1,721,078 research outputs found
Premature adrenarche, hirsutism and primary amenorrea: Which diagnosis?|Pubarca precoce, irsutismo, amenorrea e bassa statura: Quale diagnosi?
No full textNon-classical congenital adrenal hyperplasia (NCCAH) is an autosomal recessive disease that appears during childhood with hyperandrogenic symptoms. The article reports a case of NCCAH due to 21-hydroxylase deficiency in a 14-year-old girl with premature adrenarche, hirsutism and primary amenorrea. Premature adrenarche is a benign diagnosis, but can also result from severe pathology, which makes it necessary to find the cause of premature exposition to androgens. In conjunction with history and clinical examination, first line investigations should include determination of serum androgen concentrations, along with bone age, proceeding to synacthen stimulation test (for 17OHP levels) and adrenal ultrasound if indicated
Lead-bismuth eutectic recrystallization studies for the Megapie target
No full textThe expansion behaviour after freezing of the lead-bismuth eutectic (LBE) with 44.5% lead and 55.5% bismuth is described according to the reported theory. The issue of the vessel structural integrity after LBE recrystallization was dealt with by experimental and numerical studies performed in the frame of the Megapie (Megawatt Pilot Experiment) project. We have identified the important elements which, in the case of LBE solidification inside the Megapie target, play a role in the reduction of the possible vessel over-stressing; among them, the LBE yield strength has been tested under significant experimental conditions. The resulting suggestions can also be related to the design and to the freezing procedures for other LBE technology facilities
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
The use of everolimus in the treatment of epilepsy in a child affected by tuberous sclerosis
No full textTuberous sclerosis complex (TSC) is a multisystem genetic disorder affecting cellular differentiation and proliferation, resulting in a variety of hamartomatous lesions that may affect virtually every organ system of the body. TSC is caused by inactivating mutations in one of two genes, TSC1 and TSC2, resulting in increased activity of the mammalian Target of Rapamycin (mTOR). Epilepsy is the most common symptom and also the most common medical disorder in TSC. The paper presents a case of a 9- year-old male patient with TSC and drug resistant epilepsy. The first symptoms of epilepsy manifested when the patient was 4 years old. Epilepsy became drug resistant at the age of 9 years, despite polytherapy. The patient was treated with everolimus, a rapamycin analogous, this way a reduction of more than 90% of seizures, the disappearance of sleep-activated epileptic discharges and cognitive improvement were obtained. Treatment was safe and well tolerated. The dysregulation of mTOR pathway does not induce only the growth of TSC cortical tubers but also involves abnormal cell excitability and the use of mTOR inhibitors could stop the epileptogenic process and may help to control seizures with an overall improvement in quality of life
Trace metal distribution in spinels from ultramafic bedrocks with different degree of serpentinization: insight from the HP-LT Voltri Massif (Ligurian Alps)
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