[Differential diagnosis of Becker-type muscular dystrophy from polymyositis].
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Adult↗
Biomedical subjects
M Hara
Publications and source records attributed to M Hara.
Mechanical unfolding of alpha2-macroglobulin molecules with atomic force microscope.
alpha2-Macroglobulin was derivatized with a sulfhydryl cross-linker and sandwiched between a mica substrate and a silicon nitride tip, both coated with gold, of an atomic force microscope and force curve measurement was carried out. An extensive downward deflection of the cantilever was observed in the retracting realm of the curve, when and only when the substrate was covered with the derivatized protein. The result was interpreted in terms of the mechanical stretching and unfolding of a single or a few protein molecules.
Cross-Linking Reagents↗
Paraquat toxicity and oxidative damage. Reduction by melatonin.
The ability of melatonin to protect against paraquat-induced oxidative damage in rat lung, liver, and serum was examined. Changes in the levels of malondialdehyde (MDA) plus 4-hydroxyalkenals (4-HDA) and reduced and oxidized glutathione concentrations were measured. Paraquat (50 mg/kg) was injected i.p. into either Sprague-Dawley or Wistar rats with or without the co-administration of 5 mg/kg melatonin. Paraquat alone increased MDA + 4-HDA levels in serum and lungs of both rat strains, with these increases being abolished by melatonin co-treatment. Paraquat also decreased reduced glutathione levels and increased oxidized glutathione concentrations in lung and liver; these changes were negated by melatonin. The effect of melatonin on paraquat-induced mortality was also studied. Paraquat at a dose of 79 mg/kg was lethal for 50% of animals within 24 hr; when administered together with melatonin, the LD50 for paraquat increased to 251 mg/kg.
Animals↗
Identification of pentosidine as a native structure for advanced glycation end products in beta-2-microglobulin-containing amyloid fibrils in patients with dialysis-related amyloidosis.
beta-2-Microglobulin (beta-2m) is a major constituent of amyloid fibrils in patients with dialysis-related amyloidosis (DRA). Recently, we found that the pigmented and fluorescent adducts formed nonenzymatically between sugar and protein, known as advanced glycation end products (AGEs), were present in beta-2m-containing amyloid fibrils, suggesting the possible involvement of AGE-modified beta-2m in bone and joint destruction in DRA. As an extension of our search for the native structure of AGEs in beta-2m of patients with DRA, the present study focused on pentosidine, a fluorescent cross-linked glycoxidation product. Determination by both HPLC assay and competitive ELISA demonstrated a significant amount of pentosidine in amyloid-fibril beta-2m from long-term hemodialysis patients with DRA, and the acidic isoform of beta-2m in the serum and urine of hemodialysis patients. A further immunohistochemical study revealed the positive immunostaining for pentosidine and immunoreactive AGEs and beta-2m in macrophage-infiltrated amyloid deposits of long-term hemodialysis patients with DRA. These findings implicate a potential link of glycoxidation products in long-lived beta-2m-containing amyloid fibrils to the pathogenesis of DRA.
Amyloid↗
Cold air-induced bronchoconstriction is mediated by tachykinin and kinin release in guinea pigs.
In the present study, we investigated the role of acetylcholine, tachykinins and kinins in the bronchoconstriction induced by cold air inhalation. Cold air was delivered to anaesthetised, artificially ventilated guinea pigs through a tracheal cannula. Inhalation of cold air increased the maximum total pulmonary resistance (RL) in a time-dependent manner, reaching a maximum after 15 min of exposure. The increase in RL induced by exposure to cold air for 10 min was not affected by pretreatment with atropine (1.4 mu mol/kg, i.v.); it was abolished by the tachykinin NK2 receptor antagonist, SR 48968 (0.3 mu mol/kg, i.v.) and was reduced by 58% by the kinin B2 receptor antagonist, HOE 140 (0.1 mu mol/kg, i.v.). These findings suggest that cold air induces bronchoconstriction in guinea pigs via a cascade that involves the release of kinins and tachykinins.
Animals↗
Pravastatin-induced rhabdomyolysis in a patient with mixed connective tissue disease.
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Enzyme Inhibitors↗
Increased matrix metalloproteinases as possible cause of osseoarticular tissue destruction in long-term haemodialysis and beta 2-microglobulin amyloidosis.
