StudyRareStudyRare

Jacobs syndrome (47,XYY)

Log in to star

Last updated 2mo ago

Log in to add personal notes on this page.

A tall adolescent male is referred for behavioral concerns and learning difficulties. Physical exam is unremarkable except for tall stature. Karyotype obtained during workup reveals 47,XYY.

  • Karyotype: 47,XYY
  • Paternal nondisjunction (meiosis II)
  • Tall stature
  • Normal fertility (usually)
  • Learning difficulties (speech/language delays common)
  • Behavioral issues in some (historically overstated)
  • Normal physical exam (no distinctive dysmorphisms)
  • Karyotype confirms 47,XYY and is diagnostic
  • Often an incidental finding on prenatal cell-free DNA screening, chromosomal microarray, or a karyotype ordered for developmental or behavioral concerns
  • Prenatal positive screens require confirmation by diagnostic testing (amniocentesis or CVS karyotype)
  • Educational support as needed
  • Supportive care

Reference Links