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T Kamiya

Publications and source records attributed to T Kamiya.

At least 19 recordsLinked to original sources
PubMed1992

Three distinct candidate point mutations of the von Willebrand factor gene in four patients with type IIA von Willebrand disease.

Type IIA von Willebrand disease (vWD) is the most common type II vWD and is characterized by the selective loss of large and intermediate sized multimers. One explanation for this disorder has been postulated to be a qualitative defect in von Willebrand factor (vWF) which results in increased susceptibility to proteolysis at the bond between residues Tyr842 and Met843. Four missense mutations that may cause type IIA vWD have recently been identified near the cleavage site. We analyzed the molecular basis for type IIA vWD in six patients. A 512 bp DNA sequence spanning the proteolytic cleavage site was targeted for PCR amplification and sequencing. We exploited a difference in restriction sites between the vWF gene and the pseudogene and have designed allele-specific oligomer used with PCR to distinguish these two genes. Three candidate missense mutations; Ser743 (TCG)----Leu (TTG), Leu799 (CTG)----Pro (CCG), and Arg834 (CGG)----Trp (TGG) were identified in 4 out of 6 patients. The amino acid substitution at Arg834 has been reported previously, but the other substitutions at Ser743 and Leu799 are novel candidate mutations locating 99 and 43 amino acids to the N-terminal side of the cleavage site, respectively. Our results indicate that amino acid substitutions located relatively distant from the cleavage site may also be involved in type IIA vWD.

Base Sequence↗
PubMed1992

A female hemophilia A combined with hereditary coagulation factor XII deficiency: a case report.

A 2-year-old Japanese girl with easy bruising and arthropathy was demonstrated to have severe hemophilia A (Factor VIII activity: less than 0.01 U/ml). She had normal 46XX karyotype. Her brother also had hemophilia A, and her mother and grandmother seem to be hemophiliac carriers. Additionally, activated partial thromboplastin time (APTT) of the patient was disproportionately prolonged and there were reduced levels of coagulation factor XII in the patients and members of the maternal trait which are compatible with heterozygous factor XII deficiency. Her father had both normal factor VIII and factor XII levels. Southern blotting analysis of genomic DNA from the propositus and family members with factor VIII and factor XII DNA probes revealed no gross alterations. This patient represents a female hemophilia A combined with heterozygous factor XII deficiency. Nonrandom inactivation of a normal X-chromosome (extreme lyonization) may be the basis for the expression of hemophilia A in this female patient.

Blood Coagulation↗
PubMed1992

Immunohistochemical staining patterns of tenascin in invasive breast carcinomas.

Eighty-two cases of primary invasive breast carcinoma and adjacent "normal" mammary glands were examined immunohistochemically for tenascin expression and distribution. Formalin-fixed tissues pretreated with actinase were processed by the avidin-biotin complex method using anti-human tenascin monoclonal antibody (RBC1). In normal mammary glands, tenascin was distributed around the ducts and ductules but not around the acini. In carcinomas, a high incidence of tenascin-positive cases (greater than 67%) was seen with various histological appearances, with the exception of lobular carcinoma where a low incidence was found (25%). Although intense staining was seen around cancerous foci when compared with normal mammary glands, tenascin was often expressed at cancer-mesenchymal junctions with dense fibrotic stroma, but not at junctions with active inflammatory change and a loose fibrotic stroma. Tenascin, expression is not an all-or-none marker for mammary malignancy and the staining pattern suggests either a role in stimulating cancer cells or a host defence mechanism accompanied by a desmoplastic response to them.

Adenocarcinoma, Mucinous↗
PubMed1992

Late death after arterial switch operation for transposition of the great arteries.

Fifty-nine patients survived for more than 1 month after an arterial switch operation (ASO). Diagnoses in these patients included transposition of the great arteries in 27, transposition of the great arteries with ventricular septal defect in 28, and double-outlet right ventricle in four. There were six late deaths (10%) during the follow-up period, and all of them occurred suddenly and unexpectedly. Four of the six late deaths were in patients who had undergone ASO in the neonatal period. Late deaths occurred from 40 days to 10 months after the operation. Autopsies were performed in all six patients. The cause of these late deaths was acute myocardial infarction. Five patients died of subendocardial infarction resulting from stenosis of the left main coronary artery. On pathologic examination, a fibrocellular intimal thickening was noted at the proximal region of the right and left coronary arteries, which resulted in 80% stenosis on average.

