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ホシノ ジユンイチ
Hoshino Jiyun'ichi
星野 純一 所属 医学部 医学科（東京女子医科大学病院） 職種 教授・基幹分野長 |
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| 言語種別 | 英語 |
| 発表タイトル | Diagnostic Utility of Urinary Sediments in Light-Chain Proximal Tubulopathy (LCPT): Report of Three Cases |
| 会議名 | Kidney Week 2024 |
| 主催者 | American Society of Nephrology |
| 学会区分 | 国際学会及び海外の学会 |
| 発表形式 | ポスター掲示 |
| 講演区分 | 一般 |
| 発表者・共同発表者 | ◎Nakai Anna, Manabe Shun, Seki Momoko, Ushio Yusuke, Kataoka Hiroshi, Hoshino Junichi, |
| 発表年月日 | 2024/10/25 |
| 国名 | アメリカ合衆国 |
| 開催地 （都市, 国名） |
San Diego, USA |
| 開催期間 | 2024/10/24～2024/10/27 |
| 概要 | Introduction
LCPT is characterized by crystals within proximal tubular epithelial cells and is typically associated with Fanconi syndrome. However, in cases without Fanconi syndrome, diagnosing LCPT is challenging. Microscopic analysis of urinary sediments may be valuable for LCPT, as cells and casts might contain light chain-derived crystals. Although their diagnostic value remains underreported, we present three cases of LCPT without Fanconi syndrome, where urinary sediments with crystals were crucial in understanding their condition. Case Description A 54-year-old female with macroglossia, carpal tunnel syndrome, subcutaneous nodules, and IgA-κ type M protein was diagnosed with AL amyloidosis. Urine protein was 5.3 g/gCr, and serum creatinine (sCr) was 1.09 mg/dL. However, urinary sediments revealed crystals within tubular epithelial cells, inconsistent with amyloidosis. Kidney biopsy was negative for amyloid deposition, and LCPT was diagnosed. A 55-year-old male with psoriasis and hypertension experienced a gradual decline in renal function. sCr level was 1.92 mg/dL. Urinary sediments revealed crystals within tubular epithelial cells and casts. Light chain staining of the sediments was positive for kappa light chain only. IgG-κ type M protein was identified, and kidney biopsy demonstrated LCPT with significant tubular casts containing crystals. A 66-year-old female presented with fever, edema, and impaired renal function. sCr level was 2.41 mg/dL. IgG-κ type M protein was detected. Urinary sediments were suspicious for crystals within tubular epithelial cells and macrophages. Immunostaining and electron microscopic analysis of cell blocks confirmed crystals in both cell types. Kidney biopsy disclosed LCPT and crystals within interstitial macrophages. Moreover, a biopsy of a subcutaneous soft mass detected histiocytes containing crystals. These findings led to a diagnosis of Crystal-Storing Histiocytosis. Discussion Direct observation of tubular epithelial cells and casts in urine supported the diagnosis of LCPT. Immunostaining and electron microscopic analysis were informative for identifying the origin of crystals, the type of light chain, and the cell type containing crystals, deepening the understanding of the patient’s condition without invasive kidney biopsy. Further consideration is required. |