Ocular amyloid involvement after liver transplantation for polyneuropathy.
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Biomedical subjects
Publications and source records attributed to Y Ando.
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We present high resolution angle resolved photoemission data of the bilayer superconductor Bi(2)Sr(2)CaCu(2)O(8+delta) (Bi2212) showing a clear doubling of the near E(F) bands. This splitting approaches zero along the (0,0)-->(pi,pi) nodal line and is not observed in single layer Bi(2)Sr(2)CuO(6+delta) (Bi2201), indicating that the splitting is due to the long sought after bilayer splitting effect. The splitting has a magnitude of approximately 75 meV near the middle of the zone, extrapolating to about 110 meV near the (pi,0) point. The existence of these two bands also helps to clear up the recent controversy concerning the topology of the Fermi surface.
Using a cDNA glass microarray, the expression of 1081 genes in immature and mature dendritic cells (DCs) of two different individuals has been studied. The upregulation of mRNA transcripts of genes encoding the transcription factor ZFM1, Mos proto-oncogene serine/threonine-protein kinase, B-cell-specific transcription factor, preB-cell growth stimulating factor, ets translocation variant 6, and epidermal growth-factor-like CRIPTO was for the first time detected in DCs. Using semiquantitative RT-PCR analysis the upregulation of the transforming growth factor-alpha, integrin alpha 6 and ZFM 1 transcription factor in mature DCs was confirmed in samples from four different individuals. On the other hand, the downregulation of renin-binding protein transcript was detected in mature DCs using a cDNA microarray. For the first time, the expression of renin-angiotensin system genes was evaluated during maturation of DCs in samples from four donors by semiquantitative RT-PCR. A possible role of the renin-angiotensin system in DCs is discussed.
BACKGROUND: Although the choroid6 plexus of the brain is one of the most important production sites of transthyretin (TTR), the metabolism of TTR secreted in cerebrospinal fluid (CSF) remains to be elucidated. METHODS: To perform qualitative analysis of variant TTR in CSF of patients who underwent a sequential liver transplantation using an explanted familial amyloidotic polyneuropathy (FAP) ATTR Val30 Met patient's liver, levels and forms of TTR of the two patients were analyzed by means of enzyme linked immunosorbent assay (ELISA) and matrix-assisted laser desorption/time-of-flight mass spectrometer (MALDI/TOF-MS), respectively. RESULTS: After the operation, variant TTR levels in serum increased, and in CSF, a significant peak of free form of ATTR Val30 Met was detected in the transplanted patients whose CSF had shown no variant TTR before the operation. CONCLUSIONS: These findings suggest that the variant TTR can cross-the blood-CSF barrier and migrate into CSF from blood circulation. Because leptomeningeal amyloidosis occurs in FAP ATTR Val30 Met as the progression of the disease, this information suggests that in addition to peripheral neuropathy, disorders of the central nervous system (CNS) should be given an attention in patients who underwent sequential liver transplantation using an explanted FAP ATTR Val30 Met patient's liver.
We present a study of the magnetic susceptibility chi in carefully detwinned La 2-xSr(x)CuO4 single crystals in the lightly doped region (x = 0-0.03), which demonstrates a remarkable in-plane anisotropy of the spin system. This anisotropy, chi(a)/chi(b), is found to persist after the long-range antiferromagnetic (AF) order is destroyed by hole doping, suggesting that doped holes break the AF order into domains in which the spin alignment is kept essentially intact. It turns out that the freezing of the spins taking place at low temperatures is also notably anisotropic, implying that the "spin-glass" feature is governed by the domain structure as well.
We describe the unusual case of a 51-year-old woman with spinocerebellar ataxia type 1 (SCA1) who showed choreiform movements in addition to cerebellar ataxia. To date, extrapyramidal signs including involuntary movements have been rarely reported in SCA1. Surface electromyogram in our patient revealed grouped discharges whose duration was longer than that of chorea observed in HD, indicating that the involuntary movements represented choreoathetosis rather than pure chorea. These choreiform movements have not been seen in non-hereditary spinocerebellar ataxia. Therefore, if "sporadic" cases of cerebellar ataxia show such movements, the possibility of genetic origin of the ataxia is high and a surveillance of various forms of hereditary spinocerebellar ataxia including SCA1 is required.
The emergence and the evolution of the metallic charge transport in the La2-xSrxCuO4 system from lightly to optimally doped samples (x = 0.01-0.17) are studied. We demonstrate that in high-quality single crystals the in-plane resistivity shows a metallic behavior for all values of x at moderate temperatures and that the hole mobility at 300 K changes only by a factor of 3 from x = 0.01 to 0.17, where its x dependence is found to be intriguingly similar to that of the inverse antiferromagnetic correlation length. We discuss an incoherent-metal picture and a charged-stripe scenario as candidates to account for these peculiar features.
We report the result of our accurate measurements of the a- and b-axis resistivity, Hall coefficient, and the a-axis thermopower in untwinned YBa(2)Cu(3)O(y) single crystals in a wide range of doping. It is found that both the a-axis resistivity and the Hall conductivity show anomalous dependences on the oxygen content y in the 60-K phase below the pseudogap temperature T(*). The complete data set enables us to narrow down the possible pictures of the 60-K phase, with which we discuss a peculiar role of the pseudogap in the charge transport.
