• Childhood Kidney Diseases
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• v.11 no.2
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• pp.288-293
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• 2007

Thrombotic thrombocytopenic purpura(TTP) is a rare but life-threatening multi-system disorder characterized by the classic pentad of clinical features that includes fever, microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities and renal dysfunction. TTP has been rarely reported to simultaneously present with systemic lupus erythematosus (SLE). While it is important to distinguish between the two diseases of therapeutic implication, cases of concurrent TTP and SLE help to elucidate the pathophysiology that underlies each condition. We describe two adolescents with synchronous TTP and SLE, and review the literature.

• Tuberculosis and Respiratory Diseases
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• v.43 no.3
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• pp.461-466
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• 1996

Diffuse alveolar hemorrhage is a very rare manifestation in Henoch-Schoenlein purpura. Recently we experience a case of diffuse alveolar hemorrhage associated with Henoch-Schoenlein purpura which was diagnosed by typical clinical manifestation and renal biopsy. A 25 year old male was admitted due to hemoptysis and dyspnea. Chest X-ray, HRCT and BAL revealed diffuse alveolar hemorrhage. He also had a history of skin rash, polyarthralgia, and hematochezia with abdominal pain. Renal biopsy which was taken for the evaluation of microscopic hematuria showed IgA nephropathy. Under the diagnosis of Henoch-Schoenlein purpura, we treated him with solumedrol pulse therapy, plasma-pheresis and prednisolone with cytoxan. After then he showed marked improvement in clinical manifestation and was discharged with prednisolone and cytoxan.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.38-45
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• 2005

Purpose : The purpose of this study was to analyze the therapeutic effect of plasmapheresis in various pediatric diseases. Methods : Therapeutic plasmapheresis was performed by COBE Spectra centrifugation. Nine cases were included in this study. The number an[;. method of plasmapheresis, together with the progress and prognosis of each case were retrospectively reviewed. Results : The patients' ages ranged from 26 mont]Is to 16 years of age, and the mean age was 9.9 years. There were S males and 4 females. The underlying diseases requiring plasmapheresis included 2 cases of hemolytic uremic svndrome(HUS), 1 case of lupus nephritis, 2 cases of rapidly Progressive glomerulonephritis(RPGN), 1 case of focal segmental glomorulosclerosis(FSGS), 1 case of systemic vasculitis after pulmonary hemorrhage, 1 case of acute renal failure associated with pulmonary hemoIThage, and 1 case of acute rejection after renal transplantation. The average number of plasmapheresis performed was 6.2 times with a range of 3 to 13 times. The patients with HUS, lupus nephritis, ANCA positive systemic vasculitis induced by pulmonary hemorrhage and ARF-associated pulmonary hemorrhage showed a good response to therapeutic plasmapheresis, but the patients with RPGN, refractory FSGS, and acute rejection after renal transplantation were not responsive to treatment. The most common side effect was hypocalcemia which was rarely symptomatic. Vital signs were not compromised. Conclusion : Although it is presumptuous to generalize the therapeutic effects of plasma pheresis in different diseases due to the small number of study subjects, this study shows that plasmapheresis may be an effective therapeutic modality in various pediatrics diseases and should be considered as a therapeutic option.