Immunolocalization of matrix metalloproteinases (MMPs) and tissue inhibitors of matrix metalloproteinases (TIMPs) in periarticular tissues of beta 2-microglobulin amyloidosis patients was investigated. MMP-1 (interstitial collagenase) the most strongly expressed of the MMPs, was localized in the synovial lining cells, mesenchymal cells in granulation tissue and nodular amyloid deposits, and chondrocytes within areas of cartilage erosion. Expression of MMP-1 was correlated with the degree of macrophage infiltration and synovial cell hyperplasia, but it was not correlated with the degree of amyloid deposition or haemodialysis period. Expression of MMP-1 appeared more intense than that of TIMP-1 and TIMP-2 in highly inflammatory cases. MMP-2 was mildly expressed in the interstitial fibroblasts and MMP-3 was faintly stained in the extracellular matrix of the synovial membrane. MMP-9 (gelatinase B) was found to be strongly positive in the osteoclasts which increased in the progressing osteolytic lesion from the destructive arthropathy. These results suggest involvement of MMPs in inflammation with an imbalance between expression of MMPs and TIMPs being closely related to pathogenesis of the destructive arthropathy.
Amyloidosis↗
Genetic basis of the neurovirulence of type 1 polioviruses isolated from vaccine-associated paralytic patients.
We examined four type 1 polioviruses isolated from the stools of patients with vaccine-associated paralytic poliomyelitis in China. All of these isolates were shown to be Sabin derived viruses by restriction fragment length polymorphism assay after polymerase chain reaction and by sequencing of the viral genome encoding the viral coat protein, VP1. However, the same analysis of the 3D coding region suggested that two of the four isolates had the sequence of wild type poliovirus in the tested region. Furthermore there were also point mutations in the 5' non-coding region. One was a single base change from U to C at nucleotide position 525, and the other three were from G to A at position 480. All the four strains were more neurovirulent that Sabin type 1 virus in transgenic mice with human poliovirus receptor gene. The data showed that the nucleotide positions of type 1 poliovirus which were identified to be in favor of the high neurovirulence were indeed changed during natural transmission, and suggested that the point mutation alone or a recombination of the vaccine type with wild type genome results in an acquisition of neurovirulence.
Animals↗
Quantitative renography with the organ volume method and interporative background subtraction technique.
When quantification of renal activity is performed by planar imaging, many correction factors must be considered. To obtain quantitative renal images and renogram, we have examined our proposed method by using the organ volume for scatter, attenuation, and background activity, and the interporative background subtraction (IBS) technique in phantom and clinical studies. A renal phantom study was performed by varying the renal depth from 3 to 11 cm and the kidney-to-background activity concentration ratio from 5 to 80. Planar images were properly corrected for scatter, attenuation and background activity by our method and the corrected images were compared with the images obtained by the conventional method for the estimation of true renal activity. Clinical Tc-99m DTPA dynamic data for both a good and a poor renal function were also corrected by our method and volume-corrected renograms were obtained. For the phantom study, depth-independent images were obtained and these images gave a good estimation of the true count rate. In the clinical study, the conventional renogram was especially modified to allow for oversubtraction of background counts in the early phase (0-4 min). In conclusion, our proposed correction method can assess renal function qualitatively and quantitatively in both static and dynamic planar renal imaging.
Evaluation Studies as Topic↗
Central motor conduction time in patients with periventricular lucencies.
Central motor conduction time and regional cerebral blood flow were measured before and 20 min after intravenous injection of 17 mg/kg acetazolamide in 10 patients with periventricular lucencies (PVL) and 10 age-matched healthy controls. Central motor conduction time was measured using a magnetic stimulator and regional cerebral blood flow was measured by stable xenon computed tomography method. The central motor conduction time was significantly longer in the patients with PVL than in the healthy controls and was shortened significantly by the intravenous injection of acetazolamide in the patients with PVL. The blood flow not only in the periventricular white matter but also in the cerebral cortex and the cerebral white matter was significantly lower in the patients with PVL than in the healthy controls. The intravenous injection of acetazolamide increased significantly the regional cerebral blood flow except in the PVL areas. The prolongation of the central motor conduction time may be at least partly related with decreased blood flow in the cerebral cortex and cerebral white matter.
