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Biomedical subjects

M Miyao

Publications and source records attributed to M Miyao.

At least 163 records · Page 9Linked to original sources
PubMed1987

Congenital lactic acidosis.

For use in screening for disorders of pyruvate metabolism in cultured skin fibroblasts, we developed a sensitive assay method for measuring the rates of decarboxylation of (1-14C)-pyruvate using dichloroacetate (DCA). By this method, disorders of the mechanism of activation of pyruvate dehydrogenase (PDH) were found in 2 of 10 patients with congenital lactic acidosis of unknown cause. We also found that DCA activated the PDH complex and the tricarboxylic acid cycle, which is an important pathway of energy metabolism, in the brain and other tissues, and lowered the lactate level in the blood and cerebrospinal fluid. Therefore, DCA therapy may be useful in treatment of chronic congenital lactic acidosis if this treatment is started sufficiently early.

Acetates↗
PubMed1987

Effects of dichloroacetate on the mechanical function of the isolated ischemic heart.

The effects of sodium dichloroacetate (DCA) on the activity of the pyruvate dehydrogenase complex (PDH) and the mechanical function of the isolated ischemic rat heart were investigated. Ischemia was induced by restriction of coronary flow. Perfusion with DCA caused a decrease in the tension developed (DT) and the maximal rate of tension development (+ dT/dt max), and an increase in the resting tension (RT) at low flow rates (0.6 and 0.15 ml/min). However, it did not affect the DT or +dT/dt max of normoxic hearts (3 ml/min) or hearts at zero flow rate. DCA increased PDH activity in hearts at all flow rates. It was concluded that this effect of DCA was associated with a decrease in lactate production, rather than an increase in the lactate/pyruvate ratio.

Acetates↗
PubMed1987

Clinical features and cardiopulmonary function of patients with atrophic heart in Duchenne muscular dystrophy.

The clinical features and cardiopulmonary function of 5 patients with atrophic heart in Duchenne muscular dystrophy (DMD) were studied by echocardiography and spirometry. Atrophic heart was defined as a state where the left ventricular end-diastolic volume decreased markedly during long-term follow-up (over 5 years). The patients with atrophic heart were more emaciated and showed more severe motor disability than the controls (18 DMD patients with a normal left ventricular cavity size). The pre-ejection period/ejection time ratio was significantly higher in patients with atrophic heart than in controls, and the maximal diastolic posterior wall velocity was reduced. The forced vital capacity was lower in patients with atrophic heart than in controls, but the one-second forced expiratory volume rate was similar in the 2 groups. Three patients with atrophic heart died. Examination at autopsy showed that their hearts were small and of low weight, and showed not only fibrosis, but also brown atrophy and waxy degeneration. These findings indicate that cardiopulmonary function is significantly reduced in DMD patients with atrophic heart.

Adolescent↗
PubMed1987

1 alpha-hydroxyvitamin D3 treatment of three patients with 1,25-dihydroxyvitamin D-receptor-defect rickets and alopecia.

Three patients with clinically different severities of vitamin D-dependent rickets, type II, with alopecia, which is 1,25-dihydroxyvitamin D-receptor-defect rickets and is particularly resistant to treatment with calciferol analogues, were treated with large doses of 1 alpha-hydroxyvitamin D3 (1 alpha-(OH)D3) and 2 g of calcium lactate. Except for the alopecia, all of the abnormalities of patients 1 and 2 were reversed by treatment with 3 micrograms/kg/d of 1 alpha-(OH)D3, and those of patient 3, who had the severest manifestations, were reversed by treatment with 6 micrograms/kg/d. The serum 24,25-dihydroxyvitamin D concentrations of the three patients were low before treatment and those of patients 1 and 2 increased during treatment. These findings suggest that in patients 1 and 2, 25-hydroxyvitamin D-24-hydroxylase was stimulated via a 1,25-dihydroxyvitamin D-receptor-mediated system by treatment with 1 alpha-(OH)D3.

24,25-Dihydroxyvitamin D 3↗
PubMed1987

[Pharmacokinetic and clinical experience with flomoxef in bacterial infection in children].

