Conditions / Syndrome

oculocerebrorenal syndrome

info · Syndrome · ICD-10: E72.03

A syndrome that has_material_basis_in mutation in the OCRL gene on chromosome Xq26 and that is characterized by hydrophthalmia, cataract, mental retardation, vitamin D-resistant rickets, amino aciduria, and reduced ammonia production by the kidney.

Signs and symptoms

  • Elevated circulating creatine kinase activity
  • Aminoaciduria
  • Short stature
  • Seizure
  • Camptodactyly of finger
  • Renal insufficiency
  • Hypotonia
  • Aggressive behavior
  • Developmental cataract
  • Elevated amniotic fluid alpha-fetoprotein concentration

Also known as: Lowe syndrome; lowe oculocerebrorenal syndrome; oculocerebrorenal syndrome of Lowe