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Biomedical subjects

Masaru Terai

Publications and source records attributed to Masaru Terai.

At least 19 recordsLinked to original sources
PubMed2006

Coarctation of the descending aorta, patent ductus arteriosus, deficiency of right superior vena cava, and persistent left superior vena cava in a five-month infant demonstrated by multislice computed tomography.

A 5-month-old female presented in our hospital with cyanosis, peripheral psychroesthesia, and significantly lower blood pressure of lower limbs compared with the upper limbs; suggesting aortic coarctation. The presence of patent ductus arteriosus (PDA) and persistent left superior vena cava (PLSVC) was suspected by echocardiogram. Before surgery, we performed multislice computed tomography to determine the spatial relationship between the site of aortic coarctation, PDA, and PLSVC. Although contrast material was injected from the right cubital vein, PLSVC was exclusively enhanced; therefore, deficiency of right superior vena cava was diagnosed. Coarctation of the DAo was observed in the right posterior direction of PLSVC and PDA was observed just distal to the coarctation site of the DAo and located at the right posterior direction of PLSVC. Therefore, the spatial relationship of PLSVC, coarctation of DAo and PDA could be evaluated and a direct anastomosis of the DAo and ligation of PDA were performed.

Abnormalities, Multiple↗
PubMed2006

Serum vascular endothelial growth factor in cyanotic congenital heart disease functionally contributes to endothelial cell kinetics in vitro.

BACKGROUND: Remarkable amounts of neovascularization develop in patients with cyanotic congenital heart disease who have low pulmonary blood flow and systemic cyanosis, but the factors functionally responsible for angiogenesis in cyanotic congenital heart disease have not been determined. METHODS AND RESULTS: To investigate the functional angiogenic molecules in serum from these patients, serum angiogenic activity was studied in 21 patients (systemic oxygen saturation: 82+/-1.9%) and in 17 healthy controls. Patient serum was more active in stimulating the tube formation of human umbilical vein endothelial cells (HUVECs) into capillary-like structures than control serum (150% vs 104% of internal control; p<0.001). This increased serum angiogenic activity normalized after total cardiac repair (p<0.001). The migration activity of HUVECs was also accelerated in patient serum (p=0.007). To identify the molecules in patient serum affecting tube formation of HUVECs, we examined the effects of an inhibitor or a neutralizing antibody against various angiogenic molecules on in vitro angiogenesis. Both the soluble vascular endothelial growth factor (VEGF) receptor 1 and the VEGF receptor 2 tyrosine kinase inhibitor SU5416 reduced the basal serum angiogenic activity of patients and this was reversed by a supplement of recombinant human VEGF. CONCLUSION: Our results indicate that serum VEGF functionally contributes to vascular endothelial cell kinetics in patients with cyanotic congenital heart disease.

Adolescent↗
PubMed2005

Gene expression profiling of the effect of high-dose intravenous Ig in patients with Kawasaki disease.

Kawasaki disease (KD) is an acute vasculitis of infants and young children, preferentially affecting the coronary arteries. Intravenous infusion of high dose Ig (IVIG) effectively reduces systemic inflammation and prevents coronary artery lesions in KD. To investigate the mechanisms underlying the therapeutic effects of IVIG, we examined gene expression profiles of PBMC and purified monocytes obtained from acute patients before and after IVIG therapy. The results suggest that IVIG suppresses activated monocytes and macrophages by altering various functional aspects of the genes of KD patients. Among the 18 commonly decreased transcripts in both PBMC and purified monocytes, we selected six genes, FCGR1A, FCGR3A, CCR2, ADM, S100A9, and S100A12, and confirmed the microarray results by real-time RT-PCR. Moreover, the expressions of FcgammaRI and FcgammaRIII on monocytes were reduced after IVIG. Plasma S100A8/A9 heterocomplex, but not S100A9, levels were elevated in patients with acute KD compared with those in febrile controls. Furthermore, S100A8/A9 was rapidly down-regulated in response to IVIG therapy. Persistent elevation of S100A8/A9 after IVIG was found in patients who later developed coronary aneurysms. These results indicate that the effects of IVIG in KD may be mediated by suppression of an array of immune activation genes in monocytes, including those activating FcgammaRs and the S100A8/A9 heterocomplex.

Acute Disease↗
PubMed2005

Causative organism influences clinical profile and outcome of infective endocarditis in pediatric patients and adults with congenital heart disease.

