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1.
We report on the autopsy study of a premature boy with multiple joint contractures who died soon after birth of severe lung hypoplasia. Muscle histology showed PAS-positive vacuoles, and electronmicroscopy revealed massive subsarcolemmal and intermyofibrillar accumulation of glycogen. Biochemical analysis of fresh-frozen muscle tissue disclosed increased glycogen content and a complete lack of phosphofructokinase (PFK) activity. The brain showed focal cerebral and diffuse cerebellar white matter gliosis, and patchy loss of internal granular and Purkinje cells in the cerebellar cortex. The spinal cord was normal. This report describes the first case of PFK deficiency, presenting as a lethal fetal akinesia sequence.  相似文献   

2.
In radioimmunotherapy, the long circulation times of antibody radioconjugates correlate with high relative radiation doses to nontumor tissues. Tumor/normal tissue ratios can be significantly improved by using targeting molecules with shorter circulation times. IFabs are multimers of VH-CH1-linker-VK-CK monomers. The lack of the Fc region in IFabs should lead to circulation times that are shorter than those of IgG molecules. The monomers assemble into disulfide-bond-stabilized multimers, 90% of which are 100 kDa dimers (IFab2). IFab2s should not be rapidly eliminated through kidney filtration because their molecular weight is above the threshold for renal passage. We report the first experimental in vivo tests for 125I-IFab radioconjugates derived from a humanized version of the anti-breast mucin monoclonal antibody BrE-3. Biodistributions are reported for athymic nude mice carrying human mammary tumor MX-1 xenografts. The T1/2 beta's for the different tissues ranged from 13.3 h for blood to 19.9 h for tumor. Therefore, IFab radioconjugates cleared the body with a rate comparable to that of F(ab')2 fragments. Except for stomach, tumor/nontumor dose ratios were significantly better for IFabs than for the parent antibody (BrE-3)4 days after injection.  相似文献   

3.
The vertebrate Hox genes have been shown to confer regional identity along the anteroposterior axis of the developing embryo, especially within the central nervous system (CNS) and the paraxial mesoderm. The notochord has been shown to play vital roles in patterning adjacent tissues along both the dorsoventral and mediolateral axes. However, the notochord's role in imparting anteroposterior information to adjacent structures is less well understood, especially as the notochord shows no morphological distinctions along the anteroposterior axis and is not generally described as a segmental or compartmentalized structure. Here we report that four zebrafish hox genes: hoxb1, hoxb5, hoxc6 and hoxc8 are regionally expressed along the anteroposterior extent of the developing notochord. Notochord expression for each gene is transient, but maintains a definite, gene-specific anterior limit throughout its duration. The hox gene expression in the zebrafish notochord is spatially colinear with those genes lying most 3' in the hox clusters having the most anterior limits. The expression patterns of these hox cluster genes in the zebrafish are the most direct molecular evidence for a system of anteroposterior regionalization of the notochord in any vertebrate studied to date.  相似文献   

4.
Meningiomas were studied in 60 patients retrospectively. Clinical, operative and histological findings were correlated with the occurrence and extension of peritumoural oedema as measured by computerized tomography. A relationship was found between both oedema and seizures and between oedema and tumour location. No relationship between tumour size, arachnoid breaching, WHO-grade or tumour vascularity and oedema was detected. In four patients with severe pre-operative oedema, cerebral signs and symptoms persisted despite uncomplicated tumour removal. The present study shows that peritumoural oedema is not only epileptogenic but that it can also cause irreversible cerebral damage as well. Since this study purports to demonstrate that meningiomas with intact leptomeninges can show severe peritumoural oedema, the blood barrier breakdown theory cannot be considered as the only aetiological factor.  相似文献   

