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

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

• Advances in pediatric surgery
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• v.6 no.1
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• pp.50-55
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• 2000

Pathologic lead points are found in a few intussusception patients. To evaluate the pathologic lead points in childhood intussusception, a retrospective review of 227 operated cases of intussusception treated at the Yeungnam University Hospital from January 1986 to April 1999. The patients were divided into 2 groups; idiopathic group 209 cases, (92.1 % and lead points group 18 cases, 7.9 %). Intussusception developed between age two months and six months in both groups. Enteroenteric type of intussusception was relatively more frequent in the lead point group than in idiopathic group. The lead points were veil (10 cases, 52.6 %), Meckel's diverticulum(3 cases, 15.8 %), lymphoma(3 cases, 15.8 %), ectopic pancreas(2 cases, 10.5 %), Henoch-Sch$\ddot{o}$nlein purpura(1 cases, 5.3 %). The bowel resection rate was 44.4 % in the lead point group and 8.6% in idiopathic group. The recurrence rate was 5.56 % in lead points group and 1.44 % in idiopathic group.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.175-182
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• 2005

Purpose : Interleukin 1 receptor antagonist(IL-1ra) is an endogenous antiinflammatory agent that binds to IL-1 receptor and thus competitively inhibits the binding of IL-1$\alpha$ and IL-1$\beta$. Allele 2 in association with various autoimmune diseases has been reported. In order to evaluate the influence of IL-1ra gene VNTR polymorphism on the susceptibility to HSP and its possible association with disease severity, manifested by severe renal involvement and renal sequelae, we studied the incidence of carriage rate and allele frequency of the 2 repeats of IL-1ra allele 2($IL1RN^{*}2$) of the IL-1ra gene in children with HSP with and without renal involvement. Methods : The IL-1ra gene polymorphisms were determined in children with HSP with(n=40) or without nephritis(n=34) who had been diagnosed at Busan Paik Hospital and the control groups(n=163). Gene polymorphism was identified by PCR amplification of the genomic DNA. Results : The allelic frequency and carriage rate of $IL1RN^{*}1$ were found most frequently in patients with HSP and in controls. The allelic frequency of $IL1RN^{*}2$ was higher in patients with HSP compared to that of controls($4.7\%\;vs.\;2.5\%$, P=0.794). The carriage rate of $IL1RN^{*}2$ was higher In patients with HSP compared to that of controls($8.1\%\;vs.\;6.8\%$, P=0.916). The allelic frequency of $IL1RN^{*}2$ was higher in patients with HSP nephritis compared to that of HSP($5.3\%\;vs.\;2.9\%$, P=0.356). The carriage rate of $IL1RN^{*}2$ was higher in Patients with HSP nephritis compared to that of HSP($10.0\%\;vs.\;5.9\%$, P=0.523). Among 13 patients with heavy proteinuria(>1.0 g), 11 had $IL1RN^{*}1$, 1 had $IL1RN^{*}2$ and the others had $IL1RN^{*}4$. At the time of last follow up 4 patients had sustained proteinuria and their genotype was $IL1RN^{*}1$. Conclusion : The allelic frequency and carriage rate of $IL1RN^{*}1$ were found most frequently in patients with HSP and in controls. Our study suggests that the carriage rate and allele frequency of the 2-repeats of IL-1lra allele 2($IL1RN^{*}2$) of the IL-1ra gene may not be associated with susceptibility and severity of renal involvement in children with HSP (J Korean Soc Pediatr Nephrol 2005;9:175-182)

• Clinical and Experimental Pediatrics
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• v.48 no.1
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• pp.63-67
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• 2005

