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Biomedical subjects

M Kuriyama

Publications and source records attributed to M Kuriyama.

At least 19 recordsLinked to original sources
PubMed1997

Plasma lipoprotein(a) levels and expression of the apolipoprotein(a) gene are dependent on the nucleotide polymorphisms in its 5'-flanking region.

The apolipoprotein(a) (apo[a]) gene encodes a protein component of lipoprotein(a) [Lp(a)] whose plasma levels vary widely among individuals. Hyper-Lp(a)-emia constitutes a risk factor for thromboembolic disease. We previously subclassified the apo(a) gene into four allelic types (A-D) by polymorphisms in the 5'-flanking region. To elucidate whether these polymorphisms affect the expression of apo(a), we measured plasma Lp(a) concentrations in vivo by ELISA and examined expression of the gene by an in vitro assay using its 5'-flanking region. Homozygotes of type C had significantly higher Lp(a) levels than those of type D. The relative expression of type C was also about three times higher than that of type D, which was consistent with the in vivo results. Deletion analysis revealed that the substitution of C by T (+93) led to negative regulation in expression of the gene, while the change of G to A (+121) led to positive regulation. These results indicate that the polymorphisms in the 5'-flanking region of the apo(a) gene affect the efficiency of its expression and, in part, play a role in regulating plasma Lp(a) levels.

Alleles↗
PubMed1997

Mucolipidosis III (pseudo-Hurler polydystrophy); clinical studies in aged patients in one family.

Three adult patients (38-year-old male, 86-year-old female, and 61-year-old male) in a family with mucolipidosis III (ML-III) were described. They had characteristic features of ML-III and they survived a long time. N-acetylglucosaminyl 1-phosphotransferase activity was low in fibroblasts of a patient, but its residual activity remained at a relatively high level (24.5-35.3% of controls), which may explain the benign clinical course. Odontoid dysplasia and atlanto-axial dislocation was found in one patient, and surgical treatment improved his physical disability. Bilateral carpal tunnel syndrome as well as claw hand deformities were common in all of the patients. The clinical manifestations were important for the diagnosis and the management of the patients.

Acetylglucosaminidase↗
PubMed1997

Evaluation of fulminant hepatic failure by scintigraphy with technetium-99m-GSA.

UNLABELLED: We evaluated the usefulness of hepatic receptor imaging with 99mTc-diethylenetriaminepentaacetic acid galactosyl human serum albumin (GSA) to establish the diagnosis and prognosis of fulminant hepatic failure (FHF). METHODS: Of the 20 patients, 8 had acute hepatitis and 12 had FHF. Computer acquisition of gamma-camera data started just before the injection of 185 MBq 99mTc-GSA and stopped 20 min later. Time-activity curves for the heart and liver were generated from regions of interest (ROIs) for the whole liver and precordium. A receptor index was calculated by division of the radioactivity of the liver ROI by that of the liver plus heart ROIs 15 min after the injection. An index of blood clearance was calculated by division of the radioactivity of the heart ROI at 15 min by that of the heart ROI 5 min after the injection. RESULTS: The receptor index was less than 0.83 in all patients with FHF, but it was more than 0.83 in all patients with acute hepatitis. The index of blood clearance was more than 0.72 in all patients with FHF but less than 0.72 in all patients with acute hepatitis. All six survivors of FHF had receptor indices of 0.58 or more, but in five of the six patients who later died, the receptor index was 0.58 or less. The index of blood clearance was 0.85 or less in all survivors but 0.85 or more in the same five patients who later died. CONCLUSION: Hepatic receptor imaging with 99mTc-GSA facilitated the evaluation of hepatic function reserve and was useful in establishing the diagnosis and prognosis of FHF.

Acute Disease↗
PubMed1996

Identification of AF-6 and canoe as putative targets for Ras.

