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101.
102.
Excitatory amino acid neurotoxicity has been proposed to cause the neostriatal neuronal degeneration of Huntington's disease (HD); N-methyl-D-aspartate (NMDA), alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid (AMPA), and kainate receptors have been hypothesized to play important roles in this process. We have recently reported a loss of neurons in layer VI of the cerebral cortex in HD. Using quantitative autoradiographic methods, we have now measured NMDA, AMPA, and kainate receptor binding in the frontal cerebral cortex of the brains of controls and individuals with HD. We find no change in NMDA receptor binding but a selective decrease in kainate and AMPA receptor binding in layer VI. These data suggest that cerebral cortical neurons possessing kainate or AMPA receptors may be selectively vulnerable in individuals with HD.  相似文献   
103.
A 64 year old woman presented with persistent and severe symptoms due to recurrent aspiration pneumonias associated with oesophageal reflux. She had had multiple miscarriages and her son at birth had widely spaced eyes (hypertelorism), hypospadias, bilateral undescended testes, and an imperforate anus. Her daughter has mild hypertelorism and her daughter's son had neonatal inspiratory stridor, hypospadias and hypertelorism, all features now recognised as typical of the Opitz oculo-genito-laryngeal syndrome. This syndrome is genetically heterogeneous with autosomal dominant (linked to chromosome 22q21) and X-linked (linked to Xp22) inheritance. This family's history and genetic linkage data are consistent with linkage to Xp22. The proband is a manifesting carrier of this syndrome; her history of recurrent aspiration is probably secondary to pharyngeal neuromuscular incoordination aggravating gastro-oesophageal reflux. Obtaining a family history gives a vital clue to the diagnosis of Opitz oculo-genito-laryngeal syndrome. It is also suggested that this condition should be included in the differential diagnosis of recurrent aspiration pneumonia.  相似文献   
104.
The Transforming Growth Factor-beta superfamily member decapentaplegic (dpp) acts as an extracellular morphogen to pattern the embryonic ectoderm of the Drosophila embryo. To identify components of the dpp signaling pathway, we screened for mutations that act as dominant maternal enhancers of a weak allele of the dpp target gene zerkn?llt. In this screen, we recovered new alleles of the Mothers against dpp (Mad) and Medea genes. Phenotypic analysis of the new Medea mutations indicates that Medea, like Mad, is required for both embryonic and imaginal disc patterning. Genetic analysis suggests that Medea may have two independently mutable functions in patterning the embryonic ectoderm. Complete elimination of maternal and zygotic Medea activity in the early embryo results in a ventralized phenotype identical to that of null dpp mutants, indicating that Medea is required for all dpp-dependent signaling in embryonic dorsal-ventral patterning. Injection of mRNAs encoding DPP or a constitutively activated form of the DPP receptor, Thick veins, into embryos lacking all Medea activity failed to induce formation of any dorsal cell fates, demonstrating that Medea acts downstream of the thick veins receptor. We cloned Medea and found that it encodes a protein with striking sequence similarity to human SMAD4. Moreover, injection of human SMAD4 mRNA into embryos lacking all Medea activity conferred phenotypic rescue of the dorsal-ventral pattern, demonstrating conservation of function between the two gene products.  相似文献   
105.
A method for the simultaneous determination of residues of 17 beta-trenbolone and 17 beta, 19-nortestosterone and their epimers in animal tissues is described, involving immunoaffinity chromatography clean-up and high-performance liquid chromatography with dual-wavelength UV detection. The method has been validated at 2 micrograms/kg in pig and cattle liver and corned beef with recoveries of 41% upwards. The method has been applied to the determination of incurred residues of 19-nortestosterone and trenbolone. Various alternative extraction steps for incurred trenbolone have been investigated, including direct extraction, protease digestion, heating and ultrasonic probe treatment. Glucuronidase digestion has been shown to be the most effective method for this analyte.  相似文献   
106.
Kartagener's syndrome (KS) usually includes mirror-image dextrocardia. The incidence of congenital heart disease in KS is comparable with that in the general population. This paper reports on a case of Kartagener's syndrome associated with dextrocardia, corrected transposition of the great arteries (I,D,D), ventricular septal defect, and valvar pulmonary stenosis in an 8-year-old girl.  相似文献   
107.
BACKGROUND: Whether intraoperative laparoscopic cholangiography should be routine is debatable. METHODS: We reviewed the cholangiography experience in 669 consecutive laparoscopic cholecystectomies. RESULTS: Mean age of the patients was 39 years, 78% were female, and 29% had acute cholecystitis. Cholecystectomy was completed laparoscopically in 606 (91%). Laparoscopic cholangiography was completed in 562 (93%) and 348 (62%) were routine (no preoperative indication). The mean operating time in 1996 was 61 minutes. Out of the 348 routine cholangiograms, 17 demonstrated evidence of unsuspected choledocholithiasis. Five patients had choledocholithiasis documented by laparoscopic common bile duct exploration and/or endoscopic retrograde cholangiopancreatography. Two patients had normal postoperative cholangiopancreatography. One of 10 patients managed expectantly was readmitted postoperatively with obstructive jaundice. In 4 patients, routine cholangiography revealed unexpected anatomy, and in 2, this prevented misidentification and transection of the common bile duct. CONCLUSION: Laparoscopic cholangiography is safe, quick, detects unsuspected choledocholithiasis, and can prevent common bile duct transection. It should be routine.  相似文献   
108.
109.
Despite advances in anesthetic technique and technology, intraoperative awareness continues to occur with alarming regularity. The psychological effects on patients and the medicolegal consequences to providers have created the need for a reliable monitor of the hypnotic component of anesthesia. A new technology known as bispectral analysis (BIS) has the potential to put an end to the devastating occurrence of intraoperative awareness, as well as improve the overall effectiveness of anesthetic delivery and the surgical experience. Applicable to almost any situation requiring sedation or general anesthesia, BIS's reliability is a real-time measure of hypnosis levels.  相似文献   
110.
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