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关键词： pSLE   outcome   damage   disease activity   epidemiology   Malaysian  

摘要： Background Paediatric systemic lupus erythematosus is a rare autoimmune disease with a wide spectrum of clinical presentation in different populations. We present a cohort of paediatric systemic lupus erythematosus in Malaysia where the disease features and outcomes are still largely unknown. Methods A retrospective review of all paediatric systemic lupus erythematosus patients with at least 6 months follow-up at Selayang Hospital from 2004 to 2016. Epidemiological, clinical and outcome data were collected and analysed. Results A total of 141 paediatric systemic lupus erythematosus patients, 87.9% females, were followed up for a median 6.3 years (interquartile range 3.6-9.0). The median age at diagnosis was 10.8 years (interquartile range 9.0-12.0 years), positive family history of systemic lupus erythematosus was present in 12.1% and the majority (61.7%) were of Malay ethnicity. Common presentations included fever (87.2%), vasculitic rash (72.3%) and lethargy (69.5%). At diagnosis, leukopenia (51.1%), thrombocytopenia (41.8%) and cutaneous lupus (56%) predominate with significant renal involvement (39.7%). Renal (45.4%), liver (26%) and the central nervous system (17%) were important major organs involved during the course of the disease. At diagnosis, almost all (99.3%) patients had high disease activity (mean Systemic Lupus Erythematosus Disease Activity Index score 20.1 +/- 9.6). The majority (62.4%) achieved remission or low disease activity after 6 months, maintained over the next 10 years. Damage occurred early (39.1% at 1 year) and increased with time. Ocular damage was the most common side effect (29%) and was predominantly corticosteroid related (93%). Growth retardation was significant (38.2%) with no gonadal failure or secondary malignancies. End-stage renal disease occurred in 3.1% patients whereas 53.1% had sustained renal remission. Overall mortality was 1.4%. Conclusion Despite high disease activity at diagnosis, the majority had good sustained respo

关键词： Adolescent   Childhood   Juvenile   Management   Paediatric   Paediatrician   Psoriasis   Therapy   Treatment  

摘要： Introduction Paediatric plaque psoriasis (PedPso) in children and adolescents is often diagnosed and treated for the first time by paediatricians. An early onset of psoriasis is associated with a genetic family burden, higher severity of disease and increased risk of comorbidities, sometimes starting in childhood. However, little information is available on prevalence data and the clinical management of PedPso by paediatricians. Methods A total of 191 questionnaires were sent out to paediatricians regarding their management of PedPso, with a focus on prevalence, diagnosis, initiation of therapies, screening for comorbidities and collaboration with dermatologists. Of these, 95 (49.7%) were returned and evaluated anonymously. Results Only about one-half of the responding paediatricians reported being certain in their diagnosis of PedPso, even though they regularly see moderate-to-severely affected patients. The questionnaire revealed that there are clear differences in the general management of PedPso if the paediatrician is not certain of the diagnosis of psoriasis. Compared to paediatricians certain of their diagnosis, those who are uncertain less frequently perform whole-body inspection, screen for relevant comorbidities, such as psoriasis arthritis, metabolic syndrome or mental disorders, and prescribe the use of topical or systemic therapies. No responding paediatrician reported the use of modern systemic therapies, such as biologicals, even in severely affected children. The majority of respondents rated their cooperation with dermatologists as good. Conclusion The certainty of the diagnosis, the use of system therapies and the screening for comorbidity could improve the care of PedPso through targeted training of paediatricians and intensified interdisciplinary cooperation with dermatologist.

摘要： We provide an update on the state of play with regards a newly described inflammatory condition which has arisen during the current SARS-CoV-2 pandemic. The condition has been named paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 or multisystem inflammatory syndrome in children. This condition has shown significant similarities to Kawasaki disease and toxic shock syndrome.

摘要： Paediatric morphoea is a debilitating fibrosing disorder of uncertain aetiology, affecting the skin and subcutaneous tissues. Defining optimum management strategies in paediatric morphoea remains an ongoing challenge, owing to the varied presentations and a relative paucity of paediatric-specific studies. We performed a literature search on PubMed, MEDLINE and Google Scholar, using keywords such as 'pediatric morphea', 'juvenile localised scleroderma' and 'juvenile systemic sclerosis'. Relevant studies, including randomized trials, reviews of standard current guidelines and original research articles, were selected and results analysed before summarizing them. In Part 1 of this review, we described the epidemiology, aetiopathogenesis and clinical classification;in this part, we discuss the diagnosis, markers of disease activity, management and natural history in paediatric morphoea.

摘要： LINKED CONTENT This article is linked to Mouratidou et al papers. To view these articles, visit https://***/10.1111/apt.15667 and https://***/10.1111/apt.15899.

关键词： anxiety   day surgery   distraction   meta-analysis   mobile health intervention   nursing   pain   parents   paediatric   systematic review  

摘要： Aims To evaluate the effectiveness of web-based mobile health interventions on paediatric patients and their parents in the day surgery context, where the primary outcome was children's pre-operative anxiety and secondary outcomes were postoperative pain and parents' anxiety and satisfaction with entire course of the day surgery. Design A systematic review and meta-analysis of randomized controlled trials. Data Sources CENTRAL, CINAHL, Scopus, Ovid MEDLINE, and Web of Science were systematically searched without time limits (up to December 2018). Review Methods Studies were appraised using the Cochrane risk of bias tool. A random effect meta-analysis of children's pre-operative anxiety was performed. Results Eight studies with a total of 722 patients were included in the analysis. The effectiveness of web-based mobile health interventions, including age-appropriate videos, web-based game apps, and educational preparation games made for the hospital environment, was examined in pre-operative settings. A meta-analysis (N = 560 children) based on six studies found a statistically significant reduction in pre-operative anxiety measured by the Modified Yale Pre-operative Anxiety Scale with a moderate effect size. Three studies reported parental satisfaction. Conclusion Web-based mobile health interventions can reduce children's pre-operative anxiety and increase parental satisfaction. Web-based mobile health interventions could be considered as non-pharmacological distraction tools for children in nursing. There is not enough evidence regarding the effectiveness of reducing children's postoperative pain and parental anxiety using similar interventions. Impact Web-based mobile health interventions reduce children ' s pre-operative anxiety and could therefore be considered as non-pharmacological distraction tools for children in nursing.

