NA

Intense, Q-switched, neodynium laser pulses (1 -9 joules), were focused into a vacuum chamber containing 5 mil and .5 mil Mylar targets to investigate the absorption of laser radiation by the subsequent plasma produced. Absolute measurements of the incident and transmitted laser energies were obtained for various background pressures and incident beam intensities. The data show that the absorbed energy for both the 5 and .5 mil targets is linearly dependent on the input energy. At the same time, however, the percentage of energy absorbed is greater in the 5 mil target (78-90%) than in the .5 mil target (44-73%). Increased plasma temperatures both at higher input energies, and from the .5 mil targets may partially account for these observed results. Time resolved measurements of the incident and transmitted laser power pulses were also taken in order to determine the time dependent behavior of the absorption process. The results of these measurements substantiated that greater percentages of energy were absorbed by the 5 mil target. In addition, the measurements revealed a decrease, followed by an unexpected increase, in the transmitted power which would indicate that optimum absorption conditions exist relatively early during the expansion of the plasma.http://archive.org/details/absorptionoflase00kuniNANACaptain, United States ArmyApproved for public release; distribution is unlimited

The competitiveness of domestic rice production in East Africa: A domestic resource cost approach in Uganda

The rapid increase of rice imports in sub-Saharan Africa under the unstable situation in the world rice market during the 2000s has made it an important policy target for the countries in the region to increase self-sufficiency in rice in order to enhance food security. Whether domestic rice production can be competitive with imported rice is a serious question in East African countries that lie close, just across the Arabian Sea, to major rice exporting countries in South Asia. This study investigates the international competitiveness of domestic rice production in Uganda in terms of the domestic resource cost ratio. The results show that rainfed rice cultivation, which accounts for 95% of domestic rice production, does not have a comparative advantage with respect to rice imported from Pakistan, the largest supplier of imported rice to Uganda. However, the degree of non-competitiveness is not serious, and a high possibility exists for Uganda’s rainfed rice cultivation to become internationally competitive by improving yield levels by applying more modern inputs and enhancing labour productivity. Irrigated rice cultivation, though very limited in area, is competitive even under the present input-output structure when the cost of irrigation infrastructure is treated as a sunk cost. If the cost of installing irrigation infrastructure and its operation and maintenance is taken into account, the types of irrigation development that are economically feasible are not large-scale irrigation projects, but are small- and microscale projects for lowland rice cultivation and rain-water harvesting for upland rice cultivation

Transition state in the folding of α-lactalbumin probed by the 6-120 disulfide bond

The guanidine hydrochloride concentration dependence of the folding and unfolding rate constants of a derivative of α-lactalbumin, in which the 6-120 disulfide bond is selectively reduced and S-carboxymethylated, was measured and compared with that of disulfide-intact α-lactalbumin. The concentration dependence of the folding and unfolding rate constants was analyzed on the basis of the two alternative models, the intermediate-controlled folding model and the multiple-pathway folding model, that we had proposed previously. All of the data supported the multiple-pathway folding model. Therefore, the molten globule state that accumulates at an early stage of folding of α-lactalbumin is not an obligatory intermediate. The cleavage of the 6-120 disulfide bond resulted in acceleration of unfolding without changing the refolding rate, indicating that the loop closed by the 6-120 disulfide bond is unfolded in the transition state. It is theoretically shown that the chain entropy gain on removing the cross-link from a random coil chain with helical stretches can be comparable to that from an entirely random chain. Therefore, the present result is not inconsistent with the known structure in the molten globule intermediate. Based on this result and other knowledge obtained so far, the structure in the transition state of the folding reaction of α-lactalbumin is discussed

Low-frequency maternal novel MYH7 mosaicism mutation in recurrent fetal-onset severe left ventricular noncompaction: a case report

BackgroundLeft ventricular noncompaction (LVNC) is a rare inherited cardiomyopathy with a broad phenotypic spectrum. The genotype-phenotype correlations in fetal-onset LVNC have not yet been fully elucidated. In this report, we present the first case of severe fetal-onset LVNC caused by maternal low-frequency somatic mosaicism of the novel myosin heavy chain 7 (MYH7) mutation.Case presentationA 35-year-old pregnant Japanese woman, gravida 4, para 2, with no significant medical or family history of genetic disorders, presented to our hospital. In her previous pregnancy at 33 years of age, she delivered a male neonate at 30 weeks of gestation with cardiogenic hydrops fetalis. Fetal echocardiography confirmed LVNC prenatally. The neonate died shortly after birth. In the current pregnancy, she again delivered a male neonate with cardiogenic hydrops fetalis caused by LVNC at 32 weeks of gestation. The neonate died shortly after birth. Genetic screening of cardiac disorder-related genes by next-generation sequencing (NGS) was performed which revealed a novel heterozygous missense MYH7 variant, NM_000257.3: c.2729A > T, p.Lys910Ile. After targeted and deep sequencing by NGS, the same MYH7 variant (NM_000257.3: c.2729A > T, p.Lys910Ile) was detected in 6% of the variant allele fraction in the maternal sequence but not in the paternal sequence. The MYH7 variant was not detected by conventional direct sequencing (Sanger sequencing) in either parent.ConclusionsThis case demonstrates that maternal low-frequency somatic mosaicism of an MYH7 mutation can cause fetal-onset severe LVNC in the offspring. To differentiate hereditary MYH7 mutations from de novo MYH7 mutations, parental targeted and deep sequencing by NGS should be considered in addition to Sanger sequencing

Diagnostic Validity of Combining HTLV-1 Serology and Immunophenotyping in Adult T-Cell Leukemia

