Dent Disease

**Semantic type:** Disease or Syndrome

**Definition:** An X-linked, recessive disorder of the proximal renal tubules that presents during childhood, and is characterized by low-molecular weight proteinuria, hypercalciuria, hypophosphatemia rickets, nephrocalcinosis, nephrolithiasis, and progressive kidney failure.

**Synonyms:** - X-Linked Recessive Hypophosphatemic Rickets - X-linked Recessive Hypophosphatemic Rickets