Dent disease type 1

Orpha code: 93622OMIM code: 310468

Definition

A rare X-linked monogenic renal tubular disease, characterized by manifestations of complex proximal tubule dysfunction with low-molecular-weight (LMW) proteinuria, hypercalciuria, nephrolithiasis, nephrocalcinosis, and progressive renal failure. Extra-renal involvement is absent.

Disease data
Classification

Clinical subtype

Synonyms
Kamica nerkowa typu 1
ORPHA code
93622
OMIM code
310468
ICD10 code
N25.8
ICD11 code
-

No additional description.

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