***TASK*** the task is to summarize an input biomedical literature in six sentences 
    ***INPUT*** the input is a biomedical literature 
    ***OUTPUT*** the output is the summary of an input biomedical literature in six sentences 
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    Input: Abstract . Between 1980 and 1994 , 38 children with severe forms of Schönlein-Henoch purpura glomerulonephritis were entered into a prospect i ve study to evaluate methylprednisolone pulse therapy on the outcome of nephropathy in terms of clinical symptoms and histopathological changes . The patients were considered at risk of developing chronic renal failure when they presented with a nephrotic syndrome and /or had 50 % or more crescentic glomeruli . Initial renal biopsies were obtained from all patients and revealed diffuse proliferative endocapillary glomerulonephritis in 2 , focal and segmental glomerulonephritis in 4 , and endo- and extracapillary glomerulonephritis in 32 , 21 of whom had 50 % or more glomeruli with crescents . Patients were treated with intravenous pulse methylprednisolone ( 3 days ) followed by oral prednisone ( 3.5 months ) . At the latest follow-up , 1–16 years after initiation of therapy , 27 children had clinical ly recovered , 3 showed minimal urinary abnormalities , 4 persistent nephropathy , and 4 had progressed to end-stage renal failure . Sequential renal biopsies were obtained from 30 patients , 7–25 months after initiation of therapy . The clinical outcome correlated well with of the activity ( hypercellularity , cellular and fibrocellular crescents , and interstitial edema with mononuclear cell infiltrates ) and the chronicity ( fibrous crescents , glomerular sclerosis , tubular atrophy , and interstitial fibrosis ) indexes of post-therapy biopsies . Of particular interest were the post-therapy biopsies of the 18 patients who clinical ly recovered . They showed a significant decrease of the activity index from 5.1±1.1 to 0.4±0.8 with a decrease or even a disappearance of IgA deposits , while the chronicity index remained low ( 0.4±0.8 compared with 1.4±1 ) . Although uncontrolled , our study suggests that methylprednisolone pulse therapy is effective in those patients at risk of progression of their nephropathy , especially if started early during the course of the disease before the crescents become fibrous Background : Henoch-Schönlein Purpura ( HSP ) is a common childhood vasculitis with manifestations in numerous organ systems , including glomerulonephritis . Patients with more severe HSP-associated glomerulonephritis may develop chronic renal failure . Currently , no widely accepted treatment protocol s exist for patients with significant renal involvement . Methods : We retrospectively review ed the clinical courses of 12 children ( mean age 9 years ) with HSP glomerulonephritis treated with high-dose corticosteroids plus oral cyclophosphamide . All patients had nephrotic-range proteinuria , and all had significant histopathologic changes on biopsy , including crescentic nephritis in 10 patients . Treatment consisted of either intravenous pulse methylprednisolone or oral prednisone followed by oral cyclophosphamide ( 2 mg/kg/day ) for 12 weeks , along with either daily or alternate-day oral prednisone . Prednisone was tapered following completion of cyclophsophamide . Results : Serum albumin rose significantly after treatment from 2.8 ± ( SD ) 0.5 to 3.7 ± 0.4 g/dl ( p < 0.001 ) , and there was a concurrent reduction in proteinuria , as reflected by decreasing serial protein-to-creatinine ratios : from 6.3 ± 4.4 to 0.8 ± 0.8 ( p = 0.002 ) . Renal function remained normal in all patients . Hypertension developed during treatment in 10 patients , all but 1 of whom were normotensive at last follow-up , 35 ± 17 months following biopsy . Conclusions : We conclude that treatment of children with HSP nephritis with high-dose corticosteroids plus oral cyclophosphamide is safe and , as in nephrotic syndrome , appears to significantly reduce proteinuria which is a known risk factor for the development of renal insufficiency in HSP . Further studies with larger numbers of patients should be conducted to confirm this finding Objective To describe the extrarenal symptoms and clinical course of Henoch – Schönlein purpura ( HSP ) . Design A prospect i ve national multicentre trial with 6-month follow-up . Patients A total of 223 