| 1. |
- Berg, Stefan, 1959, et al.
(författare)
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Autoinflammatory Disorders
- 2016
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Ingår i: Primary Immunodeficiency Diseases. - Berlin, Heidelberg : Springer. - 9783662529096 ; , s. 393-435
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Bokkapitel (refereegranskat)
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| 2. |
- Berg, Stefan, 1959, et al.
(författare)
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Autoinflammatory disorders
- 2017
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Ingår i: Primary Immunodeficiency Diseases. Definition, Diagnosis, and Management, 2nd ed.. - Berlin, Germany : Springer. - 9783662529072
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Bokkapitel (övrigt vetenskapligt/konstnärligt)
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| 3. |
- Berg, Stefan, 1959, et al.
(författare)
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Irregular Recurrent Fever : Chapter 113
- 2019
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Ingår i: Pediatric Immunology. A Case-Based Collection with MCQs. Nima Rezaei (red.). - Cham : Springer Nature Switzerland AG. - 9783030212629 ; , s. 617-621
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Bokkapitel (övrigt vetenskapligt/konstnärligt)abstract
- Non-classifiable periodic fever syndromes are common Patients may have recurrent fevers or continuous chronic inflammation, together with different combinations of arthralgia/arthritis, mouth ulcers, lymphadenopathies, conjunctivitis, rashes, pleuritic pain, splenomegaly, hepatomegaly and abdominal pain Inheritance varies from no apparent pattern to autosomal dominant inheritance Many patients respond to colchicine as a reasonable first-line treatment
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| 4. |
- Berg, Stefan, 1959, et al.
(författare)
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Long Episodes of Rash and Fever : Chapter 100
- 2019
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Ingår i: Pediatric Immunology : A Case-Based Collection with MCQs, Volume 2. Rezaei, N. (red.). - Switzerland AG : Springer Nature. - 9783030212629 ; , s. 527-531
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Bokkapitel (övrigt vetenskapligt/konstnärligt)
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| 5. |
- Berg, Stefan, 1959, et al.
(författare)
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Rash and Fever since Two Weeks of Age : Chapter 102
- 2019
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Ingår i: Pediatric Immunology. A Case-Based Collection with MCQs. Rezaei, N. (red.). - Cham : Springer Nature. - 9783030212629 ; , s. 539-543
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Bokkapitel (övrigt vetenskapligt/konstnärligt)abstract
- Cryopyrin-associated periodic syndrome (CAPS) is an umbrella term today used for three formerly described conditions in order of increasing severity familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), and chronic infantile neurologic cutaneous and articular syndrome (CINCA), also known as neonatal-onset multisystem inflammatory disease (NOMID) CAPS is an autosomal dominant disease that starts early in life Mutations in CAPS give rise to a gain-of-function in the NLRP3 inflammasome Somatic mosaicism should be considered in patients with clinical CAPS and no mutation detected in the NLRP3 with Sanger sequencing CAPS is characterized by a varying degree of systemic inflammation, urticaria-like rash, musculoskeletal symptoms, and a risk of amyloidosis and neurologic sequelae Treatment with IL-1 blockade is generally very effective in CAPS
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| 6. |
- Berg, Stefan, 1959, et al.
(författare)
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Sudden Dizziness, Somnolence and Diplopia : Chapter 111
- 2019
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Ingår i: Pediatric Immunology. A Case-Based Collection with MCQs.. - Cham : Springer Nature. - 9783030212629 ; , s. 603-609
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Bokkapitel (övrigt vetenskapligt/konstnärligt)abstract
- Deficiency of adenosine deaminase 2 (DADA2) is a autosomal recessive disease caused by mutations in CECR1 Mutations in CECR1 cause a deficiency of the enzyme adenosine deaminase type 2 (ADA2) DADA2 phenotype has a wide spectrum and is characterized by the presence of three main features: (1) vascular inflammation, (2) immunodeficiency, and (3) coagulopathy, that may or may not overlap in the individual patient The vascular-inflammatory manifestations include livedo reticularis/racemosa, stroke, vasculitis, recurrent fever episodes and increased inflammatory markers The risk for stroke is high in DADA2 The phenotype may be almost indistinguishable to polyarteritis nodosa (PAN) TNF-blockade is an effective treatment for the vasculitis and inflammatory manifestations Patients with severe disease especially with hematological manifestations and immunodeficiency may benefit from HSCT
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| 7. |
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| 8. |
- Brown, Kelly, 1973, et al.
