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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.

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.

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.

Autoinflammatory disease was introduced as a concept in 1999, demarcating an entirely new group of diseases in clinical, immunological, and conceptual terms. During recent years, the preconditions for the definition of autoinflammatory conditions have changed. This includes the recent discovery of a number of monogenic autoinflammatory conditions with complex phenotypes that combine autoinflammation with defects of the adaptive and/or innate immune system, resulting in the occurrence of infection, autoimmunity, and/or uncontrolled hyperinflammation in addition to autoinflammation. Further, there are strong indications that classical IL-1-driven autoinflammatory diseases are associated with activation of adaptive immunity. As suggested by this development, we are of the opinion that an all-encompassing definition of autoinflammatory diseases should regard autoinflammatory conditions and innate dysregulation as inseparable and integral parts of the immune system as a whole. Hence, in this article, we try to advance the conceptual understanding of autoinflammatory disease by, proposing a modification of the definition by Daniel Kastner et al., which allows for a congruent and precise description of conditions that expand the immunological spectrum of autoinflammatory disease.

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., (© 2024 The Authors. Acta Paediatrica published by John Wiley & Sons Ltd on behalf of Foundation Acta Paediatrica.)

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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Sundqvist, Christenson, Wekell, Björnsdottir, Dahlstrand Rudin, Sanchez Klose, Kallinich, Welin, Björkman, Bylund, Karlsson-Bengtsson and Berg.)

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.

Aim: To develop a coherent programme that addresses the need for continuing professional development in general paediatrics and educational skills for senior paediatricians at outpatient clinics in Sweden today., Methods: Educational needs in the target group were investigated using a questionnaire. Themes and sub-themes extracted from responses informed the curriculum formulation of the continuing professional development (CPD) programme, which was completed using a variety of learning, assessment and evaluation methods., Results: Forty-six paediatricians identified 355 clinical situations. Competencies in general paediatrics and educational skills were incorporated in a CPD programme, implemented in western Sweden between 13 October 2016 and 23 May 2019, with 23 learning modules and 18 participants (male/female 3/15, median age 55 years). The participants' evaluation emphasised the importance of adult learning principles. Their responses to open reflective questions on 23 May 2109 suggested that the programme offered a learning environment in which they could develop their paediatric and educational practices and improve their mentorship, networks and work-based learning environment. They also described an enhanced feeling of joy at work., Conclusion: The involvement of experienced paediatricians in the programme formulation may be inspirational to clinicians and contribute to the definition, revitalisation and prioritisation of general paediatrics in Sweden in the future., (© 2021 The Authors. Acta Paediatrica published by John Wiley & Sons Ltd on behalf of Foundation Acta Paediatrica.)

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., Competing Interests: The authors declare that they have no competing interests., (© 2021 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group.)

Severe primary paediatric immunodeficiency syndromes are rare and potentially fatal unless suspected, diagnosed and treated early. We provide clinical guidance and support for on-call consultant paediatricians working in secondary level hospitals on how to recognise and manage children with these conditions. Our paper addresses four conditions that risk the most severe outcomes if they are not adequately cared for during on-call periods, such as weekends: severe combined immunodeficiency, haemophagocytic lymphohistiocytosis, severe congenital neutropaenia and chronic granulomatous disease. CONCLUSION: On-call paediatricians are provided with advice on handling the most severe primary immunodeficiencies., (©2019 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

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.

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a complex autoinflammatory disease with a clinical phenotype characterised by recurrent episodes of fever, systemic inflammation and symptoms and signs depicted in disease acronym. Although PFAPA is the most common autoinflammatory disease among children in many parts of the world, the condition is still an enigma, which include the regular episodes, the prompt responses to corticosteroids, the genetic bases for the familial clustering and therapeutic effects of tonsillectomy. This review explores PFAPA syndrome with the aim of describing the current clinical and scientific understanding of the condition., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

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.

Autoinflammatory disease was introduced as a concept in 1999, demarcating an entirely new group of diseases in clinical, immunological, and conceptual terms. During recent years, the preconditions for the definition of autoinflammatory conditions have changed. This includes the recent discovery of a number of monogenic autoinflammatory conditions with complex phenotypes that combine autoinflammation with defects of the adaptive and/or innate immune system, resulting in the occurrence of infection, autoimmunity, and/or uncontrolled hyperinflammation in addition to autoinflammation. Further, there are strong indications that classical IL-1-driven autoinflammatory diseases are associated with activation of adaptive immunity. As suggested by this development, we are of the opinion that an all-encompassing definition of autoinflammatory diseases should regard autoinflammatory conditions and innate dysregulation as inseparable and integral parts of the immune system as a whole. Hence, in this article, we try to advance the conceptual understanding of autoinflammatory disease by, proposing a modification of the definition by Daniel Kastner et al., which allows for a congruent and precise description of conditions that expand the immunological spectrum of autoinflammatory disease.

Unlabelled: There have been remarkable developments in the field of autoinflammatory diseases over the last 20 years. Research has led to definitions of new conditions, increased understanding of disease mechanisms and specific treatment. The polygenic autoinflammatory condition of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) is the most common autoinflammatory disorder among children in many parts of the world. The clinical features often include clockwork regularity of episodes, prompt responses to corticosteroids and therapeutic effects of tonsillectomy, but the disease mechanisms are largely unknown., Conclusion: This review discusses the emerging understanding of autoinflammatory diseases, with special emphasis on PFAPA., (©2016 The Authors. Acta Paediatrica published by John Wiley & Sons Ltd on behalf of Foundation Acta Paediatrica.)