Ras (Ha-Ras, Ki-Ras, N-Ras) is implicated in the regulation of various cell functions such as gene expression and cell proliferation downstream from specific extracellular signals. Here, we partially purified a Ras-interacting protein with molecular mass of about 180 kDa (p180) from bovine brain membrane extract by glutathione S-transferase (GST)-Ha-Ras affinity column chromatography. This protein bound to the GTP gamma S (guanosine 5'-(3-O-thio)triphosphate, a nonhydrolyzable GTP analog).GST-Ha-Ras affinity column but not to those containing GDP.GST-Ha-Ras or GTP gamma S.GST-Ha-Ras with a mutation in the effector domain (Ha-RasA38). The amino acid sequences of the peptides derived from p180 were almost identical to those of human AF-6 that is identified as the fusion partner of the ALL-1 protein. The ALL-1/AF-6 chimeric protein is the critical product of the t (6:11) abnormality associated with some human leukemia. AF-6 has a GLGF/Dlg homology repeat (DHR) motif and shows a high degree of sequence similarity with Drosophila Canoe, which is assumed to function downstream from Notch in a common developmental pathway. The recombinant N-terminal domain of AF-6 and Canoe specifically interacted with GTP gamma S.GST-Ha-Ras. The known Ras target c-Raf-1 inhibited the interaction of AF-6 with GTP gamma S.GST-Ha-Ras. These results indicate that AF-6 and Canoe are putative targets for Ras.

Amino Acid Sequence↗
PubMed1996

Choroideremia with leukoencephalopathy and arylsulfatase A pseudodeficiency.

A 33-year-old male patient was admitted to our hospital because of progressive gait disturbance and involuntary movement of the neck. He showed choroideremia, distal motor neuropathy, and leukoencephalopathy on T2-weighted brain magnetic resonance imaging (MRI). Choroideremia is a rare X-linked, progressive, degenerative disease of retina and choroid. There have been some reports of choroideremia patients with neurological complications. Recent studies have assigned its genetic locus to a small segment of Xq21.3 and it encodes a protein that resembles component A of rat Rab geranyl-geranyl transferase, a protein essential for cell function. This patient did not have the reported genetic abnormalities for choroideremia. Known disorders causing leukoencephalopathy were not detected except for a partial deficiency of arylsulfatase A (17.3% of normal controls in lymphocytes and 13.7% in fibroblasts). Deficiency of arylsulfatase A activity occurs in the late infantile, juvenile, and adult forms of metachromatic leukodystrophy (MLD) which is also an inherited disorder of myelin metabolism, but because of its unstability, it occurs in normal individuals and in patients with other neurological diseases. Consequently, we suspect that this patient had partial deficiency of arylsulfatase A and choroideremia as predisposing factors for white matter degeneration.

Adult↗
PubMed1996

Cytotoxin and urease activities of Helicobacter pylori isolates from Japanese patients with atrophic gastritis or duodenal ulcer.

The vacuolating cytotoxin and urease secreted by Helicobacter pylori are thought to be virulent factors. Because vacuolation is potentiated by the presence of ammonium ion, which is produced by urease in vitro, it is of interest to examine whether cytotoxin and urease work reciprocally in the development of atrophic gastritis or duodenal ulcer. In the present study, patients (all H. pylori-positive) were divided into four groups: mild atrophic gastritis (group 1; nine patients), severe atrophic gastritis (group 2; 36 patients), duodenal ulcer with mild atrophic gastritis (group 3; 19 patients) and duodenal ulcer with severe atrophic gastritis (group 4; 12 patients). Cytotoxin production and urease activity of H. pylori isolated from these patients were analysed. Cytotoxin production was observed in four of nine (44.4%), 28 of 36 (77.8%), 11 of 19 (57.9%) and eight of 12 (66.7%) isolates from groups 1, 2, 3 and 4, respectively. Cytotoxin-producing H. pylori isolates were found significantly more in patients with severe atrophy than in patients with mild atrophy (P = 0.048). The mean of relative activity of cytotoxin in H. pylori isolate was 1.6 +/- 2.3, 7.9 +/- 7.4, 5.8 +/- 6.0 and 9.0 +/- 9.1 in groups 1, 2, 3 and 4, respectively. Helicobacter pylori isolates from severe atrophy or duodenal ulcer patients in groups 2 or 4 possessed significantly higher activity than those from non-ulcer patients in group 1 (P = 0.017 and 0.030, respectively). The mean of urease activity was 8.6 +/- 4.6, 10.0 +/- 5.9, 10.0 +/- 8.5 and 11.2 +/- 7.7 IU/mg in groups 1, 2, 3 and 4, respectively. These differences indicated no statistical significance. In each H. pylori isolate, the production of cytotoxin and urease were independent, which indicated that there was no reciprocal effect between them in vivo. Thus, cytotoxin-producing H. pylori isolates were more prevalent in patients with severe atrophic gastritis and the cytotoxin activities of H. pylori isolates from the patients with severe atrophic gastritis or duodenal ulcer were much higher than those from the patients with mild atrophic gastritis, which suggested that vacuolating cytotoxin may be a disease-inducing factor.

