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Biomedical subjects

N Kono

Publications and source records attributed to N Kono.

At least 127 records · Page 7Linked to original sources
PubMed1991

[Early and late operative results of simultaneous aortic valve and ascending aorta replacement].

Thirty-four patients of ascending aortic aneurysm associated with aortic regurgitation were treated with simultaneous aortic valve and ascending aorta replacement utilizing composite graft, until December 1990. Twenty-four patients of the group were diagnosed as Marfan's syndrome and 17 had aortic dissection. For the operative procedure, Bentall's technique were employed in 25 patients and other modifications in nine. Operative death was observed in three cases (8.8%) due to low output syndrome, caused by coronary ostium abnormality, all in Marfan's syndrome. Late death was observed in six including 2 hospital deaths of cerebro-vascular disturbance and sepsis. Other causes of death were rupture of residual aneurysm (in 3) and LOS at reoperation (in 1). Hospital survivors remarkably improved in NYHA class and in cardiac size. Actuarial survival in 3, 5, 7, and 10 years were 78%, 72%, 72%, and 62% respectively. Therefore, surgical result of composite graft technique in our institution proved to be reasonable as others. However, long term result of the procedure should be carefully evaluated, because of the anatomical and histopathological peculiarity of the disease.

Adult↗
PubMed1991

[Hemochromatosis].

Explore the source record for details and available documents.

Diabetes Mellitus↗
PubMed1990

Tissue specificity in expression and alternative RNA splicing of human phosphofructokinase-M and -L genes.

Mode of the expression of phosphofructokinase (PFK) -M and -L genes was examined in various human tissues including muscle, placenta, liver, kidney, pancreas, stomach and reticulocytes. The gross level of mRNA expression of PFK-M and -L genes was estimated by Northern analysis. Polymerase chain reaction was used to detect mRNA expressed at low levels in these tissues. Tissue-specific expression of alternatively spliced PFK-M gene transcripts was also determined by polymerase chain reaction. The results indicated that alternative splicing of PFK-M gene transcripts was controlled in a tissue-specific manner.

Base Sequence↗
PubMed1990

Genetic defect in muscle phosphofructokinase deficiency. Abnormal splicing of the muscle phosphofructokinase gene due to a point mutation at the 5'-splice site.

The genetic defect in muscle phosphofructokinase deficiency (type VII glycogenosis, Tarui disease) was investigated. Six cDNAs for muscle phosphofructokinase, including a full-length clone, were isolated from a non-amplified library of muscle from a patient. By sequence analysis of these clones, a 75-base in-frame deletion was identified. The rest of the sequence was identical to that of the normal cDNA, except for a silent base transition at position 516 (ACT (Thr) to ACC (Thr]. The deletion was located in the 3'-terminal region of exon 13 (numbered with reference to the rabbit muscle phosphofructokinase gene (Lee, C.-P., Kao, M.-C., French, B.A., Putney, S.D., and Chang, S.H. (1987) J. Biol. Chem. 262, 4195-4199]. Genomic DNA of the patient was amplified by polymerase chain reaction. Sequence analysis of the amplified DNA revealed a point mutation from G to T at the 5'-end of intron 13. This mutation changed the normal 5'-splice site of CAG:GTATGG to CAG:TTATGG. A cryptic splice site of ACT:GTGAGG located 75 bases upstream from the normal splice site was recognized and spliced in the patient.

Base Sequence↗
PubMed1990

Decreased xanthine oxidase activities and increased urinary oxypurines in heterozygotes for hereditary xanthinuria.

Two brothers with hereditary xanthinuria (xanthine oxidase deficiency) and several members of their family were studied. In both subjects, plasma and urinary concentrations of uric acid were low whereas xanthine and hypoxanthine concentrations were markedly elevated. Xanthine oxidase activity was virtually absent in the patients' duodenal mucosa, a finding that established the diagnosis of hereditary xanthinuria. In their parents (obligate heterozygotes), the duodenal xanthine oxidase activity was about 50% of that in control subjects (father 9.3 and mother 12.8 mU/g tissue compared with 21.3 +/- 5.0 mU/g tissue, mean +/- SD). The residual xanthine oxidase from the parents exhibited normal kinetics with respect to hypoxanthine. The parents' urinary xanthine and hypoxanthine concentrations were significantly greater than those of control subjects, while their plasma concentrations of oxypurines were normal. Similar findings were observed in at least 6 other relatives, a finding that suggested that they were heterozygotes. This study suggests that obligate hereditary xanthinuria heterozygotes have only 50% of the xanthine oxidase activity of controls; this deficiency results in a partial metabolic blockage at this enzymatic step in heterozygotes.

