[Magnetic resonance urography in children].
Explore the source record for details and available documents.
Adolescent↗
Biomedical subjects
P Vera
Publications and source records attributed to P Vera.
[Diagnosis and treatment of soft-tissue tumors].
The diagnostic and therapeutic management of patients with soft-tissue tumors would be similar to the approach used for bone tumors if it were not for one crucial factor: the absolute necessity to recognize a sarcoma. The predominant features are the size of the tumor and its superficial or deep localization. If the tumor is small and superficial, biopsy can be associated with immediate resection without risk of dissemination to the deep tissues: this is the biopsy-resection approach. If the tumor is deep or superficial but large sized, search for locoregional spread with MRI is necessary before undertaking any surgical procedure. MRI can help guide the biopsy and plan resection if the tumor is a sarcoma. A first biopsy is necessary to establish the histological diagnosis and elaborate the therapeutic strategy. Samples should be sent immediately to the pathology lab which should examine sterile fresh tissue. Experience has demonstrated that proper rules for diagnosis and treatment are not necessarily applied initially in approximately one-fourth of all subjects with a malignant soft-tissue tumor. Besides the medical problems caused by this situation, the patient loses a chance for cure. When the tumor is a sarcoma, surgery is the basis of treatment. Complementary radiation therapy may be necessary, particularly for high-grade tumors or if the surgical margin was insufficient. Systemic or locoregional chemotherapy can also be used for high-grade or non-resectable tumors.
Biopsy↗
Frontotemporal dementia, motor neuron disease and tauopathy: clinical and neuropathological study in a family.
We report a familial disorder occurring in three patients that presented as frontotemporal dementia (FTD). A neuropathological study was performed in a 58-year-old patient, who developed FTD 2 years prior to the onset of motor neuron disease (MND), and died at age 62. Lesions indicative of associated MND were observed: neuronal loss in the anterior horns of the spinal cord, Bunina bodies, axonal spheroids, degeneration of the pyramidal tracts, and of FTD: decreased neuronal density and laminar microvacuolation of layers II and III in the frontal and temporal cortex. Ubiquitin-only-immunoreactive changes were found in the spinal cord and medulla, but were absent from the temporal and frontal cortex. There were also widespread deposits of various neuronal and glial inclusions containing abnormally phosphorylated tau protein, the Western blotting pattern of which was characterized by two major bands of 64 and 69 kDa. There were no abnormalities of the entire coding sequences of microtubule-associated protein tau (MAPT) and copper-zinc superoxide dismutase (SOD(1)) genes. Our results suggest that FTD associated with MND can be caused by a larger spectrum of neuropathological lesions than commonly accepted.
Blotting, Western↗
Ultrasonic flaw detection using radial basis function networks (RBFNs).
Ultrasonic flaw detection has been studied many times in the literature. Schemes based on thresholding after a previous matched filter use to be the best solution, but results obtained with this method are only satisfactory when scattering and attenuation are not considered. In this paper, we propose an alternative solution to thresholding detection method. We deal with the usage of different flaw detection methods comparing them with the proposed one. The experiment tries to determinate whether a given ultrasonic signal contains a flaw echo or not. Starting with a set of 24,000 patterns with 750 samples each one, two subsets are defined for the experiments. The first one, the training set, is used to obtain the detection parameters of the different methods, and the second one is used to test the performance of them. The proposed method is based on radial basis functions networks, one of the most powerful neural network techniques. This signal processing technique tries to find the optimal decision criterion. Comparing this method with thresholding based ones, an improvement over 25-30% is obtained, depending on the probability of false alarm. So our new method is a good alternative to flaw detection problem.
Algorithms↗
Experimental vertical transmission of Piscirickettsia salmonis and in vitro study of attachment and mode of entrance into the fish ovum.
