A novel sacsin mutation in a Japanese woman showing clinical uniformity of autosomal recessive spastic ataxia of Charlevoix-Saguenay.
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Adult↗
Biomedical subjects
S Okawa
Publications and source records attributed to S Okawa.
Reduction of noise from magnetoencephalography data.
A noise reduction method for magnetoencephalography (MEG) data is proposed. The method is a combination of Kalman filtering and factor analysis. A state-space model for a Kalman filter was constructed using the forward problem in MEG measurement. Factor analysis provide estimations of noise covariances required by the Kalman filter to eliminate independent additive sensor noise. The proposed method supports independent component analysis (ICA), which is difficult to use in MEG analysis owing to the sensor noise. Numerical experiments were conducted to investigate the performance of the proposed method. In a single dipole case where the maximum signal-to-noise ratio (SNR) was -10 dB, approximately equivalent to raw MEG data, noise-free signals were successfully estimated from noisy data; a 0.02 s delay of the peak latency and 15-40% of attenuation of the peak amplitude were observed. Moreover, in a multiple dipole case, independent components preprocessed with the proposed method had high correlation, 0.88 at the lowest, with correlation of 0.69 and 0.52 for those preprocessed with conventional bandpass filters. The results show that the noise reduction method reduces sensor noise effectively. High SNR-independent components are obtained by the proposed method. Real MEG data analysis was also demonstrated. The proposed method extracted auditory evoked responses from unaveraged single-trial data.
Algorithms↗
Massive accumulation of M and H subunits of neurofilament proteins in spinal motor neurons of neurofilament deficient Japanese quail, Quv.
Quiver (Quv) is a non-sense mutation of neurofilament protein L subunit (NF-L) that causes neurofilament deficiency with preserved microtubules in Japanese quail. Anti-NF-M and anti-NF-H mAbs stained cell bodies of motor neurons in Quv embryo spinal cords much more intense than those in control spinal cords. Volume of motor neurons in Quv spinal cords increased to 2.3 times of control motor neurons. Immunoblot of Quv spinal cords revealed a relative increase in non- and hypo-phosphorylated NF-M and NF-H, and a decrease in the total amount of NFs. Quv sciatic nerves showed faintly reacted phosphorylated NF-M and NF-H. These results suggest that deficiency of assembled neurofilament results in decreased axonal transport of NFs and accumulation of NFs in cell bodies of spinal motor neurons.
Animals↗
Coronary risks after high-dose gamma-globulin in children with Kawasaki disease.
OBJECTIVES: The goals of the present study were to develop a predictive coronary risk scoring system after intravenous gamma-globulin (IVGG) therapy of any dose for the different preparations currently used in the treatment of children with Kawasaki disease and to determine the predictive value of the system. The previously reported scoring systems were based on treatment with high-dose IVGG therapy at limited doses and were determined using investigative methods. METHODS: Four hundred and fifty-one patients were randomized into one of three groups and received either i.v. polyethylene glycol-treated human immunoglobulin at a dose of either 200 (n = 147) or 400 mg/kg per day (n = 152) or freeze-dried sulfonated human immunoglobulin at 200 mg/kg per day (n = 152) for 5 consecutive days. We documented 31 cases of coronary abnormalities (CA). Univariate and multivariate logistic regression was performed using 49 clinical variables and the resulting predictive model was validated. RESULTS: The duration of fever (odds (I day)/odds (- 5 days)= 0.158; 95% confidence interval (CI) 0.0385-0.648), hemoglobin (odds (Q1 = 10.3)/odds (Q3 = 11.6) = 3.97; 95% CI 1.92-8.20), IgG (odds (Q1 = 1,900)/odds (Q3=2,658)=2.72, 95% CI 1.18-6.25) and IgA (odds (Q1 =72)/odds (Q3= 160) = 0.415; 95% CI 0.253-0.680) levels after completion of gamma-globulin infusion were independent predictors. The model is quasi-cross validated and has acceptable sensitivity and selectivity. The estimated risk and observed occurrence of CA coincide. CONCLUSIONS: Determinants of the risk of CA after IVGG therapy are a longer duration of fever, a lower IgG level, a higher IgA level and a lower hemoglobin level after IVGG infusion. This model is applicable for IVGG doses from 1 to 2 g/kg and for at least two different gamma-globulin preparations.
Child↗
Surface composition and structure of titanium polished with aqueous slurry of ferric oxide.
