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Biomedical subjects

M Miyao

Publications and source records attributed to M Miyao.

At least 217 records · Page 12Linked to original sources

Activation of branched-chain alpha-ketoacid dehydrogenase complex by alpha-chloroisocaproate in normal and enzyme-deficient fibroblasts.

A method has been developed for the activation of the branched-chain alpha-ketoacid dehydrogenase complex by alpha-chloroisocaproate, an inhibitor of branched-chain alpha-ketoacid dehydrogenase kinase in human cultured skin fibroblasts. The enzyme could be activated by pretreating the cells with alpha-chloroisocaproate before they were disrupted for measurement of the activity. After this treatment, the activity was 2- to 3-fold that of untreated cells (24.8-81.4 pmol/min per mg protein). The enzyme activity in fibroblasts from a patient with maple syrup urine disease was measured by this procedure. After activation by alpha-chloroisocaproate, the activity of fibroblasts from the patient was only 10-14 pmol/min per mg protein (10% of that of controls), and was almost the same as that of the untreated cells from this patient. These results show that it is important to consider the activation state of branched-chain alpha-ketoacid dehydrogenase complex when assaying it in disrupted cells.

3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)↗

Hereditary renal hypouricemia in children.

The renal handling of urate was investigated in four children with hereditary renal hypouricemia and in their parents. The urate/creatinine clearance ratios in the four patients were 1.02 +/- 0.28, 0.93 +/- 0.11, 1.03 +/- 0.24, and 1.46 +/- 0.26, markedly higher than those in control subjects. Except for a partial response to pyrazinamide (change in clearance ratio from 1.46 to 1.07) in one patient, pyrazinamide and benzbromarone did not affect the clearance ratios in our patients. In the parents, the urate/creatinine clearance ratios were intermediate between those of the patients and control subjects, but responses to pyrazinamide and benzbromarone were normal. These data indicate that our patients have a combined defect in renal urate reabsorption, and that one of them might be subclassified as having the hypersecretion of defect. Results also show that heterozygotes can be identified by testing their urate/creatinine clearance ratio.

Benzbromarone↗

Short latency somatosensory evoked potentials to peroneal nerve stimulation in normal Japanese children.

Short latency somatosensory evoked potentials (SSEP) were elicited by stimulation of the peroneal nerve in 68 normal children of 39 weeks to 15 years old. In all subjects, three positive potentials (P1, P2 and P3) and one negative potential (N1) were consistently recorded. A further positive potential (P4) after N1 was not always observed. There was no change of wave form with development. P1, P2, P3 and N1 might be generated in subcortical structures; caudal cervical spine, brainstem, thalamus and thalamocortical pathway, respectively. The latency of each peak per one meter body length decreased with age until 5 or 6 years of age. Moreover, the latency between peaks per one meter body length also decreased with age until 5 to 6 years of age. These findings are consistent with the development of SSEP on median nerve stimulation and with the developmental phenomenon of spinal conduction velocity, and might be related to the increase in the diameter and the progressive myelination of nerve fibers.

Adolescent↗

Diagnosis of partial deficiency of the pyruvate dehydrogenase complex in biopsied muscle.

We have measured the total activity of pyruvate dehydrogenase (PDH) complex, by in vitro activation with a broad specificity protein phosphatase, and the basal activity, supposed to be present in vivo, in biopsied muscles from three patients with PDH complex deficiency and 11 patients with lactic acidemia. Results showed that the total PDH complex activity must be determined in biopsied muscles for the diagnosis, because the basal activities of two of three patients with PDH complex deficiency overlapped those of two patients with lactic acidemia whose total activities were within normal range.

Adult↗

Effect of respiratory acidosis on ventricular shunt flow and hemodynamics in dogs with ventricular septal defect.

The effects of respiratory acidosis on ventricular shunt flow and hemodynamics were studied in 20 anesthetized dogs with a ventricular septal defect and a normal pulmonary vascular bed. The interventricular shunt flow was measured directly by using a specially designed electromagnetic flow probe. Respiratory acidosis was produced by hypoventilation and tachypnea with constant minute volume. Hypoxemia was also induced by hypoventilation, but not by tachypnea with constant minute volume. Systemic vascular resistance was increased in severe hypoventilation at 100 and 50 ml of tidal volume, and tachypnea at 100 ml of tidal volume. However the increase of pulmonary vascular resistance was observed in only severe hypoventilation: arterial pH 6.9, PaO2 24 mmHg, and PaCO2 88 mmHg. Left to right ventricular shunt flow and pulmonary blood flow were increased significantly with no change of systemic blood flow in both conditions of respiratory acidosis. The diastolic fraction of shunt flow was increased significantly. These findings indicate that the increase of left to right shunt flow in respiratory acidosis might be one of the risk factors of congestive heart failure for the patients with ventricular septal defect.

