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This report presents a case of nephrotic syndrome and renal failure that developed in a 53-year-old female with metastatic breast carcinoma. She was diagnosed to have osteolytic bone metastases 5 years prior to admission, and had been administered pamidronate with a total dose of approximately 6800 mg. A renal biopsy revealed tubulointerstitial damage and marked wrinkling and retraction of the glomerular basement membrane with hypertrophy and hyperplasia of the epithelial cells, compatible with the collapsing form of focal segmental glomerulosclerosis (FSGS). Despite the discontinuation of pamidronate after admission, her renal function gradually decreased. She was finally managed with continuous palliative care for advanced malignancy through a shared effort, and died 96 days after undergoing the renal biopsy. Although the clinical impact of the pamidronate-associated kidney injury on the longitudinal changes in renal function remains to be delineated, it is therefore reasonable to consider that the collapsing FSGS associated with tubulointerstitial damage may have resulted in the irreversible renal injuries that were observed in the current case. Further studies and accumulated experience with renal biopsy are required to better determine the relationship between pathological alterations and prognostic characteristics among patients with pamidronate-associated renal impairments.

ネフローゼ症候群を呈し, 先天性片腎無形成を伴ったKlippel-Feil症候群と診断された患者が, 慢性腎不全に進行し, CAPD導入となったので報告する. 症例は, 42歳女性. 9年前顔面浮腫を呈し, ネフローゼ症候群と診断. 精査にて片腎であり, 短頸, 頸椎癒合, 側彎を認め, Klippel-Feil症候群と診断. 腎生検は行われなかった. 3度妊娠するも腎機能障害のため2度は, 人工中絶, 1度だけ中絶を勧められるも出産した. その後外来通院せず平成7年12月呼吸困難にて近医入院, ダブルルーメンカテーテルにて血液透析施行. 当院紹介されるも動脈奇形にて内シャント造設できず, negative selectionにて平成8年1月CAPD導入となった. Klippel-Feil症候群にて腎不全を起こした報告は稀であり, ここに報告した.

A case of human T-cell lymphotrophic virus type I (HTLV-I)-associated myelopathy (HAM) occurring in a 62-year-old Japanese male with a history of hemodialysis and repeated blood transfusions due to chronic renal failure (CRF) is reported. The patient was a lifelong resident of Chiba Prefecture, a nonendemic area for HTLV-I infections. The clinical course was characterized by abrupt onset and rapid progression of neurological signs and symptoms, which appeared to have responded quite well to prednisolone. Although patients with a history of CRF, hemodialysis, and transfusion of blood units which were not screened for anti-HTLV-I antibodies appear to belong to the high risk group for HTLV-1 infections and subsequent development of HAM, reports on such cases have been scanty. Only 3 cases have been reported to date. It appears quite possible that many patients with CRF and HAM remain misdiagnosed.

We report on the case of a 45 year old male with focal segmental glomerulosclerosis (FSGS) in whom steroid-resistant proteinuria was reduced rapidly by plasma exchange. In 1994, he was admitted to our hospital because of massive proteinuria of several years' duration. Renal biopsy confirmed the diagnosis of FSGS. Proteinuria was suppressed partially with the use of dipyridamole. Though oral prednisolone (PSL, 30 mg/day) was effective initially, relapse occurred during PSL tapering. Doses of PSL up to 30 mg/day or additional mizoribine were ineffective. The patient was readmitted for a trial of plasma exchange in April 2000. Four sessions of plasma exchange with albumin replacement over 2 weeks immediately reduced the proteinuria from 3.2 g/day to 0.6 g/day without any change in medication. After discharge, proteinuria remained suppressed for more than 6 months despite a reduction of PSL dose to 15 mg. The rapid and long lasting effect of plasma exchange in the present case argues for the role of a putative circulatory factor in the pathogenesis of proteinuria in FSGS.