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

• Childhood Kidney Diseases
• /
• v.4 no.1
• /
• pp.40-47
• /
• 2000

Purpose: Renal involvement is the most important prognostic factor of HSP. Therefore, the pathogenesis and prognostic factors in renal involvement have been studied by many researchers. The aim of this study was to evaluate the clinical risk factors and the role of TNF-$\alpha$ in renal involvement of HS purpura. Methods: The subjects of this study were 12 patients of HS purpura, 7 patients of HS nephritis, and 5 age-matched controls. We have analysed the rist factors for renal involvement in clinical symptoms and collected the sera and urines of all subjects in acute and convalescent stage. The concentration of TNF-$\alpha$ in the collected sera and urines were measured by sandwich ELISA and compared with that of age-matched controls. Results: Statistical analysis showed that persistent purpura increased the risk of developing renal involvement (P=0.0018). and serum TNF-$\alpha$ levels in the acute stage of patients with renal involvement($11.45{\pm}7.01$ pg/ml) were significantly higher than those of without renal involvement($6.32{\pm}1.31$pg/m1) and of age-matched controls($5.99{\pm}1.34$pg/m1)(P=0.012, 0.027, respectively). However, urine TNF-$\alpha$ levels have no correlation with renal involvement. On investigation of serum TNF-$\alpha$ levels in acute stage of HS purpura, persistent purpura had a significantly higher increase(P=0.038). Conclusion: Serum concentration of TNF-$\alpha$ is a risk factor and has a predictable value along with clinical risk factors, such as, persistent purpura for renal involvement in HS purpura. Also, the effectiveness of the specific treatment fur antagonizing TNF-$\alpha$ in HS nephritis may need further study.

• Childhood Kidney Diseases
• /
• v.21 no.2
• /
• pp.61-68
• /
• 2017

Purpose: To investigate differences in clinical features, blood/urinary findings, and prognosis in different age groups of patients with Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP). Methods: A total of 469 patients with HSP were analyzed retrospectively from June 2003 to February 2016. We classified patients into child or adult groups based on their age. Results: The adult group had more patients with anemia (child vs. adult; 7.5% vs. 16.4%), and higher immunoglobulin A (IgA) (30.0% vs. 50.0%) levels, C-reactive protein (34.2% vs. 54.0%) and uric acid (3.1% vs. 12.1%) levels than the child group. The child group was highly positive for Mycoplasma pneumoniae immunoglobulin M (IgM) (34.4%). More patients in the child group presented with high levels of antistreptolysin O (24.7% vs. 2.9%) and high C4 (11.5% vs. 4.2%). Low C3 (1.1% vs. 10.2%) levels, and renal involvement with gross hematuria (8.6% vs. 21.5 %), nonnephrotic proteinuria (1.1% vs. 11.2%), and nephrotic syndrome (1.1% vs. 6.0%) were common in the adult group. Adults also had poorer renal outcomes [persistent hematuria/proteinuria (10.5% vs. 32.8%), and chronic kidney disease (0% vs. 11.2%)] than the child group. Risk factors for renal involvement such as older age and higher level of uric acid were only found in the child group. The risk factors for poor renal outcome were nephrotic syndrome in the child group and gross hematuria in the adult group. Conclusion: In this study, child and adult groups presented with different clinical manifestations of HSP. We found that risk factors for renal involvement included age and high uric acid level in the child group. Moreover, nephrotic syndrome in the child group and gross hematuria in the adult group increased the risk of poor renal outcome.

• Clinical and Experimental Pediatrics
• /
• v.46 no.6
• /
• pp.576-584
• /
• 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
• /
• v.17 no.2
• /
• pp.149-153
• /
• 2013

We report the case of a 14-year-old girl, diagnosed with atypical thrombotic microangiopathy (TMA). The patient presented with persistent fever, nausea, and newly developed peripheral edema. Her laboratory findings indicated chronic anemia with no evidence of hemolysis, thrombocytopenia, or elevated serum creatinine level. A few days after hospitalization, acute renal failure and fever worsened, and proteinuria developed. On day 40 of hospitalization, she experienced a generalized tonic seizure for 5 min, accompanied by renal hypertension. Brain magnetic resonance imaging revealed posterior reversible leukoencephalopathy syndrome. After steroid pulse therapy, a renal biopsy was performed because of delayed recovery from thrombocytopenia. The biopsy findings showed features of thrombotic microangiopathic hemolysis with fibrinoid change restricted. Current diagnostic criteria for TMA have focused on thrombotic thrombocytopenic purpura and hemolytic uremic syndrome, and diagnosis is based on the clinical presentation and etiology, with the consequence that idiopathic and atypical forms of TMA can be overlooked. Developing effective tools to diagnose TMA, such as studying levels of ADAMTS13 or testing for abnormalities in the complement system, will be the first step to improving patient outcomes.

• Childhood Kidney Diseases
• /
• v.4 no.1
• /
• pp.25-32
• /
• 2000

Purpose: This retrospective study of 126 children with symptomless primary hematuria was undertaken to determine the distribution of various histologic types by renal biopsy, clinical outcome according to the biopsy findings and also to find out feasibility of performing renal biopsy in these children. Patients and Methods : Study population consisted of 126 children with symptom-less primary hematuria who have been admitted to the pediatric department of Kyung-poot National University Hospital for the past 11 years from 1987 to 1998 and renal biopsy was performed percutaneously. Hematuric children with duration of less than 6 months, evidences of systemic illness such as SLE or Henoch-Schonlein purpura, urinary tract infection, and idiopathic hypercalciuria were excluded from the study. Results : Mean age of presentation was 9.2${\pm}$3.3 years (range ; 1.5-15.3 years) and male preponderance was noted with male to female ratio of 2:1. IgA nephropathy was the most common biopsy finding occuring in 60 children ($47.6\%$), followed by MsPGN in 13 ($10.3\%$), MPGN in 5 ($3.9\%$), TGBM in 6 ($4.7\%$), Alport syndrome in 2 ($1.6\%$), FSGS in 1 ($0.8\%$), and in 39 children ($30.9\%$), 'normal' glomeruli were noted. Recurrent gross hematuria was more common than persistent microscopic hematuria (84 versus 42), and especially in IgA nephropathy, recurrent gross hematuria was the most prevalent pattern of hematuria. In 58 out of 126 cases ($46.0\%$), hematuria was isolated without accompa-nying proteinuria and this was especially true In cases of MsPGN and 'normal' glomer-uli by biopsy finding. Normalization of urinalysis (disappearance of hematuria) in IgA nephropathy, MsPGN and 'normal' glomuli group were similar and it was $14\%,\;27\%\;and\;21\%$ respectively during 1-2 years of follow-up period, and $37.1\%,\;40\%\;and\;35\%$ respectively during 3-4 years of follow-up periods. However, abnormal urinalysis persi-sted in the majority of children with MPGN, TGBM. Alport syndrome and FSGS. Renal function deteriorated progressively in 6 cases (3 with IgA nephropathy, 2 with Alport syndrome and 1 with TGBM). Conclusion : In summary, present study demonstrates that in 126 children with symptomless primary hematuria, IgA nephropathy was the most common biopsy findings followed by MsPGN, MPGN, TGBM, Alport syndrome and FSGS, and 'normal glomeruli' was also seen in 39 cases ($30.9\%$). Renal histology could not be predictable on the clinical findings, so that to establish appropriate long-term planning for these children, we would recommend to obtain precise histologic diagnosis by renal biopsy.