Familial Mediterranean fever - an important disease in a globalised world Familial Mediterranean fever (FMF) is characterized by recurrent febrile attacks during 1/2-3 days associated with peritonitis, pleuritis and arthritis. FMF is the most common monogenic autoinflammatory disease in the world, with over 100 000 affected individuals. It is particularly common in individuals with an origin in the eastern Mediterranean Basin, where the disease has a prevalence of 100-200 per 100 000. The gene for FMF (MEFV) was identified in 1997 with an autosomal recessive inheritance; however, a significant proportion (≈25%) of clinical patients lack two mutations. MEFV codes for the protein pyrin, whose exact function still needs to be defined. The most serious complication of FMF is amyloid A amyloidosis, in particular renal amyloidosis. FMF is efficiently treated with daily doses of colchicine resulting in an almost normal life expectancy and amyloidosis confined to non-compliant patients. In today's globalized world we need to adapt to a new context that includes inherited conditions, which have historically been uncommon in our part of the world. One of these conditions is FMF, that should primarily be suspected in individuals with an origin in the eastern Mediterranean Basin and recurrent attacks of fever.

Objective: We aimed to investigate if aberrant intracellular production of NADPH oxidase-derived reactive oxygen species (ROS) in neutrophils is a disease mechanism in the autoinflammatory disease SAPHO syndrome, characterized by synovitis, acne, pustulosis, hyperostosis and osteitis, as has previously been suggested based on a family with SAPHO syndrome-like disease., Methods: Neutrophil function was explored in a cohort of four patients with SAPHO syndrome, two of whom were sampled during both inflammatory and non-inflammatory phase. Intracellular neutrophil ROS production was determined by luminol-amplified chemiluminescence in response to phorbol myristate acetate., Results: Cells from all patients produced normal amounts of ROS, both intra- and extracellularly, when compared with internal controls as well as with a large collection of healthy controls assayed in the laboratory over time (showing an extensive inter-personal variability in a normal population). Further, intracellular production of ROS increased during the inflammatory phase. Neutrophil activation markers were comparable between patients and controls., Conclusion: Dysfunctional generation of intracellular ROS in neutrophils is not a generalizable feature in SAPHO syndrome. Secondly, serum amyloid A appears to be a more sensitive inflammatory marker than CRP during improvement and relapses in SAPHO syndrome., (© The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology.)

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.

Aim: To define the clinical competencies currently required by on-call consultant paediatricians in Sweden and to describe how these competencies can be acquired by using an outcome-based continuing professional development (CPD) programme., Methods: The overall aims and objectives of the programme were formulated to meet the current responsibilities of an on-call consultant. It was delineated during a precourse needs assessment of 26 consultants, before being implemented with 16 participants, using a variety of learning, assessment and evaluation methods., Results: The precourse needs assessment identified 217 clinical situations and 17 themes, and 139 subthemes were developed. During the programme evaluation, carried out using a reflective questionnaire, participants stated that they had improved their understanding of the role of, and demands on, the on-call consultant. They had also felt more confident on-call, had built a network of colleagues and updated their knowledge in relevant fields., Conclusion: We defined and implemented a competency-based CPD programme to meet the changing demands faced by on-call consultant paediatricians. The aims and objectives of the programme were formulated according to a needs assessment among consultant paediatricians active in on-call services. Evaluation of the programme indicates that participants moved in the direction of the overall objectives., (©2013 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

Objective: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is an autoinflammatory disease of unknown etiology that primarily affects preschool-aged children. PFAPA syndrome is characterized by recurrent attacks of fever and symptoms of inflammation consistent with the disease acronym. Since autoinflammatory diseases are, by definition, mediated by cells of the innate immune system, the aim of this study was to evaluate the functional features of neutrophils, the most abundant innate immune cell in the circulation, in children with PFAPA syndrome., Methods: Blood polymorphonuclear leukocytes (PMNs), obtained from patients with PFAPA syndrome during both febrile and asymptomatic, afebrile phases of the disease, as well as from healthy children (afebrile controls) and children with fever and abdominal pain (febrile controls), were analyzed for 3 key neutrophil characteristics: 1) apoptosis (measured by annexin V/7-aminoactinomycin D staining), 2) production of reactive oxygen species (ROS) (measured by luminol/isoluminol-amplified chemiluminescence), and 3) priming status (measured as responsiveness to galectin-3 and up-regulation of CD11b)., Results: Compared to PMNs obtained from patients with PFAPA syndrome during an afebrile interval and those from febrile controls, PMNs obtained from patients during a PFAPA syndrome flare produced elevated levels of intracellular NADPH oxidase-derived ROS, had significantly diminished rates of spontaneous apoptosis, and displayed signatures of priming. In contrast, PMNs from afebrile patients with PFAPA syndrome had a significantly elevated rate of spontaneous apoptosis compared to PMNs from afebrile controls., Conclusion: These findings demonstrate that 3 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 children with PFAPA syndrome., (Copyright © 2013 by the American College of Rheumatology.)

The plight of children in many low-income countries continues to be reminiscent of conditions in 19th century Sweden. At the threshold of the 21st century, we would urge global cooperation in child health with the aim of realising a broader concept of health and development in low-income countries. This would enable the reduction of mortality to be accelerated and child health to be improved by targeting sick children for specific intervention, combined with preventive and health promotion measures.