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Biomedical subjects

N Miyamoto

Publications and source records attributed to N Miyamoto.

At least 73 records · Page 4Linked to original sources
PubMed1999

Familial paroxysmal dystonic choreoathetosis: clinical findings in a large Japanese family and genetic linkage to 2q.

BACKGROUND: Paroxysmal dystonic choreoathetosis (PDC) is a rare familial movement disorder that has been mapped to chromosome 2q31-36. OBJECTIVE: To study the first Japanese family with PDC clinically and genetically. PATIENTS AND METHODS: We studied a large Japanese family in which at least 17 members in 6 generations have been affected by PDC. We interviewed and examined 26 family members, 8 of whom revealed choreoathetosis-like and dystonialike involuntary movement and 1 of whom revealed no involuntary movement but only muscle stiffness such as the aura of paroxysmal dystonic choreoathetosis (PDC). Genetic linkage studies of this family were carried out with polymorphic DNA markers. RESULTS: The attacks of involuntary movement or muscle stiffness were precipitated by ovulation, menstruation, emotional stress, or caffeine or alcohol ingestion. Magnetic resonance imaging of the brain revealed no abnormalities. Clonazepam therapy was effective for reducing the attacks, and ingestion of garlic was believed by patients to be effective for softening the attacks. An affected woman with only muscle stiffness showed remission after hysterectomy for hysteromyoma. This woman also had the disease haplotype and transferred it to her typical PDC-affected daughter. Maximal pairwise logarithm of odds scores exceeding 2.00 were obtained at D2S2250, D2S1242, D2S377, D2S2148, and D2S126. The PDC gene was demonstrated by linkage analyses to be located in a 15.3-centimorgan interval lying between D2S371 and D2S339 based on pairwise and multipoint logarithm of odds scores and obligate recombination events in affected individuals. CONCLUSIONS: Linkage of PDC to chromosome 2q32-36 was confirmed in a Japanese family. The clinical characterizations of this family with PDC include that ovulation seems also to be a precipitating factor of the attacks and that hysterectomy seems to be effective for softening the attacks. Although low-dose clonazepam treatment was most effective, garlic use was believed by affected members to be effective for softening the attacks. Furthermore, based on the results of clinical and genetic analyses, we suggest that muscle stiffness without involuntary movement may represent a forme fruste of PDC.

Athetosis↗
PubMed1999

Gene expression during osteoclast-like cell formation induced by antifusion regulatory protein-1/CD98/4F2 monoclonal antibodies (MAbs): c-src is selectively induced by anti-FRP-1 MAb.

Human blood monocytes can differentiate into osteoclast-like cells when they are cultured in the presence of anti-FRP-1. Messenger (mRNA) expression of markers related to osteoclasts was analyzed during differentiation of osteoclasts from monocytes. As markers related to osteoclasts, we selected cathepsin-K, carbonic anhydrase (CA) II, vacuolar H(+)-ATPase (v-ATPase), vitronectin receptor (VNR), tartrate-resistant acid phosphatase (TRAP), osteopontin (OPN), galectin-3, c-src, c-fos, and c-fms. The mRNAs other than c-src mRNA were expressed in freshly isolated monocytes or monocytes incubated with control antibody or anti-FRP-1 monoclonal antibody (MAb) for 14 days. Of these mRNAs, cathepsin-K, CA II, v-ATPase, VNR, TRAP, OPN, and c-fms mRNAs were expressed at higher levels in the osteoclast-like cells than those in monocytes cultured with control antibody. On the other hand, galectin-3 mRNA was expressed at lower levels in the osteoclast-like cells, and there was no significant difference in c-fos mRNA expression between the monocytes cultured with control antibody and anti-FRP-1 MAb. c-src mRNA could not be detected in monocytes freshly isolated or incubated with control antibody. Surprisingly, expression of c-src mRNA was induced in monocytes by anti-FRP-1 MAb and was detectable as early as 3 h after anti-FRP-1 MAb treatment, indicating that c-src is selectively induced by anti-FRP-1 MAb treatment. Furthermore, the osteoclast-like cells expressed calcitonin receptor. Receptor activator of NF-kappaB (RANK) mRNA was detectable in freshly isolated monocytes or monocytes cultured with control antibody or anti-FRP-1 MAbs. Maximal expression of RANK was observed in osteoclast-like cells. On the other hand, no receptor activator of NF-KB ligand (RANKL) mRNA was detectable in any of the samples, suggesting that anti-FRP-1 mAb can induce osteoclast-like cells from blood monocytes without RANKL.

