• Childhood Kidney Diseases
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• v.20 no.1
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• pp.23-28
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• 2016

Purpose: We investigated whether serum levels of insulin growth factor-1 (IGF-1) and insulin growth factor binding protein-3 (IGFBP-3) are valuable in predicting clinical outcomes or are correlated with other laboratory findings in children with Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP). Methods: We examined 27 children who were consecutively admitted to our hospital with HSP between January 2011 and February 2012. Blood tests (C-reactive protein, white blood cell count, platelet count, erythrocyte sedimentation rate, albumin, immunoglobulin A, complement C3, antineutrophil cytoplasmic antibody, IGF-1, IGFBP-3) and urine tests were performed upon admission. IGF-1 and IGFBP-3 were resampled in the recovery phase. Controls included 473 children whose IGF-1 and IGFBP-3 were sampled for evaluating their growth, at the outpatient department of pediatric endocrinology in our hospital. IGF-1 and IGFBP-3 were compared between the HSP children and controls, and between the acute and recovery phases in HSP children. The ability of these values to predict clinical outcomes including renal involvement was analyzed using bivariate logistic regression analysis (BLRA). Results: IGF-1 and IGFBP-3 were not different between the HSP children and controls ($148.7{\pm}117.6$ vs. $69.2{\pm}96.9$, P=0.290: $3465.9{\pm}1290.9$ vs. $3597.2{\pm}1,127.6$, P=0.560, respectively). There was no significant difference in IGF-1 or IGFBP-3 between acute and recovery phases. Based on the BLRA, no variable, including IGF-1 and IGFBP-3, could predict clinical outcomes including the presence of nephritis Conclusion: We concluded that IGF-1 and IGFBP-3 do not predict clinical outcomes of HSP, including renal involvement, in this study.

• Childhood Kidney Diseases
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• v.2 no.2
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• pp.118-124
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• 1998

Purpose : There is no specific treatment guidelines for Henoch-$Sch{\ddot{o}}nlein$(HS) nephritis. Therefore we performed this study to observe the effect of long term steroid therapy combined with azathioprine Methods : Treatment protocols; 1) Steroid pulse therapy: methylprednisolon 30 mg/kg/dose, maximum 1 gm, intravenolisly 6 times for alternate day. 2) Oral steroid was given 2 mg/kg/day for 1 month, 1 mg/kg/day for following 1 month and alternate day oral steroid combined with azathioprine 2 mg/kg/day for 2 years. Results : Time period from HSP to onset of HS nephritis was between 2 weeks to 5 months with mean $7.4{\pm}7.4$ weeks. Clinical remission were seen in 4 cases out of 5 ($80\%$). Mean time period with disappearance of proteinuria and microscopic hematuria were $5{\pm}2.4$ month and $13.3{\pm}2.9$ month respectively. On pathologic findings by ISKDC, 3 cases were grade IIIb, 2 cases were grade IV in first kidney biopsies and showed pathologic improvement in follow up tidneybiopsiesafterlyearstreatment. Conclusion : As there is no definitive treatment for HS nephritis so far, our study of long term oral steroid therapy with azathioprine was effective in clinical and histologic aspect. Therefore further study in HS nephritis with in a large group will be needed in the future.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.185-194
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• 2007

Purpose : $Henoch-Sch\ddot{o}nlein$ Purpura(HSP) is a self-limited systemic small vessel vasculitis, however, renal involvement is considered to contribute to the outcome of this disease. Therefore, identifying the renal risk factors in HSP and prevention of renal involvement are important. The aim of this study is to investigate whether early steroid administration in HSP could reduce the rate of renal involvement. Methods : We retrospectively studied two hundred children with HSP. We had administrated steroids orally to resolve of severe abdominal pain, joint and scrotal symptoms. We analyzed the relationship between the steroid therapy to relieve systemic symptoms and the subsequent renal involvement in HSP. Results : There were no significant differences in the incidence and duration of renal involvement according to steroid administration and its duration. In HSP patients with renal manifestations, steroid administration group showed a tendency of hematuria and steroid non-administration group showed a tendency of proteinuria, however, we could not find statistically significant differences in each group. There was no significant difference in the duration of purpura presence according to steroid administration. However, persistent purpura increased the incidence and the duration of renal involvement. Conclusion : Early steroid administration did not reduce the risk of renal involvement, there-fore, steroid could not prevent delayed nephritis in children with HSP On the other side, Persistent Purpura, known to be not related to steroid therapy, was associated with renal involvement. We suggest that early steroid administration could not be useful in preventing the renal involvement in HSP. (J Korean Soc Pediatr Nephrol 2007;11:185-194)