Abnormalities, Multiple↗
PubMed1992

Systemic administration of dynorphin A(1-13) markedly inhibits different behavioural responses induced by cocaine in the mouse.

The effects of systemic administration (i.p.) of dynorphin A(1-13) on the cocaine-induced behavioural alterations in the mouse were determined by using multi-dimensional behavioural analyses, based upon a capacitance system. A 1.0 mg/kg dose of cocaine did not influence behaviour, while increasing doses to 3-30 mg/kg produced a significant increment in the frequency of behaviour, such as linear locomotion, circling, rearing and grooming. Although a 1.0 mg/kg dose of dynorphin A(1-13) alone produced a significant decrease in grooming behaviour, larger doses (3.0 and 10.0 mg/kg) of the peptide failed to affect different behaviour. The cocaine (3.0 mg/kg)-induced increases in linear locomotion, circling and rearing behaviour were significantly inhibited by dynorphin A(1-13) (10.0 mg/kg). The inhibitory effects of dynorphin A(1-13) (10.0 mg/kg) were antagonized by the opioid antagonist Mr 2266 (5.6 mg/kg). It is thus possible that the systemic administration of dynorphin A(1-13) inhibits different behavioural responses induced by cocaine through the blood-brain barrier, although the instability of amino acid bonds or the relatively large molecular weight of dynorphin A(1-13), may result in the failure to demonstrate opioid activity by the peptide after systemic administration.

Analgesics↗
PubMed1992

Flow cytometric analysis of effects of cytokines on the expression of varicella-zoster virus glycoproteins.

Varicella-zoster virus (VZV)-infected human embryonic fibroblast (HEF) cells were stained with monoclonal antibodies directed against VZV glycoprotein I, II and IV, and then labeled with fluorescein isothiocyanate (FITC)-conjugated goat anti-mouse IgG. The cells were analyzed by flow cytometry. VZV-infected cells expressing VZV glycoproteins were clearly distinguished from uninfected cells. This method was useful for analyzing expression of VZV glycoproteins in different experimental conditions. Interferon alpha, beta, and gamma and tumor necrosis factor (TNF)-alpha reduced the percentage of positive cells and the mean fluorescence intensity of the cells expressing VZV glycoproteins. Interleukin(IL)-1 beta, IL-6 and TNF-beta had little effect on the expression of VZV glycoproteins.

Cells, Cultured↗
PubMed1992

Poly(ADP ribose) polymerase-defective mutant cell clone of mouse L1210 cells.

By a sequential mutation and selection utilizing N-methyl-N'-nitro-N-nitrosoguanidine as a mutagen, we succeeded in separating a poly(ADP ribose) polymerase-defective mutant clone (Cl-3527) from a mouse L1210 cell clone (Cl-3). The enzyme activity per cell in Cl-3527 cells was only 8% of that in wild type L1210 (CCL 219) cells. Immunoblot analysis of the enzyme protein in crude extracts of the mutant and wild type cells revealed that the enzyme defect was manifested as the loss of a 113-kDa wild type enzyme band in Cl-3527. Further analysis of partially purified enzyme from Cl-3527 by immunoblotting revealed that the molecular size of the enzyme in Cl-3527 was 108 kDa and that the amount of the mutant enzyme protein was markedly decreased in Cl-3527. The mutant enzyme was much more heat-labile than the wild type enzyme but the Km for NAD+, requirements for Mg2+ and nicked DNA, and the inhibition by 3-aminobenzamide, a potent inhibitor of the enzyme, however, were not so different from those of wild type enzyme. The mutant cells showed prolonged doubling time, increased temperature-sensitivity, increased percentage of active enzyme on a treatment of cells at high temperature, and increased expression of plasma membrane NADase, compared to wild type cells. Introduction of wild type ADPR pol gene into Cl-3527 cells partially restored the ADPR pol activity and the heat-resistance.

