The national incidence and clinical picture of SLE in children in Australia - a report from the Australian Paediatric Surveillance Unit.

OBJECTIVES: The objectives of this paper are to prospectively determine the incidence of paediatric systemic lupus erythematosus (pSLE) in Australia as well as describe the demographics, clinical presentation and one-year outcome. STUDY DESIGN: Newly diagnosed cases of pSLE were ascertained prospectively from October 2009 to October 2011 through the Australian Paediatric Surveillance Unit (a national monthly surveillance scheme for notification of childhood rare diseases) as well as national subspecialty groups. Questionnaires were sent to notifying physicians at presentation and at one year. RESULTS: The annual incidence rate was 0.32 per 10(5) children aged less than 16 years. The incidence was significantly higher in children of Asian or Australian Aboriginal and Torres Strait Islander parents. Approximately one-third of children underwent a renal biopsy at presentation and 7% required dialysis initially although only one child had end-stage kidney disease (ESKD) at one-year follow-up. CONCLUSION: The incidence of pSLE in Australia is comparable to that worldwide with a significantly higher incidence seen in children of Asian and Australian Aboriginal and Torres Strait Islander backgrounds. Renal involvement is common but progression to ESKD, at least in the short term, is rare.

Pilot study of fitness training and exercise testing in polyarticular childhood arthritis.

OBJECTIVE: To 1) assess the safety and feasibility of laboratory-based exercise testing in juvenile idiopathic arthritis (JIA), 2) test the safety and feasibility of a 3-month exercise program in JIA, 3) assess pain during exercise in JIA, 4) compare ratings of perceived effort (RPE) with heart rate (HR) achieved, and 5) estimate the training effect on metabolic efficiency of gait as measured by submaximal exercise testing. METHODS: Nine children with JIA were enrolled in a 12-week circuit training program involving pool, stationary bicycle, treadmill, and Fitball. They underwent formal exercise testing before and after the program, underwent a full joint assessment, were administered the Childhood Health Assessment Questionnaire and Juvenile Arthritis Functional Status Index, and were assessed for overall quality of life and health-related quality of life. A visual analog scale was used to assess pain during testing and training, and the Borg scale was used to measure RPE. RESULTS: Children with JIA were able to participate in exercise testing without any significant problems. Children with severe hip disease dropped out of the exercise program due to pain during the exercise sessions and worsened arthritis symptoms. Target HR was achieved and correlated with RPE in the bicycle and treadmill sessions. Submaximal exercise testing showed an improvement with a small to moderate effect size. CONCLUSION: This study suggests that it is safe, feasible, and acceptable for children with arthritis, in the absence of severe hip involvement, to participate in formal exercise testing and structured fitness programs.

Predictors of disease course and remission in systemic juvenile idiopathic arthritis: significance of early clinical and laboratory features.

OBJECTIVE: To determine whether the disease course in systemic juvenile idiopathic arthritis (JIA) can be characterized as monophasic, polycyclic, or persistent, and to determine whether early clinical and laboratory characteristics can be used to predict the disease course and time to remission. METHODS: Forty-five children with systemic JIA diagnosed between 1996 and 2000 were followed up with a standardized data collection protocol, including data on clinical and laboratory features (mean followup 4.9 years). Disease was considered inactive if the clinical and laboratory features were normal. Three definitions of remission were applied to classify disease course. Predictors of disease course were evaluated using multiple logistic regression. Predictors of time to remission were evaluated using Cox proportional hazards regression. RESULTS: When applying a definition of remission requiring inactive disease while not receiving any medications for a period of 3 months, 42.2%, 6.7%, and 51.1% of the patients were classified as having monophasic, polycyclic, and persistent disease, respectively. Fever and active arthritis at 3 months (R2 = 0.42, area under the receiver operating characteristics curve [AUC] = 0.76) and an erythrocyte sedimentation rate (ESR) >26 mm/hour and corticosteroid use at 6 months (R2 = 0.49, AUC = 0.92) were predictive of a nonmonophasic course. Absence of active arthritis, an ESR of <26 mm/hour, and no requirement for corticosteroid therapy at 3 and 6 months were predictors of an earlier time to remission. CONCLUSION: The disease course in systemic JIA can be characterized as monophasic, polycyclic, or persistent using a definition of remission requiring 3 months of inactive disease while not receiving any therapy. Features at 3 and 6 months are predictive of the disease course and time to remission.

A prospective study of the immediate and delayed adverse events following intravenous immunoglobulin infusions.

AIM: To document the incidence of immediate and delayed adverse events (AE) following intravenous immunoglobulin (IVIG) infusion in children. METHODS: Immediate and delayed adverse events were prospectively recorded for 345 infusions in 58 children receiving IVIG for immunodeficiency (n = 33) or immunomodulation (n = 25). For each infusion adverse events were documented during the infusion and by follow up interview 4-7 days later. RESULTS: Immediate adverse events occurred in 10.3% and delayed adverse events in 41.4% of children treated during the study period. Three and a half per cent of the infusions were associated with immediate AE and 20.9% with delayed adverse events. Headache was the most common delayed AE, occurring in 24.1% of patients and 12.8% of infusions. CONCLUSIONS: Delayed adverse events to IVIG infusions are common in children. They occur more frequently than immediate adverse events and are the cause of significant morbidity. Recognition of the high frequency of delayed adverse events is important in the care of children receiving IVIG therapy.

Circumcision for the prevention of urinary tract infection in boys: a systematic review of randomised trials and observational studies.

OBJECTIVE: To undertake a meta-analysis of published data on the effect of circumcision on the risk of urinary tract infection (UTI) in boys. DATA SOURCES: Randomised controlled trials and observational studies comparing the frequency of UTI in circumcised and uncircumcised boys were identified from the Cochrane controlled trials register, MEDLINE, EMBASE, reference lists of retrieved articles, and contact with known investigators. METHODS: Two of the authors independently assessed study quality using the guidelines provided by the MOOSE statement for quality of observational studies. A random effects model was used to estimate a summary odds ratio (OR) with 95% confidence intervals (CI). RESULTS: Data on 402,908 children were identified from 12 studies (one randomised controlled trial, four cohort studies, and seven case-control studies). Circumcision was associated with a significantly reduced risk of UTI (OR = 0.13; 95% CI, 0.08 to 0.20; p<0.001) with the same odds ratio (0.13) for all three types of study design. CONCLUSIONS: Circumcision reduces the risk of UTI. Given a risk in normal boys of about 1%, the number-needed-to-treat to prevent one UTI is 111. In boys with recurrent UTI or high grade vesicoureteric reflux, the risk of UTI recurrence is 10% and 30% and the numbers-needed-to-treat are 11 and 4, respectively. Haemorrhage and infection are the commonest complications of circumcision, occurring at rate of about 2%. Assuming equal utility of benefits and harms, net clinical benefit is likely only in boys at high risk of UTI.

Cerebral ptosis revisited.

We studied nine patients with cerebral ptosis and gaze deviation following an acute stroke. The brains of all nine showed an extensive lesion in the fronto-parieto-temporal regions. In five the stroke was left hemispheric. The assumption is that cerebral ptosis may have a bihemispheric representation and results from damage to the connections rather than to a specific area.