clinical features

Last reviewed 01/2018

Children usually present in the first year or two of life with:

  • features of Fanconi syndrome
  • failure to thrive
  • vomiting
  • lethargy
  • irritability
  • features of progressive renal tubular and glomerular damage
  • photophobia is common, associated with the deposition of crystals in the cornea and iris
  • often the hair becomes blond due to the inhibition of melanin formation

Milder adolescent and adult forms are known.