Purpose : Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) is a systemic vasculitis, characterized by cutaneous palpable purpura, gastrointestinal(GI) symptoms, arthritis and renal involvement. In general, the prognosis is determined by GI complication as well as the severity of nephritis. In this study, we analyzed the statistical relationship between the GI symptom and other clinical findings for assessing the prognosis, and evaluated abdominal ultrasonographic findings for early diagnosis of this disease with atypical clinical presentation and early detection of serious GI complications. Methods : One hundred seventy seven patients with HSP in the Department of Pediatrics, Wonkwang University Hospital from January 1994 to June 2004, were enrolled. We retrospectively analyzed charts about clinical and abdominal ultrasonographic findings, and classified our patients into two groups(GI-Sx(-), GI-Sx(+)) for statistical analysis. Results : The ratio of female to male is 1.5 : 1. The peak age incidence was five to eight years in 95 cases(53%). The GI symptoms appeared in 117 cases(66%), which include abdominal pain 115 (98 %), tenderness 45(38%), nausea and vomiting 35(30%), bloody stool 10(8.5%), diarrhea four(3.4%), rebound tenderness four(3.4%), and also intussusception and appendicitis were complicated in five and two cases respectively. GI-Sx(+) group had an increased risk of renal involvement and relapse than the GI-Sx(-) group. But there were no relationships about sex and age incidence, or other clinical and laboratory findings between two groups. Ultrasonographic findings in 98 patients with GI symptoms included small bowel thickening in 70 cases(71%) in which duodenum, jejunum and ileum were involved in 71%, 45.7%, 40% respectively, small bowel dilatation in 41 cases(42%), lymph node swelling in 46 cases(47%), and ascites in 25 cases(25.5%). Conclusion : GI symptoms in patients with HSP suggested increased risk of renal involvement and relapse. Abdominal ultrasonography could be helpful in the early diagnosis on atypical clinical presentation and early detection of serious GI complication in these patients.

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

• Clinical and Experimental Pediatrics
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• v.49 no.7
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• pp.784-789
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• 2006

Purpose : $Henoch-Sch{\ddot{o}}nlein$ purpura(HSP) is a systemic vasculitis involving small vessels of skin, gastrointestinal(GI) tract and kidney. Digestive involvement of HSP can be serious with massive GI bleeding, perforation, and intussusception. However, some patients do not respond to conventional corticosteroid therapy. In this study, we investigated the efficacy of intravenous immunoglobulin (IVIG) for serious digestive manifestations not responding to steroid. Methods : From April 1999 to January 2005, 22 children diagnosed as HSP with severe GI symptoms were included. Initially, all patients were treated with intravenous methylprednisolone. IVIG 2 g/kg of body weight was infused in children refractory to steroid therapy. Clinical data were reviewed retrospectively. Results : Among 22 children, 12 children underwent IVIG therapy. The mean duration of corticosteroid therapy was $5.61{\pm}4.9$ days before IVIG therapy, and 11 of 12 patients experienced disappearance of GI manifestations after the initiation of IVIG infusion. In one patient, IVIG was ineffective in relieving abdominal pain, but melena subsided. Comparison of the duration of hospitalization between IVIG group and corticosteroid group revealed no significant difference($12.8{\pm}7.6$ days vs. $13.2{\pm}7.8$ days, P=0.777). But, the total duration of abdominal pain decreased in IVIG group although the difference between two groups was not significant($8.8{\pm}8.1$ days vs. $14.8{\pm}16.9$ days, P=0.306). Among 10 children treated with steroid only, 2 children were operated for bowel perforation and intussusception. In contrast, there was no perforation in 12 children who underwent IVIG therapy. Conclusion : IVIG could be the alternative therapy to corticosteroids in children with severe digestive manifestations of HSP.

• Childhood Kidney Diseases
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• v.4 no.2
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• pp.120-126
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• 2000

Purpose : This retrospective study has been undertaken to find out the clinical outcome of children with HS nephntis and its relationship with initial clinical presentation and/or renal pathologic finding. Patients and methods : Study population consisted of 59 children with HS nephritis who have been admitted to the Pediatric department of Kyungpook University Hospital from 1987 to 1999, and biopsy was done with indications of heavy proteinuria (> 1 g/m2/day) lasting over 1 month, nephrotic syndrome, and persistent hematuria and/or proteinuria over 1 year. Patients were divided clinically into 3 groups ; isolated hematuria, hematuria with proteinuria and heavy proteinuria (including nephrotic syndrome). Biopsy findings ore graded from I-V according to International Study of Kidney Disease in Children (ISKDC). Results : Mean age of presentation was $8.1{\pm}3.0$ years and slight male preponderance m noted (33 boys md 26 girls). Histopathologic grading showed Grade I ; 2, Grade II ; 44, and Grade III ; 13 cases. Clinical outcome at the follow-up period of 1-2 year (49 cases) and 3-4 years (30 cases) shooed normal urinalysis in 75 (30.6$\%$) and 18 cases (60.0$\%$), persistent isolated hematuria in 20 (40.8$\%$) and 2 cases (6.7$\%$), hematuria with proteinuria in 11 (22.5$\%$) and 8 cases (26.6$\%$), and persistent heavy proteinuria in 3 (6.1$\%$) and 2 cases (6.7$\%$) respectively. Clinical outcome according to histopathologic grading showed the frequency of normalization of urinalysis being lower in Grade III compared to grade I or II. Clinical outcome according to initial clinical presentation showed no relationship to the normalization or urinalysis at follow-up periods. However, 15-20$\%$ of children with initial heavy proteinuria showed persistent heavy proteinuria (3 out of 20 cases at 1-2 years, and 2 out of 10 case at 3-4 years of follow-up periods). Conclusion : The majority of children with HS nephritis (histopathologic grade I, II, III) improved within 3-4 years and persistent heavy proteinuria was seen only in a kw of children with initial clinical presentation of heavy proteinuria.

