Disease evolution in systemic juvenile idiopathic arthritis: an international, observational cohort study through JIRcohort.

BACKGROUND: Systemic juvenile idiopathic arthritis (systemic JIA) is a severe disease with both systemic and joint inflammation. This study aims to identify predictors of disease evolution within the systemic JIA population enrolled in the Juvenile Inflammatory Rheumatism cohort (JIRcohort). METHODS: Observational patient cohort study with 201 recruited children from 4 countries (3 European, 1 North Africa) from 2005 until 2019, using retrospectively (2005-2015) and prospectively (2015-2019) routine care collected data. RESULTS: Sixty-five patients with complete follow-up data for 24 months after first diagnosis were classified as monophasic (n = 23), polyphasic (n = 6) or persistent group (n = 36) corresponding to their evolution (unique flare, recurrent flares, or persistent disease activity respectively). The patients of the persistent group were more likely to have an earlier disease onset, before the age of 6 (OR 2.57, 95%-CI 0.70-9.46), persistence of arthritis at 12-months post-diagnosis (OR 4.45, 95%-CI 0.58-34.20) and higher use of synthetic DMARD (sDMARD, OR 5.28, 95%-CI 1.39-20.01). Other variables like global assessment by physician and by patient and C Reactive Protein levels at 12-months post-diagnosis were assessed but without any predictive value after adjusting for confounding factors. CONCLUSIONS: Our results suggest that the earlier disease onset, the persistence of arthritis throughout the first year of disease evolution and the need of sDMARD might predict a persistent disease course.

Breaking down the fences among registries on autoinflammatory diseases: the E-Merge project.

BACKGROUND: Among the various numbers of different autoinflammatory diseases (AIDs), the absolute majority of them remains rare, with a single representative in large populations. This project, endorsed by PRES, supported by the EMERGE fellowship program, and performed in line with the Metadata registry for the ERN RITA (MeRITA), has the objective of performing a data synchronization attempt of the most relevant research questions regarding clinical features, diagnostic strategies, and optimal management of autoinflammatory diseases. RESULTS: An analysis of three large European registries: Eurofever, JIR-cohort and AID-Net, with a total coverage of 7825 patients from 278 participating centers from different countries, was performed in the context of epidemiological and clinical data merging. The data collected and evaluated in the registries does not cover only pediatric patients, but also adults with newly diagnosed AIDs. General aspects of the existing epidemiological data have been discussed in the context of patient global distribution, potential diagnostic delays, access to genetic testing, and the availability of the treatment. CONCLUSIONS: In general, the results indicate a great potential for upcoming collaborative work using existing data in cohorts that enhance the quality of medical care performed for patients with autoinflammatory diseases.

Laparoscopic hernia repair: a two-port technique.

OBJECTIVE: Various ventral and incisional hernia repair techniques exist and have largely replaced the open ones. The purpose of this study was to document the 2-port technique and demonstrate that it is feasible, efficient, and safe. To our knowledge, this is the largest report on this topic to date in the English-language literature. METHODS: Forty patients with ventral hernias (VH) or incisional hernias (IH) underwent laparoscopic repair with a 2-port technique. The technique involves insertion of one 10-mm to 12-mm balloon port and one 5-mm port, usually on the left side as laterally as possible. A mesh is inserted through the balloon port site and secured to the abdominal wall by using either 4 peripheral or 1 central Prolene suture. Helical fasteners are used to attach the mesh to the abdominal wall. RESULTS: Forty patients with 47 hernias underwent repair. Operating time ranged from 15 minutes to 70 minutes. Early complications were seen in 5 patients and included 1 small bowel enterotomy, 2 small bowel obstructions (SBO) with bowel adhering to the visceral side of the mesh, 1 wound infection, and 1 seroma. Late postoperative complications occurred in 8 patients (20%) who experienced persistent abdominal pain that resolved without any treatment. There was one recurrence during a mean follow-up of 23.5 months. CONCLUSION: Laparoscopic herniorrhaphy with the 2-port technique offers an efficient, safe, and effective repair for ventral and incisional hernias.

Direct trocar insertion technique: an alternative for creation of pneumoperitoneum.

OBJECTIVES: The direct trocar insertion technique (DTI) for the creation of pneumoperitoneum has been described as an alternative to open and Veress needle (VN) techniques. This study assesses the safety and feasibility of direct trocar insertion without a pre-existing pneumoperitoneum in patients undergoing elective laparoscopic procedures. METHODS: From November 2001 to February 2006, we retrospectively studied 196 (146 women and 50 men) consecutive patients. A single consultant laparoscopic surgeon performed all operations. The mean patient age was 57 years (range, 22 to 81). The procedures included 186 laparoscopic cholecystectomies, 3 laparoscopic appendectomies, 1 laparoscopic Nissen, 5 laparoscopic groin hernia repairs, and 1 conversion to open surgery. RESULTS: Creation of pneumoperitoneum with DTI was feasible in 99.5% of patients. No major complications were associated with the technique. Immediate minor postoperative complications included 1 (0.5%) wound infection and 3 (1.5%) hematomas. At mean follow-up of 23 months, 4 (2%) umbilical wound stitch granulomas and 1 (0.5%) incisional hernia from the umbilical port site were observed. CONCLUSION: This study shows that that when performed by an experienced laparoscopic surgeon the direct trocar insertion technique is a safe and effective alternative for creation of pneumoperitoneum.