Antibodies, Monoclonal↗
PubMed1999

The KOR-SA3544 antigen predominantly expressed on the surface of Philadelphia chromosome-positive acute lymphoblastic leukemia cells is nonspecific cross-reacting antigen-50/90 (CD66c) and invariably expressed in cytoplasm of human leukemia cells.

We previously reported a novel monoclonal antibody KOR-SA3544 which predominantly reacted with a surface antigen (sSA3544) expressed on Philadelphia chromosome (Ph1)-positive acute lymphoblastic leukemia (ALL). In the present study, we demonstrate that the antibody specifically recognized nonspecific cross-reacting antigen (NCA)-50/90 (CD66c), one of the carcinoembryonic antigen (CEA)-related glycoproteins encoded by a member of the CEA gene family. In addition, we show that the SA3544 antigen (NCA-50/90) was invariably expressed in cytoplasm of all of the human leukemic cell lines examined (sSA3544-positive B-lymphoid two, sSA3544-negative T or B-lymphoid and non-lymphoid 24) regardless of the presence or absence of surface expression of this antigen. Immunoelectromicroscopic examination revealed that the cytoplasmic antigen was mainly present in granules in sSA3544-positive leukemia cells, whereas it was diffusely present in cytosol in sSA3544-negative leukemia cells. Thus, among members of the CEA family, NCA-50/90 was first demonstrated to be expressed not only on the surface of some leukemia cells, but also in cytoplasm of various types of leukemia cells.

Antibodies, Monoclonal↗
PubMed1999

p16/MTS1/INK4A gene is frequently inactivated by hypermethylation in childhood acute lymphoblastic leukemia with 11q23 translocation.

The p16 gene encoding a specific inhibitor of cyclin-dependent kinases 4 and 6 has been reported to be inactivated at a variety of rates in malignant tumors. We studied frequency and mechanism of inactivation of the p16 gene in various types of childhood acute lymphoblastic leukemia (ALL) using 36 leukemic cell lines established from children (B precursor-ALL, 28; B-ALL/Burkitt's lymphoma, 3; and T-ALL, 5). On Southern blot, homozygous deletions or hemizygous deletions with rearrangement were detected in 14 cell lines. The expression of p16 protein was not observed on Western blot in 18 of 22 cell lines with intact p16 gene, but induced in 16 cell lines after treatment with the demethylating agent, indicating the silencing of the p16 gene by hypermethylation. Of note, the p16 gene was inactivated by hypermethylation of the 5' CpG island in nine of nine cell lines with 11q23 translocation, but was restored with the treatment of the demethylating agent. Partial methylation of the p16 gene was also demonstrated in three of eight primary leukemia samples with this translocation, suggesting that the p16 gene inactivation by hypermethylation might play a role in the leukemogenesis and disease progression of ALL with 11q23 translocation.

Blotting, Southern↗
PubMed1999

A computer model of myocardial disarray in simulating ECG features of hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) was simulated with a computer heart model having a realistic shape and rotating fiber orientation in order to elucidate possible mechanisms for abnormal ECG findings. The disarray of myocardial muscle in HCM was simulated by assigning random fiber direction and isotropic electrophysiologic properties to abnormal hypertrophic regions, in contrast to the anisotropic modeling for normal myocardium. With these models, main ECG features including abnormal Q wave and QS pattern were reproduced and were comparable with clinical findings. This study suggests that the change in anisotropy in the hypertrophic myocardium is likely to be the main factor responsible to the ECG features of HCM.

