Quantitative Analysis of Endocardial and Epicardial Left Ventricular Myocardial Deformation in Patients with Cardiac Amyloidosis

Article信州医学雑誌 67(1): 49-62(2019)journal articl

Left ventricular deformation and torsion assessed by speckle-tracking echocardiography in patients with mutated transthyretin-associated cardiac amyloidosis and the effect of diflunisal on myocardial function

AbstractBackgroundMutated transthyretin-associated (ATTRm) amyloidosis with heart failure is associated with decreased longitudinal left ventricular (LV) myocardial contraction, as measured by strain Doppler echocardiography. We sought to clarify whether speckle-tracking echocardiography (STE) would provide useful information in patients with ATTRm cardiac amyloidosis.MethodsOne hundred twenty-three consecutive patients with ATTRm amyloidosis were divided into 3 groups. Group 1 had no evidence of cardiac involvement (n=47), group 2 had heart involvement but no congestive heart failure (CHF) and/or serum brain natriuretic peptide (BNP) levels <100pg/mL (n=35), and group 3 had heart involvement and CHF and/or serum BNP levels ≥100pg/mL (n=41). All patients underwent standard 2-dimensional (2D), Doppler echo, and STE.ResultsBy standard 2D and Doppler echo, differences in parameters were only apparent between group 3 and groups 1 and 2. Global circumferential strains by STE at each LV level and LV torsion were different between group 1 and groups 2 and 3, but not between group 2 and group 3. In contrast, global longitudinal LV strain showed significant intergroup differences (−17.3±2.3%, −13.3±2.3%, −9.9±3.3% for groups 1 to 3, respectively, P<0.0001). Radial strain also showed significant intergroup differences for each basal LV segment. Among 41 patients who could have been followed up after 1year, 34 patients with diflunisal treatment had shown improvement in apical rotation and torsion without deterioration in multidirectional strains.ConclusionATTRm cardiac amyloidosis is characterized by progressive impairment in longitudinal and basal LV radial function when global circumferential shortening and torsion remain unchanged

Heme-dependent autophosphorylation of a heme sensor kinase, ChrS, from Corynebacterium diphtheriae reconstituted in proteoliposomes

AbstractCorynebacterium diphteriae employs the response regulator, ChrA, and the sensor kinase, ChrS, of a two-component signal transduction system to utilize host heme iron. Although ChrS is predicted to encode a heme sensor, the sensing mechanism remains to be characterized. In this report, ChrS expressed in Eshcherichia coli membranes was solubilized and purified using decylmaltoside. ChrS protein incorporated into proteoliposomes catalyzed heme-dependent autophosphorylation by ATP. Other metalloporphyrins and iron did not stimulate kinase activity. The UV–Vis spectrum of hemin in the ChrS–proteoliposomes indicated that heme directly interacts with ChrS. This is the first functional reconstitution of a bacterial heme-sensing protein

Upper limb neuropathy such as carpal tunnel syndrome as an initial manifestation of ATTR Val30Met familial amyloid polyneuropathy

This is an electronic version of an article published in Amyloid 2010, Vol. 17, No. 1 : Pages 32-35. Amyloid is available online at: http://informahealthcare.com/doi/pdf/10.3109/13506121003619369We report here two patients with amyloidogenic transthyretin (ATTR) Val30Met familial amyloid polyneuropathy (FAP) who developed numbness in both hands and were diagnosed as having bilateral carpal tunnel syndrome (CTS). In both patients systemic TTR amyloidosis consisting of polyneuropathy affecting both upper and lower limbs and/or autonomic dysfunction gradually appeared after surgery for CTS. Although CTS associated with TTR amyloidosis has been known as an initial symptom in some patients with ATTR non-Val30Met FAP and those with senile systemic amyloidosis, this is the first report of ATTR Val30Met FAP patients starting with upper limb neuropathy including CTS-like symptoms. It is also notable that both patients had no genealogical relationship with two Japanese endemic foci of this disease.ArticleAMYLOID. 17(1):32-35 (2010)journal articl

Phase behavior and superprotonic conductivity in the Cs_(1-x)Rb_xH_2PO_4 and Cs_(1-x)K_xH_2PO_4 systems