5.
The aim of the present study was to evaluate critically the protection afforded by hypothermia against ischemic injury to the hippocampus. Hypothermic treatment was applied selectively to the brain during a 5 min carotid artery occlusion in gerbils. Following a period of recovery, two independent measures were used to assess hippocampal function: (1) an open field test of spatial memory (assessment was made during the first 10 d after ischemia) and (2) measurement of evoked potentials from area CA1 in hippocampal slices (3 weeks after the ischemic episode). The functional outcome portrayed by these tests was compared to a morphological evaluation of CA1 pyramidal cells at three rostrocaudal levels. All evaluations were carried out in the same animals. We found converging evidence that intraischemic hypothermia provides virtually complete protection against a 5 min episode of cerebral ischemia. Animals treated with hypothermia performed as well as sham-operated controls in a spatial memory task, had field potentials that were indistinguishable from normal animals and CA1 cells appeared normal when assessed histologically. In contrast, ischemia at normothermia resulted in a deficit in open field behavior (p < 0.01), diminished field potentials in stratum radiatum (p < 0.01), and near total loss of pyramidal cells in dorsal CA1 (p < 0.01). There was a remarkably high correlation between these diverse measures (r ranged from 0.7 to 0.9, p < 0.01), which provides strong support for the use of hypothermia as an effective treatment for ischemia. This study introduces a novel approach for the evaluation of putative anti-ischemic treatments: combining behavioral, electrophysiological, and histological measures.(ABSTRACT TRUNCATED AT 250 WORDS)  相似文献   

6.
The dental health of 4-year-old children in the city of Ume?, northern Sweden, has been followed in cross-sectional studies conducted in 1967, 1971, 1976, 1980, 1987 and 1992. Similar methods and criteria were used. Dental caries and background factors (fluoride usage, toothbrushing frequency and diet) were recorded. In 1971, 1987 and 1992 signs of tooth trauma and presence of malocclusion were also recorded, the latter also in 1976. The present paper summarizes the results of these studies. Between 1967 and 1992 the mean dmfs values declined from 7-8 to 1-8. The decline was greatest between 1967 and 1980 and then levelled off. Toothbrushing frequency was similar over the years, but parental help with brushing was more common in the more recent studies. The use of fluoride toothpaste increased. About 30% of the children had experienced tooth trauma. A unilateral crossbite was recorded in 18% and 16% of the children in 1971 and 1992, respectively, and an anterior open bite in about 35% and 41%, respectively; both types of malocclusion were related to a dummy- or finger-sucking habit.  相似文献   

7.
The study presents the methodology and selected results of the Lazio Dialysis Registry. The registry, begun in 1994, documents basic demographic data, minimal clinical information and vital status of all end-stage renal disease patients on dialysis treatment in Lazio region. At June 1994 the register included 2834 patients; during the next 12 months, 618 new patients had been notified. In one-year follow-up, among prevalent patients, 308 deaths and 90 renal transplants, and among new patients, 80 deaths and 9 renal transplants were observed. The mean age of the studied population was 59.6 (SD 15.1) for prevalent patients and 61.9 (SD 15.3) for new patients. Among prevalent patients 58.8% were males and 41.2% females, among new patients these figure were 62.1% and 37.9%, respectively. The prevalence rate was 55 per thousand population and the incidence rate was 12.0. The leading cause of renal failure was glomerulonephritis in prevalent patients (27.7%), among new patients the most representative group was nephropathies of unknown origin (27.0%). About 85% were on hemodialysis treatment, and about 4% on peritoneal dialysis. The cumulative survival rates during the one-year follow-up was 81.9%, among patients with age less than 65 years a better survival for males (94.9%) than females (90.1%) was observed. The results of the first year of activity show that a population-based registry of patients on chronic dialysis treatment can be an useful tool for service planning and epidemiological research.  相似文献   

8.
Caretaker recognition of clinical utility of respiratory signs and symptoms in the prediction of pneumonia was examined in a prospective study of infants and children in four cities in Egypt. In all 688 children aged 2 months-5 years presenting with a history and/or physical examination findings of cough and difficult or fast breathing were recruited from out-patient health facilities. The validity of caretaker terms was determined using paediatrician observation of standard respiratory signs and symptoms, x-ray diagnosis and pulse oximetry as standards. The sensitivity of 'nahagan' (Egyptian Arabic for fast breathing) for identifying elevated respiratory rate was 78% +/- 4, and was slightly higher for < 12 month olds (85% +/- 5) versus children aged 1-5 years (74% +/- 5). 'Sedro tale nazel', which describes the chest as moving up and down, was a sensitive (86% +/- 3) and specific (60% +/- 4) indicator of chest indrawing. 'Tazyeek' (wheeze) had a sensitivity of 75% +/- 3 and specificity of 66% +/- 4 when compared to paediatrician assessment of wheezing during physical examination. Although not specific, the caretaker terms, 'nahagan' or 'nafas seria' (fast breathing) and 'sedro tale nazel' (chest indrawing), either spontaneously or after asking, were sensitive (> 71%) indicators of radiologic pneumonia and oxygen desaturation, and therefore can be used to prompt timely health seeking behaviour in these settings.  相似文献   

