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Biomedical subjects

K Sugai

Publications and source records attributed to K Sugai.

At least 91 records · Page 5Linked to original sources

[Anesthesia for a patient with spinocerebellar degeneration who developed atrioventricular block].

A 54-year-old female with a history of spinocerebellar degeneration (SCD) and myoma uteri had general anesthesia for the abdominal total hysterectomy. Though the operation was uneventfully performed, various atrioventricular blocks occurred. These might be related to the SCD. Hypertonic state of the vagus was considered to be the quite possible cause of the block. Myocardial degeneration associated with the SCD was also an undeniable factor of the block. No arrhythmia occurred and symptoms of the SCD were not aggravative after the anesthesia and the patient was discharged uneventfully.

Anesthesia, General↗

[A family of congenital combined deficiency of factor V and von Willebrand factor].

A family with inherited combined deficiency of factor V and von Willebrand factor (vWF) is reported. Hematological examination of 41 year-old female proband and her younger brother revealed prolonged prothrombin time and Kaolin partial thromboplastin time. The level of both factor V activity and factor V antigen markedly decreased, below 15% of normal. The decreased levels of factor VIII activity and vWF activity are also seen. Furthermore, abnormal mobilities were observed in crossed immunoelectrophoresis. The protein C, S antigens and activities, and protein C inhibitor activity were within normal. Four sons have received the 50% levels of factor V from their parents. One of them also showed the 50% of factor VIII and vWF activities. From above results, this family is thought to be a case of inherited deficiency of factor V and vWF, which are transmitted as an autosomal trait apparently.

Adult↗

Positron emission tomography using pyruvate-1-11C in two cases of mitochondrial encephalomyopathy.

Positron emission tomography (PET) using pyruvate-1-11C was carried out to investigate the in vivo metabolism of pyruvate in the brains of patients with mitochondrial encephalomyopathy and Leigh's disease. Two epileptic patients were studied as control subjects. Radioactivity was eliminated from the brain tissue of the epileptic patients soon after injection of pyruvate-1-11C. PET images of mitochondrial encephalomyopathy patients showed an increase in radioactivity in the cerebral cortex, basal ganglia and thalamus, with elimination of radioactivity being slower than that of epileptic patients. One patient with Leigh's disease showed similar PET images. PET using pyruvate-1-11C is useful for the evaluation of mitochondrial energy metabolism in the brain.

Brain Diseases↗

Muscle histochemistry in myotubular (centronuclear) myopathy.

We report the clinical and histochemical findings in 7 patients with myotubular (centronuclear) myopathy aged from 2 months to 32 years. The clinical symptoms varied from patient to patient. Three patients developed severe muscle weakness and hypotonia with respiratory distress from infancy, and 4 had muscle weakness from 2-5 years of age with no apparent delay in developmental milestones. In addition to an increased number of fibers with centrally placed nuclei, there were 3 other histochemical characteristics of this disorder, i.e., type 1 fiber predominance, type 1 fiber hypotrophy and type 2B fiber deficiency. Other histological findings included a peripheral halo in the sarcoplasm on NADH-TR staining and an increased number of undifferentiated type 2C fibers, indicating a delay in muscle fiber growth and differentiation due to a probable defective neural supply in the developing muscles.

Adult↗

Decreased blood flow and oxygen metabolism in the cerebellum, brain stem and thalamus in a case with Menkes kinky hair disease.

Cerebral blood flow and oxygen metabolism were measured in a five-year-old boy with atypical Menkes kinky hair disease (MKHD) by using positron emission tomography (PET). The patient was diagnosed as having atypical MKHD because of low serum and urinary copper levels, and clinical symptoms. The CT revealed mild to moderate degrees of brain atrophy predominantly in the cerebellum. The PET demonstrated marked decreases of cerebral blood flow and oxygen metabolism in the cerebellum, brain stem and thalamus. These findings seem to reflect the neuropathological abnormalities observed in MKHD. PET seems to be more sensitive than CT in detecting abnormalities in the affected structures. However, because this case is atypical the question of whether typical cases show similar features on the PET remains.

Brain Diseases, Metabolic↗

Effects of glycyrrhizin (SNMC: stronger Neo-Minophagen C) in hemophilia patients with HIV infection.

Glycyrrhizin (GL) not only has an inhibitory effect on HIV replication but also exhibits interferon-inducing and natural killer (NK)-enhancing effects and improves liver dysfunction. Thus, large doses of GL (200-800 mg/day) were intravenously administered for more than 8 weeks to 9 hemophilia A patients with HIV infection (asymptomatic carrier, AC). Lymphocyte count increased in all 9 cases. OKT4 OKT8 ratio was elevated in 6 out of the 9 cases and OKT4-positive lymphocytes increased in 8 out of the 9 cases; 66.7% and 88.9% improvement, respectively. Changes in NK cell activity and mitogenic responsiveness to PHA, Con A and PWM were not significant. Liver dysfunction, noted in 4 cases, clearly improved. Serum electrolytes, protein, lipids, and renal function were within normal levels and no serious side-effects were observed during treatment. On the other hand, in 3 cases of hemophilia without HIV infection, the number of OKT4 lymphocytes was not significantly altered during treatment. From these results, large dose administration of GL to HIV-positive hemophilia patients (AC) seems to be effective in preventing development of AC into AIDS by raising the number of decreased OKT4 lymphocytes and improving liver dysfunction.

