Methylmalonic aciduria with pathological fracture.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to F Inoue.
Explore the source record for details and available documents.
The lithocholic acid-sensitive mutant cells were isolated from CHO-9 cells mutagenized with ethylmethane sulfonate, through clonal selection on 96-well dishes. The isolated mutant cells (LSJ-11) were 3-fold more sensitive than controls in the survival to lithocholic acid and were also sensitive to other bile acids such as deoxycholic acid and cholic acid. As a preliminary characterization, it was found that the mutant cells were also sensitive to cytoskeleton-disturbing agents such as colchicine, colcemide, taxol and cytochalasin B. This is the first observation that a bile acid-sensitive mutant was isolated and was partially characterized.
Administration of monosodium L-glutamate (MSG) to neonatal mice produces a hypothalamic syndrome consisting of stunted growth and later development of obesity. We assayed plasma insulin (IRI), thyroxine (T4) and insulin-like growth factor-I (IGF-I) to investigate their roles in the growth of the mice. Two mg/g body weight of MSG was injected into newborn male mice daily for five successive days after birth. Plasma IRI levels were increased on the after 8 weeks of age in MSG-treated mice. There was no significant difference between the plasma T4 levels in MSG-treated mice and those in controls at any age studied. In contrast to this, plasma IGF-I levels in MSG-treated mice were reduced at one week and after. These results suggest that a decreased plasma IGF-I level contributes to the retarded linear growth which develops soon after the administration of MSG, and hyperinsulinemia contributes to the later development of obesity in MSG-treated mice.
The tracheal tumor is uncommon, and tracheal inflammatory pseudotumor seems to be rare with only four cases reported in the Japanese literature. We here in report a case of tracheal inflammatory pseudotumor in a 61-year-old Japanese man presenting with dyspnea and wheezing 2 weeks after a sub total gastrectomy Billroth I for gastric cancer in april 1991. At bronchofiberscopic examination a rounded tracheal mass 40 mm distal to the vocal cord was found. High vascular on the surface and obstruction of about 90% of the tracheal lumen was noticed. Surgery was attempted immediately and 25 mm of the trachea was excised. The histopathological findings revealed an inflammatory pseudotumor. We concluded that the tumor was caused by injury during tracheal intubation.
Opioid receptors solubilized from rat brain membranes with digitonin were partially purified with a newly prepared affinity resin, AF-Amino Toyopearl, coupled with a mu-antagonist Tyr-Pro-Tyr-Tyr at the C-terminus of the peptide. The purified materials were reconstituted with an inhibitory GTP-binding protein (Gi) in liposome. From displacement analyses, two binding states, with a high and a low affinities for the mu-agonist [D-Ala2,Me-Phe4,Gly-ol5]enkephalin, were observed in the reconstituted system with Gi, only a low-affinity state was observed in the reconstituted system without Gi. The results suggested that the purified materials contained the mu-opioid receptors and could functionally couple with Gi as observed in the cell membranes.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
A 3 year old girl was admitted to hospital in an emaciated condition and with polydipsia in October 1974. Following the diagnosis of diabetes mellitus, she received treatment with insulin. On the first admission, a systolic murmur was noted at the apex of the heart. In 1981, the murmur was found to be continuous with a systolic click, and echocardiography demonstrated a mitral valve prolapse. In 1982, electrocardiography revealed left ventricular hypertrophy, and the patient's X-ray showed vertebral kyphoscoliosis. Ophthalmological examination revealed slightly impaired visual acuity and a mild case of cataracts in 1986. The patient grew to be tall and thin with arachnodactylia of the hands, fingers, feet and toes. These symptoms and findings were compatible with Marfan syndrome, although the ophthalmological findings are not specific for this disease. This patient is the first case in Japan of Marfan syndrome associated with insulin-dependent diabetes mellitus, although the relation between Marfan syndrome and IDDM remains unclear.
A case of Mycoplasma pneumonia complicated with severe hemolytic anemia, which occurred as a result of a high titer of cold agglutinin is presented. A 49 year-old male was admitted because of fever, jaundice and dyspnea. Chest x-ray showed diffuse small nodular infiltrates throughout both lung fields. Laboratory studies disclosed the following values: Hb 4.6 g/dl, Ht 13.9%, reticulocyte 11.5%, direct and indirect Coombs' test positive, haptoglobin 38 mg/dl, ESR 145 mm/hr, cold agglutinin titer 1:2,048 mycoplasma antibody titer 1:640, PPD negative. The diagnosis of autoimmune hemolytic anemia associated with Mycoplasma pneumonia was made, and treatment with minocycline and prednisolone observed striking clinical improvement. It was suggested that the cold exposure was possibly a major factor in the pathogenesis of hemolysis in this patient.
