1. Academic Validation
  2. Severe hypertelorism, midface prominence, prominent/simple ears, severe myopia, borderline intelligence, and bone fragility in two brothers: new syndrome?

Severe hypertelorism, midface prominence, prominent/simple ears, severe myopia, borderline intelligence, and bone fragility in two brothers: new syndrome?

  • Am J Med Genet A. 2007 Feb 1;143A(3):229-34. doi: 10.1002/ajmg.a.31594.
Hanan A Hamamy 1 Ahmad S Teebi Kamaldine Oudjhane N N Shegem K M Ajlouni
Affiliations

Affiliation

  • 1 National Center for Diabetes, Endocrinology and Genetics, Amman, Jordan.
Abstract

We report on two brothers, born to double first cousin Jordanian Arab parents, with a syndrome comprising severe hypertelorism with upslanted palpebral fissures, brachycephaly, abnormal ears, sloping shoulders, enamel hypoplasia, and osteopenia with repeated fractures. Both have severe myopia, mild to moderate sensori-neural hearing loss and borderline intelligence. Results of chromosome analysis were normal as was a FISH assay for subtelomeric rearrangements. The father has mild hypertelorism but the family history is otherwise unremarkable. We think that this represents a previously unrecognized autosomal or X-linked recessive syndrome.

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