Effect of the Surrounding Magnetic Environment of Temporal Bone on the Fluid Signal Intensity in Human Inner Ear Using a Combined T2 Preparation Pulse and Fluid Attenuated Inversion Pulse Technique.

Purpose: Recently, the utility of non-contrast MR endolymphatic hydrops imaging was reported, but the pitfall was indicated based on T2 preparation pulse sensitiveness to local static magnetic field (B0) inhomogeneity. The purpose of this study is to clarify the effects of surrounding magnetic environment of temporal bone to lymphatic fluid signal intensity on the T2 preparation and fluid attenuated inversion recovery pulse combination (T2prep 3D-FLAIR) technique in human inner ear study.

Methods: We prepared a custom-made phantom comprising a chicken leg bone submersed in saline.

Effects of Deep Learning Reconstruction Technique in High-Resolution Non-contrast Magnetic Resonance Coronary Angiography at a 3-Tesla Machine.

Purpose: To evaluate the effects of deep learning reconstruction (DLR) in qualitative and quantitative image quality of non-contrast magnetic resonance coronary angiography (MRCA).

Methods: Ten healthy volunteers underwent conventional MRCA (C-MRCA) and high-resolution (HR) MRCA on a 3T magnetic resonance imaging with a voxel size of 1.8 × 1.

[Postoperative cholangitis of balloon-occluded transarterial chemoembolization (B-TACE) for hepatocellular carcinoma].

In 35 patients who underwent balloon-occluded transarterial chemoembolization (B-TACE) for hepatocellular carcinoma (HCC) since January 2013, 5 patients (14%) had postoperative cholangitis, 1 of whom required drainage of a liver abscess. Four of these patients(80%)were treated with cisplatin (CDDP)-epirubicin (EPI)-Lipiodol (Lp) emulsion, and 1 was treated with EPI-Lp emulsion.The balloon was located and inflated at the lobar level (C: conventional)in 3 patients (60%) and at the subsegmental or more distal level (SS: superselective) in 2 patients (40%).

Unusual biliary myoepithelial carcinoma in liver-case report and immunohistochemical study.

Myoepithelial carcinoma is a well-known tumor of salivary gland, representing 1% of all salivary gland tumors. They have also been reported in other sites as skin/soft tissue, breast and lung. This paper reports a rare case of primary myoepithelial carcinoma in the liver, as well as discusses the findings of immunohistochemistry.

Prevalence of subclinical coronary artery disease in ischemic stroke patients.

Background: Recently, ischemic stroke has emerged as a new coronary artery disease (CAD) risk equivalent. Our purpose is to study the prevalence of CAD in ischemic stroke patients compared with that in non-stroke patients.

Methods And Results: We measured coronary calcium score (CCS) in 151 ischemic stroke patients without known CAD (stroke group) and compared it with 151 age- and sex-matched non-stroke patients (control group).

Randomized trial of peginterferon α-2a plus ribavirin versus peginterferon α-2b plus ribavirin for chronic hepatitis C in Japanese patients.

Background: Pegylated interferon (PegIFN) plus ribavirin is the standard therapy for patients with chronic hepatitis C genotype 1. Although several randomized clinical trials have compared PegIFNα-2a with PegIFNα-2b, these 2 regimens have not been directly compared in Asian patients. We, therefore, compared the safety and antiviral efficacy of these agents in Japanese patients.

Safety and short-term therapeutic effects of miriplatin-lipiodol suspension in transarterial chemoembolization (TACE) for hepatocellular carcinoma.

Aim: To determine the safety and usefulness of a novel anticancer drug, miriplatin, in transarterial chemoembolization (TACE) for unresectable hepatocellular carcinoma.

Patients And Methods: Patients (n=115) who underwent TACE with miriplatin-lipiodol suspension (miriplatin group), and control patients (n=131) who underwent TACE with cisplatin-lipiodol suspension (CDDP group) took part in this study.

Results: The overall incidence of adverse events was significantly lower in the miriplatin group.

Fragmented QRS is associated with torsades de pointes in patients with acquired long QT syndrome.

Background: Acquired long QT syndrome (LQTS) is a disease due to a secondary repolarization abnormality induced by various predisposing factors. In contrast to congenital LQTS, risk factors that produce acquired LQTS include organic heart diseases that often exhibit depolarization abnormality. Although various repolarization parameters have been evaluated in acquired LQTS, the existence of depolarization abnormality in association with torsades de pointes (TdP) has not been reported.

