• Childhood Kidney Diseases
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• v.13 no.2
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• pp.146-152
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• 2009

Purpose : We performed this study to evaluate the incidence and clinical significance of antiphospholipid antibodies (aPL Ab) in Korean children with Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP). Methods : The medical records of 62 patients (31 boys and 31 girls) aged $46.0{\pm}3.1$ (1-16) years with a clinical diagnosis of HSP based on the EULAR/PReS criteria were reviewed retrospectively. From the years 2007 to 2009, the sera from children with acute HSP were tested for aPL Ab such as LA, anti-cardiolipin antibody and anti-${\beta}_2$ glycoprotein I antibody. Results : LA was positive in 18 (29%) of the 62 patients with HSP and We divided the patients into the two groups LA positive group (N=18) and LA negative group (N=44). There were no significant differences between the two groups with regard to abdominal pain, arthralgia and renal involvement, but LA positive group had significantly higher C-reactive protein ($4.3{\pm}7.2$ mg/dL vs. $1.3{\pm}1.8$ mg/dL, P=0.035), erythrocyte sedimentation rate ($37.5{\pm}26.2$ mm/hr vs. $25.1{\pm}22.6$ mm/hr, P= 0.039), IgM ($148.1{\pm}48.4$ mg/dL vs. $114.9{\pm}41.5$ mg/dL, P=0.024), C3 ($143.1{\pm}21.9$ mg/dL vs. $129.7{\pm}24.5$ mg/dL, P=0.048) and C4 levels ($30.9{\pm}6.3$ mg/dL vs. $24.9{\pm}7.8$ mg/dL, P=0.002) compared with LA negative group. Conclusion : We found that the incidence of positive aPL Ab tests was relatively higher in Korean children with HSP and the presence of aPL Ab was associated with acute inflammatory process of HSP. These results suggest that the aPL Ab are involved in the pathogenesis of HSP in children.

• 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.4 no.2
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• pp.228-232
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• 2001

Gastrointestinal involvement occurs in two thirds of children with Henoch-Sch$\"{o}$nlein purpura (HSP) and intussusception is by far the most common abdominal complication. Intussusception in HSP almost originates in the small bowel, which is in contrast with idiopathic intussusception. Earlier diagnosis and prompt treatment of intra-abdominal complications can reduce the mortality and ultrasound is the imaging modality of choice in evaluation the bowel manifestations of HSP. We report a case of jejuno-jejunal intussusception associated with HSP in a 5-year-old boy who presented with diffuse abdominal pain and vomiting after a few days of HSP onset. Abdominal ultrasound demonstrated intussusception in the jejunum with well defined target appearance because of the thickened intussusceptum, which disappeared on the computerized tomographic examination checked approximately 24 hours later. A brief review of literature was made.

• 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.

• Journal of Yeungnam Medical Science
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• v.25 no.1
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• pp.58-63
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• 2008

Henoch-Sch$\ddot{o}$nlein purpura (HSP) is a leukocytoclastic vasculitis of small vessels with deposition of IgA, commonly resulting in skin, joint, gastrointestinal, and kidney involvement. HSP is an uncommon disorder in adults and accounts for 0.6% to 2% of adult nephropathy. We report a case of HSP with acute renal failure successfully treated with corticosteroid. In this case, the patient presented with vasculitic purpuric rash on lower extremity, arthralgia in the wrist, abdominal pain, hematochezia, oliguria and azotemia. Abdominal CT showed wall thickening of the small and large bowels. Skin biopsy revealed leukocytoclastic vasculitis. Percutaneous renal biopsy showed no crescent formation, but mesangial IgA and $C_3$ deposits were observed by immunofluorescence. The patient was treated with corticosteroid (1mg/kg per day) and hemodialysis. After treatment, renal function improved and purpuric lesion, arthralgia and abdominal pain disappeared. Thus, when adults present with purpuric rash and rapidly progressive glomerulonephritis (RPGN), HSP should be a diagnostic consideration.

• Childhood Kidney Diseases
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• v.14 no.2
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• pp.230-235
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• 2010

Patients with moderate to severe degrees of Henoch-Sch$\ddot{o}$nlein purpura (HSP) nephritis receive high-dose intravenous methylprednisolone pulse therapy (IMPT). Although the regimen is generally safe and effective, various complications occasionally develop. administration of excessive corticosteroid can induce urinary potassium wasting leading to hypokalemia. Polyuria, one of the complications of hypokalemia, is related to both increased thirst and mild nephrogenic diabetes insipidus. And hypokalemia itself also impairs the maximal renal urinary concentration ability. Although polyuria or nocturia after IMPT is not common, it is correctable immediately by oral potassium supplementation. Therefore, during IMPT, careful history taking of nocturia as well as monitoring urine volume, serum and urine potassium level at regular follow-up are necessary because even mild hypokalemia can provoke urine concentrating ability defect. We experienced a case of 11 year-old boy with HSP nephritis who suffered from hypokalemia-induced polyuria with nocturia right after IMPT.

