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Biomedical subjects

A Igata

Publications and source records attributed to A Igata.

At least 19 recordsLinked to original sources
PubMed1992

Truncal hypesthesia in patients with Minamata disease.

Patients with Minamata disease (methylmercury intoxication) usually suffer from the glove and stocking type hypesthesia in the extremities. Recently it is believed that the sensory disturbance of the disease may be a manifestation of the cerebral involvement. The aim of this study was to determine the pain threshold of their extremities and body by algesimeter. The results showed that a majority of the patients had elevated pain thresholds in the body and the extremities, and suggest that the pain impairment is not responsible for the peripheral nerve involvement.

Aged↗
PubMed1992

[The significance and limit of thermography at present].

Thermography has been reported to be usefull in the detection and characterization of nervous diseases. Recently, however, some papers report that it is not useful. In this paper, problems including its history, and measures to solve them are discussed. It is proposed that methods to distinguish patients from normal subjects and information that cannot be obtained by other tests, are needed.

Arterial Occlusive Diseases↗
PubMed1990

Nationwide survey of HTLV-I-associated myelopathy in Japan: association with blood transfusion.

To study the epidemiology of human T-cell lymphotropic virus type I (HTLV-I)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) in Japan, we conducted two nationwide surveys between October 1986 and March 1989. A total of 710 patients with HAM (definite HAM, 589; probable HAM, 121) were reported. Of the 589 patients with definite HAM, 69% were residents of the areas with the highest prevalence HTLV-I in Japan. To determine the importance of blood transfusion in the pathogenesis of HAM/TSP, we performed a case-control study in the Kagoshima district in southern Japan. Significantly more patients with HAM reported a history of blood transfusion (26/129, or 20%) than did subjects in a health survey of the general population (41/1,290, or 3%; odds ratio = 7.7, p less than 0.001) or than did hospitalized neurological patients (6/119, or 5%; odds ratio = 4.8, p less than 0.001). Furthermore, the cumulative percentages of the intervals between blood transfusion and the onset of the symptoms of HAM fit a lognormal curve, suggesting that transfusion was an important common exposure. Blood transfusion probably transmitted HTLV-I to the patients with transfusion-associated HAM because there was a significant decrease in the number of patients with the transfusion-associated HAM who received blood after implementation of nationwide screening of blood donors in 1986 (p = 0.004). In the first 2 years, screening the blood supply in Japan appears to have decreased the number of reported patients with HAM by 16%.

Humans↗
PubMed1990

Thin-layer chromatography of urinary neutral oligosaccharides: the detection of blood group-related oligosaccharides and screening for lysosomal storage disease.

We devised an improved technique of thin layer chromatography, which permitted the high resolution of urinary neutral oligosaccharides and the qualitative determination of blood group related oligosaccharides as well as oligosaccharides pathologically secreted in lysosomal storage diseases. This procedure can be used inscreening for disorders associated with abnormal excretion of oligosaccharides, as well as in the purification of oligosaccharides.

ABO Blood-Group System↗
PubMed1990

Leucocyte alpha-1,4- and alpha-1,6-glucosidase activities towards oligosaccharides in late onset glycogenosis type II.

We describe the partial characterization and some properties of leucocyte alpha-glucosidase towards disaccharides with the alpha-1,4 (maltose) and alpha-1,6-glucosidic linkage (isomaltose) and tetrasaccharides with the alpha-1,4 (maltotetraose) and alpha-1,6-glucosidic linkage (tetrasaccharide, Glc alpha 1----6Glc alpha 1----4Glc alpha 1----4Glc, which was isolated from the urine of a patient with glycogenosis type II). Leucocyte alpha-glucosidase showed optimal activity towards all four oligosaccharides under two conditions, acidic (pH 4.0-4.5) and neutral (pH 6.0-6.5) regions. Our comparative studies on enzyme kinetics showed that leucocyte alpha-glucosidase was able to hydrolyze both the 1----4 isomers and the 1---6 isomers at acidic and neutral pH. Acid alpha-glucosidase could hydrolyze maltose about 10 times faster than isomaltose, and maltotetraose about 5 times faster than tetrasaccharide isolated from urine. In leucocytes of the patient with late onset glycogenosis type II, acid alpha-glucosidase activities towards maltose, isomaltose, maltotetraose and tetrasaccharide isolated from urine showed 75.3%, 67.4%, 76.5% and 41.4% of normal control values, respectively. Neutral alpha-glucosidase activities towards these four oligosaccharides were normal. Tetrasaccharide with alpha-1,6-glucosidic linkage might be accumulated by the impaired hydrolysis in the circulation as well as the leakage of undegraded glycogen to the circulation from the affected muscle.