9.
In cultures of dissociated rat hippocampal neurons, persistent potentiation and depression of glutamatergic synapses were induced by correlated spiking of presynaptic and postsynaptic neurons. The relative timing between the presynaptic and postsynaptic spiking determined the direction and the extent of synaptic changes. Repetitive postsynaptic spiking within a time window of 20 msec after presynaptic activation resulted in long-term potentiation (LTP), whereas postsynaptic spiking within a window of 20 msec before the repetitive presynaptic activation led to long-term depression (LTD). Significant LTP occurred only at synapses with relatively low initial strength, whereas the extent of LTD did not show obvious dependence on the initial synaptic strength. Both LTP and LTD depended on the activation of NMDA receptors and were absent in cases in which the postsynaptic neurons were GABAergic in nature. Blockade of L-type calcium channels with nimodipine abolished the induction of LTD and reduced the extent of LTP. These results underscore the importance of precise spike timing, synaptic strength, and postsynaptic cell type in the activity-induced modification of central synapses and suggest that Hebb's rule may need to incorporate a quantitative consideration of spike timing that reflects the narrow and asymmetric window for the induction of synaptic modification.  相似文献   

10.
In a study with 146 male Sprague-Dawley rats, 3 doses each of cathodal or anodal direct current were delivered bilaterally to coagulate lateral hypothalamic tissue. Increases in hypothalamic tissue damage were associated with more instances of aphagia, greater amounts of glandular, but not rumenal, gastric pathology (GP), and greater weight loss. Both anodal and cathodal lesions produced aphagia and similar amounts of GP, but anodal lesions also facilitated weight loss independently of tissue damage. It is suggested that extensive chromatolysis surrounding anodal lesion cavities may be related to the postoperative effects. When anodal electrolytic lesions or cortical suction ablations were used to vary the location of neural damage, eating of dry food in the presence of high GP was observed in Ss with lesions ventral and medial to an area in the dorsolateral hypothalamus that was associated with aphagia. When aphagia was accompanied by severe GP, the brain lesions typically encroached extensively on more ventromedial areas. Moreover, aphagia sometimes was observed with only negligible GP. It is suggested that GP is not primary to the expression of the aphagia that follows lateral hypothalamic damage. (44 ref) (PsycINFO Database Record (c) 2010 APA, all rights reserved)  相似文献   

11.
In this study, 96 women (mean +/- SD, 65.3 +/- 13.2 years) were investigated by bone mineral densitometry (DXA, Hologic QDR 2000) and quantitative ultrasound (QUS) of the tibia (n = 96; Sound-Scan2000, Myriad) and phalanges (n = 84: DBM Sonic 1200, IGEA). We observed a good correlation of QUS measurement with bone mineral content (BMD) on lumbar spine and both hips (Ward and Neck). Correlation of the two QUS-techniques measuring at different skeletal sites within the same patient was good. QUS instruments measuring at the various skeletal sites and their suitability for screening patients at risk of osteoporosis are discussed. The usefulness of the different QUS methods and the best measuring site for the assessment of fracture risk in osteoporotic patients still have to be established.  相似文献   

12.
Early (pre-seroconversion) infection with human immunodeficiency virus type 1 (HIV-1) was identified in 50 of 267 participants in the Multicenter AIDS Cohort Study. These 50 men had a positive EIA result, which detected IgM antibody (n = 35), p24 antigen, or serum HIV RNA (n = 15) at their last "seronegative" visit. At that visit, the mean CD4 lymphocyte number (890/mm3 vs. 1038/mm3) was significantly lower than in men who subsequently seroconverted but had no evidence of early infection. The decline in CD4 cells was slower and the duration of AIDS-free time longer in the 19 men who were symptomatic in comparison to the 31 asymptomatic men with early infection, but differences were not significant.  相似文献   