Anisotropy↗
PubMed1999

[One-hour protocol stress myocardial scintigraphy: prospective study of diagnostic accuracy for the detection of coronary artery stenosis].

A one-hour protocol for stress myocardial scintigraphy using technetium-99m-tetrofosmin (Tc-tetrofosmin) was compared with coronary arteriography for the detection of coronary artery stenosis in 90 consecutive patients without prior myocardial infarction, revascularization procedure or vasospastic angina. Tc-tetrofosmin stress myocardial scintigraphy acquired a rest image 20 min after intravenous administration of Tc-tetrofosmin (185 MBq, 1/5 vial) using a three-head gamma camera collecting 20-sec views over 360 degrees. The stress test using bicycle ergometer was performed and administration of Tc-tetrofosmin (740 MBq, 4/5 vial) was repeated at the peak stress point. The stress image was acquired 15 min after the second injection with 5-sec views over 360 degrees. Coronary arteriography revealed the presence of significant coronary artery stenosis (> 75%) in 56 vessels of 45 patients, including 35 patients with single-vessel disease, 5 with two-vessel disease, 2 with three-vessel disease, and 3 with left main trunk disease. The overall sensitivity and specificity for the detection of coronary artery disease by visual analysis were 91.1% and 77.8%, respectively, and by quantitative analyses (using bull's-eye method) were 95.6% and 91.1%, respectively. The individual stenotic vessel sensitivities in the right coronary artery, left anterior descending artery, and left circumflex artery were 84.6%, 90.9%, and 78.6%, respectively. The specificities were 97.3%, 95.9%, and 100.0%, respectively. These results suggest that stress myocardial scintigraphy using the present new protocol is a promising approach for the detection of coronary artery stenosis.

Angina Pectoris↗
PubMed1999

[A case of myasthenia gravis accompanied by steroid-resistant nephritic syndrome and CD57+ lymphocytes expansion in peripheral blood].

A 38-year-old woman showed symptoms of myasthenia gravis (MG) three months after receiving thymectomy for malignant thymoma. She was treated with anti-acetylcholine esterase drugs and azathioprine over 10 years with two exacerbations, which were controlled by plasmapheresis and large amounts of steroid. Nephrotic syndrome developed suddenly at the age of 48, was progressive even after azathioprine withdrawal, and resistant to several immunosuppressive therapies such as steroids and cyclosporine A, and plasmapheresis. She died of systemic infection one-and-a-half years after the onset of nephrotic syndrome. Immunological studies revealed several abnormalities of cellular immunity. The expansion of gamma-delta T cells and CD57+ lymphocytes in peripheral blood was characteristic findings. These cells are thought to originate from the extrathymic process. Nephrotic syndrome has been thought to be sometimes complicated with thymoma. Although some pathogenetic possibilities about combination of nephrotic syndrome and thymoma were supposed, none has yet been clarified. As we noticed the remarkable increase in the number of CD57+ cells, we examined its proportion in the peripheral blood of patients with MG and/or thymoma, as well as in individuals without sickness. The study revealed the expansion of CD57+ cells in MG thymoma patients (32.3 +/- 15.9%), compared with healthy controls (15.2 +/- 5.4%), MG non-thymoma patients (20.3 +/- 11.5%), and thymoma non-MG patients (15.2 +/- 12.0%) statistically (Mann-Whitney's U test). Therefore, we supposed that the peripheral CD57+ cell expansion was associated with parathymic immunological abnormalities, such as MG, thymoma, and nephrotic syndrome.

Adult↗
PubMed1999

[Utility of an infrared ear thermometer as an intraoperative core temperature monitor].

