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ANCA vasculitis should be considered in seronegative symmetrical polyarthritis by looking for ANCA in a second line biological test. Methotrexate is the first treatment option to be considered. In case of insufficient response or failure, rituximab seems an interesting option in this context.Autoimmune and autoinflammatory diseases (AIDs) are a heterogeneous group of diseases. They can occur in childhood and account for significant morbidity and mortality. Transitioning from pediatric to adult healthcare can be difficult for patients and their families. It can interfere with patient follow-up and management, and eventually lead to complications. Although recommendations exist for the successful transition of patients with chronic diseases, few are specifically adapted to children and adults with AIDs (Suris et al., 2015-Solau-Gervais, 2012). The French working group on transition of the rare autoimmune and autoinflammatory diseases presents its reflections and recommendations for a successful transition. Preparation for transition should start early. Its goals are to empower adolescents by providing them with the knowledge to manage their own care, respond appropriately to changes in their condition, and evolve within the adult healthcare system. This requires the active participation of the patient, his or her family, as well as the pediatric and adult medical teams. The transition process involves multidisciplinary care and dedicated therapeutic education programs. Finally, the identification of medical specialists by region, trained in rare AIDs and accompanied by expert patients, may improve the management of patients with rare AIDs from adolescence to adulthood.

Sarcopenia (core muscle loss) has been used as a surrogate marker of frailty. We investigated whether sarcopenia would adversely affect survival after thoracoabdominal aortic aneurysm repair.

We retrospectively reviewed prospectively collected data from patients aged 60years or older who underwent thoracoabdominal aortic aneurysm repairs from 2006 to 2016. Imaging was reviewed by 2 radiologists blinded to clinical outcomes. The total psoas index was derived from total psoas muscle cross-sectional area (cm

) at the mid-L4 level, normalized for height (m

). Patients were divided by sex-specific total psoas index values into sarcopenia (lower third) and nonsarcopenia (upper two-thirds) groups. Multivariable modeling identified operative mortality and spinal cord injury predictors. Unadjusted and adjusted survival curves were analyzed.

Of 392 patients identified, those with sarcopenia (n=131) were older than nonsarcopenic patients (n=261) (70.0years vs 68.0years; P=.02) and more frequently presented with al after thoracoabdominal aortic aneurysm repair but was associated with greater risk for delayed and persistent paraplegia.In this paper, the stability problem for a class of nonlinear systems in the form of strict-feedback with applying input quantization has been addressed. By considering a sector-bounded hysteresis quantizer, signal quantization has been achieved. The employed quantizer can reduce the potential chattering which can occur in some approaches. By using a common Lyapunov function (CLF) and the backstepping method, a control scheme has been introduced to stabilize the uncertain nonlinear system. Compared with the recent papers, in order to handle the quantization error, one of the sector-bounding features has been utilized straightly instead of decomposing the quantized input into linear and nonlinear parts, in this case, the possible disturbance-like term has been ignored. The designed control scheme does not need the global Lipschitz assumption over the system mismatched nonlinearities. Besides, the asymptotic stability of system trajectories to the origin is guaranteed and the imposed restrictions over quantization design parameters such as quantization density have been eliminated. Finally, in the simulation results, the accuracy and efficiency of the this control scheme are shown.Coronavirus 2019 (COVID-19) has had a significant impact on the world. Different countries introduced various public health and social measures (PHSMs) against the coronavirus. This paper aims to (a) examine how national policies on PHSMs were framed and which PHSMs were adopted; (b) compare the similarities and differences of PHSMs advocated by different countries; and (c) examine whether these measures have changed with time. JHU395 research buy We performed a content analysis of 160 COVID-19 policy documents on the websites of the WHO and ten countries' government websites on COVID-19 between December 1, 2019 and May 31, 2020. Results showed that although the initial responses to COVID-19 in different countries varied, there was a homogenization of PHSMs over time by May 31, 2020, almost all the countries we studied implemented the major PHSMs that the WHO recommended, except Sweden, which applied only part of the major PHSMs recommended by the WHO. The differences among countries were in the speediness, strictness and resourcefulness of the PHSMs implementation. We suggest that a timely and effective utilization of the integrated package of health measures with the support of adequate resources may help the efficient implementation of PHSMs.

This study aimed to explore the diagnostic value and the correlation of the combined detection of interleukin-1β (IL-1β), interleukin-6 (IL-6), and tumor necrosis factor-α (TNF-α) with sepsis-induced cardiomyopathy (SIC).

Admitted to our hospital from January 2017 to January 2019, 96 patients with SIC (a study group) and 90 patients with sepsis (a control group) were enrolled. The three cytokines were determined and the diagnostic value of their combined detection for SIC was analyzed.

The cytokines were remarkably higher in the study group (p<.001). The combined detection of the three had a better diagnostic value for SIC (p<.001). The three cytokines were independent risk factors for the death of patients with SIC.

IL-1β, IL-6, and TNF-α in SIC patients rise markedly. The combined detection of the three has a better predictive value for patients with SIC and is closely related to the patients' prognoses, so it may be crucial in diagnosing and treating the disease.

