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Biomedical subjects

M Osame

Publications and source records attributed to M Osame.

At least 289 records · Page 16Linked to original sources
PubMed1994

Immunoadsorption therapy for myasthenia gravis.

The results of a multicentre trial were analysed to evaluate the efficacy of immunoadsorption therapy for severe generalised myasthenia gravis. Twenty patients with myasthenia gravis who were concurrently receiving high dose prednisolone and azathioprine therapy were treated with an affinity-type adsorbent, using tryptophan-linked polyvinyl alcohol gel (IM-TR), according to a standardised treatment protocol. The 20 patients received five adsorption treatments within a period of 10 days. In 11, pronounced improvement of myasthenic weakness was seen and long-term remission was maintained. The treatment was especially effective in patients with thymic hyperplasia. Circulating acetylcholine receptor (AChR) antibodies were reduced by about 60% by treating one plasma volume. There was no difference in the rate of removal of the AChR antibodies between patients with thymic hyperplasia and patients with thymoma. No serious complications occurred during 100 procedures. It was concluded that the immunoadsorption therapy with IM-TR is useful in controlling symptoms in patients with severe myasthenia gravis who are otherwise unresponsive.

Adolescent↗
PubMed1994

Abnormal expression of laminin suggests disturbance of sarcolemma-extracellular matrix interaction in Japanese patients with autosomal recessive muscular dystrophy deficient in adhalin.

Dystrophin is associated with several novel sarcolemmal proteins, including a laminin-binding extracellular glycoprotein of 156 kD (alpha-dystroglycan) and a transmembrane glycoprotein of 50 kD (adhalin). Deficiency of adhalin characterizes a severe autosomal recessive muscular dystrophy prevalent in Arabs. Here we report for the first time two mongoloid (Japanese) patients with autosomal recessive muscular dystrophy deficient in adhalin. Interestingly, adhalin was not completely absent and was faintly detectable in a patchy distribution along the sarcolemma in our patients. Although the M and B2 subunits of laminin were preserved, the B1 subunit was greatly reduced in the basal lamina surrounding muscle fibers. Our results raise a possibility that the deficiency of adhalin may be associated with the disturbance of sarcolemma-extracellular matrix interaction leading to sarcolemmal instability.

Adult↗
PubMed1994

Dystrophin-related protein in Becker muscular dystrophy.

A benign Becker muscular dystrophy (BMD) patient with a marked decrease in dystrophin exhibited remarkable expression of dystrophin-related protein (DRP) on most of the muscle cell membrane. A phenotypic Duchenne muscular dystrophy patient with a truncated form of dystrophin exhibited no DRP expression on the muscle cell membrane except for the neuromuscular junction. Increased DRP expression might compensate for a lack of dystrophin in some BMD patients.

Adolescent↗
PubMed1994

Effects of LDL-apheresis on serum lipoprotein (a), C4b binding protein, protein C, protein S, and complement components.

The short-term effects of low-density lipoprotein (LDL) apheresis using a dextran sulfate-cellulose (DSC) column equipped with a plasma separator using a polysulfone (PS) membrane filter on the serum total cholesterol, lipoprotein(a) (Lp(a)), C4b binding protein (C4bp), protein C and protein S and complement components levels were examined in a patient with familial hypercholesterolemia (heterozygote, type IIa). PS/DSC-LDL apheresis markedly lowered the total cholesterol by 69.9 +/- 2.9%, serum Lp(a) level by 80.2 +/- 3.4%, and C4bp by 81.1 +/- 2.5% after 8 apheresis sessions. All the above parameters gradually returned to the baseline levels within 10 days. The free protein S level was not significantly changed, while the C4bp/protein S complex level was markedly decreased relative to the decrease in C4bp. The protein C level was moderately reduced by 29%. Almost all serum complements and complement co-factors as well as C4bp were moderately to markedly decreased after LDL apheresis, but returned to the initial levels within a few days. PS/DSC-LDL apheresis effectively eliminated both serum LDL and Lp(a), and did not have an adverse effect on fibrinolysis or complement cascade in the blood.

Adult↗
PubMed1994

[Tropical spastic paraparesis (HAM/TSP) in different regions of the world. Subclinical and geographic differences in neurophysiologic studies].

Tropical spastic paraparesis is the same clinical entity all over the world. It has been suggested that some clinical presentation differences are due to not well defined environmental factors that act along with the virus, modifying the start and progression of the disease. We analyzed 25 publications on the disease coming from different parts of the world, aiming to unravel regional differences. In these reports, we found different degrees of subclinical abnormalities in visual and somatosensory evoked potentials, nerve conduction studies, superior and inferior limb F wave and electromyography. It is concluded that genetic, environmental and even cultural factors may influence the clinical presentation of tropical spastic paraparesis and explain the detected differences in the reports analyzed in this study.

