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31.
Margaret J Bowers Nick J Orr Si Dempsey H Denis Alexander 《Blood coagulation & fibrinolysis》2006,17(5):409-411
We present a case of Bernard Soulier syndrome in a 9-year-old boy caused by a novel genetic mutation. This child was shown to be homozygous for a single nucleotide deletion (c.1077delG) in the GP1BA gene not previously reported. Clinically, the boy has become refractory to platelet transfusions with both allo-antibodies and iso-antibodies and a massive transfusion requirement for ongoing haemorrhage. We describe the critical role that the blood product transfusion continues to play in the management of Bernard Soulier syndrome and discuss therapeutic options in these patients. 相似文献
32.
In-vitro/in-vivo correlation of pulsatile drug release from press-coated tablet formulations: a pharmacoscintigraphic study in the beagle dog. 总被引:1,自引:0,他引:1
Manish Ghimire Fiona J McInnes David G Watson Alexander B Mullen Howard N E Stevens 《European journal of pharmaceutics and biopharmaceutics》2007,67(2):515-523
The aim of the current study was to investigate the in-vitro and in-vivo performance of a press-coated tablet (PCT) intended for time delayed drug release, consisting of a rapidly disintegrating theophylline core tablet, press-coated with barrier granules containing glyceryl behenate (GB) and low-substituted hydroxypropylcellulose (L-HPC). The PCTs showed pulsatile release with a lag time dependent upon the GB and L-HPC composition of the barrier layer. In-vivo gamma-scintigraphic studies were carried out for PCTs containing GB:L-HPC at 65:35 w/w and 75:25 w/w in the barrier layer in four beagle dogs, in either the fed or fasted state. The in-vivo lag time in both the fed and fasted states did not differ significantly (p>0.05) from the in-vitro lag time. Additionally, no significant difference (p<0.05) between in-vivo fed and fasted disintegration times was observed, demonstrating that in-vivo performance of the PCT was not influenced by the presence or absence of food in the gastrointestinal tract. A distinct lag time was obtained prior to the appearance of drug in plasma and correlated (R2=0.98) with disintegration time observed from scintigraphic images. However, following disintegration, no difference in pharmacokinetic parameters (AUC(0-6 dis), K(el), Cmax) was observed. The current study highlighted the potential use of these formulations for chronopharmaceutical drug delivery. 相似文献
33.
Summary Dozens of novel cysteine proteinases have been identified in positive single-stranded RNA viruses and, for the first time, in large double-stranded DNA viruses. The majority of these proteins are distantly related to papain or chymotrypsin and may be direct descendants of primordial proteolytic enzymes. Virus genome synthesis and expression, virion formation, virion entry into the host cell, as well as cellular architecture and functioning can be under the control of viral cysteine proteinases during infection. RNA virus proteinases mediate their liberation from giant multidomain precursors in which they tend to occupy conserved positions. These proteinases possess a narrow substrate specificity, can cleave in cis and in trans, and may also have additional, nonproteolytic functions. The mechanisms of catalysis, substrate recognition and RNA binding were highlighted by the recent analysis of the three-dimensional structure of the chymotrypsin-like cysteine proteinases of two RNA viruses. 相似文献
34.
Fix Joseph A. Alexander Jose Cortese Margot Engle Karen Leppert Paula Repta A. J. 《Pharmaceutical research》1990,7(4):384-387
Short-chain alkyl esters of L-dopa were administered to rats and mice via oral and rectal routes. Plasma L-dopa esters and L-dopa were determined in the systemic and portal circulation by HPLC. A comparison of isopropyl, butyl, and 4-hydroxybutyl esters of L-dopa demonstrated significantly higher levels of the esters in both systemic and portal blood samples following rectal administration than following oral administration. In most cases, oral administration resulted in nondetectable (<0.01 µg/ml) levels of the esters in plasma. Correspondingly, the plasma levels of L-dopa itself were consistently higher following rectal administration. At very high oral doses (500 mg L-dopa equivalents/kg body weight), systemic plasma levels of the butyl ester could be detected (1.25 µg/ml at 10 min), which might indicate saturation of the esterase activity of the small intestine. These studies indicate that the systemic availability of L-dopa from short-chain alkyl esters of L-dopa may be best optimized by rectal administration, which avoids the relatively high esterase activity characteristic of the small intestine. 相似文献
35.
36.
