Genetic and clinical advances in Prader-Willi syndrome

Curr Opin Pediatr. 1996 Dec;8(6):618-24. doi: 10.1097/00008480-199612000-00013.

Abstract

Prader-Willi syndrome is a developmental disorder with distinctive dysmorphic features, specific neurobehavioral attributes, and a characteristic learning profile. Advances continue to be made in understanding the factors associated with the loss of imprinted gene expression within chromosome 15q11-q13. These advances are helping providers make certain diagnoses early and are helping scientists uncover new genetic pathways. In addition, efforts to further understand the role of recombinant growth hormone therapy in Prader-Willi syndrome and the genetic information responsible for the neurobehavioral profile are additional targets for research.

Publication types

  • Review

MeSH terms

  • Child, Preschool
  • Chromosome Deletion*
  • Chromosomes, Human, Pair 15*
  • Female
  • Genomic Imprinting
  • Human Growth Hormone / metabolism
  • Human Growth Hormone / therapeutic use
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Prader-Willi Syndrome / drug therapy
  • Prader-Willi Syndrome / genetics*
  • Prader-Willi Syndrome / physiopathology
  • Prader-Willi Syndrome / psychology

Substances

  • Human Growth Hormone