Acetazolamide↗
Detection of feline herpesvirus 1 DNA by the nested polymerase chain reaction.
The thymidine kinase region of feline herpesvirus 1 (FHV 1) genome in ocular/nasal swabs from cats with clinical manifestations of upper respiratory disease was amplified by nested polymerase chain reaction (nested PCR). Two primer pairs were prepared for nested PCR. FHV 1 DNA in ocular/nasal swabs was extracted using instaGene-DNA purification matrix. Nested PCR for the FHV 1 culture supernatants was ten times as sensitive as single PCR. On comparing viral isolation with single PCR and nested PCR for the detection of FHV 1 in ocular/nasal secretions, of 5 samples that yielded infectious virus in cell culture, 3 (60%) were positive in single PCR and 5 (100%) were positive in nested PCR. When 22 ocular/nasal swabs that did not yield FHV 1 were assayed, 3 were negative in both single PCR and nested PCR, 2 were positive in both single and nested PCR and 17 were positive in only nested PCR. Thus, FHV 1 was detected in 19/22 (86.4%) by the nested PCR and in 2/22 (9%) by single PCR. These results show that nested PCR is 4.8 (24 positive samples/5 positive samples) times as sensitive as single PCR.
Animals↗
Tachykinins mediate the potentiation of antigen-induced bronchoconstriction by cold air in guinea pigs.
The role of tachykinins in the potentiation of antigen-evoked bronchoconstriction induced by inhalation of cold air was studied in guinea pigs. Cold air was delivered through a tracheal cannula to anesthetized, artificially ventilated guinea pigs sensitized with ovalbumin and pretreated with atropine (1.4 micromol/kg). Inhalation of cold air increased total pulmonary resistance (RL) in a time-dependent manner; inhalation of cold air for 10 or 15 minutes, but not for 5 minutes, produced a significant increase in RL. Aerosolized ovalbumin (5 breaths) increased RL in a dose-dependent manner (0.5% to 5%). Inhalation of cold air for 5 minutes significantly enhanced both the peak and the duration of the increase in RL induced by 0.5% ovalbumin. The tachykinin neurokinin 2-receptor antagonist, SR 48968 (0.3 micromol/kg intravenously) inhibited both the peak and the duration of the bronchoconstriction induced by 5-minute inhalation of cold air and ovalbumin (0.5%), whereas it did not affect the response to ovalbumin (0.5%) alone. These findings suggest that exposure to cold air potentiates the bronchoconstriction response to antigen and that this potentiation is mediated by tachykinin release from sensory nerves.
Airway Resistance↗
Nerve growth factor (NGF) induces increase in multi-ubiquitin chains and concomitant decrease in free ubiquitin in nuclei of PC12h.
Changes in Ubiquitin-immunoreactivity after nerve growth factor (NGF) treatment were investigated in PC12h cells. Ubiquitin-immunoreactivity was increased in the nucleus of NGF-treated cells. The quantitative analysis revealed that, after 7 days of NGF treatment, almost 20% of cells had ubiquitin-immunoreactive nuclei and the frequency was increased thereafter. Levels of free ubiquitin and multi-ubiquitin chains were measured by radioimmunoassay (RIA) and enzyme-linked immunosorbent assay (ELISA), respectively. Measurements were carried out for four subcellular fractions: urea- and water-soluble extracts of nuclei and cytoplasm. Decrease in free ubiquitin was observed in water-soluble cytoplasmic extracts of NGF-treated cells, though increase in multi-ubiquitin chains in the same fraction was not observed. As for nuclei, increase in multi-ubiquitin chains and concomitant decrease in free ubiquitin were found in the water-soluble extracts after NGF treatment. Levels of multi-ubiquitin chains did not change in urea-soluble cytoplasmic extracts as well as nuclear urea-soluble ones after NGF treatment. These results indicated that multi-ubiquitination of nuclear proteins is increased during NGF-induced neuronal differentiation of PC12h cells.
Animals↗
Anaerobic capacity and maximal oxygen uptake during arm stroke, leg kicking and whole body swimming.
