[A case of primary sclerosing cholangitis with marked polyclonal hypergammaglobulinemia; effect of corticosteroid therapy].
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Biomedical subjects
Publications and source records attributed to K Yuasa.
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A 58-year-old man was admitted to our hospital because of angina pectoris with severe intermittent claudication. Angiography showed triple-vessel disease of the coronary artery and complete obstruction of the bilateral common iliac arteries from their origins. Both femoral arteries were patent by collateral supplies. Combined revascularization of coronary and femoral arteries was performed. Coronary arteries were bypassed with in situ left internal thoracic artery, gastroepiploic artery and saphenous vein graft. Bilateral femoral arteries were bypassed with externally supported Dacron graft from ascending aorta through the preperitoneal space. The patient recovered well and postoperative angiography revealed all bypass grafts patent.
The characteristics of the corneal endothelium of 134 eyes which underwent extracapsular cataract extraction and intraocular lens implantation from 1984 to 1990 were studied. We classified the initial 29 eyes as group 1 and the most recent 105 eyes as group 2. Endothelial cell loss of group 1 was much larger than that of the latter group, reflecting the degree of surgical damage. Endothelial cell of both groups decreased with index functions. The coefficient of variation of both groups showed no significant change during the observation period. The hexagonal cells of both groups decreased once after surgery, and it increased after one year in group 1, and after three months in group 2. However the late phase of the change of hexagonal cells appeared to have no relation to the degree of surgical damage. The change of hexagonal cells was supplemented by the appearance of pentagonal cells and heptagonal cells.
We have reported two cases of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) associated with Graves' disease. Case 1: a 45-year-old woman noticed a diffuse goiter, palpitation and emaciation in 1977. Laboratory studies confirmed that she had Graves' disease, and she was treated with antithyroid drug. In 1986, when the hyperthyroidism was subsided, she showed progressive symmetrical weakness and numbness in her limbs, and she was almost in tetraplegia at 1987. Markedly slowed motor and sensory nerve conductions and elevated CSF proteins as well as clinical manifestations confirmed the diagnosis of CIDP. Following corticosteroid-pulse therapy and plasmapheresis resulted in good recovery in both motor and sensory impairment, though two-times of relapses were observed. Case 2: a 33-year-old man first noticed weakness in his legs in 1977, motor and sensory disturbances progressed for 12 years. Slowed nerve conduction, high CSF proteins and two-times of relapses in early phase indicated that the CIDP was the diagnosis. In 1989 he complained general fatigue, hyperhidrosis and body-weight loss. The serum thyroid hormone levels were high, and other laboratory studies confirmed the presence of Graves' disease. The cases with both CIDP and Graves' disease has rarely been reported. The background mechanism of this association is not well understood, but the susceptibility to CIDP and Graves' disease may be related to the HLA antigens and immunoglobulin Gm allotypes of which are the genes linked to the major histocompatibility complex and controlling immune responses. The present two cases commonly shared several HLA-DR antigens, but their significance should be confirmed by examining many cases.
Parallel studies of radionuclide bone marrow imaging and bone scanning are helpful in the early diagnosis of skeletal metastasis. In bone marrow imaging, most lesions are observed as a local defect. We had two cases of nonmetastatic lesions which appeared as local defects in bone marrow imaging. The first case was a male Hodgkin's disease patient, aged 48, who had been treated with frequent chemotherapy, including the administration of a large quantities of steroids. He complained of slight pain in the left shoulder. Without increased uptake in bone scanning, abnormal accumulation of 67Ga-citrate and a local defect in bone marrow imaging appeared, corresponding to localization of the pain. Suspecting bone marrow metastasis, we performed magnetic resonance imaging (MRI). An area of slightly decreased intensity in T1-weighted spin-echo images and lower intensity than fat tissue in T2-weighted images were observed, although it was slightly enhanced by Gd-DTPA. This lesion was diagnosed by biopsy as a bone infarction. The second case was that of a 69-year-old male lung cancer patient. Though no abnormality was revealed by bone scanning or 67Ga-citrate scintigraphy, an apparent defect at the 10th thoracic vertebra was observed in bone marrow imaging. It was not accompanied by pain. MRI was also performed in this case. This was depicted as a clearly defined high intensity area. This was diagnosed as a fat island, and no change has been seen in the seven months of follow up. In conclusion, it is necessary to consider the possibility of nonmetastatic lesions, when local defects appear in bone marrow imaging performed on cancer patients.