Adult↗
PubMed1990

[Database for patients with Duchenne muscular dystrophy].

We had a plan to arrange database for patients with Duchenne muscular dystrophy in Japan. The purpose of this projects will make it possible to design accurate and serial evaluation of patients and conduct therapeutic trial. The items of input are composed of two cards. One card is basic card which fills out mainly patients' basic and genetic informations, the other is course card which records symptoms, muscle strength, joint range of motion, laboratory studies including pulmonary function testing and therapeutic trials. These cards are written by physicians of dystrophic wards in national hospital twice a year and are sent to the statistical coordinating center of database. On the other hand outputs include annual report and optional output which are requested by each investigator. Now 519 basic cards and 1026 course cards have completed. In this paper we tried to output about some results of the data obtained. These outputs include the age of initial gait and difficulty in walking, mean muscle strength by functional grade and the change of muscle strength during the administration of some drugs.

Adolescent↗
PubMed1990

The risk of development of HTLV-I-associated myelopathy/tropical spastic paraparesis among persons infected with HTLV-I.

Using data obtained in national surveys of human T-lymphotropic virus type I (HTLV-I)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) conducted in Japan in 1987 and 1988, we estimated the yearly and lifetime risk that HAM/TSP will develop in an HTLV-I-infected person. "Definite" HAM/TSP was defined as slowly progressive myelopathy with antibodies to HTLV-I in both serum and cerebrospinal fluid. Estimates of HTLV-I infection rates in eight endemic prefectures, by age group and sex, were obtained from serologic studies of blood donors; population figures, by age group, sex, and prefecture, were obtained from the census. Of 589 definite cases of HAM/TSP reported nationally, 397 occurred in residents of the eight endemic prefectures; of these, 170 reported onset of illness during the years 1982-1988 (average incidence, 24.3 cases/year). Using the estimated HTLV-I infection rates and the 1985 census figures, we estimated the number of HTLV-I-infected persons in the eight prefectures in 1985 at 794,800. We therefore estimated the incidence of HAM/TSP among HTLV-I-infected persons at 3.1 x 10(-5) cases/year; assuming a lifetime of 75 years, the lifetime incidence is approximately one quarter of 1%. This estimate is important in counseling persons such as blood donors found to be infected with HTLV-I.

Adolescent↗
PubMed1990

Lysosomal acid lipase deficiency in rats: lipid analyses and lipase activities in liver and spleen.

We report the biological characterization of an animal model of a genetic lipid storage disease analogous to human Wolman's disease. Affected rats accumulated cholesteryl esters (13.3-fold), free cholesterol (2.8-fold), and triglycerides (5.4-fold) in the liver, as well as cholesteryl esters (2.5-fold) and free cholesterol (1.33-fold) in the spleen. Triglycerides did not accumulate, and the levels actually decreased in the spleen. Analysis of the fatty acid composition of the cholesteryl esters and triglycerides showed high percentages of linoleic acid (18:2) and arachidonic acid (20:4) in both organs, especially in the liver. No accumulation of phospholipids, neutral glycosphingolipids, or gangliosides was found in the affected rats. Acid lipase activity for [14C]triolein, [14C]cholesteryl oleate, and 4-methyl-umbelliferyl oleate was deficient in both the liver and spleen of affected rats. Lipase activity at neutral pH was normal in both liver and spleen. Heterozygous rats showed intermediate utilization of these substrates in both organs at levels between those for affected rats and those for normal controls, although they did not accumulate any lipids. These data suggest that these rats represent an animal counterpart of Wolman's disease in humans.

Animals↗
PubMed1989

Lymphocyte alpha-glucosidase in late-onset glycogenosis type II.

We describe the biochemical characterization of lymphocyte alpha-glucosidase in a 23-year-old man with intermediate clinical features between the childhood and adult forms of glycogenosis type II (Pompe's disease). Acid alpha-glucosidase activity was markedly reduced, but immunologic cross-reactive material against human liver acid alpha-glucosidase protein could be detected, and its amount was normal. In this patient, the disorder was induced by the catalytically inactive enzyme with a normal amount of enzyme protein.

Adult↗
PubMed1989

Hyperestrogenemia in neuromuscular diseases.

