• Childhood Kidney Diseases
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• v.7 no.2
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• pp.166-173
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• 2003

Purpose : The cause and pathogenesis of $Henoch-Sch\"{o}nlein$ purpura has been studied for many years but the results are disappointing. Recently the hypothesis that abnormalities involving the glycosylation of the hinge region of immunoglobulin Al(IgAl) may have an important role in the pathogenesis of $Henoch-Sch\"{o}nlein$ purpura is being approved. $Henoch-Sch\"{o}nlein$ purpura is the most common vasculitis Ihat affects children and the prognosis is good. But if kidney invovement occurs, the course may be chronic and troublesome. So we evaluated children with $Henoch-Sch\"{o}nlein$ purpura especially from the point of epidemiology and clinical manifestations. Methods : Investigation of 124 children who were diagnosed with $Henoch-Sch\"{o}nlein$ purpura at Inje University Ilsan Paik Hospital from December 1999 to July 2003 was performed retrospectively through chart review. Efforts were made to get informations about the profile, epidemiology, clinical manifestations, progress of the disease and recurrence rate of patients. Results : The patients were 69 boys and 55 girls, with a mean age of $6.1{\pm}2.7$ years at the time of data collection. The male to female ratio was 1.25 : 1. The occurrence rate was much higher in autumn(from September to November, 31.5%) and winter(from December to February, 28.2%) than in spring and summer, with a peak in November. Joint involvement was shown in 66.9% of patients mostly on the foot/ankle(75.9%), knee(39.8%). Seventy(56.5%) out of 124 patients had abdominal pain and 10 patients(8.1%) showed bloody stools. Renal involvement was observed in 24 patients(19.4%) after 21.1 days on the average. IgA was elevated in 10 of 21 patients(47.6%). $C_3$ and $C_4$ levels were normal in 40 of 49 patients (81.7%) and 47 of 48 patients(97.9%), respectively Antistreptolysin-O(ASO) titer was elevated over 250 Todd units in 29 of 62 Patients(46.8%). Mycoplasma antibody titer was elevated in 21 of 49 patients(42.9%) equal or greater than 1:80. Radiologic studies were peformed in 23 patients. Seven patients(30.4%) showed bowel wall thickening and one of them received intestinal resection and anastomosis operation due to terminal ileum necrosis. Eighty four patients took steroid 1.4 mg/kg/day in average. Recurrence rate was 2.5 in 37 patients(29.8%). Conclusion : $Henoch-Sch\"{o}nlein$ purpura in childhood appears most in about 6 years of age. The occurrence rate is much higher in autumn and winter relatively. Diagnosis can be made through the perspective history taking and the inspection of clinical manifestations, but the laboratory findings are not of great help. A small portion of the patients might show abdominal pain or arthritis before purpura develops, therfore various diagnosis can be made. Radiologic evaluation should be performed to avoid surgical complications in cases accompanying abdominal pain, and long term follow up should be needed especially in patients suffering from kidney involvement. In about 30% of the patients $Henoch-Sch\"{o}nlein$ purpura would recur. Steroid can be used safely without side effects.

• Childhood Kidney Diseases
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• v.5 no.1
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• pp.59-63
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• 2001

There are a considerable number of reports suggesting a common pathogenesis of IgA nephritis(IgANn) Henoch-$Sch{\ddot{o}}nlein$ Purpura(HSP). In previous reports, a patient develops IgAN after kidney transplantation for HSP nephritis, one of Identical twin boys, developed IgAN and the other HSP, and a boy with IgAN later developed HSP. We report two cases, one with IgAN who later developed HSP and the other with HSP who later developed IgAN, suggesting that IgAN and HSP have a common pathogenesis. (J. Korean Soc Pediatr Nephrol 5 : 59- 63, 2001)

• Childhood Kidney Diseases
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• v.1 no.2
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• pp.176-178
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• 1997

Henoch-Scholein purpura is a systemic vasculitis of small blood vessels. It is characterized by nonthrombocytopenic purpura, abdominal pain, arthralgia and renal involvement. In Henoch-Scholein purpura, severe abdominal pain may be relived by steroid, but occasionally unresponsive to steroid and conventional analgesics therapy. We tried IV-gamma globulin for severe abdominal pain, unresponsive to steroid, analgesics, and antispasmodics therapy in HSP, and experienced dramatic symptomatic improvement. So we report three cases of IVIG therapy against severe abdominal pain in HSP with a brief review of literatures

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.12 no.2
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• pp.226-229
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• 2009

Gastrointestinal involvement in Henoch-Sch$\"{o}$nlein purpura (HSP) is common. However, both acute pancreatitis and acute renal failure appear to be very rare complications of HSP. We describe a case of HSP with acute pancreatitis and hypovolemic acute renal failure in a 7-year-old girl who presented with a vasculitic purpuric rash involving the lower extremities, abdominal pain, hematochezia, vomiting, and oliguria. Laboratory findings showed increased serum levels of amylase, lipase, and creatinine. An abdominal CT scan revealed diffuse enlargement of the head and body of the pancreas. The patient was successfully managed with conservative treatment, including corticosteroids, and then her pancreatic enzymes and renal function returned to normal. Acute pancreatitis should be differentiated from other causes of acute abdomen in HSP to avoid unnecessary surgery.

