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Biomedical subjects

T Kamiya

Publications and source records attributed to T Kamiya.

At least 271 records · Page 15Linked to original sources

Blood clotting factor IX BM Nagoya. Substitution of arginine 180 by tryptophan and its activation by alpha-chymotrypsin and rat mast cell chymase.

Factor IX BM Nagoya (IX Nagoya) is a natural mutant of factor IX responsible for severe hemophilia B. A patient with this mutant is characterized by a markedly prolonged ox brain prothrombin time. IX Nagoya was purified from the patient's plasma by immunoaffinity chromatography with an anti-factor IX monoclonal antibody column. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis showed that treatment of IX Nagoya with factor XIa/Ca2+ resulted in cleavage only at the Arg145-Ala146 bond. Reversed-phase high performance liquid chromatography of a trypsin digest of IX Nagoya showed an aberrant peptide, which was further digested with proteinase Asp-N. Primary structure analysis of one of the Asp-N peptides revealed that Arg180 is replaced by Trp. An essentially complete (99%) amino acid sequence of IX Nagoya was obtained by sequencing fragments derived from a lysyl endopeptidase digest in which no other substitutions in the catalytic triad or substrate binding site were found. We also found that IX Nagoya is activated by alpha-chymotrypsin or rat mast cell chymase by monitoring the rate of factor X activation using a fluorogenic peptide substrate in the presence of factor VIII, phospholipids, and Ca2+. These results indicate that the substitution of Arg180 by Trp impairs the cleavage by factor XIa required for activation of this zymogen and that the substitution causes hemophilia BM.

Amino Acid Sequence↗

Increase in murine monoclonal-antibody-defined urinary antigens in patients with bladder cancer and benign urogenital disease.

Monoclonal antibodies (MAbs) were obtained from hybridoma clones established by cell fusion between P3X63Ag8.653 mouse myeloma cells and spleen cells of mice or rats hyperimmunized against human bladder cancer tissue or BC47 rat bladder cancer cells. RBS-31 and RBS-85 mouse MAbs and RBA-1 rat MAb were raised against BC47 cells and HBP-1 MAb was raised against human bladder cancer tissues. Urinary antigens detected by these MAbs were quantitatively assayed by means of ELISA using 50 microliters of 1:2 diluted urine samples. The cut-off value of the assay was set up as the mean + 4 X SD of the mean using data from the healthy individual urine samples. The reactivity of all healthy control urine samples were under the cut-off value (negative). By contrast, urine from bladder cancer patients reacted positively with the RBS-31 MAb at 72%, with the RBS-85 MAb at 63%, with the RBA-1 MAb at 51% and with the HBP-1 MAb at 35%. The urine samples from some patients with renal calculi, acute cystitis or complicated urinary tract infections showed only a weak reactivity with our MAbs. As for extra-bladder cancers, some patients with renal, renal pelvis, prostate or ureter cancer, but no patients with esophageal, gastric, colon or liver cancer or leukemia, had reactive urinary antigens.

Adult↗

Effect of ethanol on hepatocarcinogenesis initiated in rats with 3'-methyl-4-dimethylaminoazobenzene in the absence of liver injuries.

In this study special care was taken to discriminate between the direct effect of ethanol on hepatocarcinogenesis and secondary effects such as choline deficiency or fatty liver. Rats were divided into 2 groups, D and N. Group D was initiated using 3'-Me-DAB, while the control group N was not initiated. Groups N and D were divided into 4 sub-groups. Each subgroup was given, in the drinking water, one of the following ethanol solutions: 0, 5, 10 or 15% for 45 weeks. Liver tumors were induced only in D groups. Evidence obtained indicates that: (a) in the N group, ethanol did not cause any apparent disorders in histology and lipid metabolism, and (b) in the D group no significant differences were observed in the incidence of HCCs and other liver lesions. Ethanol thus does not appear to enhance hepatocarcinogenesis, at least in the absence of liver injury.

Animals↗

Enzymic and nonenzymic mono ADP-ribosylation of proteins in skeletal muscle.