Electromyography↗
PubMed1994

[Idiopathic interstitial pneumonia that responded to intermittent intravenous administration of high-dose cyclophosphamide].

A 78-year-old man was admitted to the hospital with exertional dyspnea and fever. Chronic idiopathic interstitial pneumonia (IIP) had been diagnosed a year before. Follow-up chest X-ray examination showed diffuse reticular shadows and progressive shrinkage of both lower lobes. Chest CT scan revealed honeycomb appearance of both lower lung fields. Moderate hypoxemia was found by arterial blood gas analysis. The patient responded to an orally administered corticosteroid. Because tapering of the medicine caused exacerbation of the hypoxemia, methylprednisolone pulse therapy was given with poor response. Then high-dose (750 mg) of cyclophosphamide (CPM) were given intravenously 6 times, every 3 or 4 weeks. After the second administration of CPM, decreases in hypoxemia and in blood LDH levels were observed. This treatment allowed as to taper the dose of oral corticosteroid from 60 mg/day to 15 mg/day. This case suggests that intravenous administration of high-dose CPM may be effective against IIP.

Aged↗
PubMed1994

A multicenter case-control study of HTLV-I associated uveitis. Study Group for HTLV-I Associated Ocular Diseases.

To elucidate the epidemiology of human T-lymphotropic virus type I (HTLV-I) associated uveitis (HAU), a multicenter case-control study was carried out by the collaboration of university hospitals throughout Kyushu and Okinawa in southwestern Japan where HTLV-I is endemic; two institutions in the non-endemic metropolitan areas of Tokyo and Yokohama also participated. A total of 426 cases of endogenous uveitis were registered during the five-month period between September 1992 and January 1993; the etiology and clinical entity of about half of the cases were definable, and for the remaining half were unknown. Assessment of antibodies to HTLV-I revealed that the group of entity-undefined uveitis cases showed a significantly high seroprevalence when compared with age- and sex-matched controls. Estimate of the risk of HTLV-I provided supportive evidence for its etiologic contribution to a considerable proportion of hitherto undefined isolated uveitis cases. The prevalence of HAU thus defined was found to correspond to the seroprevalence in the relevant areas. Occasional cases were found among residents in the nonendemic metropolitan areas who had moved from the endemic areas.

Adolescent↗
PubMed1994

Clinical features of HTLV-I associated uveitis determined in multicenter collaborative study. Study Group for HTLV-I Associated Ocular Diseases.

The elucidation of the clinical features of human T-lymphotropic virus type I (HTLV-I) associated uveitis (HAU) was advanced by the results of a multicenter study performed by the collaboration of university hospitals throughout Kyushu and Okinawa and two institutions in the Tokyo-Yokohama metropolitan areas of Japan. A total of 426 cases of endogenous uveitis were registered during the five-month study period between September 1992 and January 1993. The etiology and clinical entity remained unknown for about half of these cases. A series of 50 cases of HTLV-I seropositive, entity-undefined uveitis were suspected to be HAU, and analyses of these cases provided the following clinical features of this recently defined uveitis entity. 1) middle-aged adult of either sex develops acute, inflammatory uveal disease and presents with visual haze or floaters, or both; 2) active disease consists of granulomatous or nongranulomatous reactions accompanied by vitreous opacities and retinal vasculitis with rare retinochoroidal exudative changes; 3) the disease resolves with response to corticosteroids, although leaving sequelae in occasional cases; 4) the disease affects one or both eyes, and up to half of cases show recurrent disease within years; 5) the visual outcome is usually favorable; 6) the disease occurs as an isolated disorder, but may sometimes be complicated by HTLV-I associated myelopathy. Hyperthyroidism is also associated in occasional cases; 7) the titer of serum HTLV-I antibodies is significantly high compared with that of HTLV-I carriers, suggesting an immune mechanism as the pathogenesis of HAU.

Adolescent↗
PubMed1994

[Therapy for HAM/TSP and AIDS].

(1) We evaluated efficacy of several treatments for HTLV-I-associated myelopathy (HAM) on the basis of our study on 254 HAM patients and of literature review. Improvement of motor disability more than fair response was obtained as follows: 82% in prednisolone, 69% in interferon-alpha, 92% in fosfomycin, 82% in high-dose vitamin C, 72% in blood purification therapy, 70% in heparin, 59% in salazosulfapyridine, 56% in thyrotropin-releasing hormone, 55% in erythromycin, 50% in mizoribine. (2) In the absence of clear guideline, the efficacy of zidovudine in the AIDS dementia complex has been demonstrated. There are also efficacy of amytriptylinein controlling HIV headache, corticosteroid in mononeuritis multiple and inflammatory myositis, hydrocortisone in autonomic neuropathy and plasmapheresis in distal sensory neuropathy respectively. Otherwise, it is emphasized that ddI, ddC and d4T have peripheral neuropathy as major, dose related side effect.