13.
This study measured validity and reproducibility of unaided clinical diagnosis, fibre-optic transillumination (FOTI), and bite-wing radiology in the diagnosis of approximal caries. Sixty models were made using extracted premolars and molars, each containing four teeth with six contacting approximal surfaces. The teeth were examined first using unaided clinical examination and then using FOTI. Bite-wing radiographs were then taken of the teeth set in the models and examined. The three examinations were repeated after 1 week. Histological sections of the undecalcified teeth were prepared following their removal from the models, and those showing signs of caries were examined to give the valid state of disease in each surface. The diagnostic threshold was caries penetrating into dentine. The reproducibility of all three methods was acceptable with kappa values exceeding 0.6. All specificity values exceeded 0.95. Statistically significant differences were seen between sensitivities for clinical (0.38) and bite-wing (0.59) diagnosis and between clinical and FOTI (0.67) examination, but not between bite-wing and FOTI. It is concluded that the validity of FOTI is at least as high as that of bite-wing radiology, and both are superior to unaided clinical diagnosis.  相似文献   

14.
BACKGROUND: Myocardial ischemia increases tissue electrical resistivity leading to cell-to-cell uncoupling, and this effect is delayed by ischemic preconditioning in isolated myocardium. Alterations in myocardial resistivity elicited by ischemia in vivo may influence arrhythmogenesis and local ST-segment changes, but this is not well known. METHODS AND RESULTS: Myocardial impedance (resistivity [omega x cm] and phase angle [degrees]), epicardial ST segment, and ventricular arrhythmias were analyzed during 4 hours of coronary artery occlusion in 11 anesthetized open-chest pigs; these were compared with 13 other pigs submitted to a similar coronary occlusion preceded by ischemic preconditioning. Myocardial resistivity rose slowly during the first 34+/-7 minutes of occlusion (237+/-41 to 359+/-59 omega x cm), increased rapidly to 488+/-100 omega x cm at 60 minutes, and reached a plateau value (718+/-266 omega x cm, ANOVA; P<.01) at 150+/-69 minutes. By contrast, phase-angle changes began after 17 minutes of ischemia (-3.0+/-1.6 degrees to -4.2+/-1.2 degrees at 29+/-8 minutes) and evolved faster thereafter (-12.5+/-5.3 degrees at 144+/-56 minutes). Marked changes in myocardial impedance were observed during the reversion of ST-segment elevation that occurred 1 to 4 hours after occlusion, but impedance changes were less apparent during the early ST-segment recovery seen at 15 to 35 minutes of ischemia. The second arrhythmia peak (30+/-5 minutes) coincided with the fast change in tissue impedance, and both were delayed (P<.05) by ischemic preconditioning. CONCLUSIONS: A rapid impairment of myocardial impedance occurs after 30 minutes of coronary occlusion, and its onset is better defined by shift in phase angle than by rise in tissue resistivity. Phase 1b arrhythmias are associated with marked impedance changes, and both are delayed by preconditioning. Reversion of ST-segment elevation is partially associated with impairment of myocardial impedance, but other factors play a role as well.  相似文献   

15.
OBJECTIVE: To investigate p53 and c-jun oncoproteins and proliferating cell nuclear antigen (PCNA) in transitional cell urinary bladder carcinomas (TCCs) and to determine their relationships to tumour grade, stage and survival. MATERIALS AND METHODS: The expression of p53, c-jun and PCNA was studied using immunohistochemistry in formalin-fixed, paraffin-embedded tissues in a series of 110 TCCs. RESULTS: 58% of our cases were positive for p53 and 88% for c-jun. A statistically very significant correlation (p < 0.0001) was observed between p53 and c-jun (r = 0.781), p53 and PCNA (r = 0.772), c-jun and PCNA (r = 0.831) as well as between each of the two oncoproteins and the histological grade and clinical stage (p < 0.001). There was no correlation of either p53, PCNA or c-jun with clinical outcome in terms of patients survival. CONCLUSION: p53 and c-jun proteins' overexpression are strongly related to rapid tumour cell proliferation and hence with aggressive growth in urinary bladder TCC. PCNA score remains an important prognostic index in transitional cell carcinoma of the bladder.  相似文献   

