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Biomedical subjects

T Kamiya

Publications and source records attributed to T Kamiya.

At least 415 records · Page 23Linked to original sources

Genetic linkage study of Duchenne muscular dystrophy and hemophilia A.

We studied a family in which three boys had both Duchenne muscular dystrophy and hemophilia A. Seven other males had only hemophilia A. Genetic linkage study showed a maximum likelihood estimate of recombination frequency of these two X-linked recessive traits of 0.16 with 95% confidence limits of 0.045 and 0.50. The loci of the two diseases may be closer than previous estimates.

Adolescent↗

A trial procedure to prevent aneurysm formation of the coronary arteries by steroid pulse therapy in Kawasaki disease.

Sixty patients with Kawasaki disease in the acute phase were studied using a two-dimensional echocardiography. Coronary lesions in Kawasaki disease were characterized echocardiographically by an increase of echo density of the coronary artery and by dilatation or aneurysm of the coronary artery. These findings appeared in the sequence mentioned above in patients who were studied serially. In the group treated with corticosteroid, 62% of the patients showed a disappearance or an improvement of the coronary dilatation or aneurysm, while only 33% of untreated patients showed such an improvement. In patients with coronary aneurysms on the initial echocardiogram, 53% of the pulse therapy group showed an improvement, but none of the untreated group did. A similar result was obtained by an analysis of its therapeutical effect, according to the number of vessels involved. Thus, corticosteroid pulse therapy was shown to be effective for the prevention of coronary arterial aneurysms in Kawasaki disease, if it was given in a sufficient dose in the acute phase of this disease.

Adrenal Cortex Hormones↗

[Myocardial imaging in patients with Kawasaki disease: exercise stress imaging and serial studies].

Thallium-201 myocardial imaging was performed in 80 children with coronary arterial lesions due to Kawasaki disease in order to assess the value of serial and exercise myocardial imagings. In eight of these children, abnormalities of the image were noted. Twenty children had serial thallium studies with an interval of three to 18 months, and five of these showed changes in the image including appearance of a new perfusion defect in one patient and improvement of perfusion defects in four. These changes of the myocardial image were correlated well with coronary angiographic findings obtained within a few days of the isotope studies. It was noted that the changes of the myocardial image were more frequently observed at relatively recent period recovered from Kawasaki disease. Exercise myocardial imaging using a bicycle ergometer was performed in eight children with coronary arterial lesions. In three an evidence of improvement of the myocardial perfusion was noted immediately after exercise as well as on the delayed image. In one patient, a decrease of the perfusion in the apex and inferior wall was noted immediately after exercise. On the observed image, image of the apex improved but that of the inferior wall remained hypoperfused. Thus thallium-201 myocardial imaging was considered to permit the best noninvasive documentation of ongoing myocardial perfusion abnormalities in patients with Kawasaki disease. Exercise myocardial imaging was found to be useful in differentiating the viable from nonviable myocardium. It was to be emphasized that quantitative evaluation by computer-assisted analysis was particularly valuable in detecting small areas and in a comparison of the myocardial images.

Adolescent↗

[Clinical study of membranous ventricular septal defect in infancy and childhood by two-dimensional echocardiography].

A two-dimensional echocardiographic study was performed in patients (pts) with isolated ventricular septal defect (VSD). Group I included 48 pts with membranous VSD (aged from 3 months to 11 years) diagnosed by cardiac catheterization and angiocardiography. In 25 of 27 pts demonstrated a defect only by two-dimensional echocardiography (2DE), mean pulmonary artery pressure (MPAP) was higher than 25 mmHg and 26 of these 27 pts showed a large left to right shunt of more than 30 per cent, and the remaining one pt had Eisenmenger complex. In 15 of 16 pts demonstrated VSD with the pouch formation of the septal leaflet (PSL) by 2DE, MPAP was lower than 25 mmHg. These 16 pts showed various left to right shunt ratios (small to large). In all 4 pts demonstrated VSD with membranous septal aneurysm (MSA) and 1 pt demonstrated no findings by 2DE, MPAP was low (under 25 mmHg) and a left to right shunt was small. Group II included 400 pts with membranous VSD (aged 11 days to 15 years) diagnosed clinically. All 221 pts demonstrated VSD had PSL or MSA. PSL was demonstrated in 261 pts, MSA in 27 pts, both PSL and MSA in 33 pts and no findings in 79 pts by 2DE. Group III included 41 pts with spontaneous closed VSD (aged 3 months to 8 years) diagnosed clinically. PSL was demonstrated in 15 pts, MSA in 11 pts and no findings in 15 pts by 2DE. On the basis of these observation, PSL and MSA may play an important role in spontaneous diminution or closure of membranous VSD, and in most of VSD pts without PSL and MSA pulmonary hypertension is shown and spontaneous diminution of VSD size can not be anticipated. Visualization of PSL and MSA by 2DE may predict the prognosis and is clinically very useful in the management of pts with isolated membranous VSD.

Blood Pressure↗

[Myocardial imaging in patients with transposition of the great arteries: comparison between Mustard and Rastelli operations].

