• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.7 no.1
• /
• pp.54-60
• /
• 2004

Purpose: Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP) is a small-vessel vasculitic disease that most often affects the skin. Abdominal symptoms precede the typical purpuric rash of HSP in 14~36%. It is a challenge to diagnose HSP in the absence of a rash, because there are no biologic tests that can identify HSP with certainty, so we tried to find out the characteristic features of HSP gastroenteropathy without purpura before diagnosis. Methods: This study included 82 children with HSP who had been admitted or visited outward of the Department of Pediatrics, Pusan National University Hospital from 1995 to 2000. The cases that the onset of purpura preceded or coincided that of abdominal pain were defined as purpura-positive group. The cases that the onset of abdominal pain preceded purpura more than 1 week and purpura was not presented till diagnosed as HSP gastroenteropathy were defined as purpura-negative group. We compared and analyzed the clinical features of the two groups by reviewing the medical records retrospectively. To ensure the diagnosis of HSP gastroenteropathy, we conducted upper GI series, abdominal ultrasonogram, abdominal CT, endoscopy and/or skin biopsy. Results: The number of cases of purpura-positive group and purpura-negative group were 72 and 10, respectively. There is no difference between two groups in the incidence of clinical symptoms and laboratory findings. Children with HSP gastroenteropathy had characteristic erosive or ulcerative lesions in the stomach or duodenum on esophagogastroduodenoscopy, or mural thickening of the small bowel on abdominal ultrasonogram, CT or upper GI series. Skin biopsy revealed leukocytoclastic vasculitis in 3 of them, although biopsy specimen was taken from any areas of normal- appearing skin. In purpura-negative group, 9 patients improved by steroid therapy. Conclusion: In purpura-negative group, there is no diagnostic feature on the laboratory findings and clinical features. Therefore, to diagnose HSP gastroenteropathy in patients with abdominal pain in the absence of the characteristic rash, careful observation of clinical features and laboratory data, and prompt application of available diagnostic tools such as gastrointestinal endoscopy, radiologic study and skin biopsy are recommended. Early use of corticosteroid may reduce the suffering in these patients.

• Pediatric Infection and Vaccine
• /
• v.14 no.1
• /
• pp.62-68
• /
• 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
• /
• 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.2 no.1
• /
• pp.41-49
• /
• 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
• /
• v.24 no.1
• /
• pp.1-13
• /
• 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.

• Clinical and Experimental Pediatrics
• /
• v.46 no.11
• /
• pp.1118-1123
• /
• 2003

Purpose : Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) is a systemic vasculitis, characterized by cutaneous purpura, abdominal pain, arthralgia and renal involvement. The clinical features of HSP have been reasonably well documented but there are still many gaps in our understanding of HSP. The aim of this study was to present the clinical features of 125 children with HSP and compare them with previous reports, placing particular emphasis on clinical information. Methods : We collected the clinical data of 125 patients with acute HSP who visited Chungbuk National University Hospital from March 1992 to April 2002. Data were expressed as the mean or $mean{\pm}SD$ and statistical analysis was performed using Chi-square approximation. P<0.05 was considered as significant. Results : The patient population consisted of 87 boys and 38 girls ranging in age from one to 14 years. HSP occurs throughout the year, but this study shows seasonal skewing, with most patients presenting from fall through spring and a paucity of cases in summer. All patients had non-thrombocytopenic purpura concentrated on the buttocks and lower extremities. Purpuric lesions were also scattered on the arms and occaisionally on the face and ears, but the trunk was largely spared. A recurrence of purpura was defined as the reappearance of a rash or other symptoms following resolution of disease for at least two weeks. The mean number of recurrences was 0.51. Eighty eight patients(70.4%), 18 patients(14.4%) and 67 patients(53.6%) complained of abdomianl pain, gastrointestinal bleeding and arthralgia, respectively. Nephritis occurred in 48(38.4%) patients. Fifteen boys (17.2%) developed epididymitis. Neurologic features occurred in 13(10.4%) and two(15%) of these were seizures. Conclusion : HSP all showing purpura as defined is characterized by various clinical features, including abdominal pain, arthralgia, epididymitis and nephritis which could occur before the appearance of purpura. Therefore, we suggest that the possibility of HSP should be considered in children before invasive procedures, even if the above symptoms and signs present without purpura.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.12 no.2
• /
• pp.156-162
• /
• 2009

