Familial generalized delayed eruption of the dentition with short stature.
1. Prolonged retention of the deciduous dentition, subsequent delay in eruption of the permanent dentition, and retardation in dental maturation are displayed outside the limits of normal variation. 2. The pedigree study of this family reveals the trait of noneruption to be distributed in a characteristic pattern of autosomal dominance. 3. Short stature was noted in this family and placed in the lowest limits of short stature as recorded in standard reference charts. 4. Additional findings included concave midfacial appearance, frontal bossing, delayed skeletal maturation, presence of Wormian bones in the lamboidal suture region of the skull, and strikingly large fontanelles in infancy. 5. The pedigree described represents a syndrome of short stature and delayed eruption of the dentition.
D000293:Adolescent; D000328:Adult; D002648:Child; D006130:Growth Disorders / Q000235:genetics* / Q000503:physiopathology; D006801:Humans; D010375:Pedigree; D013997:Time Factors; D014078:Tooth Eruption; D014094:Tooth, Deciduous / Q000503:physiopathology