著者

山縣 邦弘 小山 哲夫 小林 正貴 成田 光陽

出版者

Japanese Society of Nephrology

雑誌

日本腎臓学会誌 (ISSN:03852385)

巻号頁・発行日

vol.32, no.10, pp.1045-1052, 1990

---

This study was undertaken to analyze the mechanisms of immune complex formation in Heymann nephritis. We isolated two different RTE antigens by gel filtration and prepared rabbit antisera against these antigens. By the indirect immunoflurescence method using normal rat renal tissues, the 65, 000 molecular weight antigen (=β) was observed not only in RTE, but also in the glomerular epithelium, epithelium of the small intestine, liver and spleen. On the other hand, the 35, 000 molecular weight antigen (=γ) existed in RTE and epithelium of the small intestine. When rats were injected intravenously with rabbit antiserum against β, glomerular depositions were observed within two hours. In rats injected with rabbit antiserum against r, no glomerular deposition was seen with-in 2 days, but fine granular depositions were observed after 6 days. When rat kidneys were perfused with rabbit antiserum against γ in saline by a single pass method, no glomerular deposition was seen. However, in rat kidneys perfused with preformed soluble γ-anti γ IC, fine granular depositions along the capillary walls were seen soon after the perfusion. Further the antigen which was reacted with anti γ antiserum was isolated from normal rat serum by immuno-affinity chromatography. These facts suggest that the mechanisms of IC formation may be due to not only in situ immune complex formation but also circulating immune complex deposition in Heymann nephritis.

著者

金子 洋子 楊川 堯基 林 苑子 張 紅 塚原 知樹 松永 恒明 多留 賀功 石津 隆 小林 正貴 野口 雅之

出版者

一般社団法人 日本内科学会

雑誌

日本内科学会雑誌 (ISSN:00215384)

巻号頁・発行日

vol.108, no.5, pp.999-1006, 2019-05-10 (Released:2020-05-10)

参考文献数

6

---

53歳,男性.浮腫にて受診.蛋白尿,心肥大,四肢疼痛ならびに腎不全を認め,αガラクトシダーゼA(α-galactosidase A:GLA)酵素活性の低下,腎生検にて糸球体上皮細胞内に高電子密度物質の沈着,責任遺伝子変異を認め,Fabry病と診断した.末期腎不全のため透析導入し,酵素補充療法(enzyme replacement therapy:ERT)を開始したが,心肥大は進行し,心不全のため死亡した.病理解剖にて多臓器に高電子密度沈着物を認めた.近親者3症例にERTを継続している.本疾患は,早期に診断し加療介入することが重要である.