International meeting on Wolf-Hirschhorn syndrome: Update on the nosology and new insights on the pathogenic mechanisms for seizures and growth delay

Am J Med Genet A. 2020 Jan;182(1):257-267. doi: 10.1002/ajmg.a.61406. Epub 2019 Nov 25.

Abstract

"An International Meeting on Wolf-Hirschhorn Syndrome (WHS)" was held at The University Hospital La Paz in Madrid, Spain (October 13-14, 2017). One hundred and twenty-five people, including physicians, scientists and affected families, attended the meeting. Parent and patient advocates from the Spanish Association of WHS opened the meeting with a panel discussion to set the stage regarding their hopes and expectations for therapeutic advances. In keeping with the theme on therapeutic development, the sessions followed a progression from description of the phenotype and definition of therapeutic endpoints, to definition of genomic changes. These proceedings will review the major points of discussion.

Keywords: 4p-; WHS; antiepileptic drugs; hepatoadenomas; seizures.

Publication types

  • Congress
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Chromosome Deletion
  • Chromosomes, Human, Pair 4 / genetics
  • Chromosomes, Human, Pair 4 / immunology*
  • Developmental Disabilities / epidemiology
  • Developmental Disabilities / genetics*
  • Developmental Disabilities / pathology
  • Female
  • Humans
  • Phenotype
  • Seizures / epidemiology
  • Seizures / genetics*
  • Seizures / therapy
  • Spain / epidemiology
  • Wolf-Hirschhorn Syndrome / epidemiology
  • Wolf-Hirschhorn Syndrome / genetics*
  • Wolf-Hirschhorn Syndrome / therapy