The acceptors of endogenously catalyzed monoADP-ribosylation in the cell free extract from rat skeletal muscle was searched. The main acceptor proteins in particulate were electrophoretically 52, 80, 100, and greater than 200 kDa proteins in the presence of SDS, while that in cytosol were 36 and 39 kDa proteins. Although no ADP-ribosylation was observed in particulate when the substrate NAD+ was replaced by ADP-ribose, the same ADP-ribose adducts were also formed with higher degree in cytosol. These results indicate that an enzymic and nonenzymic monoADP-ribosylation occur separately in cytosol and particulate. One acceptor, 36 kDa protein, appears to be glyceraldehyde-3-phosphate dehydrogenase.

Adenosine Diphosphate Ribose↗

A new polyvinylchloride blood bag plasticized with less-leachable phthalate ester analogue, di-n-decyl phthalate, for storage of platelets.

To compare changes in platelets stored in the new di-n-decyl phthalate (DnDP)-plasticized polyvinyl chloride (PVC) bag with those in a di-(2-ethylhexyl) phthalate (DEHP)-plasticized PVC bag, single-donor apheresis platelet concentrates (PCs), 133 +/- 11 x 10(7) platelets per ml (n = 7), were stored with 94 +/- 3 ml of plasma in a new 1-liter bag with a surface area of 44 +/- 7.1 cm2 per 10(10) platelets. Oxygen and carbon dioxide gas diffusion properties of PVC-DnDP films were respectively, 1.6 and 2 times those of standard PVC-DEHP films. The amounts of DnDP leaked into the plasma of PCs were low at 0.58 +/- 0.06 mg per bag after 5-day storage, which is about one-eightieth the amount of DEHP leaked. The pH of PCs in PVC-DnDP bags amounted to 6.99 +/- 0.03 after 5-day storage, with glycolysis accelerated somewhat in the new bags. However, the platelet oxygen consumption was no different from that in the PVC-DEHP bags. Platelet aggregation and responses to hypotonic shock were significantly better in the new bags at the end of storage. Shape changes of platelets into spherical forms with dendrites were more frequently observed in PVC-DnDP bags than in PVC-DEHP bags. The study indicated that platelets stored in the new DnDP-plasticized PVC bags have retained aggregation and responses to hypotonic shock more than platelets in the PVC-DEHP bags, but spherical forms and anaerobic metabolism increased in the new bags.

Adenosine Triphosphate↗

A novel second-generation polyolefin container for storage of single-donor apheresis platelets.

A non-blow-molded LE-2 polyolefin (PO) container was developed to store single-donor apheresis platelet concentrates (PCs) processed by the Haemonetics Plasma Collecting System (PCS) at 22 degrees C for over 1 day. We molded PO containers with heat-sealing of light-weight polymer alloy films with a thickness of 0.25 mm. The film was made of polymer blends consisting of polypropylene, styrene ethylene butylene styrene-block copolymer, and ethylene ethylacrylate copolymer. The PO container with enough strength and flexibility in routine practice has 2 and 1.6 times higher oxygen and carbon dioxide gas transfer properties than standard polyvinyl chloride (PVC) plastic containers. PCs (1-1.9 x 10(11) platelets) processed from 450 ml platelet-rich plasma were stored in 0.6-liter PO containers at 22 degrees C with flatbed agitation for up to 6 days. The pH of PCs was well maintained at the mean values of 7.0 in PO containers after 6 days in the well-oxygenated condition. The energy metabolism of stored platelets was determined. Oxygen consumption rates of platelets stored in PO containers averaged 1.5 nmol/min/10(9) platelets. The rates of glucose consumption and lactate production were 0.4 and 0.8 nmol/min/10(11) platelets, respectively. The rates of adenosine triphosphate (ATP) generation of platelets, 9.7 nmol/min/10(9) platelets, in PO containers did not differ from those in the PVC containers. Aggregation responses to adenosine diphosphate and hypotonic shock response of platelets were better maintained in PO containers. The morphological changes into sphere forms with projections and the appearance of unclassified forms were more frequently observed in PO than in PVC containers.(ABSTRACT TRUNCATED AT 250 WORDS)

Blood Component Removal↗

Follow up study of cardiac involvement in Emery-Dreifuss muscular dystrophy.