Bacterial Proteins↗
PubMed1996

[The effect of recombinant human colony stimulating factor (G-CSF) on the function of human blood and urinary leukocytes].

To study the function of leukocyte in urinary tract infection (UTI), the effect of granulocyte colony-stimulating factor (G-CSF) and various osmolarities on the function were studied. The superoxide production of blood leukocytes increased by treatment of G-CSF with its dose dependency. This effect was not observed in urinary leukocytes. This increased function was well preserved even in higher osmotic atmosphere when previously treated by the G-CSF. Therefore, urinary leukocyte activated by G-CSF and migrating from the blood through the tissue may still have its function and work in the patients with UTI.

Granulocyte Colony-Stimulating Factor↗
PubMed1996

Profile of Helicobacter pylori cytotoxin derived from two areas of Japan with different prevalence of atrophic gastritis.

BACKGROUND AND AIM: To clarify the roles of Helicobacter pylori cytotoxin in gastric atrophy, the cytotoxin positive rate and cytotoxin activity in Fukui and Okinawa, where the prevalence of atrophic gastritis and gastric cancer risk are quite different, were studied. MATERIALS: Seventy three strains from Fukui and 51 from Okinawa were examined. METHODS: The validation of atrophy was done by endoscopy, being confirmed with histology. The supernatant of liquid H pylori culture media was concentrated 20-fold, serially diluted, using doubling dilutions, and scored from 1 to 8. The semi-quantitated cytotoxin activity was expressed as the maximum dilution score yielding > 50% A431 cell vacuolation, being standardised with bacterial density. RESULTS: The cytotoxin activity of the strains from Fukui was highly diverse compared with that from Okinawa, although the cytotoxin positive rate was not different. In Fukui strains, the grade of atrophy and the cytotoxin activity were correlated (p < 0.05). In addition, the cytotoxin activity of the strains from all patients in Okinawa, most of whom showed closed-type/mild atrophy, was significantly lower than that of the strains from the patients with open-type/severe atrophy in Fukui (6.46 (5.53) v 9.76 (8.80), p < 0.05), (mean (SEM)). CONCLUSION: The difference in profile of the cytotoxin activity in the two areas was related to the difference in the prevalence of atrophic gastritis.

Adult↗
PubMed1996

Pneumonitis during interferon and/or herbal drug therapy in patients with chronic active hepatitis.

We report four cases of acute pneumonitis due either to interferon, or a herbal drug, "Sho-saiko-to", or both in combination, in patients with chronic active hepatitis, focusing on its pathogenesis and response to prednisolone therapy. These cases shared common clinical features: fever, dry cough, dyspnoea, hypoxaemia, diffuse infiltrates both on chest radiography and chest computed tomography, restrictive pulmonary functional impairment, and alveolitis on examination of transbronchial lung biopsy, all of which suggest acute interstitial pneumonia. Furthermore, lymphocytosis was observed in association with the dominant CD8+ T-cell subset in bronchoalveolar lavage fluid. A lymphocyte stimulation test using peripheral blood was positive to interferon in one case and to Sho-saiko-to in another. All patients responded to oral prednisolone therapy. Peripheral soluble interleukin-2 receptor levels decreased in parallel with improvement in the clinical course. All patients were free of symptoms with a follow-up of 1-3 yrs. We conclude that interferon- and/or Sho-saiko-to-induced acute pneumonitis may be due to allergic-immunological mechanisms rather than toxicity, and that peripheral levels of soluble interleukin-2 receptor appear to be good markers of disease activity.

Adult↗
PubMed1996

Study of hereditary cerebellar degeneration in cats.

