Abstract for presentation at 11th International Congress of Human Genetics

De novo duplication (19)(q13.1q13.3); second case with a mild phenotype

  • Julie McGaughran, Queensland Clinical Genetics Service, Australia
  • Jennifer Bryan, Mater Health Services, Australia
  • Duplication of chromosome 19q is an uncommon cytogenetic finding. Most cases have been reported as the result of an unbalanced translocation. Of those cases that are the result of a pure duplication, most have significant physical and developmental problems with a resultant poor prognosis.
    The case presented here has relatively mild features. She was born following a normal pregnancy weighing 4.2kg at term. There were no early concerns about her growth or development. She had been assessed by an ophthalmologist because of a family history of retinitis pigmentosa. Her eyes were normal. At 8 months she had physiotherapy as she had low muscle tone and was not rolling or sitting. Investigations carried out at this time showed a de novo duplication, 46,XX,dup(19)(q13.1q13.3).ish dup(19)(q13.1q13.3)(wcp19+,D19S238E+). At 11 months, she had remained relatively tall for her age with a relatively large head. She had mild plagiocephaly and wide spaced eyes. She had 2 grooves in her right ear lobe and bilateral preauricular pits. A cerebral CT scan was normal. She was sitting and rolling and had 2-3 words. She was about 2 months behind in most areas of development.
    This is only the second case with a duplication of 19q with a relatively mild phenotype. The features will be discussed in more detail.

    Conference Organiser - ICMS Pty Ltd