(författare)
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Profile of blood cells and inflammatory mediators in periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome.
- 2010
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Ingår i: BMC pediatrics. - : Springer Science and Business Media LLC. - 1471-2431. ; 10
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Tidskriftsartikel (refereegranskat)abstract
- ABSTRACT: BACKGROUND: This study aimed to profile levels of blood cells and serum cytokines during afebrile and febrile phases of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome to advance pathophysiological understanding of this pediatric disease. METHODS: A cohort of patients with a median age of 4.9 years experiencing 'typical PFAPA' episodes participated in this study. Blood cells and serum cytokines were analyzed by CBC analysis and multiplex ELISA. RESULTS: Oscillations in the concentration of blood cells during the afebrile and febrile phases of typical PFAPA syndrome were observed; novel findings include increased monocytes and decreased eosinophils during a febrile episode and increased thrombocytes in the afebrile interval. Relatively modest levels of pro-inflammatory cytokines were present in sera. IFNγ-induced cytokine IP10/CXCL10 was increased after the onset of fever while T cell-associated cytokines IL7 and IL17 were suppressed during afebrile and febrile periods. CONCLUSIONS: Identification of dysregulated blood cells and serum cytokines is an initial step towards the identification of biomarkers of PFAPA disease and/or players in disease pathogenesis. Future investigations are required to conclusively discern which mediators are associated specifically with PFAPA syndrome.
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| 9. |
- Holmgren, Daniel, 1945, et al.
(författare)
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Bakjoursutbildning för barnläkare kan stärka patientsäkerheten: Fortbildningsprojekt utvärderas i västra götalandsregionen
- 2016
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Ingår i: Läkartidningen. - 0023-7205. ; 113:10, s. 1-5
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Tidskriftsartikel (refereegranskat)abstract
- As part of a mandate to provide continuing professional development (CPD) for paediatricians in western Sweden and to support patient safety, we have addressed the professional demands which are imposed on today’s consultant paediatricians on call, by designing and implementing a CPD programme for this group. Learning objectives for specific learning modules were developed from a pre-course needs assessment among consultants. The implemented programme included a variety of learning, assessment and evaluation methods. Each specific learning module began with a reading assignment exploring the key concept. This prepared the participants for the case discussion, in which they would analyse, reflect and achieve a deeper level of understanding. We believe that the educational methods used and the approach of the programme are applicable to specialist training as well as to CPD programmes in general, as part of a lifelong learning process. © 2016, (Publisher). All rights reserved.
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| 10. |
- Holmgren, Daniel, 1945, et al.
(författare)
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Combining learning for educators and participants in a paediatric CPD programme
- 2019
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Ingår i: BMC Medical Education. - : Springer Science and Business Media LLC. - 1472-6920. ; 19
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Tidskriftsartikel (refereegranskat)abstract
- Background: Most continuing professional development (CPD) programmes do not include an educational training module. In our country, educational practice in the areas of CPD and continuing medical education relies traditionally on conventional lectures. This is in sharp contrast to the educational research that clearly demonstrates that educational programmes emphasising adult learning methods have greater potential to change physicians'clinical practice. To investigate whether lecture-oriented educators were prepared to change their educational practice towards principles of adult learning, we decided to combine learning for educators and participants in a paediatric CPD programme.The aim of the study was to investigate educators' reflections on their learning and educational practice after they have undergone an educational skills component integrated in the implementation of a CPD learning module for paediatricians and evaluate the results from the participants' perspective.Methods: The objectives of the educational skills component of the learning module were developed according to adult learning theories. The learning objectives for the CPD learning module were based on a pre-course needs assessment. Evaluations were made using questionnaires.Results: Seven of 10 participants in the educational skills component of the learning module and all the participants, 13 paediatricians and 14 nurses, who participated in the learning module, answered the questionnaires.The results of this pilot study show that educators whose main experience of teaching was based on lectures were strengthened in their practice; they defined their competence and were prepared to move towards adult learning principles. The participants in the learning module expressed a high degree of satisfaction.Conclusions: We conclude that it is feasible to combine learning for educators and participants in a paediatric CPD programme and that lecture-oriented educators are prepared to change their educational practice towards principles of adult learning.
