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Biomedical subjects

K Miyasaki

Publications and source records attributed to K Miyasaki.

At least 19 recordsLinked to original sources

A case of myasthenia gravis associated with thymoma, multiple schwannomas and monoclonal IgA gammopathy.

Myasthenia gravis, malignant thymoma, multiple schwannomas and monoclonal IgA gammopathy coexisted in a 54-year-old Japanese man. Serum acetylcholine receptor antibody activity was located in the IgG fraction, but not in the paraprotein peak. It was speculated that all these disorders might fall under the spectrum of the diseases associated with thymic abnormality or that some growth factors might induce neoplasms and activation of B cell clones of autoantibodies (including acetylcholine receptor antibody) in this case.

Autoantibodies↗

Chromium-induced pulmonary cancer. Report of a case and a review of the literature.

A 57-year-old man, whose occupational history was that of a chromate worker for about 35 years, suffered nasal perforation during and died due to pulmonary cancer. In the autopsy materials, the concentration of chromium in the lung tissues was very high, being about 90 times the amount found in normal lungs. The chromium particles in the lung tissues could not be detected by analytical electron microscope, but they could be found in the anthoracotic lesions of the lungs by means of an X-ray microanalyser. The present case was judged to be a case of chromium-induced pulmonary cancer since all the pathologic data obtained were similar to those found in similar cases reported in the literature.

Adenocarcinoma↗

Purification and characterization of the serotype c antigen from Actinobacillus actinomycetemcomitans.

The serotype c antigen from Actinobacillus actinomycetemcomitans was purified with fractional ethanol precipitation of cell-free culture supernatant, sequential ion-exchange chromatography, and gel filtration chromatography. The preparation obtained demonstrated a single precipitin line in immunodiffusion, immunoelectrophoresis, and crossed immunoelectrophoresis when rabbit antisera to serotype c whole bacterial cells were used. No immunological reaction was detected with antisera to serotype c lipopolysaccharide, indicating that lipopolysaccharide was not present in the preparation. The serotype c antigen was composed of 95% carbohydrate, 2% protein, and 3.1% phosphate. Gas chromatographic analysis of the antigen obtained from growth in either complex or chemically defined media revealed that the carbohydrate constituent was composed of 84 to 90.1% mannose, 4.8 to 16% glucose, 1.9% N-acetylglucosamine, 1.4% fucose, and 0.2% galactose. The present data suggest that A. actinomycetemcomitans serotype c antigen is predominantly a mannose-containing carbohydrate suggestive of a mannan.

Actinobacillus↗

Inoculation of dengue virus into nude mice.

When athymic nude (nu/nu) and heterozygous littermate (nu/+) mice were injected intraperitoneally (i.p.) with a mouse-adapted strain of dengue virus (DV), the following differences were noted in the course of infection. (i) The average survival time of nu/nu mice was longer than that of nu/+ mice, although the mortality ratios were not significantly different. (ii) DV persisted in some of the nu/nu mice for long periods of time without exhibiting any symptoms but they died after prolonged incubation periods. These aspects were not observed in the nu/+ mice. (iii) Infected nu/nu mice produced IgM antibody only transiently in the early stage of infection but they did not subsequently show regular IgG antibody production which normally occurred in nu/+ mice. (iv) Piamatral and perivascular mononuclear cell infiltration in the infected brain was more intense in nu/+ than in nu/nu mice. It is suggested from these data that the course of DV infection in mice is affected by the availability of thymus-derived lymphocytes (T-cells). Infectious virus was detected in various organs and tissues of infected mice. The hearts of nu/nu mice tended to show higher virus titres than those of nu/+ mice, whereas the virus concentrations in the brain, skeletal muscle and lymph node were the same in both groups of mice. Specific DV antigen was revealed by the fluorescent antibody (FA) technique in cells located in the infected tissues.

Animals↗

Primary systemic amyloidosis. A case permitting pathological and biochemical investigations.

In this 72-year-old male patient, large amounts of amyloid substances were deposited in the mesenchymal organs, but not in the parenchymal organs. Histochemical and electron microscopic studies revealed large amounts of amyloid substances and amyloid fibrils in the skeletal and smooth muscle tissues. In the skeletal muscle tissues, where large amounts of amyloid substances were deposited, glycosaminoglycan was also markedly deposited, especially chondroitin-4-sulfuric acid. The concentrations of silicon were also markedly increased in the amyloid fibrils. The concentrations of nickel and cobalt were increased but those of zinc were decreased in all organs measured, although both nickel and cobalt in another case of amyloidosis experienced were within normal values.