Purpose: The aim of this study was to investigate the clinical usefulness of upper gastrointestinal (GI) endoscopy in children with Henoch-Sch$\"{o}$nlein purpura (HSP). Methods: We retrospectively analyzed the clinical, endoscopic, and histopathologic records of children with HSP who had been admitted to the Department of Pediatrics of Gil Hospital and underwent upper GI endoscopy between January 2002 and June 2009. Patients were classified into the following two groups for statistical analysis: duodenal involvement (+) and duodenal involvement (-). Results: Fifty-one children with HSP underwent upper GI endoscopy; the mean age was 7.2${\pm}$2.9 years. The upper GI endoscopy showed abnormalities of the duodenum in 38 cases (74.5%), 22 of which had duodenal ulcers. Among the biopsy specimens obtained from the duodenum of 37 cases, 13 cases (35.1%) had leukocytoclastic vasculitis, neutrophil debri, and/or extravasation of RBCs. Steroid use was more frequent in the duodenal involvement (+) group (86.8%) than the duodenal involvement (-) group (53.8%; p=0.02). The mean length of hospitalization was 13.9${\pm}$8.43 days in the duodenal involvement (+) group and 8.1${\pm}$4.62 days in the duodenal involvement (-) group (p=0.003). The recurrence rate was significantly higher in the duodenal involvement (-) group than the duodenal involvement (+) group (p=0.027), whereas none of the other study parameters, such as the age of onset, renal involvement, and steroid use, led to significantly higher or lower recurrence rates. Conclusion: These results suggest that duodenal involvement can influence the clinical course and prognosis of HSP in children.

• Clinical and Experimental Pediatrics
• /
• v.45 no.2
• /
• pp.240-246
• /
• 2002

Purpose : It is not clear that the development of glomerular injury and aggravation by tumor necrosis factor alpha($TNF-{\alpha}$) is related to intrarenal or serum concentration of $TNF-{\alpha}$. So, we studied the relationship between the concentration of $TNF-{\alpha}$ and aggravation of glomerular damage in the Henoch-$Sch{\ddot{o}}nlein$ nephritis(HSN) and idiopathic nephrotic syndrome(INS). Methods : We collected the sera and urines of 21 patients with Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) and 22 patients with INS visited Chungbuk National University hospital from March 1998 to March 2001. The concentration of $TNF-{\alpha}$ in the sera and urines were measured by sandwich ELISA. Results : Serum $TNF-{\alpha}$ levels in the HSP patients with renal involvement were significantly higher than those without renal involvement(P=0.009). But urine $TNF-{\alpha}$ levels have no correlation with renal involvement(P=0.088). In the HSN patients, proteinuria have a significant correlation with serum $TNF-{\alpha}$ levels(P=0.004) but less correlation with urine $TNF-{\alpha}$ levels(P=0.053). Otherwise, proteinuria have no correlation with serum $TNF-{\alpha}$ levels(P=0.763) but have a significant correlation with urine $TNF-{\alpha}$ levels(P=0.007) in INS. Conclusion : These result suggest that the serum concentration of $TNF-{\alpha}$ would be important to glomerular involvement in HSP. And, it is interesting that proteinuria shows a significant relation with serum $TNF-{\alpha}$ levels in the HSN, but with urine $TNF-{\alpha}$ levels in the INS. This means the major production of $TNF-{\alpha}$ may be originated by extrarenal inflammation in the HSN and by intrarenal tubulo-interstitial damage due to proteinuria in the INS.

• Clinical and Experimental Pediatrics
• /
• v.48 no.11
• /
• pp.1244-1251
• /
• 2005

Purpose : $Henoch-Sch{\ddot{o}}nlein$ purpura(HSP) is the most common and benign systemic vasculitis in children. Few reports have focused on worse outcomes of HSP in adults. The age of onset is suggested as a main risk factor. We assessed the characteristics of adolescent-onset HSP. Methods : We retrospectively analyzed 205 cases presented from Aug. 1993 to Oct. 2003. Patients were classified as children(<10 years of age), adolescents(10-20 years of age), and adults(>20 years of age). Results : The mean age was $5.7{\pm}1.8years$ in 149 children, $13.5{\pm}2.4years$ in 38 adolescents, and $44.9{\pm}14.5years$ in 18 adults. The male to female ratio was 1.2 : 1 in children and adolescents, and 2 : 1 in adults. Previous upper respiratory infections were found in 53.4 percent of children, 32.4 percent of adolescents, and 33.3 percent of adults. Positivity of stool occult blood was more frequent in adults(50.5 percent) than in children(23.0 percent)(P<0.05). Renal involvement was found in 46 cases (30.9 percent) of children, 23 cases(60.5 percent) of adolescents, and 15 cases(83.3 percent) of adults. Recurrences occurred in 23 cases(15.4 percent) of children, nine cases(23.7 percent) of adolescents, and three cases(16.7 percent) of adults. Among the cases with renal involvement, 97.8 percent of children and 87.0 percent of adolescents improved to normal or asymptomatic urinary abnormalities. 60.0 percent of adults persisted with severe nephropathy and 13.3 percent progressed to renal insufficiency. Conclusion : Although the outcome of adolescent HSP was as good as children, the clinical manifestations were similar to those of adults. Adolescents had the highest rate of recurrences. Thus long term observations may be needed in adolescent onset HSP.

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