In order to elucidate the relationship between certain neuromuscular diseases and gonadal hormones, we measured the levels of serum estrogens and other sex-related hormones. The values were compared with those for age-matched controls. The cases, comprising bulbospinal muscular disease of the Kennedy-Alter-Sung type, Kugelberg-Welander disease, amyotrophic lateral sclerosis, and Duchenne muscular dystrophy, were all euthyroid males. The baseline levels of serum estrone were significantly higher in all of the patients than in age-matched normal subjects. Serum baseline testosterone, LH and FSH levels were all essentially normal, except low FSH levels in Duchenne muscular dystrophy. Since our patients had no overweight, liver or glandular abnormalities, we presume that the elevated serum estrone levels have resulted from increased peripheral androgen-to-estrogen conversion.

Adolescent↗
PubMed1989

Immune dysfunction in hypophosphatemic vitamin D-resistant rickets: immunoregulatory reaction of 1 alpha(OH) vitamin D3.

We investigated immunologic function in six cases with hypophosphatemic vitamin D-resistant rickets (VDRR) before and after treatment with 1 alpha-hydroxycholecalciferol (1 alpha(OH) vitamin D3). All cases suffered frequent episodes of infection, which tended to be more severe in the older patients. OKT9-, OKT10-, and OKM1-positive cells and adenosine deaminase (ADA) were significantly increased, whereas numbers and activity of natural killer (NK) cells were lower than normal before treatment. After administration of 1 alpha(OH) vitamin D3, however, the susceptibility to infection apparently decreased, and NK cell number and activity increased in all patients. ADA was also significantly decreased and remained in the normal range after treatment. These results suggest that vitamin D plays a role in the impaired immunoregulatory functions of NK cells in VDRR. Furthermore, ADA may be one parameter reflecting this immunologic impairment.

Adenosine Deaminase↗
PubMed1989

Determination of urine thiocyanate in patients with amyotrophic lateral sclerosis.

It has been reported that amyotrophic lateral sclerosis-Parkinsonism-dementia in Guam might be related to the eating of Cycas seeds, which contain cyanide. Based on this assumption, we determined the urinary thiocyanate excretion level in patients with ALS and compared this with that of other neurological diseases. The assay method was designed to use column chromatography with Amberlite IRA 402. The thiocyanate level was determined using pyridine-barbiturate method. The 24-h thiocyanate level was higher in the ALS patients of the middle stages than in the normal control group (Wilcoxon's test, P less than 0.02). There were no significant differences between the ALS patient groups of the early and terminal stages, Kugelberg-Welander disease group, Duchenne type muscular dystrophy group and control group. From these results, we concluded that ALS patients were contaminated with cyanide or thiocyanate and that, along with rapid muscular atrophy, the thiocyanate excretion levels were high.

Adult↗
PubMed1989

Partial characterization of leucocyte alpha-glucosidase in late onset glycogenosis type II.

We describe partial characterization and properties of leucocyte alpha-glucosidase from a patient with clinical features intermediate of juvenile and adult onset forms of glycogenosis type II. Acid and neutral alpha-glucosidase activities toward 4-methylumbelliferyl glucopyranoside as substrate were studied in total leucocytes, and separately in lymphocytes and granulocytes. Lymphocytes, which showed markedly reduced activities of acid alpha-glucosidase in the patient, are the most reliable peripheral blood cells for the diagnosis of glycogenosis type II. Moreover, the ratio of acid/neutral alpha-glucosidase activities, especially in lymphocytes, is a useful parameter for the diagnosis. In lymphocytes, the Km values of both acid and neutral alpha-glucosidases were essentially the same between the patient and normal controls; the Vmax value of acid alpha-glucosidase from the patient was markedly reduced, and the Vmax value of neutral alpha-glucosidase from the patient was reduced by 36% as compared with that from normal controls. Heat-inactivation experiments revealed that acid alpha-glucosidase activities of lymphocytes were relatively heat-stable, while both acid and neutral alpha-glucosidases of granulocytes were heat-labile. No differences in these properties, however, could be detected between the patient and normal controls.

Adult↗
PubMed1989

Endothelium and thrombomodulin: expression and distribution of thrombomodulin on altered endothelium and neoplastic syncytiotrophoblast.

We investigated the expression of thrombomodulin (TM) on endothelium in some pathologic states. We used the cultured endothelial cells treated with interleukin-1 (IL-1) and propagated cells by serial subculture for extended periods of time and assessed cell-surface TM molecules. We also studied the distribution of TM on surgical specimens of chorionic diseases, angiosarcoma and on several established cell lines of human choricarcinoma. Subculture of human umbilical vein endothelial cells (HUVE) up to 2 months (approximately 16 subcultures) decreased the number of cell-surface TM molecules by approximately 20% compared to the primary culture. The number of TM molecules also decreased on HUVE treated by IL-1. The treatment of the cells with IL-1 also induced change of shape. TM was found not only normal syncytiotrophoblast but also on neoplastic syncytiotrophoblast of choriocarcinoma and hydatidiform mole. However, TM was not expressed on the three established cell lines. TM was found on various types of vascular tumors, including angiosarcoma.

