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Biomedical subjects

R Koide

Publications and source records attributed to R Koide.

At least 19 recordsLinked to original sources
PubMed1995

Dentatorubral-pallidoluysian atrophy (DRPLA): close correlation of CAG repeat expansions with the wide spectrum of clinical presentations and prominent anticipation.

Dentatorubral-pallidoluysian atrophy (DRPLA) is a rare autosomal dominant neurodegenerative disease characterized by various combinations of ataxia, choreoathetosis, myoclonus, epilepsy and dementia as well as various ages of onset. We have identified a specific unstable trinucleotide repeat expansion in a gene on the short arm of chromosome 12 as the pathogenic mutation for DRPLA. We investigated how the degree of the expansion of the CAG repeat affects the clinical manifestations of DRPLA. The sizes of the expanded alleles were well correlated with the ages of onset (r = -0.6955, P < 0.001). Patients with progressive myoclonus epilepsy (PME) phenotype had larger expansions (62-79 repeats) and earlier ages of onset (onset before age 20). Furthermore, most of the patients with PME phenotype inherited their expanded alleles from their affected fathers. On the other hand, patients with non-PME phenotype showed later ages of onset (onset after age 20) and smaller expansions (54-67 repeats). When ages of onset of each clinical symptoms are compared with sizes of the CAG repeat, there is again a remarkably high correlation of the sizes of CAG repeat with each of the clinical symptoms. Thus the wide variation in clinical manifestations of DRPLA can now be clearly explained based on the degree of CAG repeat expansion, which strongly indicates that the expanded alleles are intimately involved in the neuronal degeneration in dentatofugal and pallidofugal systems.

Adult↗
PubMed1995

[Central ocular hypotensive effect of noradrenaline].

We investigated the effects of topical and central administration of noradrenaline (NA) on the intraocular pressure (IOP) in pigmented rabbits. Unilateral instillation of NA (200 micrograms) produced no significant change in IOP. On the other hand, intracerebroventricular (ICV) administration of NA (0.01-1 micrograms) decreased IOP in both eyes in a dose-related fashion. The ocular hypotensive effect of NA was diminished by sympathectomy of the superior cervical sympathetic nerve or by ICV pretreatment with yohimbine hydrochloride 1-10 micrograms). These results suggest that the hypotensive effects of contrally administered NA might be mediated by alpha 2-adrenoceptor stimulation in the central nervous system.

Administration, Topical↗
PubMed1995

[An intraocular lens power calculation for high myopia].

The accuracy of intraocular lens power calculation formulas for the axial high myopia were examined, especially regarding the point of the predictive refraction. We examined 170 eyes with axial lengths of 27 mm or longer, with postoperative visual acuity of 0.5 or more, and postoperative astigmatism of less than +/- 2D. Five formulas were tested for accuracy in predicting postoperative refraction. They were the L-SRK, SRK, SRK II, SRK/T, and Binkhorst formulas. The SRK formula tended to predict less myopia than the actual postoperative refraction. The SRK II and Binkhorst formulas predicted more myopia than the actual postoperative refraction. The best results were produced by the L-SRK and SRK/T formulas. The accuracy of the L-SRK formula predictions were measured for each of the four myopic levels. The same was done for the SRK/T formula. For both formulas, there was no statistically significant difference in accuracy of prediction for the four myopic categories. The two formulas are considered to be useful for high myopic cases.

Humans↗
PubMed1995

Dentatorubral-pallidoluysian atrophy (DRPLA). Molecular basis for wide clinical features of DRPLA.

Dentatorubral-pallidoluysian atrophy (DRPLA) is a rare autosomal dominant neurodegenerative disorder characterized clinically by various combinations of myoclonus, epilepsy, cerebellar ataxia, choreoathetosis, dementia and psychiatric symptoms. Based on the phenomenon of anticipation, the gene for DRPLA was recently identified. DRPLA is caused by unstable expansion of a CAG repeat in the gene located on the short arm of chromosome 12. As have been observed in Huntington's disease and SCA1, there is a strong correlation between the age of onset and the size of CAG repeats. Furthermore, patients with larger repeats tend to show a PME (progressive myoclonus epilepsy) phenotype as well as earlier ages of onset. More prominent anticipation and larger intergenerational increase of CAG repeats in paternal transmission can be accounted for by the meiotic instability of CAG repeats in male gametogenesis. Comparison of size distributions of CAG repeats in Japanese, African-American and white populations revealed that 7.4% of the Japanese alleles had greater than 19 repeats, whereas none of the whites and 1% of the African-American alleles were of this size. The results may account for the ethnic predilection of DRPLA.

