• Tuberculosis and Respiratory Diseases
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• v.63 no.6
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• pp.531-536
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• 2007

Wegener's granulomatosis is a systemic vasculitis of the medium and small arteries, as well as of the venules, arterioles, and occasionally large arteries, and primarily involves the upper and lower respiratory tracts and the kidneys. Renal symptoms of Wegener's granulomatosis are indistinguishable from those of vasculitis such as Henoch-$Sch\ddot{o}nlein$ purpura and microscopic polyangiitis. This case, though initially diagnosed as Henoch-$Sch\ddot{o}nlein$ purpura, was confirmed as Wegener's granulomatosis from a lung biopsy fifteen years after the initial diagnosis. We report this case with a review of the literature.

• 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.

• 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.7 no.1
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• pp.73-76
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• 2003

A girl aged 21 months with $Henoch-Sch\"{o}nlein$ purpura(HSP) developed heavy proteinuria with hematuria 8 days after the appearance of purpuric rash, swelling and tenderness of both ankle joints. Her clinical and laboratory features demonstrated nephrotic and nephritic syndrome. The percutaneous renal biopsy revealed diffuse mesangial proliferative glomerulonephritis. Unlike usual HSP nephritis, immunoglobulin A deposition was not detected in the mesangium or the capillary of the glomeruli. Instead, numerous subepithelial electron-dense deposits('humps') mimicking acute poststreptococcal glomerulonephritis were found.

• Childhood Kidney Diseases
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• v.12 no.2
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• pp.164-169
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• 2008

Purpose : There have been some reports on the prevalence of positive antineutrophil cytoplasmic antibody(ANCA) in Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP), but the results were conflicting. We performed this study to evaluate the clinical significance of ANCA(c-ANCA and p-ANCA) in Korean children with HSP. Methods : The medical records of 30 patients(13 boys and 17 girls) aged 6.0$\pm$1.9(5-12) years with a clinical diagnosis of HSP based on the EULAR/PReS criteria were reviewed retrospectively. From the years 2007 to 2008, the sera from children with acute HSP were tested for antineutrophil cytoplasmic antibodies(ANCA). The target antigens of these autoantibodies are proteinase 3(c-ANCA) or myeloperoxidase(p-ANCA). Results : Palpable purpura was seen in all 30 patients(100%), abdominal pain in 20(67%), arthralgia in 17(57%), and renal involvement in 11(37%). Laboratory findings showed leukocytosis in 4 patients(13%), thrombocytosis 18 in(60%), and elevated erythrocyte sedimentation rate in 18(60%). Anti-streptolysin O titers were elevated in 7% of the patients and no patient showed elevation of serum IgA level. The sera from 29 patients were negative for c-ANCA and p-ANCA by indirect immunofluorescence, but only one patient had weakly positive results, which became negative at follow-up. Conclusions : We conclude that c-ANCA or p-ANCA is not an important serologic marker in children with HSP, because it was neither diagnostically nor immunologically specific in children with HSP. These results suggest that ANCA are not involved in the pathogenesis of HSP in children.

• Childhood Kidney Diseases
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• v.10 no.1
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• pp.40-44
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• 2006

[ $Henoch-Sch{\"{o}}nlein$ ] purpura(HSP) is a systemic small vessel vasculitis characterized by cutaneous purpura, arthritis, abdominal pain and nephritis. The characteristic rash of HSP consists of palpable purpura on the buttocks and lower extremities. Bullous lesions often appear in adults with HSP, whereas they are very rare in children with HSP. In this case report, the patient presented with arthralgia and abdominal pain and had hemorrhagic bullae as a prominent manifestation of the disease. The skin biopsy of the patient revealed typical leukocytoclastic vasculitis of dermal vessels and prominent IgA and fibrinogen deposits on capillary walls by direct immunofluorescence. We confirmed the diagnosis of HSP and observed improvement of clinical symptoms and signs within a few days after corticosteroid treatment. We therefore report a case with a review of the literature.

• 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.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.

• 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.