Homozygous beta thalassemia in an African-American pediatric patient.
Homozygous beta-thalassemia in an African-American pediatric patient is rare. In homozygous, beta-thalassemia there are characteristic changes due to severe chronic hemolytic anemia. Orofacial characteristics of homozygous beta-thalassemia result from expansion of bone marrow causing skull and facial deformities. This expansion causes clinically recognizable maxillary hyperplasia, severe protrusion of the middle third of the face, and anterior displacement of the incisors producing a typical faces historically referred to as "Cooley's face." The orofacial characteristics of an African American pediatric patient are described in detail.
DeBall
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Department of Pediatric Dentistry, University of Mississippi School of Dentistry, Jackson 39216, USA.
Gordy
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FM
D044383:Black People; D002648:Child; D002652:Child Behavior; D017742:Dental Care for Chronically Ill; D005147:Facial Bones / Q000002:abnormalities; D019066:Facies; D006720:Homozygote; D006801:Humans; D008297:Male; D008310:Malocclusion / Q000209:etiology; D019767:Maxillofacial Abnormalities / Q000209:etiology / Q000473:pathology*; D017086:beta-Thalassemia / Q000150:complications / Q000235:genetics / Q000473:pathology*; D001741:Black or African American