Adult↗
PubMed1992

[A study on HTP organic signs].

A special version of the House-Tree-Person (H-T-P) technique was developed to differentiate organic psychosis from other disorders. H-T-P data of 126 psychiatric inpatients, including 16 patients with organic psychoses, 50 schizophrenics, 50 borderlines and 10 patients with other psychoses, were evaluated for the presence or absence of 29 possible features of houses, trees and persons. These features included those common to all three objects, as well as those specific to each object. Results for patients with organic psychoses were compared with those of patients with other disorders. The comparison disclosed that seven of the original 29 H-T-P features had diagnostical discrimination among treatment groups, giving higher H-T-P organic scores.

Diagnosis, Differential↗
PubMed1992

[Ocular hypotensive effect of alpha-adrenoceptor agonist and antagonist in the conscious pigmented rabbit].

It has been reported that some of the topically-used antiglaucomatics have a central ocular hypotensive effect. In this study, the influence of topical and intracerebroventricular (i.c.v.) administration of phenylephrine, clonidine, guanfacine, prazosin, yohimbine on the intraocular pressure (IOP) was investigated in the rabbit. Male pigmented rabbits were used throughout the experiments. For measurement of IOP, an applanation pneumatonograph was used. By unilateral topical administration of phenylephrine, an increase in IOP in the eye in which instillation was performed was observed. On the other hand, a slight decrease in IOP was observed by similar treatment of prazosin and yohimbine. No significant change of IOP in the contralateral eye was observed with these drugs. On the contrary, unilateral topical administration of clonidine or guanfacine decreased the IOP of both eyes. Furthermore, the decrease of IOP was more remarkable in the contralateral eye compared to the eye which received instillation. The IOP of both eyes was decreased in a dose-related fashion by i.c.v. administration of clonidine or guanfacine. The ocular hypotensive effects of clonidine were diminished by the pretreatment by i.c.v. administration with yohimbine. These results suggest that the ocular hypotensive effect of clonidine and guanfacine is due to their alpha 2-adrenoceptor stimulation in the central nervous system.

Administration, Topical↗
PubMed1992

[The role of sympathetic cervical ganglion in the effect of clonidine for lowering intraocular pressure].

The contribution of sympathetic cervical ganglion to the mechanism of action of clonidine for lowering intraocular pressure (IOP) was investigated. Pigmented rabbits were used. The animals were divided into 3 groups: normal control group (group 1), animals in which the bilateral cervical sympathetic trunks had been amputated presynaptically (group 2), animals in which the superior cervical ganglion (SCG) had been bilaterally dissected (group 3). Changes in IOP were measured after topical application of clonidine unilaterally. In group 1, IOP was significantly decreased in eye treated by clonidine and contralateral eyes compared to pretreatment values. Decrease of IOP in the contralateral eye was greater than that in the treated eye. In group 2, IOP was decreased in the contralateral eye but increased in the eye treated by clonidine. Pretreatment of yohimbine administered orally antagonized the ocular hypotensive effect of clonidine in a dose-related manner. In group 3, where SCGs were dissected, no changes in IOP were observed in both eyes by unilateral administration of clonidine. These results suggested that the bilateral ocular hypotensive effect of clonidine administered unilaterally is in part due to direct action on the SCG and that the alpha 2 receptor in the SCG plays some role in the regulation of IOP.

Animals↗
PubMed1991

Immunoelectron microscopic observation of cells in the rat retinal containing gamma-aminobutyric acid and catecholamine.