Aged↗

Infantile convulsion suspected of pyridoxine responsive seizures.

A 6-month-old boy, having suffered repeated convulsive siezures since birth, died of severe epilepsy, the disease remaining unknown. However, when a brother, born after the death of the present case, displayed the same clinical symptoms and was diagnosed clinically as having pyridoxine dependent convulsion, the deceased became suspected of having been a case of familial pyridoxine dependency. Pathologically, bilateral cerebellar hemorrhages were found accompanied by subdural and subarachnoidal hemorrhages. Histologically, neuronal loss and diffuse astrocyte hyperplasia were found in the brain: Beside marginal gliosis, proliferations of astroglia and glial fibers were found, especially in the pons, medulla oblongata, and cervical cord, although the fibrillary gliosis may be representative for a physiological intermediate state. Foamy cells were found in the lymph nodes, but not in the brain. Observed in the kidneys were substances deposited in the glomeruli and Bowman's capsules and materials in the tubular lumen.

Brain↗

Parkinsonism following encephalitis of unknown etiology.

The patient, a clinical case of parkinsonism, was a 32-year-old man, born in 1942, long after the prevalence of von Economo's lethargic encephalitis in Japan. Anatomically, the neurons in the substantia nigra of the mid-brain were extensively degenerated, and presented Alzheimer's neurofibrillary tangles. At the same time, melanin pigment was scattered in the tissue and was phagocytized by glia cells. Perivascular cuffing was observed in the frontal lobe, parietal lobe, temporal lobe, hippocampus, and thalamus as well as in the substantia nigra. Neuronophagia was noted in the thalamic nuclei. The present case was believed to have parkinsonism not clinically or pathologically related to von Economo's encephalitis or to Japanese encephalitis, but following a mild encephalitis of unknown etiology.

Adult↗

Hemochromatosis associated with brain lesions--a disorder of trace-metal binding proteins and/or polymers?

A 68-year-old man, after having been diagnosed as having hepatic disease at about the age of 41 years, had been hospitalized frequently until his death. Blood sugar, iron, and copper had not increased during his illness. Although the diagnosis of liver cirrhosis had been made and he had been receiving therapy, various neurologic symptoms without disturbances of consciousness appeared six months before his death. Autopsy revealed hemochromatosis, liver cirrhosis, and pancreatic fibrosis. A large amount of iron had accumulated in the liver, the pancreas, and the thyroid gland, while considerable numbers of ceroid and lipofuscin pigment granules had accumulated diffusely in the brain. Abnormal astrocytes of the Alzheimer II type were diffusely distributed in the brain and contained no intranuclear glycogen which stained positive with the carmine stain. No spongy changes were seen in the deeper layers of the cerebral cortex. Chemical analyses for trace metals in the brain, liver, and kidneys revealed a large amount of iron and increased copper in the liver, and considerable quantities of copper, manganese, calcium, and mercury in the brain. Because of changes in the erythrocyte sedimentation rate and marked thymol turbidity seen before and after the occurrence of the neurologic symptoms, this man was suspected of having disorders of the trace-metal binding proteins and/or of their polymers.

Aged↗

An unusual case which began with subcutaneous panniculitis followed by fever, severe hepatic involvement and hyperlipidemia.

A 25-year-old man was at first dermatologically suspected as suffering from Weber-Christian syndrome because of subcutaneous panniculitis, but his skin lesions disappeared completely during the course. Hyperlipidemia, disturbances in liver function, and leukemoid reaction became remarkable and he died of subarachnoid hemorrhage eleven months after onset. Necropsy revealed subarachnoid hemorrhage at the base of the brain, lipogranulomatous and inflammatory lesions in the upper lobe of the left lung, a remarkable fatty liver, splenomegaly, pericarditis, and foam cells in the spleen, liver, and bone marrow. A comparison with 57 autopsy cases of Weber-Christian syndrome reported in the literature showed our case to be an exceptional instance of Weber-Christian syndrome, if the present case is not to be regarded as a different disease entity.

Adipose Tissue↗

Arhinencephaly (holoprosencephaly) associated with external hydrocephaly.

