The authors evaluated endothelial function in patients with MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke) by flow-mediated vasodilation (FMD) and found a significant ...decrease vs controls. Two years of supplementation with oral l-arginine, a nitric oxide precursor, significantly improved endothelial function to control levels and was harmonized with the normalized plasma levels of l-arginine in patients. l-Arginine therapy improved endothelial dysfunction and showed promise in treating strokelike episodes in MELAS.
Acute encephalopathy/encephalitis is one of the most important causatives of mortality and neurological deficit during childhood. The aim of this retrospective observational study was to investigate ...clinical variables and therapeutic options associated with the outcome of children with acute encephalopathy/encephalitis.
Relationships between the clinical information at admission and the neurological outcome evaluated using Pediatric Cerebral Performance Category Scale (PCPC) at 12 months after admission were assessed in 43 patients who were treated at 10 Japanese paediatric intensive care units.
Sixteen patients were cared for at normothermia, whereas mild hypothermia was applied to 27 children. In univariate analysis, ages ≤ 18 months, marked elevation in serum lactate dehydrogenase (LD) and aspartate transaminase, diagnosis of either acute necrotising encephalopathy or haemorrhagic shock and encephalopathy syndrome and longer hypothermic periods were associated with increased risks of death or severe neurological deficit, whereas hypothermia showed pivotal effects: the outcome of children cooled after 12 h of diagnosis was statistically invariant with normothermic children, but was significantly worse compared with children cooled ≤ 12 h. In multivariate analysis, younger ages and elevated serum LD were associated with adverse outcomes, whereas early initiation of cooling was related to favourable outcomes. For normothermic children, PCPC scores were dependent on the computed tomographic findings suggestive of cerebral oedema, serum LD levels and Glasgow Coma Scale at admission. For hypothermic children, PCPC scores depended on longer delays in cooling initiation.
Without therapeutic hypothermia, the outcome of children was determined by variables suggestive of the severity of encephalopathy/encephalitis at admission. Hypothermia may have pivotal impacts on the outcome of children according to the timing of cooling initiation following acute encephalopathy/encephalitis.
Objective It has known that a prevalence of Rett syndrome (RTT) is approximately 1.0 per 10,000 females. However, we have never Japanese data. In 2010, the recent revised criterion for diagnosis of ...RTT was published. Here, we performed nationwide survey of RTT, according to the new criterion, to clarify the Japanese epidemiology of RTT. Methods We selected 1,020 hospitals or institutes by stratified sampling from 2,918 ones specialized for pediatrics and pediatric neurology in Japan. We sent the first questionnaire to them to ask the number of RTT patients each institute followed from 2008 to 2009. After responded, we asked clinical details with the second questionnaire. Results We received 677 responses from 1,020 hospitals (total response rate: 66.4%), and they counted 480 definite RTT patients and 79 atypical patients. The estimated total number of definite RTT patients was 1011 (95% confidential interval, 778–1244), and the estimated prevalence of RTT patients in Japan was 0.90 per 10,000 girls. It revealed the local clustering of hospitals, which provide the medical service for RTT patients. Interestingly, over 80% of five main symptoms appeared in typical RTT patients until 3 year-old. Conclusion This is the first nationwide survey of RTT in Japan. It gave a prevalence of concomitant with previous studies in other countries. We have established the clinical and genetic database of Japanese RTT patients. Next, we have to use the database for clinical research to explore biological markers and treatment, with or without international collaborations.