The solid acid compound CsH_2PO_4 (CDP) adopts a cubic structure of high conductivity above 228 °C, rendering it attractive as a fuel cell electrolyte for intermediate temperature operation. This superprotonic phase is stable from the phase transition temperature, T_s, to the dehydration temperature, T_d, where the latter depends on water vapor pressure (e.g. T_d = 290 °C at p_(H_2O) = 0.8 atm). In this work we examine the possibility of modifying these temperatures and thereby, amongst other characteristics, fuel cell operating conditions by introduction of Rb and K as substituents for Cs in CDP. The phase behavior of the Cs_(1−x)Rb_xH_2PO_4 and Cs_(1−x)K_xH_2PO_4 pseudo-binary systems are determined by in situ X-ray diffraction (XRD) and thermal analysis. It is found that RbH_2PO_4 (RDP) and CDP are entirely miscible both below and above the transition to the cubic phase. With increasing Rb concentration, T_s increases and T_d decreases. In contrast, K has limited solubility in CDP, with a 27 at.% solubility limit in the cubic phase, and both T_s and T_d decrease with K content. The eutectoid temperature in the Cs_(1−x)K_xH_2PO_4 system is 208 ± 2 °C and the K solubility decreases sharply below this temperature. In both systems, conductivity decreases monotonically with increasing substituent concentration. Furthermore, even after normalization for cation size, the impact of K is greater than that of Rb, suggesting local disruptions to the proton migration pathway, beyond global changes in unit cell volume. Although this investigation shows unmodified CDP to remain the optimal fuel cell electrolyte material, the study provides a possible framework for elucidating proton transport mechanisms in superprotonic conductors

CD4/CD8 Double-negative Mycosis Fungoides: A Case Report and Literature Review

Mycosis fungoides, the most common subtype of cutaneous T-cell lymphoma, displays a broad spectrum of clinical, histological and phenotypic variants with different prognostic impacts. The classic immunophenotype is CD3+/CD4+/CD45RO+memory T cells. CD4/CD8 double-negative mycosis fungoides is rare. Here we describe the clinicopathological features of CD4/CD8 double-negative mycosis fungoides in a 55-year-old female with a review of the literature. Although the CD4/CD8 double-negative phenotype appears to be associated with an unusual clinical presentation, it does not appear to confer prognostic significance

Determination of organic carbon in coastal marine particles with a CHN analyzer : effects of inorganic carbon

無機炭素を含む海洋中粒子の有機炭素・窒素をCHN元素分析計で定量する場合には，予め酸処理により無機炭素（PIC）を除去する必要があるが，沿岸域試料で，酸処理なしでのCHN分析計の測定値を有機炭素と見做して報告している例が稀に見られる。そこで，瀬戸内海で採取された懸濁粒子，沈降粒子，および堆積物を用いて，酸処理の有無が粒状有機炭素（POC）の分析結果に及ぼす影響と，そこから派生する問題について検討した。一部の懸濁粒子や，沈降粒子および海底堆積物中の炭素の測定値は，酸処理なしの値が酸処理ありの値よりも顕著に高く，C/chl. a，C/N比なども過大評価されることが確認された。以上のことから，瀬戸内海を含む沿岸域試料でも，分析前の酸処理が必須であることが明らかとなった。Organic carbon and nitrogen were generally determined with a CHN analyzer after removing carbonate by acidification of samples with liquid acid or HCl vapor. However, some workers have conducted the CHN measurements for coastal marine particles without the acidification and reported their analytical values as POC. Here, we compared the carbon values of the CHN measurements with or without acid treatment (HCl vapor) to suspended, settling particles and sediments in Seto Inland Sea. As a result, the carbon values of samples without acidification were significantly higher than those of untreated ones. Overall, our results indicate the acidification of samples is an indispensable procedure for determining POC in various marine particles

Hypotaurine is an Energy-Saving Hepatoprotective Compound against Ischemia-Reperfusion Injury of the Rat Liver

Metabolome analyses assisted by capillary electrophoresis-mass spectrometry (CE-MS) have allowed us to systematically grasp changes in small molecular metabolites under disease conditions. We applied CE-MS to mine out biomarkers in hepatic ischemia-reperfusion. Rat livers were exposed to ischemia by clamping of the portal inlet followed by reperfusion. Metabolomic profiling revealed that l contents of taurine in liver and plasma were significantly increased. Of interest is an elevation of hypotaurine, collectively suggesting significance of hypotaurine/taurine in post-ischemic responses. Considering the anti-oxidative capacity of hypotaurine, we examined if supplementation of the compound or its precursor amino acids could affect hepatocellular viability and contents of taurine in liver and plasma. Administration of hypotaurine, N-acetylcysteine or methionine upon reperfusion comparablly attenuated the post-ischemic hepatocellular injury but with different metabolomic profiling among groups: rats treated with methionine or N-acetylcysteine but not those treated with hypotaurine, exhibited significant elevation of hepatic lactate generation without notable recovery of the energy charge. Furthermore, the group treated with hypotaurine exhibited elevation of the plasma taurine, suggesting that the exogenously administered compound was utilized as an antioxidant. These results suggest that taurine serves as a surrogate marker for ischemia-reperfusion indicating effectiveness of hypotaurine as an energy-saving hepatoprotective amino acid