Piscirickettsia salmonis is a pathogenic bacterial agent causing septicaemic disease in salmon. Since its isolation in Chile in 1989, P. salmonis has continually produced high mortality rates in salmon farms. Little information exists regarding the mechanisms of vertical transmission of this pathogen. Experimental vertical transmission was established in the present study by inoculation of male and female rainbow trout broodstock with P. salmonis. The bacterium was subsequently detected using indirect immunofluorescence in milt and coelomic fluid of the majority of inoculated broodstock (14/15). Bacteria were detected in the fry when 1 or both parents were inoculated, although none of the infected fry presented signs of the disease. P. salmonis was also detected in progeny obtained through fertilisation ova from non-inoculated females incubated in a medium containing a bacterial suspension, demonstrating transmission during the process of fertilisation. Ova infected in vitro were examined at sample periods from 30 s to 60 min using scanning electron microscopy. This demonstrated that the bacterium attaches to the ova by means of membrane extensions, structures which we have called 'piscirickettsial attachment complex' (PAC) and which would allow later penetration into the ovum.
Animals↗
Ictal SPECT in children with epilepsy: comparison with intracranial EEG and relation to postsurgical outcome.
In order to validate the ability of ictal single photon emission computed tomography (SPECT) to localize the epileptogenic zone (EZ) in children, we compared in 20 patients aged from 10 months to 17 years (mean 6.5 years) the topography of the area of increased ictal perfusion (IPA), determined on the basis of ictal minus interictal scan values, with that of the EZ determined by intracranial EEG recordings and assessed its relationship with the postsurgical outcome. Eighteen patients had symptomatic epilepsy and 10 had extratemporal epilepsy. All patients except one had an ictal injection (mean time lag from clinical seizure onset was 18 s). Ictal and interictal SPECT images were successively co-registered, normalized, subtracted, smoothed and superimposed on MRI. All patients with ictal injection exhibited one or several IPAs. The topography of the 'highest' IPA, i.e. the maximal cerebral blood flow (CBF) change between ictal and interictal SPECT, significantly colocalized with the site of onset of the discharge, and that of the lower IPAs with that of the area of propagation (P < 0.0001). At a threshold of 30% of the maximal CBF change, the IPAs detected the onset of the discharge with a sensitivity of 0.80 and a specificity of 0.70. The highest IPA localized the EZ in 12 out of 15 patients. In the three others it missed the EZ and showed the area of propagation because of rapid seizure propagation or of infraclinical seizure onset. Among the patients with favourable surgery outcome, the highest IPA colocalized with the resected area in 70% of cases. Ictal SPECT could therefore plays an important role as a non-invasive presurgical method of investigation by optimizing the placement of intracranial electrodes, thus improving the postsurgery outcome of paediatric partial epilepsy.
Adolescent↗
Impact of Wiener filter in determining the left ventricular volume and ejection fraction using thallium-201 gated SPECT.
Patient morphology, as well as the acquisition and reconstruction parameters, may influence the evaluation of the left ventricular volume (LVV) and left ventricular ejection fraction (LVEF) using gated single-photon emission computed tomography (SPECT). The purpose of this study was to examine the influence of gender and reconstruction filter on the measurement of LVV and LVEF using 201Tl gated SPECT. Using a static torso phantom, a female shape was created by the addition of two saline solution-filled balloons fixed on the anterior rib cage. The following parameters were similar for all acquisitions: 90 degrees dual-head gamma camera; 32 projections; 64x64 matrix (pixel size=6.77x6.77 mm); two 20% energy windows centred at 70 and 167 keV. The following acquisition times were tested: 1.25, 10, 20, 30 and 40 s per projection, leading to a total of 10 successive acquisitions. The effect of over-sampling was tested by 2.5 post-acquisition zooming. All SPECT images were successively reconstructed using filtered back-projection with Butterworth and Wiener filters. The effect of gender and reconstruction filter was also studied in 30 patients (15 males and 15 females) with a low likelihood of coronary artery disease. LVVs were calculated using QGS software. By multivariate analysis, the following factors influenced the accuracy of phantom measurement using QGS software: zooming (F=49, P<0.0001), phantom shape (F=61, P<0.0001) and filter type (F=240, P<0.0001). LVV was underestimated in the female shape phantom, even when using the Wiener filter. In patients, LVV and LVEF measurements were independently influenced by gender (P<0.0001) and filter (P<0.0001), but not by zooming. In conclusion, it was demonstrated that LVV was significantly decreased in the female shape phantom, suggesting a significant impact of breast interposition. This underestimation was minimized by use of the Wiener filter. In patients, the impact of the Wiener filter on the assessment of LVVs and LVEF was powerful, but independent of gender, and failed to correct the underestimation of LVVs and the overestimation of LVEF in females.
