Sato Masayo, Manabe Shun, Itabashi Mitsuyo, Horita Shigeru, Hirose Orie, Kawashima Moe, Nishida Miki, Kataoka Hiroshi, Taneda Sekiko, Mochizuki Toshio, Nitta Kosaku
Department of Nephrology, Tokyo Women's Medical University, Japan.
Department of Clinical Laboratory Medicine, Tokyo Women's Medical University Hospital, Japan.
Intern Med. 2022 Apr 15;61(8):1205-1209. doi: 10.2169/internalmedicine.7372-21. Epub 2021 Oct 12.
A slowly progressive middle-aged man initially diagnosed with thin basement membrane nephropathy based on extensive thinning of the glomerular basement membrane (GBM) was subsequently diagnosed with Alport syndrome (AS) by a serial renal biopsy eight years later. The ultrastructural analysis of the second biopsy indicated thickening and wrinkling with mild reticulation in the GBM, consistent with AS. However, a retrospective analysis of the first biopsy revealed mild attenuation of type IV collagen α5 chain staining, suggesting a potential diagnosis of AS, despite the lack of ultrastructural features of AS. We herein report the clinical usefulness of type IV collagen staining in the early diagnosis of AS.
一名病情缓慢进展的中年男性最初基于肾小球基底膜（GBM）广泛变薄被诊断为薄基底膜肾病，八年后经系列肾活检被诊断为Alport综合征（AS）。第二次活检的超微结构分析显示GBM增厚、起皱并伴有轻度网状改变，符合AS表现。然而，对第一次活检的回顾性分析发现IV型胶原α5链染色有轻度减弱，提示尽管缺乏AS的超微结构特征，但仍有可能诊断为AS。我们在此报告IV型胶原染色在AS早期诊断中的临床应用价值。
