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Biomedical subjects

Y Fukuyama

Publications and source records attributed to Y Fukuyama.

At least 163 records · Page 9Linked to original sources
PubMed1989

[Dramatic effects of high-dose intravenous gammaglobulin in each patient with intractable dermatomyositis and polymyositis].

A high-dose intravenous gamma-globulin (IVGG) therapy was given to two children; one with intractable dermatomyositis (case 1) and the other with polymyositis (case 2). Case 1, a 3-year-6-month-old girl, only poorly responded to the standard oral prednisolone therapy and a pulse therapy with high-dose methyl-prednisolone. This patient showed a complete remission after a course IVGG therapy, 100 mg/kg/day iv. for 5 days, which has been maintained for 6 months up to the present. Case 2, a 13-year-6-month-old girl, also showed little improvement after the oral prednisolone therapy, and transient improvement during plasma exchange therapy. This patient showed fairly good response after three repeated courses of IVGG therapy, but not complete remission.

Adolescent↗
PubMed1989

[Genetic studies of Duchenne muscular dystrophy families using the dystrophy families using the dystrophin cDNA].

The cDNA probes for the Duchenne muscular dystrophy (DMD) gene can detect deletions in over 50% of affected males and provide a highly accurate diagnostic test in the affected families. We present the results of our recent molecular genetic studies of five DMD families (six affected males and 16 non-affected family members) by using the dystrophin cDNA. Five molecular deletions were identified in the DNA samples of six affected males by studying abnormalities of Hind III and Bgl II fragments detected by the entire dystrophin cDNA. We could not find any abnormality in a family by the same DNA analysis. Deletion was found at two different loci; in two of six probands, it was located near 5' region of the gene seen by cDNA segment 1-2 a, while in the other three, near the center of the gene covered by cDNA segment 8. In three females of the five families, we detected novel fragments, the size of which was altered due to molecular deletion. These results greatly facilitated diagnosis of the carrier status in females. We studied also the dystrophin location in the biopsied skeletal muscle (femoral muscle) in a family, by means of indirect immunofluorescence staining using antiserum against dystrophin. Dystrophin was positive in the surface membrane of muscles from a non-affected brother, mother and sister, but it was absent in the muscle from a DMD proband.

DNA Probes↗
PubMed1989

[Case-control study of moyamoya disease].

The cause of Moyamoya disease is still unknown. We made a research about the factors closely related to Moyamoya disease through a case-control study. The number of cases studied was 66. Controls were selected from among patients' friends, matched as to sex, age and residential area. Questionnaires were sent to the cases and the respective controls by mail. The questions were about past history, developmental history, school records, habitual factors, dietary habits and pets. The first symptoms and age at onset were also surveyed in the cases. The response rate was 84.8% (56 cases) of the cases and 76.5% (101 cases) out of the 132 controls. There was no significant difference in the prevalence of tonsillitis, conjunctivitis, otitis media, and bronchitis. Odds ratio of fever of unknown origin is 2.793 and X2 is 7.213. Diseases whose odds ratio was over 1 were herpes, appendicitis, bronchitis, asthma, anemia, dental caries, head injury and drug allergy. But all of them were not significantly prevalent. Odds ratios of school records were 4-9 and X2 were 4-17 from elementary to junior high school. Odds ratio of western dishes was 2.709 and X2 was 5.189. There was no significant difference as to pets kept. We could not find overt relationships between Moyamoya disease and diseases of head and neck like tonsillitis.

Adolescent↗
PubMed1988

Ataxia-ocular motor apraxia: a syndrome mimicking ataxia-telangiectasia.

We report 14 patients with a slowly progressive syndrome featuring ataxia, choreoathetosis, and ocular motor apraxia in both the horizontal and vertical planes. Although the neurological signs were indistinguishable from those of ataxia-telangiectasia, the onset tended to be later and none of the patients had evidence of multisystemic involvement. Specifically, there was no tendency to frequent infections, and immunoglobulins, alpha-fetoprotein, T- and B-lymphocyte markers, and chromosomes 7 and 14 were normal in all tested patients. The simultaneous absence of telangiectasias and of other nonneurological manifestations made ataxia-telangiectasia an unlikely diagnosis. We suggest that these patients suffer from an unusual type of spinocerebellar degeneration. This syndrome has been observed in different populations from three continents, with a genetic pattern suggesting recessive autosomal inheritance.

Adolescent↗
PubMed1988

Phase 1 study of a new antipsychotic drug, OPC-4392.

