The early childhood intervention provided in the first 3 years of life had effects on heavier LBW premature infants' IQ and verbal performance at age 5 years that were not observed for lighter LBW premature infants. The intervention did not affect health or behavior at age 5 years in either LBW stratum.
Nine children with the Williams syndrome were evaluated for physical, neurodevelopmental, and behavioral characteristics to record the natural history of this disorder. The study subjects, who ranged in age from 10 years to 20 years, generally showed lower than expected cognitive functioning with four of the nine functioning in the severely retarded range. However, all the children showed uneven developmental profiles, compared to measured IQ, with reading abilities exceeding the expected level and visual-motor skills deficient for overall performance expectations. All but one child had evidence of supravalvular aortic stenosis on echocardiography, but there was little morbidity from cardiovascular disease in this group of patients. Although all had grown at or below the fifth percentile in early childhood, seven now were above the fifth percentile for height. Personality attributes that characterize younger children with Williams syndrome persisted in this group of older children.
The Williams syndrome is a sporadic disorder generally characterized by a peculiar pattern of craniofacial abnormalities (elfin facies), prenatal and postnatal growth deficiency, mental retardation, variable congenital heart disease (including supravalvular aortic stenosis), and occasionally infantile hypercalcemia.1 Its etiology, pathogenesis, and possible relationship to vitamin D and calcium metabolism have not as yet been determined.2
Previous reports of children with this syndrome make reference to their overall mental retardation as determined by IQ as measured by various psychological tests.1,3 Frequent mention is also made of these children's tendency to an unusual, outgoing personality.1,3 Other observers describe a unique type of mental deficiency with poor motor coordination but an unusual command of language, often resulting in a superficial overestimation of cognitive abilities.3
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