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Biomedical subjects

T Ishihara

Publications and source records attributed to T Ishihara.

At least 307 records · Page 17Linked to original sources

[Non-Fukuyama type merosin-positive congenital muscular dystrophy with delayed muscle fiber type differentiation: a case report].

A patient with non-Fukuyama type merosin-positive congenital muscular dystrophy (nonFCMD) who had severe muscle weakness leading to early death was reported. He was the first product of epileptic mother who had been placed on phenobarbital and phenytoin. The patient had severe respiratory failure and muscle weakness at the neonatal period, and died at 4 months of age. Multiple joint contractures were also noted at birth. Serum creatine kinase was within normal limits (123 IU/l). Electromyography showed a myogenic pattern. Brain computed tomographic (CT) scan and magnetic resonance imaging (MRI) were normal without white matter lucency or pachygyria. Muscle biopsy revealed dystrophic changes and type 2C fiber predominance. Dystrophin, dystrophin-associated glycoproteins and merosin were all positively demonstrated. Although patients with merosin-positive nonFCMD have relatively mild clinical course, our patient had severe muscle weakness with fatal outcome. Defect in muscle fiber maturation and differentiation, such as an increase of undifferentiated type 2C fibers, may be a major factor to influence muscle symptoms in non FCMD.

Humans↗

A case of malignant melanoma: disease progression correlated with serum levels of 5-S-Cysteinyldopa (5-S-CD) and intercellular adhesion molecule-1 (ICAM-1).

A 36-year-old Japanese female, in whom malignant melanoma was detected from symptoms of brain metastasis, died 7 months later. The serum levels of 5-S-Cysteinyldopa (5-S-CD) and intercellular adhesion molecule-1 (ICAM-1) correlated with the disease progression. These values may be useful markers of disease progression for malignant melanoma, because the material is easily, frequently, and non-invasively obtained at regular intervals.

Adult↗

[Bacteriological, pharmacokinetic and clinical studies of SY5555 dry syrup in the pediatric field].

Bacteriological, pharmacokinetic and clinical studies on SY5555 dry syrup (powder which is dissolved before use), a new penem antibiotic for oral use, were performed. The following results were obtained. 1. Antibacterial activities. MICs of SY5555, clavulanic acid/amoxicillin (CVA/AMPC), cefotiam (CTM), cefpodoxime (CPDX), cefaclor (CCL) and cefdinir (CFDN) were determined against clinically isolated Staphylococcus aureus, coagulase negative staphylococci, Streptococcus pneumoniae, Streptococcus pyogenes, Haemophilus influenzae, Moraxella catarrhalis, Escherichia coli and Enterobacter cloacae at a dose of 10(6) CFU/ml. MICs of SY5555 against S. aureus, CNS, S. pneumoniae, S. pyogenes, H. influenzae, M. catarrhalis, E. coli and E. cloacae were 0.2, 0.2, 0.2, < or = 0.025, 0.78, 0.2, 0.78 and 3.13 micrograms/ml, respectively, showing excellent antibacterial effects on these pathogens. Although the effects of SY 5555 against H. influenzae and E. coli were slightly inferior to those of CPDX and CFDN, the drug showed the most excellent antibacterial effect on other strains as compared with the control drugs. 2. Absorption and excretion In this study, plasma concentrations and urinary recovery rates were examined after administration of SY5555 at doses of 5 and 10 mg/kg (potency) after meals. With both 5 and 10 mg/kg doses, peak plasma concentrations were reached 1 hour after administration, at 0.25-2.61 micrograms/ml (mean 1.47 micrograms/ml) and 1.08-2.17 micrograms/ml (mean 1.74 micrograms/ml), respectively. The plasma levels rapidly decreased to 0.06-0.19 micrograms/ml (0.12 micrograms/ml) and 0.0503-0.0637 micrograms/ml) after 6 hours. The half-lives 1.12 hours in the 5 mg/kg group and 1.0 hour in the 10 mg/kg group. The urinary recovery rates were determined in the first 8 hours after administration in the 5 mg/kg and 6 hours in the 10 mg/kg group, and the values were as low as 1.05-12.3% and 1.6-4.33%, respectively. 3. Clinical results The clinical responses were examined in a total of 73 cases including 4 acute pneumonia, 13 acute bronchitis, 11 tonsillitis, 3 pharyngitis, 12 scarlet fever, 2 pertussis, 6 urinary tract infection, 6 otitis media, 7 lymphadenitis, 2 staphylococcal scalded skin syndrome, 2 phlegmon, 4 impetigo and 1 purulent parotitis. The treatment was effective or better in 66 of 70 cases with an efficacy rate of 94.3% (3 undeterminable cases were excluded). Bacteriological effects were examined during the clinical course for detected or suspected pathogens found before administration of SY5555. The effects were determined in 50 cases including 7 cases of polymicrobacterial infections, 57 strains in total. Eight strains, however, persisted, hence the overall eradication rate was 86.0%.(ABSTRACT TRUNCATED AT 400 WORDS)

Absorption↗

[Blood gas changes in Duchenne type muscular dystrophy].

