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Lafora disease is a fatal genetic disorder characterised by neurotoxic deposits of malformed insoluble glycogen. In humans it is caused by mutation in the EPM2A or NHLRC1 genes. There is a known mutation in miniature wirehaired dachshunds which has not been documented in other dog breeds, including beagles, in which the disease is relatively commonly reported. This case report describes the causative defect in two affected beagles, namely the same massive expansion as in miniature wirehaired dachshunds of a 12‐nucleotide repeat sequence that is unique to the canine NHLRC1 gene. This is the first mutation described in beagles with Lafora disease, and so far the only Lafora disease genetic variant in dogs.  相似文献   
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Tomasek O  Tukac V 《Avian diseases》2007,51(4):989-991
Eight captive-bred horned parakeets (Eunymphicus cornutus) and four captive-bred Major Mitchell cockatoos (Cacatua leadbeateri) from the same aviary tested positive for psittacine circovirus (PsCV) DNA in whole blood by nested-polymerase chain reaction (PCR). The chronic form of disease with feather fragility and loss was observed in three horned parakeets. Infection in other individuals was subclinical. Immunosuppression, either hematologically or as susceptibility to secondary infections, was not observed. Treatment consisted of the administration of beta-(1,3/1,6)-D-glucan from oyster mushroom (Pleurotus ostreatus). Excluding two accidentally dead parakeets, four out of the original six horned parakeets, and all Major Mitchell cockatoos were negative for PsCV DNA in whole blood in 7-9 mo after the treatment was started. Even though the absence of PsCV DNA in blood does not signify elimination of the virus from the whole organism, these preliminary results indicate a possible effect of beta-glucan in the treatment of PsCV infection. To the author's knowledge, this is the first report of PsCV in horned parakeets.  相似文献   
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The authors analysed epidemiological data of the Hungarian tick-borne encephalitis epidemic from the past seven decades. A total of 911 meningitis serosa cases were described from 1930-1950 s by local hospital physicians, indicating that the virus had been present in the country decades before its official identification in 1952. The virus spread freely in the 1950s–1960s, occupying almost all habitats where ticks occurred in large numbers. The increasing number of cases drove authorities to classify this illness as a notifiable disease in 1977 and to organize the first measures to stop the epidemic. Statistical analysis revealed that the large-scale vaccination launched from the 1990s was responsible for the sharp decrease in the number of human cases from 1997. A significant negative correlation was found between the number of vaccine doses sold and human cases 6 years later. The TBEV endemic area covers 16.57% of the territory and 16.65% of the population of the country. In the last 10 years, 186,000 vaccine doses/year in average were enough to keep the incidence of human TBEV infections between 0.45 and 0.06/100,000 persons. A 20-year-long study found evidence for easing clinical signs in TBEV-infected hospitalized patients. Statistics found a sharp decrease in the number of samples sent for TBEV diagnosis after 1989. Male dominance of patients was characteristic of the epidemics since the 1940s, but now analysis of detailed data from the 1981–2021 period (60.5%–87.5%) proved the statistical significance of this dominance. Obviously, the voluntary vaccination programme was the tool which broke the spread of the epidemic. Widespread public awareness of the disease and the tick vector, probable evolutionary spread of less pathogenic virus strains supplemented with the vaccination campaign led to a negligible level of human TBE cases in Hungary in the last years.  相似文献   
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Our previous study revealed the expression of substance P (SP) and calcitonin gene‐related peptide (CGRP) in sensory distal ganglion of the vagus (nodose ganglion) neurons in the pig. As these neuropeptides may be involved in nociception, the goal of these investigations was to determine possible expression of vasoactive intestinal polypeptide (VIP), SP and CGRP in the pituitary adenylate cyclase‐activating polypeptide‐immunoreactive (PACAP‐IR) porcine nodose perikarya. Co‐expression of these substances was examined using a double‐labelling immunofluorescence technique. To reveal the ganglionic cell bodies, the pan‐neuronal marker protein gene product 9.5 (PGP 9.5) was used. Quantitative analysis of the neurons revealed that 67.25% of the PGP 9.5+ somata in the right‐side ganglion and 66.5% in the left side, respectively, co‐expressed PACAP‐IR. Moreover, 60.6% of the PACAP‐IR cells in the right‐side ganglion and 62.1% in the left, respectively, co‐expressed VIP. SP‐IR was observed in 52.2 and 39.9% of the right and left ganglia, respectively. CGRP was found in 27.7 and 34.1% of the right and left distal ganglion of the vagus, respectively. High level of co‐expression of PACAP with VIP, SP and CGRP in the distal ganglia of the vagus sensory perikarya directly implicates studied peptides in their functional interaction during nociceptive vagal transduction.  相似文献   