关键词： Adherence   Easypod (TM)   Growth hormone deficiency   Paediatric patients   Small for gestational age   Turner syndrome  

摘要： Background: The easypod (TM) injection device allows automatic recording and transmission of adherence data from patients receiving recombinant human growth hormone (rhGH [Saizen (R)]) to treat growth disorders. This analysis aimed to evaluate the adherence of Saizen (R) administered via easypod (TM) in a cohort of Greek patients from the easypod (TM) connect observational study (ECOS). Methods: The phase IV, open-label, multicentre, observational, and longitudinal ECOS study (EMR200104-520, NCT01363674) enrolled patients treated for a minimum of 6 months and up to 3 years. The primary endpoint was to assess the mean rate of adherence to treatment at different time points, where good adherence was defined as 85%. Change in height, height standard deviation score (SDS), height velocity and height velocity SDS were evaluated after 1 year of treatment as secondary endpoints, together with the impact of adherence on growth outcomes using the Spearman's product moment. Results: Of the 180 patients enrolled, 86 were included in the analysis. The mean adherence to Saizen (R), as recorded via easypoe (TM), was high at each individual time point, and was maintained at 95.5% after 1 year of treatment. Clinically meaningful positive changes were also noted for all of the secondary endpoints (median increase in height = 7.25 cm, height SDS = 0.32, median height velocity = 7.62 cm/year and height velocity SDS = 1.65). However, no significant correlation was noted between adherence and growth outcomes. Conclusions: rhGH replacement therapy using Saizen (R) with easypod (TM) led to full compliance to the treatment in a representative Greek population from ECOS, and provided additional insights on how the easypod (TM) device can assist physicians in monitoring adherence and help to optimise linear growth in paediatric patients with growth disorders.

关键词： Cardiomyopathy   Mitochondria   Paediatrics   Heart failure  

摘要： Background Primary mitochondrial disorders (PMD) are rare conditions resulting in progressive multi-organ failure. Cardiovascular involvement (CVI) has been reported in paediatric patients. However, its age-related prevalence, clinical presentation and prognostic impact are unresolved. We detailed CVI in a cohort of children diagnosed with PMD over two decades at a tertiary referral centre. Results We enrolled 86 PMD patients (M/F = 30/56;mean age 6.4 +/- 8.58 years). CVI was detected in 31 patients (36%), with mean age at onset of 5.7 +/- 7.8 years including the pre- and neonatal phase in 14, often representing the first sign of PMD (42% of those with CVI). Heart disease resulted more common in males and in children with specific aetiologies (Barth, TMEM70 and MELAS syndromes). Hypertrophic, non-compaction and dilated cardiomyopathies were the prevalent disorders, although pulmonary arterial hypertension was also found. Adverse cardiac events (heart failure, resuscitated cardiac arrest, ICD/PM implantation, sudden death) occurred in 19% of children with CVI over a follow-up period of 5.4 +/- 4.3 years. All-cause mortality was higher in patients with CVI compared to those without CVI (45.1% vs 21.8%;p < 0.01);female sex, age at onset < 5 years, acute heart failure at presentation and diabetes also proved independent predictors of outcome. Conclusion Cardiovascular involvement occurred in over one-third of children diagnosed with PMD, often at a very early age, and was associated with adverse prognosis. Final outcome of PMD-related CVI was influenced by the specific underlying aetiology, suggesting the need for tailored management of heart failure and sudden death prevention.

关键词： Active appearance model   automatic modelling   finite element analysis   patient-specific model   unilateral crossbite  

摘要： The evaluation of temporomandibular joint (TMJ) dysfunction using finite element models is a time consuming process that requires extensive technical knowledge. We combined a statistical active appearance model with automated modelling algorithms to biomechanically study the relationship between TMJ malformations and dysfunction in radiographs from 20 paediatric patients with unilateral crossbite. A fitting algorithm (fitting error < 4%) recognised the TMJ shape and adjusted the dimensions of each patient-specific 2D FE model, which was then used to compute 2 different joint movements. Significant functional differences were observed between the crossbite and non-cross bite sides, and the shape-function relation was verified.

关键词： Probiotics   Paediatrics   Necrotizing enterocolitis   Acute infectious diarrhoea   Allergy   Functional gastrointestinal disorders  

摘要： During the last decade several paediatric studies have been published with different possible indications for probiotics, leading to a global increase of probiotics' market. Nevertheless, different study designs, multiple single/combined strains and small sample size still leave many uncertainties regarding their efficacy. In addition, different regulatory and quality control issues make still very difficult the interpretation of the clinical data. The objective of this review is to critically summarise the current evidence on probiotics' efficacy and safety on a different number of pathologies, including necrotizing enterocolitis, acute infectious diarrhoea, allergic diseases and functional gastrointestinal disorders in order to guide paediatric healthcare professionals on using evidence-based probiotics' strains. To identify relevant data, literature searches were performed including Medline-PubMed, the Cochrane Library and EMBASE databases. Considering probiotics strain-specific effects, the main focus was on individual probiotic strains and not on probiotics in general.