• Childhood Kidney Diseases
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• v.16 no.1
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• pp.51-53
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• 2012

Henoch-Sch$\ddot{o}$nlein purpura (HSP) is the most common vasculitis in children and is characterized by cutaneous purpura, arthritis, abdominal pain, and nephritis. Bullous skin lesions are rare in children. We report a case involving a 9-year-old female with HSP who displayed rapidly evolving hemorrhagic bullae from the primary purpuric lesions during systemic corticosteroid therapy. The bullae disappeared within 7 days of systemic corticosteroid therapy. Some scar lesions of the skin developed on acute phase recovered completely after 6 months. Bullae should not be considered as a poor prognostic factor of HSP and its renal outcome. Skin biopsy in HSP children with bullae is not necessary if clinical diagnostic criteria of HSP are met. However, further evaluation of more pediatric HSP with bullae is needed to get the clearer conclusions. We report a 9-year-old female with HSP who showed the rapidly evolving hemorrhagic bullae from primary purpuric lesions during systemic corticosteroid therapy.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.185-194
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• 2007

Purpose : $Henoch-Sch\ddot{o}nlein$ Purpura(HSP) is a self-limited systemic small vessel vasculitis, however, renal involvement is considered to contribute to the outcome of this disease. Therefore, identifying the renal risk factors in HSP and prevention of renal involvement are important. The aim of this study is to investigate whether early steroid administration in HSP could reduce the rate of renal involvement. Methods : We retrospectively studied two hundred children with HSP. We had administrated steroids orally to resolve of severe abdominal pain, joint and scrotal symptoms. We analyzed the relationship between the steroid therapy to relieve systemic symptoms and the subsequent renal involvement in HSP. Results : There were no significant differences in the incidence and duration of renal involvement according to steroid administration and its duration. In HSP patients with renal manifestations, steroid administration group showed a tendency of hematuria and steroid non-administration group showed a tendency of proteinuria, however, we could not find statistically significant differences in each group. There was no significant difference in the duration of purpura presence according to steroid administration. However, persistent purpura increased the incidence and the duration of renal involvement. Conclusion : Early steroid administration did not reduce the risk of renal involvement, there-fore, steroid could not prevent delayed nephritis in children with HSP On the other side, Persistent Purpura, known to be not related to steroid therapy, was associated with renal involvement. We suggest that early steroid administration could not be useful in preventing the renal involvement in HSP. (J Korean Soc Pediatr Nephrol 2007;11:185-194)

• The Journal of Internal Korean Medicine
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• v.28 no.3
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• pp.570-585
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• 2007

A clinical study of 106 cases of $Henoch-Sch{\ddot{o}}nlein$ purpura was made. The author analyzed the result of Korean traditional medical therapy. It was observed more frequently in female at the sex ratio of 1:1.3. The most prevalent age group was $4{\sim}9$ years(56.6%). In seasonal incidence, the cases were frequent during spring(50%), and the main specific history was upper respiratory tract infection(48.1%). The duration of contraction was $420.9{\pm}1079.3$ days. The adults were more chronic than children (p<0.05), The clinical manifestation showed in the skin (100%), the gastrointestinal tract(62.3%), the joints(64.2%), the kidney(25.3%), genitourinary system(11.3%), headache(6.6%), and scalp edema(4.7%) respectively. The first outbreak of symptoms was showing of skin purpura in 77 cases(72.6%) the highest frequency. and the gastrointestinal tract and joints were 16(15.1%) and 12 cases(11.3%) respectively. Of all patients, 22 cases(20.8%) experienced recurrences and 47 patients(44.3%) experienced hospitalization treatment. In the patients group which were recurrence experiences, the recurrence rate was high with patients(63.2%) who usually enjoyed meats and convenience foods and in patients(84%) who treated URI with chemical drugs(p<0.05). The patients group(68.9%) that took steroids also experienced a relatively high recurrence rate. Out of the patients(68.9%) who took treatment with steroids, 46.6% experienced side effects, in orders moon face(100%), weight gain(47.1%), appetite increase(52.9%), and acne. alopecia(11.8%), etc. There was a statistically significant relationship between the period of taking the steroid and the side effects(p<0.05), As the result of the herbal medicine treatment(treatment period: $6.7{\pm}6.2$ weeks, a follow-up survey during $15{\sim}24$ months), 67 patients(91.8%) among the 73 patients(68.9%) under 18 years old recovered completely but 3 cases(4.5%) recurred. 23 patients(69.7%) among the 33 patients(31.3%) above 19 years recovered completely but 5 cases(21.7%) recurred.

• Childhood Kidney Diseases
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• v.20 no.2
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• pp.92-96
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• 2016

Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP) is the most common vasculitis in children, mainly affecting the small vessels of the skin, joints, gastrointestinal tract, and kidneys. Although most cases of HSP resolve spontaneously without sequelae, serious nephrological and intestinal problems may occur in some cases. We experienced a case of HSP complicated by simultaneous intussusception and nephritis in a 14-year-old boy who developed a sudden abdominal pain and gross hematuria on the 11th day after onset of the disease. Imaging studies revealed intussusception that required emergency laparotomy. Despite treatment with steroid and angiotensin-converting enzyme inhibitors, nephritis and nephrosis progressed for 4 weeks, and renal biopsy was performed to confirm the diagnosis. Cyclosporin A therapy was started, and remission of proteinuria was achieved after 5 months. However, the nephritis recurred and worsened to end-stage renal failure during 15 years of follow-up.

