• Childhood Kidney Diseases
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• v.21 no.2
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• pp.61-68
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• 2017

Purpose: To investigate differences in clinical features, blood/urinary findings, and prognosis in different age groups of patients with Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP). Methods: A total of 469 patients with HSP were analyzed retrospectively from June 2003 to February 2016. We classified patients into child or adult groups based on their age. Results: The adult group had more patients with anemia (child vs. adult; 7.5% vs. 16.4%), and higher immunoglobulin A (IgA) (30.0% vs. 50.0%) levels, C-reactive protein (34.2% vs. 54.0%) and uric acid (3.1% vs. 12.1%) levels than the child group. The child group was highly positive for Mycoplasma pneumoniae immunoglobulin M (IgM) (34.4%). More patients in the child group presented with high levels of antistreptolysin O (24.7% vs. 2.9%) and high C4 (11.5% vs. 4.2%). Low C3 (1.1% vs. 10.2%) levels, and renal involvement with gross hematuria (8.6% vs. 21.5 %), nonnephrotic proteinuria (1.1% vs. 11.2%), and nephrotic syndrome (1.1% vs. 6.0%) were common in the adult group. Adults also had poorer renal outcomes [persistent hematuria/proteinuria (10.5% vs. 32.8%), and chronic kidney disease (0% vs. 11.2%)] than the child group. Risk factors for renal involvement such as older age and higher level of uric acid were only found in the child group. The risk factors for poor renal outcome were nephrotic syndrome in the child group and gross hematuria in the adult group. Conclusion: In this study, child and adult groups presented with different clinical manifestations of HSP. We found that risk factors for renal involvement included age and high uric acid level in the child group. Moreover, nephrotic syndrome in the child group and gross hematuria in the adult group increased the risk of poor renal outcome.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.157-165
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• 2003

Purpose : $Henoch-Sch\"{o}nlein$ purpura(HSP) is a systemic vasculitis that involves multiple organs, especially the kidney, which is the most important organ in determining the prognosis of the disease. The morbidity of HSP nephritis in adults is low and there have been little research done on its clinical course so far. Therefore, we have compared the clinical course of HSP nephritis in children and adults in Korea. Methods : We retrospectively analyzed 81 cases of HSP nephritis in children younger than 15 years of age, and 25 cases of adults older than 15 years of age who were admitted to Yonsei University Medical College Severance Hospital from Jan. 1986 to May 2003. Results : The male to female ratio was 1.5 : 1 in children and 1.3 : 1 in adults. The incidence of HSP nephritis for both age groups was found to be increased during the autumn and winter. Infection was the predisposing factor in 39 cases(48.1%) of children, 16 cases(64.0%) of adults, and drugs were the predisposing factor in 8 cases(9.9%) of children and 4 cases (16.0%) of adults. All patients initially presented with microscopic hematuria. Thirteen cases (16.0%) of children and 7 cases(28.0%) of adults initially showed proteinuria of nephrotic range. Thirty four cases(42.0%) of children and 4 cases(16.0%) of adults showed normal urinalysis after treatment. Asymptomatic urinary abnormalities were found in 41 cases(50.6%) of children and 18 cases(72.0%) of adults. Complications such as nephrotic syndrome and hypertension were found in 3 cases(3.7%) of children and 2 cases(8.0%) of adults. Three children(3.7%) and 1(4.0%) adult required dialysis or renal transplantation. Follow-up renal biopsies were performed on 21 children, of whom 10 cases(47.6%) did not show any histologic change, 9 cases(42.9%) showed low grade changes, and 2 cases(9.5%) showed high grade changes. Prognosis was gloomy when proteinuria of nephrotic range and high grade of abnormal histology were present at diagnosis, and there was no significant difference between the two groups(P<0.05) Conclusion : This study showed that there was no difference in terms of the clinical features and courses between the children and adults with HSP nephritis. Proteinuria of nephrotic range and the severity of abnormal histologic changes at diagnosis were found to be associated with a bad prognosis, therefore we recommend that patients with these features require long term follow-up and management.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.18 no.1
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• pp.39-47
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• 2015

