スギヤマ　ヒトシ   杉山　斉    所属   川崎医療短期大学  教育部 医療介護福祉学科    職種   教授
論文種別	原著
言語種別	英語
査読の有無	査読あり
表題	A nationwide cross-sectional analysis of biopsy-proven Fabry nephropathy: the Japan Renal Biopsy Registry.
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	27(2),pp.141-150
著者・共著者	Makoto Nasu, Naoki Nakagawa, Shigeo Hara, Junko Yano, Yuka Kurokawa, Nao Nakamura, Hitoshi Yokoyama, Akira Shimizu, Hitoshi Sugiyama, Hiroshi Sato, Kei Fukami
発行年月	2023/02
概要	BACKGROUND:Fabry disease (FD) is an X-linked inherited disease where renal complications are associated with a poor prognosis. However, little is known about the prevalence of Fabry nephropathy (FN) in patients with chronic kidney disease (CKD). We extracted FN data from the Japan Renal Biopsy Registry, analyzed the prevalence of FN, and examined the correlation between clinical characteristics and renal involvement according to sex differences and hemi- and heterozygosity in patients with FD.METHODS:A total of 38,351 participants who underwent renal biopsy were retrospectively enrolled, and FN was determined. The clinical characteristics of FD patients were examined based on sex differences.RESULTS:Twenty-nine patients (0.076%) (19 males and 10 females, mean age: 43.7 ± 15.5 years old) were diagnosed with FN. Median estimated urinary protein (UP) and mean eGFR levels were 0.9 [interquartile range (IQR) [0.7-1.6] g/gCr and 67.1 ± 36.8 mL/min/1.73 m2, respectively. Mean systolic blood pressure (SBP) was 126.4 ± 17.1 mmHg and diastolic blood pressure was 76.1 ± 12.6 mmHg. An inverse correlation between eGFR and logarithm UP levels was observed (r2 = 0.23, p = 0.02), SBP was positively associated with logarithm UP (r2 = 0.34, p = 0.004) overall and inversely associated with eGFR (r2 = 0.25, p = 0.007) regardless of sex, and SBP was an independent determinant of proteinuria (p = 0.004) and eGFR (p = 0.007).CONCLUSIONS:The prevalence of biopsy-proven FN was 0.076%. Since SBP is associated with eGFR regardless of zygosity, strict SBP control might be necessary to prevent progression to end-stage kidney disease in both male and female patients with FN.
DOI	10.1007/s10157-022-02287-w
PMID	36329296