We studied changes in blood gas data from 32 patients with Duchenne type muscular dystrophy (DMD), who were followed for more than 5 years. Regression lines for each blood gas variable were obtained by the least square regression method, from 276 observations. The mean change per year was calculated by averaging the individual slopes. All blood gas variables (PaO2, PaCO2, pH, and [HCO3-]) correlated significantly with age the regression lines were: PaO2 = 95.6-0.45x Age, PaCO2 = 34.7 + 0.70x Age, pH = 7.397-0.0023x Age, [HCO3-] = 20.7 + 0.28x Age. The rates of change were -0.74 Torr/year for PaO2, 1.07 Torr/year for PaCO2, -0.0028 per year for pH and 0.55 mEq/l/year for [HCO3-]. The slope of each regression line was within the average individual yearly change +/- one standard deviation. AaDO2 did not change significantly with age, but chronic respiratory acidosis worsened because of alveolar hypoventilation. As an index of the severity of DMD, PaCO2 was better than PaO2, because the former was more strongly correlated with age and the values of PaCO2 deviated less from the the regression line.

Adolescent↗

[Extramedullary relapse in childhood acute lymphoblastic leukemia: experience at the Kyushu Yamaguchi Children's Cancer Study Group].

Since October 1984, children with acute lymphoblastic leukemia (ALL) were treated with six protocols of the Kyushu-Yamaguchi Children's Cancer Study Group (KYCCSG). We reviewed cases in whom the first relapse sites of ALL were CNS, testis and ovary. Between October 1984 and July 1993, 302 children with ALL were registered. Ten children (3.3%) developed CNS-L. After treatment for CNS-L, four cases were alive, however one of them has had several episodes of CNS-L. Testicular relapse occurred in 8 boys and three of them are alive. One girl developed ovarian and uterine involvement 7 months after completion of treatment and she survived after intensive chemotherapy for an additional 3 years. Before September 1990, children with ALL received 18Gy cranial irradiation in standard risk group and 24Gy in high risk group. Since October 1990, children with ALL received no cranial irradiation in low risk group, 15Gy in intermediate risk group and 18Gy in high risk group. Incidence of CNS-L has not increased in children treated with protocols, in which cranial irradiation was reduced.

Adolescent↗

Inflammatory response in facioscapulohumeral muscular dystrophy (FSHD): immunocytochemical and genetic analyses.

To investigate the nature of the inflammatory response in facioscapulohumeral muscular dystrophy (FSHD), we analyzed mononuclear cells in muscle sections obtained from 18 FSHD patients and 8 controls. Monoclonal antibodies reactive for T cells, T cell subsets, B cells, and NK cells were used for cell typing. Macrophages were identified by acid phosphatase reaction. The localization of perforin, granzyme A, MHC-I and -II, dystrophin, and alpha-actinin antigens was also examined. We found that all FSHD patients, both familiar and sporadic cases, had greater amounts of mononuclear cellular infiltrates in muscle than controls, in whose specimens only few extra vascular mononuclear cells were counted. Seventy-two percent (13 of 18) of the patients had more than 50 inflammatory mononuclear cells per 1000 muscle fibers, and 33% (6 of 18) patients had numerous inflammatory cells exceeding 600 per 1000 muscle fibers (1835 +/- 482 SE). Nonnecrotic fibers invaded by mononuclear cells with either T8+, perforin+, or granzyme A+ were not observed in FSHD, while a few degenerating fibers were superficially invaded by T cells and macrophages. Occasional T cells were observed moving through the blood vessel wall. The increased number of necrotic fibers was paralleled by an increased number of inflammatory cells (r = 0.783, P = 0.0001). Genetic analysis, using the probes p13E-11, pFR-1, D4S139, and D4S163, was done in 6 patients (3 familiar, 3 sporadic) who had numerous inflammatory infiltrates. These 6 patients had small (< 28 kb) EcoRI fragments associated with the disease, and the disease was linked to 4q35. These results suggest that, in chromosome 4-linked FSHD: (1) inflammatory changes in muscle are a common histological feature; (2) mononuclear cellular infiltrates may enhance muscle fiber damage; but (3) T-cell-mediated cytotoxicity directed against muscle fibers is unlikely. We speculate that the immune effector mechanism in FSHD is different from that in previously reported inflammatory myopathies and Duchenne muscular dystrophy.

