Biomedical subjects
K Ohno
Publications and source records attributed to K Ohno.
[Studies on pig erythrocyte lipids. II. The isolation and the identification of individual lipids with some quantitative aspects on the composition of erythrocyte lipids].
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[Studies on pig erythrocyte lipids. I. Fractionation of erythrocyte lipids by the simple combination of silicic acid and cellulose column chromatography].
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The semi-quantitative isolation of phosphatidyl-serine from pig erythrocytes, using cellulose column chromatography.
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[Studies on respiratory stimulation by remeflin. (2. Clinical experiences)].
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Effective segmental chemoembolization of advanced hepatocellular carcinoma with tumor thrombus in the portal vein.
PURPOSE: Evaluate therapeutic results of transcatheter segmental hepatic artery chemo-oily-embolization (segmental TAE) against advanced hepatocellular carcinomas (HCC) accompanied by portal tumor thrombus (PTT). METHODS: Segmental TAE was performed in nine patients with hepatic cirrhosis and advanced HCCs accompanied by PTT. Four subsegmental portal branches were obstructed by PTT in three patients, and two were obstructed in the remaining six patients. TAE was performed into the targeted segmental hepatic artery followed by embolization with gelatin sponge particles. RESULTS: In one patient undergoing subsequent resection, a marked anticancer effect on the PTT was demonstrated histologically. In the eight other patients who did not undergo subsequent resection, the cumulative survival rates were 67% at 6 months, 44% at 1 year, and 22% at 2 years. No serious complications were noted. CONCLUSIONS: This therapeutic approach is thought to be a useful treatment for HCC with PTT, because it reinforces anticancer effects and can be performed more safely than conventional transcatheter arterial embolization.
NPC1 gene mutations in Japanese patients with Niemann-Pick disease type C.
Complementary and genomic DNAs isolated from the fibroblasts of 10 Japanese (7 late infantile, 2 juvenile, and 1 adult form of the disease) and one Caucasian patient with Niemann-Pick disease type C were analyzed for mutations in the NPC1 gene. Fourteen novel mutations were found including small deletions and point mutations. A one-base deletion and a point mutation caused splicing errors. The mutations were not clustered in any particular region of the gene and were found both in and out of the transmembrane domains. Three patients were homozygous, five were compound heterozygous, and the remaining three were suspected of being compound hetrozygous with an unknown error in one of their NPC1 alleles. Of the 14 mutations, the G1553A substitution that caused a splicing error of exon 9 appeared to be relatively common in Japanese patients, because two patients were homozygous and one patient was compound heterozygous for this mutation.
Cerebrovascular integrity in protein-deprived rats.
Protein-deprivation does not increase the permeability of the blood-brain barrier in rats aged 3.5--12 months. PA, the product of cerebrovascular permeability (P) to 14C-sucrose and of cerebral capillary surface area (A), is very low in mature rats that have been maintained either on an 8% or 25% casein diet, and equals about 8 X 10(-6) sec-1 in both groups. There is a tendency for the calculated distribution volume of 14C-sucrose within the brain to decline in protein-deprived rats. Conscious, partially immobilized, protein-deprived rats have the same mean blood pressure, heart rate, arterial plasma pH and adreno-sympathetic response to stress (as measured by plasma norepinephrine and epinephrine concentrations) as do normally fed animals.
Type C Niemann-Pick disease. Detection and quantification of cholesterol-accumulating cells in bone marrow.
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Carbohydrate-deficient glycoprotein syndrome: electrophoretic study of multiple serum glycoproteins.
The serum glycoproteins in three patients with the carbohydrate-deficient glycoprotein (CDG) syndrome were examined using isoelectric focusing in polyacrylamide gels, SDS-PAGE, and a two-dimensional electrophoretic technique. Increases in isoforms with higher isoelectric points (pI's) were observed in all asparagine-N-linked glycoproteins tested. Abnormal components of transferrin, antithrombin III and orosomucoid had smaller molecular weights, about 3000 and 6000 Da, than normal ones. Antithrombin III gave an increased cathodal band. Its pI and molecular weight were the same as those of a physiological variant of antithrombin III (beta fraction), which is known to lack the asparagine-N-linked oligosaccharide chain at asparagine-135. The evidence suggests that a deficiency of asparagine-N-linked oligosaccharide transfer occurs in multiple serum glycoproteins in patients with CDG syndrome. Thus, examination of serum by isoelectric focusing in polyacrylamide gels and SDS-PAGE should be very useful for differentiating the CDG syndrome type I from clinical and biochemical variants.
Focal meningoencephalitis localized in the left occipital lobe.
A rare case of suspected focal meningoencephalitis localized in the left occipital lobe was described. A 2-year-old girl, following upper respiratory illness, suddenly became lethargic and experienced a right-sided hemiconvulsion beginning from a right-side gaze. Cranial MRI clearly demonstrated high intensity on T2-weighted imaging in the cortex, white matter, and leptomeninges of the left occipital lobe, which disappeared in the convalescent phase. We consider that the MRI abnormalities reflected focal inflammation and edema, and corresponded to the presumed ictal focus.
Frequency of the Prader-Willi syndrome in the San-in district, Japan.
