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ササキ タマキ
Tamaki Sasaki
佐々木 環 所属 川崎医科大学 医学部 臨床医学 腎臓・高血圧内科学 職種 教授 |
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| 論文種別 | 原著 |
| 言語種別 | 英語 |
| 査読の有無 | 査読あり |
| 表題 | A case series of Fabry diseases with CKD in Japan. |
| 掲載誌名 | 正式名：Clinical and experimental nephrology 略 称：Clin Exp Nephrol ISSNコード：14377799/13421751 |
| 掲載区分 | 国外 |
| 巻・号・頁 | 28(5),pp.404-408 |
| 総ページ数 | 5 |
| 著者・共著者 | Oi Yusei, Hajime Nagasu, Naoki Nakagawa, Seigo Terawaki, Takahito Moriwaki, Seiji Itano, Seiji Kishi, Tamaki Sasaki, Naoki Kashihara, Takanobu Otomo |
| 発行年月 | 2024/01/09 |
| 概要 | BACKGROUND:It is well known that kidney injury is vital organ damage in Fabry disease (FD). Renin-angiotensin system (RAS) inhibitors are known to reduce proteinuria in patients with chronic kidney disease (CKD) by dilating the glomerular export arteries and reducing intraglomerular pressure. This improvement in intraglomerular pressure, although lowering the glomerular filtration rate, is thought to prevent renal damage and be renoprotective in the long term. RAS inhibitors may be effective in FD patients with proteinuria to prevent the progression of kidney disease, however, the degree to which they are used in clinical practice is unknown.METHODS:The J-CKD-DB-Ex is a comprehensive multicenter database that automatically extracts medical data on CKD patients. J-CKD-DB-Ex contains data on 187,398 patients in five medical centers. FD patients were identified by ICD-10. Clinical data and prescriptions of FD patients between January 1 of 2014, and December 31 of 2020 were used for the analysis.RESULTS:We identified 39 patients with FD from the J-CKD-DB-Ex including those with suspected FD. We confirmed 22 patients as FD. Half of the patients received RAS inhibitors. RAS inhibitors tended to be used in CKD patients with more severe renal impairment.CONCLUSIONS:This case series revealed the actual clinical practice of FD patients with CKD. In particular, we found cases in which patients had proteinuria, but were not treated with RAS inhibitors. The database was shown to be useful in assessing the clinical patterns of patients with rare diseases. |
| DOI | 10.1007/s10157-023-02439-6 |
| PMID | 38193991 |