Kosin Med J.  2017 Jun;32(1):90-98. 10.7180/kmj.2017.32.1.90.

A Case of Steroid Resistant Minimal Change Disease Associated with Portal Vein Thrombosis Treated by Combined Immunosuppressive Agents

• Jung HJ
• Lee SM
• Rha SH
• Kim SE
• Son YK
• Kim KS
• An WS

• KMID: 2384837
• DOI: http://doi.org/10.7180/kmj.2017.32.1.90

Abstract

Minimal change disease (MCD) is a common cause of nephrotic syndrome and relatively well responds with steroid treatment. However, nearly half of patients with MCD experience recurrence of nephrotic syndrome. Thromboembolic events including renal vein thrombosis may occur in patients with MCD, but portal vein thrombosis rarely occurs. We experienced a case of frequent relapse/steroid dependent MCD with nephrotic syndrome progressed to steroid resistance associated with portal vein thrombosis. This patient showed complete remission of MCD and resolution of portal vein thrombosis after treatment with corticosteroid, cyclosporine, mycophenolate mofetil, and anticoagulant.

Keyword

Minimal change disease; Nephrotic syndrome; Recurrence; Steroid; Thrombosis

MeSH Terms

Cyclosporine
Humans
Immunosuppressive Agents*
Nephrosis, Lipoid*
Nephrotic Syndrome
Portal Vein*
Recurrence
Renal Veins
Thrombosis
Venous Thrombosis*
Cyclosporine
Immunosuppressive Agents

Figure

• 

  Fig. 1 Renal histologic finding. A. Light microscopy. ×400, PAS (October, 2003). A glomerulus is normal in size and shows normal cellularity. B. Light microscopy. ×40, PAS (December, 2012). There is no focal and segmental glomerulosclerosis on lots of glomerulus. C. Light microscopy. ×400, PAS (December, 2012). Compared to the previous biopsy (October, 2003), there is no interval change. D. Electron microscopy. (December, 2012). The electron microscopic examination shows effaced foot process of epithelial cell (arrow).

• 

  Fig. 2 Abdominal ultrasonography image. A. Internal hyperechoic material suggesting a portal vein thrombosis was shown on main portal vein (December, 2013). B. Regression of portal vein thrombosis with normal portal venous flow was shown on doppler ultrasonography (March, 2015).

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  Fig. 3 Long-term treatment course of steroid resistant minimal change disease. A. Treatment with steroid, cyclosporine, and mycophenolate mofetil (MMF) resulted in complete remission. B. Changes of urine protein to creatinine ratio and serum albumin levels. The left side Y-axis shows urine protein to creatinine ratio (g/g), while the right side Y-axis shows albumin levels (g/dL).

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