194268 Sensory processing patterns of children with Smith Lemli Opitz syndrome

Tuesday, November 10, 2009

Victoria S. Spencer , Department of Psychiatry, Kennedy Krieger Institute, Baltimore, MD
Harolyn Belcher, MD , Kennedy Krieger Family Center/Johns Hopkins Department of Pediatrics, Kennedy Krieger Insitute, Baltimore, MD
Forbes D. Porter, MD, PhD , National Institute for Child Health and Human Development (NICHD), National Institutes of Health, Bethesda, MD
Sandra Conley , National Institute for Child Health and Human Development (NICHD), National Institutes of Health, Bethesda, MD
Elaine Tierney, MD , Department of Psychiatry, Kennedy Krieger Institute, Baltimore, MD
Smith Lemli Opitz syndrome (SLOS) is an autosomal recessive disorder causing impairment of cholesterol biosynthesis. SLOS is associated with multiple malformations and mental retardation, affecting up to 1:15,000 births per year. Research examining sensory processing patterns of individuals with SLOS is scant. The purpose of this study is to examine the association between sterol levels (cholesterol, 7-dehydrocholesterol (7DHC), 8-dehydrocholesterol (8DHC)) and sensory processing patterns of children with SLOS. Fifteen children, mean age 8.3 years (S.D. 3.8), with SLOS had caregivers who completed the Sensory Profile questionnaire (SPQ). SPQ is a standardized measure of multiple domains of sensory processing. Lower scores on the SPQ are equivalent to a greater sensory deficit. Rank correlation coefficients were calculated for sterol levels and sensory processing patterns. There were statistically significant negative correlations between 7DHC and low endurance, sensory sensitivity, fine motor, touch, oral, sensory processing related to endurance, modulation related to body position and movement (p<0.05) and 8DHC and low endurance, poor registration, touch, oral and sensory processing related to endurance (p<0.05). This study provides evidence of associations between precursor sterol levels and sensory processing impairment in children with SLOS. Adverse sensory motor effects were found as cholesterol precursors increased in individuals with SLOS. Understanding the association between sensory motor processing and cholesterol may assist in early identification and reduction of adverse outcomes associated with SLOS.

Learning Objectives:
1. Analyze the sensory processing patterns of children with Smith Lemli Opitz syndrome. 2. Identify associations between sensory processing patterns and cholesterol and cholesterol precursor levels in children with Smith Lemli Opitz syndrome.

Presenting author's disclosure statement:

Qualified on the content I am responsible for because: I was involved in the preliminary research process, including data input and development of the database and I had an integral role in analyzing the data collected. I worked very closely with Dr. Tierney, the senior author on the project
Any relevant financial relationships? No

I agree to comply with the American Public Health Association Conflict of Interest and Commercial Support Guidelines, and to disclose to the participants any off-label or experimental uses of a commercial product or service discussed in my presentation.

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