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A hereditary kidney disease characterized by proteinuria, type IV renal tubular acidosis, microscopic hematuria and hypertension that may lead to end-stage renal failure in the second to sixth decade of life.
Features include very common findings: Reduced kidney function (renal insufficiency), Protein in the urine (proteinuria), Nephrotic syndrome, and Hypertension and others; and sometimes findings: Cerebral hemorrhage.
Organ System | Phenotype Count | Example Features |
|---|---|---|
Kidneys and urinary system | 6 | Reduced kidney function (renal insufficiency), Protein in the urine (proteinuria), Nephrotic syndrome |
Phenotype severity distribution: 9 very common features.
Estimated prevalence: <1 in 1,000,000 (VERY_RARE).
No clinical trials have been registered for fibronectin glomerulopathy.
9 publications have been identified in PubMed for fibronectin glomerulopathy. Research spans Case Report / Case Series (100%).
Sannino A (2026). [PMID: 42143752](https://pubmed.ncbi.nlm.nih.gov/42143752/). *G Ital Nefrol*. [Case Report / Case Series]
Shi B (2026). [PMID: 41642109](https://pubmed.ncbi.nlm.nih.gov/41642109/). *Clinical nephrology*. [Case Report / Case Series]
Chen X (2026). [PMID: 42068364](https://pubmed.ncbi.nlm.nih.gov/42068364/). *Pediatr Nephrol*. [Case Report / Case Series]
Wei J (2026). [PMID: 42150947](https://pubmed.ncbi.nlm.nih.gov/42150947/). *Transplant Proc*. [Case Report / Case Series]
Nagayama Y (2025). [PMID: 40120032](https://pubmed.ncbi.nlm.nih.gov/40120032/). *CEN case reports*. [Case Report / Case Series]
Tsighe AZ (2025). [PMID: 40529320](https://pubmed.ncbi.nlm.nih.gov/40529320/). *Case reports in nephrology and dialysis*. [Case Report / Case Series]
Data assembled from 4 of 12 sources · Last updated Sep 18, 2026, 7:12 PM UTC
European rare disease database
Genetic and Rare Diseases Info Center
Heart and blood vessels |
1 |
Hypertension |
Brain and nerves | 1 | Cerebral hemorrhage |
Kalmár T (2025). [PMID: 39859354](https://pubmed.ncbi.nlm.nih.gov/39859354/). *International journal of molecular sciences*. [Case Report / Case Series]
Chen Q (2025). [PMID: 41656823](https://pubmed.ncbi.nlm.nih.gov/41656823/). *Zhong nan da xue xue bao. Yi xue ban = Journal of Central South University. Medical sciences*. [Case Report / Case Series]
Choi JY (2024). [PMID: 39285372](https://pubmed.ncbi.nlm.nih.gov/39285372/). *BMC nephrology*. [Case Report / Case Series]
AI-curated news mentioning fibronectin glomerulopathy
Updated Sep 1, 2026
A 17-year clinical course study demonstrates successful management of late-onset nephrotic syndrome in patients with FN1-associated fibronectin glomerulopathy. This research highlights the potential for long-term control of this rare condition.
A case report highlights the occurrence of concomitant fibronectin glomerulopathy and chronic active antibody-mediated rejection in a renal allograft. This study contributes to the understanding of complex renal transplant complications.