Hereditary transthyretin amyloidosis with hydrocephalus at 27 years old: A case report.

Unlabelled: Hereditary transthyretin amyloidosis is autosomal dominant and results from mutations in the transthyretin gene. The Val30Met variant is the most common genetic mutation, although mutations vary within populations. More than 150 mutations in transthyretin have been reported; however, the Leu111Glu (p.

Clinical utility of the BIWACO score for patients with atrial fibrillation after percutaneous coronary intervention.

No predictive clinical risk scores for net adverse clinical events (NACE) have been developed for patients with atrial fibrillation (AF) after percutaneous coronary intervention (PCI). We evaluated NACE to develop clinically applicable risk-stratification scores in the Bleeding and thrombotic risk evaluation In patients With Atrial fibrillation under COronary intervention (BIWACO) study, a multicenter survey which has enrolled a total of 7837 patients. We also investigated the current status and time trends for the use of antithrombotic drugs.

Diverse phenotypic expression associated with the same genetic variant in female heterozygote patients of Anderson-Fabry disease: a case series.

Background: Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder resulting from a mutation of alpha-galactosidase A gene (), causing deficiency in alpha-galactosidase activity. The enzyme deficit can lead to storage of globotriaosylceramide in various organs including heart. Studies suggest that vasospastic angina (VSA) is associated with AFD.

Ventricular fibrillation associated with vasospastic angina pectoris in Fabry disease: a case report.

Background: Fabry disease (FD) is an X-linked lysosomal storage disorder resulting from a deficiency in alpha-galactosidase A. The major causes of death due to cardiac complications include life-threatening arrhythmias. In addition, life-threatening arrhythmias may be related to myocardial fibrosis assessed by late gadolinium enhancement (LGE).

Effects of Tween 80 on cellulase stability under agitated conditions.

The mechanism of the increase in the hydrolysis rate and yield by the addition of Tween 80 to the hydrolysis reaction of filter paper was investigated under static and agitated conditions. The increase in the hydrolysis rate by addition of Tween 80 was observed under the agitated condition only. The effects of Tween 80 on the changes in the protein concentration of individual cellulase components were investigated in the absence of substrates.

Characteristics of enzyme hydrolysis of cellulose under static condition.

The effect of enzyme loading under static and agitated conditions was investigated. Enzymatic hydrolysis of 10 w/v% de-lignified cellulose slurry such as filter paper, avicel and pulp was conducted under agitated (120 rpm) and static condition, and the enzyme loading ranging from 1.2 to 120 mg-protein/g-dry substrate.

Associations of D-dimer and von Willebrand factor with atherosclerosis in Japanese and white men.

Objective: D-dimer and von Willebrand factor (vWF) are associated with atherosclerosis. We recently reported that in a post-World War II birth cohort, Japanese men in Japan had lower levels of atherosclerosis than white men in the United States (U.S.

Intima-media thickness of the carotid artery and the distribution of lipoprotein subclasses in men aged 40 to 49 years between whites in the United States and the Japanese in Japan for the ERA JUMP study.

In men in the post-World War II birth cohort, that is, men aged 40 to 49 years, whites in the United States had significantly higher levels of intima-media thickness of the carotid arteries (IMT) than the Japanese in Japan (Electron-Beam Tomography and Risk Assessment Among Japanese and US Men in the Post World War II Birth Cohort [ERA JUMP] study). The difference remained after adjusting for traditional risk factors. Primary genetic effects are unlikely, given the degree to which IMT is increased in the Japanese who migrated to the United States.

Difference in carotid intima-media thickness between Korean and Japanese men.

Purpose: Koreans are recently at higher risk for coronary heart disease than are the Japanese. We aimed to evaluate levels of carotid intima-media thickness (IMT) and coronary risk factors in Korean and Japanese men in the post-World War II birth cohort.

Methods: We conducted a population-based study of 352 randomly selected healthy men 40 to 49 years of age: 102 Koreans in Ansan City, Gyeonggi-do, Korea and 250 Japanese in Kusatsu City, Shiga, Japan.

Fish consumption and early atherosclerosis in middle-aged men.

To investigate the association between fish consumption and early atherosclerosis, we analyzed the relationship between fish consumption and average intima-media thickness (AveIMT) by carotid ultrasound in middle-aged Japanese men. Participants were 250 randomly selected, community-based Japanese men aged 40 to 49 years without a prior history of cardiovascular disease. AveIMT was calculated from the mean of 1-cm lengths of both the right and the left carotid arteries at 8 locations.

Much lower prevalence of coronary calcium detected by electron-beam computed tomography among men aged 40-49 in Japan than in the US, despite a less favorable profile of major risk factors.

Background: Since World War II (WWII), exposures to westernized lifestyle have occurred in many non-Western countries, including Japan. National surveys showed that risk factor profiles for atherosclerosis around 1990 were similar in men in the post WWII birth cohorts in the US and Japan. We compared the degree of coronary calcium and other factors in men in the post WWII birth cohort: men aged 40-49 in the US and Japan.

C-S bond formation reaction between a phenolate and disulfide-bridged dicopper(I) complexes.

A novel C-S bond formation reaction took place, when a lithium phenolate derivative was treated with a disulfide-bridged dicopper(I) complex or a bis(micro-thiolato)dicopper(II) complex under very mild conditions. The reaction has been suggested to proceed via a disulfide-bridged (micro-phenoxo)dicopper(I) complex as the common reaction intermediate. Copper(II) complexes of the modified ligands containing a thioether group (products of the C-S bond formation reaction) have been isolated and structurally characterized by X-ray analysis as model compounds of the active site of galactose oxidase.

