X-linked ichthyosis: relation between cholesterol sulphate, dehydroepiandrosterone sulphate and patient's age
✍ Scribed by Delfino; Procaccini; Illiano; Milone
- Book ID
- 104458668
- Publisher
- John Wiley and Sons
- Year
- 1998
- Tongue
- English
- Weight
- 384 KB
- Volume
- 138
- Category
- Article
- ISSN
- 0007-0963
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✦ Synopsis
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.