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| 11. |
- Holmgren, Daniel, 1945, et al.
(författare)
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Integrating a pedagogic course in a CPD programme for paediatricians at out-patient clinics
- 2021
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Ingår i: Journal of European CME. - : Informa UK Limited. - 2161-4083. ; 10:1
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Tidskriftsartikel (refereegranskat)abstract
- Equipping paediatricians for the challenges of the explosive development of knowledge and specialised health care calls for a well-planned continuing professional development (CPD) strategy which updates paediatric competencies and the pedagogic skills among paediatricians. The purpose of the study was to evaluate the effects of a pedagogic course, integrated into a CPD programme for paediatricians at out-patient clinics. The pedagogic course comprised three learning components, participation in a CPD programme, during two and a half years, a pedagogic learning module and a pedagogic assignment. The objectives of all the learning activities, including the pedagogic course, were developed according to adult learning theories. Evaluations were made using questionnaires. Seventeen paediatricians participated in the CPD programme; 13 of them completed the pedagogic learning module and six the full pedagogic course, including the pedagogic assignment - teaching at one's own clinic. Evaluation of the pedagogic assignment at the participants' own clinics by 64 co-workers revealed that the co-workers appreciated the training activities and would recommend them to a colleague. We conclude that it is possible to combine medical and pedagogic education in a CPD programme for paediatricians and that the participants were able to digest and apply the pedagogic principles used in the course.
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| 12. |
- Holmgren, Daniel, 1945, et al.
(författare)
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Paediatric on-call consultants' learning within and beyond the objectives of a coherent CPD program.
- 2022
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Ingår i: BMC medical education. - : Springer Science and Business Media LLC. - 1472-6920. ; 22:1
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Tidskriftsartikel (refereegranskat)abstract
- Evaluations, using questionnaires, of a two-year long CPD program for on-call consultant paediatricians, showed that the overall objective of the program was largely met. We stipulate that the coherency of the CPD program contributed to the learning. To gains a deeper understanding of the participants learning within and beyond the overall objectives of the program, we decided to conduct an interview study enrolling participants from the first two CPD courses.Nine experienced paediatric consultants were interviewed 1-4 years after completing a coherent two-year long CPD program, focusing on what and how they learned. The interviews were audio-recorded and transcribed as text, analysed, and categorised using qualitative content analysis.What the participants learned: improved medical competences, greater confidence in the role of an on-call consultant, better understanding of the role of an on-call consultant and importance of professional networks. Several categories were outside the overall objective, at personal level: an understanding of one's own and other's competences, taking responsibility for one's own CPD and managing things one does not know. At professional level: more secure as an individual and with colleagues. How itwas learned: relevant objectives, preparatory material and case discussions were important. Participants learned by preparing, repeating, reflecting, and participating actively, and by applying what they learned in clinical practice. The participants learned from one other over a period of two years, when they also got to know one another and created networks. A safe learning environment imposed demands and enabled participants to define their competence and learn accordingly.This study describes what and how on-call consultant paediatricians learned during a coherent two-year long CPD program. The learning took place within and beyond the framework of the overall objectives. The study suggests that evaluation methods based on objectives may be blind to important areas of learning and need to be combined with qualitative methods that examine a broad impact of learning. Taken together, the analysis of what and how the participants learned shows that they were better equipped to work as consultant on call and deal with the things they did not know.
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| 13. |
- Islind, Anna Sigridur, 1985-, et al.