Structures and redox reactivities of copper complexes of (2-pyridyl)alkylamine ligands. Effects of the alkyl linker chain length.

Ligand effects on the structures and redox reactivities of copper complexes have been examined using (2-pyridyl)alkylamine derivatives as the supporting ligands, where particular attention has been focused on the effects of the alkyl linker chain length connecting the tertiary amine nitrogen atom and the pyridine nucleus: N[bond]CH(2)[bond]Py (Pym) vs N[bond]CH(2)CH(2)[bond]Py (Pye). X-ray crystallographic analysis of the copper(I) complex of tridentate ligand (Phe)L(Pym2) [N,N-di(2-pyridylmethyl)-2-phenylethylamine] (complex 1) has demonstrated that it possesses a trigonal pyramidal geometry in which a d[bond]pi interaction with an eta(1)-binding mode exists between the metal ion and one of the ortho carbons of the phenyl ring of the ligand side arm (phenethyl). The result shows sharp contrast to the d[bond]pi interaction with an eta(2)-binding mode existing in the copper(I) complex of (Phe)L(Pye2) [N,N-di[2-(2-pyridyl)ethyl]-2-phenethylamine] (complex 2).

Noninvasive assessment of significant right coronary artery stenosis based on coronary flow velocity reserve in the right coronary artery by transthoracic Doppler echocardiography.

Background: Coronary flow velocity reserve (CFVR) measured by transthoracic Doppler echocardiography (TTDE) has been reported to be useful for the noninvasive assessment of coronary stenosis in the left anterior descending artery. However, the measurement of CFVR in the right coronary artery by TTDE has not yet been validated in a clinical study.

Objective: The aim of this study was to evaluate whether CFVR by TTDE can detect significant stenosis in the right coronary artery.

Interconversion between bis(mu-thiolato)dicopper(II) and disulfide-bridged dicopper(I) complexes mediated by chloride ion.

An unprecedented clean interconversion between a novel bis(mu-thiolato)dicopper(II) complex (1) and a disulfide-bridged dicopper(I) complex (3) through a disulfide-bridged (mu-chloro)dicopper(I) complex (2) mediated by chloride ion has been established for the first time, providing a new redox chemistry of the transition metal-sulfur complexes. Crystal structures of 1 and 3 as well as spectroscopic features of the compounds are reported.

An early predictor of left ventricular remodeling after reperfused anterior acute myocardial infarction: ratio of peak E wave velocity/flow propagation velocity and mitral E wave deceleration time.

Several studies have demonstrated that the ratio of peak E wave velocity/flow propagation velocity (E/FPV) using color M-mode Doppler echocardiography and the mitral E wave deceleration time make it possible to estimate left ventricular filling pressure. Recent studies have indicated that deceleration time can predict left ventricular dilation after acute myocardial infarction. The purpose of this study was to determine whether the early assessment of deceleration time and E/FPV could predict left ventricular dilation after acute myocardial infarction.

Noninvasive assessment of coronary flow velocity and coronary flow velocity reserve in the right coronary artery by transthoracic Doppler echocardiography: comparison with intracoronary Doppler guidewire.

The aim of this study was to evaluate whether coronary flow velocity (CFV) and coronary flow velocity reserve (CFVR) in the posterior descending right coronary artery can be reliably measured by transthoracic Doppler echocardiography (TTDE). In 17 patients, CFV in the posterior descending right coronary artery was measured with TTDE at the time of Doppler guidewire examination. CFV was measured by both methods at baseline and under hyperemic conditions.

Genetic variation in aldehyde dehydrogenase 2 and the effect of alcohol consumption on cholesterol levels.

Unlabelled: Moderate drinkers with a defective alcohol dehydrogenase type 3 (ADH3) genotype have higher high-density lipoprotein (HDL) levels and a decreased risk of coronary artery disease (CAD). We examined the interaction between the aldehyde dehydrogenase type 2 (ALDH2), alcohol intake, and HDL levels in 826 men and 1295 women in a rural town in Japan. The ALDH2 genotype of each subject was determined by polymerase chain reaction (PCR) analysis.

Noninvasive estimation of pulmonary capillary wedge pressure by color M-mode Doppler echocardiography in patients with acute myocardial infarction.

The peak early diastolic filling velocity/flow propagation velocity (E/FPV) by color M-mode Doppler provides a better estimate of pulmonary capillary wedge pressure (PCWP) than transmitral or pulmonary venous flow. However, the value of E/FPV for the assessment of PCWP has not been evaluated in patients with acute myocardial infarction. We investigated the correlation between E/FPV and PCWP and whether PCWP can be estimated from E/FPV in patients with acute myocardial infarction.

Evaluation of left ventricular systolic and diastolic global function: peak positive and negative myocardial velocity gradients in M-mode Doppler tissue imaging.

Objectives: To evaluate a new indicator of left ventricular global function: Myocardial velocity gradient (MVG) M-mode Doppler tissue imaging (DTI).

Background: MVG is a new indicator of regional left ventricular function and global left ventricular diastolic function. However, it is unclear whether MVG also is an indicator of left ventricular global function in comparison with invasive indices.