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Focal segmental glomerulosclerosis in atypical polycystic kidney disease

Article information

Korean J Intern Med. 2017;32(4):766-767
Publication date (electronic) : 2017 January 5
doi : https://doi.org/10.3904/kjim.2016.232
Eun Hui Bae1, Hyunsuk Kim2, Sung Sun Kim3, Seong Kwon Ma1, Soo Wan Kim1
1Department of Internal Medicine, Chonnam National University Medical School, Gwangju, Korea
2Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea
3Department of Pathology, Chonnam National University Medical School, Gwangju, Korea
Correspondence to Soo Wan Kim, M.D. Tel: +82-62-220-6271 Fax: +82-62-225-8578 E-mail: skimw@chonnam.ac.kr
Received 2016 July 22; Revised 2016 July 22; Accepted 2016 July 22.

A 77-year-old man presented complaining of generalized edema for 1 week. He had hypertension and polycystic kidney disease (PKD), which was diagnosed 45 years ago. Blood and urine tests showed low serum albumin (2.3 g/dL) and severe proteinuria (6.38 g/day). Abdominal computed tomography revealed multiple cysts with thin wall calcification in the left kidney, and several cysts in the right kidney and the liver (Fig. 1). Ultrasonography-guided percutaneous renal biopsy for the right kidney revealed focal segmental glomerulosclerosis (Fig. 2). The patient was prescribed diuretics, an angiotensin receptor blocker, aspirin, statin, a β blocker, and an aldosterone antagonist. One month after starting the medicines, he showed improved symptoms and laboratory findings (serum albumin, 3.2 g/dL; urine albumin excretion, 654.8 mg/g creatinine), which further improved after another 3 months (serum albumin, 3.8 g/dL; urine albumin excretion, 128.4 mg/g creatinine).

Figure 1.

Abdominal computed tomography shows multiple cysts in the (A, B) left and right kidney and (C) several cysts in the liver.

Figure 2.

(A, B) Microscopic findings show endocapillary foam cells and extracapillary cytoplasmic hyaline droplets (A, periodic acid-Schiff stain, ×400; B, Jones methenamine silver stain, ×400). (C, D) Electron microscopy reveals swollen podocytes and diffuse effacement of the foot processes.

Nephrotic syndrome is a rare coincidence in atypical PKD. Percutaneous renal biopsy can be performed in selected patients.

Notes

No potential conflict of interest relevant to this article was reported.

Acknowledgements

This study was supported by a grant (CRI13903-21) from the Chonnam National University Hospital Biomedical Research Institute.

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Copyright © 2017 The Korean Association of Internal Medicine

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Figure 1.

Figure 1.

Abdominal computed tomography shows multiple cysts in the (A, B) left and right kidney and (C) several cysts in the liver.

Figure 2.

Figure 2.

(A, B) Microscopic findings show endocapillary foam cells and extracapillary cytoplasmic hyaline droplets (A, periodic acid-Schiff stain, ×400; B, Jones methenamine silver stain, ×400). (C, D) Electron microscopy reveals swollen podocytes and diffuse effacement of the foot processes.