MRI findings of pelvic radiation changes in 42 patients were correlated with the tumor and critical tissue dose, time post treatment, and clinical symptoms. The severity of tissue changes was graded. The ability of MRI to differentiate post radiation tissue changes from residual or recurrent tumor was also correlated. Radiation tissue toxicity increased significantly when the dose exceeded 4,500 cGy, with the incidence of marked rectal changes rising from 8% to 44% with a dose greater than 4,500 cGy. All grades of tissue change were seen in the rectum of time from start of therapy. All patients who exhibited clinical grade 2 or 3 rectal changes showed moderate or severe changes on MRI. Grade 1 MRI changes indicative of mucosal edema were present in 33% of patients with no clinical symptoms. In conclusion, the gradation and sequence of MRI changes following radiation therapy to the pelvis have been documented and correlated with clinical findings. With its potential for distinguishing radiation change from recurrent tumor. MRI should prove to be of value in the assessment of the post-radiation pelvis.
Fifty-eight patients with symptomatic congestive heart failure were examined for T-lymphocyte subsets in the peripheral blood using two-color laser flow cytometry as a noninvasive diagnostic procedure. The final diagnosis established by catheterization and endomyocardial biopsy were dilated cardiomyopathy (DCM, n = 24), myocarditis (MC) by the Dallas criteria (n = 12), and coronary heart disease (CHD, n = 16). The CD8+CD11- (cytotoxic T) subset was significantly low in patients with DCM (13.9 +/- 4.4 vs. controls, p less than 0.05) in comparison with MC (20.7 +/- 10.9) and CHD (22.3 +/- 5.9). Moreover, the CD4+2H4+ (suppressor/inducer T) subsets were higher in patients with DCM (27.3 +/- 6.9 vs. controls, p less than 0.01) than in those with MC (17.3 +/- 7.8) and CHD (15.6 +/- 7.9). The CD4/CD8 and CD4+2H4+/CD8+CD11- ratio were examined and compared with those of normal controls (NC n = 16). The CD4+2H4+/CD8+CD11- ratio was clearly higher in patients with DCM (2.2 +/- 0.9 vs. controls, p less than 0001) than in those with MC (1.1 +/- 0.6) CHD (0.9 +/- 0.7). A CD4+2H4+/CD8+CD11- ratio of greater than 1.6 was considered to facilitate diagnosis of dilated cardiomyopathy with 79% sensitivity and 70% specificity. There was no significant increase in the ratios between MC and CHD. However, the proportion of the CD8+Leu7+ (natural suppressor) subset of circulating T lymphocytes in patients with MC was statistically higher (19.1 +/- 6.3% vs. controls, p less than 0.05) than in DCM or CHD. An elevated ratio of CD4+2H4+/CD8+CD11- among peripheral blood lymphocytes may thus be a useful marker for differential diagnosis of dilated chronic cardiomyopathy from myocarditis and coronary heart disease.
The purpose of the present study was to investigate the influence of muscle fibre composition and stature on fractionated patellar reflex times in ten healthy untrained men (mean age: 23.3 years, SD 3.1; mass: 65.9 kg, SD 8.5; height: 172.3 cm, SD 5.3). Biopsies were taken from the right vastus lateralis muscle. Using staining for myofibrillar adenosine triphosphatase after pre-incubation at pH 4.3 and 4.6, muscle fibres were classified into slow twitch (ST), fast twitch, oxidative-glycolytic (FTa) and fast twitch, glycolytic (FTb) fibres. Total patellar reflex time (TRT) and its fractionated components--reflex latency (LAT) and reflex motor time (MT)--were obtained from the mean of ten trials in each subject whilst performing Jendrassik's maneuvre. The TRT, LAT and MT were 77.7 ms, SD 16.5, 23.4 ms, SD 1.3 and 54.2 ms, SD 16.3, respectively. The LAT was significantly correlated to the percentage number of ST (r = 0.758, P less than 0.05) and FTa fibres (r = -0.657, P less than 0.05), fast twitch:slow twitch ratio (r = -0.799, P less than 0.01) and to the height of the subjects (r = 0.901, P less than 0.001), whereas TRT and MT were not significantly correlated with either fibre types or the height of the subjects. From these results it can be concluded that the LAT during the patellar reflex is influenced by muscle fibre composition and the length of the sensory and/or motor nerve.