BACKGROUND: Studies on microorganisms in a large cohort of patients with congenital heart disease (CHD) and infective endocarditis (IE) are rare. Using a nationwide survey, the relationship between causative microorganisms and clinical profiles in patients with CHD and IE was investigated. METHODS AND RESULTS: Data from 188 patients with CHD (pediatric patients (n=113), mean age, 6.2+/-4.9 years; adult patients (n=75), mean age 28.4+/-13.4 years) and IE from 60 institutions were analyzed. Causative microorganisms were Streptococcus species (94:50.0%), Staphylococcus species (68:36.2%), Haemophilus species (9:4.8%), Candida (5:2.7%), Pseudomonas species (4:2.1%) and other unclassified microorganisms (8:4.3%). Staphylococcal IE was observed significantly higher in perioperative IE (11/16), in cyanotic patients (32/73) and patients younger than 1 year old (11/16). Streptococcal IE was observed significantly higher in acyanotic patients (64/109) and patients aged 16 years or older (48/75). Total mortality was 20/188 (10.6%) and was high for candidial (2/5; 40%) and pseudomonal IE (2/4; 50%). Mortality was highly associated with younger age, especially infants (5/16), and methicillin-resistant staphylococcal IE (6/15). CONCLUSIONS: The causative microorganisms are significantly related to the clinical profile and outcome in patients with IE and CHD. These results form the basis for selecting appropriate antibiotics for prevention and management.

Adolescent↗
PubMed2004

Detection of antigen in bronchial epithelium and macrophages in acute Kawasaki disease by use of synthetic antibody.

BACKGROUND: Kawasaki disease (KD) is the most common acquired cardiac disease in children in developed nations. The etiology is unknown, but a ubiquitous infectious agent appears to be likely. Immunoglobulin A plasma cells infiltrate inflamed tissues in acute KD, producing oligoclonal, antigen-driven antibodies. METHODS: To identify antigens important in the pathogenesis of KD, oligoclonal KD antibodies were prepared in vitro and tested by immunohistochemistry experiments on tissues from patients with acute KD and from control subjects and were also tested for reactivity with human inflammatory proteins. RESULTS: By use of synthetic antibody A, specific binding to a cytoplasmic antigen in proximal bronchial epithelium was observed in 10 of 13 patients with acute KD but in 0 of 9 control subjects (P=.001). A subset of macrophages was positive in at least 1 inflamed tissue from all 17 patients with acute KD. Antigen was detected in 9 of 12 acute KD coronary artery aneurysms but in 0 of 10 control coronary arteries (P<.001). The antigen is not immunoglobulin or any of 40 common inflammatory proteins. CONCLUSIONS: We report the first demonstration of a KD-associated antigen in the tissues targeted by the disease. Our findings are consistent with the theory that KD is caused by a previously unidentified respiratory infectious agent with tropism for vascular tissue.

Antibodies, Monoclonal↗
PubMed2004

Efficacy of intravenous immune globulin therapy combined with dexamethasone for the initial treatment of acute Kawasaki disease.

UNLABELLED: We studied the effects of a new regimen consisting of intravenous immune globulin (IVIG) combined with dexamethasone (DEX) on clinical outcome and serum levels of vascular endothelial growth factor (VEGF) in the initial treatment of Kawasaki disease (KD). A total of 46 KD patients received 0.3 mg/kg per day DEX plus heparin i.v. for 3 consecutive days, together with 2 g/kg IVIG over 4 to 5 days (DEX group). Low-dose acetylsalicylic acid was started after completion of DEX therapy. The control group consisted of 46 KD patients retrospectively treated earlier with 2 g/kg IVIG over 4 to 5 days plus higher dose acetylsalicylic acid (CONTROL group). No serious adverse effect was noted in either group. There were no differences in baseline and post-treatment laboratory data except for C-reactive protein between the groups. Post-treatment C-reactive protein in the DEX group (median 0.9 mg/dl, range 0.0 to 24.7 mg/dl) was lower than that (1.2 mg/dl, range 0.2 to 19.5 mg/dl) in the CONTROL group ( P=0.033 by Mann-Whitney U test). In addition, the mean duration of fever after the first IVIG infusion was 2.2 days (median 1 day, range 1 to 12 days) in the DEX group and 2.8 days (2 days, 1 to 16 days) in the CONTROL group ( P=0.015 by Mann-Whitney U test). The new regimen did not reduce VEGF levels. Two patients in each group developed small- or medium-sized coronary artery aneurysms. CONCLUSION: Although this regimen did not affect coronary outcome, intravenous immune globulin therapy combined with dexamethasone for the initial treatment of Kawasaki disease was safe and may accelerate the resolution of systemic inflammation.