newly diagnosed paediatric HSP patients . Results Purpura was the initial symptom in 73 % of the patients and was preceded by joint or gastrointestinal manifestations in the rest by a mean of 4 days . Joint symptoms , abdominal pain , melena , nephritis and recurrences occurred in 90 % , 57 % , 8 % , 46 % and 25 % of the patients , respectively . Orchitis affected 17/122 ( 14 % ) of the boys . Seven patients developed protein-losing enteropathy characterised by abdominal pain , oedema and serum albumin under 30 g/l , and an additional 49 patients had subnormal albumin levels without any proteinuria . Positive fecal occult blood ( 26/117 , 22 % ) and α1-antitrypsin ( 7/77 , 9 % ) suggested mucosal injury even in the patients without gastrointestinal symptoms . HSP was often preceded by various bacterial , especially streptococcal ( 36 % ) and viral infections . Previous streptococcal infection did not induce changes in the level of complement component C3 . Recurrences were more frequent in patients > 8 years of age ( OR 3.7 , CI 2.0 to 7.0 , p<0.001 ) and in patients with nephritis ( OR 4.6 , CI 2.3 to 8.9 , p<0.001 ) . Patients with severe HSP nephritis had more extrarenal symptoms up to 6 months . There was no difference in the clinical course between the prednisone-treated and non-treated patients during the 6-month follow-up . Conclusions Serum albumin is often low in HSP patients without proteinuria , due to protein loss via the intestine . Although corticosteroids alleviate the symptoms , they seem not to alter the clinical course of HSP during 6 months of follow-up Abstract .Nine Japanese children with severe proteinuric Henoch-Schönlein purpura nephritis ( HSPN ) received prompt initiation of oral prednisolone ( 1.5 mg/kg/day ) combined with an 8-week course of cyclophosphamide ( 2 mg/kg/day ) therapy . All underwent renal biopsy before and after treatment . At presentation , urine protein excretion and histologic indices of the mean activity index , the mean chronicity index and the tubulointerstitial ( TI ) scores in the patients were 5.0±1.4 , 4.7±1.0 , 3.9±1.6 and 3.7±0.5 g/day , respectively . Urine protein excretion , the activity index and the TI scores decreased significantly at the second renal biopsies obtained at a mean interval of 23 months after the first [ 0.3±0.3 , 2.4±0.5 and 1.4±0.7 g/day ( P<0.01 ) , respectively ] , while the chronicity index did not change . At the latest observation ( mean interval 78 months ) , all except two showed negative proteinuria while no patient showed renal impairment . Although this case series is without controls , our experience suggests that early treatment with oral prednisolone and cyclophosphamide may be beneficial to a proportion of patients with severe proteinuric HSPN BACKGROUND There have been few controlled studies of combined therapy with multiple drugs , including immunosuppressives , for severe Henoch-Schoenlein nephritis ( HSPN ) . We evaluated the efficacy of methylprednisolone and urokinase pulse therapy combined with cyclophosphamide for patients with HSPN of at least grade IVb . METHODS We studied 37 patients who had been diagnosed with HSPN of at least grade IVb . Of them , 20 ( Group A ) were treated with  methylprednisolone and urokinase pulse therapy , and 17 ( Group B ) were treated with methylprednisolone and urokinase pulse therapy combined with cyclophophamide . We analysed the clinical features , laboratory and pathological findings of the two groups retrospectively . RESULTS After 6 months of treatment , mean urinary protein excretion in Group B had significantly decreased compared with Group A , and the activity index of both groups at the second biopsy was lower than that at the first . Furthermore , at the second biopsy , the chronicity index of Group B was lower than that of Group A. Four patients of Group A but none of Group B had persistent nephropathy ( P<0.05 ) . CONCLUSIONS Our study suggests that methylprednisolone and urokinase pulse therapy combined with cyclophosphamide is useful for patients with severe HSPN BACKGROUND Previous studies suggested that leukotrienes ( LT ) were involved in the pathogenesis of Henoch-Schönlein purpura ( HSP ) . This study investigated the efficacy of an add-on therapy with montelukast in the treatment of HSP . METHODS In this four-center , double-blind , placebo-controlled , parallel paired comparative study , 130 