive intussusceptions requiring extensive small bowel resection. Our experience is compared with that of other authors by a review of the literature highlighting important points in the diagnosis and management of complications in Peutz-Jeghers syndrome.     

Evaluation of the Appropriateness of Pacemaker Mode Selection in Bradycardia Pacing:

Although guidelines for selection of the appropriate pacing mode have been published, little data is available on how closely these are followed in the clinical setting. All 738 patients (men 412, women 326; age 73.4 +/- 0.46 years; range 19-101 years) who underwent pacemaker implantation from 1996 to 2000 were reviewed to determine if the appropriate mode was selected based on the ACC/AHA guidelines with the data collected prospectively. Demographic, investigational, and implantation data including the presence of sinus disease and/or atrioventricular block, diagnosis, indication for pacing, ACC/AHA class indication for device therapy, recommended ACC/AHA mode, implanted mode, and reason for not using the recommended mode were entered into an SPSS data base. Of 738 patients, 708 were cross-tabulated for a match to the guidelines of which 358 (50.6%) had a mode selected that did not conform. The reasons were advanced physical disability (16%), physician choice without identifiable reason (21%), rate modulation selected without identifiable indication (16%), DDD implanted instead of VDD (25%), advanced age (9%), rare need for pacing (6%), a need for specific device features (5%), and unstable stimulation thresholds or difficult venous access (2%). In the treatment of bradyarrhythmias, deviation from the ACC/AHA indicated mode occurred in a substantial proportion of pacing system implantations. However, in many, the deviation appeared appropriate considering the patient's clinical status. Nevertheless, in a smaller proportion of patients the deviation appeared inappropriate requiring rectification. The two outstanding categories were: (1) elderly denied a dual chamber system with no clinical explanation and (2) selection of rate-modulated devices without any indication of chronotropic incompetence.     

Hepatic resection. Pillars of success built on the foundation of 15 years of experience

Two hundred eight-eight hepatic resections performed over the past 15 years are discussed. The safety and success achieved are attributed to the original work in Malaysia on the anatomy of the liver and its anomalies, the use of surgical instruments specially designed for hepatic resection, various types of resections devised and studies on aids to liver regeneration after resection. The diversity of the principles and practice of surgery in the Western countries compared with those in Malaysia is illustrated.     

Recruitment of the oncoprotein v-ErbA to aggresomes

Aggresome formation, a cellular response to misfolded protein aggregates, is linked to cancer and neurodegenerative disorders. Previously we showed that Gag-v-ErbA (v-ErbA), a retroviral variant of the thyroid hormone receptor (TRα1), accumulates in and sequesters TRα1 into cytoplasmic foci. Here, we show that foci represent v-ErbA targeting to aggresomes. v-ErbA colocalizes with aggresomal markers, proteasomes, hsp70, HDAC6, and mitochondria. Foci have hallmark characteristics of aggresomes: formation is microtubule-dependent, accelerated by proteasome inhibitors, and they disrupt intermediate filaments. Proteasome-mediated degradation is critical for clearance of v-ErbA and T(3)-dependent TRα1 clearance. Our studies highlight v-ErbA's complex mode of action: the oncoprotein is highly mobile and trafficks between the nucleus, cytoplasm, and aggresome, carrying out distinct activities within each compartment. Dynamic trafficking to aggresomes contributes to the dominant negative activity of v-ErbA and may be enhanced by the viral Gag sequence. These studies provide insight into novel modes of oncogenesis across multiple cellular compartments.     

Molecular Detection of Reticuloendotheliosis Virus (REV) Integration in Avian Poxvirus in North Eastern India

Avipoxviruses are responsible for decreased egg production, reduced growth and increased mortality in poultry. The disease has re-emerged as a new threat to poultry farmers as a number of outbreaks have been reported worldwide. Several workers have characterized the field strains of fowlpox virus and found incorporation of reticuloendotheliosis (REV) provirus in their genome. Here, the authors present the cases of poxvirus infection in three avian species viz., poultry, turkey and pigeon in north eastern India and found REV genes within the genome of poxvirus isolated from poultry and turkey. The presence of REV in the flocks of the region need to be further investigated as it reflects the emerging threats to the poultry industry at large and poultry rearing in the region in particular.     

Clinical, genetic and fertility studies of Indians with beta S-globin gene and the influence of Hb S on Plasmodium falciparum malaria infection

Clinical studies were carried out on mild Indian sickle cell anaemia in Malaysia, and genetic and fertility studies were carried out on 101 families with and without sickle-cell haemoglobin (Hb S). The Indian sickle cell anaemia patients reached adulthood, and pregnancies and deliveries were uneventful without blood transfusion. There was no foetal wastage and the number of children produced was not significantly different from that in families without Hb S. 28 Indian patients hospitalized with Plasmodium falciparum malaria infection were also examined for their beta S genotype. P. falciparum malaria infection occurred much more frequently in individuals without Hb S than in Hb S carriers.