Outcome in congenital stridor (laryngomalacia)

Twenty-one children who had been diagnosed as having laryngomalacia by direct laryngoscopy in infancy were reviewed 7 to 12 years later. The natural history of the disease is documented. A wide variation in the time of onset and duration of the stridor was found and there was a high incidence of feeding difficulties. A previously reported association with mental retardation or cerebral palsy is not confirmed. 4 out of the 21 children had early speech problems.     

Menkes's syndrome. Report of a patient treated from 21 days of age with parenteral copper

In an infant with Menkes's steely-hair syndrome, early treatment (from 21 days of age) with parenteral copper failed to halt the disease. In addition to urinary tract abnormalities, panlobular emphysema was present a finding not previously noted in the syndrome.     

Factor V deficiency in Philadelphia-positive chronic myelogenous leukemia

Factor V deficiency has been identified in 8 of 8 patients 7--20 yr of age, with Philadelphia-positive (Ph1+) chronic myelogenous leukemia (CML). In these 8 patients, factor V deficiency was not due to hepatic dysfunction, factor V inhibitors, or disseminated intravascular coagulation. In 3 patients, factor V activity rose 10%--12% (0.10--0.12 U/ml) after the infusion of 28--31 ml/kg body weight of fresh frozen plasma (FFP). The rise persisted less than 14 hr. The mean measured postinfusion rise in factor V was 18% of the expected rise calculated from the volume of FFP infused in the patients' plasma volume. In 4 patients, a small transient rise in factor V activity occurred after splenectomy or plateletpheresis. Factor V deficiency was completely corrected after a marked reduction in bone marrow cellularity in 2 patients with Ph1+ CML treated with extensive chemotherapy, total body irradiation, and bone marrow transplantation. Factor V deficiency was retrospectively observed in 6 of 20 patients, ages 20--80 yr, with Ph1+ CML and 3 of 6 patients with other myeloproliferative disorders. The factor V deficiency appears to be associated with the large myeloid- megakaryocytic cell mass characteristic of CML and other myeloproliferative disorders.     

Extraretinal representations in area V4 in the macaque monkey.

Several neurophysiological studies have shown that the visual cerebral cortex of macaque monkeys performing delayed match-to-sample tasks contains individual neurons whose levels of activity depend on the sample the animal is required to remember. Haenny et al. (1988) reported that the activity of neurons in area V4 of monkeys performing an orientation matching task depends on the orientation for which the animal is searching. It was proposed that these neurons contribute to a representation of the orientation being sought. We have further characterized these neurons by recording visual responses from individual neurons during multiple behavioral tasks. Animals were trained to perform an orientation match-to-sample task using either a visual or a tactile orientation sample. In a set of 89 neurons examined using both types of sample, 25% showed statistically significant effects of sample orientation regardless of whether the sample was visual or tactile. Most of these preferred the same sample orientation in both conditions. These results allow us to specify the nature of the information signaled by these neurons more precisely than has previously been possible. For 193 units tested using one of the matching tasks, responses were also recorded while the animal performed a simple fixation task. In this task the animal was not required to attend to the visual stimuli that were presented. A few neurons that were responsive during the matching task were silent during fixation, but a comparable number was much more responsive during fixation. Across the whole population there was no systematic change in either responsivity or selectivity for orientation under the two conditions.     

Interaction of platelet factor four with cultured vascular endothelial cells

Platelets secrete a low-molecular-weight protein, platelet factor four (PF-4), which binds to and neutralizes heparin and related sulfated glycosaminoglycans (GAGs). To examine the interactions of PF-4 with the GAGs present on endothelial cell surfaces, we incubated 125I-PF-4 with cell suspensions derived from confluent monolayers of cultured bovine aortic endothelium. Binding of 125I-PF-4 was inhibited by a 100-fold excess of nonradioactive PF-4 and varied with duration and temperature of incubation. At 4 degrees C, binding reached equilibrium at 20 minutes with kd = 2.87 mumol/L and Bmax of 63.83 pmol/10(5) cells. Binding capacity was reduced 83.4% by brief incubation of endothelial cells with trypsin and 46.67% by incubation with Flavobacterium heparinase, but was unchanged by chondroitin-ABCase treatment. At 37 degrees C, PF-4 was internalized by confluent monolayer of bovine aortic endothelial cells primarily through low-affinity adsorptive endocytosis. The internalized PF-4 was degraded to amino acids and small peptides with 50% conversion after 18-hour incubation. These studies demonstrate that a secreted platelet protein can bind to and enter endothelial cells. Binding may explain the rapid clearance of released PF-4 from plasma and could have important local effects on endothelial structure and function.     

Superparamagnetic iron oxide-enhanced MR imaging: pulse sequence optimization for detection of liver cancer

The effects of magnetic resonance (MR) pulse sequences and timing parameters on tumor-liver contrast were studied in an animal model of metastatic liver cancer. Six spin-echo (SE), three inversion-recovery (IR), and four gradient-echo (GRE) sequences were evaluated at 0.6 T before and after injection of super-paramagnetic iron oxide. GRE techniques, irrespective of echo time and flip angle, showed the greatest change in signal intensity (enhancement) of the liver after administration of iron oxide. Single-acquisition GRE sequences (16 seconds) matched the contrast-to-noise ratio (C/N) performance of the most effective 6.4-minute SE sequences. Multiexcitation GRE sequences showed tumor-liver C/Ns per unit time that were significantly (P less than .05) higher than those achieved with SE and IR sequences. GRE sequences, which recruit intravoxel dephasing as an additional source of transverse relaxation enhancement (T2*), show a higher C/N per unit time and in this respect seem superior to SE and IR sequences for MR imaging with superparamagnetic iron oxide.     

Duodenal hematoma: the ring sign in MR imaging

Proper management of duodenal hematoma requires that an accurate diagnosis be made using noninvasive radiological methods. Conventional imaging may be nonspecific if there is no history of trauma or coagulopathy. Two cases of duodenal hematoma that were imaged by magnetic resonance (MR) and computed tomography (CT) are described. In both cases the hematoma had a well-defined concentric ring configuration on MR images, a finding which helped establish the diagnosis. MR imaging may provide tissue-specific characterization of duodenal hematomas.