(författare)
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Co-creating a Digital Symptom Tracker : An App as a Boundary Object in the Context of Pediatric Care
- 2022
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Ingår i: IFIP Advances in Information and Communication Technology. - Cham : Springer Science+Business Media B.V.. - 1868-4238. ; 660 IFIP, s. 79-93
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Tidskriftsartikel (refereegranskat)abstract
- The rise of digital health has provided new opportunities for patients to be more actively involved in their health and wellbeing. Despite the increased use of mobile health apps, there is still a lack of research on patient self-monitoring, and few studies have focused on children with chronic diseases and their parents. In this study, we draw from a case of the design of a mobile application – a symptom tracker – to continuously monitor children with periodic fever and the theoretical concept of boundary objects, to understand the role of digital artifacts in current healthcare practice. The research approach is qualitative, building on interview data with parents and experiences from the co-design process involving researchers, physicians, and other key stakeholders. The aim of the paper is to contribute with a better understanding of how an app for tracking children’s fever (a symptom tracker) can support the pediatricians as well as the parents and their children during the treatment process. The research question is: In what ways can a symptom tracker increase stakeholder involvement and how may this affect their relationship boundaries and collaborations? Our findings suggest that the symptom tracker can be seen as a boundary object that binds the children, parents, and pediatricians treating them by connecting the app to the context of both the patients and healthcare practice. We argue that such an object (symptom tracker) can function as external support and, thereby, an essential part of the treatment process.
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| 14. |
- Pettersson, Jonas, 1971-, et al.
(författare)
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Using scenario-based training to promote information literacy among on-call consultant pediatricians.
- 2017
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Ingår i: Journal of the Medical Library Association. - : University Library System, University of Pittsburgh. - 1536-5050 .- 1558-9439. ; 105:3, s. 262-267
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Traditionally, teaching hospital staff to search for medical information relies heavily on educator-defined search methods. In contrast, the authors describe our experiences using real-time scenarios to teach on-call consultant pediatricians information literacy skills as part of a two-year continuing professional development program.CASE PRESENTATION: Two information-searching workshops were held at Sahlgrenska University Hospital in Gothenburg, Sweden. During the workshops, pediatricians were presented with medical scenarios that were closely related to their clinical practice. Participants were initially encouraged to solve the problems using their own preferred search methods, followed by group discussions led by clinical educators and a medical librarian in which search problems were identified and overcome. The workshops were evaluated using questionnaires to assess participant satisfaction and the extent to which participants intended to implement changes in their clinical practice and reported actual change.CONCLUSIONS: A scenario-based approach to teaching clinicians how to search for medical information is an attractive alternative to traditional lectures. The relevance of such an approach was supported by a high level of participant engagement during the workshops and high scores for participant satisfaction, intended changes to clinical practice, and reported benefits in actual clinical practice.
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| 15. |
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| 16. |
- Rydenman, Karin, 1982, et al.
(författare)
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Antibiotic prescriptions to children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis
- 2024
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Ingår i: Acta Paediatrica, International Journal of Paediatrics. - 0803-5253 .- 1651-2227. ; 113:8, s. 1927-1933
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Tidskriftsartikel (refereegranskat)abstract
- Aim: To investigate the rate of dispensed antibiotic prescriptions to children and adolescents with PFAPA and compare this with the rate for children in the general population. Furthermore, to compare dispensed antibiotic prescription rates before and after a diagnosis of PFAPA was established. Methods: Patients aged 0–17 years and diagnosed with PFAPA between 1 January 2006 to 31 October 2017 were included retrospectively. Data on dispensed drug prescriptions were obtained from the Swedish National Prescribed Drug Register. Results: The PFAPA cohort received more antibiotic prescriptions than the general population in all but one of the age groups and time periods that were analysed. The largest difference was seen in 2014–2017 in the youngest age group (0–4 years) when children with PFAPA received 1218 antibiotic prescriptions per 1000 person years compared to 345 in the general population (IRR 3.5; 95% CI 2.8–4.4). The yearly number of antibiotic prescriptions to PFAPA patients was reduced from 2.1 before diagnosis to 0.8 after diagnosis, a reduction of 62%. Conclusion: This study shows higher rates of dispensed antibiotic prescriptions for children with PFAPA than in the general population. The reduction of prescriptions after an established PFAPA diagnosis indicates that antibiotics were previously incorrectly prescribed for PFAPA episodes.
