研究者詳細 - 橋口　明典

論文 - 橋口　明典

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FGGS経過中ネフローゼ症候群を発症,再生検でFSGSを伴うMCDと診断しステロイド治療が著効した一例

吉見公志, 大島洋一, 圓谷泰章, 加藤亜唯, 橋口明典, 小松素明

日本腎臓学会誌（(一社)日本腎臓学会） 67 （ 6-E ） 858 - 858 2025年09月

共著, ISSN 0385-2385

COL4A1遺伝子変異により糸球体基底膜および尿細管基底膜に構造変化を認めたHANAC症候群の一例

吉田隆人, 中山尭振, 吉本憲史, 菱川彰人, 萩原あいか, 橋口明典, 畔上達彦, 林香

日本腎臓学会誌（(一社)日本腎臓学会） 67 （ 6-E ） 780 - 780 2025年09月

共著, ISSN 0385-2385

短腸症候群・在宅中心静脈栄養管理中に急速な腎機能悪化を呈しANCA関連血管炎と診断した一例

安井雅晴, 中山尭振, 吉本憲史, 菱川彰人, 萩原あいか, 橋口明典, 畔上達彦, 林香

日本腎臓学会誌（(一社)日本腎臓学会） 67 （ 6-E ） 839 - 839 2025年09月

共著, ISSN 0385-2385

腎再生検での特徴的な免疫蛍光所見によりPGNMIDの診断に至った一例

熊谷聡佑, 中村共生, 三守賢, 伊藤亘, 中山尭振, 吉本憲史, 菱川彰人, 萩原あいか, 畔上達彦, 橋口明典, 林香

日本腎臓学会誌（(一社)日本腎臓学会） 67 （ 6-E ） 800 - 800 2025年09月

共著, ISSN 0385-2385

腎小葉間動脈の壊死性血管炎を認めた顕微鏡的多発血管炎の一例

冨田巌, 加藤亜唯, 圓谷泰章, 大島洋一, 橋口明典, 小松素明

日本腎臓学会誌（(一社)日本腎臓学会） 67 （ 6-E ） 810 - 810 2025年09月

共著, ISSN 0385-2385

著明な肉芽腫性間質性腎炎を認めた腎サルコイドーシスの一例

渡邉一生, 大島洋一, 中山尭振, 加藤亜唯, 橋口明典, 小松素明

日本腎臓学会誌（(一社)日本腎臓学会） 67 （ 6-E ） 866 - 866 2025年09月

共著, ISSN 0385-2385

An adolescent presenting with IgA nephropathy and persistent decreased kidney function after COVID-19 vaccination during follow-up for asymptomatic hematuria: a clinicopathological study

Morisawa K., Takahashi T., Matsuoka K., Hashiguchi A., Yamanaka M., Hamada R., Honda M.

Cen Case Reports 14 （ 4 ） 635 - 640 2025年08月

共著

Although the coronavirus disease 2019 (COVID-19) vaccine has been proven to be effective and safe in most adults and children, various diseases, including IgA nephropathy, sometimes occur as an adverse effect. We herein describe a case of IgA nephropathy in a 16-year-old, male patient with persistent kidney dysfunction following COVID-19 vaccination and present the clinicopathological course of the disease. The patient presented to the outpatient clinic with a history of gross hematuria 6 days after receiving the COVID-19 vaccine. Prior to the current presentation, he was being examined regularly at an outpatient clinic for asymptomatic hematuria. His mother had received a diagnosis of IgA nephropathy, and his younger brother had received a diagnosis of asymptomatic hematuria. A blood test of this patient demonstrated elevated serum creatinine, and IgA nephropathy was pathologically diagnosed (Oxford classification M0E1S1T0C1). Prednisolone and immunosuppressants were administered promptly to treat the decreased kidney function and the pathology. Nevertheless, the failure of his kidney function to recover to the state it was in prior to this episode may have led to the formation of chronic lesions, causing irreversible kidney tissue damage. Some patients with IgA nephropathy, asymptomatic hematuria or a family history of kidney-related symptoms may experience kidney dysfunction after COVID-19 vaccination and require prednisolone or immunosuppressive therapy to stem the progressive deterioration of their kidney function. Prior to receiving the COVID-19 vaccine, patients with any of these conditions should be provided with an appropriate explanation of the risks and be asked for their consent to be vaccinated.