Purpose: To determine clinically useful biochemical markers reflecting disease activity and/or gastrointestinal (GI) tract involvement in Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP). Methods: A total of 185 children with HSP and 130 controls were included. Laboratory data indicating inflammation, standard coagulation, and activated coagulation were analyzed for the HSP patients, including measurements of the hemoglobin level, white blood cell (WBC) count, absolute neutrophil count (ANC), platelet count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) level, prothrombin time, activated partial thromboplastin time, and fibrinogen, D-dimer, and fibrin degradation product (FDP) levels. The clinical scores of the skin, joints, abdomen, and kidneys were assessed during the acute and convalescence phases of HSP. Results: The WBC count, ANC, ESR, and CRP, fibrinogen, D-dimer, and FDP levels were significantly higher in the acute phase compared with the convalescent phase of HSP (p<0.05). The total clinical scores were more strongly correlated with the D-dimer (r=0.371, p<0.001) and FDP (r=0.369, p<0.001) levels than with inflammatory markers, such as the WBC count (r=0.241, p=0.001), ANC (r=0.261, p<0.001), and CRP (r=0.260, p<0.001) levels. The patients with GI symptoms had significantly higher ANC (median [interquartile range], 7,138.0 [4,446.4-9,470.0] vs. 5,534.1 [3,263.0-8,153.5], p<0.05) and CRP (0.49 [0.15-1.38] vs. 0.23 [0.01-0.67], p<0.05), D-dimer (2.63 [1.20-4.09] vs. 1.75 [0.62-3.39]), and FDP (7.10 [0.01-13.65] vs. 0.10 [0.01-7.90], p<0.05) levels than those without GI symptoms. Conclusion: D-dimer and FDPs are more strongly associated with disease activity and more consistently reflect GI involvement than inflammatory markers during the acute phase of HSP.

• Childhood Kidney Diseases
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• v.20 no.1
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• pp.23-28
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• 2016

Purpose: We investigated whether serum levels of insulin growth factor-1 (IGF-1) and insulin growth factor binding protein-3 (IGFBP-3) are valuable in predicting clinical outcomes or are correlated with other laboratory findings in children with Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP). Methods: We examined 27 children who were consecutively admitted to our hospital with HSP between January 2011 and February 2012. Blood tests (C-reactive protein, white blood cell count, platelet count, erythrocyte sedimentation rate, albumin, immunoglobulin A, complement C3, antineutrophil cytoplasmic antibody, IGF-1, IGFBP-3) and urine tests were performed upon admission. IGF-1 and IGFBP-3 were resampled in the recovery phase. Controls included 473 children whose IGF-1 and IGFBP-3 were sampled for evaluating their growth, at the outpatient department of pediatric endocrinology in our hospital. IGF-1 and IGFBP-3 were compared between the HSP children and controls, and between the acute and recovery phases in HSP children. The ability of these values to predict clinical outcomes including renal involvement was analyzed using bivariate logistic regression analysis (BLRA). Results: IGF-1 and IGFBP-3 were not different between the HSP children and controls ($148.7{\pm}117.6$ vs. $69.2{\pm}96.9$, P=0.290: $3465.9{\pm}1290.9$ vs. $3597.2{\pm}1,127.6$, P=0.560, respectively). There was no significant difference in IGF-1 or IGFBP-3 between acute and recovery phases. Based on the BLRA, no variable, including IGF-1 and IGFBP-3, could predict clinical outcomes including the presence of nephritis Conclusion: We concluded that IGF-1 and IGFBP-3 do not predict clinical outcomes of HSP, including renal involvement, in this study.

• Childhood Kidney Diseases
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• v.12 no.2
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• pp.170-177
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• 2008

Purpose : The aim of this study was to evaluate whether the incidence of relapse or nephritis might be influenced by the duration of corticosteroid therapy in children with Henoch-Schonlein purpura(HSP). Methods : We retrospectively analyzed 186 children with a diagnosis of HSP in two major hospitals in Ilsan, Korea from the years 2000 to 2003. To evaluate whether renal involvement or relapse might be influenced by the duration of corticosteroid therapy in children with HSP, one pediatric nephrologist from hospital A, maintained corticosteroid therapy for at least 2 weeks(Group A, n=94). The other from hospital B used only during the symptomatic period(Group B, n=92). Results : There were no significant differences in age, sex, body weight, white blood cell count, hemoglobin, hematocrit, platelet count, serum protein and albumin levels between the two groups. The incidence of abdominal pain or arthralgia also did not differ between two groups. However, the duration of steroid therapy was significantly longer in Group A than in Group B and the cumulative dose of prednisolone was also higher in Group A than in Group B. The development of nephritis was more frequent in Group A. Conclusion : The longer duration of steroid use was not associated with the decreased rate of nephritis. Therefore, corticosteroids should be used carefully in a selected group of HSP children, and be tapered rapidly after control of the acute symptoms.

