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Biomedical subjects

T Ogata

Publications and source records attributed to T Ogata.

At least 145 records · Page 8Linked to original sources

Microphthalmia with linear skin defects syndrome in a mosaic female infant with monosomy for the Xp22 region: molecular analysis of the Xp22 breakpoint and the X-inactivation pattern.

This paper describes a female infant with microphthalmia with linear skin defects syndrome (MLS) and monosomy for the Xp22 region. Her clinical features included right microphthalmia and sclerocornea, left corneal opacity, linear red rash and scar-like skin lesion on the nose and cheeks, and absence of the corpus callosum. Cytogenetic studies revealed a 45,X[18]/46,X,r(X)(p22q21) [24]/46,X,del(X)(p22)[58] karyotype. Fluorescence in situ hybridization analysis showed that the ring X chromosome was positive for DXZ1 and XIST and negative for the Xp and Xq telomeric regions, whereas the deleted X chromosome was positive for DXZI, XIST, and the Xq telomeric region and negative for the Xp telomeric region. Microsatellite analysis for 19 loci at the X-differential region of Xp22 disclosed monosomy for Xp22 involving the critical region for the MLS gene, with the breakpoint between DXS1053 and DXS418. X-inactivation analysis for the methylation status of the PGK gene indicated the presence of inactive normal X chromosomes. The Xp22 deletion of our patient is the largest in MLS patients with molecularly defined Xp22 monosomy. Nevertheless, the result of X-inactivation analysis implies that the normal X chromosomes in the 46,X,del(X)(p22) cell lineage were more or less subject to X-inactivation, because normal X chromosomes in the 45,X and 46,X,r(X)(p22q21) cell lineages are unlikely to undergo X-inactivation. This supports the notion that functional absence of the MLS gene caused by inactivation of the normal X chromosome plays a pivotal role in the development of MLS in patients with Xp22 monosomy.

Chromosome Mapping↗

Occurrence of okadaic acid-producing Prorocentrum lima on the Sanriku coast, northern Japan.

Prorocentrum lima was found to be distributed on the surface of the algae, Sargassum confusum and Carpopeltis flabellata collected at the Sanriku coast, northern Japan. Chemical analysis of cultured cells revealed that Sanriku strains of P. lima produce okadaic acid, a toxin responsible for diarrhetic shellfish poisoning. The Sanriku strain grew well in T1 medium at 15 degrees C at which tropical strains do not grow, indicating that it is a local strain which adapts to cooler environments.

Adaptation, Biological↗

Severe form of juvenile corneal stromal dystrophy with homozygous R124H mutation in the keratoepithelin gene in five Japanese patients.

AIM: To confirm the mutation of the keratoepithelin gene in patients with a severe form of superficial juvenile granular corneal dystrophy (GCD). METHODS: Five Japanese probands in whom GCD was diagnosed after histopathological examination and who developed severe manifestations of GCD in their first decade of life were investigated. Other affected family members of two probands were also examined. All probands were the offspring of consanguineous parents. DNA was extracted from their peripheral blood leucocytes and mutational analysis of the gene was performed by the polymerase chain reaction and direct sequencing. RESULTS: Four of the five probands underwent their first keratectomy or keratoplasty in their teens and subsequently underwent a second or third keratoplasty. Each proband had a homozygous G-->A transition at codon 124, replacing Arg-->His, of the keratoepithelin gene. Their moderately affected family members were heterozygous for the mutation. CONCLUSIONS: This finding suggests that the severity of the corneal phenotype depends on the dose effect of the mutant gene.

Adult↗

Hypergonadotropic hypogonadism in a 3-year-old girl with blepharophimosis, ptosis, and epicanthus inversus syndrome.

We report on ovarian dysfunction in a 3-year-old girl with blepharophimosis, ptosis, and epicanthus inversus syndrome (BPES). A gonadotropin releasing hormone test showed hyperresponses of luteinizing hormone (<0.2-->7.2 mIU/ml) and follicle-stimulating hormone (7.1-->44.8 mIU/ml), and a human menopause gonadotropin test yielded no estradiol response (13-->11 pg/ml). The results suggest that primary ovarian failure in type I BPES can take place in early childhood.

Blepharophimosis↗

An elderly man with Klinefelter syndrome associated with hypertrophic cardiomyopathy, sick sinus syndrome, and coronary arteriovenous fistula.