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| 17. |
- Rydenman, Karin, 1982, et al.
(författare)
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Chapter 104. Recurrent Fever and Sore Throat
- 2019
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Ingår i: Pediatric Immunology. A Case-Based Collection with MCQs. Rezaei N. (red.). - Cham : Springer. - 9783030212612 ; , s. 553-559
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Bokkapitel (övrigt vetenskapligt/konstnärligt)
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| 18. |
- Rydenman, Karin, 1982, et al.
(författare)
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Epidemiology and clinical features of PFAPA: a retrospective cohort study of 336 patients in western Sweden
- 2022
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Ingår i: Pediatric Rheumatology. - : Springer Science and Business Media LLC. - 1546-0096. ; 20
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Tidskriftsartikel (refereegranskat)abstract
- Background: Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is generally regarded as the most common autoinflammatory disease, but the epidemiology of the disease is largely unknown. The objectives of this study were to estimate the annual incidence and describe the clinical features of PFAPA in a large cohort from western Sweden. Methods: The study retrospectively included children < 18 years of age diagnosed with PFAPA between 2006 and 2017 at three hospitals: NU Hospital Group, Skaraborg Hospital and Queen Silvia Children’s Hospital. Patients were identified by searching for relevant diagnostic ICD-10 codes in the comprehensive electronic medical records and data were retrieved by reviewing case records. To estimate incidence, patients with symptom onset from January 1, 2006, to December 31, 2016, were included. Population data for the study area during this period were retrieved from Statistics Sweden. Results: In this study, 336 patients with PFAPA were identified. Of these, 156 (46%) were girls and 180 (54%) were boys. Almost 90% of the children with PFAPA (291 patients) experienced their first symptoms before the age of 5 years and fewer than 3% presented at ages above 10 years. Pharyngitis was the most common symptom during febrile episodes, followed by cervical adenitis and aphthous stomatitis. Fourteen percent of the patients displayed atypical features, of which skin rash was the most common. To calculate incidence, 251 patients with symptom onset during the study period were identified. The mean annual incidence was estimated at 0.86/10,000 for children < 18 years of age and 2.6/10,000 for children < 5 years of age. Conclusions: This study adds to the understanding of the epidemiology of PFAPA syndrome by presenting incidence rates based on a large cohort and in different age groups in a population-based setting. It also shows the distribution of age of onset of PFAPA, with a peak in 1-year-olds and waning at older ages. Signs and symptoms of PFAPA syndrome were similar in children with symptom onset before vs. after 5 years of age.
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| 19. |
- Rydenman, Karin, 1982, et al.
(författare)
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PFAPA syndrome - An important differential diagnosis in children with recurrent fever : PFAPA-syndrom – en viktig differentialdiagnos hos barn med återkommande feberepisoder.
- 2019
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Ingår i: Läkartidningen. - 1652-7518. ; 116
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Forskningsöversikt (refereegranskat)abstract
- Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis (PFAPA) syndrome is the most common autoinflammatory disorder among children in many parts of the world and an important differential diagnosis in children presenting with recurrent fever episodes. Commonly, PFAPA has an onset under the age of 5 years. Fever episodes in PFAPA usually last 3-6 days and are associated with one or more of the cardinal symptoms aphthous stomatitis, pharyngitis and cervical adenitis. The fever episodes typically recur with an interval of 3-6 weeks, often with a striking regularity. During the episodes, the patient has elevated inflammatory variables such as CRP and serum amyloid A (SAA) and may sometimes have additional symptoms such as abdominal pain, nausea and leg pain. Between the fever episodes, the patient is typically free of symptoms with normalized inflammatory variables and grows normally. Awareness and recognition of PFAPA is key to providing the patient with adequate treatment and avoiding misdiagnosis.
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| 20. |
- Rydenman, Karin, 1982, et al.