• Childhood Kidney Diseases
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• v.17 no.2
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• pp.92-100
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• 2013

Purpose: To investigate the clinicopathologic effects of cyclosporine A (CsA) in children with diseases characterized by mesangial immunoglobulin A deposits such as immunoglobulin A nephropathy (IgAN) and Henoch-Sch$\ddot{o}$nlein purpura nephritis (HSPN). Methods: We retrospectively reviewed the clinicopathologic outcomes of 54 children (IgAN, 36; HSPN, 18) treated with CsA. The starting dose of CsA was 5 mg/kg per day, and it was administered in 2 divided doses. The degree of proteinuria and pathologic changes in renal biopsies were evaluated before and after CsA treatment. Results: The mean protein to creatinine ratio decreased from $3.7{\pm}1.5$ to $0.6{\pm}0.4$(P <0.001), and 32 (59.2%) children achieved complete remission of proteinuria after 1-year CsA treatment. Among the 54 children, 24 maintained normal renal function and 25 exhibited microscopic hematuria or proteinuria at the end of CsA treatment. In the HSPN group, 3 children whose initial biopsies indicated class IIIb disease showed class II disease on follow-up, and the follow-up biopsies of 2 children who had class II disease indicated the same class II disease. In the IgAN group, cortical tubular atrophy occurred in 1 child, and no child with IgAN had cortical interstitial fibrosis or tubular atrophy after 1-year CsA treatment. No significant complications were found in the children treated with CsA. Conclusion: Our findings indicate that CsA treatment is effective and beneficial in reducing massive proteinuria and preventing progression to end-stage renal failure in children with glomerular diseases characterized by IgA deposits, such as IgAN and HSPN, within 1 year of treatment.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.4 no.2
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• pp.181-190
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• 2001

Purpose: The aim of this study is to investigate the usefulness of intestinal ultrasonography (US) and upper gastrointestinal endoscopy in the early diagnosis of Henoch-Sch$\"{o}$nlein purpura (HSP) with the gastrointestinal (GI) symptoms preceding the emergence of the skin lesion. Methods: The clinical, intestinal US and upper gastrointestinal endoscopic records of 85 patients (88 cases) with GI symptoms relating to HSP presenting between January 1999 and April 2001 were reviewed. Results: 1) GI symptoms were observed in 52 cases (59%) and skin, joint, renal and scrotal manifestations were observed in 88 (100%), 64 (73%), 15 (17%), 3 cases (3%) respectively. 2) Out of 52 cases with GI symptoms, abdominal pain was observed in all cases (100%). Positive stool occult blood, nausea and vomiting, abdominal tenderness, melena or tarry stool, diarrhea, hematemesis, rebound tenderness and rigidity were observed in 28 (50%), 17 (33%), 17 (33%), 12 (23%), 6 (12%), 4 (8%), 1 (2%) and 1 case (2%) respectively in order of frequency. 3) Intestinal US examination was performed in 27 cases with HSP and GI symptoms (52 cases). Out of 27 sonographic examinations 22 showed abnormal findings. Thickening of the duodeno-jejunal wall was observed in 16 cases (73%). Free peritoneal fluid, enlarged mesenteric lymph node, ileus and abnormal gall bladder were seen in 8 (36%), 8 (36%), 4 (18%) and 1 case (5%) respectively. In three cases of HSP without GI symptoms, those changes were absent. 4) In all of five cases with HSP and GI symptoms, endoscopic study showed mucosal edema and multiple hemorrhagic erosions especially at the second portion of the duodenum. Biopsy specimens from the duodenum of 2 cases out of 5 endoscopic examinations showed acute inflammatory infiltrates in the mucosa with hemorrhage. 5) Both intestinal US and endoscopic studies were performed in 4 cases with HSP and GI symptoms simultaneously. Out of 4 those cases, 3 cases showed the thickened duodeno-jejunal wall on the intestinal US, which suggested erosive hemorrhagic duodenitis by endoscopic findings. Conclusion: The typical but nonpathognomonic intestinal US findings including the thickening of the duodeno-jejunal wall and upper gastrointestinal endoscopic findings including hemorrhagicerosive duodenitis, in children with GI symptoms, should be considered a manifestation of HSP, even in the absence of skin lesion.