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.19 no.3
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• pp.175-185
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• 2016

Purpose: To investigate the epidemiology, clinical manifestations, investigations and management, and prognosis of patients with Henoch-Schonlein purpura (HSP). Methods: We performed a retrospective review of 212 HSP patients under the age of 18 years who were admitted to Inje University Sanggye Paik Hospital between 2004 and 2015. Results: The mean age of the HSP patients was 6.93 years, and the ratio of boys to girls was 1.23:1. HSP occurred most frequently in the winter (33.0%) and least frequently in the summer (11.3%). Palpable purpura spots were found in 208 patients (98.1%), and gastrointestinal (GI) and joint symptoms were observed in 159 (75.0%) and 148 (69.8%) patients, respectively. There were 57 patients (26.9%) with renal involvement and 10 patients (4.7%) with nephrotic syndrome. The incidence of renal involvement and nephrotic syndrome was significantly higher in patients with severe GI symptoms and in those over 7 years old. The majority of patients (88.7%) were treated with steroids. There was no significant difference in the incidence of renal involvement or nephrotic syndrome among patients receiving different doses of steroids. Conclusion: In this study, the epidemiologic features of HSP in children were similar to those described in previous studies, but GI and joint symptoms manifested more frequently. It is essential to carefully monitor renal involvement and progression to chronic renal disease in patients ${\geq}7$ years old and in patients affected by severe GI symptoms. It can be assumed that there is no direct association between early doses of steroids and prognosis.

• Childhood Kidney Diseases
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• v.2 no.1
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• pp.41-49
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• 1998

Treatment of $Henoch-Sch\"{o}nlein$ purpura nephritis(HSPN) accomanied by nephrotic syndrome is still controversal, even though both corticosteroids and immunosuppressants have been used for therapy. Azathioprine(AZA) is a chemical analog of the physiologic purines-adenine, guanine, and hyoxanthine and an antagonist to purine metabolism which may inhibit RNA and DNA synthesis and is mainly used for immunosuppressive agent. We studied the effects of AZA in HSPN accompanied by nephrotic syndrome and evaluating the clinical status and histopathologic changes by sequential biopsies following the treatment. Fifteen patients with nehprotic syndrome either initially or during the course of HSPN confirmed by renal biopsies were treated with AZA(2 mg/kg/day) and prednisolone (0.5-1 mg/kg/day qod) for 8months. Folow up renal biopsy was done after treatment in 11 patients. The clinical status of the patients on admission were C(12 cases) and B(3 cases). Improvement of clinical status were showed in 12 cases, but 3 cases were not improved and 1 case was aggrevated after AZA treatment. Complete remission of proteinuria were in 8 cases(53.3%), partial remission were in 4 cases(26.7%) and persistence of proteinuria and hematuria were in 3 cases(20.0%). The loss of hematuria were in 10 cases(66.7%). Histopathologically and immunopathologically, 4 cases were improved. This study suggests that, although control studies are needed, AZA could be used in the treatment of HSPN accompanied by nephrotic syndrome.

• Childhood Kidney Diseases
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• v.17 no.2
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• pp.92-100
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• 2013

