Peter N. McLaine

A randomized, placebo-controlled trial of prednisone in early Henoch Schönlein Purpura (ISRCTN85109383)

BackgroundHenoch Schönlein Purpura (HSP) is the most common systemic vasculitis of childhood. There is considerable controversy over whether children with HSP should be treated with corticosteroids. The goal of this study was to investigate whether early corticosteroid administration could reduce the rate of renal or gastrointestinal complications in children with HSP.MethodsForty children with HSP, seen in the emergency room of a tertiary-care, paediatric centre, entered a randomized, double-blind, placebo controlled study. The treatment group (n = 21) received oral prednisone, 2 mg/kg/day for one week, with weaning over a second week, while the placebo group (n = 19) received an identical appearing placebo. Co-primary outcomes were the rate of renal involvement at one year and the rate of acute gastrointestinal complications. Co-primary outcomes were analysed using Fishers Exact test.ResultsAt one year, there was no difference in the rate of renal involvement (3/21 prednisone group vs. 2/19 placebo group, P = 1.0). There was also no statistically significant difference in the rate of acute gastrointestinal complications (2/21 prednisone group vs. 3/19 placebo group, P = 0.7). Two children in the placebo group did experience intussusceptions compared with none in the prednisone group (P = 0.2).ConclusionsEarly prednisone therapy in HSP does not appear to reduce the risk of renal involvement at one year, or the risk of acute gastrointestinal complications. There may be a reduced risk of intussusception. The routine, early use of prednisone in uncomplicated HSP cannot be recommended at this time.

Prevalence of hypertension in children with primary vesicoureteral reflux

This study was undertaken to determine the prevalence of hypertension in childdren with primary, uncomplciated vesicoureteral reflux (VUR) and to evaluate the relationship between blood pressure (BP), grade and duration of reflux, and renal scarring. Subjects were identified retrospectively during a 17-year period; of 146 subjects who agreed to participate, 129 (88.4%) were female. Mean age at diagnosis was 5.0 years (range, 1 month to 16 years), and at follow-up was 14.4 years (range, 5 months to 21 years). Mean durtion of follow-up was 9.6 years. Renal scarring was detedted in 34.3% of patients by intravenous pyelogram, ultrasonography, or both. The BP at diagnosis was linearly related to the grade of reflux, but values were not higher than expected norms for age. At follow-up, mean systollc and diastolic BP were at the 41.6 percentile and the 18.7 percentile, respectively. No patients BP was above the 55th percentile. After a mean follow-up period of 10 years, we conclude that primary, uncomplicated VUR, regardless of the number of documented urinary tract nfections, duration and severity of reflux, modality of therapy, presence of renal scarring, and duration of follow-up, is not associated with the development of hypertension. Hypertension does not appear to be a complication of VUR and urinary tract infection unless there is preexisting dysplasis.

Haemolytic uraemic syndrome is an immune-mediated disease: role of anti-CD36 antibodies.

Haemolytic uraemic syndrome (HUS) is a disorder in which platelet microthrombi are formed that have a particular propensity to deposit in the kidney microvasculature, resulting in impaired renal function and thrombocytopenia. The mechanism of formation of these microthrombi is not known. In this study, we showed that plasma from five adult and six paediatric cases of HUS caused aggregation and release of adenosine triphosphate from normal platelets. The plasma reacted against platelet lysate in a protein blot and all samples showed reactivity against a band at 88 kDa, corresponding to the membrane antigen CD36. This was confirmed by probing with Mo91, a monoclonal antibody to CD36. CD36 was also identified in the immune complex formed by incubation of patient plasmas with normal platelet lysate. In other studies, bands of 32 and 7·7 kDa were obtained when purified verotoxin was protein blotted and probed with either patient plasma or with anti‐CD36 antibody Mo91 suggesting structural homologies between CD36 and verotoxin. While a direct cause–effect relationship is not yet established, the data support the concept of an immunological pathogenesis for HUS and suggest that molecular mimicry involving one or both of the homologous domains in membrane‐bound CD36 and verotoxin lead to the development of antibodies capable of inducing the pathophysiological events characteristic of HUS.

Idiopathic unilateral gross hematuria in the pediatric patient.

Microscopic hematuria is not an uncommon finding in the pediatric population. Since gross hematuria elicits parental and physician concern, its presence may result in extensive investigation. Unilateral hematuria is an unusual feature of gross hematuria, particularly in the pediatric patient. Although a number of structural and pathologic entities have been described to account for the laterality, in the majority of instances no overt underlying process has been uncovered. Unilateral hematuria appears to be benign, idiopathic, and has a prognosis no different than other idiopathic hematurias in the pediatric age group.

Experiences with HUS in Canada: what have we learned about childhood HUS in Canada?

Experiences with childhood hemolytic uremic syndrome (HUS) in Canada will focus on the development of the Canadian Pediatric Kidney Disease Research Centre (CPKDRC) and the results of our collaborative research over a 13-year period (1985-1998).

Sequelae of haemolytic uraemic syndrome

Twenty two patients with previous episodes of haemolytic uraemic syndrome (HUS) were investigated for evidence of deficits in cognitive, behavioural, and academic function. Patients were pair matched with 22 controls for age (+/- 1 year), gender, and socioeconomic status. HUS patients had numerically lower cognitive and achievement scores and higher behavioural problem ratings than their controls on every measure. None of the group differences was significant at the 0.01 level. Significance values between 0.10 and 0.01 were obtained for the Wechsler full scale and verbal intelligence quotient scores and for several of the achievement measures and behaviour ratings. These results were conservatively interpreted as trends and are considered to provide preliminary indications of a post-HUS deficit in behaviour, verbal intelligence, and the verbally based skills of reading comprehension and vocabulary. The findings provide interim guidelines for follow up care but require confirmation and elaboration in a larger study.