17th Annual PRADER-WILLI SCIENTIFIC CONFERENCE

Salt Lake City, UT

JULY 11, 2002 

SESSION I – Genetics     

•  DNA Methylation of the UBE3A and ATP10C Genes Differs from the Other Imprinted Genes in the AS/PWS Region

• Imprint establishment in Prader-Willi and Angelman syndromes is controlled by two major transcription factors acting at alternative promoters of Snurf-Snrpn during gametogenesis and preimplantation development

• Four genes, including the related and ancient NIPA1 and NIPA2 genes within the Prader-Willi/Angelman syndromes deletion region, have undergone evolutionary transposition mediated by HERC2- or adjacent duplicons

• Phenotypic Variablility and Genetic Findings in Three Siblings with PWS

• Growth Hormone Therapy in Very Young Children with Prader-Willi syndrome: Preliminary Data

SESSION II – Metabolism  

• Effect of Growth Hormone Administration on Body Composition, Lumbar Spine-Bone Mineral Density, Resting Energy Expenditure, Pulmonary Function, Sleep and IQ and Metabolic Parameters in Children with Prader-Willi Syndrome
   

• Neuroanatomical Correlates of Satiation and Hunger in Adults with Prader-Willi Syndrome: A Study Using fMRI
   

• Elevated Plasma Ghrelin Levels May Contribute to Obesity in Prader-Willi Syndrome
   

• Insights into energy homeostasis of a Prader-Willi syndrome mouse model

SESSION III – Obesity/Feeding   

• Multidisciplinary approach for the treatment of obesity in two adult subjects with PWS

• Comparison of PWS and PWS-Like Individuals Clinically, Cognitively and Biochemically

• Body Composition and Fat Analysis in Prader-Willi Syndrome

• Impact of Supplemental Therapies and Nutritional Support on the Duration of Nasogastric Feedings in Infants with Prader-willi syndrome

SESSION IV – Medical     

• UNEXPECTED DEATH AND CRITICAL ILLNESS IN PRADER-WILLI SYNDROME:  REPORT OF NINE INDIVIDUALS

• Disappointing clinical experience with topiramate in patients with Prader-Willi Syndrome: adverse behavioral effects and renal tubular acidosis

• INCIDENCE OF HEPATIC ABNORMALITIES IN INDIVIDUALS WITH PRADER-WILLI SYNDROME

• Compulsivity, GABA and Gender Differences in PWS

• Estimate of Long Term Health Care Costs for Adults with Prader-Willi Syndrome

• Reversal of Severe Right Heart Failure in PWS

July 2002