Pharmacokinetic and clinical studies were performed on flomoxef (FMOX, 6315-S), a new oxacephem antibiotic, as follows. 1. Pharmacokinetics Serum concentrations of FMOX were measured in 2 cases given 20 mg/kg bolus injection. In the 2 cases, peak concentrations of the drug were 44.3 and 197 micrograms/ml at 15 minutes, T1/2 (beta) were 0.76 and 0.47 hour and AUC were 44.8 and 169.5 micrograms.hr/ml, respectively. Urinary recovery rates for these cases during 6 hours were 83.1 and 54.9%, respectively. The extremely high peak serum concentration in one case may be attributed to dehydration. 2. Clinical efficacy FMOX was administrated intravenously to 12 patients, 6 with pneumonia, 2 with cellulitis, 1 each with bronchitis, tonsillitis, purulent lymphadenitis and subcutaneous abscess, in doses of 55.0-120.0 mg/kg (average 82.2 mg/kg) t.i.d. for 4-13 days (average 6.2 days). The overall efficacy rate was 100%, with excellent responses in 10 and good in 2. Bacteriological efficacy was excellent; 4 of 5 strains were eradicated and 1 strain was decreased. No clinical side effect was observed. Laboratory abnormality was observed in 1 case with transient eosinophilia. The above results suggested that FMOX would be an useful antibiotic for treating pediatric bacterial infections.

Age Factors↗
PubMed1987

[Clinical experience with cefuzonam in bacterial infection of children].

Clinical studies were performed on cefuzonam (L-105, CZON), a new cephem antibiotic, as follows. Cerebrospinal fluid (CSF) and serum concentrations. CSF and serum concentrations of CZON were measured in 1 case of septic arthritis without meningitis. One hour after 50 mg/kg intravenous bolus injection, the CSF and serum concentrations were 0.10 and 18.1 micrograms/ml, respectively, and CSF to serum concentration ratio was 0.55%. Clinical efficacy CZON was administered to 15 patients in doses ranging 54.5 approximately 212.4 mg/kg/day (94.1 mg/kg/day on average) t.i.d. or q.i.d. for 4 approximately 12 days (6.5 days on average). Of those patients, 9 were with pneumonia, one each was with bronchitis, with tonsillitis, with septic arthritis, with septicemia, with purulent meningitis and with urinary tract infection. The overall efficacy rate was 100%, i.e., efficacy was excellent in 12, good in 3. Bacteriological efficacy was excellent, i.e., 8 of 8 strains were eradicated. Side effects were observed in 2 cases, i.e., one case with loose stool and another with eruption. Laboratory abnormalities to the drug were not observed during the treatment. The above results suggested that CZON would be a useful antibiotic for treating pediatric bacterial infections.

Adolescent↗
PubMed1986

Rapid diagnosis of vitamin D-dependent rickets type II by use of phytohemagglutinin-stimulated lymphocytes.

The interactions of 1,25-dihydroxyvitamin D3 [1,25-(OH)2D3] with phytohemagglutinin (PHA)-stimulated lymphocytes from normal subjects and three patients with vitamin D-dependent rickets (DDR) type II were investigated. Impaired nuclear uptake and normal cytosol binding of [3H]1,25-(OH)2D3 were observed with PHA-stimulated lymphocytes of these patients as with their cultured skin fibroblasts. Furthermore, the incorporation of [14C]thymidine into PHA-stimulated lymphocytes of the patients was not reduced by 1,25-(OH)2D3, which is known to inhibit proliferation of various cells. These findings suggest that 1,25-(OH)2D3 receptors are reduced or absent in patients with DDR type II. Thus, the capacities of cytosol binding and nuclear uptake of 1,25-(OH)2D3 in PHA-stimulated lymphocytes seem to reflect those of endo-organs such as the intestine and bone. These findings show that a test of the effect of 1,25-(OH)2D3 on thymidine incorporation into PHA-stimulated lymphocytes is useful for rapid diagnosis of DDR type II.

Calcitriol↗
PubMed1986

Involvement of thiol proteases in galactosialidosis.

The activities of Z-Phe-Arg-NMec(ZPA) hydrolase, cathepsin B and cathepsin H and the concentration of endogenous thiol protease inhibitor in fibroblasts from patients with galactosialidosis were found not to be significantly different from those in control fibroblasts. Culture for 5 days with thiol protease inhibitors such as leupeptin, E-64 or Z-Phe-Phe-CHN2 partially restored the beta-galactosidase activity of fibroblasts from patients, but did not affect the beta-galactosidase activity of fibroblasts from control subjects. However, culture with leupeptin, but not other protease inhibitors, increased the ZPA hydrolase and cathepsin B activities of fibroblasts from both patients and controls 2- to 4-fold. Sephadex G-75 chromatography showed that the activity of high molecular weight ZPA hydrolase, which was initially predominant in fibroblasts, decreased markedly during their culture with leupeptin, while the activities of lower molecular weight ZPA hydrolase and cathepsin B increased about 5-fold. These results suggest that high molecular weight ZPA hydrolase, which is presumably cathepsin J, degrades beta-galactosidase, and that the defect in galactosialidosis is impaired protection of beta-galactosidase from degradation.

Cathepsin B↗