(författare)
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Tonsillectomy reduces the family impact of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome and improves health-related quality of life in affected children
- 2023
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Ingår i: Orphanet Journal of Rare Diseases. - 1750-1172. ; 18:1
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Tidskriftsartikel (refereegranskat)abstract
- Background: Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is an autoinflammatory disorder that primarily affects young children, and typically gives rise to fever episodes that recur monthly for several years. This study investigated the impact of PFAPA syndrome on the families of affected children, the health-related quality of life (HRQOL) of children with the syndrome, and how these factors were influenced by tonsillectomy. Methods: This prospective cohort study included 24 children with typical PFAPA syndrome that were referred for tonsillectomy, of whom 20 underwent the procedure. The control group consisted of randomly selected children from the general population. Family impact and HRQOL were measured using the standardized, validated questionnaires Pediatric Quality of Life Inventory™ (PedsQL™) Family Impact Module (FIM) and PedsQL™ 4.0 Generic Core Scales (GCS). Parents to children with PFAPA completed the questionnaires before and 6 months after their child underwent tonsillectomy, and HRQOL was measured both between and during PFAPA episodes. The Wilcoxon signed-rank test was used to compare data before and after tonsillectomy in the patient group, while the Mann–Whitney test was used for comparison of the patient and control groups. Results: Before tonsillectomy, children with PFAPA had significantly lower scores than the control group on the PedsQL™ FIM and the PedsQL™ 4.0 GCS during fever episodes. After tonsillectomy, all patients improved with diminished febrile episodes, which resulted in significantly higher scores regarding both family impact and HRQOL at the time of follow-up. HRQOL of in children with PFAPA improved after tonsillectomy even when compared to afebrile intervals before the procedure. The differences between PFAPA patients and controls were eliminated after tonsillectomy. Conclusion: PFAPA syndrome has a profound negative impact on the families of affected children. Tonsillectomy that leads to cessation or reduction of fever episodes eases the impact of the disease on the family. HRQOL in children with PFAPA is low during febrile episodes and similar to healthy controls in between episodes. The improvement of HRQOL in patients with PFAPA after tonsillectomy compared to the afebrile intervals before tonsillectomy highlights that the constantly recurring fevers may affect the children’s well-being even between fever episodes.
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| 21. |
- Sparud Lundin, Carina, 1964, et al.
(författare)
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From uncertainty to gradually managing and awaiting recovery of a periodic condition- a qualitative study of parents´ experiences of PFAPA syndrom
- 2019
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Ingår i: BMC Pediatrics. - : Springer Science and Business Media LLC. - 1471-2431. ; 19:99, s. 1-9
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Tidskriftsartikel (refereegranskat)abstract
- BackgroundThe prevalence of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is unknown. Although an uncommon condition, it is considered to be the most common autoinflammatory disease among children in many parts of the world. The knowledge of the consequences of the recurrent fever episodes for the child and its family are limited. This study explores the experiences of parents regarding the impact of the disease on the child's general well-being, the family's situation and how the family handles the associated challenges.MethodsA qualitative approach was used, applying a modified version of Grounded theory for design, data collection and analysis. Data was collected from two different sources: communication between parents of children with PFAPA in a closed Facebook group and face-to face interviews with one of the parents of children diagnosed with PFAPA (6 mothers and 2 fathers).ResultsParents described a lengthy process of how everyday life becomes affected by their child's recurrent fever episodes. This process is depicted in the following Grounded Theory core category: From uncertainty to gradually managing and awaiting recovery. The categories Uncertainty, Assurance, Gradually managing and Recovery describe the experienced illness trajectory. The illness representation illustrates the experiences/impacts of the periodic condition in the subcategories: Harmlessness-Severity, Disclosure of diagnosis, Impact on daily life and Regularity-Unpredictability. The children's well-being was highly affected by the symptoms during episodes. Parents experienced increased stress with constant fatigue, social constraints of family life and restricted career opportunities. Nevertheless, hope of recovery was constantly present.ConclusionsPFAPA is associated with a considerable burden on the child and the parents in daily life. Obtaining a diagnosis enables parents to move from a state of uncertainty towards a sense of coherence while awaiting recovery. Because of limited general knowledge of the condition and its impact on daily life, health care professionals need to become aware of the parents' efforts to mitigate the consequences of the recurrent episodes for the child and for the family as a whole.