Adult T-cell leukemia (ATL) is heterogeneous and sometimes equivocal to other T-cell neoplasms. Detecting anti- HTLV-1 antibodies is significant for a first screening not only for HTLV-1 infection but also for the HTLV-1-related disorders of ATL and TSP/HAM. The purpose of the present study was to investigate the diagnostic validity of HTLV-1 serology in ATL. The serologic results by a gelatin particle agglutination (PA) assay were highly sensitive (100%) and specific (99.5%) for the results of polymerase chain reaction (PCR) assay in 666 healthy blood donors who live in an area endemic for the HTLV-1 virus. Of 7,536 hospitalized patients, 189 patients with ATL were serologically screened. There were 1,140 patients (15.2%) infected by chance with HTLV-1, showing specificity, sensitivity, positive predictive value (PV), and negative PV of 84.3%, 100%, 14.2%, and 100%, respectively. Since the low positive PV (14.2%) was useless, we tried combining the anti-HTLV-1 assay with the immunophenotyping necessary for the diagnosis of lymphoid neoplasms. This combination gave nearly 100% positive and negative PV, and could prove to be useful in diagnosing ATL with the probability of 98%, especially for epidemiologic studies

Late-onset spastic ataxia phenotype in a patient with a homozygous DDHD2 mutation

Autosomal recessive cerebellar ataxias and autosomal recessive hereditary spastic paraplegias (ARHSPs) are clinically and genetically heterogeneous neurological disorders. Herein we describe Japanese siblings with a midlife-onset, slowly progressive type of cerebellar ataxia and spastic paraplegia, without intellectual disability. Using whole exome sequencing, we identified a homozygous missense mutation in DDHD2, whose mutations were recently identified as the cause of early-onset ARHSP with intellectual disability. Brain MRI of the patient showed a thin corpus callosum. Cerebral proton magnetic resonance spectroscopy revealed an abnormal lipid peak in the basal ganglia, which has been reported as the hallmark of DDHD2-related ARHSP (SPG 54). The mutation caused a marked reduction of phospholipase A(1) activity, supporting that this mutation is the cause of SPG54. Our cases indicate that the possibility of SPG54 should also be considered when patients show a combination of adult-onset spastic ataxia and a thin corpus callosum. Magnetic resonance spectroscopy may be helpful in the differential diagnosis of patients with spastic ataxia phenotype.ArticleSCIENTIFIC REPORTS. 4:7132 (2014)journal articl

Severity and Progression Rate of Cerebellar Ataxia in 16q-linked Autosomal Dominant Cerebellar Ataxia (16q-ADCA) in the Endemic Nagano Area of Japan

16q22.1-linked autosomal dominant cerebellar ataxia (16q-ADCA) is a recently defined subtype of ADCA identified by a disease-specific C/T substitution in the 5' untranslated region of the puratrophin-1 gene. In Nagano, the central mountainous district of the main island of Japan, 16q-ADCA and spinocerebellar ataxia type 6 (SCA6) are the most and second most prevalent subtypes of ADCA, respectively. Both subtypes are classified into Harding's ADCA III, but little attention has been given to the differences in the severity and progression rate of cerebellar ataxia between 16q-ADCA and SCA6. We investigated the clinical severity and progression rate of cerebellar ataxia of 16q-ADCA patients using international cooperative ataxia rating scale and scale for the assessment and rating of ataxia and compared them with those of SCA6 patients. The age at onset was much higher in 16q-ADCA patients (60.1 +/- 9.8 years, n = 66) than in SCA6 patients (41.1 +/- 8.7 years, n = 35). Clinical features of 16q-ADCA were basically consistent with pure cerebellar ataxia, as well as in SCA6, but gaze-evoked nystagmus was observed less frequently in 16q-ADCA patients than in SCA6 patients. When compared at almost the same disease duration after onset, the severity of cerebellar ataxia was a little higher, and the progression rate seemed more rapid in 16q-ADCA patients than in SCA6 patients, but the differences were not significant.ArticleCEREBELLUM. 8(1):46-51 (2009)journal articl

Protocol for a Randomized, Crossover Trial : ISCHIA study

Objective: Intermittent-scanning continuous glucose monitoring (isCGM) is widely used in type 1 diabetes (T1D) patients; however, the education required to prevent hypoglycemia by using isCGM is not established. This study examines the combined effect of isCGM device usage and the education to reduce the time in hypoglycemia in comparison to conventional self-monitoring of blood glucose (SMBG). Methods: The Effect of Intermittent-Scanning Continuous Glucose Monitoring to Glycemic Control Including Hypoglycemia and Quality of Life of Patients with Type 1 Diabetes Mellitus Study (ISCHIA Study), a randomized, crossover trial, enrolls 104 T1D patients (age, 20-74 years) with T1D. Participants are randomized to use isCGM combined with structured education (Intervention period) or SMBG (Control period) for 84 days, followed by the other for a further 84 days. During the Intervention period, participants have access to the sensor glucose levels and trend arrow of the device. During the Control period, participants conduct SMBG at least three times a day, and retrospective CGM is used to record the blinded sensor glucose levels. The primary endpoint is the decrease of time in hypoglycemia ( < 70 mg/dL) per day (hour/day) during the Intervention period compared with the Control period. The secondary endpoints include other indices of glycemic control, glycoalbumin, accuracy of isCGM, diabetes-related quality of life (QOL), adherence, and cost-effectiveness. The study protocol has received Certified Review Board (CRB) approval from National Hospital Organization Osaka National Hospital (N2018002, February 14, 2019). This study is carried out in accordance with the Declaration of Helsinki and the Clinical Trials Act. The findings will be published in peer-reviewed journals. Conclusion: The ISCHIA study will contribute to the standardization of patient education regarding the prevention of hypoglycemia by using isCGM