Algorithms↗
Expression of a pathogen-induced gene can be mimicked by auxin insensitivity.
Following perception of a pathogenic attack, plants are able to develop a strong response with the corresponding activation of a plethora of defense-related genes. In this study we have characterized the mode of expression of the CEVI-1 gene from tomato plants, which encodes an anionic peroxidase. CEVI-1 expression is induced during the course of compatible viral and subviral infections, like many other defense-related genes, but is induced neither in incompatible interactions nor by signal molecules such as salicylic acid, ethylene, or methyl jasmonate. Additionally, CEVI-1 is induced in detached leaf tissues following a pathway distinct from that related to the classical wound response. We also describe the characterization of the structural CEVI-1 gene and compare the mode of expression in different transgenic plant species harboring a CEVI-1::GUS construct. Furthermore, we have isolated mutants in Arabidopsis, called dth mutants, that are deregulated in the control of expression of this gene. From the initial analysis of some of these mutants it seems that activation of CEVI-1 gene expression correlates with a defect in the perception of auxins by the plant. All these results may suggest that, during systemic infections with viruses, auxin homeostasis is one of the components participating in the regulation of the overall defense response.
Arabidopsis↗
Characterization of P69E and P69F, two differentially regulated genes encoding new members of the subtilisin-like proteinase family from tomato plants.
Subtilisin-like proteins represent an ancient family of serine proteases that are extremely widespread in living organisms. We report here the structure and genomic organization of two new transcriptionally active genes encoding proteins that belong to the P69 family of subtilisin-like proteases from tomato (Lycopersicon esculentum) plants. The two new members, P69E and P69F, are organized in a cluster and arranged in a tandem form. mRNA expression analysis and studies of transgenic Arabidopsis plants transformed with promoter-beta-glucuronidase fusions for each of these two genes revealed that they are differentially regulated, with each showing a highly specific mRNA expression pattern. P69E mRNA is expressed only in roots, while P69F mRNA is expressed only in hydathodes. A comparison of all the P69 amino acid sequences, gene structure, expression profiles, and clustered organization suggests a working model for P69 gene family evolution.
Amino Acid Sequence↗
Local and systemic induction of two defense-related subtilisin-like protease promoters in transgenic Arabidopsis plants. Luciferin induction of PR gene expression.
Following a pathogenic attack, plants are able to mount a defense response with the coordinated activation of a battery of defense-related genes. In this study we have characterized the mode of expression of the P69B and P69C genes from tomato (Lycopersicon esculentum Mill.), which encodes two closely related subtilisin-like proteases associated with the defense response. We have compared the mode of gene regulation in heterologous transgenic Arabidopsis plants harboring promoter-beta-glucuronidase (GUS) and promoter-luciferase (LUC) gene fusions for these two genes. These studies revealed that the P69B and P69C promoters are induced by salicylic acid as well as during the course of both a compatible and an incompatible interaction with Pseudomonas syringae. Furthermore, P69B and P69C expression takes place in both the local and the distal (noninoculated) leaves upon inoculation with bacteria but following different and unique tissue-specific patterns of expression that are also different to that described for most other classical PR genes. Also, we report that luciferin, the substrate for the reporter luciferase (LUC) gene, is able to activate expression of PR genes, and this may pose a problem when using this gene reporter system in studies related to plant defense.
