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March 25, 2026Kidney International ReportsOpen Access

WCN26-5465 An adult case of cyanotic nephropathy with light chain proximal tubulopathy after partial remission of membranous nephropathy

Masayoshi Ishii Daisuke Honda Hanae Wakabayashi Katsuhiko Asanuma

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Key Points

The aim is to report a case of a novel REN gene mutation leading to ADTKD and associated renal abnormalities.
Case report of a 23-year-old man with a heterozygous REN mutation
Family history of kidney disease and genetic testing for ADTKD
Magnetic resonance imaging and renal biopsy performed
MRI showed isointense signals in renal cortices indicative of cyst-like structures
Renal biopsy revealed dilated cyst-like structures with fibrosis and positive GATA binding protein 3 staining
The water-containing tubular structures may be characteristic of ADTKD-REN

Implication

A case report demonstrates a novel REN mutation causing kidney disease and structural changes in imaging.

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Cite This Study

Ishii et al. (2026) studied this question.

synapsesocial.com/papers/69c76fff8bbfbc51511e0595 https://doi.org/10.1016/j.ekir.2026.105822

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