Acute Disease↗
PubMed2004

Enhanced iNOS expression in leukocytes and circulating endothelial cells is associated with the progression of coronary artery lesions in acute Kawasaki disease.

Nitric oxide (NO) serves many vasoprotective roles, but the massive release of NO causes arterial wall degeneration. We investigated whether enhanced nitric oxide synthase (iNOS) expression in peripheral blood leukocytes and circulating endothelial cells mirrors the progression of coronary arterial lesions in 55 children with acute Kawasaki disease (KD), including 24 with and 31 without coronary artery lesions (CAL). Patients were treated with i.v. gamma-globulin at the time of diagnosis and blood samples were collected before and after treatment. The cellular origin of NO synthesis was determined by flow cytometric analysis of iNOS expression in peripheral blood, and by immunohistochemical analysis of circulating endothelial cells and coronary arteries. iNOS expression in neutrophils peaked at the time of diagnosis, but did not peak in monocytes until 2 wk post onset of disease. Levels were significantly higher in both cell types in patients with CAL (p = 0.001 and p = 0.035, respectively). In addition, the number of circulating endothelial cells and levels of iNOS expression were higher in patients with CAL (p = 0.011 and p = 0.012, respectively). Immunohistochemical analysis of the coronary arteries from three patients with acute KD revealed iNOS immunoreactivity in endothelial cells, as well as infiltrating monocytes/macrophages in the aneurysms. We conclude that the expression of iNOS in peripheral blood leukocytes, as well as circulating endothelial cells, correlates with the severity of coronary arterial wall injury and the progression of CAL in patients with acute KD.

Arteries↗
PubMed2004

CD40 ligand gene and Kawasaki disease.

Kawasaki disease (KD) is an acute systemic vasculitis syndrome of infants and young children. Although its etiology is largely unknown, epidemiological findings suggest that genetic factors play a role in the pathogenesis of KD. To identify genetic factors, affected sib-pair analysis has been performed. One of the identified peaks was located on the Xq26 region. A recent report of elevated expression of CD40 ligand (CD40L), which maps to Xq26, during the acute-phase KD, and its relationship to the development of coronary artery lesions (CAL) prompted us to screen for polymorphism of CD40L and to study the association of the gene to KD. A newly identified SNP in intron 4 (IVS4+121 A>G) is marginally over-represented in KD patients as compared to controls (109/602, 18.1 vs 111/737, 15.1%). When male KD patients with CAL were analyzed as a patient group, the SNP was significantly more frequent than in controls (15/58, 25.9%, vs 111/737, 15.1%, OR=2.0, 95% CI=1.07-3.66; P=0.030). Interestingly, this variation was extremely rare in a control Caucasian population (1/145, 0.7%). Our results suggest a role of CD40L in the pathogenesis of CAL and might explain the excess of males affected with KD.

3' Untranslated Regions↗
PubMed2003

Prognostic impact of vascular leakage in acute Kawasaki disease.

BACKGROUND: Increased microvascular permeability is an initial step of Kawasaki disease (KD). We reported that vascular endothelial growth factor (VEGF) might play a role in the vascular leakage of KD. In fatal KD, plasma leakage was extensively documented at VEGF-positive microvessels. Increases in vascular leakage cause hypoalbuminemia and noncardiogenic edema. However, the prognostic impact of vascular leakage in KD remains unclear. METHODS AND RESULTS: We compared 76 patients who became afebrile within 5 days after starting intravenous gamma globulin (IVGG) (2 g/kg over 5 days) (IVGG-responsive) with 27 patients who did not respond (IVGG-resistant). Baseline levels of serum VEGF and albumin were similar between the groups. After IVGG, VEGF levels increased (P<0.0001) and albumin levels decreased (P<0.00001) in both groups. However, the IVGG-resistant group had higher VEGF levels (P=0.029) and severe hypoalbuminemia (P<0.00001) compared with the IVGG-responsive group. Coronary aneurysms were documented in 12 patients from the IVGG-resistant group but not in the IVGG-responsive group. Then IVGG-resistant patients were divided into 2 subgroups according to the presence (n=12) or absence (n=15) of coronary aneurysms. There was no difference between subgroups in age, sex, laboratory data including albumin, and retreated doses of IVGG. However, body weight gain after IVGG was documented in patients who subsequently developed coronary aneurysms (P=0.003) but not in those who did not (P=0.967). CONCLUSIONS: These results suggest that vascular leakage may be a key feature of KD pathophysiology. The present study may provide better insights into the pathogenesis and treatment of patients resistant to IVGG in acute KD.