• Clinical and Experimental Pediatrics
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• v.49 no.12
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• pp.1354-1357
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• 2006

Acute hemorrhagic edema of infancy (AHEI) is an uncommon form of cutaneous leukocytoclastic vasculitis that occurs in infants and children younger than 2 years. AHEI is characterized clinically by marked peripheral edema and fever as well as large palpable purpuric and ecchymotic skin lesions in a target-like pattern, mainly on the face, ears and extremities, similar to the skin findings of $Henoch-Sch{\ddot{o}}nlein$ purpura (HSP). The skin lesions heal spontaneously within one to three weeks and internal organs are rarely affected. We report a case of AHEI occurring in a 23-month-old boy who was initially misdiagnosed as HSP, and was later diagnosed according to his clinical symptoms and histochemical characteristics.

• Childhood Kidney Diseases
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• v.23 no.2
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• pp.128-133
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• 2019

Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP) is a systemic vasculitis characterized by purpura, arthritis, abdominal pain, and nephritis. Gastrointestinal involvement can manifest as pain, intussusception, intestinal bleeding, and intestinal perforation. We report a case of fulminant HSP at an age of eight in 1994, with multiple complications of intra-thoracic bleeding, massive intestinal perforation, nephritis, and various skin rashes. The brisk bleeding findings of intestinal on Technetium-99m-labeled red blood cell scan ($^{99m}Tc$ RBC scan) were well matched to those of the emergency laparotomy and the resected intestine. The patient's abdominal conditions improved gradually but nodular skin eruptions developed newly apart from improving preexisting lower limb rashes and the urine findings continued abnormal, so skin and kidney biopsy were done for the diagnosis. After cyclosporine therapy, skin eruptions and urine findings returned to normal gradually. On a follow-up after 25 years in 2019, the patient is 33-year-old, healthy without any abnormality on blood chemistries and urine examination.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.9 no.2
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• pp.183-192
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• 2006

Purpose: This study was undertaken to evaluate the factors correlated with the clinical course and outcome in patients of Henoch-Sch${\ddot{o}}$nlein Purpura. Methods: The medical records of 104 children diagnosed with Henoch-Sch${\ddot{o}}$nlein Purpura (HSP) from January 1996 to April 2006 were reviewed retrospectively. The patients were divided into two groups: patients with Gastrointestinal (GI) symptoms and those without GI symptoms. When there were joint, scrotum, and renal symptoms except for skin lesion in whole HSP, those patients were excluded. The history of acute infection, duration of admission, treatment requirement, recurrence of HSP, CBC, stool occult blood test, abdominal ultrasonographic findings and GI endoscopic findings were reviewed. Results: Among 104 patients, patients with GI symptoms included 66 cases (63.5%), those without GI symptoms accounted for 38 cases (36.5%). GI symptoms included: abdominal pain in 57 cases (54.8%), vomiting 21 cases (20.2%), GI bleeding 5 cases (4.8%), nausea 3 cases (2.9%) and diarrhea 3 case (2.9%). Positive GI symptoms and GI mucosal lesions on GI endoscopy had a statistically significant correlation with increased admission duration, treatment requirement, recurrence of HSP, and positive stool occult blood. Six cases with small intestinal wall thickening were noted on abdominal ultrasonography. Six cases of hemorrhagic gastritis and hemorrhagic duodenitis, 3 cases of duodenal ulcer, 3 cases of hemorrhagic gastritis and duodenal ulcer, 2 cases of hemorrhagic duodenitis and colitis, and 1 case of colitis were noted on GI endoscopy. Conclusion: These results suggest that GI endoscopic examination may be helpful for the diagnosis and treatment of children with HSP.