Janus kinase inhibition in induction treatment of anti-MDA5 juvenile dermatomyositis-associated rapidly progressive interstitial lung disease.

Tofacitinib has an important role in pediatric rapidly progressive interstitial lung disease (ILD) associated with juvenile dermatomyositis (JDM), an otherwise potentially fatal condition. It may be useful in induction of remission and can be used safely to maintain remission. Serum ferritin and interleukin-18 are useful markers for tracking activity and response of JDM-associated ILD.

Intra-articular glucocorticoid injections in patients with juvenile idiopathic arthritis in a Singapore hospital.

INTRODUCTION: This study aimed to evaluate the efficacy and safety of intra-articular glucocorticoid (IAG) injections in our institution in children with juvenile idiopathic arthritis (JIA). METHODS: This is a retrospective assessment of IAG injections performed by the Department of Paediatrics, National University Hospital, Singapore, from October 2009 to October 2011. A total of 26 procedures were evaluated for efficacy, considering parameters such as clinical response, changes in systemic medication, length of time between repeat injections, safety, consent-taking, pre- and post-procedural advice, compliance with aseptic technique, and post-procedural complications. RESULTS: A total of 26 IAG injections of triamcinolone hexacetonide were administered over 17 occasions (i.e. patient encounters) to ten patients with JIA during the study period. After the injections, clinical scoring by a paediatric rheumatologist showed overall improvement by an average of 2.62 points out of 15. Besides six patient encounters that had an increase in systemic medication on the day of the injection, five required an increase within six months post injection, two required no adjustments, and one resulted in a decrease in medications. In all, 21 injections did not require subsequent injections. The mean interval between repeat injections was 7.8 months. Cutaneous side effects were noted in three anatomically difficult joints. Medical documentation with regard to patient progress was found to be lacking. CONCLUSION: As per the recommendations of the American College of Rheumatology, we safely used IAG injections as the first-line therapy in our group of patients with oligoarticular JIA, and/or as an adjunct to systemic therapy in our patients with JIA.

Specialist pediatric dialysis nursing improves outcomes in children on chronic peritoneal dialysis.

Chronic peritoneal dialysis (PD) for children in Singapore was instituted in 1988 at the National University Hospital with adult nurses providing dialysis services during the first 10 years. In 1998, a specialist pediatric dialysis nursing team was recruited. This study was conducted to determine the impact of dialysis nursing service on PD-related outcomes during the two nursing periods. Comparing the adult (group 1) and pediatric (group 2) nursing periods, the peritonitis rate was significantly higher in group 1 (RR 1.90; 95%CI 1.27-2.84), and this association did not weaken after adjusting for age, gender, and exit site infections. Exit site infection rate (RR 2.16; 95%CI 1.44-3.23), risk of peritonitis during the first year (RR 3.65; 95%CI 1.68-7.90), and multiple peritonitis attacks (RR 2.45; 95%CI 1.32-4.55) were higher in group 1. The peritonitis rates for adult patients cared for by the same adult nurses declined sharply from 1.05 episodes per patient-year between 1989 and 1992 to 0.41 episodes per patient-year between 1995 and 1997, however the corresponding pediatric rates did not change (1.48 to 1.06 episodes per patient-year, respectively) until the second era when specialized pediatric nurses were available. In conclusion, establishment of a specialist pediatric dialysis nursing team resulted in significant improvement in infection-related PD outcomes.

Simulating inadequate dialysis and its correction using an individualized patient-derived nomogram.

Computerized kinetic modeling is a valuable automated peritoneal dialysis (APD) prescription tool for optimizing dialysis adequacy. However, non-compliance results in failure to achieve adequacy targets. The aim of this study was to determine if a nomogram could estimate dialysis compensations for shortfalls in simulated non-compliant patients, such that total weekly urea clearance (Kt/V(urea)) targets are met. Individualized nomograms comprising a series of curves were derived from PD Adequest (ver. 2.0)-predicted Kt/V(urea) data (r (2 ) > 0.99) for different APD prescriptions. The nomogram was then used to estimate the (Nomogram-computed) average of the daily Kt/V(urea) in 14 patients. The study comprised three 1-month phases. Patients were compliant to dialysis in phase I, where Adequest-predicted Kt/V(urea) showed good agreement with both measured (r (I) = 0.72), and Nomogram-computed values (r (I) > 0.99) (p < 0.001). Conversely, in non-compliant phase II, Nomogram-computed values were lower than Adequest-predicted values (p < 0.002). In phase III, the nomogram estimated prescription adjustments required to compensate for shortfalls, such that there was significantly less difference between Nomogram-computed and Adequest-predicted Kt/V(urea) than in phase II (p = 0.005). Thus, despite non-compliance, predicted Kt/V(urea) targets were attained using the nomogram to adjust the daily APD prescriptions. This concept is potentially useful for patients desiring to compensate for inadvertent shortfalls, rather than for 'truly non-compliant' patients.