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Biomedical subjects

I Matsuda

Publications and source records attributed to I Matsuda.

At least 469 records · Page 26Linked to original sources
PubMed1981

Aarskog syndrome with isolated growth hormone deficiency.

A 5-year-old male with the Aarskog syndrome is described. He had abnormal facies, short stature, short fingers with interdigital webbing, a saddle type scrotum and mild mental retardation. In addition, he had isolated growth hormone deficiency as evidenced by the insulin, arginine, and propranolol-glucagon tests. An arginine test after short-term stimulation with estrogen further supported this diagnosis. His mother had minor abnormalities of the hands and feet, and slight mental retardation.

Child, Preschool↗
PubMed1981

An acute variant of subacute sclerosing panencephalitis: an autopsy case report.

An acute variant of subacute sclerosing panencephalitis (SSPE) was described in a 5-year-old boy who showed rapid progression of coma within 14 days of right hemiplegia. He had measles at 3 years of age. The diagnosis of SSPE was based on the following findings: high anti-measles antibody titer in the serum and in the spinal fluid, periodic complex of EEG, and typical pathological changes of the brain. Treatment with transfer factor failed to improve the worsening clinical course. It is suggested that SSPE should be considered in the differential diagnosis of acute fulminating encephalitides or intracranial vascular lesions.

Acute Disease↗
PubMed1981

Effect of pyridoxal 5-phosphate on the activity of GOT isozyme in plasma from patients with Duchenne muscular dystrophy.

GOT isozyme (soluble GOT, s-GOT and mitochondrial GOT, m-GOT) activities with and without reactivation by the co-enzyme pyridoxal 5-phosphate (PALP) in plasma from 42 patients with Duchenne muscular dystrophy (DMD), and 11 normal controls were investigated. The magnitude of the enzyme activities in patients at the early stage of DMD greatly exceeded those of controls. As the disease progressed, the levels gradually declined, but late stage values were still higher than the control values except s-GOT with PALP. At the mid stage as well as the late stage, the increase in m-GOT activity was approximately to double the unstimulated activity, indicating that, in these patients, as much as half the m-GOT in the circulation was in the inactive apoenzyme form, whereas s-GOT showed negligible increase following PALP addition at any stage.

Adolescent↗
PubMed1981

[Giant neurofibroma of the occipital scalp associated with lambda defect-case report (author's transl)].

Calvarial bone defect associated with a hugh neurofibroma in the region of the lambdoid suture was reported in a 42-year-old man with v. Recklinghausen's neurofibromatosis. An enlarging occipital tumor was first noted at age 4 similar to or approximately 5, and an operation was performed at age 12. Lambda defect was noted at that time, but the operative diagnosis was, apparently, "meningocele". Available literature was reviewed, and the significance of calvarial bone defect in the region of the lambdoid suture as a manifestation of v. Recklinghausen's disease was stressed. Lambda defect witn no sclerosing margin, particularly if associated with hypoplasia of the ipsilateral mastoid cells, has been known to represent the primary nature of the bone defect due to mesodermal dysplasia. In the present case, plain radiographs of the skull revealed abnormal sclerotic change around the bone defect. It is suggested that such an osteoblastic change may well indicate the secondary nature of the bone defect due to the presence of overlying neurogenic tumor.

Adult↗
PubMed1981

Bioavailability of phenobarbital by rectal administration.

Phenobarbital was administered to five patients, eight to 20 years of age, by four different administration routes: Intravenous, intramuscular, oral, and rectal. At each administration, extent of bioavailability, elimination rate constant, absorption rate constant, and volume of distribution of phenobarbital were calculated using a one-compartment open model. The results obtained suggest that rectal administration of phenobarbital is more reliable than intramuscular or oral administration of the drug for rapid treatment in children.

Adolescent↗
PubMed1980

Agenesis of an internal carotid artery: angiographic, tomographic and computed tomographic correlation.

