• Title/Summary/Keyword: Henoch-Sch$\ddot{o}$nlein 자반증

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Henoch-Schönlein purpura associated with Mycoplasma infection (마이코플라스마 감염증과 관련된 Henoch-Schönlein 자반증)

  • Kim, Hyung Wook;Kim, Young Dae;Kim, Woo Kyung;Lee, Chong Guk
    • Pediatric Infection and Vaccine
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    • v.14 no.1
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    • pp.62-68
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    • 2007
  • Purpose : The cause and pathogenesis of Henoch-Sch$\ddot{o}$nlein purpura (HSP) are unknown, but recently the hypothesis that immunoglobulin A may have an important role in the pathogenesis of HSP is being published and HSP associated with mycoplasma infection has been also reported. The aim of this study is to discover relation between HSP and mycoplsma infection. Methods : From Jan. 2002 to Dec. 2005, we retrospectively evaluated 98 children who were diagnosed as HSP at Ilsan Paik Hospital. 84 patients were not associated with mycoplasma infection (group A) and 14 patients were associated with mycoplasma infection (group B). We compared both groups about clinical features. Results : The ratio of male to female patients in group A and B were 1.21:1 and 1.80:1. The number of patients in group A was most in November and December, and in group B was most in November. All patients had non-thrombocytopenic purpura concentrated on the buttocks and lower extremities and joint involvement was most common on the feet and ankle in both groups. 57 (67.9%) cases in group A and 9 (64.3%) cases in group B complained of abdominal pain. And 21 (25.0%) cases in group A and 5 (35.7%) cases in group B had nephritis. Elevated mycoplasma antibody titer (${\geq}1:320$) or 4 fold-rise were noted in 14 of 98 patients (14.3%). In this study, there was one child with HSP preceded by typical mycoplasma pneumonia (mycoplasma antibody titer 1:1280). Conclusion : In this study, elevated mycoplasma antibody titer (${\geq}1:320$) or 4 fold-rise were noted in 14 of 98 patients and the difference of clinical features between group A and B was not specific. The role of mycoplasma infection in the etiology of HSP may have been implicated, so the association with mycoplasma infection should have to be proved by further controlled studies.

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Clinical Features of Henoch-Schönlein Purpura Gastroenteropathy without Purpura before Diagnosis (진단 전에 자반이 동반되지 않았던 Henoch-Schönlein 자반 위장병증의 임상적 고찰)

  • Oh, Jae Min;Park, Jae Hong
    • 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.

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The Analysis of Risk Factors and Significance of Tumor Necrosis Factor(TNE)-$\alpha$ in Henoch-$Sch\ddot{o}nlein$ Nephritis (Henoch-$Sch\ddot{o}nlein$ 자반증 환아에서 신침범에 대한 위험인자 분석 및 tumor necrosis factor-$\alpha$의 의의)

  • Song Young-Jun;Ha Tae-Sun
    • Childhood Kidney Diseases
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    • v.4 no.1
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    • pp.40-47
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    • 2000
  • Purpose: Renal involvement is the most important prognostic factor of HSP. Therefore, the pathogenesis and prognostic factors in renal involvement have been studied by many researchers. The aim of this study was to evaluate the clinical risk factors and the role of TNF-$\alpha$ in renal involvement of HS purpura. Methods: The subjects of this study were 12 patients of HS purpura, 7 patients of HS nephritis, and 5 age-matched controls. We have analysed the rist factors for renal involvement in clinical symptoms and collected the sera and urines of all subjects in acute and convalescent stage. The concentration of TNF-$\alpha$ in the collected sera and urines were measured by sandwich ELISA and compared with that of age-matched controls. Results: Statistical analysis showed that persistent purpura increased the risk of developing renal involvement (P=0.0018). and serum TNF-$\alpha$ levels in the acute stage of patients with renal involvement($11.45{\pm}7.01$ pg/ml) were significantly higher than those of without renal involvement($6.32{\pm}1.31$pg/m1) and of age-matched controls($5.99{\pm}1.34$pg/m1)(P=0.012, 0.027, respectively). However, urine TNF-$\alpha$ levels have no correlation with renal involvement. On investigation of serum TNF-$\alpha$ levels in acute stage of HS purpura, persistent purpura had a significantly higher increase(P=0.038). Conclusion: Serum concentration of TNF-$\alpha$ is a risk factor and has a predictable value along with clinical risk factors, such as, persistent purpura for renal involvement in HS purpura. Also, the effectiveness of the specific treatment fur antagonizing TNF-$\alpha$ in HS nephritis may need further study.

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A Clinical Study of Childhood Henoch-Schönlein Purpura (Henoch-Schönlein 자반증 환아의 임상적 고찰)

  • Ha, Tae-Sun;Koo, Hyun-Hoe
    • 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 Significance of Gastrointestinal Symptoms and Abdominal Ultrasonographic Findings in Henoch-Schönlein Purpura (Henoch-Schönlein 자반증에서 복부 증상의 임상적 의의와 복부 초음파 소견)

  • Choi, Eun Jung;Lee, Chang Woo;Choi, Du Young
    • 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.

