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Spec Care Dentist. 2003;23(1):22-7.

Smith-Lemli-Opitz syndrome: a review, case report and dental implications.

Author information

  • 1Caruth School of Dental Hygiene, Baylor College of Dentistry, Texas A & M University Health Science Center, 3302 Gaston Avenue, Dallas, TX 75246, USA. kmuzzin@tambcd.edu

Abstract

Smith-Lemli-Opitz syndrome (SLOS) is an autosomal recessive developmental disorder characterized by multiple congenital malformations, dysmorphic craniofacial features, and mental impairment. SLOS is caused by a deficiency of the enzyme 7-dehydrocholesterol delta7 reductase which converts 7-dehyrocholesterol to cholesterol. This error results in elevated serum levels of 7-dehydrocholesterol and decreased levels of serum cholesterol. This article describes the clinical features and medical treatment of SLOS. A case report is included, with recommended guidelines for providing safe and comprehensive dental care for individuals with SLOS.

PMID:
12887150
[PubMed - indexed for MEDLINE]
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