130,417 research outputs found
Delphi consensus on the current clinical and therapeutic knowledge on Anderson-Fabry disease
No full textManagement of Anderson-Fabry disease (AFD) is contentious, particularly regarding enzyme replacement therapy (ERT). We report results of a Delphi consensus panel on AFD management
Influence of β-carotene on lysosomal hydrolases and their natural substrates in major salivary glands of hamsters treated with 7,12-dimethylbenzanthracene (DMBA)
No full textWe evaluated the effects of P-carotene, a precursor of vitamin A, on the activity of some lysosomal hydrolases and on the levels of their natural substrates in hamster major salivary glands during experimental oral 7,12-dimethylbenzanthracene (DMBA) carcinogenesis. Sixtyfour hamsters (Cricetus auratus) were divided into four groups-group 1: untreated control; group 2: DMBA was painted three times a week in the left buccal pouch; group 3: beta-carotene was painted three times a week in the left buccal pouch; group 4: DMBA and beta-carotene were painted alternatively in the left buccal pouch. After 16 weeks, the animals were sacrificed and the activities of some lysosomal hydrolases and their natural substrates in the major salivary glands were measured. beta-Carotene when administered topically in DMBA treated animals (group 4) reduced the levels of the majority of enzymes and substrates closer to those of the untreated control group, thus outlining a mild protective effect of beta-carotene towards the DMBA carcinogenic stress. Nevertheless, the presence of some enzymes which responded negatively to the combined administration of DMBA and beta-carotene suggests the necessity.for future studies on the effect of beta-carotene at different concentrations, the systemic administration and the possibility to combine the topical beta-carotene administration with other chernopreventive drugs. (C) 2004 Elsevier Inc. All rights reserved
Early-onset basal cell carcinoma in a case of Ivemark syndrome
No full textFirst described by the Swedish paediatrician, Björn Ivemark,
(1925-2005), IS is a rare congenital heterotaxic disorder
with lateralization anomalies [1]. It accounts for 1-
3% of all congenital heart defects, with an incidence of
1 in 10-20,000 live births. To date, about 200 cases
have been reported in the literature, mostly sporadic, but
familial occurrence has also been described, suggesting
autosomal recessive inheritance, with a male preponderance
Linear atrophoderma of Moulin
No full textFirstly described by Moulin et al. in 1992 in 5 patients
[1], LAM is a distinct clinical entity characterized by
acquired atrophic bandlike skin lesions that often show
hyperpigmentation and always follow the lines of Blaschko.
No preceding infl ammation is noted, but a transient
infl ammatory stage is perhaps often unrecognized, and
there is no induration or scleroderma. Usually the condition
begins in childhood or adolescence, and there is
no evidence of any long-term progression. Histopathologically,
an irregular moderate hyperpigmentation of the
lower part of the epidermis is found, along with a few
perivascular lymphocytes in the dermis and slight thickening
of the collagen bundles, as in our case [2]. The
existence of LAM is controversial in its possible clinical
overlap with linear scleroderma or morphea. Nevertheless,
this latter is characterized by one or more linear
streaks of progressive induration that can extend through
the dermis, subcutaneous tissue, and muscle to the underlying
bone, causing signifi cant deformities [3]. The
lack of autoantibodies, such as ANA, found in 73% of
adult patients with linear scleroderma and the chronic and
unvaried course make this diagnosis unlikely in our patient,
leading to the more compatible diagnosis of LAM
[4]. The cause and pathogenesis of this disorder remain unclea
Topical preparations for the treatment of psoriasis: results of a retrospective study over 15 years.
No full textPsoriasis requires lifelong treatments that depend on the extent, clinical forms and associated conditions.To retrospectively analyze which topical treatments were used, their efficacy, and potential advantages and disadvantages.A total of 666 patients admitted for the first time over 15 years who were topically treated were retrospectively reviewed and subdivided using clinical forms and PASI into four groups and four subgroups for the applied treatments. For each treatment the mean PASI was calculated daily: on the first, third and sixth day. An X sample statistical analysis and Mann--Whitney U-test were performed. The hospitalization time and correlation with the response to treatment were analyzed.A statistically significant response was recorded for every regimen. The best combination was clobetasol propionate plus eosin on alternate days with eosin plus cade oil. The highest score was recorded for the 'en plaques' psoriasis. The average length of treatment was of 7.5 days in the best combination. No statistically significant difference among the groups was recorded with respect to the length of hospitalization and PASI.The statistically significant response for all the topical treatments analyzed and recorded in this study does not exclude a potential benefit due to hospitalization per se
MeSH term explosion and author rank improve expert recommendations
No full textInformation overload is an often-cited phenomenon that reduces the productivity, efficiency and efficacy of scientists. One challenge for scientists is to find appropriate collaborators in their research. The literature describes various solutions to the problem of expertise location, but most current approaches do not appear to be very suitable for expert recommendations in biomedical research. In this study, we present the development and initial evaluation of a vector space model-based algorithm to calculate researcher similarity using four inputs: 1) MeSH terms of publications; 2) MeSH terms and author rank; 3) exploded MeSH terms; and 4) exploded MeSH terms and author rank. We developed and evaluated the algorithm using a data set of 17,525 authors and their 22,542 papers. On average, our algorithms correctly predicted 2.5 of the top 5/10 coauthors of individual scientists. Exploded MeSH and author rank outperformed all other algorithms in accuracy, followed closely by MeSH and author rank. Our results show that the accuracy of MeSH term-based matching can be enhanced with other metadata such as author rank
Lysosomal glycosidases and their natural substrates in major salivary glands of hamsters treated with 7,12-dimethylbenzanthracene (DMBA).
No full textOro-maxillofacial diseases may influence structure and function of salivary glands. In this study, 32 hamsters were treated with topical application of 7,12-dimethylbenzanthracene (DMBA) on the buccal pouch. After 16 weeks, the animals were killed and the major salivary glands extracted. The activities of some lysosomal glycosidases and their natural substrates were measured to understand how the carcinogenetic stress and the inflammatory reaction could influence the physiology of the salivary glands. Large differences were observed in lysosomal activities among treated and untreated animals. Similarly, large differences were shown in the concentration of natural substrates, including sialic acids. These results suggest that inflammation and/or tumors induce profound changes in the biology of the salivary glands
Two novel mutations in the gene for human alpha-mannosidase that cause alpha-mannosidose
No full textGoing Beyond Counting First Authors in Author Co-citation Analysis
No full textThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
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