Cardiac resynchronization improves heart failure in one patient with Myotonic Dystrophy type 1. A case report

We report an improvement in symptoms of heart failure, a reduced left ventricular dysfunction and induced reverse remodelling in one patient with Myotonic Dystrophy type 1, showing an early onset ventricular dysfunction secondary to a complete left bundle branch block (LBBB) who underwent cardioverter defibrillator CRT (ICD- CRT) implantation.Key words: myotonic dystrophy, cardiac resynchronization therapy, sudden death     

Regional variations of muscle fibre characteristics in m. semitendinosus of growing cattle

The objectives of the present study were to elaborate an intra-muscular profile of metabolic enzyme equipment, contractile and morphometric features along the longitudinal axis of m. semitendinosus at various ages throughout the growth phase. Thirty-seven male Montbeliard cattle, about half of them castrated, were representatively allocated to various slaughter dates, scheduled at 4, 8, 12 and16 months of age. Samples were collected from proximal, medial and distal locations of m. semitendinosus. Isocitrate dehydrogenase (aerobic metabolism) and lactate dehydrogenase (anaerobic metabolism) were measured spectrophotometrically. Contractile muscle type was classified by quantification of myosin heavy chain I isoform proportion using the sensitive enzyme-linked immunosorbent assay. Mean muscle fibre area was obtained on histologically-stained cross-sections utilizing animage analysis system. Our results indicated the existence of a regular intra-muscular pattern of muscle fibre traits along the length of m. semitendinosus, with decreasing glycolytic activities and concomitantly an increase in oxidative capacity towards the distal extremity. The metabolic characteristics were in good agreement with decreasing cross-sectional muscle fibre areas and the slow myosin heavy chain I isoform proportion becoming gradually more abundant from proximal to distal regions of the muscle. Moreover, the observed gradient was found to be closely related to age and diminished with advanced physiological maturity. At the final slaughter age (16 months) no differences among the distinct portions were detected, m. semitendinosus was longitudinally homogeneous in all the characteristics studied This revised version was published online in July 2006 with corrections to the Cover Date.     

p53 tumour suppressor gene and RAS oncogenes: molecular analysis in the chronic and leukaemic phases of essential thrombocythaemia

A panel of 51 cases of essential thrombocythaemia (ET), in chronic or leukaemic phase, was investigated for p53 gene and RAS oncogenes mutations by PCR-SSCP-direct sequencing. No RAS oncogenes mutations were detected, but p53 mutations were identified in three cases: 1/27 cases (≈4%) in chronic phase not undergoing chemotherapy, 1/19 cases (≈5%) in chronic phase undergoing chemotherapy, and 1/5 cases (20%) which had progressed to leukaemia. Our results suggest that: (1) p53 gene mutations occur sporadically in the chronic phase of ET, independent of chemotherapy, and may contribute to the progression to the leukaemic phase in a limited number of ET patients; (2) the RAS genes family does not seem to be involved in the pathogenesis of ET, unlike other bcr/abl negative chronic myeloproliferative diseases (CMPDs).     

Pharmacists in a liberalised system — results from a profession‐wide survey in Iceland

Background — The study reported here was part of a multi‐study evaluation of new drug distribution legislation in Iceland. Objective — The objective of this sub‐study was to compare the satisfaction of community pharmacists and pharmacists in other settings with regard to their job in general, importance of the job, work hours, contact with patients, and responsibility. Method — A questionnaire survey was constructed, pilot tested, and mailed to all pharmacists belonging to professional societies in Iceland in March 1999. Key findings — The results show an inconsistency in whether general job satisfaction was correlated with respondents' perception of the job's importance and responsibility, more so for community pharmacists than others. Overall job satisfaction was quite high and community pharmacists felt that their contact with customers was satisfactory. However, they were more dissatisfied with their work hours, importance of their work, and responsibility than other pharmacists. Conclusion — Community pharmacists have been affected more adversely by the legislative change than their colleagues in other work settings.     