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A case of IgA nephropathy associated with Crohn’s disease treated with enteric-coated budesonide

Yoshida R., Nakayama T., Hashiguchi A., Azegami T., Hayashi K.

Cen Case Reports 14 （ 4 ） 659 - 664 2025年08月

共著

The gut–kidney axis has recently gained attention as a pathogenesis of IgA nephropathy (IgAN). In fact, the efficacy of target-release formulation of budesonide for IgAN has been reported in recent studies. On the other hand, there have been no reports yet on the efficacy of enteric-coated budesonide for IgAN complicated with inflammatory bowel disease. We report a case of IgAN with comorbid Crohn’s disease (CD) treated with a combination of enteric-coated budesonide and methylprednisolone pulse therapy. The patient was followed for 2 years. He showed initial reduction in proteinuria following treatment. However, after discontinuation of enteric-coated budesonide therapy, proteinuria recurred despite well-controlled CD. Enteric-coated budesonide combined with methylprednisolone pulse therapy may be effective in short-term reduction of proteinuria while minimizing systemic steroid side effects in IgAN associated with CD. However, the tendency for relapse suggests the need for long-term management strategies. Therefore, further research is needed to establish optimal treatment protocols for this patient population.

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Focal global glomerulosclerosis経過中にネフローゼ症候群を発症したMinimal change diseaseの1例

吉見公志, 大島洋一, 圓谷泰章, 加藤亜唯, 橋口明典, 小松素明

日本内科学会関東地方会（日本内科学会-関東地方会） 706回 np23 - np23 2025年07月

共著

【ヘマトネフロロジー】各疾患と腎障害の相互の病態関連と腎障害に対する治療　原発性マクログロブリン血症の病理と鑑別疾患

橋口明典

腎と透析（(株)東京医学社） 99 （ 1 ） 41 - 45 2025年07月

共著, ISSN 0385-2156

＜文献概要＞はじめに　原発性マクログロブリン血症(Waldenstroemマクログロブリン血症:WM)は,1944年,Jan Goesta Waldenstroemによって,口・鼻腔出血,リンパ節腫大,貧血,血小板減少,赤沈更新,高血漿粘度,骨放射線画像正常,リンパ球優位の骨髄を有した2名の患者について記述されたのが,最初の報告である。現在では,リンパ形質細胞性リンパ腫のサブセットとして,骨髄のリンパ形質細胞性リンパ腫(lymphoplasmacytic lymphoma:LPL)と血中単クローン性免疫グロブリンM(IgM)によって定義される。本稿では,WM血液腫瘍としての鑑別診断と,本疾患によって起こる腎疾患の形態分類について概説したい。

Taurine supplementation improves physical activity level in a hemodialysis patient with mitochondrial disease: a case report

Yoshida R., Mitsuno R., Nakayama T., Azegami T., Hashiguchi A., Torimitsu T., Yoshimoto N., Hisikawa A., Hagiwara A., Nakamura T., Meguro S., Katsumata M., Endo J., Matsunaga T., Yoshino J., Kanda T., Morimoto K., Monkawa T., Yoshida T., Nakahara J., Yamaguchi S., Hayashi K.

Cen Case Reports 14 （ 3 ） 366 - 373 2025年06月

共著

Mitochondrial diseases (MDs) are inherited metabolic disorders that affect multiple organ systems, including the kidneys. Variability in disease onset and phenotypic expression, combined with the absence of specific kidney pathological findings, pose significant challenges in diagnosing MD. Consequently, many undiagnosed cases of MD may exist among patients undergoing dialysis. No effective treatment for mitochondrial nephropathy has been established. We report the case of a 27-year-old female patient who presented with leg edema, nephrotic range proteinuria attributed to focal segmental glomerulosclerosis, and bilateral sensorineural hearing loss. Immunosuppressive therapy failed to achieve remission, resulting in progressive kidney function decline and eventual end-stage kidney disease. At hemodialysis initiation, worsening atypical cardiac function and hypertrophy prompted genetic testing, which identified an MT-TL1 m.3243 A > G mutation and confirmed the diagnosis of MD. After hemodialysis initiation, the patient experienced persistent fatigue and decreased physical activity levels despite dry weight management. Suspected stroke-like symptoms prompted the initiation of taurine supplementation, which significantly improved headache severity, cardiac function, and physical activity levels. This case highlights the therapeutic potential of taurine supplementation in patients with MD undergoing dialysis and the importance of maintaining clinical vigilance for MD across all stages of chronic kidney disease, even without characteristic renal pathological findings of mitochondrial nephropathy.

