EXCRETION OF (SULFATED) STEROIDS IN THE URINE AND EXCRETION OF CHOLESTEROL SULFATE IN THE FECES OF BOYS WITH RECESSIVE X-LINKED ICHTHYOSIS

The excretion of sulfated steroids was investigated in the urine and feces of six boys aged 9 months to 7 years and 10 months who had recessive X-linked ichthyosis. Profiles of urinary total steroids as well as sulfated steroids were normal. Cholesterol sulfate excretion in the urine was not elevated. In the feces 2–20% of total cholesterol was cholesterol sulfate, whereas in the feces of 28 healthy children no cholesterol sulfate was demonstrable. In the 6 patients total cholesterol excretion (500–2,500 μmol/kg feces) was also elevated in comparison with the 28 healthy controls (150–700 μmol/kg feces, mean 365 μmol/kg feces)