Thallium-201 myocardial imaging was performed in 21 patients with transposition of the great arteries (TGA). Thirteen patients were studied before operation, eight patients after Mustard operation and five patients after Rastelli operation. Five patients were studied sequentially before and after the surgery. Each image was compared with hemodynamic and angiographic findings in order to assess the usefulness of this method. In the 13 patients with preoperative TGA, it was possible to differentiate TGA with the intact ventricular septum from TGA with associated anomalies such as patent ductus arteriosus or ventricular septal defect, and also TGA with pulmonary hypertension or pulmonary stenosis from TGA without them by evaluating the degree of thallium uptake in the free wall of the pulmonary ventricle (anatomical left ventricle). The degree of thallium uptake was well correlated with systolic pressure of the pulmonary ventricle (r = 0.98). In the 13 patients with postoperative TGA, it was less correlated with the pulmonary ventricular systolic pressure (r = 0.83). Four cases sequentially studied showed a marked decrease in thallium uptake in the pulmonary ventricle 10 to 16 months after Rastelli operation. But the corrected thallium uptake (thallium activity/ventricular systolic pressure) was more increased compared with the uptake of the systemic ventricle after the surgery. This was probably related to a decrease of the pressure load to the pulmonary ventricle following the surgery and to the fact that there should be some delay of a decrease of the ventricular mass. An increased lung uptake of thallium was noted in the cases of tricuspid regurgitation after Mustard operation, but the degree of regurgitation was not clearly correlated with the increase of thallium uptake in the lung. In five cases after Rastelli operation, one patient showed a high degree of thallium uptake of the pulmonary ventricle (anatomical right ventricle), and the conduit valve calcification and severe pulmonary stenosis were confirmed by cardiac catheterization and angiography. Thus myocardial imaging provides a reliable mean for the qualitative and quantitative assessment of TGA before and after operation.

Child, Preschool↗

Postoperative assessment of arterial reconstructive surgery using Doppler flow detection.

The ankle systolic pressure was measured by the Doppler flow detector during the postoperative course after arterial reconstructive surgery in 61 limbs. The ankle systolic pressure was expressed as a pressure index (PI). There were 2 patterns in the recovery of PI after successful reconstructions. In one of these patterns, PI returned to the normal level within 24 hours. The other group represented a delayed recovery of PI. A comparative study between these 2 groups indicated that the delay in the recovery of PI may be chiefly related to a poor distal run-off. There was no relationship between the recovery pattern of PI and the late patency of reconstructed arteries. On the other hand, follow-up examination by means of the ankle pressure measurement was useful to detect new occurrences of stenotic lesions in the postoperative period.

Adolescent↗

Left ventricle volume characteristics in children with tricuspid atresia before and after surgery.

Left ventricular volume variables (left ventricular end-diastolic volume, ejection fraction, mass and systolic output) were quantified in (1) 13 patients with tricuspid atresia preoperatively (type Ia, 3 patients; type Ib, 9 patients; type IIb, 1 patient), (2) 4 patients after a shunt procedure (Blalock-Taussig, 3; Glenn, 1), and (3) 1 patient after corrective surgery. Cardiac catheterization and angiography were performed at age 1 month to 5.8 years (mean 1.4 years). All patients had decreased pulmonary blood flow. The results were compared with data in 15 patients with no significantly greater than normal in 12 of the 13 patients. Left ventricular mass was significantly greater than normal, but ejection fraction was significantly less than normal. There was a positive correlation between ejection fraction and systemic oxygen saturation. Left ventricular systolic index was normal in 9 of the 13 patients preoperatively. After a shunt procedure, left ventricular end-diastolic volume and systolic index increased significantly in all four patients, and ejection fraction improved greatly in three of the four. After corrective surgery, left ventricular end-diastolic volume and left ventricular systolic index returned to the normal range and left ventricular ejection fraction improved. These findings suggest that patients with tricuspid atresia have impaired left ventricular function.

Cardiac Volume↗

An autopsied case of an elementary school boy with sudden death four years after Kawasaki disease: on the problem of present method of cardiac mass screening of school children.

An 8 year-old boy died suddenly 4 years after the onset of Kawasaki disease. He was examined by a cardiac mass screening for school children one and a half year before the death, and was evaluated as having no sequela of Kawasaki disease. The autopsy showed coronary arterial aneurysms and obstruction with fresh and old myocardial infarction. One of the problems of the present method for cardiac mass screening for school children is that it is performed only with history taking, physical findings and electrocardiograms. This method is completely insufficient to find out coronary involvement as a sequela of Kawasaki disease. All the children with history of Kawasaki disease should be examined by two dimensional echocardiography, which is the most sensitive and specific noninvasive method to detect the coronary involvement in our experience.

Autopsy↗

Cardiac biopsy of Kawasaki disease.

Two hundred one patients (138 boys, 63 girls), 1 month to 11 years old, with Kawasaki disease underwent coronary angiography, ultrasonic tomography, myocardial imaging, and biopsy of right ventricular myocardium. Aneurysms of epicardial coronary arteries, mostly left, occurred in 26 cases (12.9%). Degeneration and proliferation of endothelium, edema, scarring, and fibrosis, slight to moderate, involved the tunicae of intramural small vessels in all cases. These changes tended to abate with time. The basic lesion of Kawasaki disease, round-cell myocarditis and fibrosis, involved all cases and persisted. Disarrangement, abnormal branching, disarray, and hypertrophy of myocytes correlated significantly with myocarditis, but not with epimural or intramural coronary angitis. As a consequence of the myocardial changes, it is suggested that some cases may terminate as myocardiopathy. Scoring criteria for vascular and myocardial changes in biopsy specimens are included.

Biopsy↗