A patient with Emery-Dreifuss muscular dystrophy was followed up from 9 to 26 years of age. Serial electrocardiographic studies showed changes ranging from early cardiac conduction abnormality as first degree atrioventricular block to sick sinus syndrome. In addition the development of cardiomyopathy was documented by electrocardiographic, echocardiographic and radionuclide studies of the heart.

Bradycardia↗

Right ventricular mechanical and energetic properties.

To formulate right ventricular (RV) mechanical and energetic properties in terms of the time-varying elastance model, Emax and the systolic pressure-volume area (PVA) of RV were measured in the excised cross-circulated heart preparation, while the left ventricle was beating unloaded. Emax of RV was constant, and independent of the RV volume, the stroke volume, the ejection velocity, and the pre-ejection period in the control contractile state. Enhancement of the contractile state with calcium increased Emax, and reduction of the contractile state with propranolol decreased Emax. The whole heart oxygen consumption (Vo2) was linearly regressed on PVA of RV, in both the control and the calcium-enhanced contractile state. Calcium elevated the regression line in a parallel manner. The slope of the regression line was (1.85 +/- 0.19) x 10(-5) ml O2/mmHg ml in the control state, and (1.57 +/- 0.44) in the calcium state. These slope values were similar to those in left ventricle (LV). We therefore conclude that mechanical and energetic properties of RV are similar to those of LV.

Animals↗

[A case of Behçet disease associated with hepatocellular carcinoma].

A 31-year-old man, suffering from Behçet disease, was admitted to our hospital because of a palpable mass in the right hypochondrium. On testing, laboratory data showed an abnormal liver function, positive HBs antigens, and a high AFP level. An abdominal CT and a SCAG revealed large tumors in the liver. The patient died of hepatic failure 6 months after the first admission and an autopsy was performed. At autopsy, hepatocellular carcinomas were found to occupy most of the entire liver. Histologically, the residual liver showed no remarkable changes, such as liver cirrhosis or chronic hepatitis. To date, few cases of Behçet disease complicating a malignant neoplasma have been reported. The possible relationship between Behçet disease and the occurrence of malignant tumors is described with a review of the literature.

Adult↗

Extragenic factor IX gene RFLP is useful for detecting carriers of Japanese hemophilia B.

Seventy-eight X chromosomes from 25 normal Japanese subjects and 22 family members with hemophilia B (coagulation factor IX deficiency) were examined with an extragenic factor IX DNA probe, pX58dIIIc at DXS99 locus. In contrast to the previously described nonpolymorphic RFLPs in the factor IX gene, DXS99 locus RFLP produced by SacI digestion was detected among those Japanese subjects with allelic frequencies of 0.48 and 0.52. The estimated heterozygosity rate of this extragenic RFLP among Japanese females was about 50%. The study of hemophilia B family members showed that DXS99 locus RFLP was informative in 9 out of 13 families tested (69.2%). No recombination events between the factor IX gene locus (F9) and DXS99 locus have been noted among nine families analyzed. DXS99 SacI RFLP is a useful gene indicator of carrier-ship of hemophilia B.

Factor IX↗

[Gallium-67 imaging in patients with myocarditis in childhood and youth].

Gallium-67 (Ga-67) myocardial imaging was performed in 19 patients (1 month to 21 years of age) with proven or suspected myocarditis. The anterior images, 48 hrs after the intravenous administration of 0.5-2.0 mCi Ga-67 citrate, were analysed using a computer. Regions of interest were set on the heart, lungs and background, excluding the sternum and vertebrae. The uptake of Ga-67 was evaluated quantitatively by the ratio of the average count of the heart to the average count of the lung (H/L). When the H/L value was higher than 130% the cardiac uptake of Ga-67 was designated positive. Histological examination was performed in 11 patients who underwent right endomyocardial biopsy. The patients were categorized according to their clinical findings. Group 1 consisted of 11 patients with clinical symptoms of dilated cardiomyopathy, six of whom had arrhythmias or elevated serum CPK-MB levels (group Ia) in the acute stage of the disease, and the remaining five had neither (group Ib). Group 2 consisted of eight patients with dysrhythmias but without heart failure. Four had elevated serum CPK-MB levels (group IIa), but the remaining four did not (group IIb). All patients in the group Ia had positive Ga-67 uptakes, and three patients in the group Ib had negative uptakes. The H/L values were significantly higher in the group Ia than in the group Ib (p less than 0.02).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Isolation right ventricular hypoplasia: report of two cases].