A female infant, weighing 2,263 g had been spontaneously delivered at the 33rd week of gestation. The only noticeable abnormality was a cleft palate, but within the next few days, she developed low temperature, spastic movements in the extremities, and depressed respiration. These abnormalities soon became worse and she died on the 12th day. Clinically, she was not suspected of having malformations of the brain, but on autopsy, an external hydrocephaly (400 ml) and small arhinencephalic brain (75 g) were noted. In the brain, the growth of the diencephalon was exceedingly poor but the growth of the rhombencephalon was relatively favorable. The olfactory bulbi and tracts in the telencephalon were absent. These findings were similar to those seen in cases of arhinencephaly (or holoprosencephaly). In the cerebral ventricles, the lateral ventricles and the IVth ventricle were enlarged, but the IIIrd ventricle could not be identified. Communication could only be found between the lateral apertures of the IVth ventricle and the subarachnoidal spaces.

Brain↗

Secondary lipidosis in leukemia.

Foam cells in the spleen, bone marrow, liver and lymph nodes were examined on the 73 reliably recorded and sampled leukemia autopsy cases encountered at Kobe University from 1958 to 1972. Although the substances stored in the foam cells were biochemically unknown, the foam cells in leukemia could be morphologically classified into two types: The one was identified with the Gaucher type, but the other was not identified with the sea-blue type and might be considered as to be the transitional type described in another report. Foam cells could be found in the spleen of 6 out of 12 cases of chronic myeloid leukemia, one out of 2 cases of chronic lymphatic leukemia, one out of 7 cases of leukemic lymphosarcoma, one out of 9 cases of acute lymphatic leukemia, and none in 3 cases of monocytic leukemia. In acute myeloid leukemia, the incidence of foam cells in the spleen was 47.5% in 40 cases, and acquired lipidoses were more frequently seen in cases under 19 years of age, in male cases, in cases with an enlarged spleen over 400 g, and in cases of over 4 months' duration.

Autopsy↗

Congenital brain and facial anomalies in the D1 trisomy syndrome--report of a case and a review of literature--.

On 58 cases of D1 trisomy, which were classified as those with seven separate D chromosomes, including the one in this report, congenital brain and facial anomalies were reviewed and discussed. Congenital brain defects in this syndrome might be classified into three groups: (I) grossly normal brain, (II) absence of olfactory bulbi and tracts, but normally-separated cerebral hemisphere, and (III) completely or incompletely uncleaved hemispheres with absence of olfactory bulbi and tracts. 25.9% of the cases were found in group I, 56.9% in group II, and 17.2% in group III, respectively. Although facial anomalies predicted the brain defects in group III, malformations of the face were not concerned with the brain defects in group I and II. There were no cases with both, normal brain and face.

Brain↗

Experimental polymer storage disease in rabbits. An approach to the histogenesis of sphingolipidoses.

Water-soluble polymer compound, polyvinyl alcohol(PVA), and water-insoluble polymer compounds, polyvinyl acetate (PVAc) and polystylol (PS), were administered in 297 rabbits. When high polymerized PVA, PVAc or PS were continuously injected intravenously for a long period of time, lesions resembled to those of Gaucher and Niemann-Pick diseases were developed. From these experimental results, pathological development of various sphingolipidoses found in the human body was discussed and pathological findings were analysed polymer-chemically from the chemical properties of the substances stored.

Animals↗

Coronary heart disease in familiar hypercholesterolemia.

Autopsy findings of a 36-year-old male with familiar hypercholesterolemia were reported and discussed. Hyperlipoproteinemia found in this case might belong to the type IIa in the classification of hyperlipoproteinemia while there was no skin lesion such as xanthoma and hypertension. Remarkable strictures due to atherosclerotic plaques in the lumens of the right and left coronary arteries and wide-spread myocardial infarction in the left ventricle were found. In the aorta just above the aortic valve there were atherosclerotic plaques in which foam cells could be seen. From the histological findings of the aortic valve the possibility that the thickening of the valve might have been induced by a similar mechanism as that of aorta and coronary arteries was suggested.

Adult↗

Renal accumulation of glycosphingolipids. Report of a case and a review of literature.

A 62-year-old woman gave clinical manifestation of liver cirrhosis. Urinary protein was false positive, no uremia was found and renal changes were entirely overlooked. Deposition of abundant lipids (globoside and ceramide trihexoside) was found in the kidneys; essentially degenerative changes of the tubular epithelia were noted. These renal changes were compared with those in Fabry's disease.

Adult↗