Intravenous immunoglobulin for severe gastrointestinal manifestation of Henoch-Schönlein purpura refractory to corticosteroid therapy (스테로이드 치료에 반응하지 않는 심한 위장관 증세의 Henoch-Schönlein 자반증 환아에서 정맥 면역글로불린 치료)

  • Yang, Hye Ran;Choi, Won Jung;Ko, Jae Sung;Seo, Jeong Kee
    • 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.

Analysis of Childhood Rapidly Progressive Glomerulonephritis (소아 급속 진행성 사구체신염의 임상적 고찰)

  • Uhm Ji Hyun;Kim Mi Jin;Lee Young-Mock;Kim Ji Hong;Lee Jae Seung;Kim Pyung-Kil;Hong Soon Won;Jeung Hyeun Joo
    • Childhood Kidney Diseases
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    • v.5 no.2
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    • pp.78-86
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    • 2001
  • Purpose: Rapidly progressive glomerulonephritis (RPGN) is characterized by the rapid increase in serum creatitnin and crescents formation involving more than $50\%$ of glomeruli. 10 patients who had been treated for RPGN were studied retrospectively for thier underlying diseases and clinical features Method: Cilinical review was performed on 10 children who were diagnosed with RPGN by clinical features and renal biopsy and followed up at department of pediatrics during tile last 10 years, from May 1990 to May 2000. Result: There were 6 males and 4 females between the ages of 2.1 and 14.3 years (mean $10.9{\pm}3.8$). 3 had Henoch-$Sch{\ddot{o}}nlein$ purpura nephritis; 2, idiopathic rapidly progressive glomerulonephritis; 2, lupus nephritis; 1, hemolytic uremic syndrome; 1, membranous glomerulonephritis and 1, microscopic polyangiitis. The most common chief complaints were gross hematuria and oliguria. Initial clinical features included proteinuria, edema, hypertension, nausea and arthralgia. Mean serum BUN was $74.2{\pm}39.1\;mg/dL$ mean serum creatinin, $3.2{\pm}1.8\;mg/dL$ and mean creatinin clearance, $26.5{\pm}13.2\;mL/min/1.73m^2$. Antineutrophil cytoplasmic antibody was positive only in microscopic polyangiitis. ANA and Anti-DNA antibody were positive in two lupus nephritis patients. Serum complements were decreased in 4 patients. All patients except Hemolytic uremic syndrome received steroid pulse therapy and immunosupressive agents. 3 patients were performed acute peritoneal dialysis and 2 patients were given plasmapheresis. At the last follow up, 1 patient was dead, 4 patients had elevated serum creatinin, 2 of these 4 patients were on chronic ambulatory peritoneal dialysis and 6 patients had normal renal function. Conclusion: Rapidly progressive glomerulonephritis is a medical emergency that requires very rapid diagnosis, classification, and therapy. Appropriate therapy selected on the basis of underlying disease mechanism can substantially improve renal survival. (J. Korean Soc Pediatr Nephrol 2001 ; 5 : 78-86)

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The Role of Colonoscopy in Children with Hematochezia (소아 선혈변에서 대장 내시경 검사의 역할)

  • We, Ju-Hee;Park, Hyun-Suk;Park, Jae-Hong
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.14 no.2
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    • pp.155-160
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    • 2011
  • Purpose: This study was performed to evaluate the role of colonoscopy in children with hematochezia. Methods: We retrospectively reviewed the medical records of 277 children who underwent colonoscopy because of hematochezia between January, 2003 and July, 2010. Results: The mean age of the patients was $6.0{\pm}4.4$ (7 days~17.8 years) years. The male to female ratio was 2.2:1. The duration between the 1st episode of hematochezia and colonoscopy was $4.9{\pm}12.1$ months. Characteristics of hematochezia included red stool (65.1%), blood on wipe (12.8%), bloody toilet (11.9%), and blood dripping (10.2%). The most proximal region of colonoscopic approach was terminal ileum (84.5%), cecum (9.5%), hepatic flexure (2.8%), and splenic flexure (3.2%). Eighty five patients (30.6%) had no specific abnormal findings. Major causes of hematochezia were polyp (26.4%), food protein induced proctocolitis (6.9%), infectious colitis (5.4%), lymphofolliculitis (5.7%), non specific colitis (5.7%), and vascular ectasia (5.1%). The hemorrhagic sites included the rectum (24.0%), rectosigmoid junction (18.1%), sigmoid colon (13.5%), ascending colon (14.2%), transverse colon (11.3%), descending colon (7.8%), cecum (8.1%), and terminal ileum (3.1%). The recurrence rate of hematochezia after colonoscopy was 19.1%. Colonoscopy was performed in 262 patients (94.6%) with conscious sedation. Endoscopic hemostasis was performed in 5 patients. Complications of colonoscopy or sedation were not found. Conclusion: The causes and lesional localization of pediatric hematochezia were diverse. Colonoscopy has an important role in the diagnosis and treatment of hematochezia in children. Total colonoscopy is recommended to detect the cause of hematochezia.