1,720,976 research outputs found
2021 ACR guideline for JIA reflects changes in practice
No full textThe new ACR guideline for the treatment of juvenile idiopathic arthritis provides an update on several important topics, including management of oligoarthritis, temporomandibular joint arthritis and systemic-onset arthritis. Overall, the new guideline reflects changes in practice, but also highlights a concerning lack of high-quality evidence
Gastrointestinal involvement in patients affected with 22q11.2 deletion syndrome
No full textObjective. Enteropathy is a very common feature in patients with primary immunodeficiencies. In patients with Del22 gastrointestinal (GI) alterations, including feeding disorders and congenital abnormalities have been often reported, mostly in the first year of life. Material and methods. Aim of this monocentric study is to better define the GI involvement in a cohort of 26 patients affected with Del22 syndrome. Anamnestic information was retrospectively collected for each patient. Weight and height parameters at the time of the screening were recorded. Plasma levels of hemoglobin, iron, ferritin, albumin, total protein, calcium, phosphorus, transaminase levels, antigliadin (AGA) IgA and IgG, and antitissue transglutaminase (anti-TGase) titers were measured. Results. A GI involvement was identified in the 58% of patients. The prominent problems were abdominal pain, vomiting, gastroesophageal reflux and chronic constipation. Weight deficiency, short stature and failure to thrive were reported in 54, 42, and 30% of the patients, respectively. The evidence of sideropenic anemia, in keeping with hypoproteinemia, impaired acid steatocrit or cellobiose/mannitol test suggested an abnormal intestinal permeability. In this cohort, a high prevalence of AGA IgA and IgG positivity was observed. Celiac disease (CD) was suspected in three patients, and in one of them confirmed by histology. In this patient, a long-lasting gluten-free diet failed to restore the intestinal architecture. Conclusions. In conclusion, GI involvement is a very common feature in Del22 patients. A better characterization of GI involvement would be very useful to improve the management of these patients
Shiga Toxin Producing Escherichia coli–Hemolytic Uremic Syndrome Mimicking Acute Severe Ulcerative Colitis in a Child With Bloody Diarrhea
No full textIncreased Relapse Rate During COVID‐19 Lockdown in an Italian Cohort of Children With Juvenile Idiopathic Arthritis
Get PDFObjective: Changes of routine disease management associated with COVID-19 lockdown might have potentially affected the clinical course of juvenile idiopathic arthritis (JIA). The aim of our study was to assess the rate of disease flare before and during COVID-19 lockdown to investigate its impact on disease course in children with JIA. Methods: A single-center retrospective study was conducted, including patients presenting with inactive JIA between September 1, 2018 and March 9, 2019 (group A) and between September 1, 2019 and March 9, 2020 (group B). For each patient, demographic and clinical data were collected. The rate of JIA flare from March 10, 2019 to June 30, 2019 for group A and from March 10, 2020 to June 30, 2020 for group B was compared. Results: Group A included 126 patients, and group B 124 patients. Statistical analysis did not show significant differences among the 2 cohorts with respect to age, sex, age at JIA onset, JIA subtype, co-occurrence of uveitis, antinuclear antibody positivity, and past or ongoing medications. The rate of disease flare during lockdown at the time of the first COVID-19 pandemic wave was significantly higher in comparison to the previous year (16.9% versus 6.3%; P = 0.009). Conclusion: Our study showed that COVID-19 lockdown was associated with a higher rate of joint inflammation in children with JIA. This finding has a considerable clinical implication, as restrictive measures may be necessary in order to contain pandemics. Our data highlight the need for rearrangement in the home and health care management of children with JIA during lockdowns
Clinical outcome measures in juvenile idiopathic arthritis: toward a new disease activity score based on the parent/patient’s perspective
Get PDFThe regular assessment of disease activity through validated and reliable tools is of utmost importance in the management of patients with juvenile idiopathic arthritis (JIA). The treatment target and the therapeutic plan should be based on shared decisions between the parent/patient and the physician. Patient/parent reported outcome measures (PCROs) provide a direct insight on the parent’s and child’s perceptions of disease course and effectiveness of therapeutic interventions. The incorporation of PCROs in the routine assessment of children with JIA may lead to more efficient and effective clinical care, by enforcing concordance with physician’s choices. The identification of valid and reliable PCROs could be crucial to remotely monitor disease activity when in-face evaluation is not possible, as happened during Coronavirus Disease 2019 pandemic and reported in the chapter 2 of this thesis.
Against this scenario, our research was aimed to develop a new tool, solely based on PCROs, for the assessment of disease activity by parent/patient. First, to identify the items suitable to be included into this new tool, we analyzed the measurement properties of following 4 PCROs for disease activity: 1) parent’s or child’s assessment of overall disease activity on a 21-numbered circle visual analogue scale (VAS) (0 = no disease activity; 10 = maximum disease activity; 2) parent’s or child’s assessment of pain on a 21-numbered circle VAS (0 = no pain; 10 = maximum pain); 3) parent’s or child’s assessment of the activity of joint disease; 4) morning stiffness duration, scored on a 10-point scale as follows: absent (score = 0); less than 15 minutes (score = 2); 15-30 minutes (score 4); 30 minutes-1 hour (score = 6); 1-2 hours (score = 8); > 2 hours (score =10). We provided evidence of the validity and reliability of these four PCROs, showing that they could be appropriate instruments to remotely monitor the disease activity of JIA, as reported in chapter 4.
On the other hand, we showed that the parent/patient global assessment of wellbeing might be an imperfect indicator of disease activity in patients with JIA, because it can be affected by several other factor irrespective of disease activity, such as persistent pain, functional ability, treatment burden, medications’ side effects and psychosocial aspects (chapter 3). Therefore, we decided to rule out this PCRO as a component of the new PCROs-based score for JIA activity.
Finally, in chapter 5, we proposed the parent/child Juvenile Arthritis Disease Activity Score (par/childJADAS) as a new disease activity tool for JIA, solely based on PCROs. The parJADAS and childJADAS are calculated as the simple linear sum of the scores of their 4 components, which are the 4 above-mentioned PCROs. The parJADAS and the childJADAS exhibited very good measurement properties, possessing good construct validity, discriminant and predictive ability, internal consistency, and responsiveness to change. A regular home-completion of parJADAS and the childJADAS through electronic devices could be used for the remote assessment of the disease activity and telehealth, leading to an improvement of the quality of care of patients with JIA
Going Beyond Counting First Authors in Author Co-citation Analysis
Get PDFThe 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
Get PDF“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
Get PDFWe 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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