南予医学雑誌19巻
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南予医誌 Vol.19 No. 1 2019-68-IntroductionNephrotic syndrome (NS) includes some patients resistant to various medications without attaining remission. Such refractory cases bother us about how and when medi-cations to be used. We experienced a pecu-liar boy who had NS refractory to intensive treatments including cyclophosphamide and cyclosporine A (CyA). Case presentationA three-year-old boy was diagnosed NS in 1991, and minimal change by renal biopsy. He could attain first remission by steroid treatment, but frequent relapses leaded him to receive not only cyclophosphamide but also CyA. Re-biopsy performed in 2003 exhibited IgA deposit on the mesangium area, considering atypical IgA nephropathy because of no staining of C3. Thereafter he had kept a relatively good condition, but still unstable, under the treatment of 5-10 mg prednisolone and 120mg CyA. During this period before admission, serum protein showed 4.5-5.0g/dl and albumin 2.0 g/dl, decreasing gradually, while T-cholesterol was 250-400mg/dl and LDL-cholesterol 120-300mg/dl. He became worse clinically, showing prominent edema on the lower legs 4-days before admission. He was trans-ported to the Hospital, because of his poor condition with anuria. Life style and his fam-ily history have nothing in particular.He was 159 cm high and 91.2 kg in weight, showing rapid weight gain over 30 kg within several months. His blood pressure was 130/84 mmHg and SpO2 was 97%. He showed severe edema on the whole body and serous ooze from his hydrotestis. Chest radiograph showed pneumonia and pleural (Figure)Clinical course and treatment regimen for a 25-year-old patient with refracory NS
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