Interactions between gamma-aminobutyric acid (GABA)- and catecholamine (CA)-containing cells in the rat retina was revealed by a double-labeling immunocytochemical technique using the antisera to GABA- and CA-synthesizing enzymes, such as tyrosine hydroxylase (TH) and phenylethanolamine-N-methyltransferase (PNMT). At the light microscopic level, GABA-, TH- and PMNT-immunoreactive (GABA-, TH- and PMNT-IR) amacrine cell bodies and their processes appeared in the inner nuclear layer and the inner plexiform layer, respectively. By electron microscopy observation, in the inner plexiform layer, GABA-, TH- or PMNT-IR amacrine cell processes were found making synaptic contacts with the axon terminals of immunonegative bipolar cells or with the processes of immunonegative amacrine cells. TH-IR amacrine cell processes formed synapse-like contacts with the GABA-IR amacrine cell perikarya and processes. In contrast, GABA-IR amacrine cell processes formed symmetric synaptic contacts onto the TH-IR as well as PNMT-IR amacrine cell processes. From these findings, it appears that the GABA- and CA-containing amacrine cells may interact with each other and play some important role in regulating the activities of bipolar cells and other unknown amacrine cells in the rat retina.

Animals↗
PubMed1990

[Estimating the quality of life in childhood cancer survivors from parent questionnaires].

Multidisciplinary clinical research on childhood cancer has achieved a marked improvement in survival rate during the last two decades. Due to the increase of long-term survivors, the survey of quality of life (QOL) of cured children is essential. But methods to estimate QOL of children have not been established. We attempted to estimate the QOL of surviving patients from the responses to a questionnaire filled out by their parents. The total number of patients treated at the National Children's Hospital from 1965-1987 was 937, of which 296 patients are still surviving. Among these survivors 108 patients were selected to be surveyed because they were being followed up by our attending physicians. The originally elaborated questionnaire was sent to parents in June 1989. Seventy-eight parents responded. The questionnaire consisted of four major questions. The first was how the QOL of the child compared to that of an ordinary child. The parents circled the appropriate level of QOL determined by our scale (1 lower, 4 the same as ordinary child, 7 excellent). The average score was 5.61 showing that parents felt their child's QOL is superior to an ordinary child. The second question concerned how the present status of the child was influenced by the experience of cancer. Answers were divided into five categories as follows: 1) Good or positive (10%); 2) nothing in particular (49%); minimum (29%); negative (9%); and other (7%). The third question concerned any anxieties. Most parents had numerous anxieties about the relapse, late effects, education, job prospects, marriage and offspring and getting the latest information. The fourth question dealt with what to tell the child as to their true diagnosis. Answers were divided as follows: absolutely no (56%); yes, but in the future (23%); yes, already done (10%); and yes, through our physician; (13%). After studying the questionnaires we interviewed the parents. We noticed that the parent/child relationship was very close. A meeting was conducted with parents and all the staff (doctors, psychologists and nurses). In conclusion, the QOL in surviving childhood cancer patients is considered to be better than that of ordinary children by their parents. This result is likely due to the excellent parent/child relationship.

Adolescent↗
PubMed1988

Improvement in the treatment of childhood cancer: analysis of survival data from the National Children's Hospital (1965-1987).

Developments in the treatment of childhood cancer have been evaluated in patients who had been treated in the National Children's Hospital from 1965 to 1987. The total number of patients was 867, of which leukemia accounted for 376, malignant lymphoma 61, neuroblastoma 174, Wilms' tumor 55, yolk sac tumor 29, rhabdomyosarcoma 36 and hepatoblastoma 30. Patients were divided into three time intervals: the 1960s, 1970s and 1980s. A marked improvement in five-year survival was recognized in Wilms' tumor and yolk sac tumor, amounting to 80%, followed by rhabdomyosarcoma, acute lymphoblastic leukemia and malignant lymphoma. There was no improvement in patients with acute non-lymphoblastic leukemia, neuroblastoma and hepatoblastoma. Prognostic factors for neuroblastoma were further analyzed, and the age of onset and stage of disease were found to have remained constant for 23 years. Factors relating to the improvement of survival were discussed.

Carcinoma, Hepatocellular↗
PubMed1988

Ocular hypotensive effects of monoamine oxidase-A inhibitors in rabbit.