Hypertrophic cardiomyopathy (HCM) is a rare cardiac complication in patients with Klinefelter syndrome. We report the case of a 67-year-old Japanese man with Klinefelter syndrome, HCM, sick sinus syndrome, and coronary arteriovenous fistula, in whom the 47XXY/46XY mosaic pattern was revealed by chromosomal study. Echocardiography revealed HCM with an interventricular septum thickness of 17 mm and a left ventricular posterior wall thickness of 10 mm. Sick sinus syndrome type III was diagnosed by paroxysmal atrial fibrillation (longest sinus arrest 9.0 sec) on 24-h Holter ECG recording. Coronary arteriovenous fistula was detected from the left anterior descending artery to the right ventricle by coronary arteriography. To our knowledge, this is the first case report of Klinefelter syndrome with HCM. As there have been a few reports of patients with Klinefelter syndrome in association with skeletal muscular diseases such as Becker-type muscular dystrophy or myotonic dystrophy, the gene mutation that causes Klinefelter syndrome may occur in the cardiac muscle. HCM may represent another variable expression of this chromosomal abnormality.

Aged↗

An electron microscopic study of Helicobacter pylori in the surface mucous gel layer.

This study describes the distribution of spiral and coccoid forms of Helicobacter (H.) pylori within the surface mucous gel layer (SMGL) on human gastric mucosae. Gastric mucosae infected with H. pylori were obtained from surgically removed stomachs of 14 cases of gastric cancer. The glycocalyx of the spiral and coccoid forms of H. pylori was examined for specific lectin labeling. Coccoid forms were identified in 11 of these cases (78.6%). The SMGL, which was from 50 to 100 microns in thickness, contained H. pylori throughout its entire thickness. Variously-sized vacuole-like clear areas were present near H. pylori. The glycocalyx on both the spiral and coccoid forms was similar in its staining with the 8 types of lectins tested. However, the staining pattern of the lectins varied among different samples. The numerous fibrillae-like filaments radiated from the surface of the bacteria and appeared to link the bacteria to the surface mucous cells or the surrounding mucus. These fibrillae-like filaments were not specifically stained by the lectin reactions, suggesting absence of sugar molecules.

Adult↗

Localization of cytoskeletal filaments during membrane rearrangement in rat parietal cells stimulated with gastrin.

When stomachs are stimulated to secrete acid, the intracellular canaliculi of the parietal cell increase and there is a concomitant depletion of the cytoplasmic tubulovesicular system. This change is believed to occur through the transformation of tubulovesicular membranes into intracellular canaliculi. This study was undertaken to examine the distribution of the cytoskeletons in rat gastric parietal cells during this process. In the resting parietal cells, actin filaments decorated with heavy meromyosin (HMM) were found in the cores of microvilli, extending from the apex of microvilli into the pericanalicular cytoplasm and forming radial networks. In some cases, these actin filaments were also associated with the tubulovesicles. Moreover, tubulovesicular membranes were rare in the 300 nm zone around intracellular canaliculi but numerous actin filaments were seen in this region. Soon after stimulation of the parietal cells by gastrin, tubulovesicles were closely associated with the intracellular canaliculi, while actin filaments networks adjacent to the canaliculi diminished and their labeling with HMM seemed less orderly. By immunocytochemistry, immunogold particles indicating ezrin were associated with microvillous membranes in the resting as well as stimulated parietal cells but were absent on the tubulovesicular membranes. When intermediate filaments were immunocytochemically investigated using anti-cytokeratin immunogold particles clearly labeled filamentous bundles present around the intracellular canaliculi, perinuclear spaces and under the basolateral cell membrane. Their localization was not changed after stimulation. These results suggest that actin filaments in the cytoplasm around the intracellular canaliculi may play a key role in the translocation of the tubulovesicles toward the intracellular canaliculi during the acid secreting process.

Actins↗

Castability of a commercial castable glass ceramics.

This study was carried out to determine the design and thickness of the cervical margin of a castable ceramic restoration from the point of view of its castability. Castability of castable ceramics was lower compared to Ag-Pd-Au and Co-Cr alloy with the use of the mesh pattern and rod pattern test (p < 0.05). It was more dependent on the pattern thickness than alloys (p < 0.05), and perfect reproducibility with non-measurable variability could be estimated to reach 1.0 mm thickness of the original pattern (p < 0.001). Marginal shape was certainly more reproducible in a right angle shoulder than in 30, or 45 degree bevels (p < 0.05). From these results, it is suggested that a right angle shoulder margin with 1.0 mm thickness is more suitable to castable glass ceramic restoration than any other design.

Analysis of Variance↗

[A case report of advanced breast cancer with remarkable response to chemoendocrine therapy (CTF + MPA)].

A 59-year-old female complaining of breast tumor with suppurative discharge was diagnosed as having advanced breast cancer (T4cN3M1-StIV), with giant liver metastasis. Seven courses of combined chemoendocrine therapy (CTF + MPA) were used. Following the chemoendocrine therapy, primary tumor, lung, pleural, supraclavicular and parasternal metastasis disappeared, and the liver metastasis was obviously diminished. These effects continued for 1 year 7 months. Although CTF + MPA chemoendocrine therapy is widely used with advanced or recurrent breast cancer, a clearly effective case has almost never been reported. The reason for the remarkable effect in this case was the consistent immunity to breast cancer.