IL-1β, IL-6, and TNF-α in SIC patients rise markedly. The combined detection of the three has a better predictive value for patients with SIC and is closely related to the patients' prognoses, so it may be crucial in diagnosing and treating the disease.

Liddle syndrome is an autosomal dominant hereditary disease caused by a single gene mutation. link2 Typical clinical manifestations are early-onset hypertension and hypokalaemia and can be treated using ENaC blockers (amiloride and aminopterin).

This report describes a 17-year-old male with hypertension and hypokalaemia. link3 We performed a Captopril inhibition test and a postural stimulation test for the diagnosis and typing of primary aldosteronism.

The serum renin was low, and aldosterone was high, so the patient was initially misdiagnosed as primary aldosteronism. After a genetic analysis, a diagnosis of Liddle syndrome was made due to the presence of an SCNN1B p.Pro617Ser mutation. After diagnosis, the patient was administered one tablet of amiloride twice a day (each tablet contains 2.5mg of amiloride hydrochloride and 25mg of hydrochlorothiazide 25mg). The patient's blood pressure (average of 120-135/70-80mmHg) and serum potassium levels (3.6-4.0mmol/L) returned to normal and were well-controlled after treatment.

The patient is an atypical case of Liddle syndrome; genetic analysis is helpful and essential for diagnosis.

The patient is an atypical case of Liddle syndrome; genetic analysis is helpful and essential for diagnosis.

Tocilizumab is an interleukin-6 receptor-blocking agent proposed for the treatment of severe COVID-19; however, limited data are available on their efficacy. The aim of this study was to assess the effect of tocilizumab on the outcomes of patients with COVID-19 pneumonia by using propensity-score-matching (PSM) analysis.

A retrospective observational analysis of hospitalized COVID-19 adult patients admitted to the Vall d'Hebron Hospital was performed between March and April 2020. We used the logistic regression to analyze the effect of tocilizumab on mortality, as main outcome, and PSM analysis to further validate their effect. Secondary outcomes were length-of-stay (LOS) and intensive-care-unit (ICU) stay. Same outcomes were also assessed for early tocilizumab administration, within 72h after admission. Patients were selected by matching their individual propensity for receiving therapy with tocilizumab, conditional on their demographic and clinical variables.

A total of 544 COVID-19 patients were included, 197 (36.2%) were treated with tocilizumab of whom 147 were treated within the first 72h after admission; and 347 were included in the control group. After PSM analyses, the results showed no association between tocilizumab use and overall mortality (OR=1.03, 95%CI 0.63-1.68). However, shorter ICU-stay in the tocilizumab group was found compared to the control group (Coefficient -4.27 95%CI -6.63 to -1.92). Similar results were found in the early tocilizumab cohort.

The administration of tocilizumab in patients with moderate to severe COVID-19 did not reduce the risk of mortality in our cohort of patients, regardless of the time of administration.

The administration of tocilizumab in patients with moderate to severe COVID-19 did not reduce the risk of mortality in our cohort of patients, regardless of the time of administration.Wounds are conditions largely present in the clinical routine, and even though frequent, their complete resolution can be challenging. Several solutions can aid or stimulate the healing process, and for this reason, this work used a stabilized solution of 4% sodium hypochlorite for the treatment of excisional wounds in mice. This study was carried out in two distinct stages in the first stage, the optimal concentration of the chlorinated solution was determined by using the sponge implantation technique in mouse subcutaneous tissue to evaluate the dose-response curve; and in the second phase, this concentration was tested in an experimental model of excisional skin wounds in mice. Soluble collagen, hemoglobin, myeloperoxidase (MPO) and N-acetyl-β-D-glycosaminidase (NAG) activity were assessed, and total, type I and type III collagen deposition were quantified in both stages. Based on the results presented in the sponge implantation study, the chlorinated solution at 150 ppm (0.015%) was chosen for use in a preclinical trial of skin healing in mice. At 1, 3, 7 and 14 days of treatment, the % wound area repair in the group treated with 150 ppm chlorinated solution was higher when compared to the control group, with statistical differences at all time points (*p≤ 0.05 and **p≤ 0.01). 150 ppm chlorinated solution obtained from a stabilized 4% sodium hypochlorite solution was effective in accelerating cutaneous excision wound repair in mice, showing a positive influence on tissue repair.

Use of an LMA ProSeal™ laryngeal mask airway (P-LMA; Teleflex) with no neuromuscular block is considered a safe alternative to tracheal intubation in short-duration paediatric laparoscopic surgery. However, few studies have evaluated surgical conditions of short-duration paediatric laparoscopic surgery using this anaesthetic technique. We assessed surgical conditions for paediatric laparoscopic inguinal hernia repair using P-LMA with and without neuromuscular block.

Sixty-six patients undergoing laparoscopic inguinal hernia repair were randomised to receive a neuromuscular block (train-of-four 1-2 twitches) using rocuronium or no neuromuscular block with the P-LMA. All operations were performed by the same surgeon who determined the surgical conditions using the Leiden-surgical rating scale (L-SRS). Secondary outcomes included perioperative data, haemodynamics, and adverse events.

Neuromuscular block improved surgical conditions compared with no neuromuscular block mean (standard deviation) L-SRS 4.1 (0.