OBJECTIVE: To elucidate the nature of ataxia observed in 3 cats spanning 2 generations. DESIGN: Experimental breeding was attempted to confirm heritability of the disease and establish the mode of inheritance; the original 3 cats and their offspring were studied. ANIMALS: Seven diseased cats spanning 3 generations and 11 neurologically normal cats. PROCEDURE: Cats were examined by use of the following methods: clinical observation, hematologic and serum biochemical examinations, neurologic examination, electrodiagnostics, magnetic resonance imaging, lysosomal enzyme activity assay, horizontal transmission test, and virologic and pathologic examinations. RESULTS: All kittens (1 male and 3 females) obtained by backcrosses developed pure cerebellar dysfunction from the age of 7 to 8 weeks onward. It became progressively worse, but not fatal, between 1 and 2.5 months. Prenatal or perinatal infection with feline panleukopenia virus, inherited lysosomal storage diseases, including gangliosidosis and mannosidosis, and feline hereditary neuroaxonal dystrophy were excluded. Magnetic resonance imaging indicated that size of the cerebellum of diseased cats was markedly reduced. Cerebellar cortical degeneration, especially with extensive destruction of Purkinje cells, was observed microscopically. CONCLUSION: The disease was concluded to be cerebellar degeneration of a new clinical form in cats having an autosomal recessive mode of inheritance. CLINICAL RELEVANCE: When cerebellar dysfunction is diagnosed in a cat, hereditary cerebellar degeneration of this type should be considered in the differential diagnosis.

Animals↗
PubMed1996

[Tumor markers in urological malignancies].

Present status of tumor markers in urological malignancies for diagnosis and follow-up was reviewed. Although many researches have been performed, specific tumor markers in kidney, urothelium and penis cancers have not identified. In testicular tumors, AFP and beta-subunit of HCG are widely used. Especially, using biological half time, these substances are very useful in the judgement of presence of residual tumor or tumor recurrence. In prostate cancer, the determination of PSA has been confirmed to be the most useful tumor marker in solid tumor. World standardization of PSA assays and evaluation of PSA subtypes are necessary.

Biomarkers, Tumor↗
PubMed1996

[Unilateral multicystic dysplastic kidney in an adult: report of a case].

A case of unilateral multicystic dysplastic kidney in a 52-year-old woman is reported. Two abnormal round-like calcifications in left upper quadrant abdomen were found on upper gastrointestinal roentgengraphy for health screening. The computerized tomographic (CT) scan revealed a very small left kidney which was not enhanced and the ring and diffuse calcifications in it. It was not visualized by drip infusion pyelography. Left nephrectomy was performed. The kidney, which was very small, had two cysts the walls of which were calcified. The left ureter and renal vessels were absent. Histological appearance shows typical multicystic dysplastic kidney (MCDK). Literature is reviewed. However, the therapy for MCDK is still controversial.

Calcinosis↗
PubMed1996

[Clinical analysis of 683 prostatic cancer patients in the Tokai Urological Cancer Registry].

Of 815 patients with prostatic carcinoma registered in Tokai Urological Cancer Registry between 1989 and 1991, 683 patients followed up until April to July 1992 were analyzed. The patients were between 48 and 94 years old (mean 73.2). The clinical stage was A in 96 (14.1%), B in 118 (17.3%), C in 117 (17.1%) and D in 352 (51.5%) patients. The histological study well differentiated adenocarcinoma in 150 (22%) patients, moderately differentiated adenocarcinoma in 305 (44.6%), and poorly differentiated adenocarcinoma in 194 (28.4%) patients. There were 12 undifferentiated adenocarcinomas and 22 were not classified. The incidence of patients with poorly differentiated adenocarcinoma increased with the progression of clinical stage. Hormonal therapy was the main treatment (92.8%) and a variety of hormonal therapies with surgery or chemotherapy were attempted through out the clinical stages. Overall survival rate at 5 years was 48.6%. The 5-year survival rage for stage A, B, C and D carcinoma was 84.7, 93.1, 51.8 and 30.3%, respectively. Significant differences in the survival of patients were noted among stage A or B, stage C and stage D (Logrank test: p < 0.0001). The 5-year survival rate was 70.2% for well differentiated, 53.0% for moderately differentiated, and 32.6% for poorly differentiated adenocarcinoma (Logrank test: p < 0.0001). Disease-specific death was observed in 128 patients (66%), and cardio- or cerebrovascular death accounted for 16 (8%) deaths.

