The outcomes of children with severe proliferative lupus nephritis (LN) were examined using a new mycophenolate and cyclosporine-based (MMF-CSA) induction protocol. Sixteen children with LN (WHO class III and IV), 31.3% of whom required dialysis at induction, were retrospectively studied. Median MMF dose was 942 mg/m( 2)/day. Thirteen patients (81%) with persistent proteinuria received CSA. Clinical and laboratory parameters were compared at pre-induction, 6 and 12 months. Treatment outcome was defined by Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), renal function, haematuria, proteinuria and serological markers (complements C3, C4 and anti-dsDNA). Comparing these parameters at induction, 6 months and 12 months, respectively, SLEDAI (25.4 +/- 8.7 versus 3.2 +/- 2.9 versus 2.9 +/- 2.8), serum C3 (47 +/- 21 versus 107 +/- 27 versus 111 +/- 38 mg/dl), C4 (12 +/- 14 versus 23 +/- 14 versus 22 +/- 11 mg/dl) and urine protein (6.97 +/- 7.09 versus 0.98 +/- 1.56 versus 0.21 +/- 0.13 g/ day/1. 73 m(2)) improved significantly (p < 0.05). Anti-dsDNA titres decreased in 73% by 6 and 12 months (p < 0.05). Complete renal remission was achieved in 7/16 (43.8%) at 6 months and 12/16 (75%) at 12 months, the rest achieving partial remission with no treatment failures. In conclusion, a combination MMF-CSA protocol is an effective therapeutic alternative for induction of children with severe proliferative LN, resulting in significant clinical and serological improvement with minimal adverse effects.

We have previously reported the one-year outcomes of 16 children with severe proliferative lupus nephritis (LN) who were treated using a multi-targeted induction protocol based on intravenous (IV) pulse methylprednisolone (MP), mycophenolate mofetil (MMF) and cyclosporine (CSA). This study examined the long-term renal outcomes of these 16 children, followed up for a median duration of 9.2 years (range 5.8-14.2 years). Primary treatment outcome was complete renal remission. Secondary outcomes included patient and renal survival as well as relapse-free and event-free survival. All patients achieved complete renal remission within 24 months (median 8.7 months, range 4.0-24.0 months). Comparing clinical and laboratory parameters at induction and last follow-up, respectively, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score (25.4 ± 8.7 vs. 0.4 ± 0.8), serum complement C3 (47 ± 21 vs. 107 ± 27 mg/dL), estimated glomerular filtration rate (eGFR) (72 ± 57 vs. 109.7 ± 43 ml/min/1.73 m2) and urine protein (6.97 ± 7.09 vs. 0.2 ± 0.02 g/day/1.73 m2) improved significantly (p < 0.05). Kaplan-Meier survival analysis showed a cumulative ten-year renal relapse-free survival of 73.3% when considering relapses with severe proteinuria >1 g/day/1.73 m2. Cumulative probability that hospitalization would not be required was 93.8% at one year, and 71.4% at ten years. Our multi-targeted protocol for induction and maintenance therapy in Asian children with severe proliferative LN resulted in good long-term patient survival and renal preservation, with a good safety profile.

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.

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.

Bullous eruptions are rare cutaneous manifestations of systemic lupus erythematosus. We report a case of an 8-year old Filipino girl with vesiculobullous systemic lupus erythematosus (SLE) and membranous lupus nephritis on kidney biopsy who presented with clinical nephrotic features of generalized edema, proteinuria, hypoalbuminemia and hyperlipidemia. The 2012 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria for SLE were met. Immunohistopathologic examination of the skin lesion revealed a sub-epidermal split with neutrophilic infiltrates along the dermo-epidermal junction, moderate perivascular, periadnexal and interstitial infiltrates composed of predominantly neutrophils with neutrophilic dusts, lymphocytes, plasma cells, rare eosinophils and increased dermal mucin. Direct immunofluorescence showed strong continuous linear IgG deposits along the basement membrane and weak linear IgM and IgA deposition along the basement membrane zone (BMZ). To our knowledge, this is the first report of vesiculobullous SLE in a Filipino child. This case is a rare form of cutaneous lupus in children. Bullous SLE (BSLE) should be considered in the differential diagnosis of children presenting with generalized bullous eruptions.