Claus Jacob Alexander Y. Safronov Sonia Wilson H.Allen O. Hill Tim F. Booth 《Journal of electroanalytical chemistry (Lausanne, Switzerland)》1997,431(1):7-10
A novel chiral redox-active ferrocene compound (FcVI) with amphiphilic properties has been synthesized. Cryogenic Transmission Electron Microscopy (cryo-TEM) has been used to estimate the shape and size of the FcVI aggregates in solution. Uni- and multi-lamellar vesicles (between 40 and 300 nm in diameter) were observed in water. Large particles (of more than 1 μm in diameter) with a hexagonal fine structure were found in 50 mM aqueous Na2SO4 solution. Sonication transformed the latter into ‘rosette’-like structures. Cyclic voltammetry has been employed to investigate the electrochemical properties of FcVI. The amphiphile adsorbed on graphite electrodes and a reversible electrochemical behaviour, characteristic of ferrocene, was observed with redox potentials between 330 and 350 mV. 相似文献
37.
A. Robert Spitzer Shalom Stahl David Yarnitsky Ernest W. Johnson John R. Wilson R. A. C. Hughes Stefania Morino Giovanni Antonini Kiyotoshi Kaneko Yoji Ohnishi Tetsushi Atsumi Isao Hozumi Tadashi Miyatake Tetsuo Furukawa James P. Knochel Ikuo Mineo Seiichiro Tarui Francis O. Walker Andrew J. Gitter Walter C. Stolov Nicholas J. Capozzoli 《Muscle & nerve》1996,19(4):531-538
38.
Armin W. Geiger M.D. F.I.C.A. Alexander M. Zarubin Ph.D. Marc Hertel Anke Fahrenkamp M.D. Gert von Bally Hans H. Scheld M.D. F.I.C.A. 《The International journal of angiology》1995,4(1):46-50
Dysfunction of heart valve prostheses—mechanical as well as biological—is a common problem in cardiac surgery. The reasons for the valve failures are still not well understood. Biological valves especially have an unsatisfactory durability; degeneration and calcification very often lead to the failure of the valves. In our opinion, hidden defects present in the valve material prior to implantation of the valves is a plausible explanation for the dysfunction. Hitherto there has been no technique to detect these defects without destructing the specimen. Holographic interferometry proved to be applicable forin vitro evaluation of mechanical heart valve prostheses. In the present paper we describe application of this method to biological valves. Nine porcine bioprostheses and four fresh porcine aortic valves were investigated by means of holographic interferometry. In eight of nine bioprostheses, the results showed irregularities of the leaflet structure which depend on anomalies of the connective tissue of the leaflets of the valves. To make sure that these findings are not due to normal variations of the morphology, the investigations were carried out with fresh and unfixated porcine aortic valves. In the latter, no such anomalies of the structure were detected. The results obtained confirm the above hypothesis on the origin of the later valve dysfunction. Thus, holographic interferometry tests of bioprostheses prior to their implantation prevent the use of potentially dysfunctional valves.Presented at the 35th World Congress, International College of Angiology, Copenhagen, Denmark, July 1993 相似文献
39.
Familial Sneddon's syndrome 总被引:4,自引:0,他引:4
Alexander Lossos Tamir Ben-Hur Ziva Ben-Nariah Claes Enk Moshe Gomori Dov Soffer 《Journal of neurology》1995,242(3):164-168
We report the familial occurrence and apparent autosomal dominant inheritance of Sneddon's syndrome with variable clinical expression. The proband, a 40-year-old woman, presented with livedo reticularis and progressive neurological deterioration following a stroke. The diagnosis was confirmed by cerebral angiogram and skin biopsy, both showing the characteristic findings. Two of the patient's sisters were reported to have been similarly affected in the past. Her mother, two additional siblings and five of her seven children exhibited various vasospastic skin phenomena. Familial aggregation of this disorder may be common and a genetic basis may be involved in its pathogenesis. 相似文献
40.
In his autobiography, Dr. Wilder Penfield relates the medical history of his sister, Mrs. Ruth Inglis, who developed a right frontal oligodendroglioma. Penfield performed the initial craniotomy on her, and after her death, reported the case in a paper on frontal lobe function in humans. Although Penfield has provided more than adequate information on certain aspects of his sister's illness, little has been published concerning her subsequent operation performed by Dr. Harvey Cushing at the Peter Bent Brigham Hospital. We feel this case is significant for several reasons. First, it sheds some light on Penfield's personal relationship with Cushing and his sister; it also shows the deep interest that Dr. Cushing took in his patients. Second, this case nicely illustrates how Cushing used his postoperative drawings to make his operative notes more precise. Finally, we see a sample of the work done by Dr. Louise Eisenhardt as pathologist at the Peter Bent Brigham Hospital. 相似文献