We studied the long-term outcome of 64 children with biopsy-proven Schönlein-Henoch purpura (SHP) nephritis over 1-23 years of follow-up. Overall renal survival 10 years after onset was 73%. Multivariate logistic regression analysis identified initial renal insufficiency (P=0.004), nephrotic syndrome (P=0.037), and the severity of histological alterations, as defined by the proportion of glomerular crescents (P=0.051), as significant independent predictors of progressive renal failure. Four patients followed for more than 19 years showed glomerular damage after transient recovery. Eight children with crescentic nephritis associated with a rapidly progressive course and/or persistent nephrotic syndrome were treated by at least seven sessions of plasma exchange (PE) within 16 weeks of onset of purpura. During treatment serum creatinine levels dropped in each patient from a mean of 2.3 to 1.1 mg/dl, followed by a rebound increase. Repeated courses of PE in 5 patients produced comparable responses. Four patients undergoing PE reached end-stage renal disease at 1.2.-3.7 years after onset, whilst 3 finally were in preterminal renal failure (creatinine 3.2-6.1 mg/dl after 7-13.5 years), and 1 patient reached a normal glomerular filtration rate. Our experience suggests that initial renal insufficiency is the best single predictor of the further clinical course in children with SHP nephritis. Early PE appears to delay the progression in some patients with severe, rapidly progressive forms of the disease.

Within a collaborative project of 14 transplant centers, prospective recipients of cadaver kidney grafts were randomized to receive either three pretransplant transfusions or transplants without transfusions. RESULTS; The graft survival rate was significantly higher in the 205 transfusion recipients than in the 218 patients who did not receive transfusions (at 1 year: 90+/-2% vs. 82+/-3%, P=0.020; at 5 years: 79+/-3% vs. 70+/-4%, P=0.025). Cox regression analysis showed that this effect was independent of age, gender, underlying disease, prophylaxis with antilymphocyte antibodies, and preformed lymphocytotoxins. CONCLUSIONS; Transfusion pretreatment improves the outcome of cadaver kidney transplants even with the use of modern immunosuppressive regimens.

The majority of children with renal allografts have diminished growth and reduced final height. Impaired allograft function and glucocorticoid treatment are the main contributing factors. Since recombinant human growth hormone (rhGH) treatment was able to counteract the growth depressing effects of glucocorticoids in experimental uremia, an open-labeled prospective study in 17 short children with renal allografts was designed to investigate the efficacy of rhGH therapy (30 IU/m2/week) with special emphasis on the safety regarding graft function and carbohydrate metabolism. Height velocity in prepubertal children (N = 10) increased from baseline median 2.2 cm/year to 7.9 cm/year after one year (P < 0.01), 7.2 cm/year after two years (P < 0.01), and 5.5 cm/year (P < 0.05) after three years of rhGH therapy. This resulted in a normalization of height in three out of seven patients after two years and in three out of five after three years of therapy. Growth stimulation in pubertal children was less consistent. Bone maturation paralleled chronological age. The effect of rhGH treatment on longitudinal growth may be partially attributable to the improved ratio between the serum concentration of the insulin-like growth factor (IGF)-I and its major binding protein (BP) IGFBP-3 leading to a normal IGF bioactivity. The incidence of acute rejection crises in the study group (corrected for time after grafting) did not differ from that of untreated retrospective "controls" (0.10 vs. 0.12 episodes per patient and year). No systematic effect of rhGH on glomerular filtration rate assessed by repeated inulin and creatinine clearances was noted.(ABSTRACT TRUNCATED AT 250 WORDS)

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