Congenital absence of one internal carotid artery was found by angiography in a woman of 52 years who had subarachnoid hemorrhage. The absence of the bony carotid canal on the affected side substantiated the congenital nature of this rare vascular anomaly. We believe this to be the first report of absence of the bony carotid canal and the intracavernous portion of the internal carotid artery confirmed by computed tomography and cavernous sinography.

Carotid Artery, Internal↗
PubMed1980

Ornithine transcarbamylase (OTC) in white blood cells.

A radiochemical assay method of ornithine transcarbamylase (OTC) was developed using labeled carbamyl phosphate as a substrate. The enzyme activities determined by this method in peripheral white blood cells from ten normal subjects were 1.32 +/- 0.95 nmoles/mg/hr and the apparent Km's, when assayed at pH 8.5, were 6.4 mM for ornithine and 0.6 mM for carbamyl phosphate. On the contrary, the apparent Km's of human liver OTC were 0.6 mM for ornithine and 0.12 mM for carbamyl phosphate. The average OTC activity of granulocytes was 1.0 nmoles/mg/hr, whereas that of mononuclear cells was 0.4 mmoles/mg/hr. Lymphoid cell lines were established from three normal subjects and an OTC-deficient infant. All these cell lines demonstrated no OTC activity. When arginine was removed from the medium and replaced by ornithine, the lymphoid cells were unable to grow in culture. On autoradiography, the lymphoid cells showed labelling at incubation in the presence of 14C-citrulline, but not with 14C-ornithine.

Amino Acid Metabolism, Inborn Errors↗
PubMed1980

A new variant of Lowe oculocerebrorenal syndrome.

A case is reported of a 5-year-old boy with Lowe syndrome. The patient was unusual in that he had only mild and transient acidosis with no rickets. The corneal opacities in the parents suggest that this disease might be autosomal recessive.

Bicarbonates↗
PubMed1980

Serum vitamin E concentration in patients with severe multiple handicaps treated with anticonvulsants.

Serum vitamin E concentrations were measured in 47 severely handicapped patients, aged from 4 to 23 years, and in 22 controls. Thirty-three of the handicapped patients with seizures were treated with phenytoin and phenobarbital; the remaining 14 patients were not treated. The serum vitamin E levels were lower in the handicapped than in controls. Among the handicapped, those treated with anticonvulsants showed much lower levels of serum vitamin E than those untreated. Ten patients under anticonvulsant therapy were selected to receive d-1-alpha tocopherol acetate, 100 mg/day, based on their low serum vitamin E levels (range of 0.27 to 0.61 mg/100 ml). After one month of tocopherol treatment, both their serum vitamin E levels and hemolysis tests returned to normal. During a three-month tocopherol treatment period, both the frequencies of seizure attacks and the electroencephalogram (EEG) patterns remained unchanged. Supplementation with vitamin E is recommended in some patients under anticonvulsant therapy.

Adolescent↗
PubMed1980

[Multiple cerebral tuberculomata involving brainstem and cerebellum--report of a case (author's transl)].

A case of multiple cerebral tuberculomata involving the pons and cerebellum was presented. The lesions were demonstrated by CT as isodense to slightly dense foci. All four intra-axial lesions showed homogeneous enhancement following an intravenous injection of the contrast medium, and one of them was surrounded by a small area of low density, probably representing the perifocal edema. The patient responded well to chemotherapy with streptomycin, hydrazid and rifampicin : cranial nerve signs and long tract signs clearing rapidly and the enhancing lesions and mass effect on CT disappearing concomitantly. Although cerebral tuberculoma is nowadays very rare in Japan, still a high index of suspicion should always be entertained during the investigation of patients showing solitary or multiple enhancing lesions with no or slight degree of perifocal edema on CT, and a trial of antituberculous drugs should be given before the incurable malignancy is presumed or the lesion is explored surgically.

Brain Diseases↗