Arabidopsis↗
Arabidopsis dth9 mutation identifies a gene involved in regulating disease susceptibility without affecting salicylic acid-dependent responses.
To determine which components of the plant defense response make important contributions to limiting pathogen attack, an M(2) mutagenized population of a transgenic Arabidopsis line was screened for mutants showing constitutive expression of beta-glucuronidase activity driven by the promoter region of the CEVI-1 gene. The CEVI-1 gene originally was isolated from tomato plants and has been shown to be induced in susceptible varieties of tomato plants by virus infection in a salicylic acid-independent manner. We report here the characterization of a recessive mutant, detachment9 (dth9). This mutant is more susceptible to both virulent and avirulent forms of the oomycete Peronospora and also exhibits increased susceptibility to the moderately virulent bacterial pathogen Pseudomonas syringae pv maculicola ES4326. However, this mutant is not affected in salicylic acid metabolism and shows normal expression of pathogenesis-related (PR) genes after pathogen attack. Furthermore, after inoculation with avirulent pathogens, the dth9 mutant shows a compromised systemic acquired resistance response that cannot be complemented by exogenous application of salicylic acid, although this molecule is able to promote normal activation of PR genes. Therefore, the dth9 mutation defines a regulator of disease susceptibility that operates upstream or independently of salicylic acid. Pleiotropy is also evident in the dth9 mutant in the sense that the shoots of dth9 plants are insensitive to the exogenously applied auxin analog 2,4-dichlorophenoxyacetic acid.
Arabidopsis↗
[Primary intraosseous rhabdomyosarcoma].
We report a case of primary intraosseous pleiomorphic rhabdomyosarcoma located in the pelvis of a 21-year-old woman followed for 4 years. The lytic tumor involved the acetabulum and the isthma with moderate extension to soft tissue. First line chemotherapy was unable to arrest tumor progression. Hemipelvectomy with saddle prosthesis reconstruction was performed, but septic complications dictated a secondary inter-ilio-abdominal amputation. Recurrence-free remission was achieved for 4 years, suggesting this was indeed a primary tumor. Primary intraosseous rhabdomyosarcomas are exceptional. Bone localizations generally suggest metastasis from a primary tumor often situated in an intraperitoneal localization. When search for a primary tumor is negative, intraosseous lesions can be considered as primary tumors warranting curative treatment. Radical surgical resection is recommended within the framework of a multidiscipinary management protocol associating radiotherapy and chemotherapy to improve prognosis.
Adult↗
A genomic cluster containing four differentially regulated subtilisin-like processing protease genes is in tomato plants.
Screening of a genomic library from tomato plants (Lycopersicon esculentum) with a cDNA probe encoding a subtilisin-like protease (PR-P69) that is induced at the transcriptional level following pathogen attack (Tornero, P., Conejero, V., and Vera, P. (1996) Proc. Natl. Acad. Sci. U. S. A. 93, 6332-6337) resulted in the isolation of a cluster of genomic clones that comprise a tandem of four different subtilisin-like protease genes (P69A, P69B, P69C, and P69D). Sequence analyses and comparison of the encoded proteins revealed that all are closely related (79 to 88% identity), suggesting that all are derived from a common ancestral gene. mRNA expression analysis as well as studies of transgenic plants transformed with promoter-beta-glucuronidase fusions for each of these genes revealed that the four genes exhibit differential transcriptional regulation and expression patterns. P69A and P69D are expressed constitutively, but with different expression profiles during development, whereas the P69B and P69C genes show expression following infection with Pseudomonas syringae and are also up-regulated by salicylic acid. We propose that these four P69-like proteases, as members of a complex gene family of plant subtilisin-like proteases, may be involved in a number of specific proteolytic events that occur in the plant during development and/or pathogenesis.
Amino Acid Sequence↗
Developmental regulation of a cytosolic ascorbate peroxidase gene from tomato plants.