AIDS Dementia Complex↗
PubMed1994

[Detection of HTLV-I proviral DNA by PCR method from bronchoalveolar lavage cells of HTLV-I carriers].

To elucidate whether or not human T-lymphotropic virus type I (HTLV-I) infected cells exist in the lungs of HTLV-I carriers, we examined bronchoalveolar lavage (BAL) cells for the presence of HTLV-I proviral DNA by the polymerase chain reaction (PCR) method. The cells were harvested from HTLV-I seropositive individuals with or without various respiratory diseases. The HTLV-I pX region was detected from separated BAL cells by dot blot hybridization after PCR in all 14 HTLV-I seropositive individuals tested. Four out of these 14 individuals were asymptomatic HTLV-I carriers, while the other ten had symptoms, including those of bacterial pneumonia, chronic respiratory tract infection, interstitial pneumonia, atypical mycobacterial infection, bronchitis, mediastinal lymphadenitis and bronchial asthma. HTLV-I proviral DAN was detected in BAL cells from patients with a normal proportion of lavage lymphocytes and a normal CD4/CD8 ratio. These findings suggest that HTLV-I infected cells may commonly exist in the lower respiratory tract and alveolar space without producing characteristic symptoms/signs, and that any causal relation to the pulmonary lesion must therefore be carefully elucidated, in HTLV-I carriers.

Aged↗
PubMed1993

Vesnarinone inhibits production of HIV-1 in cultured cells.

Vesnarinone, a synthetic oral cardiotonic agent that has been used for treatment of patients with congestive heart failure, was found to inhibit replication of HIV-1 in a peripheral blood lymphocytes model and in chronically infected macrophages at clinically achieved concentrations. Vesnarinone has no direct inhibitory activity against the reverse transcriptase of HIV-1, syncytium formation in short term assays, or retroviral protease. In addition, vesnarinone inhibits production of TNF-alpha and IL-6 by human peripheral blood mononucleated cells stimulated with LPS. These observations suggest that vesnarinone may be therapeutically useful in patients infected with HIV-1.

Antiviral Agents↗
PubMed1993

Immunohistochemical characteristics of polymyositis in patients with HTLV-I-associated myelopathy and HTLV-I carriers.

The immunohistochemical characteristics of polymyositis in patients with HTLV-I-associated myelopathy (HAM) and HTLV-I carriers were studied. Infiltrating cells were predominantly T cells and were not different from in the control group. All specimens contained positively staining muscle fibers for MHC class I antigens, but class II antigens were also expressed in some muscle fibers in 1 patient with HTLV-I-negative polymyositis, 3 patients with HTLV-I-positive polymyositis, and all 5 HAM patients with polymyositis. Expression of the neural cell adhesion molecule was lowest in the HAM patients. These findings may suggest a different immune environment in polymyositis with HAM.

Antigens, CD↗
PubMed1993

Long-term follow-up of patients with Duchenne muscular dystrophy receiving ventilatory support.

We, retrospectively, examined the clinical course, decline in pulmonary function, and requirements for ventilatory assistance in 54 patients with Duchenne-type muscular dystrophy (DMD) who were followed in the muscle disease ward of the National Hospital in Kagoshima, Japan, over the past 20 years. The percentage of the predicted vital capacity (%VC) declined in relation to age and stage of disease. Most patients required assisted ventilation when the %VC fell below 10%. Twenty patients were treated with a negative pressure chest respirator. Six of these died at the mean age of 23.2 years after being on the respirator for a mean period of 18 months. Fourteen patients are surviving at a mean age of 23.5 years after being on the respirator for a mean period of 39 months.

Adolescent↗
PubMed1993

Histochemical and ultrastructural pathology of skeletal muscle in a patient with abetalipoproteinemia.

Pathological examination was carried out of the skeletal muscle of an 8-year-old boy with abetalipoproteinemia. The patient complained of diarrhea, and showed a deficiency of betalipoprotein, decreased fat-soluble vitamins, acanthocytosis and a mild increase in serum creatine kinase. The prominent histochemical finding was punctate deposits of acid phosphatase activity in most fibers. Ultrastructural lesions revealed a number of giant lysosomes. Although these pathological findings seemed to be related to vitamin E deficiency, other pathological findings such as concentric laminated bodies or filamentous bodies were also observed. The clinical course and the changes in the pathological findings in our patient after long-term vitamin E therapy need to be observed.

Abetalipoproteinemia↗