Adenocarcinoma↗
PubMed1996

[Prognosis in stage a prostate cancer].

In 39 hospitals in the Tokai region of Japan, 815 cases with prostatic cancer were registered between 1988 and 1991, 91 of which (13.3%) were diagnosed as stage A. Eighty cases of stage A cancer were subclassified into stage A1 (33 cases) or stage A2 (47 cases). A detailed investigation was performed on these 80 subclassified cases with respect to tumor markers, treatment methods and prognosis. The tumor marker, prostate specific antigen (PSA) was positive in 18.5% of the A1 cases and 53.8% of the A2 cases. Endocrine therapy was administered in 45.5% of the A1 and 78.7% of the A2 cases. There was 1 (3.0%) case of progression in the stage A1 group, and 2 cases in stage A2 group. The 5-year survival rate was 97.0% for stage A1 and 88.6% for stage A2. The prognosis of prostatic cancer in stage A was fairly good in both the A1 and A2 subclasses in our series.

Aged↗
PubMed1995

Secretion of active human lysozyme by Acremonium chrysogenum using a Fusarium alkaline protease promoter system.

We constructed expression vectors for Acremonium chrysogenum using a Fusarium alkaline protease promoter region and tested their potential as secretion systems for foreign proteins using the human (h)-lysozyme gene as an indicator. The gene encoding h-lysozyme was linked to the coding region of (1) the carboxy terminal of the alkaline protease pre peptide, (2) the carboxy terminal of the prepro peptide, (3) three amino acids of the mature protein preceded by the prepro peptide and (4) the carboxy terminal of chicken lysozyme signal peptide, inserted into the genomic DNAs of A. chrysogenum and expressed under the control of the alkaline protease promoter. The transformants of A. chrysogenum with each of these plasmids secreted enzymatically active h-lysozyme. A maximum yield in excess of 40 mg l-1 was obtained when h-lysozyme was linked to the carboxy terminal of alkaline protease prepro peptide. The majority of the amino terminal sequence of the purified h-lysozyme from the culture supernatant was identical with that of authentic h-lysozyme, but it showed some heterogeneity.

Acremonium↗
PubMed1995

An atypical variant of Fabry's disease in men with left ventricular hypertrophy.

BACKGROUND: Fabry's disease is considered very rare. Left ventricular hypertrophy is one of the common manifestations in adults with classic hemizygous disease. Recently, several cases of an atypical variant of hemizygous Fabry's disease, with manifestations limited to the heart, have been reported. Therefore, we assessed the incidence of hemizygosity for Fabry's disease among male patients with left ventricular hypertrophy. METHODS: We measured plasma alpha-galactosidase activity in 230 consecutive male patients with left ventricular hypertrophy. Clinical manifestations were assessed, endomyocardial biopsies were performed, and the patients were screened for mutations in the alpha-galactosidase gene. RESULTS: Seven of the 230 patients with left ventricular hypertrophy (3 percent) had low plasma alpha-galactosidase activity (0.4 to 1.2 nmol per hour per milliliter; 4 to 14 percent of the mean value in normal controls). These seven unrelated patients, ranging in age from 55 to 72 years, did not have angiokeratoma, acroparesthesias, hypohidrosis, or corneal opacities, which are typical manifestations of Fabry's disease. Endomyocardial biopsy was performed in five patients and revealed marked sarcoplasmic vacuolization in all five. Samples from four patients were examined by electron microscopy and revealed typical lysosomal inclusions with a concentric lamellar configuration in all four. Two patients had novel missense mutations in exon 1 (Ala20Pro) and exon 6 (Met296lle). The remaining five had no mutations in the coding region of the alpha-galactosidase gene, but the amounts of the alpha-galactosidase messenger RNA were markedly lower than normal. CONCLUSIONS: Seven unrelated patients with atypical variants of hemizygous Fabry's disease were found among 230 men with left ventricular hypertrophy (3 percent). Fabry's disease should be considered as a cause of unexplained left ventricular hypertrophy.

Adolescent↗