Acute Disease↗
PubMed2003

Analyses of fatal cases of Kawasaki disease in Japan using vital statistical data over 27 years.

BACKGROUND: Chronological changes in mortality and case fatality rates from Kawasaki disease covering an extended period in Japan are still unknown. METHODS: We analyzed 679 deaths of patients in Japan whose underlying cause was Kawasaki disease, by using the data of vital statistics between 1972 and 1998. RESULTS: The male-to-female ratio of the number of deaths was 2.07 and the mean age at death in males was higher. Two unusual increases in the epidemic years, 1982 and 1986, were observed in the chronological changes of the number of deaths. The mortality rate of males was higher than that of females, with a few exceptional years, and the annual mortality rates were high in three epidemic years. The age-specific mortality rate was highest in infants under one year of age. Prefectures with high mortality rate clustered in some regions. The case fatality rate decreased annually, declining to as low as 0.2% among those who were born in 1986 and thereafter; and unusual increases in the case fatality rate affected by these three epidemic years were not repeated. CONCLUSIONS: The case fatality rate from Kawasaki disease in Japan decreased during the 27 years of observation: improvements in treatment might account for this.

Age Factors↗
PubMed2002

Systemic production of vascular endothelial growth factor and fms-like tyrosine kinase-1 receptor in acute Kawasaki disease.

BACKGROUND: Increased vascular permeability is an important event during the initial process of Kawasaki disease (KD). One potential responsible candidate for the induction of vascular hyperpermeability is vascular endothelial growth factor (VEGF). METHODS AND RESULTS: We investigated the expression of VEGF and its receptors (flt-1, KDR) in acute KD tissues at 7 days to 5 weeks of illness. Neuropilin-1, which enhances the binding of VEGF(165) to KDR, was also studied. Abundant expression of VEGF and flt-1 was documented immunohistochemically in many organs from acute KD, including heart and lung. VEGF and flt-1 were colocalized in all vessels that showed edema. These molecules resided in endothelium and vascular media and also in migrating smooth muscle cells in neointima and infiltrating macrophages. Compared with controls, coronary vessels of acute KD had upregulation of VEGF and flt-1 but not KDR or neuropilin-1. KDR was expressed by vessels at 7 days of illness but not later in the illness. Plasma proteins were more extensively bound to the extracellular matrix in coronary vessels in acute KD than controls. Furthermore, elevation of serum VEGF levels was correlated with low serum albumin in acute KD (n=220, r=-0.53, P<0.001). CONCLUSIONS: These findings suggest that VEGF and flt-1 are upregulated in blood vessels in many organs of acute KD. Expression of KDR was limited to the early stage of acute KD. The roles of VEGF in acute KD may involve promotion of vascular permeability and macrophage activation. Low serum albumin may indicate overproduction of VEGF in acute KD.

Acute Disease↗
PubMed2002

Influence of early repair of tetralogy of fallot without an outflow patch on late arrhythmias and sudden death: a 27-year follow-up study following a uniform surgical approach.

Pulmonary regurgitation and older age at the time of repair may have a large impact late after repair on subsequent mortality of patients with tetralogy of Fallot. We aimed to identify whether age at repair, and preservation of the pulmonary valve, had a favorable influence on morbidity and mortality. We also analyzed risk factors for late death subsequent to surgical repair. We identified 167 patients who, between 1965 and 1975, and at a mean age of 6 years, underwent total repair of tetralogy of Fallot by a single surgical team without use of an outflow patch. All patients were known to have survived for at least 30 days after repair, and late mortality was identified though the use of hospital records, interviews, and death certificates. The 29-year actuarial survival rate was 86%, with 24 late deaths. Of these deaths, seven occurred suddenly (4.2%). Morbidity was analyzed in 99 of the patients by means of a written questionnaire and telephone interview. It proved possible to analyze ventricular and valvar function in 50 of the patients. Survivors experienced no re-intervention, and 89% of them were in class I of the grading system of the New York Heart Association. We found evidence of 3 episodes of sustained ventricular tachycardia (3.0%), and two episodes of atrial tachycardia (2.0%). Of the 50 patients in whom serial examinations were available, 18 had pulmonary regurgitation of moderate degree or greater, and none had more than moderate tricuspid regurgitation, with a mean QRS duration of 148 ms and an ejection fraction for the left ventricle of 50%. Older age at repair (p = 0.03), and longer periods of cardiac arrest during repair (p = 0.02), were associated with late mortality. Although the mortality was similar to that observed in previous reports, our operative method might have a better effect in terms of late morbidity.

