• Clinical and Experimental Pediatrics
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• v.46 no.10
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• pp.1040-1043
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• 2003

Henoch-Shonlein purpura(HSP) is a systemic small-vessel vasculitis that primarily affects the skin, gastrointestinal tract, joints, and kidneys. The nervous system may be involved, less commonly than other organs. When the central nervous system(CNS) was involved, headache, changes in mental status, seizures, and focal neurologic deficits have been reported. Hypertension, uremic encephalopathy, metabolic abnomalities, electrolyte abnormalities, or cerebral vasculitis were suggested as possible causes of the neurologic manifestation. Diagnosis of vasculitic involvement of CNS is difficult. Magnetic resonance imaging of the brain is the modality of choice for the evaluation of the CNS disease. Steroid or plasmapheresis are used in treatment of cerebral vasculitis. We experienced a case of 9-year-old boy who had presented with Henoch-Schonlein purpura nephritis complicating encephalopathy accompanied by hypertension and cerebral vasculitis. Brain MRI showed multiple small nodular-linear pattern enhancing lesions in whole cerebral hemispheres and focal increased T2 signal in the right basal ganglia. We used intravenous immunoglobulin in treatment of cerebral vasculitis. We report this case with a brief review of related literature.

• Clinical and Experimental Pediatrics
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• v.45 no.7
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• pp.884-890
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• 2002

Purpose : Henoch-Schonlein purpura(HSP) nephritis has been reported to vary from 25 to 50% among HSP patients and is a common cause of chronic glomerulonephritis in children. In our study, we evaluated the distribution and the association of the Insertion/Deletion(I/D) polymorphism of angiotensin converting enzyme(ACE) gene with clinical manifestations, particularly proteinuria in children with HSP nephritis, compared with that in HSP. Methods : ACE gene polymorphism was determined in children with HSP nephritis(n=33) and HSP(n=28) who were diagnosed in Busan Paik hospital from January 1996 to June 2001. The I/D polymorphism of ACE gene was determined by PCR amplication of genomic DNA. Results : The ACE I/D genotype frequency was DD : 25%, ID : 50%, II : 25% in HSP and DD : 24 %, ID : 46%, II : 30% in HSP nephritis, there was no significant difference in the genotype and allele frequencies between two groups. When statistical analysis was done according to the presence of D allele, the amount of 24-hour urinary protein excretion and the incidence of moderate to heavy proteinuria(>$500mg/m^2/day$) at onset and last follow-up were higher in DD/ID genotype than in those in II genotype, but these differences were not statistically significant. Conclusion : We suggest a lack of association between I/D polymorphism of ACE gene and clinical manifestations in children with HSP nephritis. However, further follow-up studies based on a sufficient number of patients and long term follow up periods are necessary to confirm the role of I/D polymorphism of ACE gene in children with HSP nephritis.

• Childhood Kidney Diseases
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• v.24 no.1
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• pp.1-13
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• 2020

Immunoglobulin (Ig)A vasculitis nephritis (IgAVN), also referred to as Henoch-Schönlein purpura nephritis, is a relatively benign disease in children. However, two 24-year European cohort studies have reported high sustained rates of hypertension, severe proteinuria, and renal dysfunction in patients with IgAVN. Notably, the incidence and exacerbation rates of proteinuria, hypertension, and renal dysfunction during pregnancy were high even in women who recovered from IgAVN before pregnancy. Patients with IgAVN need lifelong care. Trials have been performed to investigate early biomarkers and genes associated with poor prognosis to identify high-risk patients in whom IgAVN may progress to severe renal disease. Urinary IgA/cr, IgM/cr levels, and HLAB35 and angiotensinogen gene expression were shown to be predictors of progression of IgAVN to severe renal dysfunction. The 2019 Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) initiative group published guidelines for pediatric IgAVN, following the Kidney Disease: Improving Global Outcomes (KDIGO) guidelines established in 2012. Compared with the KDIGO guidelines, the SHARE guidelines recommend earlier corticosteroid administration in cases of mild proteinuria (>0.5 g/d). Clinical trials of targeted budesonide delivery to the distal ileum, monoclonal antibody targeting C5, eculizumab and anti-CD20 monoclonal antibody administration, among others are currently underway in patients with IgA nephropathy. It is expected that newer therapeutic agents would become available for IgAVN in the near future. This review summarizes IgAVN with emphasis on recently published literature, including possible preventive strategies, predictive biomarkers for progression of IgAVN, and various treatments.