Adolescent↗

[Status epilepticus in two patients with Sotos syndrome].

Two patients with Sotos syndrome showed very intractable and prolonged status epilepticus, resulting in poor outcomes. Clinical seizures and EEG abnormalities in patients with Sotos syndrome are sometimes noted, but they are usually mild. These two patients showed hypoplasia of corpus callosum on MRI. We considered the mechanism of intractable seizures, and emphasized the importance of careful management for their seizures and EEG abnormalities.

Brain Diseases↗

[Amyloidosis].

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Amyloidosis↗

[Respiratory failure: respiratory disorder during sleep in patients with myotonic dystrophy].

Myotonic dystrophy (MyD) patients have been reported to show severe nocturnal desaturation related to sleep respiratory disorders. However, the reason of respiratory failure in MyD has remained unclear. In this study, ten patients with MyD underwent overnight polysomnography to evaluate the mechanisms which would cause respiratory failure, compared with Duchenne muscular dystrophy (DMD) patients in these three views: 1) sleep-related nocturnal desaturation, 2) histopathological evaluation of respiratory muscles, and 3) abnormalities of respiratory center. Nocturnal desaturation was more prominent in MyD rather than DMD. Apnea-hypopnea index (AHI) was higher in MyD than DMD. Type of respiratory disorder during sleep was mainly central apnea-hypopnea pattern, including Cheyne-Stokes respiration. In histopathological findings, central core change in respiratory muscle related to respiratory muscle fatigue was found less frequently in MyD than DMD. In respiratory center function, MyD showed hyporesponse to both alveolar hypercapnic and hypoxic stimulation. However, DMD showed normal response to both stimulations. We concluded that respiratory failure in patients with MyD would be attributed to respiratory center disorder rather than respiratory muscle weakness, which is the main cause of respiratory failure in patients with DMD.

Humans↗

Potential flow of frontal midline theta activity during a mental task in the human electroencephalogram.

The movement of potential field (potential flow) of frontal midline theta activity (Fm theta) as well as its potential distribution was examined in 7 subjects by using optical flow detection techniques in image processing. Electroencephalograms (EEGs) over the fronto-central region were recorded from 13 electrodes near the frontal midline (Fz) while the subjects were performing a mental task. The potential flow of Fm theta was estimated on a frame consisting of a square grid with Fz at its center. In regions anterior to Fz, the direction of potential flow was from lateral to medial, whereas it was from medial to lateral in regions posterior to Fz. The peak-to-trough amplitude distribution was round or oval with a maximum just anterior to Fz. The source density distribution showed the greatest potential along the midline in the frontal region and bilaterally symmetric smaller maximum areas mostly in the prefrontal regions. Our findings suggested the presence of 2 different source areas of opposite direction in each hemisphere in spite of a round or oval amplitude distribution.

Brain Mapping↗

Elevated basic fibroblast growth factor in the serum of patients with Duchenne muscular dystrophy.

The mechanism whereby dystrophin deficiency leads to excessive fibrosis and muscle degeneration is not known. The absence of dystrophin in skeletal muscle is associated with reduced plasma membrane stability as evidenced by elevated serum levels of the cytoplasmic enzyme creatine kinase. Basic fibroblast growth factor, a cytoplasmic polypeptide growth regulator that stimulates connective tissue synthesis, induces satellite cell proliferation, and suppresses myogenic differentiation, is made by skeletal muscle. We hypothesize that dystrophin deficiency leads to the constant release of basic fibroblast growth factor, which in turn contributes to fibrosis and muscle weakness by stimulating connective tissue and suppressing skeletal muscle differentiation. As an initial step in testing this hypothesis, we measured basic fibroblast growth factor in the serum of Duchenne muscular dystrophy patients. We found that whereas basic fibroblast growth factor was undetectable in the serum of normal individuals (n = 200), levels were elevated in 11 of 18 patients with Duchenne muscular dystrophy.

Adolescent↗

Reduced cerebellar blood flow and oxygen metabolism in spinocerebellar degeneration: a combined PET and MRI study.

