Rare diseases · Sign or symptom

Proximal tubulopathy

HP:0000114

What it means

Dysfunction of the proximal tubule, which is the portion of the duct system of the nephron of the kidney which leads from Bowman's capsule to the loop of Henle.

Rare diseases that can present with this7

The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions. A sign on this page is a conversation to have with a clinician, not a conclusion.

Part of the broader category

Also called: Proximal renal tubule defect · Proximal renal tubulopathy · Proximal tubular defect · Proximal tubule dysfunction · Selective proximal tubular damage

Proximal tubulopathy

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This page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.