• Childhood Kidney Diseases
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• v.10 no.2
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• pp.174-181
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• 2006

Purpose : To analyse the results of the renal biopsies and the clinical diagnoses of patients who had undergone percutaneous kidney biopsies in the department of pediatrics at Kyunghee University Hospital for 22 years from 1984 to 2005. Methods : We retrospectively reviewed the medical records of 1559 patients and analyzed the chief complaints that led to a renal biopsy, age, sex, histopathologic findings and diagnosis. Routine kidney biopsies were performed by automated gun biopsy guided by real time ultrasonography. The diagnoses were made based on the specimen's light microscopy, immunofluorescence microscopy and electron microscopy findings and clinical symptoms and signs. Results : The mean age of the patients was 10 years with the male to female ratio being 1.3:1. The chief complaints that led to a renal biopsy included hematuria only(753 cases, 48.3%), proteinuria only(125 cases, 8.0%) and hematuria combined with proteinuria(537 cases, 34.4%). The most frequent histopathological finding was primary glomerular disease(75.4%) which included IgA nephropathy(30.1%) and mesangial proliferative glomerulonephritis(27.6 %). Systemic disease comprised 11.4% which included Henoch-$Sch\ddot{o}nlein$ nephritis(10.5%) and lupus nephritis(0.8%). Alport syndrome was found in 1.1% of cases which was attributed to hereditary causes. 628 children(40.3%) visited the clinic due to abnormal school urine screening abnormalities and among these, 237 children had mesangial proliferative glomerulonephritis and 234 children who had IgA nephropathy were managed thereafter. Conclusion : IgA nephropathy and mesangial proliferative glomerulonephritis were the two major forms of primary glomerulonephritis found in Korean children who had kidney biopsies from 1984 to 2005.

• Clinical and Experimental Pediatrics
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• v.55 no.4
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• pp.136-142
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• 2012

Purpose: Urinary excretion of N acetyl-beta-D glucosaminidase (NAG) and ${\beta}_2$-microglobulin (${\beta}_2$-M) was increased in the presence of proximal tubular damage. Based on these urinary materials, we investigated the ability of expecting renal function in chronic glomerular diseases. In this study, we evaluated the relationship between glomerular filtration rate (GFR) urinary NAG, and urinary ${\beta}_2$-M. Methods: We evaluated 52 children with chronic kidney disease at the Chung-Ang University Hospital between January 2003 and August 2009. We investigated the 24-hour urinalysis and hematologic values in all 52 patients. Serum creatinine, creatinine clearance (Ccr), serum cystatin C, urinary ${\beta}_2$-M and urinary NAG were measured. Results: Out of 52 patients, there were 13 children with minimal change in disease, 3 children with focal segmental glomerulosclerosis, 17 children with immunoglobulin A nephropathy, 15 children with Henoch-Sch$\ddot{o}$nlein purpua nephritis, 3 children with poststreptococcal glomerulonephritis, and 1 child with thin glomerular basement membrane disease. In these patients, there were significant correlation between the Ccr and urinary NAG (r=-0.817; $P$ <0.01), and between the GFR (as determined by Schwartz method) and urinary NAG (r=-0.821; $P$ <0.01). In addition, there was a significant correlation between the GFR (as determined by Bokencamp method) and urinary NAG (r=-0.858; $P$ <0.01). Conclusion: In our study, there was a significant correlation between the GFR and urinary NAG, but there was no correlation between the GFR and urinary ${\beta}_2$-M, suggesting that the GFR can be predicted by urinary NAG in patients with chronic glomerular disease.