Age Factors↗
PubMed2002

Atresia of the orifice of the coronary sinus after surgery.

We describe a patient with a coronary arteriovenous fistula, atresia of the orifice of the coronary sinus, and persistence of the left superior caval vein. Depression of her ST segments was revealed by exercise electrocardiography long after the initial surgical reconstruction of the coronary sinus.

Arteriovenous Fistula↗
PubMed2002

Mortality and risk factors for late deaths in tetralogy of Fallot: the Japanese Nationwide Multicentric Survey.

OBJECTIVES: We have compared mortality and risk factors for late deaths in patients with tetralogy of Fallot undergoing surgical repair in 1972 and 1982 in a Japanese multicentric study, examining in particular the impact of time of repair. BACKGROUND: There is limited information on the effect that time of repair, and our constantly changing approach to it, has on late outcome in repaired tetralogy of Fallot. METHODS: We analysed the Japanese registry of deaths occurring after surgical repair of tetralogy of Fallot. We studied two postoperative 1-year cohorts of survivors of surgery performed in 12 centers. Of the patients, 122, aged 29+/-12 years, had undergone repair in 1972, their age at repair being 9.6 years. An additional 186 patients, aged 23+/-8.7 years, had been repaired in 1982 at the age of 7.7 years. RESULTS: Annual mortality, as judged per 100,000 population of patients with tetralogy of Fallot, declined from 0.387 in 1972 to 0.196 in 1982. Significant differences were deaths following surgery (27% vs. 13%, p < 0.001), patching of the subpulmonary outflow tract (48% vs. 89%, p < 0.001), and transjunctional patching (13% vs. 63%, p < 0.001). Late death was observed in 6 vs. 3 patients (9/308, 2.9%). The actuarial rate of survival calculated over 14 years was 97% vs. 98%. Reoperation was performed in 5 vs. 9 patients (14/308, 4.5%). Risk factors for late death were age at repair (p = 0.01), and history of reoperation (p < 0.001). Transjunctional patching (p = 0.01) proved to be associated with late mortality only in patients repaired in 1972. CONCLUSIONS: Late survival was excellent, with a low incidence of reoperations in both groups of patients. The era of repair has a big influence on total and operative mortality, but has only a small impact on late mortality. Ongoing analysis of follow-up will possibly reveal subsequent changes with time.

Adolescent↗
PubMed2002

Peripheral blood eosinophilia and eosinophil accumulation in coronary microvessels in acute Kawasaki disease.

BACKGROUND: Early stage Kawasaki disease (KD) histopathology includes perivasculitis and vasculitis of the microvessels. The lesions then extend to larger vessels. Therefore the analysis of microvessel lesions is important to better understand the initial pathogenesis of KD vasculitis. METHODS AND RESULTS: We studied epicardial microvessel lesions (<50 microm) and aneurysm lesions of paraffin-embedded cardiac tissues from 4 Japanese patients who died 7 to 22 days after KD onset. The cellular composition in the microvessel lesions was different from that in coronary aneurysm lesions; eosinophils were preferentially accumulated in the microvessel lesions. The average population of eosinophils was 16% of total infiltrated cells in the microvessel lesions, whereas it was 3% in the intima of aneurysm walls. We examined peripheral blood eosinophil cell counts in 95 KD patients and 95 febrile age-matched controls. Baseline eosinophil cell counts in KD patients were higher than those in febrile control patients (361 +/- 441 65 +/- 133; < 0.0001). Eosinophilia (>350 cells/microl) before therapy was documented in 36% of KD patients, but in only 4% of febrile controls ( < 0.0001). Sixty-six KD patients (69%) developed eosinophilia within 2 weeks of illness. CONCLUSIONS: Because the numbers of circulating eosinophils in the body are tightly regulated, eosinophil accumulation in blood or tissues may reflect the host's immune response against KD related antigen(s).

Child, Preschool↗