Severe congestive heart failure with cardiac liver cirrhosis 10 years after orthotopic liver transplantation for familial amyloidotic polyneuropathy.

Reported herein is an autopsy case of familial amyloidotic polyneuropathy (FAP) with cardiac liver cirrhosis associated with amyloid cardiomyopathy after liver transplantation. At 47 years of age a Japanese woman with a transthyretin Val30Met mutation and sensorimotor polyneuropathy underwent liver transplantation; no postoperative deterioration related to the graft or polyneuropathy occurred. However, cardiovascular dysfunction associated with amyloid deposition gradually worsened.

Aging and transthyretin-related amyloidosis: pathologic examinations in pulmonary amyloidosis.

Although aging is closely related with the onset of senile systemic amyloidosis (SSA) caused by wild-type transthyretin (TTR), the effect of aging on amyloid formation has remained unclear in familial amyloidotic polyneuropathy (FAP), caused by variant- and wild-type TTR. The aim of this study was to elucidate the effects of aging and/or other factors in FAP on amyloid formation in the lung, one of the most important target organs of amyloid deposition in SSA. Pulmonary amyloid distribution was determined using 19 autopsied lung samples from patients with FAP amyloidogenic TTR (ATTR) V30M, the most common type of FAP.

Role of extracellular superoxide dismutase in patients under maintenance hemodialysis.

Background: It has been well documented that free radical injury is involved in the progression of chronic renal failure. Extracellular superoxide dismutase (EC-SOD), localized on the endothelial cell surface, plays an important role in reducing oxidative stress especially in the vessels by binding to the endothelial cell surface via the heparin-binding domain. Although EC-SOD Arg213Gly, which cannot bind on endothelial cells, has been considered a polymorphism, the effect of EC-SOD on hemodialysis patients has not been well examined.

Studies on unfolded beta-microglobulin at C-terminal in dialysis-related amyloidosis.

Background: In 1997, Stoppini et al reported that monoclonal antibody specific to the C-terminal 92-99 of beta(2)-microglobulin (beta(2)m) had been capable of inhibiting fibrillogenesis of beta(2)m in vitro. Meanwhile, recent studies have indicated that an acidifying procedure can unfold conformation of the precursor protein, leading to fibril formation of beta(2)m as well as a transthyretin.

Methods: We thus prepared monoclonal antibody specific to the C-terminal 92-99 (mAb 92-99), and investigated its reactivity in plasma ultrafiltrate and amyloid tissues from 18 hemodialysis patients with dialysis-related amyloidosis (DRA).

Muscular amyloid angiopathy with amyloidgenic transthyretin Ser50Ile and Tyr114Cys.

Among patients with familial amyloid polyneuropathy (FAP), those with transthyretin Val30Met mainly show distally predominant weakness and atrophy, whereas some FAP patients, including those with transthyretin Ser50Ile and Tyr114Cys, show muscle weakness and atrophy that is dominant proximally, simulating myopathy. To clarify the cause of proximally dominant muscular atrophy in patients with FAP transthyretin Ser50Ile and Tyr114Cys, we investigated the distinctive features of muscle specimens of patients with FAP, 3 of who had Val30Met, 2 Ser50Ile, and 2 Tyr114Cys transthyretin. All specimens showed transthyretin amyloid around blood vessels and perimysium, and neurogenic denervation patterns.

Near-infrared spectrophotoscopy of finger venules in assessment of autonomic dysfunction.

The authors evaluated morphologic changes in the venules of the finger using near-infrared spectrophotoscopy in patients with autonomic dysfunction, such as familial amyloidotic polyneuropathy and multiple-system atrophy. Abnormalities of the venules, such as tortuosity, irregular venous caliber, and microaneurysm-like change, and a linear negative correlation between the degree of orthostatic hypotension and the degree of vasoconstriction of the venules were observed.

[New molecular marker in dialysis setting--misfolded beta 2-microglobulin].

More than 25 clinical settings in amyloidosis have been acknowledged in which a peculiar criminal protein, a precursor protein, has been identified. As of now, however, the mechanism of amyloidogenesis, by which a precursor protein is transformed irreversibly into an amyloid protein, remains to be clarified. We speculated that a study of the molecular conformation of beta 2-microglobulin (beta 2 m), a precursor protein in dialysis-related amyloidosis (DRA), might provide a typic model of amyloidogenesis in other precursor proteins.