Animals↗
PubMed1992

Activation of lymphocytes by varicella-zoster virus (VZV): expression of interleukin-2 receptors on lymphocytes cultured with VZV antigen.

The expression of human leukocyte antigen (HLA-DR) and interleukin-2 receptor (IL-2R; alpha and beta subunits) was significantly increased in peripheral blood mononuclear cells (PBMC) from varicella-zoster virus (VZV)-seropositive donors when PBMC were cultured with VZV antigen for 6 days. The increased expression of HLA-DR and IL-2R was observed in both CD4+ and CD8+ lymphocytes, which suggests that both types of lymphocytes from VZV-seropositive donors were activated when PBMC were cultured with VZV antigen. Adding anti-IL-2R alpha did not inhibit lymphocyte proliferation in response to VZV antigen, although marked inhibition was obtained by adding anti-IL-2R beta. No IL-2 was detected in the supernatant of PBMC cultured with VZV antigen; however, antibody to IL-2 inhibited lymphocyte proliferation on exposure to VZV antigen. These results indicate that IL-2 and expression of IL-2R beta are essential to lymphocyte activation by VZV antigen.

Adult↗
PubMed1992

Retinoic acid-specific induction of a protein kinase C isoform during differentiation of HL-60 cells.

Human promyelocytic leukemia cells (HL-60) were treated with several differentiation inducers, then the changes in the activity of cytosolic protein kinase C (PKC) isoforms were examined by hydroxylapatite chromatography and the species of the isoforms were determined immunologically. In three undifferentiated HL-60 cell lines examined, PKC alpha and beta isoforms were present, but PKC gamma isoform was not detected. When the cells were induced by dimethylsulfoxide, dibutyryl cAMP, or nicotinamide to differentiate into granulocytes, these two PKC isoforms each increased to about 2- to 3-fold. When retinoic acid was used as the inducer, in addition to PKC alpha and beta, a third PKC isoform appeared. This isoform was clearly distinct from rat PKC alpha, beta, and gamma, immunologically. This isoform showed a distinctly lower Ca(2+)-requirement (3 microM) than that of PKC alpha or beta (100 microM) and was more dependent on cardiolipin and phosphatidylethanolamine, compared with PKC alpha, beta, and gamma. These results suggest that while the increases in the activities of PKC alpha and beta isoforms are common in the differentiation program initiated by several inducers, including retinoic acid, the emergence of an unclassified PKC isoform is a retinoic acid-specific process.

Animals↗
PubMed1992

[Chemical composition of gallstones in Santa Cruz--Bolivia].

The knowledge of the gallstones chemical composition is very important from view point of lithogenesis and possible medical therapy of dissolution. The composition (chemical) of gallstones in 30 consecutive cholecystectomized patients were studied in the 2nd. Department of Surgery of Hospital San Juan de Dios, Santa Cruz, Bolivia (altitude 1900 m). All gallbladder stones were washed, counted, measured and dried before sending them to Japan to be processed. The composition of gallstones was analyzed by infrared absorption spectroscopy. Cholesterolemia, age, sex and number of stones were analyzed in cases of pure cholesterol stones. The incidence of pure cholesterol stones was 56.6%, statistically similar to be reported by other authors, but very low if compared with the valley area of Bolivia (Cochabamba altitude 2300 m) where hypothyroidism is frequent. Hypercholesterolemia had little relation with the pure cholesterol gallstones. There was no difference in the chemical composition and the size of the stones, but while their number was small, cholesterol stones were more frequent. The low incidence of pure cholesterol stones may show a bad nutritional condition of our population (poor fat and protein content) and this result is important in order in using the new advance: lithodissolvent drugs.

Adolescent↗
PubMed1992

[Pulmonary uptake of thallium-201 in patients with congenital heart disease: comparison between total anomalous pulmonary venous connection and tetralogy of Fallot].