Acquired Immunodeficiency Syndrome↗

[The effects of ischemia and postischemic reperfusion on the brain cholinergic system of the rat].

Using experimental cerebral ischemia induced in rats with 4-vessel occlusion, the effects of 2-hour ischemia (ligation group) and 30-minute ischemia followed by 72-hour reperfusion (reperfusion group) on cholinergic system (ChS) was investigated with acetylcholine receptor (AChR), choline acetyltransferase (CAT) and acetylcholine esterase (AChE) as indicators of ChS in seven sections of the brain. The activity of CAT was reduced just after ischemia and tended to decrease further after reperfusion. CAT is known as the specific enzyme of cholinergic neuron (ChN), so the reduction of activity of CAT probably means that the ischemic change of ChN was irreversible after 30-minute occlusion. The activity of AChE was also reduced just after ischemia but tended to recover after reperfusion. AChE is not specific in ChN, so the activity of this enzyme may also reflect activity of postsynaptic neuron. The recovery tendency of AChE, therefore, may have the meaning that the ischemic change of the postsynaptic neuron was reversible. The number of AChR was found to have the tendency to increase after ischemia in more sections of reperfusion group compared with ligation group, which is supposed to show that compensatory reactive increase of the receptor may be retained after reperfusion.

Acetylcholinesterase↗

[Epidural anesthesia for a patient with Charcot-Marie-Tooth disease, bronchial asthma and hypothyroidism].

The patient was a 75-year-old male with a history of Charcot-Marie-Tooth disease (CMT), bronchial asthma and hypothyroidism simultaneously. Epidural anesthesia was planned for the open reduction of a femur fracture. CMT is one of the hereditary motor and sensory neuropathies, and there may be no report in Japan that epidural anesthesia was applied to a patient with CMT. It has been suggested that neuromuscular disease is a contraindication to epidural anesthesia, but the patient had such a severe gait disturbance that he might not be able to walk by himself postoperatively without any aggravation of the neuropathy. In addition, there existed the other simultaneous complications, especially severe respiratory insufficiency. Therefore we dared to administer epidural anesthesia. The epidural anesthesia was safely performed during the operation and there was no untoward effects postoperatively.

Aged↗

The interaction of pancuronium and vecuronium with cardiac muscarinic receptors.

The interaction of vecuronium, a monoquaternary analogue of pancuronium, with the cardiac muscarinic receptors in canine hearts was investigated in vitro by [3H] QNB binding assay and compared with that of pancuronium. Both pancuronium and vecuronium, which are nicotinic antagonists of competitive types, inhibited the binding of [3H] QNB to cardiac muscarinic receptors with values of IC50 of 5.41 X 10(-7) mol/l and 3.97 X 10(-6) mol/l respectively. According to the Kd and Bmax values on the Scatchard plot, pancuronium, while not influencing the number of receptors, had a 3-fold greater inhibitory effect than vecuronium on the affinity of the muscarinic receptors. The KI values of vecuronium and pancuronium showed that pancuronium had a 7.3-fold greater affinity with the receptors than vecuronium. We concluded that vecuronium had a direct inhibitory action on the binding of [3H] QNB to the canine heart muscarinic receptors, but this action was much weaker than pancuronium.

Animals↗

The control of hypertension with dibutyryl cyclic AMP.

The effects of the rapid infusion of large doses of dibutyryl cyclic AMP (DBcAMP) were studied to clarify the clinical usefulness of its vasodilating action in 32 middle-aged patients, who underwent various types of surgery and developed systolic hypertension of over 160 mmHg during general anaesthesia. DBcAMP was given i.v. with an infusion pump at a rate of 0.6 mg kg-1 min-1 for 20 min. In all patients just after the infusion, systolic arterial pressure decreased from 174.0 +/- 20.7 to 129.0 +/- 23.9 mmHg, diastolic pressure decreased from 93.1 +/- 13.4 to 64.8 +/- 13.3 mmHg, heart rate increased from 81.2 +/- 15.7 to 91.5 +/- 19.5 beats min-1, and urine volume increased from 69.4 +/- 54.8 to 182.7 +/- 143.5 ml h-1. In three patients, cardiac index increased from 3.44 to 4.24 l min-1 m-2. In seven patients, tachycardia exceeding 120 beats min-1 developed. DBcAMP was also effective in patients with a history of hypertension. The strongest antihypertensive effect was observed in patients anaesthetized with nitrous oxide-oxygen and enflurane. We speculate that DBcAMP is useful to control hypertension and may be particularly indicated in patients with cardiac failure, renal disorders and essential hypertension.

