The spectrum of clinical presentation of fatty acid oxidation defects (FAOD) continues to expand. One FAOD, L-3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency has been associated with liver disease in pregnancies involving a heterozygous mother carrying an affected fetus. Hepatic carnitine palmitoyltransferase (CPT I) deficiency typically presents as a Reyelike syndrome in children between 8 and 18 mo. of age. We have investigated a family in which the mother developed liver disease consistent with acute fatty liver of pregnancy (AFLP) and hyperemesis gravidarum in her two successive pregnancies. Neither child nor their mother was found to carry the common LCHAD G1528C mutation. Both children were subsequently shown to have absent activity of CPT I. This is the first report of CPT I deficiency presenting as maternal illness in pregnancy.
This study evaluated the impact of premature birth on the development of local and global motion processing in a group of very low birthweight (<1500 g), 5- to 8-year-old children. Sensitivity to first- and second-order local motion stimuli and coherence thresholds for global motion in random dot kinematograms were measured. Relative to full-term controls, premature children showed deficits on all three aspects of motion processing. These problems could not be accounted for by stereo deficits, amblyopia, or attentional problems. A history of mild retinopathy of prematurity and/or intraventricular hemorrhage increased risk, but deficits were observed in some children with no apparent ocular or cerebral pathology. It is important to note that, despite the observed group differences, individual profiles of performance did vary; the results suggest that these three forms of motion processing may involve separate neural mechanisms. These findings serve to increase our understanding of the organization and functional development of motion-processing subsystems in humans, and of the impact of prematurity and associated complications on visual development.
This study reports on the results of a randomized controlled trial that evaluated a caregiver-based intervention program for children with autism in community day-care centers. Thirty-five preschool children with a DSM III-R diagnosis of autism or pervasive developmental disorder were randomized to an experimental or control group. Children in the experimental group were enrolled in day care and their parents and child care workers received a 12-week intervention consisting of lectures and on-site consultations to day-care centers. In addition, supportive work was undertaken with families. Control subjects received day care alone. In the experimental group, there were greater gains in language abilities, significant increases in caregivers' knowledge about autism, greater perception of control on the part of mothers, and greater parent satisfaction. We conclude that this research design demonstrated that the intervention was significantly superior to day care alone.
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