Adult↗
PubMed1990

Evidence for alternative RNA splicing and possible alternative promoters in the human muscle phosphofructokinase gene at the 5' untranslated region.

Three mRNA species for human muscle phophofructokinase containing heterogeneous 5' untranslated sequences were identified through cDNA cloning. Type A mRNA was essentially the same as that reported previously (Nakajima, H., et al. (1987) FEBS Lett. 223, 113). Type B mRNA was considered to be the major gene product, which contained an extra non-coding sequence within the 5' untranslated region of type A mRNA. Amplification of mRNA by polymerase chain reaction revealed that types A and B mRNAs shared a common precursor RNA, and were alternatively spliced. Type C mRNA, homologous to the cDNA sequence from a placenta library (Sharma, P. M. et al., (1989) Gene 77, 177), was considered to be under the control of an alternative promotor.

Base Sequence↗
PubMed1990

Glucose infusion abolishes the excessive ATP degradation in working muscles of a patient with McArdle's disease.

A 23-year-old woman with McArdle's disease performed mild leg exercise on a bicycle ergometer. Saline or 10% glucose solution was infused throughout exercise. After exercise with saline infusion, her plasma concentrations of ammonia, hypoxanthine and creatine kinase increased greatly. Conversely, after exercise with glucose infusion, there were no appreciable changes in these plasma substances. In addition, she noticed that glucose infusion relieved her from muscle symptoms during exercise. These findings suggest that glucose infusion to patients with McArdle's disease ameliorates excessive ATP degradation in exercising muscles.

Adenosine Triphosphate↗
PubMed1990

Preventive effect of a new immunosuppressant FK-506 on insulitis and diabetes in non-obese diabetic mice.

We investigated the effect of an immunosuppressant FK-506 on histological change of islets, the onset of diabetes, and the change of spleen cell subsets in female non-obese diabetic mice. Mice administered intraperitoneally with FK-506 from 5 to 20 weeks of age showed marked suppression of mononuclear cell infiltration (insulitis) at 10 weeks of age. Among the subsets of the spleen cells, a significant decrease in the population of Thyl.2-positive T cells (pan-T), L3T4-positive T cells (mainly helper/inducer), and Lyt2-positive T cells (mainly suppressor/cytotoxic) was observed in FK-506-treated mice. Furthermore, glucose tolerance of the mice at 15 weeks of age was clearly improved. Cumulative incidence observed up to 40 weeks of age was 86% in control mice and 23% in FK-506-treated mice (p less than 0.01). These data indicate that FK-506 has a preventive effect on insulitis and diabetes by the suppression of cell-mediated autoimmunity in non-obese diabetic mice.

Animals↗
PubMed1990

Biliary sludge and microcalculi in intrahepatic bile ducts. Morphologic and X-ray microanalytical observations in 18 among 1,179 consecutively autopsied livers.

To clarify the morphology and pathogenesis of intrahepatic calculi in the incipient stage, we examined biliary sludge and microcalculi in intrahepatic bile ducts by morphologic and X-ray microanalytical methods in 18 (1.5%) among 1,179 autopsied livers. The hepatobiliary conditions of these 18 livers were intra- and extrahepatic biliary obstruction in 14 cases, hepatic fibrosis in three cases, and cirrhosis with hepatocellular carcinoma in the remaining one. Grossly, brown-pigmented microcalculi were observed floating in biliary sludge. Microscopically, the biliary sludge was composed of mucin, fibrinous materials, desquamated epithelial cells and a few bilirubin granules. The microcalculi were embedded in the sludge and consisted of mucin and precipitates of bilirubin with a granular, lamellar or amorphous appearance. Bacterial colonies were recognized in both the sludge and microcalculi in all but three cases. Intrahepatic bile ducts harboring sludge and microcalculi showed a minimal to moderate degree of glandular proliferation with mucin production. X-ray microanalysis disclosed that the sludge contained little calcium ion, whereas microcalculi were calcium-rich. These findings suggest that biliary obstruction, bacterial infection and mucin hypersecretion play an important role in the formation of intrahepatic biliary sludge and microcalculi, and that sludge is causally related to the formation of intrahepatic microcalculi. Intrahepatic microcalculi and biliary sludge may represent a pathogenetic sequence in the early stage of calcium bilirubinate hepatolithiasis.

