[A case of progressive dystonia with serum anti-neuronal antibodies against to the basal forebrain cholinergic neurons].
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Biomedical subjects
Publications and source records attributed to R Oka.
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In vivo anesthetic activity of alkyl acetates in mice was studied in relation to hydrophobicity and the in vivo effect on membrane fluidity. The anesthetic potency (AD50) of alkyl acetates was determined; AD50 shows the i.p. dose required to anesthetize 50% of mice from the treated group. We used log P (n-octanol/water partition coefficient) as an operational definition of hydrophobicity. Membrane fluidity was determined using 1,6-diphenyl-1,3,5-hexatriene (DPH) as fluorescence probe. Log (1/AD50) was a parabolic function of log P, and the value of log P that corresponds to the minimum AD50 was estimated to be 2.08. Brain synaptosomal membranes were prepared from mice 30 min after dosing with each of the three alkyl acetates applied at 1.5-fold AD50: n-butyl, n-amyl, and n-hexyl acetate. In each alkyl acetate group, most of the animals were anesthetized (> 68%). Decreased membrane fluidity was observed for the animals that were anesthetized while no change in the fluidity was seen for the animals that were not anesthetized. The results suggest an involvement of decreased DPH fluidity in alkyl acetate-induced anesthesia.
Povidone-iodine sugar ointment is an excellent preparation for the treatment of decubitus. It has been used as an intrahospital preparation made according to the formula each hospital decided on from experience. Although commercial products have also been developed and used, they are too expensive. The efficacy of a povidone-iodine sugar ointment formulation which can be prepared by a single method and which has the stability and antibacterial activity equal to commercially available products was evaluated. As the test drugs, one commercially available product (UP), and three preparations with different formulas (P-1, P-2 and P-3) were used. All of these test drugs were stored at 20 and 40 degrees C. Specimens were sampled immediately after storage and after 20, 60, 90, 120 and 150 days and examined pharmaceutically (measurement of pH value and determinations of available iodine and sucrose levels). For the determination of bacteriological effects, 5 standard strains of 5 genera and 5 strains of methicillin-resistant Staphylococcus aureus (MRSA) were used and the time required to kill the bacteria was determined. For UP and P-3, no changes were seen pharmaceutically after 150 days of storage at 20 and 40 degrees C. However, MRSA could not be killed within 30 min. P-1 and P-2 showed remarkable changes pharmaceutically after 60 days of storage at 40 degrees C and could not be used any more. It became possible to make a preparation of povidone-iodine sugar ointment which has a stability almost similar to that of UP. Moreover, such a preparation can be made at low cost. However, since the bactericidal activity against MRSA was not higher than those of other drugs, the future task is to improve the bactericidal activity.
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We report a very rare case of an infant with coronary arterial fistula in whom the communication between a dilated right coronary artery and the coronary sinus was found. The patient was 1-year-old. She had pulmonary congestion and cardiomegaly due to the left-to-right shunt through the fistula, and underwent ligations of the fistula. Ligations of the fistula was indicated because the patient was too small to undergo a fistula resection and coronary arterial bypass grafting. Although the patient had a successful post-operative course, we are paying attention to the aneurysmal change of the dilated right coronary artery proximal to the site of ligations.
We report a case of a newborn with Shone's anomaly in whom the parachute mitral valve, mild subaortic stenosis, hypoplastic aortic arch, and severe coarctation of the aorta were found. The patient was 10 days old, who had symptoms of low output syndrome due to the progressive obstruction of patent ductus arteriosus, underwent "extended aortic arch anastomosis" to repair the hypoplastic aortic arch and severe coarctation of the aorta. This surgical procedure, resulting in a successful post-operative course of the patient, might be effective in such anomalies.
