1. X-linked ichthyosis: relation between cholesterol sulphate, dehydroepiandrosterone sulphate and patient's age.
- Author
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Delfino M, Procaccini EM, Illiano GM, and Milone A
- Subjects
- Adolescent, Adult, Age Factors, Aged, Child, Child, Preschool, Chromatography, Gas, Humans, Male, Middle Aged, Cholesterol Esters blood, Dehydroepiandrosterone Sulfate blood, Ichthyosis, X-Linked blood
- Abstract
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.
- Published
- 1998
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