A case of primary malignant lymphoma of the breast in a 52 year old woman is described herein. She presented with a painless tumor of the right breast, which was elastic-hard and measured 2 X 1 cm, although there was no lymphadenopathy noted in the axilla or any other regions. Both the biopsy carried out prior to surgery and frozen sections revealed evidence of malignancy, however, histologically, it was difficult to differentiate between malignant lymphoma and carcinoma until staining with the monoclonal antibody MB1 was performed. This allowed a diagnosis of B cell malignant lymphoma of the diffuse large-cell type to be made. The patient remains alive and well 24 months after her mastectomy.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
In order to elucidate the relation between the hepatotoxicity of salicylate (SA) and the pathogenesis of Reye's syndrome (RS), urea production, gluconeogenesis and ketogenesis were investigated in isolated perfused rat livers in the presence of salicylate (SA) and oleate. Although urea formation from 0.5 mM NH4Cl, 2 mM ornithine and 0.3 mM oleate was not inhibited by infusion of SA, 3 mM SA caused a 26% decrease of ketogenesis, 85% decrease of 3-hydroxybutyrate/acetoacetate ratio (30HB/AcAc) and 45% increase of oxygen consumption. Glucose production from 2 mM pyruvate in the presence of 0.3 mM oleate decreased by 33% after administration of 3 mM SA, and 30HB/AcAc ratio also decreased by 33%. The decrement of gluconeogenesis and that of the 30HB/AcAc ratio were very close. These results suggested that ATP production was maintained but that the intra-mitochondrial redox state was changed to a more oxidized state after SA administration in perfused rat livers. This change in redox state could be responsible for the decrease of gluconeogenesis. Metabolic characteristics found in RS were not obtained by infusion of 3 mM SA and 0.3 mM oleate in rat livers. Therefore, some other factors in addition to SA seem necessary to establish an animal model of RS.
Digitonin-solubilized opioid receptors from rat brain were purified with an affinity resin, AH-Sepharose coupled with [D-Ala2, D-Leu5]enkephalin (DADLE). Radioreceptor binding assay showed that the purified materials had specific opioid-binding activity of 310 pmol/mg protein on DADLE binding. Analyses by polyacrylamide gel electrophoresis in the presence of sodium dodecyl sulfate (SDS-PAGE) revealed that the materials were rich in two polypeptides; the major component had a molecular weight of 62000-64000. To establish the materials responsible for binding opiates, the purified materials were cross-linked with 125I-labeled beta-endorphin using bis[2-(succinimidooxycarbonyloxy)-ethyl]sulfone as a cross-linker. The molecular weight of 62000-64000, the major band of the purified materials on SDS-PAGE, agreed closely with that determined by the cross-linking experiment. The results suggest that the purified materials contained opioid-binding materials (opioid receptors).
Formation of free radicals from aflatoxin B1 was demonstrated in systems of a) NADPH/microsome, b) H2O2/peroxidase, c) UV irradiation, d) silver oxide oxidation and e) alkaline dimethyl sulfoxide. In a) to c), radical spots were detected in thin-layer chromatograms. Among several known metabolites of aflatoxin B1, aflatoxin M1 was active in generation of free radical. These results suggest a possible role of free radical metabolites in the cytotoxicity of this hepatocarcinogen.
We performed a genotypic study on lymphoid cells from a patient with adult T-cell leukemia (ATL). Clonal proliferation of human lymphotropic virus type-1(HTLV-1)-infected helper T-cells was detected in the primary skin tumors, while no clonality of the virus-infected lymphocytes was observed in the peripheral blood. Following intensive chemotherapy, however, we detected the presence of two genotypically different HTLV-1-infected lymphocyte clones in different samples taken from the peripheral blood. These data demonstrated the exchange of the dominant neoplastic clone in the clinical course of ATL, suggesting the possibility that a certain clone among many repertoires of HTLV-1-infected T-cells has a capability of leukemogenesis, instead of the primary tumour cells.
A 37-year-old male was admitted to hospital for jaundice and ascites. An ultrasonogram revealed a hypoechoic mass located in the right anterior inferior segment. A needle specimen of the liver was taken, and its biopsy led to a histological diagnosis of a hepatocellular carcinoma. The patient subsequently underwent an operation wherein it was found that the resected tumor that was taken was not clearly demarcated from the cirrhotic parenchyma. The histological diagnosis was an adenomatous hyperplasia. In a portion of this adenomatous hyperplasia, highly consistent hepatic cells and pseudogland formations were seen, confirming the diagnosis of a well-differentiated hepatocellular carcinoma within an adenomatous hyperplasia.
Three siblings with neonatal familial hyperparathyroidism diagnosed at age 4 months, 2 months, and 5 days, respectively, were treated. Hypercalciuria, nephrocalcinosis, and renal tubular acidosis were present in each child. In all three, there were higher responses of serum parathyroid hormone to serum calcium and higher elevation of serum calcium with oral calcium loading. The metabolism of vitamin D and calcitonin seemed to be intact. Hypercalcemia associated with the abnormal response of parathyroid hormone secretion disappeared when the children passed the age of approximately 2 years, although renal tubular acidosis and nephrocalcinosis remained. An autosomal recessive inheritance seems likely.