• Childhood Kidney Diseases
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• v.19 no.1
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• pp.39-42
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• 2015

Henoch-Sch$\ddot{o}$nlein purpura can result from exposure to an antigen after infection with several types of organisms. However, Henoch-Sch$\ddot{o}$nlein purpura caused by a primary Epstein-Barr virus infection has been rarely reported. Here, we report the case of a 32-month-old female patient who presented with Henoch-Sch$\ddot{o}$nlein purpura. Based on abnormal liver function test results and positive results for Epstein-Barr virus infection markers, a diagnosis of Epstein-Barr virus hepatitis manifesting as Henoch-Sch$\ddot{o}$nlein purpura was made. Treatment with methyl-prednisolone and hydration improved the symptoms, and a switch to oral steroids was effective in completely alleviating the purpura. No recurrence was noted and no liver function abnormalities were detected during the follow up period.

• Childhood Kidney Diseases
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• v.16 no.2
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• pp.132-137
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• 2012

Microscopic polyangiitis (MPA) is systemic small vessel vasculitis that is very rare in childhood. MPA is characterized by pauci-immune necrotizing small vessel vasculitis without clinical or pathological evidence of necrotizing granulomatous inflammation. Approximately 90% of patients have glomerulonephritis that is accompanied by a variety of other organ involvement. A 10-year-old girl visited our clinic with clinical manifestations suggestive Henoch-Sch$\ddot{o}$nlein purpura nephritis such as purpuric skin rash, abdominal pain, arthralgia on both knees, massive proteinuria and microscopic hematuria. So initially we suspected Henoch-Sch$\ddot{o}$nlein purpura nephritis. However, later her perinuclear-antineutrophil cytoplasmic antibodies(p-ANCA) test was positive, and her renal biopsy was consistent with microscopic polyangiitis. We began steroid therapy, combined with cyclophosphamide, ACE inhibitor. Currently she is a 12-year old, and until now she has been regularly examined in the outpatient. We report a case of microscopic polyangiitis initially suspected with Henoch-Sch$\ddot{o}$nlein purpura nephritis.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.86-90
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• 1997

The author experienced a case of $Henoch-Sch\"{o}nlein$ purpura with epididymitis in a 6-year-old boy who was admitted to our hospital due to left scrotal pain and the relapse of purpura on lower extremities for 3 days. Scrotal ultrasonography on admission revealed homogenously enlarged left epididymal head and small amount of fluid collection in left tunical space. The size of left epididymal head decreased gradually with no more evidence of fluid collection on day 7 and recovered completely on day 21. The author reports a case of $Henoch-Sch\"{o}nlein$ purpura with epididymitis with brief review of related literatures.

• Advances in pediatric surgery
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• v.6 no.1
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• pp.45-49
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• 2000

Henoch-Sch$\ddot{o}$nlein purpura is a systemic vasculitis of unknown etiology that is probably related to an autoimmune phenomenon. Henoch-Sch$\ddot{o}$nlein purpura is characterized by a purpuric rash, arthralgia, nephritis, and gastrointestinal manifestations. We reviewed 169 children hospitalized with a diagnosis of Henoch-Sch$\ddot{o}$nlein purpura between 1989 and 1998. One-hundred thirty-nine (82.2 %) had gastrointestinal findings including abdominal pain, nausea, vomiting and gastrointestinal bleeding. Surgical consultations were obtained for ten children, and laparotomy was performed in five. Three children suspected of having appendicitis underwent appendectomy. None had appendicitis. One child thought to have been intestinal strangulation was found to have hemorrhagic edema of the proximal jejunum and of the distal ileum. Another child underwent resection for an hemorrhagic infarct of the distal ileum. A high index of suspicion of this disease entity in the differential diagnosis of abdominal pain in children can avoid unnecessary laparotomy in most cases. However, life-threatening gastrointestinal complications may occur in low percentage of cases. Prompt recognition and adequate radiologic evaluation of the abdominal manifestation of this entity is necessary for early surgical intervention.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.82-85
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• 1997

The incidence of nephritis associated with Henoch-Sch nlein purpura varies, but glomerulonephritis consistently accounts for most of the associated morbidity and mortality. A very small number of Henoch-Sch nlein purpura develop rapidly progressive glomerulonephritis. A three-year old male patient who showed acute nephritic nephrotic syndrome developed abdominal pain, arthralgia and multiple purpurae on lower extremities later. Peritoneal dialysis was done at the 6th hospital day and continued for 7 months. Renal biopsy disclosed crescentic glomerulonephritis (with 81% crescent formation) and methylprednisolone pulse therapy was done. These days, his general condition is good, but serum creatinine levels are 1.2-1.3 mg/dL. This case was reviewed briefly with the literatures.

• 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.7 no.1
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• pp.30-37
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• 2003

Purpose : $Henoch-Sch\"{o}nlein$ purpura(HSP) is usually a self-limited disease with a good eventual outcome. The prognosis of HSP is mainly determined by the renal involvement. In this study, We evaluated children with biopsy-proven $Henoch-Sch\"{o}nlein$ purpura nephritis about the clinical outcome correlated with renal manifestation and morphologic findings. Methods : The clinical features, initial laboratory and pathologic findings, and clinical out-come were evaluated in 60 children with biopsy-proven $Henoch-Sch\"{o}nlein$ purpura nephritis at Yonsei University Severance Hospital during the period from Jan. 1990 to Dec. 2002. Results : The ratio of male to female patients was 1.2:1. The interval between the onset of $Henoch-Sch\"{o}nlein$ purpura and renal manifestation was less than 3 months in 81% of the patients. Initial renal manifestation was microscopic hematuria in 100% of patients, isolated hematuria in 15%, acute nephritic syndrome in 7%, nephrotic syndrome In 22% of patients. Renal manifestation correlated with clinical outcome. Grade II and III were the most common in histologic grades of ISKDC. Renal pathologic finding correlated with clinical outcome. Conclusion : Renal manifestation and pathologic findings correlated with the clinical out-come. It is necessary to evaluate the correlation between pathologic findings and treatment.

• Clinical and Experimental Pediatrics
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• v.48 no.2
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• pp.224-227
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• 2005

Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) is an IgA mediated immune complex vasculitic disease characterized by non-thrombocytic purpura, arthritis, gastrointestinal manifestations, and glomerulonephritis. HSP related glomerulonephritis induces hypoproteinemia and edema in some cases. Protein-losing enteropathy is another rare but known manifestation of HSP leading to hypoproteinemia and edema. We report a 6-year-old girl with HSP who showed edema caused by intestinal protein loss, evidenced by elevated fecal alpha 1 antitrypsin clearance.

• Pediatric Infection and Vaccine
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• v.4 no.2
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• pp.271-275
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• 1997

We experienced a case of Henoch-Sch$\ddot{o}$nlein purpura associated with Mycoplasma pneumoniae pneumonia in a 28 month old male who suffered from cough, abdominal pain and both leg swelling and pain. Physical examination showed varying sized purpura, characteristic of Henoch-Sch$\ddot{o}$nlein purpura, below both knee. Laboratory test revealed Mycoplasma pneumoniae antibody titer >1:2,560 and cold agglutinins titer 1:64. Chest X-ray showed peribronchial blurring in both lung fields. The patient was treated with midecamycin and prednisolone for 7 days and responded to the treatment well. The authors report a case of Henoch-Sch$\ddot{o}$nlein purpura with Mycoplasma pneumoniae pneumonia with brief review of related literatures.

• 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.