Diabetic ketoacidosis

Diabetic ketoacidosis (DKA) is the most common acute hyperglycaemic emergency in people with diabetes mellitus. A diagnosis of DKA is confirmed when all of the three criteria are present — ‘D’, either elevated blood glucose levels or a family history of diabetes mellitus; ‘K’, the presence of high urinary or blood ketoacids; and ‘A’, a high anion gap metabolic acidosis. Early diagnosis and management are paramount to improve patient outcomes. The mainstays of treatment include restoration of circulating volume, insulin therapy, electrolyte replacement and treatment of any underlying precipitating event. Without optimal treatment, DKA remains a condition with appreciable, although largely preventable, morbidity and mortality. In this Primer, we discuss the epidemiology, pathogenesis, risk factors and diagnosis of DKA and provide practical recommendations for the management of DKA in adults and children

Lost bones: differential diagnosis of acro-osteolysis seen by the pediatric rheumatologist

Abstract Introduction Acro-osteolysis is a radiographic finding which refers to bone resorption of the distal phalanges. Acro-osteolysis is associated with various conditions and its presence should prompt the clinician to search for the underlying etiology. The aim of this review is to discuss disorders with which acro-osteolysis is associated and their distinguishing features, with a focus on the pediatric population. Methods A targeted literature review was performed using the term “acro-osteolysis” in combination with other key terms. The primary search results were supplemented using reference citations. Articles published prior to the year 2000 were included if they described additional associations not encountered in the more recent literature. Results Genetic disorders (particularly primary hypertrophic osteoarthropathy and skeletal dysplasias) and rheumatic diseases (particularly psoriatic arthritis and systemic sclerosis) are the most frequently encountered conditions associated with acro-osteolysis in children. Hyperparathyroidism, neuropathy, local trauma and thermal injury, and spinal dysraphism should also be included in the differential diagnosis. Conclusion Although acro-osteolysis is uncommon, its presence should prompt the clinician to consider a differential diagnosis based on clinical and radiographic features

Proposed core set of items for measuring disease activity in systemic juvenile idiopathic arthritis

Objective.To date, there are no standardized disease activity tools for systemic juvenile idiopathic arthritis (sJIA). We developed a core set of disease activity measures for sJIA.Methods.We conducted a validation study in patients with sJIA recruited from 3 Canadian institutions. Disease activity scores were based on questionnaires, clinical factors, and laboratory measures. The physician’s global assessment was our criterion standard. We determined the strength of association of each item with the criterion standard. We then surveyed international experts to determine the top 10 items. Finally, we used the experts’ responses to generate a proposed core set of disease activity measures.Results.We enrolled 57 subjects — 26 with moderately or severely active disease, and 31 with mildly active or inactive disease. Items that most strongly correlated with the criterion standard were number of active joints (r = 0.79), parent’s global assessment of disease activity (r = 0.53), erythrocyte sedimentation rate (ESR; r = 0.62), and C-reactive protein (CRP; r = 0.61). The response rate from international experts was 82% (154/187). Items with the most votes, in descending order, were number of active joints, number of days with fever in the preceding 2 weeks, patient’s and parent’s global assessments of disease activity, sJIA rash, ESR, CRP, and hemoglobin level.Conclusion.We propose a core set of items for measuring disease activity in sJIA. Future research should be aimed at further validation of this core set in the international context.</jats:sec

Hallmark trials in ANCA-associated vasculitis (AAV) for the pediatric rheumatologist

Abstract Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) refers to a complex group of systemic vasculitides that are characterized by primary small-to-medium sized blood vessel inflammation with the presence of autoantibodies known as ANCA. AAV diseases include Granulomatosis with Polyangiitis (GPA), Eosinophilic Granulomatosis with Polyangiitis (EGPA), and Microscopic Polyangiitis (MPA). AAVs are challenging conditions associated with high cumulative disease and treatment related morbidity and mortality. Given its rarity and the resulting paucity of pediatric-specific clinical trial evidence, pediatric rheumatologists have had to often extrapolate from adult literature for management and therapeutic decisions. The aim of this review is to provide a comprehensive overview of the important findings and overall conclusions of critical landmark clinical trials in the induction and maintenance treatments in adult AAV for the pediatric rheumatologist. This review also highlights the outcomes of recent pediatric AAV observational studies and discusses the future research priorities in pediatric AAV management

Internet Program for Physical Activity and Exercise Capacity in Children With Juvenile Idiopathic Arthritis: A Multicenter Randomized Controlled Trial

Objective. To determine the effects of Rheumates@Work, an internet-based program supplemented with 4 group sessions, aimed at improving physical activity, exercise capacity, health-related quality of life (HRQoL), and participation in children with juvenile idiopathic arthritis. Methods. Patients were recruited from 3 pediatric rheumatology centers in The Netherlands for an observer-blinded, randomized controlled multicenter trial. Physical activity level, time spent in rest, light, and moderate-to-vigorous physical activity (MVPA) were recorded in a diary and with an accelerometer, before intervention, after intervention, and at followup after 3 and 12 months (intervention group only). Exercise capacity was assessed using the Bruce treadmill protocol, HRQoL was assessed with the Pediatric Quality of Life Inventory generic core scale, and participation in school and in physical education classes were assessed by questionnaire. Results. The intervention group consisted of 28 children, and there were 21 children in the control group. MVPA, exercise capacity, and participating in school and physical education classes improved significantly in the intervention group. HRQoL improved in the control group. No significant differences were found between groups. The effect of Rheumates@Work on physical activity and exercise capacity lasted during the 12 months of followup. Improvements in physical activity were significantly better for the cohort starting in winter compared to the summer cohort. Conclusion. Rheumates@Work had a positive, albeit small, effect on physical activity, exercise capacity, and participation in school and physical education class in the intervention group. Improvements lasted for 12 months. Participants who started in winter showed the most improvement. Rheumates@Work had no effect on HRQoL