1,721,032 research outputs found
Mechanisms of bone impairment in sickle bone disease
Full text linkSickle bone disease (SBD) is a chronic and invalidating complication of Sickle cell disease (SCD), a multisystem autosomal recessive genetic disorder affecting millions of people worldwide. Mechanisms involved in SBD are not completely known, especially in pediatric age. Among the hypothesized pathogenetic mechanisms underlying SBD are bone marrow compensatory hyperplasia and bone ischemic damage, both secondary to vaso-occlusive crisis (VOC), which leads to cell sickling, thus worsening local hypoxia with a negative impact on osteoblast recruitment. Furthermore, the hypoxia is a strong activator of erythropoietin, which in turn stimulates osteoclast precursors and induces bone loss. Hemolysis and iron overload due to a chronic transfusion regimen could also contribute to the onset of bone complications. Vitamin D deficiency, which is frequently seen in SCD subjects, may worsen SBD by increasing the resorptive state that is responsible for low bone mineral density, acute/chronic bone pain, and high fracture risk. An imbalance between osteoblasts and osteoclasts, with a relative decrease of osteoblast recruitment and activity, is a further possible mechanism responsible for the impairment of bone health in SCD. Moreover, delayed pubertal growth spurt and low peak bone mass may explain the high incidence of fracture in SCD adolescents. The aim of this review was to focus on the pathogenesis of SBD, updating the studies on biochemical, instrumental, and biological markers of bone metabolism. We also evaluated the growth development and endocrine complications in subjects affected with SCD
EFFECTS OF PHYSICAL ACTIVITY IN CHILDREN WITH SEVERE HAEMOPHILIA
No full textRecurring bleeding lead to artropathy with disability. In the past sports and physical activity was denied to children with severe haemophilia for the risk of trauma. Nowadays the preventive administration of lack factor (prophylaxis regimen) guarantees a good hemostatic coverage. Children in prophylaxis will be encouraged to practice sports. The movement strengthens bones and muscles preventing displaced articular motions. Moreover children during physical activity play with peer favoring social integration. Unfortunately Italian law for sports medicine certification is not clear if children with haemophilia (in prophylaxis) can play sports in organized trainings and competition
Antithyroid treatment improves thrombocytopenia in a young patient with graves' disease
No full textAutoimmune thyroid disorders, including Graves' disease and Hashimoto's thyroiditis, have been reported in patients with primary immune thrombocytopenia (ITP). Several etiopathogenetic mechanisms connecting thyroid diseases and thrombocytopenia have been described. Thrombocytopenia is often documented in patients with Graves' disease, due to reduced platelet life span in hyperthyroidism, immune dysregulation and genetic predisposition (HLA B8 presence). We present the case of a 14-years old girl, who was referred to our Pediatrics Unit, because of contemporary appearance of weight loss, profuse sweating and episodes of recurrent epistaxis. A complete health team, made up of hematologists and endocrinologists, met in consultation in order to reach a diagnosis. A suppression of serum TSH concentrations, the presence of anti-TSH receptor antibodies, and at the same time an immune thrombocytopenia with positive anti-platelet antibodies, have been detected. Furthermore, a positive direct and indirect Coombs test without hemolytic anemia, ANA positivity, and a C4 consumption have been documented. The patient started treatment with thiamazole with progressive improvement of thyroid function and thrombocytopenia, requiring only an intravenous immunoglobulin infusion on one time. A multidisciplinary follow-up has been scheduled, in order to monitor the multi-organ immune dysregulation. This report documents a significant improvement of thrombocytopenia after antithyroid treatment in a young subject affected with Graves' disease
Il badminton (gioco del volano) ed il bambino emofilico:una nuova "opportunità" per educare alla corretta motricità
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Going Beyond Counting First Authors in Author Co-citation Analysis
Full text linkThe 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
Variations on the Author
Full text link“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
Appropriate Similarity Measures for Author Cocitation Analysis
Full text linkWe provide a number of new insights into the methodological discussion about author cocitation analysis. We first argue that the use of the Pearson correlation for measuring the similarity between authors’ cocitation profiles is not very satisfactory. We then discuss what kind of similarity measures may be used as an alternative to the Pearson correlation. We consider three similarity measures in particular. One is the well-known cosine. The other two similarity measures have not been used before in the bibliometric literature. Finally, we show by means of an example that our findings have a high practical relevance.information science;Pearson correlation;cosine;similarity measure;author cocitation analysis
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