This report describes the characterisation of a gene (APX20) from tomato plants that encodes a cytosolic isoform of ascorbate peroxidase which is involved in the detoxification of intracellular H2O2. Expression analysis of promoter-GUS fusions in transgenic plants reveals that the gene is under strict developmental control, becoming transcriptionally active in cells which are apparently undergoing mechanical stimulation generated during the different phases of growth. We show that the APX20 gene contains a large 5' leader intron which is required to confer constitutive gene expression in leaves, but not in other organs of the plant. Based on these observations, we propose that the observed transcriptional regulation of this gene may constitute a basic mechanism for deployment of antioxidative defences in plants, which act to limit the deleterious effects of H2O2 generated during normal plant development.
5' Untranslated Regions↗
Single-photon emission computed tomography: ictal perfusion in childhood epilepsies.
Explore the source record for details and available documents.
Child↗
Identification of a novel peptide motif that mediates cross-linking of proteins to cell walls.
A cDNA clone representing a member of a novel class of cell wall proteins was isolated from tobacco plants. We have designated this protein NtTLRP for tyrosine- and lysine-rich protein. It is structurally related to the previously identified TLRP from tomato plants, sharing a high amino-acid sequence similarity at the C-terminal region. This region contains what appears to be a novel peptide motif which we call CD for cysteine-rich domain, and which is common to several other cell-wall proteins. By using a functional test in transgenic plants, we demonstrate that the presence of the CD domain is per se sufficient to cross-link previously soluble proteins to the cell wall. We present evidence that NtTLRP is cross-linked and specifically localizes to the cell wall of lignified cells. The highly localized deposition of NtTLRP in these cells indicates that this class of cell-wall proteins may have a specialized function in the formation of xylem tissue.
Amino Acid Sequence↗
A tomato homeobox gene (HD-zip) is involved in limiting the spread of programmed cell death.
Antisense suppression in transgenic tomato plants of H52, a gene encoding a new homeodomain protein of the HD-Zip class, produces a conditional lethal phenotype. The transgenic lines that survive exhibit spontaneous misregulation of cell death control in leaves, which, once initiated, propagates and engulfs the entire leaf. Activation of defence genes, over-accumulation of ethylene and conjugated salicylic acid, and growth reduction of virulent pathogens also occurs in these plants. In wild-type plants, H52 is up-regulated upon infection, mirroring the generation of the oxidative burst which normally precedes the hypersensitive response (HR). Thus, H52 appears to be a transcription factor involved in cellular protection by limiting spread of programmed cell death in plants.
Amino Acid Sequence↗
Identification of a new pathogen-induced member of the subtilisin-like processing protease family from plants.
By using biochemical, immunological, and molecular strategies we have identified and cloned a cDNA encoding a protease from tomato (Lycopersicon esculentum) plants (P69B) that is part of a proteolytic system activated in the plant as a result of infection with citrus exocortis viroid. This new protease is closely related, in terms of amino acid sequence and structural organization, to the previously identified pathogenesis-related subtilisin-like protease (Tornero, P., Conejero, V., and Vera, P. (1996) Proc. Natl. Acad. Sci. U. S. A. 93, 6332-6337). The 745-residue amino acid sequence of P69B begins with a cleavable signal peptide, contains a prodomain and a 631-residue mature domain which is homologous to the catalytic modules of bacterial subtilisins and eukaryotic Kex2-like proteases. Within the catalytic domain, the essential Asp, His, and Ser residues that conform the catalytic triad of this family of proteases are conserved in P69B. Northern blot and reverse transcriptase-polymerase chain reaction analysis demonstrated widespread induced expression of the 2.5-kilobase hybridizing mRNA in plant tissues as a consequence of viroid infection. We propose that P69B is a member of a complex gene family of plant Kex2/subtilisin-like proteases presumably involved in a number of specific proteolytic events activated during pathogenesis in plants and that takes place in the extracellular matrix.
Amino Acid Sequence↗