Cholesterol supplementation does not improve developmental progress in Smith-Lemli-Opitz syndrome - 24/08/11
, Mark Ruggiero, MD, Kersti Petit-Kekel, BS, Louise S Merkens, PhD, William E Connor, MD, Robert D Steiner, MD
Reprint requests: Darryn M. Sikora, PhD, CDRC/Oregon Health and Science University; PO Box 574, Portland, OR 97207-0574.Abstract |
Objective |
Smith-Lemli-Opitz syndrome (SLOS) results in multiple malformations, growth deficiency, and mental retardation. Cholesterol supplementation has been used for several years to treat symptoms of SLOS. We assessed the developmental progress of children and adolescents with SLOS over a 6-year period.
Study design |
Patients with SLOS (n=14) received continuous cholesterol supplementation as part of a longitudinal study. Assessment of their developmental progress in the areas of cognitive, motor, and adaptive skills occurred every 6 to 12 months. The progress of each subject over time and the progress of the group as a whole were analyzed by using a repeated-measures design and multiple t tests.
Results |
Developmental quotients did not improve over time for children with SLOS receiving cholesterol. In addition, baseline cholesterol levels, rather than age when supplementation began or increase in cholesterol levels, best predicted developmental outcome.
Conclusions |
These results suggest that cholesterol supplementation in its current form does not improve the developmental progress of children and adolescents with SLOS.
Le texte complet de cet article est disponible en PDF.Abbreviations : CNS, 7-DHC, DQ, GCRC, SLOS
Plan
 | Supported by Oregon Child Health Research Center (PHS HD33703), the General Clinical Research Center (PHS RR003344 and RR003344-33S3), a CAP award to R. D. S., the American Academy of Pediatrics Section on Genetics and Birth Defects, the Collins Foundation, the Smith Lemli Opitz Advocacy and Exchange Group, and PHS HL64618 to W. E. C. |
Vol 144 - N° 6
P. 783-791 - juin 2004 Retour au numéro
Article précédent
- Lifestyle factors and the development of bone mass and bone strength in young women
- Tom Lloyd, Moira A Petit, Hung-Mo Lin, Thomas J Beck
- Growth of pediatric intensive care units in the United States from 1995 to 2001
- Adrienne G Randolph, Calle A Gonzales, Lynelle Cortellini, Timothy S Yeh
Bienvenue sur EM-consulte, la référence des professionnels de santé.
L’accès au texte intégral de cet article nécessite un abonnement.
Déjà abonné à cette revue ?