The effects of monoamine oxidase-A (MAO-A) inhibitors with epinephrine on intraocular pressure in the pigmented rabbit were studied. MAO-A inhibitors were used topically with or without various concentrations of epinephrine. For the measurement of intraocular pressure, applanation pneumatonography was used and tissue MAO activities were determined by radiometric assay. After topical administration with clorgyline, MAO-A activities in the bulbar conjunctiva and the iris-ciliary body were remarkably inhibited, whereas MAO-B inhibition was minimal. Maximal reduction of intraocular pressure with 0.05% epinephrine was 3.2 mmHg. Single administration of clorgyline, amiflamine, moclobemide or CGP 11305-A caused decreases in the intraocular pressure of 2.0, 2.5, 1.8 and 2.4 mmHg, respectively. In the coadministration experiments with epinephrine, the ocular hypotensive effects of epinephrine were potentiated with clorgyline, amiflamine, moclobemide and CGP 11305-A (6.6, 4.8, 5.6 and 5.8 mmHg). On the contrary, they were not influenced by the MAO-B inhibitor deprenyl. These results indicated that MAO-A inhibitors potentiated the ocular hypotensive effects of epinephrine, and that the coadministration of a reversible MAO-A inhibitor with epinephrine might be useful for patients with glaucoma.

Animals↗
PubMed1987

Serial determination of serum neuron-specific enolase in patients with neuroblastoma and other pediatric tumors.

The importance of determination of serum neuron-specific enolase (NSE) in patients with neuroblastoma has been emphasized by several authors. However, the specificity and sensitivity of NSE have not yet been well studied in tumors of infancy and childhood, nor is the role of serial determination of NSE in monitoring these patients fully understood. Concentrations of serum NSE were determined by a newly developed radioimmunoassay technique in 241 samples from 111 patients. NSE was also assayed in sera of nude mice bearing human pediatric tumors (16 samples), as well as in 30 tumor specimens. Eighty-two serum samples from 19 patients with neuroblastoma all showed NSE values (mean 120.2 ng/mL, range 16.2 to 722.0 ng/mL) elevated beyond the upper border of the normal range (14.6 ng/mL), even though four of the 19 patients had normal urinary excretion of 3-methoxy-4-hydroxymandelic acid (VMA) and 3-methoxy-4-hydroxy-phenylacetic acid (HVA). Twelve of these patients were monitored with serial NSE determinations, and their serum NSE were found to correlate well with the tumor burden, but were transiently modified by chemotherapeutically induced cell death. All 68 samples from nine patients, free of neuroblastoma at assessment, showed NSE values within the normal range. Thirteen of 25 patients with tumors other than neuroblastoma, however, showed serum NSE values mildly elevated beyond the upper border of the normal range (mean of the 25 patients 36.7 ng/mL, range 5.0 to 234.0 ng/mL). Results from our nude mouse study and from NSE analysis of the tumor extracts paralleled the clinical results.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1987

[Bone metastasis of malignant solid tumors in childhood].

A total of 452 cases of childhood malignant solid tumors were treated over the last twenty years at the National Children's Hospital. These included 175 cases of neuroblastoma, 64 cases of Wilms' tumor, 65 cases of malignant lymphoma, 45 cases of soft tissue sarcoma, 31 cases of hepatoma, 20 cases of malignant teratoma, 17 cases of testicular tumor, 7 cases of ovarian tumor and 28 cases of other forms of malignant solid tumor. Bone metastasis was observed in 62 of 175 cases of neuroblastoma, 3 of 64 cases of Wilms' tumor, one of 65 cases of malignant lymphoma, 4 of 45 cases of soft tissue sarcoma, one case of pulmonary blastoma and one case of osteogenic sarcoma, giving a total occurrence of bone metastasis in 72 of the 452 cases. The main sites of bone metastasis in neuroblastoma were the skull (61.4%), femur (56.8%), orbit (27.3%) and spine (22.7%). The average values of serum calcium and alkaline phosphatase activity showed no significant difference. The patients with bone metastasis were treated with a combination of radiation therapy and intensive chemotherapy, resulting in temporary improvement. The survival of patients with stage IV neuroblastoma with bone metastasis was worse than that of similar patients without bone metastasis.

Bone Neoplasms↗
PubMed1984

Mass screening for neuroblastoma in infants in Japan. Interim report of a mass screening study group.

A mass screening system for the early detection by means of a vanillylmandelic acid test of neuroblastoma in 6-month-old infants in Japan has been developed in eight districts. 16 of the 281 939 infants screened by this test had neuroblastoma, equivalent to a very high incidence of 1 in 17 621. 15 of the 16 children with neuroblastoma are alive; the other child died 1 month after surgery. This mass screening system for neuroblastoma used in infancy can help to improve prognosis in infants with this malignant disorder.

Adrenal Gland Neoplasms↗