Abnormalities of skeletal muscle function and metabolism are common in patients with congestive heart failure (CHF) and appear to contribute to systemic exercise limitation. Although the mechanism for these differences is unclear, one possibility is skeletal muscle atrophy. In 21 CHF patients and 12 sex- and age-matched sedentary control subjects, we quantified muscle size as maximal cross-sectional area (MCSA) of thigh muscles measured by magnetic resonance imaging and determined the relationship between muscle size and muscle function. Muscle strength was measured as maximum force developed during isometric contractions, and muscle endurance was quantified as the decline in force during 15 consecutive isokinetic knee extensions (measured as ratio of mean peak torque of last 3 and first 3 extensions). MCSAs of thigh muscles (141 +/- 28 vs. 167 +/- 47 cm2, P < 0.05) and knee extensors (62 +/- 13 vs. 75 +/- 13 cm2; P < 0.05) were both significantly smaller in patients than in control subjects. These differences persisted after normalization for body size. Isometric strength was less, but not significantly so, in patients (126 +/- 39 vs. 135 +/- 43 Nm; P = NS), but muscle endurance was markedly impaired (endurance ratio 0.67 +/- 0.14 vs. 0.83 +/- 0.11; P < 0.05). A strong correlation was found between isometric strength (r = 0.76) and MCSA of knee extensors, but only a weak correlation between dynamic endurance and MCSA was seen. We conclude that muscle size is smaller in CHF patients but that maximal force generated per area of muscle is not impaired.(ABSTRACT TRUNCATED AT 250 WORDS)
A medical examination of 932 Vietnamese refugees was conducted within 1 month of their resettlement in Japan between 1989 and 1991. A variety of abnormalities were detected, including parasitic disease (78% prevalence), anemia (12%), HBsAg positive state (14%), liver dysfunction (10%), hypertension (0.8%), active pulmonary tuberculosis (2%) and syphilis (0.7%). These rates were still as high as the prevalence in previous studies of earlier immigrants from Vietnam. The high frequency of infectious diseases in recent Vietnamese refugees compared with the Japanese community leads to a recommendation for continuing medical examinations and treatment for new Vietnamese refugees.
We described the pharmacokinetics of acetylsalicylic acid and salicylic acid in 5 children with prior Kawasaki disease receiving buffered aspirin tablets or intravenous aspirin DL-lysine. Oral aspirin is characterized by its longer mean residence time in the body (MRT), an indicator for duration of exposure, than intravenous aspirin (1.18 vs. 0.37 h). The apparent elimination half-life (t1/2) of acetylsalicylic acid was also longer after oral than after intravenous administration of aspirin (40 vs. 17 min). The mean absorption time was calculated to be as long as 0.8 h. In contrast, the two modes of administration gave virtually identical results regarding MRT of salicylic acid proper and its t1/2. After oral administration of aspirin, 51% of the dose is absorbed intact as acetylsalicylic acid while 23% is converted to salicylic acid presystemically, indicating that 74% of aspirin is actually absorbed. Our data suggest that an equivalent intravenous aspirin dose to achieve the same salicylic acid concentration is about 70% of the oral dose. However, the area under the curve of acetylsalicylic acid itself, a more potent cyclooxygenase inhibitor than salicylic acid, would be about 1.5 times higher than that following oral administration. The clinical consequences of this exposure difference remain uncertain.
In patients with congestive heart failure (CHF), the poor relationship between systemic exercise performance and cardiac function, together with morphologic and metabolic abnormalities in skeletal muscle, raises the possibility that skeletal muscle function may be impaired and limit systemic exercise performance. We assessed strength and endurance of the knee extensors during static and dynamic exercise in 16 patients with Class I-IV CHF and eight age-matched sedentary controls and related these measurements to systemic exercise performance. To assess skeletal muscle function independent of peripheral blood flow, endurance was repeated under ischemic conditions. Strength was not significantly different in the two groups. Dynamic endurance, quantified as the decline in peak torque during 15 successive isokinetic knee extensions, was significantly reduced in the patients compared to controls during aerobic (peak torque 65 vs. 86% of initial for exercise at 90 deg/s and 60 vs. 85% for exercise at 180 deg/s; P less than 0.002 for both), and during ischemic exercise (56 vs. 76% of initial torque; P less than 0.01). Static endurance, defined as the time required for force during a sustained maximal voluntary contraction to decline to 60% of maximal, was reduced in the patients compared to controls (40 +/- 14 vs. 77 +/- 29 s; P less than 0.02). There were highly significant relationships between systemic exercise performance and skeletal muscle endurance at 90 and 180 deg/s in the patients with CHF (r = 0.90 and 0.66, respectively). These findings indicate that skeletal muscle endurance is impaired in patients with CHF, that this abnormality is in part independent of limb blood flow, and that these changes may be important determinants of systemic exercise performance.