The effect of bifemelane hydrochloride on plasma beta-endorphin-like immunoreactivity (beta-En-LI) levels in rats was studied. Bifemelane hydrochloride (25 mg/kg) was injected i.p., and the animals were decapitated at various times after the administration. The plasma beta-En-LI levels were measured by radioimmunoassay. The effect of bifemelane hydrochloride on beta-En-LI release from the anterior pituitary was also investigated by means of an in vitro experiment. The beta-En-LI content in the hypothalamus and pituitary gland did not change significantly after bifemelane hydrochloride injection. The plasma beta-En-LI levels decreased significantly in a dose-related manner with a nadir at 40 min after the injection. The beta-En-LI release in vitro from the anterior pituitary was inhibited with the addition of bifemelane hydrochloride. The findings suggest that bifemelane hydrochloride acts on the anterior pituitary to inhibit beta-En-LI release.
We examined whether or not patients with Wegener's granulomatosis have autoantibodies against the respiratory tract and kidney, by using the direct avidin-biotin-glucose oxidase complex method. On immunostaining, the IgG fractions obtained from the sera of 2 patients with Wegener's granulomatosis and of 7 controls were coupled with biotin, and each couple was used as the primary antibody. The serum IgG sample from 1 patient with Wegener's granulomatosis definitely reacted with epithelial cells of the nasal mucosa, with the strongest reaction in basal layer. The sample from another patients with Wegener's granulomatosis showed a similar but weaker reaction. Control sera showed negative reactions in 6 subjects (3 with rheumatoid arthritis, 1 with allergic granulomatous angiitis and 2 normal volunteers) and a very weak reaction in 1 patient with poly-arteritis nodosa. These observations suggest the presence of an autoantibody reacting with some cell components of the nasal epithelium in patients with Wegener's granulomatosis.
A case of native valve endocarditis due to Acinetobacter calcoaceticus in a patient with dental caries is presented. The aortic, mitral and tricuspid valves were affected and showed vegetation by echocardiography in the affected valves. In spite of a good response to antibiotic therapy, multiple embolisms resulted in the patient's death. An autopsy confirmed the vegetation, which was calcified and contained no bacteria.
A 65-year-old man with idiopathic myocarditis is described. He was admitted with symptoms of acute heart failure. Examination revealed left ventricular hypokinesis. Results of an endomyocardial biopsy showed "resolving myocarditis". Immunological studies of the peripheral blood showed a low helper-suppressor (CD4/CD8) ratio on admission and depressed natural killer (NK) cell activity coincident with the onset of myocarditis. We considered that this immunological imbalance may have occurred during the progression of myocarditis to dilated cardiomyopathy.
The effects of large doses of thyroid hormone, thyrotropin (TSH) or thyrotropin-releasing hormone (TRH) on pro-TRH concentrations in the rat hypothalamus, cerebrum, cerebellum, brain stem, stomach and eye were studied. The rats were administered T4 (2.5 mg/kg), T3 (375 micrograms/kg), TRH (1.0 mg/kg) or bovine TSH (1.25 IU/kg) i.p. and seven rats in each subgroup were decapitated at 4 or 24 h after the injection. Pro-TRH, TRH, TSH and thyroid hormone were measured by each radioimmunoassay. Immunoreactive pro-TRH (ir-pro-TRH) and immunoreactive TRH (ir-TRH) were found in the hypothalamus, cerebrum, brain stem, stomach and eye. Ir-pro-TRH concentrations in the hypothalamus decreased significantly after T4 and T3 injection and tended to decrease after TRH and TSH injection, but not significantly. In contrast, ir-pro-TRH concentrations in other tissues showed no changes after T4, T3, TRH or TSH injection. Ir-TRH concentrations in tissues did not change significantly after these hormone injection. The plasma TSH levels decreased significantly, while these of thyroid hormone increased significantly after thyroid hormone injection. Elution profile of acetic acid extracts of hypothalamus, stomach or eye was identical to that of synthetic pro-TRH. The findings suggest that the large dose of thyroid hormone inhibits pro-TRH synthesis in the hypothalamus, and that TRH synthesis in the tissues except the hypothalamus may not be regulated by thyroid hormone.
