• 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.

• 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.

• Tuberculosis and Respiratory Diseases
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• v.63 no.6
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• pp.531-536
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• 2007

Wegener's granulomatosis is a systemic vasculitis of the medium and small arteries, as well as of the venules, arterioles, and occasionally large arteries, and primarily involves the upper and lower respiratory tracts and the kidneys. Renal symptoms of Wegener's granulomatosis are indistinguishable from those of vasculitis such as Henoch-$Sch\ddot{o}nlein$ purpura and microscopic polyangiitis. This case, though initially diagnosed as Henoch-$Sch\ddot{o}nlein$ purpura, was confirmed as Wegener's granulomatosis from a lung biopsy fifteen years after the initial diagnosis. We report this case with a review of the literature.

• 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.7 no.2
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• pp.166-173
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• 2003

Purpose : The cause and pathogenesis of $Henoch-Sch\"{o}nlein$ purpura has been studied for many years but the results are disappointing. Recently the hypothesis that abnormalities involving the glycosylation of the hinge region of immunoglobulin Al(IgAl) may have an important role in the pathogenesis of $Henoch-Sch\"{o}nlein$ purpura is being approved. $Henoch-Sch\"{o}nlein$ purpura is the most common vasculitis Ihat affects children and the prognosis is good. But if kidney invovement occurs, the course may be chronic and troublesome. So we evaluated children with $Henoch-Sch\"{o}nlein$ purpura especially from the point of epidemiology and clinical manifestations. Methods : Investigation of 124 children who were diagnosed with $Henoch-Sch\"{o}nlein$ purpura at Inje University Ilsan Paik Hospital from December 1999 to July 2003 was performed retrospectively through chart review. Efforts were made to get informations about the profile, epidemiology, clinical manifestations, progress of the disease and recurrence rate of patients. Results : The patients were 69 boys and 55 girls, with a mean age of $6.1{\pm}2.7$ years at the time of data collection. The male to female ratio was 1.25 : 1. The occurrence rate was much higher in autumn(from September to November, 31.5%) and winter(from December to February, 28.2%) than in spring and summer, with a peak in November. Joint involvement was shown in 66.9% of patients mostly on the foot/ankle(75.9%), knee(39.8%). Seventy(56.5%) out of 124 patients had abdominal pain and 10 patients(8.1%) showed bloody stools. Renal involvement was observed in 24 patients(19.4%) after 21.1 days on the average. IgA was elevated in 10 of 21 patients(47.6%). $C_3$ and $C_4$ levels were normal in 40 of 49 patients (81.7%) and 47 of 48 patients(97.9%), respectively Antistreptolysin-O(ASO) titer was elevated over 250 Todd units in 29 of 62 Patients(46.8%). Mycoplasma antibody titer was elevated in 21 of 49 patients(42.9%) equal or greater than 1:80. Radiologic studies were peformed in 23 patients. Seven patients(30.4%) showed bowel wall thickening and one of them received intestinal resection and anastomosis operation due to terminal ileum necrosis. Eighty four patients took steroid 1.4 mg/kg/day in average. Recurrence rate was 2.5 in 37 patients(29.8%). Conclusion : $Henoch-Sch\"{o}nlein$ purpura in childhood appears most in about 6 years of age. The occurrence rate is much higher in autumn and winter relatively. Diagnosis can be made through the perspective history taking and the inspection of clinical manifestations, but the laboratory findings are not of great help. A small portion of the patients might show abdominal pain or arthritis before purpura develops, therfore various diagnosis can be made. Radiologic evaluation should be performed to avoid surgical complications in cases accompanying abdominal pain, and long term follow up should be needed especially in patients suffering from kidney involvement. In about 30% of the patients $Henoch-Sch\"{o}nlein$ purpura would recur. Steroid can be used safely without side effects.

• 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.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.

• Clinical and Experimental Pediatrics
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• v.45 no.3
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• pp.406-412
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• 2002

Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) is one of the most common vasculitic diseases of childhood, referred to as a leukocytoclastic vasculitis affecting small vessels. Although HSP related gastrointestinal symptoms are seen in up to 80% of patients during acute illness, these symptoms are usually transient. However, some patients with HSP have gastrointestinal major surgical complications such as intussusception, bowel infarction, necrosis, stricture, and perforation. We experienced a rare case of HSP-related ileal perforation developed after corticosteroid treatment. We report a case with HSP-related intestinal perforation and assess the effect of corticosteroid on the outcome of abdominal pain in children with HSP.

• Clinical and Experimental Pediatrics
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• v.45 no.12
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• pp.1601-1605
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• 2002

Henoch-Sh${\ddot{o}}$nlein Purpura(HSP) is an immuologically mediated systemic vasculitis of small blood vessels affecting predominantly the skin, gastrointestinal tract, joints, and kidneys. Clinical neurological manifestations such as headaches, behavioral changes, mental changes, seizures, and visual loss are described, but neurological complication are rare during the course of HSP. We experienced a case of an 8 year-old male with HSP who presented with seizures. Magnetic resonance imaging(MRI) showed multiple high signal intensity in both cortical and subcortical areas of frontal and parieto-occipital lobes and magnetic resonance(MR) angiogram showed stenosis of cerebral arteries, compatible with MRI and MR angiogram findings of cerebral vasculitis. We report this case with related literature.

• Journal of Trauma and Injury
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• v.26 no.3
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• pp.238-242
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• 2013

Idiopathic thrombocytopenic purpura (ITP) is the condition of having an abnormally low platelet count with an unknown cause. Acute subdural hematomas (ASDHs) usually develop in trauma patients and often involve a high bleeding tendency. However, ITP patients rarely have a large ASDH, and when a traditional decompressive craniectomy is performed on patients with coagulopathy, the mortality rate is higher because of the greater bleeding risk. We report the case of an ITP patient with a large ASDH who treated with a burr-hole trephination and irrigation and who was discharged without any neurological deficit.