Cast plates were prepared from commercial titanium. The plates were polished with a slurry of fine ferric oxide powder. The surface composition and structure were investigated by electron probe microanalysis (EPMA) and X-ray photoelectron spectroscopy (XPS). In the high pressure-polished surface, iron was non-uniformly distributed but oxygen was mostly uniformly distributed, while in a light pressure-polished surface, iron and oxygen were uniformly distributed though at lower and higher concentrations, respectively. EPMA state analysis and XPS suggested that the iron might exist as Fe2+ in the outermost surface, while it might be in a metallic state in the inner surface layer.
Copper↗
New partition technique for two-chamber pressure casting unit for titanium.
Although titanium has been widely used in prosthetic appliances, such as cast removable partial denture frameworks, high quality castings are still difficult to obtain. This study proposes a new partition technique using a metal foil for improving castability of a two-chamber pressure casting unit. The metal foil was formed beforehand to bring it in contact with the mold crucible wall so that no clearance was left behind it. Using this formed foil, the mold cavity can be completely isolated from the upper chamber and can be maintained in a vacuum even after the rupture of the foil. Accordingly, a large casting force is generated very quickly, and as a result, castability is expected to be significantly improved. This expectation was confirmed by the experiments using wax patterns including thin plate and mesh plate.
Aluminum↗
Surface contamination of titanium by abrading treatment.
This study investigated the contamination of abraded Ti surfaces. Using a polishing machine, specimens were abraded with waterproof SiC grit papers under water cooling. The abraded surfaces were examined using element analysis, X-ray diffraction, and hardness tests. Contaminant deposits with dimensions reaching about 30 microns were observed throughout the surface. In these deposits, Ti was apparently reduced by about 10% and replaced by Si and O. The chemical bond state of the Si was similar to that of SiC or a titanium silicide. The O was solute in Ti, which increased the surface hardness. The contaminant deposits were amorphous or very thin. The contamination of Ti, the extent of which was related to hardness, resulted from a reaction with abrasives.
Dental Polishing↗
Possible segregation caused by centrifugal titanium casting.
The possibility of the segregation under solidification process using a centrifugal casting machine was investigated using an electron probe microanalyzer with elemental distribution map, line analysis and quantitative analysis. When a very small quantity of platinum was added to local molten titanium during the casting process, macroscopic segregation was observed under conditions of density difference of 0.1 g/cm3 at the most, confirming that the centrifugal force of the casting machine is extremely strong. When a Ti-6Al-4V alloy was cast, however, no macroscopic segregation was observed. The centrifugal force of the casting machine examined in the present study hardly results in the body-force segregation in this titanium alloy.
Alloys↗
[Prediction of high risk group of patients with hepatitis-C-virus type liver cirrhosis developing to liver neoplasm].
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Alanine Transaminase↗
[Effects of lactitol on fecal bacterial flora in patients with liver cirrhosis and hepatic encephalopathy].
Lactitol, a non-absorbable synthetic disaccharide, was administered at a dose of 36g/day for 3-4 weeks to 8 patients with liver cirrhosis and hepatic encephalopathy in order to investigate its effects on fecal bacterial flora and clinical symptoms of hepatic encephalopathy. Lactitol significantly increased occupation ratio (ratio to total bacterial number) of anaerobic Bifidobacterium (before administration 7.1% --> after 4 weeks 46.0% (p < 0.05) as well as bacterial count of Lactobacillus. On the other hand, bacterial counts of Bacteroides and Clostridium, which are considered to be NH3-producing bacteria, and that of total aerobic bacteria were not markedly changed, but their occupation ratio were decreased after the administration. Further, tendencies toward decreased fecal pH, increased frequency of defecation and soft stools were observed. As for clinical efficacy, a decrease in blood ammonia concentration, improvement in mental state and flapping tremor were also observed.
Aged↗
A multicenter, randomized, controlled trial of intravenous gamma globulin therapy in children with acute Kawasaki disease.