We investigated the utility of an infrared ear thermometer (M 10, Terumo) as an intraoperative core temperature monitor. Temperatures of the axilla and inner ear were measured before and after anesthesia. Bladder, rectal, and forehead deep temperatures were continuously measured using a core temperature monitor (CTM-205, Terumo) during anesthesia. At the same time, the temperature of the inner ear was measured 4 times every 15 minutes (twice from cephalad and twice from caudal directions). There were no significant differences in the measured inner ear temperatures, either between directions of measurement or between the 1st and 2nd measurements. The inner ear temperature showed a close correlation with rectal, bladder, forehead deep, and axillary temperatures (r = 0.72-0.79, P < 0.01). The smallest temperature difference in this study was found to be that between the inner ear and forehead deep temperatures (-0.10), and the limit of agreement between these temperatures was also the smallest (0.81). In conclusion, we recommended the use of an infrared ear thermometer as an intraoperative core temperature monitor, especially in lower abdominal surgery, in which neither rectal nor bladder temperature monitoring is reliable.

Anesthesia, General↗
PubMed1999

Clonal hematopoiesis in acquired aplastic anemia revealed by rearrangement of the immunoglobulin heavy chain gene-JH region.

We report on a female patient with acquired aplastic anemia whose bone marrow cells showed DNA rearrangement of the immunoglobulin-JH region that disappeared after 1 month with recovery of hematopoiesis through treatment with granulocyte colony-stimulating factor (G-CSF) and immunosuppressive drugs. The patient is now 2 years and 6 months from onset, and her hematopoiesis is almost within normal limits without medication. This finding provides new data supporting clonal hematopoiesis in acquired aplastic anemia but does not imply that the disease is resistant to immunosuppressive therapy.

Anemia, Aplastic↗
PubMed1998

Rainbow trout Sox24, a novel member of the Sox family, is a transcriptional regulator during oogenesis.

We isolated a cDNA clone encoding a novel SRY-type HMG box (Sox) protein, designated Sox24, from a rainbow trout ovary cDNA library. On the basis of the HMG box amino acid sequence, Sox24 can be categorized into the same subgroup of Sox proteins as SOX4, SOX11, and SOX22. The proteins in this group also share a highly conserved sequence at the C-terminus. The Sox24 mRNA is expressed at high levels in the ovary, and in-situ hybridization localized its expression to oocytes. The recombinant protein containing the Sox24 HMG box region bound to an AACAAT sequence strongly in a gel retardation assay. Upon co-transfection into CHO cells, the full-length Sox24 transactivated transcription from a reporter plasmid through the AACAAT binding motif. We used GAL4/Sox24 chimeras with the DNA binding domain of yeast GAL4 at the N-terminus to map the transactivation function to the C-terminal region, which included the conserved sequence. These results suggest that Sox24 plays a role as a transcriptional regulator during oogenesis.

Amino Acid Sequence↗
PubMed1998

Development of a three-electrode-lens drift tube for time-of-flight mass spectrometry.

A three-electrode-lens drift tube for time-of-flight mass spectrometry (TOF-MS) has been developed for utilizing a detector to observe photon-stimulated desorption (PSD). In spite of a small detection area, the detector has a high detection efficiency and durability to reactive gas atmosphere at high pressure. The TOF-MS performance of the drift tube was examined for PSD using single-bunch-mode synchrotron radiation on a dichlorosilane (SiH(2)Cl(2))-saturated Si(001) surface. The measured acceleration and focusing-voltage dependences of the time of flight, intensity and full width at half-maximum for the peak of H(+) and Cl(+) PSD ions are discussed in terms of the numerical calculations of ion trajectories and focusing characteristic of the drift tube.

Journal Article↗
PubMed1998

Transient increase in CD45RO expression on T lymphocytes in infected newborns.