Antineoplastic Agents, Hormonal↗

[Comparative study on quality of life between weekly and monthly chemotherapy with cisplatin, vindesine and mitomycin C in patients with non-small cell lung cancer].

A comparative study between weekly and monthly chemotherapy with CDDP, VDS and MMC was performed on the basis of QOL in patients (stage III A/III B/IV) with non-small lung cancer from September 1993 to August 1996. Arm A received CDDP (80 mg/m2) monthly on day 1, VDS (3 mg/m2) on days 1 and 8, MMC (8 mg/m2) on day 1. Arm B received CDDP (40 mg/m2), VDS (3 mg/m2) and MMC (4 mg/m2) on day 1, and followed by CDDP (20 mg/m2), VDS (1.5 mg/m2) and MMC (2 mg/m2) weekly. A diary-type QOL self-rating questionnaire was devised for this study, consisting of 5 scales including 13-item questionnaires and a face scale as the global scale. Chronological QOL data from 20 days during chemotherapy (total dose of CDDP was 80 mg/m2 in both A and B arm) were analyzed using summary measures. In the 78 eligible subjects of the study, 34 were QOL subjects of the 27 eligible subjects, 13 were in arm A and 14 in arm B. 1. The questionnaire was confirmed to be satisfactory with regard to reliability and validity as a questionnaire for the evaluation of this study by factor and correlation analysis. 2. Although each scale changed to the worth level after chemotherapy, there was no significant difference between arm A and B. 3. Summary statistics were assessed using indices of the area under the curve (AUC) and the maximum fluctuations of QOL scores (Dif max) arm B showed a tendency to smaller changes than arm A in AUC and Dif max. There was a significant difference in the physical well-being scale of Dif max. 4. Three items, including the physical well-being in arm B, revealed a better score than those of arm A. Abdominal condition showed a significant difference between the two arms. 5. No obvious difference in anti-cancer effectiveness was found between arm A and B. Arm B showed a longer median survival, less nausea and vomiting, leukopenia and thrombocytopenia compared with arm A. The above results suggested that weekly chemotherapy is as effective, less toxic and shows less influence against patients QOL than the conventional monthly chemotherapy in this study.

Adult↗

[Aortic regurgitation with stenosis due to congenitally bicuspid valve with fibrous band: a surgical case report].

A 62-year-old man with aortic regurgitation and stenosis underwent aortic valve replacement. Intraoperative findings showed fibrous band between the edge of the raphe and the ascending aorta. Such fibrous bands are usually observed in juvenile pure aortic valve regurgitation due to congenitally bicuspid valve with degenerative change and are related to cause aortic insufficiency. The present patient was comparatively old, and the aortic regurgitation was combined with stenosis, which revealed sclerotic change on histological examination.

Aortic Valve↗

[Left subclavian artery approach for insertion of IABP].

A 62-year-old man admitted with a complication of chest pain. Cardiac catheterization showed three vessel disease with ventricular dysfunction (EF 40%). And aortagram showed that he had arteriosclerosis obliterans (ASO). While he was waiting for operation, the frequency of chest pain increased. It was considered necessary for him to have IABP catheter inserted. We chose left subclavian artery as an access for the insertion of IABP catheter. The catheter was easily introduced into the descending aorta under the fluoroscopy. Emergency CABG was performed the day after insertion of IABP catheter. IABP removed the next morning of operation. Operation and postoperation course was uneventful. In this case, the insertion and the removal were possible under local anesthesia. Left subclavian approach was useful for emergency case with ASO.

Angina, Unstable↗

Protective mechanisms of adenosine in neurons and glial cells.

