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Objective To determine the frequency of incident vertebral fractures (IVF) 12 months after glucocorticoid (GC) initiation in children with rheumatic diseases and to identify children at higher risk. Methods Children with rheumatic diseases initiating GC were enrolled in a prospective observational study. Annual spine radiographs were evaluated using the Genant semiquantitative method. Spine areal bone mineral density (aBMD) was measured every 6 months. Clinical features, including cumulative GC dose, back pain, disease and physical activity, calcium and vitamin D intake, and spine aBMD Z scores, were analyzed for association with IVF. Results Seven (6%) of 118 children (95% confidence interval 2.9-11.7%) had IVF. Their diagnoses were: juvenile dermatomyositis (n = 2), systemic lupus erythematosus (n = 3), systemic vasculitis (n = 1), and mixed connective tissue disease (n = 1). One child was omitted from the analyses after 4 months because of osteoporosis treatment for symptomatic IVF. Children with IVF received on average 50% more GC than those without ( P = 0.030), had a greater increase in body mass index (BMI) at 6 months ( P = 0.010), and had greater decrements in spine aBMD Z scores in the first 6 months ( P = 0.048). Four (67%) of 6 children with IVF and data to 12 months had spine aBMD Z scores less than −2.0 at 12 months compared to 16% of children without IVF ( P = 0.011). Conclusion The incidence of VF 12 months following GC initiation was 6%; most children were asymptomatic. Children with IVF received more GC, had greater increases in BMI, and had greater declines in spine aBMD Z scores in the first 6 months. [ABSTRACT FROM AUTHOR]

A 4‐month‐old infant was declared brain‐dead 2 days after being initiated on venoarterial ECMO for a refractory septic shock. All brain death diagnostic criteria were fulfilled according to French law, and parental consent was given for organ donation. The hospital where ECMO was initiated had no authorization for organ procurement, and the donor was then transferred to the local referral center for child organ recovery with our mobile ECMO team to maintain organ perfusion. The kidneys were recovered and successfully transplanted to a child who is now well and alive. Although the transport elements of this case report are of limited relevance to an international audience as no other country, to our knowledge, has this particular organization, it does show excellent collaboration between teams to realize the goal of organ donation for this family. This is the first case describing a successful inter‐hospital transport for organ procurement of a brain‐dead infant on ECMO. Brain‐dead pediatric patients undergoing ECMO can be considered as potential organ donors to expand the donor pool. [ABSTRACT FROM AUTHOR]