Aged↗

Cerebro-oculo-hepato-renal syndrome (Arima' syndrome): a distinct clinicopathological entity.

Three children with Leber's congenital amaurosis, agenesis of the cerebellar vermis, and infantile polycystic kidneys are described. The common clinical findings of three unrelated patients (two boys and one girl) included severe visual impairment from early infancy, profound psychomotor retardation, hypotonia, nystagmus, characteristic facial appearance with blepharoptosis, and progressive chronic renal insufficiency. The two boys died of uremia at ages 13 and 12 years. The common pathological findings in these two patients consisted of minor disproportions of cerebral lobes, almost total aplasia of the cerebellar vermis, micropolygyria of the dentate nuclei, malformations of the brain stem (including pachygyria of the inferior olivary nuclei and partial absence and anomalous position of the pyramidal tracts), and infantile polycystic kidneys; there was fatty liver in one case and hepatic fibrosis in the other. The clinicopathological findings of our two patients were entirely compatible with those of patients previously reported by Arima and other Japanese authors. Therefore, these patients seem to comprise a distinct clinicopathological entity, cerebro-oculo-hepato-renal syndrome (Arima's syndrome), different from other syndromes with retinal, cerebellar, and renal abnormalities.

Blindness↗

Morphological changes of platelets during the process of platelet aggregation in gray platelet syndrome.

Morphological changes of platelets during the process of platelet aggregation in gray platelet syndrome were observed by means of light microscopy, and scanning and transmission electronmicroscopy. Light microscopic examinations showed normal platelets and slightly enlarged, gray colored ones due to a deficiency in alpha-granules by May-Giemsa stain. At the time of maximum aggregation, normal platelets aggregated completely to form a mass by addition of ADP. On the other hand, patient's platelets only aggregated loosely and each platelet could be recognized separately, resembling primary aggregation of normal platelets. When collagen was added, almost no aggregation was noted in the patient's platelets. Scanning electronmicroscopic examinations revealed the same results as seen by light microscopy. Centralization of platelet organelles and almost complete aggregation and degranulation of platelets were noted immediately after 30 sec and 3-4 min after addition of ADP and collagen on the transmission electronmicrographs in normal platelets, whereas most patient's platelets remained to be unchanged. From these results, we concluded that these morphological changes were consistent with the changes of "release abnormalities" as shown in the functional examinations. Although some relationships between alpha-granule deficiency and functional abnormalities are considered, there is no definitive opinion.

Adenosine Diphosphate↗

Plasma levels and urinary excretion of batroxobin and its defibrinogenating effects in various animal species.

The plasma levels and urinary excretion of batroxobin administered to 6 species of animals were examined by an enzyme immunoassay method. Defibrinogenating effect of batroxobin was also studied in those species. The plasma levels of immunoreactive batroxobin disappeared exponentially in all the animals and differences in half-life were observed to occur according to species. The elimination half-life of immunoreactive batroxobin in the plasma was the largest in dogs, followed by rats, monkeys, guinea pigs, mice and rabbits. The extent of the defibrinogenating effect was also noted to vary according to the species, being greatest in dogs and then monkeys, mice, rats, guinea pigs and rabbits. Following the continuous infusion of batroxobin into dogs, its level in the plasma remained high over a considerable period of time and the defibrinogenating effect lasted in corresponding to its plasma level. The urinary excretion of immunoreactive batroxobin was quite small in these species, being 0.2-1.9% of the original dose.

Animals↗

Metabolic fate of 125I-labeled batroxobin in rats and dogs.

125I-Labeled batroxobin was prepared and following its intravenous and subcutaneous administrations to rats and dogs, the blood radioactivity was determined. In the both species following the intravenous injections, the decrease in radioactivity was biexponential. Following subcutaneous administration, radioactivity became maximal at 6h and decreased in a manner similar to that of the beta-phase of the intravenous injection. The blood concentration of fibrinogen in dogs was also determined. After the intravenous injection, fibrinogen became undetectable 1h later, and appeared again in the blood at 24h. After the subcutaneous injection, the decrease was not so rapid. Fibrinogen resumed its original levels at 7 day after the administration in both the routes. Radioactivity after the both injections was excreted generally in the urine in about the same amounts. The total urinary and fecal excretions in rats and dogs were 80 and 95%, respectively. The distribution of radioactivity in the tissues was examined by counting technique and whole-body autoradiography. Radioactivity predominantly accumulated in the thyroid and stomach and could also be found in the kidneys and liver in fair amounts. The distribution patterns of radioactivity for both the routes of administrations and also for male and pregnant rats were basically the same. In fetus rats, a slight distribution was noted. From the results of gel filtration chromatography and trichloroacetic acid fractionation, [125I] batroxobin was metabolized soon after the administration to afford low molecular substances such as 125I-ion in the plasma and urine.

Animals↗