Purpose: To investigate the clinicopathologic effects of cyclosporine A (CsA) in children with diseases characterized by mesangial immunoglobulin A deposits such as immunoglobulin A nephropathy (IgAN) and Henoch-Sch$\ddot{o}$nlein purpura nephritis (HSPN). Methods: We retrospectively reviewed the clinicopathologic outcomes of 54 children (IgAN, 36; HSPN, 18) treated with CsA. The starting dose of CsA was 5 mg/kg per day, and it was administered in 2 divided doses. The degree of proteinuria and pathologic changes in renal biopsies were evaluated before and after CsA treatment. Results: The mean protein to creatinine ratio decreased from $3.7{\pm}1.5$ to $0.6{\pm}0.4$(P <0.001), and 32 (59.2%) children achieved complete remission of proteinuria after 1-year CsA treatment. Among the 54 children, 24 maintained normal renal function and 25 exhibited microscopic hematuria or proteinuria at the end of CsA treatment. In the HSPN group, 3 children whose initial biopsies indicated class IIIb disease showed class II disease on follow-up, and the follow-up biopsies of 2 children who had class II disease indicated the same class II disease. In the IgAN group, cortical tubular atrophy occurred in 1 child, and no child with IgAN had cortical interstitial fibrosis or tubular atrophy after 1-year CsA treatment. No significant complications were found in the children treated with CsA. Conclusion: Our findings indicate that CsA treatment is effective and beneficial in reducing massive proteinuria and preventing progression to end-stage renal failure in children with glomerular diseases characterized by IgA deposits, such as IgAN and HSPN, within 1 year of treatment.

• Pediatric Infection and Vaccine
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• v.14 no.1
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• pp.62-68
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• 2007

Purpose : The cause and pathogenesis of Henoch-Sch$\ddot{o}$nlein purpura (HSP) are unknown, but recently the hypothesis that immunoglobulin A may have an important role in the pathogenesis of HSP is being published and HSP associated with mycoplasma infection has been also reported. The aim of this study is to discover relation between HSP and mycoplsma infection. Methods : From Jan. 2002 to Dec. 2005, we retrospectively evaluated 98 children who were diagnosed as HSP at Ilsan Paik Hospital. 84 patients were not associated with mycoplasma infection (group A) and 14 patients were associated with mycoplasma infection (group B). We compared both groups about clinical features. Results : The ratio of male to female patients in group A and B were 1.21:1 and 1.80:1. The number of patients in group A was most in November and December, and in group B was most in November. All patients had non-thrombocytopenic purpura concentrated on the buttocks and lower extremities and joint involvement was most common on the feet and ankle in both groups. 57 (67.9%) cases in group A and 9 (64.3%) cases in group B complained of abdominal pain. And 21 (25.0%) cases in group A and 5 (35.7%) cases in group B had nephritis. Elevated mycoplasma antibody titer (${\geq}1:320$) or 4 fold-rise were noted in 14 of 98 patients (14.3%). In this study, there was one child with HSP preceded by typical mycoplasma pneumonia (mycoplasma antibody titer 1:1280). Conclusion : In this study, elevated mycoplasma antibody titer (${\geq}1:320$) or 4 fold-rise were noted in 14 of 98 patients and the difference of clinical features between group A and B was not specific. The role of mycoplasma infection in the etiology of HSP may have been implicated, so the association with mycoplasma infection should have to be proved by further controlled studies.

• Childhood Kidney Diseases
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• v.7 no.1
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• pp.30-37
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• 2003

Purpose : $Henoch-Sch\"{o}nlein$ purpura(HSP) is usually a self-limited disease with a good eventual outcome. The prognosis of HSP is mainly determined by the renal involvement. In this study, We evaluated children with biopsy-proven $Henoch-Sch\"{o}nlein$ purpura nephritis about the clinical outcome correlated with renal manifestation and morphologic findings. Methods : The clinical features, initial laboratory and pathologic findings, and clinical out-come were evaluated in 60 children with biopsy-proven $Henoch-Sch\"{o}nlein$ purpura nephritis at Yonsei University Severance Hospital during the period from Jan. 1990 to Dec. 2002. Results : The ratio of male to female patients was 1.2:1. The interval between the onset of $Henoch-Sch\"{o}nlein$ purpura and renal manifestation was less than 3 months in 81% of the patients. Initial renal manifestation was microscopic hematuria in 100% of patients, isolated hematuria in 15%, acute nephritic syndrome in 7%, nephrotic syndrome In 22% of patients. Renal manifestation correlated with clinical outcome. Grade II and III were the most common in histologic grades of ISKDC. Renal pathologic finding correlated with clinical outcome. Conclusion : Renal manifestation and pathologic findings correlated with the clinical out-come. It is necessary to evaluate the correlation between pathologic findings and treatment.

