Increase in plasma thrombomodulin in patients with systemic sclerosis.
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Aged↗
Biomedical subjects
Y Soma
Publications and source records attributed to Y Soma.
[A case of juvenile onset ataxia with dystonia, myoclonus, sensorineural hearing loss and mental retardation].
A 35-year-old female was reported who presented early onset and slowly progressive ataxia and retrocollis which appeared at the age of nine. On admission, neurological examination revealed cerebellar ataxia, dystonia of the neck and the right arm, myoclonus of the neck and the shoulder, slight mental retardation, supranuclear upper gaze palsy, and sensorineural hearing loss. Laboratory examination showed high serum CK activity. Electromyography and muscle biopsy findings suggested slight muscular involvement. CSF level of HVA and 5-HIAA were reduced. MRI demonstrated marked cerebellar atrophy and slight atrophy of the brain stem. To our knowledge, the characteristic combination of the neurological sign in this case has not been reported. This case was compared with EOCA (early onset cerebellar ataxia with retained tendon reflexes) and other juvenile onset cerebellar ataxia and dystonia.
Adult↗
[Relations between neuropsychological findings and lateral asymmetries of cerebral blood flow measured by SPECT in dementia of Alzheimer type].
We studied 16 right-handed patients clinically diagnosed as dementia of Alzheimer type (6 men, 10 women; aged 63-85, mean 72.8 years). The average duration of symptoms was 2.7 years. Dementia ranged from mild to moderately severe. None had clinical or laboratory evidence of cerebrovascular disease (Hachinski ischemic scores for all patients were 4 or below 4). All received the Wechsler Adult Intelligence Scale (WAIS), Mini-mental State Test (MMS) and Western Aphasia Battery (WAB, First Japanese edition, 1986). Regional cerebral blood flow (rCBF) was evaluated by single photon emission CT (SPECT) with 123I-N-isopropyl-p-iodoamphetamine (123I-IMP), using the Matsuda's quantitative method (Am J Physiol Imag 1: 186, 1986). Regional tracer uptake was measured in regions of interests (ROIs) over right and left frontal, temporal, parietal and occipital cortical regions; basal ganglia; and cerebellar hemispheres. The subjects were divided into three groups on the basis of lateral asymmetries in the temporal and parietal cortexes of rCBF (left < right, n = 5; right < left, n = 3; left = right, n = 8). We decided that lateral asymmetry was present when rCBF for each ROI between left and right sides differs by more than 10%. General score (MMS, T-IQ) was not correlated with asymmetry of cerebral blood flow. Verbal IQ in patients with predominant hypoperfusion of left temporal and parietal lobe was significantly lower than other group, while performance IQ and WAB constructive scores were lower in those with right hemispheric hypoperfusion (p < 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)
Aged↗
A case of systemic sclerosis with anticentromere, antitopoisomerase I, and anti-U1RNP antibodies.
We describe a case of systemic sclerosis (SSc) positive for 3 autoantibodies; anticentromere antibody, antitopoisomerase I antibody, and anti-U1RNP antibody, It is known that coexistence of 2 of these 3 antibodies is rare and the coexistence of these 3 antibodies in serum from a patient with SSc has not been reported. In addition, we examined our stock serum samples from 236 cases with SSc or related disorders for the presence of specific antinuclear antibodies and were unable to find a serum sample containing all 3 antibodies.
Autoantibodies↗
[A case of diagnostic dyspraxia without ideomotor apraxia by callosal lesion].
A case of diagnostic dyspraxia was reported. A 57-year-old right handed male had been suffering from the lack of cooperation between his right and left hands for six months. Except for decreased deep tendon reflexes in all extremities, there were no abnormal findings on neurological examination. On neuropsychological examination, he was attentive, well orientated and his spontaneous speech, comprehension, naming, repetition and reading were intact. There was peculiar dissociative behavior between his right and left hands. For instance, he put a cigarette or coin in the pocket with his right hand then his left hand took out and replaced them, and he buttoned his shirts with his right hand but then unbuttoned with his left hand. These left hand oppositional behavior to his right hand were triggered by voluntary activities of his right hand. Left unilateral agraphia was also revealed but ideomotor apraxia, compulsive manipulation of tools and grasp reflex were not demonstrated. T1-weighted MRI demonstrated irregular low signal intensity areas extending from the genu to the body of the corpus callosum. No definite lesion was detected in the medial aspect of the frontal lobe. Only small numbers of diagnostic dyspraxia have been reported and such cases without ideomotor apraxia or medial frontal lesion are even rare. MRI is very useful for detecting the lesion of the corpus callosum.