The high-frequency components of the surface QRS was analyzed quantitatively in 87 normal infants and children. The subjects were categorized in 4 groups by age; Group A: one day (n = 27), Group B: 1-5 years (n = 20), Group C: 6-10 years (n = 20), Group D: 11-15 years (n = 20), and the results were compared among the 4 groups. Signal-averaging and high-pass, bidirectional and digital filtering were used for analysis. The total duration of the QRS, the duration of the low-amplitude signals (< 40 microV) in the terminal portion of the QRS (U 40), the amplitude of the signals in the QRS (RMS; QRS) and the amplitude of the last 40 msec of the QRS (RMS 40) were measured at high-pass filter frequency settings of 25, 40, 60 and 100 Hz. Statistical analysis was performed with the unpaired t-test. Differences were considered significant if p < 0.05. The results were as follows: 1. At all filters, the durations of the QRS and U 40 were longer in the order of Groups A < B < C < D, and the amplitude of the RMS; QRS and RM 40 were greater in the order of Groups D < C < B < A. 2. There were significant differences in the QRS between the groups at all filters. 3. There were significant differences in U 40 between the groups except between Groups B and C at 25-Hz filter, but at 40-, 60-, 100-Hz filters, there were no significant differences among Groups B, C and D.(ABSTRACT TRUNCATED AT 250 WORDS)
To find reliable indicators of digoxin clearance (CLdig) in the neonatal period, we investigated the linear correlation of CLdig and the reciprocal of CLdig (1/CLdig) with serum beta 2-microglobulin (S beta 2-MG), serum creatinine, blood urea nitrogen, age, and weight on 25 occasions in 21 neonates with congenital heart disease. The S beta 2-MG value showed a significantly closer correlation to 1/CLdig (r = 0.84, p less than 0.0001) than to the other values. The regression equation was (1/CLdig) = 0.15 X (s beta 2-MG) + 0.08. Creatinine and blood urea nitrogen values correlated less closely with 1/CLdig (r = 0.67 and 0.71, respectively). Age and weight had no significant linear correlation with CLdig and 1/CLdig. Determination of s beta 2-MG values allowed an estimate of CLdig by means of the regression equation between s beta 2-MG and 1/CLdig, and permitted a prediction of the required maintenance dose of digoxin. We conclude that s beta 2-MG is a good indicator of CLdig in neonates, and that the determination of s beta 2-MG values may facilitate the advance individualization of digoxin therapy in the neonatal period.
A 39-year-old woman was admitted to our hospital complaining of hemosputum and right neck swelling. Pharyngography, neck CT scan and laryngoscopy revealed moderately differentiated squamous cell carcinoma of the right pyriform sinus. After series of examinations, it was found that she had pancytopenia, hypoplastic bone marrow, hyperpigmentation of the skin, cardiac anomaly, small stature, hypogonadism, chromosomal aberrations and consanguinity in her parents. These findings suggested that she was the congenital aplastic anemia, that is Fanconi's anemia, variant form. Although pepleomycin and corticosteroids were administrated for the treatment of squamous cell carcinoma and aplastic anemia, she died of cardiovascular shock due to massive hemorrhage. Flow cytometric analysis of squamous cell carcinoma showed an unusual aneuploidy DNA histogram. This is the first report on Fanconi's anemia with squamous cell carcinoma in Japan. It is said that chromosomal aberrations and impairment of DNA cross-links repair may play an important role developing of malignancy in Fanconi's anemia.
A new glucose 6-phosphate dehydrogenase (G6PD) variant associated with chronic nonspherocytic hemolytic anemia was discovered in Japan. The patient showed hemolytic crises after upper respiratory infections. The enzyme activity was about 3.8% of the normal. The partially purified enzyme revealed slow anodal electrophoretic mobility, high Km NADP, marked thermal-instability, and increased affinity for a substrate analogue (deamino-NADP). A particular characteristic of this enzyme was a biphasic pH curve with a greatly increased activity at low pH values. From these results, this variant was clearly different from hitherto observed G6PD variants, and was designated G6PD Asahikawa.
Since 1977, we have used a live attenuated varicella vaccine to immunize 10 children with acute leukemia. 8 patients had no adverse clinical reaction but 2 patients developed mild fever and papulovesicular rash after vaccination. All 9 tested children became seropositive after the vaccination. Also in all 3 children who were observed for more than 4 years, persistence of neutralizing antibody was detected. Most of the recipients were prevented from developing symptoms of varicella in spite of contact exposure. Two patients developed varicella when they were in severe immunosuppressive states but their symptoms were mild. None of the children developed herpes-zoster during the 6 year follow-up period. The results suggest that the varicella vaccine is effective in children with acute leukemia, and that long-term effectiveness can be expected.
A 15-month-old boy with posterior mediastinal endodermal sinus tumor is reported. The patient was admitted with recurrent hemothorax, and his serum alpha-fetoprotein level was 29,520 ng/ml. After removal of the posterior mediastinal tumor, VAC therapy (vincristine, actinomycin D, cyclophosphamide) was started. The chemotherapy was continued 18 months. The patient continues to do well with no sign of tumor and a normal alpha-fetoprotein level 53 months after the operation and 35 months after the completion of therapy. He is the youngest patient documented in the literature and successfully treated for mediastinal endodermal sinus tumor.