16.
The combination of ultrasound contrast media (US-CM) and color-coded duplex sonography (CCDS) is a very promising new diagnostic method. The principles of CCDS and US-CM are therefore briefly discussed. Different US-CM are described regarding their application and possible indications. This is followed by a summary of the first clinical experience with this new method.  相似文献   

17.
BST-1, a bone marrow stromal cell surface antigen, is a glycosyl phosphotidylinositol-anchored protein that stimulates pre-B-cell growth and has adenosine diphosphate (ADP)-ribosyl cyclase and cyclic ADP-ribose (cADPR) hydrolase activity. The two enzymatic activities are responsible for the synthesis and hydrolysis of cADPR, a novel second messenger of calcium release from intracellular calcium stores. The expression and characterization of human BST-1 in certain mammalian cell lines have been reported. We have expressed the murine BST-1 in yeast as a 6 x His-tagged secreted protein. The recombinant protein has been purified and subjected to structural and functional characterization. It has an apparent molecular mass of 38.5 kDa on SDS-PAGE gel stained with Coomassie blue and is recognized on Western blots by a rabbit polyclonal antibody against BST-1. Deglycosylation of the protein with N-glycanase produces a ladder of bands with molecular sizes ranging from 32 to 39 kDa. The protein possesses the ADP-ribosyl cyclase activity as measured using nicotinamide guanine dinucleotide as substrate.  相似文献   

18.
Computed tomography (CT), percutaneous transhepatic cholangiography (PTC) and magnetic resonance imaging (MRI) findings in a case of sclerosing cholangitis associated with Crohn's disease of the colon and terminal ileum are described. CT gives additional information on dilatation of peripheral bile ducts and confirms findings of PTC, i.e. a decreased arborization of the biliary tree, a nodular appearance of the common bile duct and multifocal bile duct strictures. CT findings could be recognized on MRI which provided no additional information.  相似文献   

19.
Joint contractures have been one of the contraindications for use of functional electrical stimulation for standing in paraplegic patients. A simulation study using a three-segment link mechanical model of the human body was performed to calculate the muscle moments at the ankles, knees, and hips during standing with and without having joint contractures. The knee and hip angles were varied in 5 degrees increments, whereas the ankle angles were varied in 1 degree increments. It was assumed that energy efficient posture was obtained with the least sum of the squared moments of the ankles, knees, and hips joints by the muscles. Ankles at 5 degrees of dorsiflexion, knees at 0 degrees, and hips at 15 degrees of extension resulted in the most energy efficient posture without joint contractures. The muscle moments increased with the increase in angle of contractures. The joint contractures at ankle angles > or = 6 degrees of plantar flexion, knee angles > or = 20 degrees of flexion, and/or hip angles > or = 20 degrees of flexion produce a potentially unstable posture. These findings suggest that some degree of joint contractures can be tolerated in paraplegic patients using functional electrical stimulation for standing.  相似文献   

20.
The structure of a mammalian class IV alcohol dehydrogenase has been determined by peptide analysis of the protein isolated from rat stomach. The structure indicates that the enzyme constitutes a separate alcohol dehydrogenase class, in agreement with the distinct enzymatic properties; the class IV enzyme is somewhat closer to class I (the "classical" liver alcohol dehydrogenase; approximately 68% residue identities) than to the other classes (II, III, and V; approximately 60% residue identities), suggesting that class IV might have originated through duplication of an early vertebrate class I gene. The activity of the class IV protein toward ethanol is even higher than that of the classical liver enzyme. Both Km and kcat values are high, the latter being the highest of any class characterized so far. Structurally, these properties are correlated with replacements at the active site, affecting both substrate and coenzyme binding. In particular, Ala-294 (instead of valine) results in increased space in the middle section of the substrate cleft, Gly-47 (instead of a basic residue) results in decreased charge interactions with the coenzyme pyrophosphate, and Tyr-363 (instead of a basic residue) may also affect coenzyme binding. In combination, these exchanges are compatible with a promotion of the off dissociation and an increased turnover rate. In contrast, residues at the inner part of the substrate cleft are bulky, accounting for low activity toward secondary alcohols and cyclohexanol. Exchanges at positions 259-261 involve minor shifts in glycine residues at a reverse turn in the coenzyme-binding fold. Clearly, class IV is distinct in structure, ethanol turnover, stomach expression, and possible emergence from class I.  相似文献   

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