• Clinical and Experimental Pediatrics
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• v.46 no.6
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• pp.576-584
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• 2003

Purpose : For the early diagnosis of Henoch-Schonlein purpura(HSP) presenting with acute abdominal pain preceding skin rash. Methods : The clinical, endoscopic and radiological records of 23 cases of HSP, presenting with gastrointestinal symptoms preceding skin rash were reviewed. Results : The intervals from the onset of abdominal pain to the development of the skin rash were one day to 30 days(median five days), most of them were within two weeks. The presenting abdominal symptoms were abdominal pain(23 cases), vomiting(16 cases), hematochezia or melena(eight cases) and hematemesis(three cases). The abnormal endoscopic findings include coalescing erythematous lesions, areas of submucosal hemorrhage and superficial erosions and ulcers. The upper gastrointestinal endoscopy showed the abnormalities in 21 of 23 cases, which were observed in the duodenum( 21 cases), the stomach(12 cases) and the esophagus(one case). Duodenitis with hemorrhage and/or erosions in the descending duodenum was the sole endoscopic abnormality in two cases and was the most marked finding in three cases. Sigmoidoscopy showed the abnormalities in six of eight cases. The abdominal ultrasonogram showed abnormalities in 12 of 17 cases, which included small bowel wall thickening(eight cases) and intramural hemorrhage(three cases). Recurrences after three months of symptom free intervals developed in four cases; three of them had persistent nephritis beyond one year. Conclusion : The erosive hemorrhagic duodenitis in the descending duodenum in the upper endoscopy and the small bowel wall thickening in the abdominal ultrasonogram can be useful findings in the diagnosis of HSP presenting with acute abdomen.

• Childhood Kidney Diseases
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• v.2 no.2
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• pp.110-117
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• 1998

Purpose : Henoch-$Sch{\ddot{o}}nlein$ purpura nephritis(HSPN) accompanied by nephrotic syndrome(NS) is known to have a poor prognosis and effective treatment is still controversial, even though both corticosteroids and immunosuppresant have been used for therapy. Cyclosporine A(CsA) is a well known immunosuppresant and widely used in renal transplantation and glomerular diseases especially steroid resistant. The aims of this study was to evaluate the therapeutic effect of CsA and to compare CsA with previously reported our data of rifampin(RFP) and azathioprine(AZA) in children with HSPN accompanied by NS. Methods : 37 HSPN patients with NS confirmed by renal biopsy were selected. Of these, 17 patients were treated with CsA(5 mg/kg/day) fur 6-8 months, 7 children were treated with RFP(10-20 mg/kg/day) for 9-12 months and 13 patients were treated with AZA(2 mg/kg/day) fur 8 months. Along with these regimens, low dose oral prednisolone(0.5-1 mg/kg, qod) was also used. Sequential renal biopsy was done in all patients 1 month after termination of treatment. Results : Complete remission rate of nephrotic syndrome was $58.8\%$ in CsA, $57.1\%$ in RFP and $38.4\%$ in AZA group after 17, 22, 11 months of mean follow-up period. Overall remission rate including partial remission was $88.2\%$ in CsA, $85.7\%$ in RFP and $84.6\%$ in AZA group. Disappearance rate of hematuria was $58.8\%$ in CsA, $57.1\%$ in RFP and $46.2\%$ in AZA group. Improvement of grade of clinical status was observed in 17 out of 17 CsA, 7 out of 7 RFP and 10 out of 13 AZA group. Improvement of pathologic class on sequencial renal biopsy was shown in 5 CsA($29.4\%$), none RFP($0\%$) and 2 AZA group($12.4\%$). Improvement on histologic immune-deposition was seen in 15 CsA($88.2\%$), 6 RFP($85.9\%$) and 4 AZA group($30.8\%$). Conclusion : In conclusion, Both CsA and RFP treated groups showed better result in complete remission rate of nephrotic syndrome and significant improvement of histologic immune-deposition compared with AZA treated group(p=0.004). So, we recommend CsA and REP rather than AZA for immunosuppresant treatment in HSPN with nephrotic syndrome.