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Biomedical subjects

K Tada

Publications and source records attributed to K Tada.

At least 523 records · Page 29Linked to original sources

Childhood epilepsy uncontrolled by phenytoin: clinical and electroencephalographic study.

Nine cases of childhood epilepsy manifesting motor convulsions uncontrolled despite high levels of phenytoin (PHT) were studied clinically and electroencephalographically. These cases consisted of five cases of partial seizures without impairment of consciousness, two cases of partial seizures (occasionally generalized seizures beginning locally), one case of predominantly unilateral seizures, and one case of generalized tonic-clonic seizures. the onset of seizures was at a rather early age, between 3 months and 9 years, and under 3 years of age in eight cases. All cases had single or multiple, cortical epileptic foci in EEG. The projection of spikes was localized to a rather limited area. Seizures of these patients were frequent. All cases, except one, did not respond to other medication. Convulsive seizures with cortical focal spike foci in EEG uncontrolled despite high levels of PHT were thought to have poor responsiveness to not only PHT itself, but also to other anticonvulsants.

Adolescent↗

Effect of adenosine deaminase replacement therapy on a child of adenosine deaminase deficiency with severe combined immunodeficiency disease.

Enzyme replacement therapy was performed for a 1-year and 5-month old boy with adenosine deaminase deficiency disease, the first case in Japan. Irradiated fresh red blood cells were administered without any clinical improvement, but there was an increase in the peripheral lymphocytes from 300/mm3 to 1849/mm3, of which 88% had T cell marker. B lymphocytes did not bear any classes of surface immunoglobulins. The proliferative responses of these lymphocytes to phytohemagglutinin, concanavalin A, pokeweed mitogen and allogeneic cells were examined. More than two-fold increase in response to these mitogens was observed in lymphocytes after treatment as compared with responsiveness before treatment, but these responses still remained to a much lesser degree than that of lymphocytes from controls.

Adenosine Deaminase↗

A lymphoblastoid cell line from an adenosine deaminase deficient patient established by Epstein-Barr virus.

Lymphoblastoid cell lines from a patient with adenosine deaminase (ADA) deficiency and his parents were established by Epstein-Barr virus (EBV). ADA activity in the lymphoblastoid cells from the patient was not detectable, while the enzyme activity in the cells from his parents was approximately a half level of the controls'. No inhibitor to ADA could be detected in the lymphoblastoid cells from the patient.

Adenosine Deaminase↗

[The topographical localization of spinal motoneurons of the rat and its numerical alternation in regard to development (author's transl)].

The topographical localization of spinal motoneurons innervating the forelimb muscles of the rat and its numerical alternation in regard to development was observed by HRP (horseradish peroxidase) method. HRP method is a histochemical technique to trace the cellular origin of axon terminal net work. When HRP is injected into the muscles, the enzyme is taken up by nerve endings and transported retrogradely by the axoplamic flow to the original cell body, and does not transported hematogenously and does not cross the synapse. The spinal motoneurons were found in spindle-shape longitudinal cell columns according to its innervation. The motoneurons supplying the biceps brachii muscle were located in the dorso-lateral nucleus on the anterior horn of the 4th cervical segment, and extended downward to the ventral nucleus in 8th cervical segment, forming a spindle-shape cell column. The thick spindle-shape cell column consisting of cells which innervated the triceps brachii muscle lied in the ventro-medial nucleus of the 6th, 7th and 8th cervical segment. The neurons innervating the flexor carpi ulnaris muscle were located in 7th and 8th cervical segment, but few in number compared to those of the biceps and triceps. The motoneurons innervating the biceps brachii muscle decreased in number with increasing age according to exponential curve. The total number of HRP-labeled neurons in two week old rat ranged from 2,932 to 2,017 and markedly decreased during first eight weeks, in eight week old rat ranged from 1,637 to 603. After this, they gradually decreased to 312 in 50 weeks on an average, where the number corresponded to about 13% of those of two week old rat. The data indicate that ventral horn has a great deal of excess neurons which has a distinct neuromuscular connection as suggested by Prestige, in the developmental stage of Xenopus laevis. In young rats (ten days to two weeks of age), HRP-labeled neurons were also present in the ventral horn on the non-injected side. These large and multigonal contralateral HRP-labeled neurons constituted a characteristic feature of spinal motoneurons, and distributed through the same segment as those on injected side. The total number of HRP-labeled neurons within non-injected side ranged from 563 to 197 and corresponded from one-fifth to one-tenth of that in the injected side. In five weeks or elder rat, no HRP-labeled neurons could be observed in non-injected side. This indicates that in immature rats the muscles are innervated bilaterally by the spinal nerve, since HRP is transported by retrograde axoplasmic flow and does not cross the synapse, and the bilateral motor innervation has disappeared with maturation. The bilateral motor innervation is therefore, presumed to be an immature mode of innervation limited to the early stage of life. Although bilateral motor innervation following the nerve injury in the limb at the early stage of life has been confirmed by ourselves, the significance of the immature mode of innervation is still hard to explain.