• Childhood Kidney Diseases
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• v.15 no.2
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• pp.184-190
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• 2011

Scrotal involvement has been reported from 2% to 38% of males with Henoch-Sch$\ddot{o}$nlein purpura (HSP). It may present before the rash occurs or even long time after it has disappeared. Scrotal involvement of HSP usually results in pain, tenderness, swelling or discoloration of scrotum. Scrotal pain sometimes mimics testicular torsion to various degrees, which can be followed by unnecessary operation. In our 2 cases, one was a 5-year and 11-month-old boy who came to our emergency department due to scrotal pain before the diagnosis of HSP, and the other was a 5-year and 1-month-old boy who came to the outpatient clinic due to scrotal pain after the resolution of HSP about 1 month before the visit. We performed Doppler ultrasonography (USG) to evaluate the acute scrotal pain in the two boys. On Doppler USG, epididymis showed increased blood flow, and testis showed normal blood flow. These findings enabled the diagnosis of acute epididymitis and excluded testicular torsion. Epididymitis was improved by conservative management including shortterm steroid therapy within 5 days. It is important to perform adequate evaluation with tools such as Doppler USG in the early course of acute scrotum of HSP, to avoid unnecessary scrotal exploration.

• Clinical and Experimental Pediatrics
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• v.45 no.3
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• pp.406-412
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• 2002

Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) is one of the most common vasculitic diseases of childhood, referred to as a leukocytoclastic vasculitis affecting small vessels. Although HSP related gastrointestinal symptoms are seen in up to 80% of patients during acute illness, these symptoms are usually transient. However, some patients with HSP have gastrointestinal major surgical complications such as intussusception, bowel infarction, necrosis, stricture, and perforation. We experienced a rare case of HSP-related ileal perforation developed after corticosteroid treatment. We report a case with HSP-related intestinal perforation and assess the effect of corticosteroid on the outcome of abdominal pain in children with HSP.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.10 no.1
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• pp.11-19
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• 2007

Purpose: Henoch-Sch$\"{o}$nlein purpura (HSP) is a systemic vasculitis involving the skin, joints, gastrointestinal tract, and kidney. Although the pathogenesis of HSP is still unclear, tumor necrosis factor (TNF-${\alpha}$) is regarded as an important cytokine contributing to the disease. The goal of this study was to determine the role of TNF-${\alpha}$ in the pathogenesis of HSP, and to evaluate the TNF-${\alpha}$ polymorphism for genetic susceptibility to HSP. Methods: From March 2004 to November 2005, 40 children with HSP and 32 healthy controls were included. Serum TNF-${\alpha}$ levels were measured using the ELISA method during the acute and convalescent phase of HSP. The genotypic and allelic frequencies of the TNF-${\alpha}$ gene polymorphisms at positions -308 and -238 were evaluated in patients and controls. Results: Serum TNF-${\alpha}$ levels were $23.17{\pm}11.31$ pg/mL in the acute phase of children with HSP and $10.56{\pm}5.59$ pg/mL in the convalescent phase (p=0.000). There was no significant correlation between the serum TNF-${\alpha}$ levels and the clinical scores of HSP (r=0.310, p=0.070). The genotypic frequency of the TNF-${\alpha}$ -308 polymorphism in children with HSP was not significantly different compared to healthy controls (GG 80%, GA 20% vs. GG 93.8%, GA 6.2%; p=0.094). The genotypic frequency of the TNF-${\alpha}$ -238 polymorphism in children with HSP was not significantly different (GG 97.5%, GA 2.5% vs. GG 93.8%, GA 6.3%; p=0.429). Conclusion: TNF-${\alpha}$ is assumed to be the main cytokine associated with the pathogenesis of HSP during the acute phase. However, the presence of TNF-${\alpha}$ gene polymorphisms at positions -308 and -238 did not distinguish children with HSP from normal controls.

• Childhood Kidney Diseases
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• v.2 no.2
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• pp.192-195
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• 1998

[ $\beta$ ]-streptococci have been reported as an etiology of Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP), but the role of $\beta$-streptococci in HSP is not yet confirmed. Our report of two HSP patients is as follows : Although the two patients' clinical symptoms resembled those symptoms of HSP, whereas serum $C_3$ decreased in one case, ASO titer increased in both cases and a histological examination of renal biopsy specimens revealed the fetures commonly described in poststreptococcal glomerulonephritis.

• Childhood Kidney Diseases
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• v.4 no.2
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• pp.161-165
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• 2000

Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) frequently follows upper respiratory infection, and one of the causes of this discase is inferred to $\beta$-hemolytic streptococcal infection, but the relationship is still unclear. familial tendency of this disease is unclear, too. Also genetic relationship of this disease has been in a controversy yet. We experienced two cases of HSP in brother and sister at similar period, and report this case with review of related literatures.

• Childhood Kidney Diseases
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• v.10 no.2
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• pp.125-131
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• 2006

Purpose : High interleukin-1 beta(IL-$1{\beta}$) expression in the skin biopsy specimens of patients with Henoch-$Sch\ddot{o}nlein$ Purpura(HSP) has been observed. We examined IL-$1{\beta}$ gene polymorphism in patients with HSP. The purpose of this study is to examine the relationship between IL-$1{\beta}$ gene polymorphism and renal involvement in HSP. Methods : Patients from mideast Korea with HSP were studied. All patients had at least 6 months of follow up. Patients and ethnically matched controls were genotyped for IL-$1{\beta}$ gene polymorphism by polymerase chain reaction-restriction fragment length polymorphism(PCR-RFLP). Results : Thirty-four patients(all younger than 15 years old) who had been diagnosed with HSP and 27 controls were examined. No allele or genotype differences between the HSP and control groups were observed. No significant association between the carriage of IL-$1{\beta}$(-511) T allele and renal involvement(P=0.525, OR:1.417, CI:0.545-3.686) was found. Conclusion : In unselected patients with HSP, carriage of IL-$1{\beta}$(-511) T allele does not appear to influence renal involvement.