1. A phase I study of OPC-4392 (OPC), a quinolinone derivative recently developed in Japan and recognized to have an agonistic effect on dopamine autoreceptors, was performed in 7 male healthy volunteers in comparison with chlorpromazine (CPZ). 2. Clinical pharmacology The main clinical symptoms of OPC were sleepiness, weakness, fatigability, heavy headedness, disturbance of concentration, nausea, etc. The severity of these symptoms increased dose-dependently, and the upper limit dosage of OPC was considered to be 5 mg for the healthy volunteers. 3. Endocrinological research The serum prolactin level decreased dose-dependently in the OPC group, whereas it rose in the CPZ group. A significant negative correlation was recognized between the OPC-plasma level and serum prolactin level as well. 4. Psychological tests In the Kraepelin test, a decrease in the average work quantity was observed in both groups, but it was less in the OPC group. 5. Pharmacokinetic study From the pharmacokinetic parameters measured, two features were recognized: one was the slowness of Tmax (4-6 hours) and the other was the length of its biological half-life (56-88 hours). It was estimated that the plasma level of OPC-4392 would take 2 weeks to reach a steady state.

Adult↗
PubMed1987

Clinical effects of MND-19 (Inosiplex) on subacute sclerosing panencephalitis--a multi-institutional collaborative study--The Inosiplex-SSPE Research Committee.

A total of 151 cases of subacute sclerosing panencephalitis (SSPE), comprising 89 cases treated with MND-19 (Inosiplex) and 62 untreated cases, were retrospectively investigated as to background characteristics, survival rate and clinical course in order to compare the findings in the 2 groups of cases. The survival rate for the cases treated with MND-19 (MND-19-treated group) was significantly higher than that for the untreated cases (control group), which was also true on stratified analysis or on smoothing of the background factors by means of Cox's multiple regression model. Investigation of the clinical course revealed that progression through the disease stages was significantly slow in the MND-19-treated group, compared with in the control group. Global rating of the clinical course showed that a prolonged remission was obtained in more MND-19-treated cases than control cases. The measles virus antibody titer was in no way affected in the former group. Side effects of MND-19 were observed in 17 of the 89 treated cases (19.1%).

Adolescent↗
PubMed1987

A case of de novo interstitial deletion 3q.

A rare chromosome abnormality consisting of interstitial deletion 3q was found in a malformed girl. Chromosome analysis using G and Q banding showed deletion of bands 3q12----3q21: 46,XX,del(3)(pter----q12::q21----qter). The clinical features of the proband included severe psychomotor retardation, craniofacial asymmetry, hypertelorism, epicanthus, high arched palate, progressive scoliosis, multiple skin pigmentations, and renal abnormalities. The parents had normal karyotypes.

Abnormalities, Multiple↗
PubMed1986

Administration of ACTH to suckling rats results in hyperkinetic behavior.

The present study on ACTH-administered suckling rats was undertaken to monitor the late behavioral sequelae due to the effects of this drug. The locomotor activity of rats treated with ACTH in the suckling period increased significantly as to the gross size of movements when compared with control rats. The increase in locomotor activity was correlated with the suppression of CNPase activity. These results show that administration of ACTH during the suckling period has suppressive effects on the development of the brain and behavior, and that these effects persist for a longer period after termination of the administration of ACTH than the direct effect of the peptide on endocrine functions.

Adrenocorticotropic Hormone↗
PubMed1986

Suppression of 2', 3'-cyclic nucleotide 3'-phosphohydrolase activity in suckling rat brain by ACTH.

Administration of ACTH at two different doses (0.05 and 0.5 microgram/g/day) to suckling rats resulted in the suppression of both the body and brain weight gains and the developmental increase in brain CNPase activity, and the suppression of the brain CNPase activity persisted for 3 weeks (up to the end of the experiment) after the cessation of ACTH administration in the suckling period, while the suppression of the body and brain weight gains was noticed only during the administration period. The authors emphasized the possibility that long-term therapy with massive doses of ACTH for infantile spasms may be hazardous to the developing brain in many ways.

Adrenocorticotropic Hormone↗
PubMed1986

High incidence of fundus hamartomas and clinical significance of a fundus score in tuberous sclerosis.

A detailed ophthalmological study was performed on a consecutive series of 100 cases with tuberous sclerosis (TS). A fundus score was given to each patient according to the size and number of fundus hamartomas to correlate them with other clinical symptoms. TS-Specific fundus hamartomas were detected in 87% of the subjects. There were no significant differences in the distribution of the scores with age, and considerably high scores were given even for younger infants and children. The fundus scores were higher in sporadic cases or the complete type than in heredofamilial cases or the incomplete type. On fluorescein fundus angiography in 25 patients with 72 hamartomas, various degrees of hyperfluorescence were found in 87% of the lesions, while no leakage was found in the rest. Other ocular findings included depigmented iris sectors in 12 cases, punched-out chorioretinal defects in 9, and unilateral visual impairment due to either primary or secondary effects of the hamartomas in 3.

Adolescent↗
PubMed1986

Cornelia de Lange syndrome associated with a suprasellar germinoma.

A case of Cornelia de Lange syndrome complicated by a suprasellar germinoma is presented. The patient was an 18 years old girl with severe mental retardation and characteristic facial and other malformed features. She was admitted because of polyuria and polydipsia. Endocrine investigation showed defects of hypothalamic-pituitary function. The most striking finding on postmortem examination was the existence of a suprasellar germinoma extending to the optic tract and pituitary lobes. We discussed a diagnostic problem caused by the coexistence of these two uncommon entities.

Adolescent↗