Acidosis, Respiratory↗

[Clinical studies on aspoxicillin in the field of pediatrics].

Clinical studies were performed as follows on aspoxicillin (ASPC), a new semisynthetic penicillin. ASPC was intravenously given to 12 patients in doses of 57.7-129.0 mg/kg on average) t.i.d. or q.i.d. for 4-7 days (5.7 days on average): 9 with pneumonia, 1 with tonsillitis, 1 with purulent lymphadenitis and 1 with urinary tract infection. The overall efficacy rate was 83.3%, i.e. efficacy was excellent in 8 (66.7%), good in 2 (16.7%), fair in 1 (8.3%) and poor in 1 (8.3%). Bacteriological efficacy was excellent, i.e. 6 of the 6 strains disappeared. No clinical side effects were observed during treatment. Laboratory abnormalities were observed in 3 cases, slight elevation of GOT in 1, slight elevations of GOT and GPT in 1 and mild eosinophilia in 1. The above results suggest that ASPC is an useful antibiotic for treating pediatric bacterial infections.

Amoxicillin↗

[Clinical experience with ceftizoxime suppositories in bacterial infections in children].

A clinical trial of ceftizoxime suppositories (CZX-S) was performed to evaluate the therapeutic effectiveness in children with bacterial infection. The subjects were 10 children comprising 4 with pneumonia, 3 with lacunar tonsillitis, 2 with pharyngitis, and 1 with UTI. They were given 1 suppository containing either 125 mg or 250 mg of CZX 2 to 4 times a day. The daily per kg body weight dose ranged from 17.1 to 60.0 mg. The result was "markedly effective" in 3, "effective" in 6, and "failure" was recorded in 1. Bacteriologically, successful eradication of causative organisms was confirmed in all the 4 children who underwent the test. No clinical side effects were observed. The only laboratory test abnormality recorded in a single patient was eosinophilia, which was not definitely ascribable to CZX-S. In conclusion, CZX-S have proved to be a clinically safe and effective antibiotic preparation in infantile infection, even in children whose treatment with conventional antibiotics is associated with difficulties.

Bacteria↗

[Laboratory and clinical studies on cefminox in the pediatric field].

Laboratory and clinical studies were performed as follows on cefminox (CMNX, MT-141), a new cephamycin antibiotic. Pharmacokinetics Serum concentrations of CMNX were measured in 4 patients given CMNX for prophylactic purpose during cardiac catheterization. In 2 patients given 20 mg/kg of CMNX by intravenous bolus injection, the average of peak serum concentration was 178.9 micrograms/ml at 15 minutes. The mean urinary recoveries in these 2 cases was 66.9% within 6 hours after injection. In 2 patients given 20 mg/kg of this drug by 1 hour drip infusion, the peak serum concentration was obtained at the time drip was completed, and the average value was 68.3 micrograms/ml. Clinical efficacy CMNX was administrated intravenously to 13 patients in dose of 52.9 approximately 96.0 mg/kg t.i.d. or q.i.d. for 4 approximately 7 days; 3 with tonsillitis, 6 with bronchitis, 1 with bronchopneumonia, 1 with UTI, 1 with lymphadenitis and 1 with enterocolitis. The overall efficacy rate was 92.3%, i.e., efficacy was excellent in 12, and poor in 1. Bacteriological efficacy was excellent, i.e., 3 of 3 strains disappeared. Side effects were observed in 3 cases, i.e., 1 case of eruption, 1 case of diarrhea and 1 case of transient eosinophilia. The above results suggest that CMNX is a useful antibiotic for treating pediatric bacterial infections.

Anti-Bacterial Agents↗

A case of congenital lymphoblastic leukemia: clinical course and significance of ultrasonography.

A case of congenital leukemia in which texture changes in ultrasonography were followed up is reported. The patient was a male infant hospitalized on day 22 after birth with multiple bluish skin nodules. Complete remission was achieved easily, but he relapsed early and died from the disease after six months. Because of L1 morphology the disease was classified as acute lymphoblastic leukemia, but the leukemic cells lacked the characteristics of lymphoid lineage on cytochemical, cytogenetic, immunological, and enzymological examination. Ultrasonography was considered a useful tool in the following respects for the treatment of the patient with congenital leukemia: The texture of the infiltration of leukemic cells into the liver was clearly visualized and changes in the images could be traced both in size and in number; the process of ventricular dilatation and brain atrophy were successively visualized.

Atrophy↗