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Background: Deficiency of interleukin-36 receptor antagonist (DITRA) is a life threatening monogenic autoinflammatory disease caused by loss of function mutations in the IL36RN gene. Affected patients develop recurrent episodes of generalized pustular psoriasis (GPP) with systemic inflammation and fever. We here review and analyze the literature on pediatric DITRA patients who have been treated by biologicals targeting inflammatory cytokines., Method: A database research was performed to identify all relevant articles on pediatric DITRA patients treated with biologicals. According to defined response criteria therapeutic efficacy was analyzed., Results: Our literature research revealed 12 pediatric patients with DITRA who have received treatment with biologicals and we add a further not yet reported patient. Out of these 13 patients 10 were homozygous including 6 with the p.Leu27Pro, 3 with the p.Arg10 Argfs* and 1 with the p.Thr123Met mutation. 3 patients were compound heterozygous. In total 28 flares were treated with biological agents- targeting IL-1, IL-17, IL-12/23 and TNF-α. Complete response was achieved in 16 flares (57%), a partial reponse was seen in 2 flares (7%), and no response was observed in 10 flares (36%). Response rates were heterogeneous among the different agents. While complete/partial/no response with inhibition of TNF-alpha could be achieved in 7 (58%)/1 (8%)/4 (33%), the inhibition of IL-17 and of IL-12/23 led in each 4 flares to a 100% complete response. IL-1 inhibition led to complete/partial response in each 1 (13%) and was not effective in 6 (76%) flares. Of note, the novel patient was successfully treated with weekly dosed adalimumab., Conclusions: DITRA is a rare disease that has to be considered in GPP with systemic inflammation and fever. It can be effectively treated with specific biological inhibition of TNF-alpha, IL-12/23 and IL- 17, while anti-IL-1 treatment seems less effective. Weekly dosed adalimumab appears to be a treatment option for pediatric patients. Further reports and studies of biological treated pediatric DITRA patients are warranted for evaluation of optimal treatment.

Introduction: The importance of research, as promoted by the CanMEDS framework, is widely acknowledged. Many medical students in Germany work on a research project as part of their doctoral thesis whilst still going to medical school. However, a significant amount of projects are abandoned unfinished, which leads to substantial wastage of resources. One reason for this is an information deficit concerning undergraduate research projects., Project Description: To counteract this, we introduced an annual event at LMU Munich called DoktaMed with more than 600 visitors each year. It combines medical convention and research fair including keynote lectures, workshops and poster sessions as well as an exhibition of research groups and institutes. DoktaMed is a peer-to-peer event organized by a team of 40 students., Results: A needs analysis before its implementation underlined the information deficit as a possible cause for the high rate of abandoned projects. In the annual evaluation, visitors of DoktaMed rate the event with an average grade of 2.1 on a six-level Likert scale (n=558, SD=1.06, with "1=very good", "6=poor"). They stated to now feel better informed about the topic and regarded visiting DoktaMed as a worthwhile investment of time., Discussion: Students are generally satisfied with the event and feel better informed after visiting DoktaMed. However, many students never visit DoktaMed for various reasons. A possible improvement would be to present a greater number of clinical studies in addition to the laboratory work that DoktaMed focuses on now., Conclusion: Evaluation after six years of DoktaMed is very promising. Visitors seem to be better informed. Nevertheless there is space for improvement in order to get more students and more faculty members involved. More studies are needed to assess long-term effects.