Two cases of the hypoplastic right ventricle are reported. Patient 1: A five-year-old boy underwent cardiac catheterization 13 days after birth which revealed that the end-diastolic volume in the right ventricle was 2 ml. It was 20 ml at the age of two years and 40 ml at five years. Thus, the right ventricular hypoplasia lessened with increasing age. Neither pulmonary nor tricuspid valvular malformation was present. Patient 2: A four-month-old boy received two-dimensional echocardiographic examination at the age of two days which revealed: 1) a small right ventricle in the apical four-chamber view, 2) a half-moon-shaped right ventricle in the subxyphoid short-axis view at the level of the great arteries, and 3) normal positioning of the septal leaflet of the tricuspid valve. Pulsed Doppler echocardiography revealed normal blood flow patterns, immediately above the pulmonary valve and below the tricuspid valve. Cardiac catheterization at the age of three months indicated isolated right ventricular hypoplasia, a diverticulum of the right ventricle, and dilated cardiomyopathy. The patient died of congestive heart failure at the age of four months. Autopsy confirmed the above diagnoses.

Child, Preschool↗

[Doppler echocardiographic estimates of pressure gradients in various types of stenoses: usefulness and limitations].

UNLABELLED: In the present study, the accuracy of Doppler estimates of pressure gradients in various types of stenoses was clinically and experimentally evaluated. Fifty-seven patients, including 23 with ventricular septal defect, 15 with aortic or pulmonary valvular stenosis, four with infundibular stenosis, and five with supravalvular aortic or pulmonary stenosis were observed. The peak systolic pressure gradient (dP (C] was obtained at the time of catheterization in all patients. Before catheterization, the maximum velocity was measured by pulsed or continuous Doppler echocardiography and the estimated systolic pressure gradient according to Doppler (dP (D] was calculated by the simplified Bernoulli equation. The experimental model was designed to create pulsatile flow through a stenosis model. Nine different stenotic model types were used, including three orifice-like stenoses and six truncated cones with heights of 10 mm and 20 mm distal to the stenosis. The orifices in their stenoses were 3, 4 and 5 mm, respectively. Glycerin solution containing Sephadex with a viscosity similar to that of blood was used as the circulation medium. Its specific gravity was 1.16 g/cm3. In each stenotic model, the maximum velocity and instantaneous systolic peak pressure gradient were measured at various water flow rates. CLINICAL RESULTS: In patients with ventricular septal defect or valvular stenosis, dP (D) correlated very well to dP (C), with the regression equation, y = 0.87x + 2.79 (r = 0.92) or y = 0.96x + 1.02 (r = 0.99). In the other patients except for three with patent ductus arteriosus, dP (D) overestimated dP (C) by 11 to 71 mmHg, and their post-stenotic areas had gradually widened according to angiographic findings.(ABSTRACT TRUNCATED AT 250 WORDS)

Aortic Stenosis, Subvalvular↗

[External conduit repair with "valved pericardial roll"].

External conduit repair was performed in 20 patients with "valved pericardial roll (VPR)" made of own pericardium (one case) or porcine pericardium (19 cases). Intraoperative measurement of peak pressure gradients across the valve ranged from 0 to 8 mmHg (mean 1), and that from the pulmonary ventricle to the pulmonary artery ranged from 0 to 38 mmHg (mean 20). Re-evaluation of 7 patients one year after surgery shows no increase in the pressure gradient except the first case, in which VPR was made of own pericardium. In pulsed Doppler echocardiographic study, no regurgitation was found in 4 patients, mild in 6, moderate in 4, severe in 1. VPR is functioning well with longest follow up at 16 months.

