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Biomedical subjects

Y Ohtani

Publications and source records attributed to Y Ohtani.

At least 181 records · Page 10Linked to original sources
PubMed1983

Childhood-type myositis and linear scleroderma.

A 5-year-old girl had linear scleroderma on the flexor surface of the right arm; muscle wasting included the shoulder girdle. IgM fluorescence on blood vessels and along dermal-epidermal junction was observed by direct immunofluorescence in biopsied skin. Biceps muscle underlying the plaque of the scleroderma showed atrophy of entire fascicles, perifascicular atrophy, and cellular infiltration around blood vessels that are quite similar to those found in childhood-type dermatomyositis. In addition, various abnormalities, including edema and thickening of basal lamina, were found on blood vessels in muscle tissue. The results suggested that the autoimmune collagen vascular disorder is responsible for this condition.

Autoimmune Diseases↗
PubMed1983

Mitral regurgitation associated with aortitis syndrome.

Three patients with mitral regurgitation (MR) associated with aortitis syndrome are presented. All had multiple lesions of the large sized arteries, calcification of the aorta, mild inflammatory findings, a chronic course, and congestive heart failure. MR was observed by ventriculography in all 3 patients. Case 1 had mitral valve prolapse and secondary systemic hypertension. Case 2 showed mildly thickened mitral valve leaflets and had moderate aortic regurgitation (AR). Case 3 had massive AR. The grade of MR was moderate in Cases 1 and 2, and massive in Case 3. The left ventricle was moderately dilated in Cases 1 and 2 but contracted sufficiently and symmetrically in all 3 patients. Other than the prolapse, no significant mitral valve deformity or left ventricular asynergy was evident by ventriculography. The incidence of MR was 3.1% of 128 patients with aortitis syndrome observed in our clinic. MR may be found in the late stage of aortitis syndrome. It may be caused by a mild valvular lesion related to aortitis syndrome and be exacerbated by increased hemodynamic loads such as those which occur in secondary hypertension and AR.

Aortic Arch Syndromes↗
PubMed1982

Carnitine deficiency and hyperammonemia associated with valproic acid therapy.

Plasma carnitine and blood ammonia concentrations were measured in 25 severely handicapped patients, ages 3 to 21 years, and 27 age-matched control subjects. Fourteen of the handicapped patients were treated with anticonvulsant drugs including valproic acid; the remaining 11 patients were treated with drugs excluding valproic acid. Plasma carnitine concentrations were lower and blood ammonia values were higher in patients treated with valproic acid than in the untreated patients and control subjects. A significant inverse relationship was found between plasma carnitine concentrations and the dosage of valproic acid, and between plasma carnitine and blood ammonia values. After oral administration of D,L-carnitine (50 mg/kg/day) for four weeks, both carnitine deficiency and hyperammonemia were corrected.

Adolescent↗
PubMed1982

Xanthine oxidase inhibitor in Duchenne muscular dystrophy.

The treatment with xanthine oxidase inhibitor, allopurinol, was evaluated in 17 patients with Duchenne muscular dystrophy (aged 2 years 9 months to 13 years 9 months) using the double blind technique. The total observed period was 27 months. The results of 100-point scale of graded functional abilities revealed that an improvement, unchange and progression of dysfunction were found in 2, 2 and 6 patients, respectively, in allopurinol group and in 0, 2 and 5 patients, respectively in placebo group. The patients' age and stage of the disease seemed to be related to the effectiveness or allopurinol treatment.

Adenosine Triphosphate↗
PubMed1982

Infantile glycogen storage myopathy in a girl with phosphorylase kinase deficiency.

A 19-month-old girl with moderate hypotonia was studied. Histochemical and electronmicroscopic findings revealed that many skeletal muscle fibers contained an excess amount of glycogen. The phosphorylase reaction was normalized only after activation with 5' AMP. Biochemical studies showed an increased glycogen content and decreased activities of phosphorylase "a" and an active form of phosphorylase kinase, whereas activities of total phosphorylase, total phosphorylase kinase, and cyclic AMP-dependent protein kinase were all in the normal range. Thus, phosphorylase kinase in the patient's muscle seemed to be a variant form, which was activated partially under the physiologic condition. This condition may be inherited as an X-linked recessive trait.

Acid Phosphatase↗
PubMed1982

Cyclodextrin-induced hemolysis and shape changes of human erythrocytes in vitro.

Cyclodextrins (CyDs) at higher concentrations were found to cause hemolysis of human erythrocytes in the order of beta- greater than alpha- greater than gamma-CyD in isotonic solution. Biphasic effects of CyDs were observed for the osmotic and heat-induced hemolysis; i.e. the protection at relatively low CyD concentrations and stimulation at higher CyD concentrations. From the scanning electron microscopic observations, CyDs induced shape changes of membrane internalization type on erythrocytes. CyDs caused the release of cholesterol from erythrocyte membrane in the order of beta- greater than gamma- greater than alpha-CyD. These results clearly indicate that CyD-induced hemolysis is probably a secondary event resulting from the membrane disruption which elicited the removal of membrane components from erythrocytes.

Cyclodextrins↗
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↗
PubMed1979

Neutral lipid and sphingolipid composition of the brain of a patient with membranous lipodystrophy.

Lipids were extracted from brains of a patient with membranous lipodystrophy (ML) and three normal patients and the neutral lipid and sphingolipid constituents were investigated. The storage of a large amount of free fatty acid was observed in the ML brain, but no cholesterol ester was found. Total lipid, cholesterol and cerebroside contents were slightly decreased in the white matter of the ML brain. The composition of free and sphingolipid fatty acids did not differ between ML and normal brains.

Adult↗
PubMed1979

Creutzfeldt-Jakob disease--alteration in ganglioside sphingosine in the brain of a patient.

Gangliosides isolated from the brain of patients with Creutzfelt-Jakob (C-J) disease were analyzed. The ganglioside current was abnormally low, and the percentage distributions of individual gangliosides and the long-chain base compositions were abnormal. The C20-sphingosine contents of all the ganglioside fractions were low. Abnormalities in ganglioside long-chain bases in adult human brain have been reported previously only in patients with inherited metabolic disorders. These abnormalities in C-J disease seem to be closely related to the cause of the disease.

Brain Chemistry↗