Nineteen cases of the Prader-Willi syndrome were collected in the San-in district, in the western part of Japan, and an epidemiological study was carried out. The male to female ratio was 1:1.11. Deletion of chromosome 15q11-13 was found in seven of 16 patients examined by means of high-resolution chromosome banding. The incidence among live-births was estimated to be 6.64 x 10(-5) (1 in 15,060 live-births) in 1980-1989, and the mutation rate was 3.32 x 10(-5) per gene per generation, as determined by a direct method. The prevalence was estimated to be 5.72 x 10(-5) (1 in 17,483) in patients under 15 years old, which was higher than that in other autosomal dominant diseases (tuberous sclerosis and congenital myotonic dystrophy) also studied in the San-in district. A birth-order effect was revealed and a slightly significant difference was found as to the fathers' age between the patients and their normal siblings. The results of these two tests are in accord with the recent discovery that this syndrome is caused by paternal chromosomal aberrations.
A Japanese case of infantile sialic acid storage disease.
We report a 4-year-old Japanese girl with infantile sialic acid storage disease. She presented with failure to thrive, coarse facial features, hepatosplenomegaly, severe mental retardation and spastic quadriplegia. Electron microscopic examination of cultured skin fibroblasts revealed multiple vacuoles and inclusion material representing distended lysosomes, thus suggesting a lysosomal storage disorder. A high concentration of free sialic acid was present in the urine and cultured fibroblasts, but bound sialic acid was not increased. The activity of a variety of lysosomal enzymes was not diminished. The MRI findings included brain atrophy and a diffuse high signal in the cerebral white matter and low signal in the basal ganglia on T2-weighted images. To our knowledge, this is the first case of infantile sialic acid storage disease described in a non-Caucasian family.
Joubert syndrome associated with unilateral ptosis and Leber congenital amaurosis.
Two siblings are described with clinical features of Joubert syndrome associated with unilateral ptosis, severe visual disturbances with normal appearing fundi, and an occipital meningocele; one child also had polydactyly. Neither child manifested responses to electroretinography and one did not manifest visual evoked potentials. The siblings were considered to have characteristics of Leber congenital amaurosis and Joubert syndrome as well as unilateral ptosis. The presence of these findings in siblings suggests a genetic basis for their disease. Careful investigations should be conducted on infants with dysplasia of the cerebellar vermis, including tests for respiratory irregularities, retinal abnormalities, and renal abnormalities to further clarify the interrelationships of these conditions.
Relief of chronic burning pain in Fabry disease with neurotropin.
Neurotropin, an extract from the inflamed skin of vaccinia virus-inoculated rabbits, was effective in the relief of sharp or burning pain induced by pyrexia, hot weather, bathing, or exercise in 2 siblings with Fabry disease. Neither neurotropin nor carbamazepine mono-therapy relieved the episodic colicky pain in 1 patient; however, therapy with both drugs eliminated the pain completely. This result suggests that the mechanisms underlying the analgesic actions of both drugs may be complementary in ameliorating the pain of Fabry disease, even though the mechanism underlying the pain has not been clearly elucidated.
Varicella with delayed hemiplegia.
We report 4 children who developed acute hemiplegia 7 weeks to 4 months after varicella infection. In 2 patients, carotid angiography demonstrated segmental narrowing and occlusion of the middle cerebral artery. Their clinical and angiographic features were similar to those associated with contralateral hemiplegia after herpes zoster ophthalmicus, the pathogenesis of which comprises cerebral angiitis due to varicella zoster viral infection. We believe that our patients had the same pathogenesis. In a survey of infectious diseases in our region, the frequency of varicella with delayed hemiparesis was roughly 1:6,500 varicella patients.
Galactose-containing amphiphiles prepared with a lipophilic radical initiator: association processes between liposomes triggered by enzymatic reaction.
A galactose-containing monomer (2-(methacryloyloxy)ethyl beta-D-galactopyranoside, MEGal) was polymerized by using a lipophilic radical initiator. The amphiphile obtained formed a liposome by mixing with bis(trans,trans-2,4-dioctadecadienoyl)phosphatidylcholine (DDPC), and the liposome obtained was physically stabilized by the polymerization of DDPC by UV irradiation. The enzymatic treatment of the galactose-containing liposomes with galactose oxidase resulted in the formation of aldehyde groups on the liposome surface. By the subsequent mixing of the liposome suspension with the amino group-containing liposome suspension, a rapid increase in turbidity was observed due to the formation of Schiff bases between the aldehyde groups and the amino groups at the interface of the liposomes. The rate of turbidity change strongly depended on the degree of polymerization of MEGal, the surface densities of galactose and amino groups on the liposome, the distance from the liposome surface to amino end groups, and the flexibility and deformability of the liposomes.
Catalytic properties of galactose oxidase to liposome-forming amphiphiles which have many pendent galactose residues.
A galactose-carrying vinyl monomer [2-(methacryloyloxy)ethyl beta-D-galactopyranoside, MEGal] was polymerized by using a lipophilic radical initiator. The amphiphiles obtained (DODA-PMEGal) formed stable liposomes by mixing with phospholipids, and the galactose residues on the liposome surface were effectively recognized and oxidized by galactose oxidase. The affinity (estimated by the 1/Km value) of galactose oxidase for the galactose residues on the liposomes was higher than those for free galactose and MEGal and dependent on the length of galactose-carrying polymer chains on the liposome surface and the fluidity of the membranes, whereas not significantly influenced by the surface density of galactose residues on the liposomes. The affinity of galactose oxidase for the galactose-carrying linear polymers, which were prepared by using an ordinary azo-type radical initiator and a chain-transfer reagent, was also higher than those for free galactose and MEGal and dependent on the degree of polymerization of MEGal. The affinity was, however, relatively much smaller than those for DODA-PMEGals incorporated in liposomes.