Apraxias↗
[Neuropsychological investigation in multiple sclerosis].
We studied neuropsychological performance in 10 patients with multiple sclerosis (MS). Neuropsychological test batteries consisted of Mini-Mental State Examination, Digist Span, Paired Associate Learning Test (PAL), Benton Visual Retention Test, Kohs Block-Design Test, Digit Symbol Subtest in WAIS, 'Kanahiroi" Test, Verbal Fluency and Wisconsin Card Sorting Test. In accordance with cerebral lesions on MRI, patients were divided into three groups. A: Multiple-confluent lesions, B: Discrete lesions, C: No lesion. In MS patients as a whole, performance of PAL, "Kanahiroi" Test and Verbal Fluency were significantly impaired compared with 10 age and education matched normal controls (p < 0.05), while other tests were not. In four out of five patients of A group, more than four neuropsychological tests showed below mean -2SD score of normal controls, whereas in patients of B and C group, less than three neuropsychological tests showed below -2SD score of normal controls. In conclusion, severity of cognitive impairment in patients with MS correlated with lesion extent on MRI. Patients with MS exhibited significant cognitive impairment on tasks of recent memory and mental processing speed. It is suggested that MS patients show the features of subcortical dementia.
Adult↗
Clinical evaluation of scleroderma spectrum disorders using a points system.
We have established a new diagnostic method using a points system to evaluate patients with early scleroderma and those with scleroderma spectrum disorders (SSD). To examine the clinical usefulness of this method, it was applied to a total of 215 cases including 97 patients with scleroderma, 32 with SSD, 28 with presumed primary Raynaud's phenomenon (RP) and 58 with other connective tissue disorders (CTD). A total score was obtained for each patient as the sum of the following five factors: (1) extent of skin sclerosis (maximum, 10 points); (2) pulmonary changes (maximum, 4 points); (3) antinuclear antibodies (maximum, 5 points); (4) pattern of Raynaud's phenomenon (maximum, 3 points); and (5) nailfold bleeding (maximum, 2 points). Of the 97 scleroderma patients, 86 (89%) had 9 or more points, and of the 32 SSD patients, 28 (88%) had 5 to 8 points. In contrast, all patients with presumed primary RP and 54 of 58 (93%) patients with other CTD had 0 to 4 points. These data suggest that this diagnostic method is very useful not only for clinical evaluation of SSD, but also for the differentiation of scleroderma and SSD from other CTD and primary RP.
Connective Tissue Diseases↗
MRI of anterior spinal artery syndrome of the cervical spinal cord.
Cervical spinal cord lesions in the anterior spinal artery syndrome were delineated on magnetic resonance images (MRI) in four patients. The lesion was always seen anteriorly in the cervical cord. On T2-weighted images, the lesions appeared hyperintense relative to the normal spinal cord, while on T1-weighted images, two chronic lesions appeared hypointense, with local atrophy of the cord. In one case, repeated T1-weighted images showed no signal abnormality 4 days after the ictus, but the lesion became hypointense 18 days later, when contrast enhancement was also recognized after injection of Gd-DTPA; this sequence of intensity changes was similar to that of cerebral infarction. The extent of the lesion seen MRI correlated closely with neurological findings in all cases. Although the findings may not be specific, MRI is now the modality of choice for confirming the diagnosis in patients suspected of having an anterior spinal artery syndrome.
Adult↗
Platelet-derived growth factor AA homodimer is the predominant isoform in human platelets and acute human wound fluid.
Platelet-derived growth factor (PDGF) is a dimeric protein composed of A- and B-chains (AA, AB, and BB). PDGF purified from human platelets has been shown to be composed primarily of the AB heterodimer. Immunoblots of total platelet extracts, cell-bound PDGF from the platelet extracts, and acute human wound fluid with PDGF A- and B-chain-specific antisera all demonstrate that the PDGF A-chain is the predominant peptide. Chemotactic and immunochemical assays of chromatographic fractions during PDGF isolation support these observations and demonstrate that PDGF AA can be separated from PDGF AB and BB by ion exchange chromatography. These studies indicate that the AA isoform constitutes the major PDGF dimer contained in human platelets and is the major form present at sites of injury during the acute phase of the wound repair response.