Aged↗
PubMed1990

Anorectal mucinous adenocarcinoma associated with latent perianal Paget's disease.

We report a case (63-yr-old man) of anorectal mucinous adenocarcinoma associated with perianal Paget's disease, found incidentally by pathologic examinations of the resected specimen. Paget's disease resided just distal to the anorectal adenocarcinoma. The area of Paget's disease was rather small. The epidermis containing Paget's cells was continuous with the anorectal adenocarcinoma, as if adenocarcinoma cells invaded directly into the epidermis. The Paget's cells were positive for neutral and acid mucus. Ultrastructurally, Paget's cells contained secretory granules, microvilli, and a few tight junctions, but lacked tonofilaments. The mucinous adenocarcinoma cells also showed histochemical and ultrastructural features that were similar to those of Paget's cells. These findings strongly suggest that the Paget's cells were derived from direct spread from the anorectal adenocarcinoma, in this case. Clinicians and pathologists should carefully examine the perianal epidermis in anorectal carcinoma, even if there are no clinical findings of perianal Paget's disease. The English literature is also reviewed briefly.

Adenocarcinoma, Mucinous↗
PubMed1990

[A case of glycogen storage myopathy with acute heart failure].

A case of 25-year-old woman with glycogen storage myopathy is reported here. She was hospitalized for acute heart failure after alcohol drinking. The electrocardiogram on admission showed marked ST elevation. Laboratory data showed elevated levels of serum myogenic enzymes but no rise in cardiomyogenic enzyme: CK 3862 IU/l CK-MB 35 IU/l, LDH 427 IU/l, GOT 203 IU/l. After several days, she recovered from acute heart failure and could walk without supporting. ST elevation in ECG and elevated myogenic enzymes were also normalized. The occurrence of acute myocardial infarction was ruled out because a coronary angiogram and 99 Tcm scintigram were normal. Physical examination revealed proximal muscular weakness and mental retardation (WAIS, total 72). Venous lactate response was normal after semi-ischemic forearm exercise. PAS staining of muscle specimen showed an excess deposit of glycogen. Ragged-red fibers were not seen on Gomori-trichrome stain. By electron microscopy, a large amount of glycogen particles were demonstrated in the subsarcolemma, but there were no abnormal mitochondrial changes. Biochemical analysis showed accumulation of glycogen in muscles: 28.7 mg/g muscle (normal 11.4 +/- 4.2 mg/g muscle). The activities of enzyme in the pathway of glycogen and glycogenosis (alpha-glucosidase, amylo-1,6-glucosidase, phosphorylase a, phosphorylase kinase, phosphofructokinase, etc.) were within normal limits. The spectrum of glycogen iodine complex was normal. Our case was different from any type of muscle glycogen storage disease previously reported. The etiology of an excess of glycogen deposit in muscles is unknown.

Acute Disease↗
PubMed1990

Ciliated hepatic foregut cyst. A mucus histochemical, immunohistochemical, and ultrastructural study in three cases in comparison with normal bronchi and intrahepatic bile ducts.

We describe herein the morphologies of three cases of ciliated hepatic cysts and compare them with those of normal bronchi and intrahepatic bile ducts. Grossly, the hepatic cysts were located in the subcapsular region. They were solitary, unilocular, and rather small (less than 4 cm in diameter). Histologically, the cyst wall consisted of four layers: pseudostratified ciliated columnar epithelia with mucous cells, subepithelial connective tissue, smooth-muscle bundles, and an outermost fibrous capsule. The epithelial cytoplasm contained neutral, carboxylated, and sulfated mucus. We noted moderate immunoreactivity to keratin, epithelial membrane antigen, carcinoembryonic antigen, DU-PAN-2 and secretory component; weak immunoreactivity to cytokeratin CAM 5.2, cytokeratin AE1 + 3, and carbohydrate antigen 19-9; and faint or negative immunoreactivity to IgA and IgM. Cilia were immunoreactive to actin and tubulin; smooth muscles were immunoreactive to actin and desmin. Ultrastructural observations revealed definite cilia arranged in a 9 + 2 pattern as well as mucous cells. These morphologic features of the hepatic cysts were similar to those of normal bronchi but different from those of normal bile ducts. Our findings suggest that ciliated hepatic cysts arise from the embryonic foregut and differentiated toward bronchial structures in the liver.

Adult↗