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中华肾病研究电子杂志 ›› 2026, Vol. 15 ›› Issue (05) : 296 -299. doi: 10.3877/cma.j.issn.2095-3216.2026.05.010

病例报告

Abernethy畸形相关IgA肾病:1例报告并文献复习
曹文雅, 郭晓莉, 赵雪, 高祎†()   
  1. 710018 西安,西北大学附属医院(西安市第三医院)肾脏内科
  • 收稿日期:2025-07-03 出版日期:2026-10-28
  • 通信作者: 高祎

IgA nephropathy associated with Abernethy malformation: a case report and literature review

Wenya Cao, Xiaoli Guo, Xue Zhao   

  • Received:2025-07-03 Published:2026-10-28
引用本文:

曹文雅, 郭晓莉, 赵雪, 高祎. Abernethy畸形相关IgA肾病:1例报告并文献复习[J/OL]. 中华肾病研究电子杂志, 2026, 15(05): 296-299.

Wenya Cao, Xiaoli Guo, Xue Zhao. IgA nephropathy associated with Abernethy malformation: a case report and literature review[J/OL]. Chinese Journal of Kidney Disease Investigation(Electronic Edition), 2026, 15(05): 296-299.

图1 肾脏病理免疫荧光、光镜和电镜图注:A为免疫荧光示IgA沿系膜区呈颗粒状沉积(免疫荧光×400);B为过碘酸雪夫染色示肾小球系膜区细胞与基质增生,部分节段性硬化伴囊内粘连,局部可见新月体形成(过碘酸雪夫染色×400);C为电镜显示系膜区及内皮下可见致密电子物沉积,足突部分融合(透射电镜×815)
图2 患者门静脉-体静脉异常分流的CT静脉成像注:A示门静脉主干发育细小,肠系膜上静脉、脾静脉扩张,走行迂曲,二者汇合后仅一小分支汇入入肝门静脉;B示左侧膈下可见一小血管汇入左肾静脉
表1 患者术前及门体分流矫正术后肝肾功能及相关指标动态变化
随访时间 总胆红素(μmol/L) 直接胆红素(μmol/L) 丙氨酸氨基转移酶ALT(U/L) 碱性磷酸酶(U/L) γ-谷氨酰转肽酶(U/L) 凝血酶原时间(s) 国际标准化比值 白细胞计数(×109/L) 红细胞计数(×1012/L) 血小板计数(×109/L)
2020-12(术前) 17.5 4.4 36 80 26 16.4 1.27 5.53 4.46 108
2021-03 11.8 3.4 64 78 32 16.9 1.38 7.80 3.87 79
2021-11 20.1 4.7 42 119 33 - - 6.71 4.26 92
2022-05 25.7 5.3 45 116 38 - - 5.86 4.02 84
2023-01 26.1 5.9 77 136 47 - - 6.20 3.73 88
2023-04(术前末次) 30.3 1.3 70 80 50 12.0 1.06 9.67 4.32 93
2023-05(术后) 17.4 5.2 62 113 72 11.2 1.11 9.04 3.79 96
2023-06(术后1个月) 33.6 9.1 23 92 28 13.2 1.2 5.43 3.33 91
2023-09(术后4个月) 14.3 3.9 22 53 20 15.5 1.23 5.20 3.52 158
2024-10(术后17个月) 40.7 9.2 26 80 27 - - 10.50 3.67 111
随访时间 白蛋白(g/L) 血氨(μmol/L) 肝性脑病 腹水 肝功能Child-Pugh分级(评分) 血清肌酐(μmol/L) 24 h尿蛋白定量(g) 尿蛋白 尿红细胞(个/μl) 尿红细胞(/高倍视野)
2020-12(术前) 20.3 - 否 中度 B级(9分) 77 1.68 2+ 253 46.0
2021-03 18.5 - 否 中度 B级(9分) 105 1.14/1.98 1+ 411 75.0
2021-11 17.3 - 否 轻度 - 85 - 3+ 251 46.0
2022-05 20.4 - 否 轻度 - 96 - 3+ 367 67.0
2023-01 18.6 - 否 中度 - 128 - 2+ 222 -
2023-04(术前末次) 21.2 323 是(Ⅲ级) 中度 C级(11分) 133 2.12 3+ - 112.0
2023-05(术后) 18.4 108/84/69 是(Ⅱ级) 中度 C级(10分) 109 0.75/0.73 1+ 60 10.8
2023-06(术后1个月) 20.7 59 否 轻度 B级(8分) 87 0.19/0.29 +- 152 27.4
2023-09(术后4个月) 18.7 21 是(Ⅰ级) 轻度 B级(8分) 119 - 1+ - 4.0~6.0
2024-10(术后17个月) 19.8 30.3 否 轻度 - 119 - 1+ - 8.0~10.0
表2 文献报道的Abernethy畸形合并肾小球疾病病例特征汇总
图3 Abernethy畸形相关IgA肾病的可能病理机制
[1]
Kumar P, Bhatia M, Garg A, et al. Abernethy malformation: a comprehensive review [J]. Diagn Interv Radiol, 2022, 28(1): 21-28.
[2]