• Tuberculosis and Respiratory Diseases
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• v.67 no.3
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• pp.226-228
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• 2009

Henoch-$Sch\ddot{o}nlein$ purpura (HSP) is an immunologically mediated systemic vasculitis of small blood vessels that primarily involves the skin, gastrointestinal tract, joints and kidneys. HSP is a common vasculitic syndrome in children who, in most cases, achieve complete recovery. Pulmonary hemorrhage is a very rare manifestation of HSP. The authors present a case of a 46-year-old male presenting with pulmonary hemorrhage and renal involvement and the diagnosis of HSP. The patient responded to prednisolone therapy.

• Childhood Kidney Diseases
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• v.2 no.2
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• pp.110-117
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• 1998

Purpose : Henoch-$Sch{\ddot{o}}nlein$ purpura nephritis(HSPN) accompanied by nephrotic syndrome(NS) is known to have a poor prognosis and effective treatment is still controversial, even though both corticosteroids and immunosuppresant have been used for therapy. Cyclosporine A(CsA) is a well known immunosuppresant and widely used in renal transplantation and glomerular diseases especially steroid resistant. The aims of this study was to evaluate the therapeutic effect of CsA and to compare CsA with previously reported our data of rifampin(RFP) and azathioprine(AZA) in children with HSPN accompanied by NS. Methods : 37 HSPN patients with NS confirmed by renal biopsy were selected. Of these, 17 patients were treated with CsA(5 mg/kg/day) fur 6-8 months, 7 children were treated with RFP(10-20 mg/kg/day) for 9-12 months and 13 patients were treated with AZA(2 mg/kg/day) fur 8 months. Along with these regimens, low dose oral prednisolone(0.5-1 mg/kg, qod) was also used. Sequential renal biopsy was done in all patients 1 month after termination of treatment. Results : Complete remission rate of nephrotic syndrome was $58.8\%$ in CsA, $57.1\%$ in RFP and $38.4\%$ in AZA group after 17, 22, 11 months of mean follow-up period. Overall remission rate including partial remission was $88.2\%$ in CsA, $85.7\%$ in RFP and $84.6\%$ in AZA group. Disappearance rate of hematuria was $58.8\%$ in CsA, $57.1\%$ in RFP and $46.2\%$ in AZA group. Improvement of grade of clinical status was observed in 17 out of 17 CsA, 7 out of 7 RFP and 10 out of 13 AZA group. Improvement of pathologic class on sequencial renal biopsy was shown in 5 CsA($29.4\%$), none RFP($0\%$) and 2 AZA group($12.4\%$). Improvement on histologic immune-deposition was seen in 15 CsA($88.2\%$), 6 RFP($85.9\%$) and 4 AZA group($30.8\%$). Conclusion : In conclusion, Both CsA and RFP treated groups showed better result in complete remission rate of nephrotic syndrome and significant improvement of histologic immune-deposition compared with AZA treated group(p=0.004). So, we recommend CsA and REP rather than AZA for immunosuppresant treatment in HSPN with nephrotic syndrome.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.1
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• pp.78-82
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• 2004

Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP) is a vasculitis of the small vessels in skin, joints, gastrointestinal (GI) tract and kidney. GI symptoms occur in up to 85% of patients and may lead to severe problems such as intussusception, obstruction, and perforation. GI symptoms may not be easily controlled, showing refractoriness to the conventional corticosteroid therapy. Although GI involvements of HSP are acute, and self-limited in most instances, they may cause fatal results in some unusual cases. In such conditions all the possible therapeutic modalities should be considered. We report two cases of severe small bowel involvement of HSP. One case presented with severe abdominal pain showing refractoriness to corticosteroid, but improved with IV immunoglobulin therapy. In the second case, HSP with transmural infarction in the small bowel could be cured with surgical intervention.

• Clinical and Experimental Pediatrics
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• v.48 no.2
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• pp.174-177
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• 2005

Purpose : We evaluated children with Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) in terms of epidemiology and clinical characteristics. Methods : A total of 424 medical records of children with HSP admitted to The Catholic University of Korea, Daejeon St. Mary's Hospital, from 1987 to 2003 were retrospectively analyzed. Results : The mean annual number of cases was $25.1{\pm}7.9$ and no one year showed an outbreak. There was a steady number of patients throughout the year with a decrease during the summer season. The male-to-female ratio was 1.3 : 1 with the median age of the patients being 6-years-old. The age distribution showed a peak at age 6 in a bell-shaped distribution curve. Purpura was noted in 100 percent of the patients, gastrointestinal involvement in 53.8 percent, joint involvement in 40.8 percent, and renal involvement in 18.9 percent. Nephrotic syndrome occurred in 1 percent of all patients. Conclusion : The epidemiologic and clinical features of HSP were similar to those of other regions in Korea and foreign nations, irrespective of time.

• Tuberculosis and Respiratory Diseases
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• v.67 no.3
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• pp.234-238
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• 2009

Henoch-$Sch\ddot{o}nlein$ Purpura (HSP) is a systemic vasculitis involving small vessels with the deposition of immune complexes containing IgA and C3, which is characterized by associated skin, joint, renal, and gastrointestinal manifestations. Although there were several causes such as autoimmunity, infection, and drugs, it also can be presented as a form of vasculitidies associated with malignancies. We report a case of HSP developed during treatment for non-small cell lung cancer in a 66-year-old man. Multiple purpuric skin lesions occurred in both legs after the first cycle of chemotherapy, which was diagnosed as HSP by clinical and pathologic examinations. Due to the itching sensation, topical steroid was applied and the patient was improved 3 weeks later without a scheduled change in chemotherapy.

• 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.