In l998, Hopkin et al. described a new disorder characterized by progressive laryngotracheal stenosis, short stature, and arthropathy. We provide extensive clinical information on two additional unrelated patients with this distinctive disorder and propose the name LAPS syndrome, to reflect the most consistently recognized features, namely, Laryngotracheal stenosis, Arthropathy, Prognathism, and Short stature.
Perfusion manometry was used to study the maturation of small intestinal motility in 15 preterm and term infants before the initiation of enteral feeding (study 1); seven of the 11 preterm infants were restudied after 3 weeks or more of enteral feeding (study 2). During study 1, the interdigestive motility of the four term infants exhibited cycles of quiescence and rhythmic activity, compatible with migrating motor complexes observed regularly in adults; in response to feeding, motility changed to irregular, phasic activity. Preterm infants, however, showed only unorganized clusters of phasic activity during fasting, and there was no consistent pattern of motor response to feeding. After 3 or more weeks of enteral feeding, interdigestive cycles with migratory motor complexes and a consistent, obvious motor response to feeding were present. Preterm infants as young as 32 weeks' gestation showed “mature” motor patterns in study 2, results suggesting that intestinal motility matures with postconceptual age.
The difficulty of diagnosing pneumonia by chest x-ray in children less than 4 years of age was studied by comparing the chest roentgenograms of 34 healthy children with the chest roentgenograms of 34 children previously diagnosed as having pneumonia. Review of these films without knowledge of the previous interpretation and without clinical information disclosed variations regarding the roentgenographic confirmation of pneumonia in 24 percent of the cases and variations regarding the location of the lesion in about 50 percent of the cases.
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