• Childhood Kidney Diseases
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• v.4 no.2
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• pp.111-119
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• 2000

Purpose : Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) is a common pediatric discase presenting most frequently with skin, gastrointestinal, joint and renal manifestations. The prognosis of HSP is mainly determined by the involvement of the kidney, but prognostic markers have not been established. We evaluated the patients who have HSP nephritis with nephrotic syndrome. Method : Clinical manifestations and laboratory findings were observed and analyzed in 34 cases with HSP which were manifested by nephrotic syndrome hospitalized at Kyung Hee university Hospital during the period from Jan. 1990 to Dec. 1998. Results : 1) Male to female ratio was 1.3:1, and mean age at onset was 8.3 year. 2) Mean duration from symptom onset to renal biopsy was 10.5 weeks. 3) Proportion of patients presenting with acute nephritis was 32.4$\%$, gross hematuria 17.6$\%$, microscopic hematuria 50$\%$. 4) The findings of renal biopsy were 20 cases of grade II, 11 cases of grade III, 2 cases of grade I, 1 case of grade IV according to classification by ISKDC. 5) Patients with grade I were recovered with no residual defect, but patients with grade IV shows active renal disease(states C). Conclusion : Among the 디le patients with Henoch-$Sch{\ddot{o}}nlein$ purpura accompanying nephrotic syndrome, more aggressive treatment might be needed in patients showing crescents formation on renal biopsy. A prospective study will be needed to explore the progression of this disease.

• Childhood Kidney Diseases
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• v.11 no.1
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• pp.106-111
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• 2007

[ $Henoch-Sch\ddot{o}nlein$ ] Purpura(HSP) is a form of vasculitis that typically affects small arteries in the skin, joints, intestinal tract and kidneys. It usually resolves spontaneously but sometimes can cause serious problems in the kidneys and intestinal tract. A 6-year-old girl with purpura, arthralgia and abdominal pain for 2 weeks was admitted. She also showed gross hematuria, generalized edema and decreased urine output. Blood pressure was in the upper normal range. Initial laboratory findings showed hypoalbuminemia, hyperlipidemia, microhematuria and nephrotic-range proteinuria(27.2 g/day). Initially, she was treated with pulse methylprednisolone, azathioprine, albumin and furosemide. Her renal biopsy revealed diffuse mesangial proliferation with strong IgA deposition. There were no crescents. On the third hospital day, she complained of severe abdominal pain and free peritoneal air was seen on abdominal X-ray. Primary repair of small bowel was performed and two pin-point sized holes were found. One week later, she still showed heavy proteinuria. Therefore, we added an ACE inhibitor and dipyridamole, and changed azathioprine to cyclosporine. One month later, the urine protein/creatinine ratio was decreased to 17.8 from 57, but heavy proteinuria has been still persisted. Here we report a rare case of a patient with HSP who had both severe nephrritc-nephrotic syndrome and small bowel perforation.

• Childhood Kidney Diseases
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• v.1 no.2
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• pp.130-135
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• 1997

Purpose : Interleukin-6(IL-6), a multifunctional cytokine, has been found to have growth and differentiation activities on a wide variety of tissues and cells, including mesangial cells. It has been known that IL-6 is an autocrine growth factor for the proliferation of mesangial cells. Several studies have been performed for revealing the clinical significance of IL-6 in mesangial proliferative glomerulonephritis. Methods : The author measured serum and urinary IL-6 in 30 patients with $Henoch-Sch\"{o}nlein$ purpura (HSP), 18 patients with HSP nephritis, and 10 normal children as a control group. Results : The serum level of IL-6 was increased significantly in the patients with HSP and HSP nephritis compared to normal control. The level of urinary IL-6 was increased significantly in the patients with HSP nephritis compared to both HSP and normal control groups. The level of urinary IL-6 was not correlated with the level of serum. Conclusion : IL-6 was correlated with the amount of proteinuria. These data suggest that IL-6 may be involved in the pathological proliferation of mesangial cell in HSP nephritis.

• Clinical and Experimental Pediatrics
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• v.48 no.1
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• pp.81-84
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• 2005

Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP) is a systemic disorder characterized by leukocytoclastic vasculitis that can affect multiple organs predominantly the skin, joints, gastrointestinal tract and kidney. Although the specific pathogenesis of HSP is not known, there are several hypotheses. Although the importance of the complement activation in glomerular injury in HSP has been suggested, the complement levels and the blood pressure in those patients are usually normal and massive proteinuria is not common. And pathologic renal changes also have been reported to show a large variety of glomerular changes. However, to our knowledge, a membranoproliferative glomerulo-nephritis (MPGN) is a rare renal clinicopathologic manifestation of HSP. We report a 6-year-old boy with HSP who developed MPGN with hypertension, massive proteinuria, and hypo-complementemia revealed activation of the classical complement pathway, although we could not exclude the possibility of other hypocomplementemic glomerulonephritis including post-streptococcal acute glomerulonephritis.