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Urinary Biomarkers Associated With Pathogenic Pathways Reflecting Histologic Findings in Lupus Nephritis

Hiramoto K., Saito S., Hanaoka H., Kikuchi J., Fukui H., Hashiguchi A., Suzuki K., Takeuchi T., Kaneko Y.

Arthritis and Rheumatology 77 （ 3 ） 298 - 310 2025年03月

共著, ISSN 23265191

Objective: There is a pressing need to understand the pathogenesis of histologic findings and identify the biomarkers for predicting the histologic severity in lupus nephritis (LN). This study aimed to identify the pathogenic signal pathway and elucidate urinary biomarkers for predicting the presence or severity of histologic findings in LN. Methods: Urine samples from patients with biopsy-proven active LN were screened for 1,305 proteins using an aptamer-based proteomic assay. The diversity and expansion of individual renal histologic features in LN were quantified to identify the urinary proteins associated with the histologic findings found in each score. Candidate urinary proteins were validated in a validation cohort. Immunohistochemical staining of the renal tissues was performed to clarify the localization of the candidate proteins. Results: Cluster analysis extracted five histologic subgroups according to their correlations with each histologic finding in LN. Protein groups that correlated with each histologic subgroup revealed a distinct pathogenesis in LN using pathway analyses. Enzyme-linked immunosorbent assay validation revealed that urinary calgranulin B (S100A9), monocyte chemotactic protein 1 (MCP-1), and insulin-like growth factor binding protein 5 (IGFBP-5) levels could specifically predict the presence and severity of active glomerular lesions, interstitial inflammation, and interstitial fibrosis, respectively. Immunohistochemical staining revealed the localization of these proteins in each lesion. Conclusion: Renal histologic findings may reflect the different pathogeneses involved in each lesion, and estimating the urinary calgranulin B, MCP-1, and IGFBP-5 levels may be useful in predicting the presence and severity of histologic findings in LN.

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Urine-to-blood urea nitrogen ratio predicts proteinuria remission in nephrotic syndrome

Mitsuno R., Nakayama T., Yoshida R., Komatsu M., Oshima Y., Iwabuchi S., Hoshi K., Itoh T., Matsumoto D., Fujii K., Hara Y., Futatsugi K., Kawaguchi T., Ando T., Matsuda H., Yamaji Y., Murakami M., Yoshino J., Hashiguchi A., Kaneko Y., Azegami T., Hayashi K.

Clinical and Experimental Nephrology 30 （ 1 ） 109 - 116 2025年

共著, ISSN 13421751

Background: Nephrotic syndrome (NS) carries a high risk of severe complications and kidney failure, necessitating reliable prognostic markers. Given the link between tubular injury and poor remission of proteinuria, markers of tubular function may be informative in NS. The urine-to-blood urea nitrogen ratio (UBUR) reflects tubular urea handling, yet its prognostic value in NS is unclear. We evaluated whether baseline UBUR predicts proteinuria remission in adult NS. Methods: This multicenter retrospective study included patients with NS who underwent kidney biopsy between January 2012 and June 2022. Patients were followed from kidney biopsy until complete remission, dialysis initiation, death, or the end of the observation period (12 months after kidney biopsy). Results: A total of 237 patients (median age, 63 years; 47% female) were included, and divided into two groups based on a UBUR cutoff of 25.4 determined by the receiver operating characteristic curve. Patients with high UBUR had a significantly higher cumulative incidence of complete remission compared to those with low UBUR (P < 0.001, log-rank test). In multivariable Cox regression analysis, high UBUR independently predicted a greater likelihood of complete remission (hazard ratio 2.30 [95% confidence interval 1.47–3.61]). This association persisted in the subgroup analyses for podocytopathies, defined as minimal change disease and focal segmental glomerulosclerosis. Conclusions: High UBUR independently predicts proteinuria remission in adults with NS. UBUR could have potential as a simple, valuable biomarker to help guide the management of NS.

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A Case of Membranous Nephropathy Associated with Syphilis: Correlative Light and Electron Microscopy Revealing the Localization of Neuron-derived Neurotrophic Factor within Subepithelial Electron Dense Deposits.