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This paper deals with the determination of the pore-size distribution of untreated and thermally modified twin samples of Norway spruce (Picea abies (L.) Karst.) and sycamore maple (Acer pseudoplatanus L.) by means of mercury intrusion porosimetry. The investigations considered oven-dried and at 22 °C and 95 % relative humidity-conditioned samples. A special sample holder and a novel method for evacuation were developed for the measurements. This was necessary both to ensure that samples were positioned at a defined distance and the conditioned samples did not dry in an uncontrolled manner. The results clearly show that the climatic state influences the results. Thermal modification greatly alters the pore-size distribution of spruce. This can be largely attributed to the formation of intercellular spaces in the middle lamella as a result of cell-wall compression due to thermal modification. The alteration of the pore structure of maple due to thermal modification is not as pronounced as that of spruce.  相似文献   
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Modern ultrasound contrast media are gas-containing stabilized microbubbles that remain intact in the circulating blood for several minutes after intravenous injection and increase the intensity of the backscattered ultrasound. When the microbubbles disappear from the blood, they can be detected in the parenchyma of the liver and the spleen for about 30 more minutes (late liver- and spleen-specific phase). The insonated microbubbles produce second harmonic ultrasound frequencies, whose detection requires nonconventional ultrasound modalities such as pulsed inversion imaging. Nonconventional ultrasound techniques can also be used without microbubbles because second harmonics can be generated by ultrasound in tissues as well. The physical principles and advantages of nonconventional ultrasound techniques are described. The circulating microbubbles can be used not only to enhance weak Doppler signals, but also to perform dynamic contrast studies. Contrast-enhanced dynamic ultrasound studies--similar to contrast-enhanced CT and MRI examinations--have been used in humans to characterize lesions noninvasively (i.e., without biopsies) found during conventional ultrasound examinations. To map the distribution of contrast medium in a nodule or in an organ, specific scanning techniques such as stimulated acoustic emission have been developed. Stimulated acoustic emission occurs when high acoustic pressure ultrasonic waves disrupt the stationary or slowly moving microbubbles. This results in the release of a large amount of harmonic ultrasound frequencies. When the stimulated acoustic emission technique is used for dynamic studies, scanning must be interrupted several times to allow the microvasculature of the lesion to refill with microbubbles (interval delay imaging). The contrast patterns of malignant and benign hepatic nodules in humans have been the most intensively studied. Another type of dynamic study in humans measures the transit time of the contrast medium; that is, how fast the peripherally injected microbubbles reach the hepatic veins. Hepatic cirrhosis can be differentiated from other diffuse parenchymal liver diseases by a shorter transit time. Introducing nonconventional ultrasound techniques and ultrasound contrast media in veterinary diagnostic imaging may have potential value; however, intensive research should be carried out before ultrasound contrast agents can routinely be used in clinical practice.  相似文献   
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Mineral properties in Earth's lower mantle are affected by iron electronic states, but representative pressures and temperatures have not yet been probed. Spin states of iron in lower-mantle ferropericlase have been measured up to 95 gigapascals and 2000 kelvin with x-ray emission in a laser-heated diamond cell. A gradual spin transition of iron occurs over a pressure-temperature range extending from about 1000 kilometers in depth and 1900 kelvin to 2200 kilometers and 2300 kelvin in the lower mantle. Because low-spin ferropericlase exhibits higher density and faster sound velocities relative to the high-spin ferropericlase, the observed increase in low-spin (Mg,Fe)O at mid-lower mantle conditions would manifest seismically as a lower-mantle spin transition zone characterized by a steeper-than-normal density gradient.  相似文献   
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