Using scanning tunneling spectroscopy, we investigated the temperature dependence of the quasiparticle density of states of overdoped Bi(2)Sr(2)CuO(6+delta) between 275 mK and 82 K. Below T(c) = 10 K, the spectra show a gap with well-defined coherence peaks at +/-Delta(p) approximately 12 meV, which disappear at T(c). Above T(c), the spectra display a clear pseudogap of the same magnitude, gradually filling up and vanishing at T(*) approximately 68 K. The comparison with Bi(2)Sr(2)CaCu(2)O(8+delta) demonstrates that the pseudogap and the superconducting gap scale with each other, providing strong evidence that they have a common origin.
OBJECTIVE: To report a case of familial amyloid polyneuropathy homozygous for the amyloidogenic transthyretin (ATTR) Val30Met gene with motor-dominant sensorimotor polyneuropathy and unusual sural nerve pathological findings. METHODS: Mass spectrometry analysis and polymerase chain reaction-restricting fragment length polymorphism were performed. A right sural nerve biopsy specimen was obtained for histological investigation. SETTING: Academic medical center. RESULTS: A 56-year-old Japanese man living in a local town (Nakajima, Japan) in Ishikawa Prefecture, a nonendemic area of type I familial amyloidotic polyneuropathy, had vitreous amyloidosis, motor-dominant sensorimotor polyneuropathy, erectile dysfunction, and urinary incontinence. He had neither orthostatic hypotension nor indolent diarrhea. Restriction enzyme analysis with EcoT22 I of amplified DNA and mass spectrometry analysis revealed homozygosity for ATTR Val30Met. Of 8 family members, 5 were evaluated and found to be heterozygous for ATTR Val30Met; a family history found no relative with the similar neurologic disorders. The sural nerve biopsy specimen showed focal edema and an amyloid deposit in the subperineural tissue, associated with moderate loss of myelinated and unmyelinated fibers. CONCLUSIONS: In addition to the findings characteristic of homozygosity for ATTR Val30Met such as vitreous amyloidosis and relatively less autonomic involvements, this case had the unique findings of motor-dominant sensorimotor polyneuropathy and unusual sural nerve biopsy specimen results.
Ultrasonography has been used in the evaluation of enlarged lymph nodes in patients with malignant lymphoma, which comprises a variety of histopathological subtypes. We assessed ultrasonic findings of infiltration in cervical lymph nodes of 47 patients with previously untreated malignant lymphoma and correlated them with histopathological subtypes. Common findings among various histopathological subtypes were hypoechoic masses, spot and/or line echoes, involvement of multiple nodes, and posterior enhancement. In addition, each histopathological subtype showed characteristic findings: Hodgkin's disease had a contour with a lobular pattern (9/10 cases), septum-like echo (9/10 cases), and tubular structure (9/10 cases) in the internal echo, reflecting histopathologically fibrous connective tissue surrounding lymph nodes and small vessels. In B-cell lymphoma, there was characteristically a tendency for lymph node fusion (19/28 cases) and spot and/or line echoes (17/28 cases), reflecting histopathological replacement and destruction of the structure of lymph node and capsule by proliferation of lymphocytes. These findings were strengthened in diffuse large B-cell lymphoma. T-cell lymphoma was distinguished from other types of lymphoma by the presence of a hilum-like echo (6/9 cases), reflecting histopathologically prominent vascularization in lymph nodes accompanied by a dilated hilum area. In summary, this preliminary study suggests that ultrasonic findings of infiltration of cervical lymph nodes in major subtypes of malignant lymphoma could reflect characteristic histopathological structures, therefore providing information helpful for differentiating them.
AIM: To determine the patterns and factors affecting dental utilisation by adolescents in Sri Lanka. DESIGN: The information was collected by means of a self-administered questionnaire. SETTING: Ten randomly selected secondary schools. PARTICIPANTS: A total of 492 Year 11 students of which 257 were from urban and 235 were from rural schools. RESULTS: Sixty one per cent of the rural and 54% of the urban students had used dental services. There was a significant difference in the care providers and the types of treatment received at the previous visit between the urban and the rural students. A majority of students in both groups utilised dental services for symptomatic reasons. According to logistic regression analysis, those students who had received advice about oral health were 1.9 times more likely to have used dental services than those who had not received advice. Also, female students were 1.5 times more likely to have used dental services than males. CONCLUSIONS: Utilisation of dental services was low in adolescents and was mainly related to symptomatic reasons. Gender, perceived need and whether the adolescents had received information about oral health were significant predictors of utilisation of dental services by adolescents in Sri Lanka.