Minezaki C, Azegami T, Hashiguchi A, Yoshifuji A, Yoshimoto N, Hagiwara A, Hishikawa A, Hayashi K

Internal medicine (Tokyo, Japan) 64 （ 14 ） 2202 - 2207 2024年12月

共著, ISSN 0918-2918

A 50-year-old man presented to our hospital with a fever, edema, and a rash. The clinical diagnosis was renal dysfunction, nephrotic syndrome, and syphilis. The patient was treated with benzylpenicillin, and his symptoms improved. A renal biopsy revealed membranous nephropathy (MN). Recently, neuron-derived neurotrophic factor (NDNF) was reported to be an antigen corresponding to syphilis-associated MN. In the present patient, immunofluorescence staining and immunoelectron microscopy revealed granular NDNF-positive findings within subepithelial deposits, suggesting the presence of NDNF-IgG immune complexes. Although the mechanism by which NDNF serves as a target antigen remains unclear, NDNF was found to colocalize within subepithelial immune complexes in syphilis-associated MN.

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The proteinuria selectivity index value predicts the remission of IgA nephropathy: a retrospective cohort study.

Mitsuno R, Nakayama T, Ito W, Maruki T, Nakamichi R, Adachi K, Yoshimoto N, Hishikawa A, Hagiwara A, Yamaguchi S, Monkawa T, Yoshino J, Hashiguchi A, Azegami T, Hayashi K

Renal failure 46 （ 2 ） 2423839 - 2423839 2024年12月

共著, ISSN 0886-022X

IgA nephropathy (IgAN) is the most common type of primary glomerulonephritis worldwide and leads to end-stage kidney disease. The proteinuria selectivity index (PSI) has been used to assess the prognosis in nephrotic syndrome, but its predictive value in patients with IgAN remains unclear. This single-center retrospective cohort study included patients who diagnosed with IgAN between March 2012 and March 2020. The PSI was calculated at the time of kidney biopsy. Patients were followed up from the time of kidney biopsy to kidney replacement therapy, death, transfer to another facility, or study completion. Ninety-four patients with a median age of 51 years were enrolled and divided according to the cutoff value of PSI determined by the receiver operating characteristic curve analysis into low-PSI (PSI <0.243, n = 39) and high-PSI groups (PSI ≥0.243, n = 55). The median follow-up duration was 70 months. Rates of remission of proteinuria and survival without a two-fold increase in serum creatinine were significantly better in the low-PSI group (both p < 0.01, log-rank test). Cox regression analysis showed that a low PSI was significantly associated with an increased likelihood of remission of proteinuria and hematuria (hazard ratio [HR] 1.96; 95% confidence interval [CI] 1.02–3.85 and HR 1.75; 95% CI 1.01–3.13, respectively), and a decreased risk of a two-fold increase in serum creatinine (HR 0.10; 95% CI 0.01–0.81). In conclusion, The PSI could have the potential to support the assessment of the prognosis of IgAN, in addition to established prognostic markers, by reflecting the overall glomerular permeability.

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Clinical relevance of proteinuria selectivity index and fractional excretion of sodium in patients with nephrotic syndrome.

Nakayama T, Azegami T, Yamaguchi S, Hirano K, Komatsu M, Fujii K, Futatsugi K, Urai H, Kawaguchi T, Itoh T, Yoshimoto N, Hagiwara A, Hishikawa A, Matsuda H, Ando T, Yamaji Y, Murakami M, Hashiguchi A, Kaneko Y, Yokoo T, Hayashi K

Scientific reports 14 （ 1 ） 23755 - 23755 2024年10月

共著

Proteinuria selectivity index (PSI) is a potential tool for histological classification and prediction of treatment response in nephrotic syndrome, but evidence is insufficient. Clinical relevance of fractional excretion of sodium (FENa) in nephrotic syndrome remains largely unexplored. This multicenter retrospective study included patients with nephrotic syndrome who underwent kidney biopsy between January 2012 and June 2022. Optimal cutoffs for predicting complete remission based on PSI and FENa were determined using receiver operating characteristic curves. Patients were divided into two groups using these cutoffs and followed until complete remission. Of the 611 patients included, 177 had minimal change disease (MCD), 52 had focal segmental glomerulosclerosis (FSGS), and 149 had membranous nephropathy (MN). Median (interquartile range) PSI were 0.14 (0.09–0.19) for MCD, 0.33 (0.23–0.40) for FSGS, and 0.20 (0.14–0.30) for MN. FENa were 0.24 (0.09–0.68), 1.03 (0.50–2.14), and 0.78 (0.41–1.28). Patients with low PSI and FENa had a higher incidence of complete remission. Cox regression analyses demonstrated that both parameters were associated with achieving complete remission (HR 2.73 [95% CI 1.97–3.81] and HR 1.93 [95% CI 1.46–2.55], respectively). PSI and FENa may be useful for histological classification and predicting remission in nephrotic syndrome.