Inhibitory effect of α-tocopherol on methylmercury-induced oxidative steress.

Objectives: The present study investigated the involvement of oxidative stress in the degeneration of the cerebellum during methylmercury (MeHg) intoxication and the protective effect of α-tocopherol (Vit E) against MeHg toxicity.

Methods: After 5 mg/kg of MeHg was administered to Wistar rats for 12 consecutive days, the cerebellum were examined histopathologically. In addition, the same amount of MeHg was administered to 3 different groups of Wistar rats: rats with a Vit E-deficient diet, rats fed 150 mg/kg of Vit E for 20 consecutive days after initial MeHg administration, and rats with an ordinary diet.

A different amyloid formation mechanism: de novo oculoleptomeningeal amyloid deposits after liver transplantation.

Background: Liver transplantation has served as a treatment for patients with familial amyloidotic polyneuropathy (FAP) because variant transthyretin (TTR), the pathogenic protein of FAP, is predominantly produced by the liver. However, the effect on amyloid formation of TTR that is synthesised by the retina and the choroid plexus remains to be elucidated in FAP patients with liver transplants.

Objective: To investigate changes in ocular tissues and the central nervous system (CNS) of FAP patients after liver transplantation.

Targeted conversion of the transthyretin gene in vitro and in vivo.

Familial amyloidotic polyneuropathy (FAP) is the common form of hereditary generalized amyloidosis and is characterized by the accumulation of amyloid fibrils in the peripheral nerves and other organs. Liver transplantation has been utilized as a therapy for FAP, because the variant transthyretin (TTR) is predominantly synthesized by the liver, but this therapy is associated with several problems. Thus, we need to develop a new treatment that prevents the production of the variant TTR in the liver.

A novel tool for detecting amyloid deposits in systemic amyloidosis in vitro and in vivo.

We synthesized (trans,trans)-1-bromo-2,5-bis-(3-hydroxycarbonyl-4-hydroxy)styrylbenzene (BSB) and used this compound to detect amyloid fibrils in autopsy and biopsy samples from patients with localized amyloidosis, such as familial prion disease, and systemic amyloidosis, such as familial amyloidotic polyneuropathy, amyloid A (AA) amyloidosis, light chain (AL) amyloidosis, and dialysis-related amyloidosis. BSB showed reactions in all Congo red-positive and immunoreactive regions of the samples examined in the study, and some amyloid fibrils in the tissues could be detected more precisely with BSB than with the other methods. In the mouse model of AA amyloidosis, injected BSB reacted with amyloid in all regions in the serial sections in which Congo red staining was positive.

Circulating level of alpha2-macroglobulin-beta2-microglobulin complex in hemodialysis patients.

Background: The presence of alpha2-macroglobulin (alpha2M) in amyloid tissue from patients with dialysis-related amyloidosis (DRA) was demonstrated by Argilés et al in 1989. Thereafter, the formation of the complex of beta2-microglobulin (beta2m) with alpha2m was confirmed directly by in vitro study. In Alzheimer's disease, complex formation of amyloid beta-peptide and alpha2M is considered to play an important role in the pathogenesis by modifying the degradation processes of amyloid protein.

Role of VLDL/chylomicron in amyloid formation in familial amyloidotic polyneuropathy.

We examined the affinity of transthyretin (TTR) for lipoproteins and the effect of lipoproteins on TTR-related amyloidogenesis using serum samples from healthy volunteers and patients with familial amyloidotic polyneuropathy (FAP) ATTRVal30Met. In both volunteers and patients, TTR levels were highest in the VLDL fraction containing chylomicrons (VLDL/CM) and next highest in the HDL fraction. Levels were lowest in the LDL fraction.

Presence of variant transthyretin in aqueous humor of a patient with familial amyloidotic polyneuropathy after liver transplantation.

To determine the origin of transthyretin (TTR) in the aqueous humor of patients with familial amyloidotic polyneuropathy (FAP), we measured TTR levels and analyzed the TTR forms in the aqueous humor of three FAP patients (one patient; liver transplanted, and two patients; non-transplanted). The total TTR levels were almost the same as reported previously in non-transplanted patients and slightly increased in a transplanted patient. Analyses with mass spectrometry in the two non-transplanted FAP ATTR V30M patients revealed that both wild type and variant TTR forms were detected in their aqueous humor samples.