X-linked ichthyosis: relation between cholesterol sulphate, dehydroepiandrosterone sulphate and patient's age.
Steroid sulphatase deficiency is a feature of recessive X-linked ichthyosis (RXLI) that causes the accumulation of sulphated steroids (SS) in various organs and cells. In a previous study, we detected elevated cholesterol sulphate (CS) and dehydroepiandrosterone sulphate (DHEAS) serum levels in a group of 15 RXLI patients selected in a narrow age range. In the present study both CS and DHEAS serum levels were qualitatively and quantitatively determined using gas-chromatographic analysis in a group of 33 RXLI patients ranging in age from 3 to 70 years. The levels of CS and DHEAS were significantly increased in all patients. Variations in SS were related both to patients' ages and clinical course of the disease; Serum SS levels start to increase in early infancy, peak at puberty, remain elevated in adults and decrease slightly in the elderly.
Delfino
M
M
Clinica Dermatologica, Università Federico II, Naples, Italy.
Procaccini
E M
EM
Illiano
G M
GM
Milone
A
A
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D002648:Child; D002675:Child, Preschool; D002788:Cholesterol Esters / Q000097:blood*; D002849:Chromatography, Gas; D019314:Dehydroepiandrosterone Sulfate / Q000097:blood*; D006801:Humans; D016114:Ichthyosis, X-Linked / Q000097:blood*; D008297:Male; D008875:Middle Aged
D002788:Cholesterol Esters; D019314:Dehydroepiandrosterone Sulfate; C007045:cholesteryl sulfate