This retrospective study investigated the outcome of 27 children (19 male) with Henoch-Schönlein purpura nephritis (HSN) of International Study of Kidney Disease in Children (ISKDC) grade 3b or higher treated with long-term immunosuppressive therapy in a single centre over a 10-year period. The mean age at presentation was 9.7 years. The median estimated glomerular filtration rate (eGFR) was 91.3 ml/min per 1.73 m(2), with the median urine protein creatinine ratio (UP:UC) 556 mg/mmol. The treatment protocol comprised daily steroids and cyclophosphamide for 8-12 weeks followed by azathioprine and a reducing regimen of alternate-day steroids for 8-12 months. After a mean follow-up period of 7 years following presentation, 37% made a complete recovery, 40.7% had persistent proteinuria, 7.4% had persistent proteinuria and were on antihypertensive therapy and 14.8% had progressed to end-stage kidney failure (ESKF). Children with poor outcome were older at presentation (p 0.005), had more crescents (p 0.015) and had heavier proteinuria 6 months post initial biopsy (p 0.023). All of the four children with ESKF had nephrotic range proteinuria and greater than 50% crescents on initial biopsy. Despite long-term immunosuppression, the majority of children with HSN grade 3b or higher will have persistent renal abnormalities on long-term follow-up.

The clinical phenotypes of specific complement immunodeficiencies vary considerably both in terms of the predominant bacterial pathogen, and the risk and type of auto-inflammatory disease. Appreciation of these phenotypic differences should help both immunologists and other specialists in their diagnosis and management of these rare and complex patients.

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The reliability of urea kinetic modelling (UKM) in paediatric haemodialysis was tested by comparing results of the classic variable volume model (UKM3), a recently introduced two-sample modification of this (UKM2) and direct quantification by a partial dialysate collection method (PDC). Urea generation rate (G) was also found from a 1-week collection of dialysate and urine (OWC). Nine children aged 2-18 years and weighing 10.6-39.9 kg were examined over 1 week (25 treatments). UKM3 and UKM2 gave almost identical results, but deviated from PDC and OWC. The two indirect methods overestimated G by 24% and 18%. However, the correlations between the results were very high for all variables and all methods (r > or = 0.96). Repeating UKM3 and UKM2 mid-week for 5 consecutive weeks, the following coefficients of variation were found: for the normalised whole body urea clearance (Kt/V) 10% and 11%, respectively; for normalised protein catabolic rate 17% and 14%. It is concluded that all tested methods can be used, but each method requires its own reference interval. Results of UKM seem to vary somewhat more than in adults. This should be considered when assessing children by such methods.