Aging↗

Congenital lactic acidosis due to pyruvate carboxylase deficiency: absence of an inhibitor of TPP-ATP phosphoryl transferase.

Two children are described who suffered from episodes of metabolic acidosis and progressive mental and motor deterioration. The patients showed periodic elevation of blood lactate, pyruvate and alanine, which was accompanied by vomiting, hypotonia or convulsions. The concentrations of lactate and pyruvate in cerebrospinal fluid were found to be increased. Liver biopsies revealed a decrease in pyruvate carboxylase activity and normal pyruvate decarboxylase activity. No inhibitor of TPP-ATP phosphoryl transferase was detected in urine from the patients. These findings suggest that congenital lactic acidosis due to pyruvate carboxylase deficiency is probably a different disease entity from Leigh's encephalomyelopathy. A possible mechanism of brain damage caused by a defect in pyruvate carboxylase is postulated.

Acidosis↗

Three-dimensional architecture of blood vessels of tendons demonstrated by corrosion casts.

Three-dimensional observation of the microcirculation of the tendon was readily and clearly demonstrated by preparing methyl methacrylate casts and observing them under the scanning electron microscope. In the muscles blood vessels made a network like a ladder surrounding every muscle fibre. The fibrous digital sheath had blood vessels made in a fine meshed cylinder. By microdissection of the vessels of the sheath the blood vessels of the vinculae and tendons were observed stereoscopically in relation to the peritendinous tissues. The casting method contributes to better understanding of vascular architecture of tendons.

Animals↗

Prenatal diagnosis and fetal pathology of Niemann-Pick disease.

Prenatal diagnosis was successfully accomplished by determining sphingomyelinase activity in the cultured amniotic fluid cells in a case of high risk pregnancy for Niemann-Pick disease (NPD), type A. No detectable activity of sphingomyelinase was found in the cultured amniotic fluid cells obtained at the 17th week of gestation. Patient's pregnancy was terminated and the aborted fetus was proved to be affected with NPD. The liver, brain and skin fibroblasts from the aborted fetus revealed a markedly diminished activity of sphingomyelinase. Sphingomyelin content in the liver of the affected fetus was found to be increased approximately sevenfold as compared with that in a control fetus liver. On the other hand, there was no increase of sphingomyelin in the brain from the affected fetus. No significant increase in cholesterol content was found in the liver and brain from the affected fetus. Electron-microscopic findings revealed membranous cytoplasmic bodies and electron dense material with vacuoles in cytoplasm of the liver cell and a number of Zebra body-like inclusions in the cerebral vessel wall. Biochemical and histological findings of the NPD fetus indicate that there is the progress of the disorder already in the midtrimester of gestation.

Amniotic Fluid↗

[Hyperargininemia].

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Amino Acid Metabolism, Inborn Errors↗