Treatment of Papillon-Lefevre syndrome with chemotherapy: report of cases

Two sisters affected by Papillon-Lefevre syndrome were described and treatment trials reported. Scaling, curettage, pro-phylaxis, and oral hygiene instructions were repeatedly given to the patients. Daily rinses with a solution of cysteine were instituted. When this was unsuccessful, a daily dose of 1 gm tetracycline was admininstered for 8 weeks, concomitant with a periodontal splint. All the treatment modalities failed. Poor patient compliance may have affected the results. A noticeable periodontal finding of PLS was the presence of unaffected primary dentition in the younger sibling.     

SERUM TRYPTOPHAN AND CATARACT

Total serum tryptophan concentrations were measured in 74 cataractous patients and 124 controls No significant diferences were observed in the mean values for the cataractous and normal subjects, 11.4 ± 2.6 and 11.3 ± 2.2 µg/ml respectively, or in the range of values 7.–17.1 and 7.3–17.4 µg/ml respectively These results do not support previous reports that serum tryptophan is elevated in cataractous subjects     

IMMUNOHISTOCHEMICAL STUDY ON THE PATTERN OF 50 KD CYTOKERATIN IN PSORIASIS

Three biopsies of normal skin and 15 biopsies collected from patients with psoriasis vulgaris were analyzed for the expression of the 50 kd cytokeratin using direct immunofluorescence and ABC technique before and after local treatment with anthralin 0.1% in a petrolatum base, with 0.05% betamethasone dipropionate cream, and finally, after PUVA treatment. Antiserum against the 50 kd anti-cytokeratin reacted with tissue sections of normal skin, staining cells in the basal layer, while the psoriatic skin sections before the various treatments showed a staining concerning the whole thickness of the epidermis. After the various therapies, the 50 kd cytokeratin immunoreactivity was observed only in the basal layer of those psoriatic skin sections that showed complete clinical clearing, while it was observed in the whole thickness of psoriatic patches that did not clear. These data suggest that the normalization of the 50 kd cytokeratin expression pattern can be considered as a marker of clinical remission of psoriasis.     

N-terminal sequence of some ribosome-inactivating proteins

The N-terminal portion of some type 1 ribosome-inactivating proteins (RIPs) isolated from the seeds of Gelonium multifiorum, Momordica charantia, Bryonia dioica, Saponaria oficinalis and from the leaves of Saponaria oficinalis are reported in the present paper. Their relationship with other RIPS is discussed.     

Transcutaneous Cardiac Pacing for Termination of Tachyarrhythmias

ALTAMURA, G., ET AL.: Transcutaneous Cardiac Pacing for Termination of Tachyarrhythmias. Transcutaneous cardiac pacing (TCP) was used for interruption of tachyarrhythmias in 31 patients: 20 with ventricular tachycardia (VT); eight with atrioventricular reentrant tachycardia (AVRT) and three had atrioventricular nodal tachycardia (AVNT). The stimulators used (Pace Aid 50/52) allow pacing at programmable rates (50–160 ppm) and output (10–200 mA at 20-msec pulse duration), when possible overdrive pacing was used. Short bursts of stimuli were delivered with increasing current intensity until interruption of the arrhythmia or to the maximum energy tolerated by the patient. VTs were interrupted in eight of the 20 patients: four of the six (67%) treated by overdrive pacing and four of the 14 (29%) were treated by underdrive pacing. Supraventricular tachycardias (SVT) were terminated in eight of the 11 patients: seven out of eight (88%) AVT, and one out of three AVNT (33%). We observed two cases of arrhythmia worsening: a VT acceleration and induction of ventricular fibrillation in a patient with AVNT. TCP was well tolerated by the majority of the patients. We conclude that TCP is an effective method for interruption of ventricular and supraventricular reentrant tachycardias, but the risk of arrhythmia worsening must be considered.