To evaluate the pulmonary extravascular space in patients with congenital heart disease, lung uptake of thallium-201 (T1-201) was quantitatively studied. A total of 50 T1-201 imagings were performed in 33 patients with total anomalous pulmonary venous connection (TAPVC); 4 preoperatively, 22 postoperatively in the early stage (within 6 months), and 24 in the late stage (7 months or later). The images consisted of 17 supracardiac TAPVC (type-I), 13 paracardiac (type-II) and 3 infracardiac (type-III). In patients with tetralogy of Fallot (T/F), T1-201 imaging was performed 15 times preoperatively, 12 in the early stage and 15 in the late stage, postoperatively. Furthermore, 29 patients with ventricular septal defect (VSD) or patent ductus arteriosus (PDA) were also studied preoperatively, and 21 in the late postoperative stage. Twenty-five patients with arrhythmias or a history of Kawasaki disease without perfusion defects were studied on T1-201 myocardial imaging. Lung uptake of T1-201 was analyzed with a computer using the anterior image of the chest, and the average count ratio of the right lung (P) to the left ventricular wall (LV) was calculated. P/LV values were compared between the patients before and after surgery, and differences in anatomical types in TAPVC were also evaluated. In TAPVC, P/LV values decreased gradually in the postoperative state, but were significantly higher than those of controls even in the late stage. In the late postoperative stage, type-I TAPVC had significantly higher P/LV values than those of type-II.(ABSTRACT TRUNCATED AT 250 WORDS)

Heart Defects, Congenital↗
PubMed1992

Surgical excision of hemophilic pseudotumor of the ilium.

The iliac hemophilic pseudotumor is a rare complication of hemophilia occurring in 1-2% of patients with Factor VIII or Factor IX deficiency. It is frequently disabling and life threatening. This report presents a comparative study of postoperative results of two cases of hemophilic pseudotumor of ilium. One patient undergoing partial resection showed a favorable postoperative course, whereas the patient with complete resection of the pseudotumor died of postoperative bleeding and sepsis. Studies on the postoperative results of these two cases indicate that careful preoperative consideration of tumor size and degree of infiltration is of the utmost importance in operative management. Early excision of tumors eliminates the possibility of endogenous infection. Even partial resection of huge tumors, leaving the lateral wall intact for compression, can promote recovery of functions.

Angiography↗
PubMed1992

[Total repair for truncus arteriosus].

Total repair for truncus arteriosus using an external conduit was performed in 12 patients from 1978 through 1989. Six cases were infants (mean age: 3.4 months) and 6 were children (mean age; 1 years 9 months). Two cases had Collet-Edwards type II truncus and the other 10 cases had type I truncus. One of the infants was associated with an interruption of the aorta and another had a severe regurgitation of the truncal valve (TrV). For external conduits, we used a non-valved conduit in one infant, a composite valved conduit of Dacron containing a heterograft valve in 4 children and a valved pericardial roll made of an autologous or porcine pericardium in 5 infants and 2 children. One infant with a severe regurgitation of the TrV needed valve replacement along with enlargement of the annulus of the TrV. One infant who had replacement of the TrV died early postoperatively. Another infant died 10 months after total repair due to an infection of an external conduit. Cardiac catheterization was performed in all 10 survivors. The mean value for the systolic pulmonary/systemic pressure ratio decreased from 0.98 +/- 0.09 preoperatively to 0.36 +/- 0.09 postoperatively. Replacement of an external conduit was performed due to a conduit stenosis in 2 children and 1 infant, 10 years and 2 months, 7 years and 9 months, and 1 year and 8 months after the total repair, respectively. In one of these 2 children, replacement of the aortic valve was performed due to a severe aortic regurgitation. We conclude that our results of total repair for truncus arteriosus were satisfactory. However, it remains to be solved how to manage an infant with truncus arteriosus associated with a severe regurgitation of the TrV.

Bioprosthesis↗
PubMed1992

[Thallium-201 myocardial imaging in cases involving transposition of the great arteries after arterial switch operation].

