Electrolyte Blood Press.  2018 Dec;16(2):23-26. 10.5049/EBP.2018.16.2.23.

Long-term Tolvaptan Treatment of Autosomal Dominant Polycystic Kidney Disease in Korea

• Kim HY
• Lee SJ
• Kim BK
• Kim M
• Bae EH
• Ma SK
• Kim SW

• KMID: 2439088
• DOI: http://doi.org/10.5049/EBP.2018.16.2.23

Abstract

A 22-year-old male patient was diagnosed with autosomal dominant polycystic kidney disease (ADPKD). He received conservative treatment with an angiotensin-converting enzyme inhibitor. Two years later, oral therapy, consisting of 60 mg tolvaptan per day, was initiated. Compared with height-adjusted total kidney volume, the rate of kidney growth reduced significantly from 7.33% to 0.66% annually, since commencement of the tolvaptan therapy. The liver enzyme profile and serum sodium level and osmolality were constantly within normal ranges. In Korea, this is the first reported case of a patient with ADPKD who received tolvaptan treatment for more than 1 year. This case demonstrates that long-term tolvaptan treatment appears to be safe, well tolerated, and effective for ADPKD.

Keyword

Autosomal dominant polycystic kidney disease; Tolvaptan; V2-antagonist; Long-term outcome

MeSH Terms

Humans
Kidney
Korea*
Liver
Male
Osmolar Concentration
Polycystic Kidney, Autosomal Dominant*
Reference Values
Sodium
Young Adult
Sodium

Figure

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  Fig. 1 Abdominal computed tomography at January 2015, April 2017, and April 2018. A) Right kidney: length, 171.30 mm; width, 120.29 mm; depth, 65.80 mm. Left kidney: length, 163.42 mm; width, 101.88 mm; depth, 79.86 mm. B) Right kidney: length, 183.88 mm; width, 122.46 mm; depth, 70.25 mm. Left kidney: length, 170.62 mm; width, 102.77 mm; depth, 86.24 mm. C) Right kidney: length, 183.90 mm; width, 126.06 mm; depth, 68.77 mm. Left kidney: length, 164.02 mm; width, 106.24 mm; depth, 86.75 mm.

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  Fig. 2 Genetic analysis using Sanger's method. Direct sequencing revealed that the 22nd intron had translocated from G to A.

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  Fig. 3 Course of serum creatinine levels (line) and estimated glomerular filtration rate (bar) during tolvaptan treatment. From May 2017, tolvaptan was initiated at a daily dose of 60 mg.

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  Fig. 4 Comparing the total kidney volume before and after tolvaptan therapy; the cyst growth rate decreased from 7.33% to 0.66%.

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