In the present study, we determined both anaerobic capacity (the maximal accumulated oxygen deficit) and maximal oxygen uptake (VO2max) during arm stroke (A), leg kicking (K), and whole body swimming (S), and compared them. The subjects were six trained college swimmers (two male and four female), aged 20 +/- 1 years. To determine VO2max for A, K and S, VO2max was measured during a 6-min swim at constant water flow rates. VO2 was measured by the Douglas bag method. Anaerobic capacity was determined by accumulated oxygen deficit during exercise lasting 2-3 min according to the methods of Medbø et al. Mean values of VO2max during A, K and S were 2.53 +/- 0.37 L min-1, 2.93 +/- 0.37 L min-1, and 3.23 +/- 0.43 L min-1, respectively. Those in A and K corresponded to 78.2% and 91.0% of that in S. Mean values of anaerobic capacity during A, K and S were 2.15 +/- 0.31 L, 2.52 +/- 1.08 L and 2.99 +/- 0.52 L, respectively. Those in A and K corresponded to 73.3% and 81.7% of that in S. Both VO2max and anaerobic capacity in S were much lower than the sum of A and K, corresponding to only 59.3% and 65.9%, respectively. These results suggest that the total energy production during S is lower than simply the sum of A and K because the potentials of both the anaerobic and aerobic energy releasing processes in the muscle groups involved in A and K cannot be fully reached during S.
Adult↗
Pit-1 gene expression in human pituitary adenomas using the reverse transcription polymerase chain reaction method.
OBJECTIVE: Previous studies of Pit-1 expression in human pituitary tumours have produced conflicting results. We have studied expression of Pit-1 mRNA in human pituitary adenomas, as well as in normal human and rat pituitaries, and results were compared with clinical, histological, and immunohistochemical features. In addition, expression of GH, PRL, and TSH-beta mRNA was also studied and compared with Pit-1 gene expression. MATERIAL AND METHODS: The adenomas consisted of 13 GH cell adenomas, 7 PRL cell adenomas, 3 TSH cell adenomas, 4 ACTH cell adenomas, and 10 clinically non-functioning adenomas. Expression of the Pit-1, its isoforms, and each hormone, was studied using the reverse transcription polymerase chain reaction. RESULTS: Pit-1 mRNA was expressed not only in normal human and rat pituitaries, but also in all GH, PRL and TSH cell adenomas. There was no correlation between Pit-1 transcripts and biological behaviour or histological findings in these three types of adenoma, suggesting that Pit-1 is generally required for the determination of cell phenotype but is insufficient for the regulation of hormonal activity and tumour growth in these pituitary adenomas. In addition, Pit-1 was also expressed in some ACTH cell (2/4) and non-functioning adenomas (7/10). Although there were no GH, PRL or TSH-beta transcripts in Pit-1 mRNA-negative ACTH cell and non-functioning adenomas, PRL mRNA was detected in all Pit-1 mRNA-positive ACTH cell adenomas and GH, PRL and/or TSH-beta mRNA were found in four of seven Pit-1 mRNA-positive non-functioning adenomas. In contrast, Pit-1 mRNA was expressed without any GH, PRL, or TSH-beta transcripts in only three non-functioning adenomas. These data suggest that expression of Pit-1 mRNA in these two types of adenomas can be mainly attributed to the presence of GH, PRL and/or TSH-beta mRNA expressing cells and that true Pit-1 transcripts found in non-functioning adenomas may be a rare event. Moreover, there were two cases which expressed Pit-1 alpha mRNA, but failed to show other Pit-1 isoform mRNA. There were, however, no clinical or histological differences between these two adenomas showing only Pit-1 alpha mRNA and the others expressing both Pit-1 alpha mRNA and other Pit-1 isoform mRNA. CONCLUSIONS: Pit-1 mRNA was expressed not only in GH, PRL and TSH cell adenomas but also in other types of adenoma. However, it is suggested that expression of Pit-1 mRNA in most ACTH cell and non-functioning adenomas can be attributed to GH, PRL and/or TSH-beta mRNA expressing cells. Further studies are necessary to elucidate the role of Pit-1 transcripts in the three non-functioning adenomas without GH, PRL and/or TSH-beta mRNA expression.
Adenoma↗
Effects of sodium ozagrel on hemostatic markers and cerebral blood flow in lacunar infarction.