PURPOSE: It has been reported that an intra-abdominal pressure (IAP) above 15 mm Hg may cause oliguria and that an IAP above 25 mm Hg may cause anuria. Because Valsalva's maneuver yields an IAP exceeding 180 mm Hg, it is presumed to affect renal perfusion. We evaluated the ability of power Doppler sonography to depict the changes in renal blood flow during Valsalva's maneuver. METHODS: Seven healthy men aged 21-24 years and 1 50-year-old man with massive ascites participated in the study. With each healthy subject lying in a supine position, longitudinal power Doppler sonograms of the kidney were obtained and analyzed semiquantitatively during Valsalva's maneuver. Also, in the patient with massive ascites, power Doppler sonography was performed before and after paracentesis. RESULTS: Along with an increase in IAP, monitored as expiratory pressure during Valsalva's maneuver, power Doppler signals decreased as indicated by both visual impression and computer scores. In the patient with massive ascites, signal intensity increased after paracentesis. CONCLUSIONS: Our results demonstrated that an increase in IAP within the physiologic range affects renal perfusion and that power Doppler sonography depicts semiquantitatively the change in renal blood flow.
SMKT (salt-mediated killer toxin), a killer toxin produced by the halotolerant yeast, Pichia farinosa, kills yeasts of several genera, including Saccharomyces cerevisiae. To elucidate the killing mechanism of SMKT, we examined the interaction of SMKT with membranes using liposomes. Leakage of calcein from calcein-entrapped liposomes was observed in the presence of SMKT. Destruction of liposomes was observed by dark-field microscopy. Comparison of intact S. cerevisiae cells with SMKT-treated cells by dark-field microscopy indicated that the spherical cell membrane is disrupted by SMKT. Using sodium carbonate extraction, we obtained direct evidence for the first time that SMKT is associated with the membrane of sensitive cells. Our results indicate that SMKT kills sensitive S. cerevisiae by interacting with the yeast cell membrane.
BACKGROUND: The increasing use of mammographic screening has led to an increased detection of ductal carcinoma in situ (DCIS) of the breast. The detailed biological characteristics of DCIS and a new classification of DCIS based on these characteristics are needed. METHODS: Immunohistochemical studies were performed to assess the expression of c-erbB-2 (ErbB-2), estrogen receptor (ER), p53 and proliferative activity (Ki-67) in 65 patients with pure DCIS and 60 with invasive ductal carcinoma (IDC). We classified pure DCIS tumors using three classifications, the architectural, Nottingham, and Van Nuys classifications. RESULTS: ErbB-2, ER and p53 staining was positive in 34%, 66% and 21% of patients with DCIS, respectively, and 58%, 42% and 33% in patients with IDC, respectively. Ki-67 stained positively in 1.5% of patients with DCIS and 11.2% of patients with IDC. The comedo type showed a high rate of positive ErbB-2 and p53 staining. The cribriform and papillary types showed a high rate of positive ER staining. Under the Van Nuys classification, ErbB-2, p53 and Ki-67 expression were highest in the group with high nuclear grade and lowest in the group with non-high nuclear grade without necrosis. CONCLUSION: Although the biological markers of IDC tended to suggest aggressive behavior more so than those of DCIS, these differences were based on the histological sub-type, comedo or non-comedo. The Van Nuys classification best defined the subgroups of DCIS with a distinct expression pattern of biological markers, and the best candidates for breast-conserving surgery.
We report a 59-year-old woman with human T-cell lymphotrophic virus type-I (HTLV-I) associated myelopathy/tropical spastic paraparesis who showed high signal in the cervical and thoracic spinal cord on T2-weighted and contrast enhancement on T1-weighted images.
PURPOSE: To determine the maximum tolerated dose (MTD) of irinotecan combined with carboplatin, to evaluate its efficacy and toxicity for patients with lung cancer, and to examine its pharmacokinetics and pharmacodynamics. METHODS: The dose of irinotecan was escalated from 40 mg/m2 per week in increments of 10 mg/m2. Carboplatin was fixed at 300 mg/m2. Multivariate regression models with an interaction term were used to evaluate synergistic pharmacodynamic interactions. RESULTS: The MTD and recommended dose of irinotecan were 60 and 50 mg/m2, respectively. Dose-limiting toxicities were grade 4 neutropenia and grade 3 or 4 diarrhea. In phase II studies, response rates were 81.3% (95% confidence interval 61.8-100%) in 16 patients with small-cell lung cancer and 22.2% (2.7-41.8%) in 18 patients with non-small-cell lung cancer. Two patients (6%) experienced grade 4 neutropenia, thrombocytopenia, and grade 3 diarrhea. The area under the plasma concentration versus time curve (AUC) of carboplatin ranged from 2.87 to 9.31 mg x min/ml, with a median of 4.66 mg x min/ml. In pharmacodynamic analyses, the log-transformed surviving fraction in platelet count (SFp) showed a significant association with the AUC of carboplatin (P=0.010), while that in neutrophil count (SFn) was not significantly correlated with any pharmacokinetic parameter. The interaction term was not significant in either case. CONCLUSIONS: These results indicate that AUC-based dosing of carboplatin is still rational in combination chemotherapy. A more sensitive method for predicting life-threatening toxicities is needed, however, because traditional pharmacokinetic parameters were not adequate tools for identifying patients at high risk of severe neutropenia and diarrhea. This combination regimen has only modest activity, and further studies are necessary to evaluate a different dose schedule.
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