Forty patients, including 39 with complete transposition of the great arteries and one with double outlet right ventricle, after arterial switch operation were studied with thallium myocardial imaging (TMI). In patients who had post-operative cardiac catheterization, the TMI findings were compared to those of catheterization studies. The right ventricular to left ventricular up-take ratio (RV/LV ratio) shown by TMI correlated well with the right-to-left ventricular systolic pressure ratio. Supravalvular pulmonic stenosis with significant pressure gradient was found in 14 patients, and all of them were noted to have an elevated RV/LV ratio. This ratio was noted to increase progressively on serial TMI studies in most patients who had significant pulmonic stenosis on first postoperative catheterization. Three patients had significant perfusion defects indicating the presence of myocardial damage. Two of them had occlusion of the coronary artery, but the other one had no coronary abnormalities able to be found by coronary arteriography. Air embolism or some other intra-operative transient insults to the myocardium were most likely to have been responsible for the perfusion defects. Thus, TMI was valuable as one of the noninvasive tools for follow-up of patients after arterial switching, particularly for those with late complications such as supravalvular pulmonic stenosis and coronary problems.

Cardiac Surgical Procedures↗
PubMed1979

Congenital deficiency of alpha 2-plasmin inhibitor associated with severe hemorrhagic tendency.

alpha(2)-Plasmin inhibitor (alpha(2)PI) is a recently characterized, fast-reacting plasmin inhibitor in human plasma that appears to play an important role in regulation of in vivo fibrinolysis. We report here a case of complete deficiency of alpha(2)PI in man. The patient, a 25-yr-old Japanese man, had a life-long severe bleeding tendency (hemarthrosis and excessive bleeding after trauma). The following tests were within normal limits: platelet count, bleeding time, thrombin time, prothrombin time, partial thromboplastin time, titers of known clotting factors, platelet glass bead retention, Factor VIII-related antigen, platelet aggregation by ADP, collagen and ristocetin, and clot retraction. Routine liver function tests were also normal. The only abnormal finding was that whole blood clot lysis was extemely rapid and was complete in 4-8 h. The concentration of plasma protease inhibitors, including alpha(2)-macro-globulin, antithrombin III, alpha(1)-antitrypsin, and C1INH, were all normal. The concentration of alpha(2)-PI in the patient's plasma, assayed by immunological methods, was <0.1 mg/100 ml (normal concentration, 6.1+/-0.88 mg/100 ml [mean+/-SE]) and functional assays showed a complete deficiency of alpha(2)PI. Addition of purified alpha(2)PI to the patient's whole blood completely corrected the accelerated fibrinolysis. The patient's parents, four siblings, and four other members of this family were asymptomatic, but the titers of alpha(2)PI in their plasmas were congruent with50% of normal pooled plasma. There were three consanguineous marriages in this family, and the alpha(2)PI deficiency appears to have been inherited as an autosomal recessive trait. We speculate that alpha(2)PI deficiency in this patient has led to uninhibited in vivo fibrinolysis that probably causes the severe hemorrhagic tendency. Thus, this study indicates the important role of alpha(2)PI in hemostasis.

Adult↗
PubMed1979

Echocardiographic and angiocardiographic features of supravalvular aortic stenosis in children.

Fourteen patients with SVAS served as subjects of echocardiographical and angiocardiographical studies and the data obtained were compared. There were significant decreases in aortic diameter at the level of ascending aorta compared to those of the aortic leaflets and significant differences of the percentage change of the aorta between the patients with SVAS and the normals. Echocardiography is, therefore, a useful noninvasive method for evaluating SVAS. If the values of percentage change of the aortic diameter obtained by echocardiogram are below -30%, the values of peak systolic pressure gradients across the supravalvular stenotic region should be higher than 20 mmHg. The value of LV M/V under 1.0 suggests that the pressure gradient is lower than 20 mmHg.

Angiocardiography↗
PubMed1979

Open heart closure of atrial septal defect in a patient with idiopathic thrombocytopenia.

A case of atrial septal defect with idiopathic thrombocytopenia was successfully operated with the aid of heart-lung machine. The operation was performed under the administration of platelet rich plasma, fresh blood and adrenocorticosteroids before, during and after the operation. With the aid of these treatments, no severe hemorrhagic diasthesis was encountered.

Female↗