Respiration (= oxygen consumption) rates and electron transport system (ETS) enzyme activities in conjunction with body carbon and nitrogen composition (for respiration) or protein (for ETS) were ...determined for over 50 copepod species from the mesopelagic (M; 500 to 1000 m), upper-bathypelagic (UB; 1000 to 2000 m) and lower-bathypelagic (LB; 2000 to 3000 m) zones of the western subarctic Pacific. Calculated specific respiration rates (SR, a fraction of body carbon respired) atin situtemperatures (3, 2 and 1.5°C for the M, UB and LB zones, respectively) were greater for the M zone copepods (mean: 1.1% body C d–1) than that for the UB and LB zone copepods (both 0.6% body C d–1). Respiration rates adjusted to those at 1°C by using a Q10value (2.0), and to those of specimens with 1 mg body nitrogen by using a body mass exponent (0.8) (e.g. adjusted metabolic rates, AMR, in μl O₂ (mg body N)–0.8h–1) were also greater for the M zone copepods than for the UB and LB zone copepods. ETS activities, measured as μl O₂ (mg protein)–0.8h–1, showed the same depth-related decline from the M zone to the LB zone. Stepwise regression analysis revealed that stage/sex, feeding type and/or reaction speeds (as judged by the presence/absence of myelin sheath enveloping axons) of copepods were possible additional variables affecting their respiration rates and ETS activities. The reduction in respiration rates and ETS activities from the M zone to the UB or LB zone is more pronounced when respiration rate data on Arctic/Antarctic epipelagic copepods is added; the same is true for ETS activities when respiration rate data is added from copepods dominant in the subarctic Pacific. The present results are compared with those of micronektonic crustaceans and fishes reported for specimens collected from 500 to 2000 m in other regions and discussed in the light of the ‘visual interactions’ hypothesis.
A 22-week fetus presented with a large left ventricular aneurysm, 24 × 21 × 18 mm in size, detected by abnormal four-chamber view, and severe fetal hydrops with pericardial effusion, ascites and skin ...edema. The aneurysm was thin-walled, hypokinetic, and had enlarged with gestational age, causing compression of the lung. Although the left ventricular function had progressively impaired as expressed by increase in Tei index, hydrops had resolved by 32 weeks of gestation, probably because of maternal digoxin therapy and successful compensation by the right ventricle, as represented by retrograde blood flow in the distal aortic arch via the patent arterial duct. Because of the significant risk of severe cardiorespiratory failure, we transported the mother to a neonatal cardiac surgical center at 38 weeks of gestation. Indeed, the baby showed severe cardiopulmonary failure after birth, showing 100% of cardiothoracic ratio on the chest X-ray film, but was saved by the successful Dor procedure, including surgical resection of the aneurysm at 10 h of life. In this case, serial echocardiographic evaluation can allow us to monitor the hemodynamics and lead to successful postnatal management.
MCP-PMT timing property for single photons Akatsu, M; Enari, Y; Hayasaka, K ...
Nuclear instruments & methods in physics research. Section A, Accelerators, spectrometers, detectors and associated equipment,
08/2004, Letnik:
528, Številka:
3
Journal Article
Recenzirano
We have measured the performance, especially the timing properties, of micro-channel plate photo-multiplier tubes (MCP-PMTs) by irradiating with single photons with/without a magnetic field. A time ...resolution of
σ=30–
35
ps
was obtained for single photons under
1.5
T
. With an MCP-PMT, a small time-of-flight counter, by means of Cherenkov light radiation instead of scintillation light has been prepared, and a time resolution
σ∼10
ps
was attained for a high-energy
π-beam by multiple photons.
We developed a simple back-calculation model that includes the age effect in which otolith size increases continuously during no-growth periods. To evaluate the validity of our proposed model, we ...back-calculated the past fish lengths and growth rates using the new model and seven traditional back-calculation models (scale-proportional hypothesis (SPH), body-proportional hypothesis (BPH), FraserLee, biological intercept, nonlinear SPH, nonlinear BPH, and modified Fry) using the otoliths of individually tag-recaptured white-spotted char (Salvelinus leucomaenis). The estimated fish lengths corresponded well to observed fish lengths for simple traditional (SPH, BPH, and FraserLee) and the new models. However, the back-calculated growth rates did not correspond to observed growth rates except for the new model. All previous models had considerable bias; growth rates of slow-growing fish were overestimated. As our model incorporating the age effect did not show such bias, this bias would be attributed to the age effect. Our proposed back-calculation model incorporating the age effect should be useful to estimate past growth rates at the individual level.
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