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We describe two cases of congenital chylothorax in siblings with important differences from previously described familial cases. Our findings support the likelihood of an autosomal recessive inheritance in some cases of this condition, rather than X-linked recessive inheritance, which has also been suggested. Autopsy findings from one of these cases and others previously described suggest that the pathophysiological mechanisms involved may be variable. 相似文献
33.
KB Reddy P Gascard MG Price EV Negrescu JE Fox 《Canadian Metallurgical Quarterly》1998,273(52):35039-35047
Signaling across integrins is regulated by interaction of these receptors with cytoskeletal proteins and signaling molecules. To identify molecules interacting with the cytoplasmic domain of the beta3-integrin subunit (glycoprotein IIIa), a placental cDNA library was screened in the yeast two-hybrid system. Two identical clones coding for a 96-amino acid sequence were identified. This sequence was 100% identical to a sequence in skelemin, a protein identified previously in skeletal muscle. Skelemin is a member of a superfamily of cytoskeletal proteins that contain fibronectin-type III-like motifs and immunoglobulin C2-like motifs and that regulate the organization of myosin filaments in muscle. The amino acid residues in the isolated clones encompassed C2 motifs 4 and 5 of skelemin. A recombinant skelemin protein consisting of C2 motifs 3-7 interacted with beta1- and beta3-integrin cytoplasmic domains expressed as glutathione S-transferase (GST) fusion proteins, but not with GST-beta2-integrin cytoplasmic tail or GST alone. The skelemin-binding region was in the membrane proximal cytoplasmic domains of the integrins. Full-length skelemin interacted with integrin in intact cells as demonstrated by the colocalization of hemagglutinin-tagged skelemin in Chinese hamster ovary (CHO) cells containing alphaIIbbeta3-integrin and by the finding that microinjection of C2 motif 4 of skelemin into C2C12 mouse myoblast cells caused spread cells to round up. A skelemin-like protein was detected in CHO cells, endothelial cells, and platelets, and this protein colocalized with beta1- and beta3-integrins in CHO cells. This study suggests the presence of a skelemin-like protein in non-muscle cells and provides evidence that it may be involved in linking integrins to the cytoskeleton. 相似文献
34.
MS Lefcoe GA Fox DJ Leasa RK Sparrow DG McCormack 《Canadian Metallurgical Quarterly》1994,105(3):885-887
Sixty-six supine portable chest radiographs done on the day of bronchoscopy in 62 critical care unit patients suspected of having pneumonia were examined in a blinded fashion by two radiologists. Quantitative culture results obtained from protected brush catheter (PBC) specimens were compared with chest radiograph scores. For one observer, the sensitivity of the chest radiograph for predicting the presence of positive culture results was 0.60, specificity was 0.29, overall agreement was 0.41, positive predictive value was 0.34, and negative predictive value was 0.55. For the second observer, the values were as follows: sensitivity, 0.64; specificity, 0.27; overall agreement, 0.41; positive predictive value, 0.35; and negative predictive value, 0.55. The kappa statistic was calculated at 0.27 indicating marginal interobserver reproducibility. We conclude the portable chest radiograph in the critical care setting is not accurate in predicting the presence of pneumonia when the diagnosis is based on quantitative cultures obtained from protected brush catheter specimens. 相似文献
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Joseph R. Fox P. Costa Kokoropoulos Gary H. Wiseman H. K. Bowen 《Journal of Materials Science》1987,22(12):4528-4531
Organosilanes of the general formula R
x
Si(OR)4–x
(where R is an alkyl group and R = CH3 or CH2CH3) were used to sterically stabilize hexane dispersions of submicrometre silica spheres. The dispersions were characterized according to sediment volume results. For 0.5m silica particles, the sediment density increased by more than a factor of three up to 50 to 55% of theoretical in the presence of organosilanes with 12 or more carbons in the R group. Solid-state13C nuclear magnetic resonance was used to characterize the powder-dispersant interaction; this technique can distinguish between carbons in the R group of the organosilane and residual organic groups in the silica. Scanning electron micrographs of filter compacts were used to further characterize the dispersions and indicated the presence of primary particles as well as small agglomerates. 相似文献
39.
Five field experiments involving P application rates from 0 to 66 kg P ha–1 were conducted on irrigated wheat at Tandojam, Pakistan. The soils belonged to two great soil groups, Torrifluvent and Camborthid. All soils were calcareous. Olsen-P contents ranged from 3.5 to 6.3 mg P kg–1. Phosphate sorption curves were developed for soils from control (no P) plots at each site. Concentrations of P in solution established by fertilization in the field as estimated from the sorption curves ranged from 0.008 to 0.16mg P L–1. Actual grain yields were converted to relative grain yields and plotted against corresponding concentrations of P in solution. Yield response to P application was obtained in each experiment. Control plot yields ranged from 57 to 89% of maximum yield of respective experiments. Phosphorus requirements of wheat were 0.032 mg L–1 for 95% yield as determined from a composite yield response curve. Predicted quantities of P required to attain 0.032 mg P L–1 ranged from 18 to 29 kg P ha–1. The results of the study suggest that the P sorption approach can be used as a rational basis for making P fertilizer recommendations for various soil-crop combinations. 相似文献
40.
Dieter Braun Manfred Rtzsch Peter Fiedler Günter Weber Heinz Hofmann Bernd Gutschalk 《大分子材料与工程》1991,187(1):101-121
Based on the example of PE-LD grades and on model substances obtained by preparative fractionation (fractions in the molecular weight range M?w = 1.2 ? 390·104 g/mol), comprehensive investigations regarding the problems of structure/properties have been carried out. The correlations of M?w with 15 physical and application technological perameters have been investigated taking into account the reaction mechanism during the manufacturing of PE-LD in the tubular and agitated autoclave reactor, resulting in the formation of so-called primary structure perameters M?n, M?w, molar mass distribution, short chain branching and long chain branching. For the interpretation of the results, among other factors, also the morphological conditions have been taken into consideration, which in connection with the molecular parameters reveal — for specific M?w-ranges — strong changes and saturation ranges, resp., for the physical and application technological properties. 相似文献