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Primary breast lymphoma mimicking triple-negative breast cancer: a case report with clinical and pathological implications.

Sawano T, Wada M, Ozaki A, Hashiguchi A, Hirooka S, Tanimoto T

Surgical case reports 10 （ 1 ） 234 - 234 2024年10月

共著, ISSN 2198-7793

BACKGROUND: Primary breast lymphoma (PBL) is a rare type of extranodal lymphoma, the diagnostic process for which presents significant challenges owing to an overlap in clinical and pathological features with those observed in triple-negative breast cancer (TNBC). However, the current literature reveals a paucity of information regarding the ramifications of potential diagnostic errors, particularly in the context of emergent therapeutic strategies for TNBC. Thus, we present a unique report of a case of PBL. CASE PRESENTATION: A 76-year-old female with no past medical or family history presented to the hospital with the chief complaint of a mass in the right breast. Two masses were palpated in the right breast: one 56 mm mass (No. 1) located at 10 o'clock, and a 21 mm large, elastic, hard mass (No. 2) at 4 o'clock. Needle biopsy was performed only on the larger 56 mm mass (No. 1). The results showed invasive carcinoma that was negative for estrogen receptor, progesterone receptor, and human epidermal growth factor receptor-2. The preoperative diagnosis was right breast cancer (T3N0M0 Stage IIB) of the TNBC subtype. The patient refused the preoperative chemotherapy recommended by the treatment team; therefore, right breast mastectomy and sentinel lymph-node biopsy were performed instead. The histopathological diagnosis of the first mass was diffuse large B-cell lymphoma (DLBCL); that of the second mass (No. 2) was an invasive breast carcinoma of no special type. Postoperative treatment consisted of endocrine therapy (letrozole) for breast cancer, while the DLBCL was treated with chemotherapy and three courses of intrathecal chemotherapy. At the time of this report, the patient is still living, and neither tumor had recurred in the 2 years following surgery. CONCLUSIONS: On rare occasions, PBL can preoperatively mimic TNBC. While this case did not lead to serious consequences, because surgery was eventually selected as the first therapy, clinicians should be aware that the diagnosis of PBL is challenging using only a core-needle biopsy and can often be misdiagnosed as TNBC.

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False-positive Serum Antiglomerular Basement Membrane Antibody due to Bovine Serum Albumin-containing Surgical Adhesive: A Case Report.

Yoshida R, Azegami T, Yamaguchi S, Hagiwara A, Hishikawa A, Yoshimoto N, Hashiguchi A, Hayashi K

Kidney medicine 6 （ 10 ） 100880 - 100880 2024年10月

共著

Antiglomerular basement membrane (GBM) disease has a poor prognosis. The rapid detection of serum anti-GBM antibody using an enzyme immunoassay, which has a high sensitivity and specificity, leads to an early diagnosis and improved prognosis. We report a case of acute kidney injury with false-positive anti-GBM antibody. A man in his early fifties underwent aortic arch replacement using bovine serum albumin (BSA)-containing surgical adhesion. After intravenous administration of vancomycin for a fever, he developed acute kidney injury without an abnormal urinalysis, and his anti-GBM antibody titer (fluorescence enzyme immunoassay [FEIA]) was 70.4 IU/mL. A kidney biopsy showed acute tubular injury and minor glomerular abnormalities without immunoglobulin G deposits, suggesting no evidence of anti-GBM glomerulonephritis. Consistent with the false-positive anti-GBM antibody test results, anti-GBM antibody determined using a chemiluminescent enzyme immunoassay was negative. A serum sample showed crossbinding to the FEIA plate from which the GBM antigen was removed. This finding indicated a nonspecific reaction to BSA, which contains a coating solution for the FEIA plate. This reaction was likely caused by anti-BSA antibody produced using BSA-containing surgical adhesion. Our findings suggest emerging challenges in diagnosing anti-GBM disease. Nephrologists must remain vigilant regarding false-positive anti-GBM antibody test results, particularly in cases evaluated with immunoassays that contain BSA.