As illustrated in Figure 1, a disturbance of the intracellular Ca2+ homeostasis is thought to be a common pathogenic factor for the generation of secondary nerve cell damage that develops after brain trauma or stroke or during the course of neurodegenerative diseases. A neuronal Ca2+ overload which may result from an excessive glutamate-evoked membrane depolarization and consecutive Ca2+ influx as well as from an activation of metabotropic receptors and consecutive intracellular Ca2+ mobilization is known to have direct toxic effects on the cytoskeleton and the cell metabolism of neurons. In addition, a Ca(2+)-dependent activation of glial cells along with the loss of physiologically required mature astrocyte functions and with the acquisition of potentially neurotoxic microglial properties, has more recently been recognized as an additive pathogenic factor. This may provide an effective target for pharmacological interference. Specifically, the reinforcement of an endogenous homeostatic regulator, which obtained its sophisticated know-how during evolution, may provide a neuroprotective therapy which can handle the complexity of the pathological process with a minor risk of pharmacological side effects. Adenosine is such an ancient molecular signal that acts on both neurons and glial cells. In neurons, adenosine activates K+ and Cl- conductances, which limits synaptically evoked depolarization, thus counteracting the Ca2+ influx through voltage-dependent and NMDA receptor-operated ion channels. This A1 receptor-mediated effect seems to be the major action by which adenosine adds directly to the protection of neurons against Ca(2+)-dependent damage. In glial cells, the prevalent effect of adenosine is its regulatory influence on the Ca2+ and cAMP-dependent molecular signaling that determines the cellular proliferation rate, the differentiation state and related functions. When mimicking the activation of metabotropic glutamate receptors in cultures of immature rat astrocytes, which largely resemble pathologically activated astrocytes, a transient Ca2+ mobilization was initiated by adenosine. This A1 receptor-mediated Ca2+ signal caused a prolonged potentiation of the A2 receptor-mediated intracellular cAMP rise. An experimentally sustained enhancement of the cAMP signaling initiated the differentiation of cultured astrocytes and the new expression of K+ and Cl- channels which are required for the physiological astrocyte function to maintain the extracellular ion homeostasis. Evidence is accumulating that a strengthening of the cAMP signaling, which can be achieved by adenosine agonists and also by the pharmacon propentofylline (an adenosine uptake blocker and phosphodiesterase inhibitor), stimulates the mRNA production of neurotrophic factors in astrocytes. In cultured microglial cells, several days' treatment with adenosine agonists or propentofylline markedly inhibited their proliferation rate, the in vitro spontaneously occurring transformation into macrophages and their particularly high formation of free oxygen radicals. Adenosine agonists also depressed the release of the potentially toxic cytokine TNF alpha and induced programmed cell death in immunologically activated microglial cells. We conclude that a pharmacological reinforcement of the endogenous cell modulator adenosine may provide neuroprotection by counteracting neuronal Ca2+ overload, by depressing potentially neurotoxic microglial functions and by regaining physiologically required properties of differentiated astrocytes. Further information about the influence of adenosine on the molecular signaling and on ischemic brain damage is given in Refs. 37 and 38, and about the implicated possible relevance for the treatment of stroke in Ref. 39.

Adenosine↗

Support of homeostatic glial cell signaling: a novel therapeutic approach by propentofylline.

A pathological glial cell activation, which forces microglia to transform into immunocompetent cells with cytotoxic properties and astrocytes to "de-differentiate," presumably adds to neurodegenerative diseases. We examined the modulatory effect of adenosine on the Ca2+ and cAMP-dependent regulation of such reactive glial cell properties in culture and tested possibilities of pharmacologic reinforcement. A strengthening of the cAMP-signaling, as could be achieved by adenosine agonists via a Ca(2+)-dependent action, favored the differentiation of proliferating astrocytes and associated neuroprotective properties (ion homeostasis, formation of trophic factors). But potentially neurotoxic properties of microglial cells were inhibited. Adenosine depressed their proliferation rate and transformation into macrophages, their particularly high formation of reactive oxygen intermediates and the release of the cytokine TNF-alpha. Similar effects were obtained with propentofylline, which acts as selective cAMP/cGMP phosphodiesterase inhibitor and also increases the effective concentration of adenosine by blocking its cellular reuptake. The recently observed induction of microglial apoptosis by elevated extracellular adenosine levels may further contribute to limit secondary nerve cell damage related to a pathological glial cell activation.

Adenosine↗

Decrease in amplified telomeric sequences and induction of senescence markers by introduction of human chromosome 7 or its segments in SUSM-1.

Introduction of human chromosome 7 by microcell-mediated chromosome transfer suppresses indefinite division of SUSM-1, an in vitro established human fibroblast line. This cell line has unusually long telomeric sequences although it lacks detectable telomerase activity. Thus, we examined whether such telomeric sequences change upon introduction of chromosome 7 or its segments. In the microcell hybrids that stopped dividing by introduction of chromosome 7, the telomeric sequences were found to be lost or markedly diminished. Introduction of various fragments (2-40 Mb) of chromosome 7 contained in radiation hybrids gave similar results. On the other hand, the telomeric sequences were not altered significantly in the unsuppressed hybrids, a revertant of one suppressed clone, or subclones of SUSM-1 used as controls. In the suppressed microcell hybrids, the distribution of a mortality marker, mortalin, was changed to the cytosolic type of mortal cells from the immortal type of perinuclear fibres. Also, senescence-associated beta-galactosidase was induced to a level similar to that of normally senesced diploid fibroblasts. These results suggest that human chromosome 7 induces senescence in SUSM-1 by suppressing its telomere maintenance mechanism, which does not depend on telomerase.

Animals↗