We studied the effect of intravenous, polyethyleneglycol-treated, human immunoglobulin, administered at 200 mg/kg per day (group A: n = 147; male 86, female 61; age < 1 year, 50) or 400 mg/kg per day (group B: n = 152; male 87, female 65; age < 1 year, 52) for five consecutive days and compared it with freeze-dried, sulfonated human immunoglobulin [group C: n = 152; male 87, female 65; age < 1 year, 51), administered at 200 mg/kg per day for five consecutive days, on the prevention of coronary artery abnormalities in Kawasaki disease. Echocardiograms were interpreted blindly and independently. Proportions of 87.1%, 95.4%, and 82.3% in groups A, B, and C, respectively, had no coronary artery abnormalities. The confidence limits of difference between the proportions of groups A and C, groups B and C, and groups B and A were -4.4% and 10.4%, 7.8% and 15.9%, and 4.0% and 10.8%, respectively. Duration of fever and serum immunoglobulin G (IgG) levels were correlated with the prevalence of coronary artery abnormalities. We concluded that intravenous, polyethyleneglycol-treated, human immunoglobulin and freeze-dried, sulfonated human immunoglobulin had clinically equivalent effects on coronary artery abnormalities, and that five daily doses of 400 mg/kg of intravenous, polyethyleneglycol-treated, human immunoglobulin is more effective than that of 200 mg/kg gamma globulin.
Acute Disease↗
Skin holes of titanium casting.
The present study describes refractories which contribute to formation of skin holes of Ti casting. Plates consisting of quartz (Qu) and cristobalite (Cr) powders and Qu- and Cr-MgO mixtures were fired at high temperatures and used as phosphate-free molds. The cross-section of the castings was investigated using an optical microscope and an electron probe microanalyzer. Whereas the castings into the Qu molds were almost complete, those into the Cr molds were incomplete. The surface reaction zone of the Qu molds formed a typical layered structure with fewer skin holes. That of the latter extended deeply into the interior with a net-shaped Ti-Si phase, cracks, and holes. Addition of MgO to Cr effectively improved the castability and changed the reaction zone to the typical layered structure. However, slender holes occurred along the mold wall. The overall area around the holes was contaminated by Si and Mg. Probably, this resulted from a reaction of forsterite with Ti. The reactivity difference between Qu and Cr was not related to the fired strength of mold.
Dental Casting Investment↗
Involvement of calmodulin- and protein kinase C-related mechanism in an induction process of peroxisomal fatty acid oxidation-related enzymes by hypolipidemic peroxisome proliferators.
Trifluoperazine, a calmodulin antagonist, suppressed the clofibric acid-evoked induction of the peroxisomal cyanide-insensitive fatty acyl-CoA oxidizing system and carnitine acetyltransferase in rat liver and also in cultured rat hepatocytes. H-7, a potent inhibitor of protein kinase C, also suppressed the induction of these enzymes by clofibric acid, bezafibrate, Wyl4,643 or mono(2-ethylhexyl)phthalate in cultured rat hepatocytes. This suppressive effect was also confirmed by the protein composition of hepatocytes treated with clofibric acid and these antagonists, where the increase in the amount of peroxisomal bifunctional enzyme by peroxisome proliferator was markedly suppressed by above two antagonists. Profile of the time-dependent changes in the activities of the two enzymes after clofibric acid treatment showed that there might be two phases in the induction process. The initial phase (0-3 days after the treatment) showed a relative low inducing rate and subsequent phase (3-5 days after the treatment) showed an abrupt induction. The suppressive effect of the above two antagonists was significant in the later phase. In a time course study of the induction process of peroxisomal catalase, bifunctional enzyme or 69 kDa integral membrane protein using immunochemical detection, the induction of the membrane protein by clofibric acid was delayed compared with that of the bifunctional enzyme, where the induction was inhibited almost completely by nicardipine. These experimental results suggest that calmodulin- and protein kinase C-dependent processes play an important role in the process of marked induction of peroxisomal enzymes and membrane protein by drugs in rat liver.
1-(5-Isoquinolinesulfonyl)-2-Methylpiperazine↗
[Effect of antiplatelet and anticoagulant therapy on secondary prevention and long-term prognosis after acute myocardial infarction in aged patients].
The long-term efficacy and side effects of antiplatelet and anticoagulant therapy for secondary prevention after the first acute myocardial infarction (AMI) were retrospectively assessed in 133 patients over 60 years of age during a mean follow-up period of 36.6 months. Seventy five patients received antiplatelet and anticoagulant therapy (group 1) and 58 patients did not (group 2). In group 1 patients, 54, 12 and 9 patients received ticlopidine, aspirin and warfarin, respectively. Mean age, sex ratio, site of AMI, max CPK value and left ventricular ejection fraction in the convalescent phase did not differ between the two groups. There were no differences between the two groups in terms of the number and kind of combination drugs such as nitrate, beta-blocker and Ca antagonist. During the follow-up period 40 patients died; 18 patients (45%) suffered cardiac death and 22 patients (55%) experienced non-cardiac death. Nineteen patients had recurrent MI and 37 patients had cardiac events which were defined in total as cardiac death, recurrent MI and unstable angina pectoris. The total mortality rate and rate of recurrent MI based on the life time table method were significantly lower in group 1 than in group 2 by the generalized Wilcoxon test. The cumulative total mortality rate in the fifth year was 24.2% in group 1 and 49% in group 2. The cumulative rate for recurrent MI in the fifth year was 7.4% in group 1 and 27.5% in group 2 (p less than 0.05). However, the rate of cardiac events did not differ between the two groups.(ABSTRACT TRUNCATED AT 250 WORDS)
Aged↗
Successful aggressive treatment against multiple intra-abdominal metastases from renal cell carcinoma 18 years after nephrectomy.