• 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.12 no.2
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• pp.170-177
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• 2008

Purpose : The aim of this study was to evaluate whether the incidence of relapse or nephritis might be influenced by the duration of corticosteroid therapy in children with Henoch-Schonlein purpura(HSP). Methods : We retrospectively analyzed 186 children with a diagnosis of HSP in two major hospitals in Ilsan, Korea from the years 2000 to 2003. To evaluate whether renal involvement or relapse might be influenced by the duration of corticosteroid therapy in children with HSP, one pediatric nephrologist from hospital A, maintained corticosteroid therapy for at least 2 weeks(Group A, n=94). The other from hospital B used only during the symptomatic period(Group B, n=92). Results : There were no significant differences in age, sex, body weight, white blood cell count, hemoglobin, hematocrit, platelet count, serum protein and albumin levels between the two groups. The incidence of abdominal pain or arthralgia also did not differ between two groups. However, the duration of steroid therapy was significantly longer in Group A than in Group B and the cumulative dose of prednisolone was also higher in Group A than in Group B. The development of nephritis was more frequent in Group A. Conclusion : The longer duration of steroid use was not associated with the decreased rate of nephritis. Therefore, corticosteroids should be used carefully in a selected group of HSP children, and be tapered rapidly after control of the acute symptoms.

• 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.9 no.1
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• pp.21-30
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• 2005

Purpose : Metabolic bone diseases have been mai or problems in children with renal diseases and steroid treatment is the main precipitating factor reducing bone mineral density(BMD). This study was performed to assess the prevalence of osteoporosis and to evaluate the clinical factors associated with decreased BMD in children with renal diseases. Methods : Forty-four children with renal diseases who were diagnosed at the Pediatric no phrology division of Ajou University hospital since Oct. 1994 were included. Using a new quantitative ultrasound device, BMD and the prevalence of osteoporosis were evaluated. The clinical and serological data were analyzed in association with decreased BMD. Results : A total of 44 patients were evaluated. The age at initial diagnosis was 6.7$\pm$4.2 years. At the time of evaluation, the chronological and bone age was 9.3$\pm$4.2 years and 8.2 $\pm$ 4.6 years, respectively. The renal diseases included nephrotic syndrome 24(54.5%), Henoch Schonlein purpura nephritis 7(15.9%), IgA nephropathy 6(13.9%), reflux nephropathy(RN) 2 (4.5%), and other renal disease 5(%). The prevalence of osteoporosis was 11%. There was no difference in the clinical factors between the long-term and the short-term treated steroid groups. Conclusion : The prevalence of osteoporosis was 12% in 44 children with renal diseases No significant factor was found in association with decreased BMD and there was no relationship between osteoporosis and steroid usage duration or cumulative dose. A new quantitative ultrasound, which is relatively easy to perform, especially in children, is expected to be in common use and will enable clinicians to evaluate metabolic bone disorders with ease.

• Childhood Kidney Diseases
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• v.2 no.1
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• pp.26-33
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• 1998

This retrospective study was designed to evaluate the prognostic value and histologic correlation of sonography in childhood nephrotic syndrome. Sixty-nine patients with proteinuria over 2g per day at the time of presentation who were treated at the Korea University Hospital were included in this review. They were 1 to 15 years old(mean age, 7.8 years) with 49 males and 20 females. In each patient an ultrasound examination was done using SPA 1000(Diasonics, C.A., U.S.A.) on admission. Tissue specimens were obtained from 46 patients. The paraffin-embedded specimens were reviewed with special reference to interstitial edema, interstitial fibrosis, tubular atrophy, global sclerosis or inflammatory cell infiltrates. Biopsy proven renal disease were minimal change disease(n=20), focal segmental glomerulosclerosis(n=7), membranous glomerulonephritis(n=2), membranoproliferative glomerulonephritis(n=1), $Henoch-Sch\"{o}nlein$ purpura nephritis(n=6), IgA nephropathy(n=5), poststreptococcal glomerulonephritis(n=2), systemic lupus erythematosus(n=1) and Alport syndrome(n=2). There was a significant relationship between increased cortical echogenicity and global sclerosis or tubular atrophy(P<0.05). But no significant relationship was found between increased cortical echogenicity and interstitial fibrosis, interstitial edema, or inflammatory cell infiltration. In biopsy-proven primary nephrotic syndrome(n=30), no significant relationship was found between the increased conical echogenicity and the interstitial edema, interstitial fibrosis, global sclerosis, tubular atrophy or inflammatory cell infiltration. But there was a significant relationship between increased cortical echogenicity and resistance to corticosteroid (P<0.05). These results suggest that increased cortical echogenicity may be due to tubular atrophy or global sclerosis in patients with proteinuria and may be an effective indicator of resistance to corticosteroid in primary nephrotic syndrome.(J Korean Soc of Pediatr Nephrol 2:26-33, 1998)