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| 22. |
- Sundqvist, Martina, et al.
(författare)
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Increased intracellular oxygen radical production in neutrophils during febrile episodes of PFAPA syndrome.
- 2013
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Ingår i: Arthritis and rheumatism. - : Wiley. - 1529-0131 .- 0004-3591. ; 65:11, s. 2971-2983
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Tidskriftsartikel (refereegranskat)abstract
- Objective: Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome is an autoinflammatory disease of unknown etiology that primarily affects preschool children. PFAPA is characterized by recurrent attacks of fever and inflammatory symptoms consistent with the disease acronym. Since autoinflammatory diseases by definition are mediated by cells of the innate immune system, we aimed at evaluating functional features of neutrophils, the most abundant innate immune cell in circulation, in PFAPA syndrome. Methods: Blood neutrophils, obtained from PFAPA patients during both febrile and asymptomatic afebrile phases of disease, as well as from healthy children (afebrile controls) and children with fever and abdominal pain (febrile controls) were analysed for three key neutrophil characteristics: (i) apoptosis (measured by Annexin V/7AAD staining), (ii) production of reactive oxygen species (ROS; by luminol/isoluminol-amplified chemiluminescence), and (iii) priming status (as responsiveness to galectin-3 and upregulation of CD11b). Results: Compared to neutrophils from both PFAPA patients in an afebrile interval and from febrile controls, neutrophils obtained during a PFAPA flare produced elevated levels of intracellular NADPH-oxidase-derived ROS, had significantly diminished rates of spontaneous apoptosis, and displayed signatures of priming. In contrast, neutrophils from afebrile PFAPA patients had a significantly elevated rate of spontaneous apoptosis compared to neutrophils from afebrile controls. Conclusions: We demonstrate that three key aspects of neutrophil innate immune function, namely apoptosis, priming, and generation of an intracellular oxidative burst are altered, most prominently during febrile attacks in PFAPA syndrome. © 2013 American College of Rheumatology.
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| 23. |
- Sundqvist, Martina, et al.
(författare)
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Severe chronic non-bacterial osteomyelitis in combination with total MPO deficiency and responsiveness to TNFα inhibition
- 2023
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Ingår i: Frontiers in Immunology. - : FRONTIERS MEDIA SA. - 1664-3224. ; 14
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Tidskriftsartikel (refereegranskat)abstract
- We describe a female patient suffering from severe chronic non-bacterial osteomyelitis (CNO) with systemic inflammation and advanced malnutrition and complete deficiency of myeloperoxidase (MPO). CNO is a rare autoinflammatory bone disorder associated with dysregulation of the innate immune system. MPO deficiency is a genetic disorder with partial or complete absence of the phagocyte peroxidase MPO. MPO deficiency has no established clinical phenotype but reports indicate increased susceptibility to infection and chronic inflammation. The patient’s symptoms began at 10 years of age with pain in the thighs, systemic inflammation and malnutrition. She was diagnosed with CNO at 14 years of age. Treatment with nonsteroidal anti-inflammatory drugs, corticosteroids, bisphosphonates or IL1-receptor antagonists (anakinra) did not relieve the symptoms. However, the patient responded instantly and recovered from her clinical symptoms when treated with TNFα blockade (adalimumab). Three years after treatment initiation adalimumab was withdrawn, resulting in rapid symptom recurrence. When reintroducing adalimumab, the patient promptly responded and went into remission. In addition to clinical and laboratory profiles, neutrophil functions (reactive oxygen species, ROS; neutrophil extracellular traps, NETs; degranulation; apoptosis; elastase activity) were investigated both in a highly inflammatory state (without treatment) and in remission (on treatment). At diagnosis, neither IL1β, IL6, nor TNFα was significantly elevated in serum, but since TNFα blockade terminated the inflammatory symptoms, the disease was likely TNFα-driven. All neutrophil parameters were normal both during treatment and treatment withdrawal, except for MPO-dependent intracellular ROS- and NET formation. The role of total MPO deficiency for disease etiology and severity is discussed.