Amino Acid Sequence↗
Alteration of the chemotactic response of human skin fibroblasts to PDGF by growth factors.
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Blood Physiological Phenomena↗
[Angiographic studies of atherosclerotic changes in coronary vein grafts after operation].
A total of 230 vein grafts were studied angiographically in 116 unselected survivors of 260 coronary bypass operations performed from May 1977 through October 1989 in order to investigate atherosclerotic changes in coronary vein grafts after operation. These patients were divided into three groups according to the interval from operation to angiography. In group A (30 patients) the interval was less than one year (mean interval 8.2 months), in group B (73 patients) from one to five years (mean interval 19.2 months) and in group C (13 patients) more than 5 years (mean interval 96.6 months). Fifty-five vein grafts were in group A, 153 vein grafts in group B and 22 vein grafts in group C. The graft patency rate of each group was 83.6%, 89.5% and 90.9% respectively (N.S.). To classify angiographic appearances we believe to be caused by atherosclerosis, we devised a grading system. Category I indicated that the graft outline was completely smooth without any irregularity; Category II indicated that less than 50% of the estimated surface area of the graft intima was irregular; Category III indicated that more than 50% of the intima was involved. Significant stenosis indicated narrowing reducing the lumen to less than 50% of the graft. Of the 203 patent grafts 181 grafts (89%) were in Category I, 22 grafts (11%) in Category II, but no graft in Category III. In group A of the 46 patent grafts 45 grafts (98%) were classified as Category I and 1 graft (2%) was classified as Category II.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
[Extended aortic reconstruction for dissecting aneurysm of the aorta].
While lifesaving palliative surgery may be selected for dissecting aneurysm of the aorta, extended reconstructive surgery has been advocated as a more curative procedure. We performed a variety of extended reconstructive surgeries, for example, replacement of the ascending aorta and the aortic arch, subtotal replacement of the thoracic aorta, replacement of the entire descending thoracic aorta and replacement of the entire descending aorta in 20 cases of dissecting aneurysm of the aorta. However, the operative mortality remained high as 35%, and the operative procedures have not yet to be standardized. We believe that it is extremely important to strive for much greater improvement in the operative results by correct selection of the operative indications, and by improving the operative techniques and patient management during surgery.
Adult↗
[A case of benign intracranial hypertension with bilateral reversible thalamic lesions on magnetic resonance imaging].
A 10-year-old girl had complained of headache, vomiting and blurred vision for one month before admission to our hospital. Her neurological findings were normal, except that the examination of the fundi revealed papilledema. The cerebrospinal fluid pressure was elevated to 220 mmH2O. The brain MRI showed bilateral and approximately symmetrical hyperintense areas located in the thalamus. These disappeared on the repeated MRI, and her symptoms regressed spontaneously within a month. Her illness was diagnosed as benign intracranial hypertension because of the favorable clinical course. These reversible thalamic lesions might be due to circulatory insufficiency associated with intracranial hypertension.
Child↗
Differential binding, biological and biochemical actions of recombinant PDGF AA, AB, and BB molecules on connective tissue cells.
We have compared the biological and biochemical properties of recombinant PDGF AA, AB, and BB using three types of fibroblastic cells: NIH/3T3, human skin fibroblast, and fetal bovine aortic smooth muscle. PDGF binding, receptor autophosphorylation, phosphatidyl inositol hydrolysis, as well as chemotactic and mitogenic responses of the cells were analyzed. PDGF-AB and PDGF-BB showed similar receptor binding, receptor autophosphorylation, and potent biological activity for all three of the cell types tested. In contrast, PDGF-AA was biologically active only for the NIH/3T3 cells in which binding sites for PDGF-AA were abundant, but was inactive for bovine aortic smooth muscle cells and human skin fibroblasts in which binding sites for PDGF-AA were absent. PDGF-AA could not induce any biochemical changes in the human skin fibroblasts or smooth muscle cells. Western blot studies with anti-Type alpha and beta PDGF receptor antibodies indicate that the NIH/3T3 cells contained both PDGF alpha and beta receptors, whereas the human skin fibroblasts and bovine smooth muscle cells contained only detectable levels of beta receptors. These results indicate that cells possessing high levels of PDGF beta receptors only are capable of responding equally well to either PDGF AB or BB.
3T3 Cells↗
Response of scleroderma fibroblasts to various growth factors.
