| 1. |
- Mazereeuw-Hautier, J., et al.
(författare)
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Management of congenital ichthyoses : European guidelines of care, part two
- 2019
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Ingår i: British Journal of Dermatology. - : WILEY. - 0007-0963 .- 1365-2133. ; 180:3, s. 484-495
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Tidskriftsartikel (refereegranskat)abstract
- These guidelines for the management of congenital ichthyoses have been developed by a multidisciplinary group of European experts following a systematic review of the current literature, an expert conference held in Toulouse in 2016, and a consensus on the discussions. These guidelines summarize evidence and expert-based recommendations and intend to help clinicians with the management of these rare and often complex diseases. These guidelines comprise two sections. This is part two, covering the management of complications and the particularities of some forms of congenital ichthyosis. What's already known about this topic? Various symptomatic treatment options exist for congenital ichthyoses, but there are no European guidelines. What does this study add? These European guidelines for the management of congenital ichthyosis may help to improve outcomes and quality of life for patients. Linked Comment: Akiyama. Br J Dermatol 2019; 180:449-450. Plain language summary available online
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| 2. |
- Mazereeuw-Hautier, J., et al.
(författare)
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Management of congenital ichthyoses : European guidelines of care, part one
- 2019
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Ingår i: British Journal of Dermatology. - : Oxford University Press (OUP). - 0007-0963 .- 1365-2133. ; 180:2, s. 272-281
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Tidskriftsartikel (refereegranskat)abstract
- These guidelines for the management of congenital ichthyoses have been developed by a multidisciplinary group of European experts following a systematic review of the current literature, an expert conference held in Toulouse in 2016 and a consensus on the discussions. They summarize evidence and expert-based recommendations and are intended to help clinicians with the management of these rare and often complex diseases. These guidelines comprise two sections. This is part one, covering topical therapies, systemic therapies, psychosocial management, communicating the diagnosis and genetic counselling.
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| 3. |
- Charlesworth, A., et al.
(författare)
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Epidermolysis bullosa simplex with PLEC mutations : new phenotypes and new mutations
- 2013
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Ingår i: British Journal of Dermatology. - : Oxford University Press (OUP). - 0007-0963 .- 1365-2133. ; 168:4, s. 808-814
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Tidskriftsartikel (refereegranskat)abstract
- Background Genetic mutations in the plectin gene (PLEC) cause autosomal recessive forms of epidermolysis bullosa simplex (EBS) associated with either muscular dystrophy (EBS-MD) or pyloric atresia (EBS-PA). Phenotype-genotype analysis has suggested that EBS-MD is due mostly to genetic mutations affecting the central rod domain of plectin, and EBS-PA to mutations outside this domain. Objectives This study aimed to describe new phenotypes of patients with EBS-MD and EBS-PA, to identify novel PLEC mutations and to establish genotype-phenotype correlations. Methods Seven patients with a suspicion of EBS linked to PLEC mutations were included. A standardized clinical questionnaire was sent to the physicians in charge of each patient. Immunofluorescence studies of skin biopsies followed by molecular analysis of PLEC were performed in all patients. Results We report the first case of nonlethal EBS-PA improving with age, the first multisystemic involvement in a patient with lethal EBS-PA, and the first patients with EBS-MD with involvement of either the bladder or oesophagus. Eleven novel PLEC mutations are also reported. Conclusions Our results confirm that EBS-PA is linked to mutations in the distal exons 1-30 and 32 of PLEC. Long-term survival is possible, with skin improvement, but a delayed onset of MD is probable. While EBS-MD is linked to PLEC mutations in all exons, in most cases one of the mutations affects exon 31. The precocity of MD seems to be linked to the type and localization of the PLEC mutation(s), but no correlation with mucosal involvement has been found.
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| 4. |
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| 5. |
- El Hachem, J., et al.
(författare)
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Securing system-of-systems through a game theory approach
- 2021
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Ingår i: Proceedings of the ACM Symposium on Applied Computing. - New York, NY, USA : Association for Computing Machinery. - 9781450381048 ; , s. 1443-1446
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Konferensbidrag (refereegranskat)abstract
- Enabling System-of-Systems (SoS) security is an important activity when engineering SoS solutions like autonomous vehicles, provided that they are also highly safety-critical. An early analysis of such solutions caters for proper security architecture decisions, preventing potential high impact attacks and ensuring people's safety. However, SoS characteristics such as emergent behavior, makes security decision-making at the architectural level a challenging task. To tackle this challenge, it is essential to first address known vulnerabilities related to each CS, that an adversary may exploit to realize his attacks within the unknown SoS environment. In this paper we investigate how to use Game Theory (GT) approaches to guide the architect in choosing an appropriate security solution. We formulate a game with three players and their corresponding strategies and payoffs. The proposal is illustrated on an autonomous quarry example showing its usefulness in supporting a security architect to choose the the most suitable security strategy.
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| 6. |
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| 7. |
- Lisova, Elena, et al.
(författare)
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Investigating attack propagation in a SoS via a service decomposition
- 2019
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Ingår i: Proceedings - 2019 IEEE World Congress on Services, SERVICES 2019. - : Institute of Electrical and Electronics Engineers Inc.. - 9781728138510 ; , s. 9-14
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Konferensbidrag (refereegranskat)abstract
- A term systems of systems (SoS) refers to a setup in which a number of independent systems collaborate to create a value that each of them is unable to achieve independently. Complexity of a SoS structure is higher compared to its constitute systems that brings challenges in analyzing its critical properties such as security. An SoS can be seen as a set of connected systems or services that needs to be adequately protected. Communication between such systems or services can be considered as a service itself, and it is the paramount for establishment of a SoS as it enables connections, dependencies, and a cooperation. Given that reliable and predictable communication contributes directly to a correct functioning of an SoS, communication as a service is one of the main assets to consider. Protecting it from malicious adversaries should be one of the highest priorities within SoS design and operation. This study aims to investigate the attack propagation problem in terms of service-guarantees through the decomposition into sub-services enriched with preconditions and postconditions at the service levels. Such analysis is required as a prerequisite for an efficient SoS risk assessment at the design stage of the SoS development life cycle to protect it from possibly high impact attacks capable of affecting safety of systems and humans using the system.
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| 8. |
- Misery, L., et al.
(författare)
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A position paper on the management of itch and pain in atopic dermatitis from the International Society of Atopic Dermatitis (ISAD)/Oriented Patient-Education Network in Dermatology (OPENED) task force
- 2021
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Ingår i: Journal of the European Academy of Dermatology and Venereology. - : Wiley. - 0926-9959 .- 1468-3083. ; 35:4, s. 787-796
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Tidskriftsartikel (refereegranskat)abstract
- Atopic dermatitis (AD) is a disease that can have a high impact on quality of life, especially due to itch and skin pain. This paper utilizes expertise from members of the International Society of Atopic Dermatitis (ISAD)/Oriented Patient-Education Network in Dermatology (OPENED) task force to review the epidemiology, pathophysiology and exacerbating factors of itch and pain in atopic dermatitis. General principles of treatment are provided, as well as a more detailed evaluation of topical and systemic therapies. Educational and psychological approaches to itch and pain in atopic dermatitis are proposed, along with expert recommendations for the management of itch and pain in atopic dermatitis.
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