PEMPHIGUS: an acronym for a disease with multiple etiologies

The acronym PEMPHIGUS is suggested to encompass the numerous factors involved in the pathogenesis and course of the disease. In the following review the authors present studies documenting these factors. The acronym can serve as a handy tool to direct the physician's investigation of a case of pemphigus, aiding in its diagnosis and in the prevention of future flare-ups.     

Timing of surgery in regurgitant lesions

For both aortic and mitral regurgitation if the patient has irreversible LV dysfunction, then they are at increased risk of postoperative LV dysfunction and death from heart failure. To justify the risk of surgery, the ideal timing for surgery is to do the same at the onset of LV dysfunction. There are several clinical and non-invasive indicators of the same.     

Inhibition of serotonin re-uptake by licorice constituents

Ovarian steroid hormones, estrogen and progestin, affect the function of the serotonin neural system by inhibiting serotonin re-uptake through allosteric interaction with the serotonin transporter (SERT) in a nongenomic mechanism. Blocking or reducing serotonin re-uptake at the synapse alleviates depression. The aim of this study was to test the effect of compounds of the isoflavan and isoflavene groups, subclasses of the flavonoids family, on serotonin re-uptake and to compare the results with the effect of other known phytoestrogens like genistein and daidzein to relate the activity of these compounds to their structure. The effect of these compounds on the re-uptake of radioactive serotonin was assayed in HEK-293 cells stably expressed the recombinant human serotonin transporter (hSERT). The results demonstrated that the isoflavans glabridin and 4'-O-methylglabridin (4'-OMeG) and the isoflavene glabrene inhibited serotonin re-uptake by 60, 53 and 47%, respectively, at 50 microM, whereas resorcinol, the isoflavan 2'-O-methylglabridin (2'-OMeG), and the isoflavones genistein and daidzein were inactive. The inhibition of serotonin re-uptake is dose dependant with glabridin and estradiol. These results emphasize the importance of the lipophilic part of the isoflavans, as well as the hydroxyl at position 2' on ring B. In conclusion, this study showed that several isoflavans are unique phytoestrogens, which like estradiol, affects the serotonergic system and inhibits serotonin re-uptake and, thus, potentially may be beneficial for mild to moderate depression in pre- and postmenopausal women.     

Validation of the Routine Assessment of Patient Progress (RAPP) in patients with psychosis in South India

The aim of the study was to validate the Routine Assessment of Patient Progress (RAPP), an instrument for assessment by nurses, among patients with psychosis in South India. One hundred consecutive patients with psychosis admitted to the Department of Psychiatry, Christian Medical College, Vellore, India, were assessed on day five and day 19 using the RAPP, the PANSS, and the CGI scales. The correlation of the RAPP with the other scales, its change over time, the inter-rater reliability, and factor structure were assessed. The subscales of RAPP correlated significantly with those of the other scales at intake and at follow-up. The RAPP scores changed significantly after treatment. Its inter-rater reliability as measured by the intra-class correlation coefficient was 0.98. The Cronbach's alpha, used to measure the internal consistency of the scale, was 0.87. The split-half reliability (Spearman-Brown) was 0.89. Principal component analysis with varimax rotation revealed six factors, which explained 67.4% of the variance. Items related to life skills explained a major proportion of the variance. The good psychometric properties, concurrent validity, high inter-rater reliability and sensitivity to change make it a useful instrument for nurses to employ to assess patients with psychoses.     

Genetic mapping and characterization of the bleeding disorder in the fawn-hooded hypertensive rat

Release of platelet dense granule contents occurs in response to vascular injury, playing an important role in platelet aggregation and primary hemostasis. Abnormalities of the platelet dense granules results in a bleeding disorder of variable severity termed "storage pool defect" (SPD). We have examined the fawn-hooded hypertensive (FHH) rat as a model of SPD in order to genetically map the locus (Bd) responsible for prolonged bleeding. Platelet function assays of the FHH rat confirmed the presence of a platelet dense granule SPD. However electron microscopy and lysosomal enzyme assays indicated differences between the FHH rat and other rodent models of SPD. Genetic mapping through the use of congenic FHH rats localized the Bd locus to an approximately 1 cM region on rat chromosome 1. Through the use of comparative mapping between species and analysis of the initial draft of the rat genome assembly, six known and thirty-four putative genes were identified in the Bd locus. None of these genes have been previously implicated in platelet function. Therefore positional cloning of the gene responsible for the bleeding disorder in the FHH rat will lead to new insights in platelet physiology, with implications for diagnosis and management of hemostatic and thrombotic disorders.     

Quality of life following surgery for anterior skull base tumors

OBJECTIVES: To evaluate patients' quality of life (QOL) after surgical extirpation of anterior skull base tumors, to elucidate different QOL domains, and to define possible predictors of functional outcome postoperatively. DESIGN: Retrospective survey. SETTING: University-affiliated medical center. PARTICIPANTS: Sixty-nine patients (76 consecutive cases) who underwent subcranial surgery between 1994 and 2002 for extirpation of anterior skull base tumors. MAIN OUTCOME MEASURE: A multidimensional, disease-specific questionnaire with 39 items was used. Six relevant domains of QOL were assessed: role of performance, physical functioning, vitality, pain, specific symptoms, and impact on emotions. RESULTS: The response rate for completing the questionnaire was 98% (40/41) after excluding patients who died (n = 13), were lost to follow-up (n = 10), and were operated on within 3 months of commencement of the study (n = 5). Thirty patients (74%) reported a significant improvement or no change in overall QOL within 6 months after surgery. The worst impact of surgery on the patients' QOL was on their financial status and emotional state. The most influential factor on QOL was malignancy leading to a significant decrease in the overall score. Radiotherapy, old age, comorbidity, and wide resection also significantly worsened QOL scores of specific domains. CONCLUSION: After subcranial extirpation of anterior skull base tumors, the overall outcome of the patients is good. Old age, malignancy, comorbidity, wide resection, and radiotherapy are negative prognostic factors for these patients' QOL.