Background/Purpose: To determine the incidence of vertebral fracture (VF) in the three years following glucocorticoid (GC) initiation, and the effect of GC on the risk of incident VF in children with rheumatic disorders (RD). Methods: Children with RD were enrolled within 30 days of GC initiation and followed for 3 years, with VF assessed at baseline and then annually using the Genant method on lateral spine radiographs. An incident VF was defined as a new fracture in a previously normal vertebral body or worsening of an existing VF. The annual and the 3 year cumulative incidences were calculated as the number of new cases divided by the number of subjects who completed VF assessment at the specified time periods. Six different time-dependent GC exposure measures were constructed: cumulative dose, average daily dose, duration on GC therapy, dose intensity (cumulative dose divided by duration on GC therapy) over 3 years, recent average daily dose and duration on GC therapy over the preceding 12 months. Extended Cox's models were used with alternative time-dependent variables, adjusting for factors at baseline including age, gender, body mass index, vitamin D and calcium supplementation, disease activity, bone mineral density, physical activity, and presence of prevalent VF. Results: 136 enrolled children (mean ± SD age 9.9 ± 4.4 years, 65% girls) held the following diagnoses: 22% juvenile dermatomyositis (JDM), 21% non-systemic juvenile idiopathic arthritis (JIA), 19% systemic lupus erythematosus (SLE), 18% systemic JIA, 12% systemic vasculitis, and 9% other RD. 9 children had VF at baseline (3 JDM, 2 systemic JIA, 2 SLE, 1 systemic vasculitis, 1 localized scleroderma), with an estimated prevalence of 6.6% (95% confidence interval (CI) 2.4-10.8%). 18 incident VF were identified in 15 children during the 3 years following GC initiation (6 JDM, 4 SLE, 2 systemic vasculitis, 2 systemic JIA, and 1 non-systemic JIA). The 3-year cumulative VF incidence was 13.0%, at a rate of 4.9 per 100 person-years. Of the 18 incident VF events, 7 occurred during the first year at an annual incidence of 6.0% (95% CI 1.7-10.3%); 6 VF occurred during the second year (annual incidence 5.6%; 95% CI 1.2-9.9%); 5 VF occurred during the third year (annual incidence 4.3%; 95% CI 1.0-8.1%). 64% of patients had mild incident VF as the worst grade, 36% had moderate fractures, and none had severe fractures. Of those with incident fractures, 21% had no complaints of back pain in the preceding 12 months. Multivariable models showed that every 100 mg/kg increase in cumulative GC dose was associated with a 30% increased incident VF risk (hazard ratio (HR) = 1.3, 95% CI 1.1, 1.6). Every 0.5mg/kg increase in average daily dose over the 3 year period was associated with a 4.2 fold increased VF risk (HR = 4.2, 95% CI 2.3, 7.7), and every 0.5 mg/kg increase in daily dose during the preceding 12 months was associated with a 2.7 fold increased incident VF risk (HR =2.7, 95% CI 1.3, 5.2). Conclusion: We have shown that 13.0% of children with RD sustained VF within three years followingGC initiation, of which 36% had moderate fractures. Cumulative dose, average daily GC dose, and recent average daily GC dose were associated with a significantly increased VF risk. Funded by CIHR FRN 64285 [ABSTRACT FROM AUTHOR]

Background/Purpose: Juvenile idiopathic arthritis (JIA) is the most common cause of chronic arthritis in childhood. Intra-articular corticosteroids injections (IAS) are a safe and effective therapeutic option for affected children. The response of the ankle joint to IAS is thought to be less favourable than other joints. To date, little evidence is available to support this observation and there is insufficient research evaluating the response of the ankle compared to other joints of the lower extremities. Methods: JIA patients 0-18 years old followed at the Montreal Children's Hospital undergoing IAS (blindly or with fluoroscopy) in the lower extremities from January 1st 2005 to December 30th 2012 were included in a retrospective cohort study. Data obtained through chart review encompassed demographics, IAS method, and response to IAS. Response was evaluated at 6 weeks, 3 months, 6 months, 12 months and 24 months post-procedure. Patients without available data for at least 3 months following intervention were excluded. Non-response to IAS was determined within the first 3 months after IAS. Time to flare was assessed in subsequent visits up to 24 months. Results: Data from 70 patients included in our study are presented. Median age at diagnosis was 4.7 years with 64% affected with oligoarthritis and 69% of female gender. Median age at the time of IAS was 7.8 years. Triamcinolone hexacetonide was administered in 98% of cases. IAS were performed in 97 knees, 50 ankles and 20 subtalar joints. At 3 months post IAS, 34.0% of ankles, 15.0% of subtalar joints and 11.3% of knees failed to demonstrate a clinical response. The median time to flare after IAS was 7.7 months for the ankles, 7.8 months for the subtalar joints and 8.1 months for the knees. Only two patients developed a complication (subcutaneous atrophy). Conclusion: To date, we report a higher rate of non-response following blind IAS ankle injections compared to other lower extremities joints. Future analysis of the entire cohort will assess the factors associated with a non-response to IAS as well as the factors associated with a longer duration of remission. Our findings may reflect inaccurate needle placement and/or non-treatment of associated tenosynovitis and suggest the need to modify the approach to IAS in the ankles by performing ultrasound-guided injections to improve outcomes. [ABSTRACT FROM AUTHOR]