children with HSP were divided into two large groups : 84 patients without nephritis and 46 patients with nephritis . For each pair of patients with the same severity of disease , one subject was r and omly allocated to one subgroup and the other allocated to the other subbroup ; one subgroup received routine treatment plus placebo treatment , while the other subgroup received routine treatment plus montelukast treatment for 3 months . The efficacy was determined using Severity Scale Score ( SSS ) . Blood eosinophil count , eosinophil cationic protein ( ECP ) , IgE , interleukin (IL)-4 , IL-5 , IL-6 , IL-8 , IL-17 , LTB4 , and urinary LTE4 were measured . RESULTS Add-on therapy with montelukast alleviated the symptoms of HSP including purpura , abdominal pain , stool occult blood , arthritis , proteinuria and hematuria , and , accordingly , shortened the length of hospital stay , and lowered blood eosinophil count , ECP , IgE , IL-4 , IL-5 , IL-6 , IL-8 , IL-17 , LTB4 , and urinary LTE4 production , and also lowered the HSP relapse rate during the 3 months of treatment , but did not alter the outcome of nephritis at the end of follow up . CONCLUSIONS Add-on therapy with montelukast alleviated the symptoms of HSP . HSP may be improved by add-on therapy with a leukotriene receptor antagonist The benefits of treating severe Henoch-Schoenlein Purpura ( HSP ) glomerulonephritis have not been established . In this study , we evaluate the outcome of 21 children with severe HSP nephritis treated with corticosteroids and azathioprine . Between 1977 and 1995 , 78 children ( age range 1 to 16 years ) were seen for evaluation of HSP . Thirty-one underwent kidney biopsy ; indications included nephritic and /or nephrotic onset ( 15 patients ) , persistently decreased creatinine clearance ( 5 patients ) , or proteinuria > 4 g/24 h ( 11 patients ) . Twenty treated patients had diffuse mesangial proliferation with crescents in 6 - 100 % ( mean 40 % ) of glomeruli . One treated patient , not biopsied due to extreme obesity , had a creatinine clearance of 49 ml/min/1.73 m2 and proteinuria of 21.3 g/24 h. These 21 patients were initially treated with azathioprine and daily oral prednisone ( 13 patients ) or i.v . methyl-prednisolone ( 8 patients ) , followed by azathioprine and alternate-day prednisone for 9 - 24 ( mean 15 ) months . The average follow-up was 32 months . Over the course of follow-up , 19 treated patients showed a decline in hematuria ( > 5 red blood cells/high power field ) from 100 % to 16 % ( p < 0.01 ) , a fall in the serum creatinine from 1.71 + /- 2.20 to 0.78 + /- 0.25 mg/dl ( p < 0.01 ) , an increase in creatinine clearance from 76 + /- 43 to 122 + /- 26 ml/min/1.73 m2 ( p < 0.01 ) , and a reduction in proteinuria from 8.8 + /- 7.5 to 0.47 + /- 0.39 g/24 h ( p < 0.01 ) . Two treated patients progressed to end-stage renal failure . There was no difference in outcome comparing patients initially treated with prednisone versus methyl-prednisolone . These observations suggest that corticosteroid and azathioprine therapy is effective in crescentic HSP nephritis Objective . To observe the clinical efficacy of the Chinese herb , Triptolide , in children with moderately severe Henoch-Schönlein purpura nephritis ( HSPN ) . Methods . From January 2007 to December 2011 , 56 HSPN children manifested by nephrotic range proteinuria 
    Output:
    
Two studies ( one involving 56 children and the other involving 54 adults ) compared cyclophosphamide with placebo or supportive treatment and found no significant benefit of cyclophosphamide .
There are no substantial changes in conclusions from this up date compared with the initial review .
From generally low quality evidence , we found no evidence of benefit from RCTs for the use of prednisone or antiplatelet agents to prevent persistent kidney disease in children with HSP .
Though heparin appeared effective , this potentially dangerous therapy is not justified to prevent serious kidney disease when fewer than 2 % of children with HSP develop severe kidney disease .
No evidence of benefit has been found for cyclophosphamide treatment in children or adults with HSP and severe kidney disease .
Because of small patient numbers and events leading to imprecision in results , it remains unclear whether cyclosporin and mycophenolate mofetil have any roles in the treatment of children with HSP and severe kidney disease