Abnormal growth regulation in lesional skin fibroblasts may be related to scleroderma pathogenesis. We report on the abnormal response of cultured fibroblasts derived from sclerotic lesions to various growth factors. We investigated the responses of skin fibroblasts (10 strains) and normal fibroblasts (9 strains) to the growth factors as PDGF, TGF-beta 1, EGF and basic FGF. Experiments were conducted during the proliferation and confluent stages. PDGF, EGF and basic FGF stimulated fibroblast growth during the proliferation and confluent stages, but the response of scleroderma fibroblasts was significantly lower than that of normal fibroblasts. TGF-beta 1 slightly stimulated confluent fibroblast growth and inhibited proliferating fibroblasts, and the response of scleroderma fibroblasts exceeded that of normal fibroblasts. The decreased response to growth-stimulating factors observed in scleroderma fibroblasts suggests that cultured fibroblasts derived from scleroderma lesions were already senescent because they have been activated by growth-stimulating factors and repeatedly divided in vivo. Thus, abnormal growth regulation of skin fibroblasts may be partially related to the pathogenesis of scleroderma.
Adolescent↗
Cerebral infarction: early detection by means of contrast-enhanced cerebral arteries at MR imaging.
The authors describe a new magnetic resonance (MR) imaging sign of acute cerebral ischemia/infarction. Abnormally contrast material-enhanced curvilinear structures were demonstrated in eight patients in the evolving area of cerebral ischemia/infarction within 26 hours after the ictus on contrast-enhanced, high-field-strength (1.5-T), T1-weighted spin-echo images. The abnormal enhancement is considered to represent cortical arterial vessels of markedly slowed circulation in areas of underlying brain injury, which will eventually progress to frank brain infarction. This was demonstrated at computed tomographic (CT) and follow-up MR examinations. Characteristically, the degree of the contrast enhancement of vessels appeared most intense in the proximal portions, and the intensity of enhancement gradually diminished in the more distal portions of these vessels as they pass over the convexities and finally disappear.
Acute Disease↗
Early detection of scleroderma spectrum disorders in patients with Raynaud's phenomenon.
Fifty patients with the chief complaint of Raynaud's phenomenon (RP) presented at our scleroderma clinic from March to December 1990. Physical examination, routine laboratory tests (blood, urine and chest X-ray), determination of the pattern of RP, antinuclear antibody (ANA) tests and examination for nailfold bleeding were performed. Three patients were diagnosed as having systemic sclerosis sine scleroderma, 15 patients as having RP with positive anticentromere antibody and 6 patients as having an incomplete form of mixed connective tissue disease. Thus, a total of at least 24 patients out of 50 (48%) were shown to have a scleroderma spectrum disorder. A definite RP pattern (triphasic or biphasic and bilateral), positive ANA and positive nailfold bleeding were strongly correlated statistically, suggesting that these are simple useful findings for the early detection of scleroderma spectrum disorders in patients with RP. We expect that there are many undiagnosed patients with an early-stage scleroderma spectrum disorder in the general population.
Antibodies, Antinuclear↗
Medical treatment or surgical intervention? A cooperative retrospective study on infective endocarditis--timing of operation.
Two hundred and five patients treated for infective endocarditis over the last 10 years were reviewed. There were 185 cases of native valve endocarditis (NVE) and 20 of prosthetic valve endocarditis (PVE). In the NVE group there were 175 clinically active patients and 10 non-active patients. The mortalities among 108 non-surgical and 57 surgical patients were 15.7% and 14.0%, respectively. Leading causes of deaths in the former were cardiac failure, embolism and cerebral hemorrhage. Patients with embolism showed significantly higher mortality. Culture negative endocarditis resulted in almost the same incidence of hospital death and urgent operation as staphylococcal endocarditis, and a higher incidence than streptococcal endocarditis. In 9 of 33 patients operated at our hospital, surgery was performed on an urgent basis and one NYHA class IV patient died. Indications for operation were intractable cardiac failure, uncontrollable infection and angina. In the PVE group, 3 of 4 patients operated in the active stage died of severe cardiac failure generated preoperatively. The only survivor was a patient operated early under stable hemodynamics. These results suggest that culture negative endocarditis should be observed as closely as staphylococcal endocarditis and early operation should be considered for patients with progressive cardiac failure, embolism and uncontrollable infection.
Anti-Bacterial Agents↗