Gopal P, Banerjee D, Karad A, et al. Gastrointestinal: congenital portosystemic shunt (Abernethy type 2) with cirrhosis of liver [J]. J Gastroenterol Hepatol, 2024, 39(5): 781-782.
[3]
Xu L, Zhang H, Liu G, et al. Abernethy malformation with unusual cardiac malformation: case report and literature review [J]. Echocardiography, 2023, 40(1): 57-60.
[4]
Schaeffer DF, Laiq S, Jang HJ, et al. Abernethy malformation type Ⅱ with nephrotic syndrome and other multisystemic presentation: an illustrative case for understanding pathogenesis of extrahepatic complication of congenital portosystemic shunt [J]. Hum Pathol, 2013, 44(3): 432-437.
[5]
魏纯淳,陈大进,黄洪锋,等.Abernethy畸形合并膜性肾病一例[J].中华肾脏病杂志,2017, 33(7): 540-541.
[6]
刘艳,王文红,刘杨,等.Abernethy畸形合并IgA肾病一例[J].中华儿科杂志,2020, 58(7): 607-608.
[7]
He X, Zhu Y, Fu H, et al. Case report: membranoproliferative glomerulonephritis, a rare clinical manifestation of Abernethy malformation type Ⅱ [J]. Front Pediatr, 2021, 9: 647364.
[8]
Wu X, Gu W, Lin Y, et al. A rare presentation of type Ⅱ Abernethy malformation and nephrotic syndrome: case report and review [J]. Open Life Sci, 2022, 17(1): 794-799.
[9]
Goyal D, Tyagi V, Mantan M, et al. Membranoproliferative glomerulonephritis in a child with congenital portosystemic shunt [J]. Pediatr Nephrol, 2024, 39(12): 3459-3462.
[10]
Stamellou E, Seikrit C, Tang SCW, et al. IgA nephropathy [J]. Nat Rev Dis Primers, 2023, 9(1): 67.
[11]
Tissandié E, Morelle W, Berthelot L, et al. Both IgA nephropathy and alcoholic cirrhosis feature abnormally glycosylated IgA1 and soluble CD89-IgA and IgG-IgA complexes: common mechanisms for distinct diseases [J]. Kidney Int, 2011, 80(12): 1352-1363.
[12]
De Smet A, Kuypers D, Evenepoel P, et al. 'Full house’ positive immunohistochemical membranoproliferative glomerulonephritis in a patient with portosystemic shunt [J]. Nephrol Dial Transplant, 2001, 16(11): 2258-2262.
[13]
Karashima S, Hattori S, Nakazato H, et al. Membranoproliferative glomerulonephritis in congenital portosystemic shunt without liver cirrhosis [J]. Clin Nephrol, 2000, 53(3): 206-211.
[14]
Dash SC, Bhuyan UN, Dinda AK, et al. Increased incidence of glomerulonephritis following spleno-renal shunt surgery in non-cirrhotic portal fibrosis [J]. Kidney Int, 1997, 52(2): 482-485.
[15]
Okamoto N, Fukazawa S, Shimamoto M, et al. Remission of membranoproliferative glomerulonephritis associated with a noncirrhotic portosystemic shunt after percutaneous transhepatic portal vein embolization [J]. NDT Plus, 2009, 2(3): 228-232.
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