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Hays-wells syndrome (aec syndrome; ankyloblepharon-ectodermal defects-cleft lip/palate syndrome)


Hays-Wells Syndrome (AEC Syndrome; Ankyloblepharon-Ectodermal Defects-Cleft Lip/Palate
Syndrome) 313d32d 313d32d 313d32d 313d32d 313d32d 313d32d 313d32d

General: Autosomal dominant disease, as initially described, but may exist as an autosomal recessive disorder.

Ocular: Ankyloblepharon, filiforme adnatum (fused eyelids).

Clinical: Coarse, wiry, sparse hair; dystrophic nails; slight hypohidrosis; scalp infections; hypodontia; maxillary hypoplasia; cleft lip and palate.

Greene SL, Michels VV, Doyle JA. Variable expression in ankyloblepharon-ectodermal defects-cleft lip and palate syndrome. Am J Med Genet 1987; 27:207-212.

Seres-Santamaria A, Arimany JL, Muniz F. Two sibs with cleft palate, ankyloblepharon, alveolar synechiae, and ectodermal defects: a new recessive syndrome? J Med Genet 1993; 30:793-795.

Shwayder TA, Lane AT, Miller ME. Hays-Wells syndrome. Pediatr Dermatol 1986; 3:399-402.




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