Cell Line↗
PubMed1989

[Oligoclonal IgG bands in patients with HTLV-I associated myelopathy--detection by agarose isoelectric focusing, transfer to cellulose nitrate and immunoperoxidase staining].

The patient with HTLV-I associated myelopathy (HAM) shows a quite uniform clinical picture characterized by slowly progressive spastic paraparesis, slight sensory disturbances and urinary frequency, and the pathogenetic relationship between spastic paraparesis and HTLV-I was established. Since then, the role of the virus in causing myelopathy has drawn increasing attention. However, we have little information about cerebrospinal fluid (CSF) abnormalities in patients with HAM. Analysis of CSF oligoclonal bands (OB) in 22 patients with HAM was reported. All of 22 patients had typical clinical signs and symptoms of HAM with high titers of anti-HTLV-I antibodies in the serum by particle agglutination method. And these antibodies against HTLV-I were confirmed by enzyme-linked immunosorbent assay and western blot. Detection or characterization of CSF OB was done by high resolution agarose gel (HARG) electrophoresis with silver staining and immunofixation method with immunostaining. Other method for detection OB was by agarose isoelectric focusing (IEF), transfer to cellulose nitrate and immunoperoxidase staining (Olsson, 1984). CSF OB was detected in 13 of 22 patients with HAM by the method of immunofixation, using HRAG. All of CSF OB reacted with peroxidase conjugated goat anti-human IgG serum. More than 3 oligoclonal bands were not detected in HRAG electrophoresis. However, CSF OB was detected in all of 22 patients by the method of Olsson (IEF, in agarose, double-antibody peroxidase labelling and avidin-biotin amplification). The majority of patients with HAM had at least 5 or more OB in the region between pH 6.8 and 9.5.(ABSTRACT TRUNCATED AT 250 WORDS)

Ampholyte Mixtures↗
PubMed1988

HLA haplotype-linked high immune responsiveness against HTLV-I in HTLV-I-associated myelopathy: comparison with adult T-cell leukemia/lymphoma.

HLA haplotypes of 27 patients with human T-lymphotropic virus type I (HTLV-I)-associated myelopathy (HAM) and 12 patients with adult T-cell leukemia/lymphoma (ATLL) were examined by analyzing HLA types of the patients and their family members. Either A11Bw54Cw1DR4DQw3, A24Bw54Cw1DR4DQ-, A24B7Cw7DR1DQw1, or A24Bw52Cw-DR2DQw1 and the related haplotypes were found in 70% of cases with HAM. None of these "HAM-associated" haplotypes was found in patients with ATLL. HLA haplotypes made up of HLA components of A26Bw62Cw3DR5DQw3 and one particular haplotype of Aw33B44Cw-DRw6DQw1 were associated with the ATLL haplotypes. These "ATLL-associated" haplotypes were also found in the patients with HAM who had no previous history of blood transfusion. The in vitro cultures of peripheral blood lymphocytes with HTLV-I virion antigens revealed that the response with HAM peripheral blood lymphocytes was remarkably higher than that with ATLL peripheral blood lymphocytes. Based on this HTLV-I-specific immune responsiveness, we can segregate the high responders in HAM (14 of 16 cases) and the low responders in ATLL (6 of 7 cases). The existence of high and low responders was also confirmed by the normal healthy individuals, whose responses were segregated with HAM-associated and ATLL-associated haplotypes. These results suggested that two ethnic groups in southern Kyushu may get the two different diseases, HAM and ATLL, because of their different immunogenetic backgrounds. The high immune response to HTLV-I seems to be an important genetic factor in the development of HAM.

Adult↗
PubMed1988

Morphometry of intramuscular nerves in amyotrophic lateral sclerosis.

Morphometric analysis of 90 intramuscular nerves from the biopsied biceps muscles of 16 cases of amyotrophic lateral sclerosis (ALS) were done and compared with controls. In all fascicles (large and small) of ALS, the total number and the numbers of large and small myelinated fibers were significantly reduced to 52, 16, and 64% of controls, respectively. A histogram of the largest fascicles revealed a unimodal distribution and a shift to the left, showing a single peak at 1 micron diameter. The results indicate proximal and distal involvement of the most remote portions of the nerves to the muscle in ALS and support previous suggestions of a neuronopathy.

Adult↗