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機能性表示食品「紅麹コレステヘルプ」およびNSAIDs服用中に発症した完全型Fanconi症候群を伴う急性腎障害の1例

星健太, 村上円人, 佐藤芳紀, 杉浦康平, 船木裕規, 橋口明典

日本腎臓学会誌（(一社)日本腎臓学会） 66 （ 7 ） 1207 - 1214 2024年10月

共著, ISSN 0385-2385

薬剤性腎障害の原因は多岐にわたるが,漢方薬やサプリメントなど医薬品以外の物質の関与も多く,病歴を漏れなく聴取することが重要である。近年,機能性表示食品「紅麹コレステヘルプ」(小林製薬,大阪,日本)による健康被害の報告が多数のぼり,腎機能障害や尿所見異常などを呈し,一部の症例では末期腎不全に至るなどの臨床像が報告されているが,原因物質は未詳である。今回われわれは,以前より軽度の推算糸球体濾過量(estimated glomerular filtration rate:eGFR)低下を指摘されていたところへ同サプリメントを3年間内服継続し,発症2ヵ月前から非ステロイド性抗炎症薬(non-steroidal anti-inflammatory drugs:NSAIDs)を使用していたところに発症した急性腎障害(acute kidney injury:AKI),後天性完全型Fanconi症候群を伴った急性尿細管障害(acute tubular injury:ATI)の1例を経験した。初診時には尿中β2ミクログロブリン(β2 microglobulin:β2MG)および好中球ゼラチナーゼ結合リポカリン(neutrophil gelatinase-associated lipocalin:NGAL)の上昇と,汎アミノ酸尿,尿糖陽性,高カルシウム尿症,高リン尿症,高尿酸尿症,II型尿細管性アシドーシスといった近位尿細管機能異常を認め,Fanconi症候群を呈したATIによるAKIと考えられた。腎生検組織所見では尿細管上皮の傷害像はみられたが,炎症性細胞浸潤は乏しかった。足突起消失(foot process effacement)は部分的であった。ステロイドを含めた免疫抑制治療は行わず保存的治療のみで腎機能障害やFanconi症候群は軽快し,現在経過観察中である。全国的に使用されているサプリメントであり,原因物質の究明が待たれるが,同サプリメントにより惹起される腎機能障害や蛋白尿などの尿所見異常の病態を中心に,文献的考察を踏まえて報告する。(著者抄録)

Effective calcineurin inhibitor treatment in adult-onset steroid-resistant nephrotic syndrome with a novel splice donor site variant of TRPC6: a case report.

Nagasaka T, Uchiyama K, Hama EY, Kojima D, Kaneko K, Yoshimoto N, Yasuda I, Yamada M, Miya F, Suzuki H, Tajima T, Yamaguchi S, Hayashi K, Kanda T, Hashiguchi A, Kosaki K, Itoh H

CEN case reports 14 （ 2 ） 208 - 216 2024年09月

共著

Transient receptor potential canonical 6 (TRPC6) variants, which were initially detected in adult-onset familial focal segmental glomerulosclerosis (FSGS), were also identified in pediatric-onset one. Here, we present a patient with adult-onset steroid-resistant nephrotic syndrome (SRNS) who harbored a likely pathogenic TRPC6 variant and partially responded to calcineurin inhibitors (CNIs). A 44-year-old woman with stable rheumatoid arthritis, systemic lupus erythematosus, and Sjögren’s syndrome was presented with nephrotic syndrome. Her renal biopsy results showed minor glomerular abnormalities. Upon admission, she was treated with steroids for around 4 weeks, but it was ineffective. After 1–2 weeks of cyclosporine A (CyA) administration, urine output increased, renal function improved without a decrease in proteinuria, and she was discharged. Her renal function was maintained for 2 months, but after a CyA dose reduction, she was again admitted to the hospital due to relapsing edema, decreased urine output, and worsening renal function. CyA was replaced by tacrolimus (TAC). A second renal biopsy showed nearly the same findings as the first except for tubulointerstitial lesions. After 1–2 weeks of TAC administration, urine output increased, and renal function improved. However, urinary protein levels did not decrease as before. After discharge, a whole exome analysis revealed a heterozygous splice donor site variant NM_004621.6;c.2644 + 1G > A in TRPC6. Genetic testing identified a novel splice donor site variant of TRPC6 in a patient with adult-onset SRNS, which prevented unnecessary steroid continuation. The safety and efficacy of CNI in TRPC6 glomerulopathy must be evaluated in future larger studies with longer follow-up.

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