The management of late metastases from renal cell carcinoma is often difficult because of multiple organ involvement. We report a case of multiple metastases from renal cell carcinoma in the duodenum, pancreas, intestine, falciform ligament and liver, 18 years after nephrectomy. The patient underwent a total pancreatectomy following a gastroduodenal arterial embolization to control duodenal bleeding, a resection of the ileum and falciform ligament at a second laparotomy and repeated hepatic arterial embolizations to control the growth of liver metastases. Aggressive treatment should be undertaken in cases of late recurrence of renal cell carcinoma after nephrectomy because of the possibly slow-growing biological character of the tumor.
Abdominal Neoplasms↗
Co-suppression by nicardipine, a calcium antagonist, of induction of microsomal lauric acid hydroxylation with peroxisome proliferation in clofibrate-treated rat liver.
The in vivo effect of nicardipine, a well-known calcium antagonist, on microsomal omega-oxidation of laurate in clofibrate-treated rat liver was studied. The 15.3-fold induction of the activity by 2 weeks administration of 0.25% clofibrate in the diet was markedly suppressed to about 6-fold by co-administration of nicardipine at 100 mg/kg body weight. Similarly, the induction of peroxisomal beta-oxidation and carnitine acetyltransferase activities were also suppressed by this simultaneous administration by more than 50%. Although clofibrate also induced the activity of reduced nicotineamide adenine dinucleotide phosphate (NADPH)-cytochrome c reductase and increased the hepatic content of cytochrome P-450, no suppressive effect of nicardipine was observed. Contrarily, nicardipine induced the reductase activity and increased the hepatic content of cytochromes P-450 and b5. These results provide the first demonstration of a calcium antagonist, e.g. nicardipine acting as inhibitor of the induction of microsomal omega-oxidation, in association with the inhibition of peroxisome proliferation in animals. The suppression of drug-induced peroxisome proliferation and microsomal omega-oxidation by the calcium antagonist may help in elucidating the causal relationship of the induction mechanisms between peroxisomal and microsomal enzymes.
Animals↗
Molten titanium flow in a mesh cavity by the flow visualization technique.
The tracer element molten (TEM) method, which provides titanium flow visualization in a mold cavity, was applied to investigate the flow in a mesh pattern. When a pressure casting machine was used, most of the mesh rods were composed of many solidification layers, which implied that the flow was laminar and almost steady. When a centrifugal casting machine was used, the flow was almost irregular and a stack of solidification layers was observed in only a few segments. These results indicate that the flow was turbulent. In a complicated cavity, such as a mesh pattern, pressure rather than centrifugal force acts as an effective casting force. Therefore, in such a cavity, favorable castability can be achieved with high pressure and laminar flow.
Dental Casting Technique↗
Cardiac sequelae of Kawasaki disease in Japan: statistical analysis.
The proportions of Kawasaki disease patients with cardiac sequelae in Japan were analyzed using nationwide survey data from the 6 1/2-year period July 1982 through December 1988. Of 46,864 cases of Kawasaki disease reported in the surveys, 7637 or 16.3% had cardiac sequelae such as dilation or stenosis of coronary arteries, myocardial infarction, and valvar lesions 1 month or more after onset. The prevalence of cardiac sequelae was particularly high in males, infants younger than 1 year, and children older than 5 years of age. In sequential observation, there was no correlation between the prevalence of cardiac sequelae and periods of high or low incidence of the disease. The prevalence of cardiac sequelae overall declined steadily over the observation period, perhaps as a consequence of increasing use of intravenous gamma globulin. However, children older than the age of 5 years manifested increasing prevalence of cardiac sequelae over the observation period, probably as a result of lower rates of intravenous gamma globulin administration.
Child↗