CEN Case Reports

, Volume 7, Issue 2, pp 259–263 | Cite as

A decreased soluble Klotho level with normal eGFR, FGF23, serum phosphate, and FEP in an ADPKD patient with enlarged kidneys due to multiple cysts

  • Takahiro KanaiEmail author
  • Kazuhiro Shiizaki
  • Hiroyuki Betsui
  • Jun Aoyagi
  • Takanori Yamagata
Case Report


Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disorder. ADPKD is characterized clinically by the presence of multiple bilateral renal cysts that lead to chronic renal failure. The cysts evolve from renal tubular epithelial cells that express the Klotho gene. Notably, Klotho acts as a co-receptor for fibroblast growth factor 23 (FGF23); in this context, it induces phosphaturia and maintains serum phosphate at a normal level. Many reports have shown that decreases in the soluble Klotho level and increases in the FGF23 level are associated with glomerular filtration rate (GFR) decline, but a recent study observed these changes in patient with normal eGFR. It remains unclear whether the decrease in the Klotho level precedes the increase in FGF23. Here, we present an ADPKD patient with enlarged kidneys due to multiple cysts who had a decreased soluble Klotho level but a normal eGFR and a normal FGF23 level. The patient’s serum phosphate level was normal, as was the fractional excretion of phosphate (FEP). This appears to be the first reported case to show a decreased soluble Klotho level plus normal eGFR, FGF23, and FEP. These results suggest that Klotho decreases before FGF23 increases and further suggest that Klotho is not required to maintain normal serum phosphate levels in ADPKD if the FEP and serum phosphate levels are normal.


ADPKD FGF23 Klotho Children 


Compliance with ethical standards

Conflict of interest

The authors have declared that no conflict of interests exists.

Consent for publication

All authors read this manuscript and approved it for publication.

Ethical standards

This study complies with the Declaration of Helsinki and was approved by the Ethics Committee of Jichi Medical University (A17-0088).

Study involving human and/or animal participants

All procedures performed in the studies involving human participants were in accordance with the ethical standards of the institutional research committee where the studies were conducted and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study and from their parents.


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Copyright information

© Japanese Society of Nephrology 2018

Authors and Affiliations

  • Takahiro Kanai
    • 1
    Email author
  • Kazuhiro Shiizaki
    • 2
  • Hiroyuki Betsui
    • 1
  • Jun Aoyagi
    • 1
  • Takanori Yamagata
    • 1
  1. 1.Department of PediatricsJichi Medical UniversityShimotsukeJapan
  2. 2.Division of Anti-aging Medicine, Center for Molecular MedicineJichi Medical UniversityShimotsukeJapan

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