Adolescent↗

[Follow-up study of coronary artery bypass grafting after Kawasaki disease--early and late postoperative evaluation].

In our hospital, 22 patients with severe coronary arterial lesion after Kawasaki disease underwent coronary artery bypass grafting (CABG) since 1982. The age of the cases at surgery ranged from 1 year to 19 years. Mean age was 8.3 years. Fifteen cases were bypassed using internal mammary artery (IMA) and 5 cases using IMA and autologous saphenous vein (SVG). Two cases were bypassed using only SVG. They have been examined until now by catheterization, by Treadmill test, and by Tl-201 myocardial imaging, around 1 month (Study 1) and around 1 year (Study 2) after surgery for the evaluation of results of CABG. One patient died from acute myocardial infarction 3 months after surgery and one patient shows return of anginal attack due to graft stenosis. Other patients are almost uneventful. In the study 1,20 (100%) IMA were patent, and 7 (88%) SVG were patent. In the study 2, 11 (92%) IMA and 6 (67%) SVG remained patent. Development of left anterior descending artery distal to IMA anastomosis was shown in 8 cases in the study 2. On the follow-up study with TI-201 myocardial imaging, disappearance or decrease of perfusion defect was seen in 7 cases (59%) in the study 1 and 9 cases (75%) in the study 2. On the Treadmill test, disappearance or improvement of ischemic change was seen in 15 cases (88%) in the study 1 and 10 cases (84%) in the study 2. These results suggest that IMA is more preferable for than SVG for young children who has an indication of CABG after Kawasaki disease.

Adolescent↗

[Anomalous origin of the left anterior descending coronary artery from the pulmonary artery associated with other anomalies: a case report].

A two-month-old boy with anomalous origin of the left anterior descending coronary artery (AOLAD), aortic stenosis (AS), coarctation of the aorta (CoA) and patent ductus arteriosus (PDA) is reported. Tachypnea was observed since birth. When he arrived at our hospital, congestive heart failure, cyanosis of his lower extremities and weak femoral pulses were observed. Radiography revealed cardiomegaly and pulmonary congestion. ECG showed low voltage in all leads, ST elevation in leads II, aVF, V5,6 and abnormal Q waves in V5,6. The tentative diagnosis by two-dimensional echocardiography (2DE) was AS, CoA, PDA, pulmonary hypertension and pericardial effusion. The abnormal coronary artery was visualized as originating from the left anterior wall of the pulmonary artery, coursing between the pulmonary artery and left atrial appendage. A definitive diagnosis of AOLAD were made by angiography. Aortic reconstruction using an artificial graft for CoA and PDA ligation was performed first. He died of myocardial infarction while waiting for his second operation for AOLAD and AS. Autopsy confirmed the aforementioned diagnoses as well as old and recent infarction of the myocardium. AOLAD is a rare congenital malformation, only six cases of which have been reported. Moreover, AOLAD complicated by other anomalies had not been previously reported. This is the first such reported case.

Abnormalities, Multiple↗

Genomic structure of the murine IL-6 gene. High degree conservation of potential regulatory sequences between mouse and human.

The genomic clone of mouse IL-6 was isolated and compared with the human IL-6 gene. The comparison revealed that the mouse IL-6 consists of five exons and four introns and that the overall organization is similar to that of the human IL-6 gene although the third intron is about 2 kb longer. The sequence similarity in the coding region is about 60%, whereas the 3'-untranslated region and the first 300-bp sequence of the 5'-flanking region are highly conserved (greater than 80%). Several sequence blocks with high homology are also found in the introns. Furthermore, sequences similar to transcriptional enhancer elements such as the c-fos serum responsive element and the consensus sequences for cAMP induction, activator protein 1 binding, and the glucocorticoid receptor binding are identified within the highly conserved 5'-flanking regions of the genes from the two species. These sequences may play an important role in transcriptional activation of the IL-6 gene.

Amino Acid Sequence↗