Nance-horan syndrome

General: Rare; X-linked oculodental trait; occurs in both s 545g62f exes; present from birth.

Ocular: Posterior sutural cataracts in females; zonular cataracts in males; decreased visual acuity; nystagmus; microcornea; punctate opacities of cornea; affected males have dense nuclear cataracts and frequently microcorneas; carrier females may show posterior Y sutural cataracts with small corneas and only slightly reduced vision.

Clinical: Dental anomalies, including supernumerary central incisors; short fourth metacarpals; diastemata in females.

Bixler D, et al. The Nance-Horan syndrome: a rare X-linked ocular-dental trait with expression in heterozygous females. Clin Genet 1984; 26:30-35.

Horan MB, et al. X-linked cataract and hutchinsonian teeth. Aust Paediatr J 1974; 10:98-l02.

McKusick VA. Mendelian Inheritance in Man; A Catalog of Human Genes and Genetic Disorders, 12^th ed. Baltimore: The Johns Hopkins University Press, 1998.

Online Mendelian Inheritance in Man, OMIM. McKusick-Nathans Institute for Genetic Medicine, Johns Hopkins University and National Center for Biotechnology Information, National Library of Medicine, February 12, 2007. World Wide Web URL: https://www.ncbi.nlm.nih.gov/omim/.

Nance WE, et al. Congenital sex-linked cataract, dental anomalies, and brachymetacarpalia. Birth Defects 1974; 10: 285-287.

van Dorp DB, Delleman JW. A family with X-chromosomal recessive congenital cataract, microphthalmia: a peculiar form of the ear and dental anomalies. J Pediatr Ophthalmol Strabismus 1979; 16:166-l71.