Fourteen patients with spinocerebellar degeneration (SCD) were subjected to MRI and PET studies. The quantitative MRI data revealed significant cerebellar and pontine atrophy in the patients with olivopontocerebellar atrophy (OPCA), and cerebellar atrophy in the patients with late cerebellar cortical atrophy (LCCA). We failed to demonstrate significant differences in the pons between LCCA patients and normal controls. PET measurements revealed decreases in cerebral oxygen metabolic rate (CMRO2) in the cerebellar hemisphere and vermis in both groups of patients. The markedly decreased cerebral blood flow (CBF) and CMRO2 in the pons were found only in the patients with OPCA. PET data corrected for the tissue shrinkage on the basis of MRI morphometry indicated a net reduction in cerebellar CMRO2 and CBF. The present study has demonstrated that a combination of functional and anatomical data offers further evidence in favour of the current acceptable classification of SCD based on clinicopathological grounds. Our data further suggest that the amount of atrophy in the cerebellum could not fully account for the decreased metabolic rates observed in PET studies.

Adult↗

Bilateral giant bullae with rapidly increasing fluid in the right bulla following operation: report of a case.

A 33-year-old Japanese male was admitted to our hospital for the treatment of left pneumothorax. He was found to have bilateral giant bullae. Retention of fluid was observed in the right giant bulla. The fluid accumulated rapidly directly after bullectomy and excision of the left giant bullae. This suggested the existence of some mechanisms of fluid retention other than an infection of the bulla alone after operation.

Adult↗

Pleural lipoma: report of a case.

We report herein the unusual case of a 45-year-old Japanese man whose chest X-rays revealed an abnormal shadow, increasing in size. A chest wall tumor was suspected, based on the findings of computed tomography (CT) of the thorax, the CT number of which was -137, chest roentgenogram, and an echograph. An open biopsy was performed to establish the final diagnosis. The resected tumor was a pedunculated pleural mass, yellowish in color, the pathological diagnosis of which confirmed a lipoma. Intrathoracic lipomas are rare, but pleural lipomas are seen even less frequently. CT, echography, and percutaneous needle biopsy have been found useful for diagnosing intrathoracic lipomas, but these examinations are not always adequate for confirming the final diagnosis. Consequently, tumor resection is essential for obtaining a pathological diagnosis.

Humans↗

Preparation of nanoparticles bearing high density carbohydrate chains using carbohydrate-carrying polymers as emulsifier.

A novel method of preparing nanoparticles bearing high density carbohydrate chains on their surface is described. Carbohydrate-bearing nanoparticles of poly(lactic acid) or polystyrene were prepared by the solvent evaporation method using a carbohydrate-carrying polystyrene derivative which served as both an emulsifier and a surface coating. The diameter of the obtained nanoparticles ranged from 80 to 300 nm depending on the concentration of the polystyrene derivative. As the concentration of the polystyrene derivatives increased the nanoparticle diameter decreased, indicating that the polystyrene derivatives worked as an emulsifier. The obtained particles were specifically aggregated by carbohydrate-specific lectin, showing that the polystyrene derivative was retained on the particle surfaces and expressed carbohydrate residues. The density of carbohydrates on the particle surfaces was determined to be 3-5 molecules per square nanometre. The particles prepared by the present method were stably dispersed and hardly aggregated in aqueous media during storage and centrifugal treatment compared with the post-coated particles that were prepared by adsorbing polystyrene particles with the polystyrene derivative. In vitro study with isolated rat hepatocytes revealed that surface carbohydrate chains were recognized by hepatocytes.

Animals↗

Isolation, characterization and epistasis of fluoride-resistant mutants of Caenorhabditis elegans.

We have isolated 13 fluoride-resistant mutants of the nematode Caenorhabditis elegans. All the mutations are recessive and mapped to five genes. Mutants in three of the genes (class 1 genes: flr-1 X, flr-3 IV, and flr-4 X) are resistant to 400 micrograms/ml NaF. Furthermore, they grow twice as slowly as and have smaller brood size than wild-type worms even in the absence of fluoride ion. In contrast, mutants in the other two genes (class 2 genes: flr-2 V and flr-5 V) are only partially resistant to 400 micrograms/ml NaF, and they have almost normal growth rates and brood sizes in the absence of fluoride ion. Studies on the phenotypes of double mutants showed that class 2 mutations are epistatic to class 1 mutations concerning growth rate and brood size but hypostatic with respect to fluoride resistance. We propose two models that can explain the epistasis. Since fluoride ion depletes calcium ion, inhibits some protein phosphatases and activates trimeric G-proteins, studies on these mutants may lead to discovery of a new signal transduction system that controls the growth of C. elegans.

Animals↗