Although infections are a significant cause of mortality and long-term morbidity of newborns, the early diagnosis of neonatal infections remains difficult. It has been shown recently that up-regulation of CD45RO expression on umbilical cord lymphocytes might be a good indicator for intrauterine viral infections. To evaluate clinical relevance of changes in CD45 isoforms to the diagnosis of neonatal infections, we examined CD45RA and CD45RO expression on total, CD4+, and CD8+ lymphocytes from newborns. Lymphocytes from 55 newborns who were hospitalized in Kiyose Metropolitan Children's Hospital or Yamanashi Medical University Hospital were analyzed. Newborns were classified into three groups according to the possibility of bacterial or viral infection; I ("no" infection, n = 24), II ("possible" infection, n = 17), and III ("definite" infection, n = 14). Results were compared between two of three groups. The CD45RO expression on total or CD4+ lymphocytes in group III (bacterial 12, viral 2) was significantly higher than that in group I or II. Particularly, the CD45RO expression on CD4+ lymphocytes was always greater than 10% in group III, and showed no overlap with the respective value in group I. After termination of infection, the increased CD45RO expression in group III gradually returned to normal levels. These results indicate that not only viral but also bacterial infections trigger transient and reversible changes in CD45 isoform expression by neonatal CD4+ T lymphocytes. This may be a novel marker for the early diagnosis of neonatal infections.

CD3 Complex↗
PubMed1998

Magnetic resonance imaging of Bland-White-Garland syndrome--a case of anomalous origin of the left coronary artery from the pulmonary trunk in a 22-year-old woman.

Selective right coronary angiography in a 22-year-old woman demonstrated an enormous right coronary artery, with the contrast medium opacifying to the left coronary artery through a well-developed collateral circulation and draining into the pulmonary artery. The diagnosis of anomalous origin of the left coronary artery from the pulmonary trunk [also known as Bland-White-Garland (BWG) syndrome] was thus established. Magnetic resonance imaging (MRI) showed a dilated right coronary artery arising from the aorta, the left coronary artery from the pulmonary trunk but not from the aorta, and well-developed channels in the interventricular septum. MRI could be a useful tool for diagnosing anomalous origin of the left coronary artery from the pulmonary trunk.

Coronary Vessel Anomalies↗
PubMed1998

Effect of pacing rate in pacing therapy in hypertrophic obstructive cardiomyopathy.

The effects of dual-chamber pacing therapy in patients with hypertrophic obstructive cardiomyopathy (HOCM) have been reported in short- and long-term studies. Almost all of these studies have reported that the key factor in pacing therapy is the setting of the atrioventricular (AV) interval. However, studies focusing on the effects of pacing rate on the hemodynamic state are rare. In this study, cardiac catheterization was performed in 2 patients during temporary pacing at various rates and AV intervals. When the pacing rate was increased slightly (to 70-90/min), AV sequential pacing decreased peak subaortic pressure gradient and increased systolic aortic pressure without increase in pulmonary capillary wedge pressure, left ventricular end-diastolic pressure, and the time constant of isovolumetric relaxation. In another case, of a patient who became refractory to AV sequential pacing therapy at an optimum AV interval, pacing at a slightly higher rate relieved syncope. Thus, AV sequential pacing therapy performed at a slightly higher rate than normal in a patient with HOCM may lead to a decreased subaortic pressure gradient and relief of symptoms without noticeable deterioration in cardiac function.

Adult↗
PubMed1998

Indications for peripartum aortic pressure monitoring in Takayasu's disease. A patient with past history of intrapartum cerebral hemorrhage.

We describe a successful delivery by prophylactic cesarean section in a patient with Takayasu's disease who had a previous history of intrapartum cerebral hemorrhage during vaginal delivery. Aortography showed occlusion of the common carotid, subclavian and vertebral arteries on the left side, severe narrowing of the right subclavian artery, and moderate narrowing of the entire abdominal aorta. Blood pressure was managed by monitoring central aortic pressure at the thoracic aorta. During the peripartum course, we recommend monitoring the central aortic pressure in patients with Takayasu's disease in whom accurate blood pressure readings can not be obtained in any extremity.

Adult↗