The present study was designed to examine the effects of sodium ozagrel on hemostatic markers and cerebral blood flow in lacunar infarction. Ten cases of lacunar infarction in which sodium ozagrel was given (administered group), 10 cases of lacunar infarction in which sodium ozagrel was not given (nonadministered group), and 10 age-matched controls in which cerebrovascular diseases were absent but risk factors were similar to those of the patients (control group) were studied. Intravenous infusion of 80 mg of sodium ozagrel was done twice a day for 2 weeks. Platelet factor 4, beta-thromboglobulin, and fibrinopeptide A were significantly higher in the administered and nonadministered groups than in the control group at the time of admission. Platelet factor 4, beta-thromboglobulin, fibrinopeptide A, and thromboxane B2 were decreased significantly by the administration of sodium ozagrel. The blood flow in the cerebral cortex was significantly lower in the administered and nonadministered groups than in the control group. The blood flows around the infarcted area, in the cerebral cortex, and in the cerebral white matter were significantly increased by the administration of sodium ozagrel. Sodium ozagrel is considered to decrease platelet aggregation and increase cerebral blood flow by decreasing thromboxane A2, which has a platelet-aggregating and a vasoconstricting action. Sodium ozagrel is considered to be effective in the acute phase of lacunar infarction.
Aged↗
Gamma knife radiosurgery for arteriovenous malformations: long-term follow-up results focusing on complications occurring more than 5 years after irradiation.
The detailed long-term follow-up results of 40 patients treated for cerebral arteriovenous malformations with gamma knife radiosurgery are presented, with special reference to postradiosurgical complications that can develop many years after irradiation. The follow-up period after radiosurgery was 54 to 205 months, excluding one mortality, with a mean and a median of 106 and 97 months, respectively. One patient (2.5%) has, to date, refused all neuroimaging follow-up examinations. Complete nidus obliteration was angiographically confirmed in 26 patients (65%) between 1 and 5 years after radiosurgery. In the remaining 13 patients (32.5%), although significant shrinkage of each nidus was angiographically demonstrated, complete obliteration was not attained during a 3- to 7-year period of follow-up after radiosurgery. Among these 13 patients, 1 underwent surgical extirpation of the nidus and 5 underwent second courses of gamma knife radiosurgery between 3 and 6 years after initial treatment; in 3 of the 5 patients, complete nidus obliteration was angiographically confirmed between 1 and 3 years after the second course of radiosurgery. There were no radiation- or arteriovenous malformation-related mortalities. However, we did experience one angiography-related mortality. We also experienced one morbidity (probably caused by hemorrhagic stroke), which developed 5 years after 2-year postradiosurgical angiography had demonstrated complete obliteration, and three radiation-related morbidities, two of which (hemiparkinsonian syndrome and visual field disturbances caused by delayed cyst formation) occurred 5.5 and 7 years, respectively, after irradiation. Furthermore, we observed another two patients who, although asymptomatic to date, showed delayed cyst formation on magnetic resonance imaging 5 and 10 years after irradiation, respectively. In total, 3 (23%) of 13 patients who underwent computed tomography and/or magnetic resonance imaging more than 5 years after radiosurgery showed delayed cyst formation. In conclusion, long-term follow-up, particularly with the use of neuroimaging techniques, is necessary even after the treatment goal has been achieved.
Adolescent↗
A case of dysembryoplastic neuroepithelial tumor of the parietal lobe with characteristic magnetic resonance imaging.
The case of a 4 year old boy with a dysembryoplastic neuroepithelial tumor (DNT) of the left parietal lobe is reported. The DNT was located mainly in the cortex and showed no mass effect on magnetic resonance imaging (MRI). T1-weighted images of the DNT showed the characteristic findings of lesion hypointensity, but with a well preserved gyrus-like configuration. This lesion was isointense to the normal cortex on proton density imaging and hyperintense on T2-weighted imaging. The characteristic features on T1-weighted and proton density imaging in this patient were useful in differentiating DNT from other types of tumors. Histologic findings in DNTs, which include the presence of both glial and neuronal cells without atypia and no definite transitional zone between the adjacent disorganized cortical cell layers, suggest that DNT is not a true neoplasm but rather a dysplastic lesion. It is clinically important to differentiate this tumor both from other benign tumors and malignant tumors which have different prognoses and therapies. The distinctive MRI findings, as well as the histologic features of DNT, support the diagnosis in the clinical setting.
Brain Neoplasms↗