Two cases of pulmonary alveolar proteinosis (PAP) with increased CEA were reported. Case 1 was a 62-year-old male with suspected pulmonary fibrosis, who was transferred to our hospital. Laboratory findings on admission revealed 31.3 ng/ml of CEA. Because he had severe dyspnea, lung biopsy was not carried out. His condition gradually deteriorated and he died of respiratory failure. Autopsy revealed he had PAP and no malignancy. Case 2 was a 48 year-old male referred to our hospital because of dyspnea. Serum CEA was 52.8 ng/ml. Microscopic examination of a transbronchial lung biopsy showed PAP. The level of CEA in bronchoalveolar lavage fluid was 151 ng/ml. Unilateral whole lung lavage was performed twice. With the improvement of chest X-ray findings, serum levels of CEA fell to normal level. The molecular weight of CEA in bronchoalveolar lavage fluid was 180,000. Immunochemical staining of CEA in lung revealed nonspecific findings.
A 44 year-old woman died after a history of chronic right heart failure for 25 years. Roentgenological studies showed marked pulmonary aneurysm, and hemodynamics at rest revealed severe pulmonary hypertension. The diagnosis of chronic pulmonary thromboembolism was made by MRI which identified a high intensity mass in the dilated right pulmonary artery. At autopsy, a large organized thrombus adhered to the atherosclerotic pulmonary artery, and pulmonary thromboembolism or hemorrhagic infarction were identified. Microscopic examination revealed intimal and medial proliferation in small arteries and plexiform lesions. In addition, hypoplasty of the portal vein and portacaval shunt, which were thought to be congenital, were present. It has been recognized that the portacaval shunt can be attributed to pulmonary hypertension. In this case it was considered that the main cause of pulmonary thrombus formation was both pulmonary atherosclerosis and pulmonary artery aneurysm caused by prolonged pulmonary hypertension. This is the first case of chronic pulmonary thromboembolism associated with congenital hypoplasty of portal vein.
We developed an analysis system for the evaluation of corneal endothelial photographs. Morphological parameters including hexagonality were analyzed semiautomatically with this system. The image analyzer used consists of an image processor MC68000 and image memories 512 x 512 x 17bytes in size. The algorithm that measures the number of apices of each cell is as follows. First, all crossing points of cell borders are detected. Then, each cell is enlarged in order by three pixels in eight directions. The number of crossing points which are overlapped with an enlarged cell is detected as the number of apices of the cell. The analysis time was 7.44 +/- 1.48 min. (mean +/- standard deviation) for 40 endothelial photographs which needed no manual trace for contrast enhancement.
When heat-killed Propionibacterium acnes (P. acnes) and lipopolysaccharide (LPS) were injected into mice, liver cell necrosis with infiltrating neutrophils and macrophages were induced. Proliferating cells in the spleen were histochemically investigated. P. acnes injection rapidly produced hyperplasia of neutrophils and macrophages in the red pulp of the spleen. The proportion of Bromodeoxyuridine positive cells reached a peak at 5 days after injection of P. acnes. These results indicate that proliferating cells in the spleen after injection of P. acnes are mainly neutrophils and macrophages, which are the same kinds of infiltrating cells into the liver after injection of P. acnes and LPS.
A growth-promoting factor for human myeloid cells was purified to apparent homogeneity from horse serum by a combination of gel filtration, blue Sepharose affinity chromatography, Mono Q anion-exchange chromatography, Mono P chromatofocusing and sodium dodecyl sulfate polyacrylamide gel electrophoresis. The growth promoter was an iron-bound, single glycopolypeptide chain with a molecular weight of 84,000, an isoelectric point of 5.4 and an amino terminal sequence of Glu-Gln-Thr-Val-Arg-Trp-Cys-Thr-Val-Ser-Asn-His-Glu-Val-Ser-Lys-. According to the results of the amino acid sequence, iron binding ability and physicochemical properties, we identified the growth-promoting factor as horse serum transferrin. It was highly active in promoting the proliferation of a human monocytic leukemia cell line, THP-1, as well as of two other human myeloid cell lines, HL-60 and K-562. It had the same activity in proliferating THP-1 cells as 5% fetal calf serum-supplemented medium. Horse serum transferrin could be substituted for human or bovine serum transferrin.