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| 24. |
- Vallo Hult, Helena, 1976-, et al.
(författare)
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Co-designing a Digital Platform : Towards e-Health and Continuous Learning in General Paediatrics
- 2017
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Ingår i: International Forum on Quality & Safety in Healthcare.
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Konferensbidrag (övrigt vetenskapligt/konstnärligt)abstract
- BackgroundThe study is part of a paediatric CPD program in Western Sweden, involving researchers and practitioners with expertise in medicine, informatics, and pedagogy. Based on previous experiences, the program provides a strong learning environment with extensive collaboration and networking among participants, while it is hard to maintain the collegial network and continue to share knowledge and experiences. Research has shown that new information technologies have potential to create learning opportunities and support reflection, collaboration and workplace learning. However, due to a history of IT-related problems in healthcare, where (despite documented benefits) many eHealth initiatives have failed in practice, health professionals are often less supportive towards such technologies. The research question is: How can IT be used for continuous learning and what are the implications of participatory design for the introduction of eHealth for workplace learning and use in clinical context?MethodInspired by participatory action research (PAR), the program is designed to offer a learning environment in which participants develop their paediatric and educational practices, as well as improve IT skills and digital competence needed as per today and in the future. In this new program a digital learning platform is introduced with the intention to contribute to formal course objectives but also to function as support for informal learning, networking and knowledge sharing during and after the CPD program, thus promoting long-term continuity of the educational outcomes. During the two-year course, the participants will develop a digital platform together with a Ph.D. student, for paediatric practice, present, and future learning as well as a web-based learning environment.OutcomeThe benefits of this project, where the focus is on eHealth as support for continuous learning, is for healthcare providers and physicians to be able to meet the demands and challenges for the future medical profession. The originality is that the digital platform in use is developed by the participants, integrated into the course and daily work and that the primary focus of the platform is to support continuous learning. This research is still in an initial stage (2016-2018), and findings are tentative. The project is linked to patient benefit, health professionals’ workplace learning, and continuous training. Children, carers or family members are not directly involved in this project, but the CPD program is built up around patient cases and focus on patient outcomes, in terms of improved safety and quality of care of the child.ConclusionThere are potential difficulties recruiting paediatric educators to the program. This is addressed by supporting the participants in their role as educators and experts in outpatient paediatrics as well as regarding how IT could support not only learning a new subject or training skills but also learning how to learn. In summary, this project highlights the importance of interdisciplinary and practice-based research for continuous improvement and quality in healthcare, especially due to new demands following the patient-centric care and new role of patients, and sheds light on both benefits and challenges of integrating IT and learning in daily clinical work
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- Vallo Hult, Helena, 1976-, et al.
(författare)
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Decreased Memory Bias via a Mobile Application : A Symptom Tracker to Monitor Children's Periodic Fever.
- 2022
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Ingår i: Studies in Health Technology and Informatics. - 0926-9630 .- 1879-8365. ; 294, s. 915-919
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Tidskriftsartikel (refereegranskat)abstract
- Memory bias, the tendency to rely on certain events over others, can become an issue in chronic illnesses, especially when symptoms are reported retrospectively. This paper examines a case where continuous symptom registration can be facilitated, memory supported, and memory bias reduced by introducing a mobile application. The aim of the paper is to report on the design of an app for collecting subjective data over an extended period to continuously follow children with periodic fever. The research approach is qualitative, building on interview data. The design method is co-design, a collaborative and participatory approach involving researchers, physicians and other key stakeholders, with focus on the views of the parents. We argue that collecting data continuously through an app moves the discussion from memory to the specific data points, which is illustrated through trends shown in the visualizations of the data. Moreover, we highlight the importance of systematically collecting data over an extended period through a data-driven approach to both forward clinical practice and research on complex, often chronic topics such as periodic fever, which is genuinely under-researched to date.
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