Biomedical subjects

K Hayashida

Publications and source records attributed to K Hayashida.

At least 37 records · Page 2Linked to original sources

PubMed1996

Effect of cilostazol, a novel anti-platelet drug, on restenosis after percutaneous transluminal coronary angioplasty.

The possible preventive effect of cilostazol, a novel anti-platelet drug, on restenosis after successful percutaneous transluminal coronary angioplasty (PTCA) was examined. One hundred and two consecutive patients, who underwent successful PTCA, were followed for 3 to 6 months. To prevent restenosis, 46 patients (60 PTCA sites) were treated with cilostazol alone (200 mg/day) (cilostazol group) and the remaining 56 (61 PTCA sites) were treated with other anti-platelet drugs and/or warfarin potassium (control group). Restenosis was defined as a more than 50% loss of the initial gain of the coronary diameter achieved by PTCA. Cilostazol did not significantly reduce the patient or lesion restenosis rate; the patient restenosis rate was 32% in the control group and 22% in the cilostazol group (P = 0.24), and the lesion restenosis rate was 30% in the control group and 23% in the cilostazol group (P = 0.44). However, the lesion non-progression rate, which was defined as the incidence of lesions with either no change or regression of coronary stenosis at the PTCA site, was significantly greater with cilostazol (37%) than in the control group (16%) (p < 0.05). Although cilostazol failed to show a significant reduction in restenosis after PTCA, the present results suggest that a further trial with a larger number of patients is needed to confirm its usefulness.

Aged↗

PubMed1996

[Anti-platelet antibody and severe thrombocytopenia during interferon-alpha therapy for chronic active hepatitis C].

We herein report a case of chronic hepatitis C where the patient developed severe thrombocytopenia during interferon therapy. The patient was a 61-year-old woman, who received interferon therapy on April 27, 1993 under the diagnosis of C type chronic active hepatitis. After 4 weeks, her platelet count had decreased to 18,000/microliters and intraoral hemorrhage had begun. Although she received 250 mg of methylprednisolone and 20 U of platelet transfusion three times, her platelet count continued to decrease to 4,000/microliters on both May 28, and on June 3, 1993, and so she was transferred to our hospital on June 4. On her second admission to our hospital, although the platelet-associated IgG (PA-IgG) had increased markedly and the megakaryocytes in her bone marrow had decreased, her platelet count had already increased to 37,000/ microliters, and this gradually returned to a normal level accompanied with a decrease of PA-IgG within one month In this case, although we found immunological abnormalities (high level of IgG, positive ANA and positive anti-smooth muscle antibody) prior to interferon treatment, we could not diagnose the patient as having suffered from autoimmune disease, including autoimmune hepatitis, because she did not satisfy the necessary criteria and because she did not have any symptoms suggesting autoimmune disease. We consider that there may be the possibility that interferon induced only an anti-platelet antibodies that caused the high level of PA-IgG and decreased the production level of platelets within the bone marrow.

Adolescent↗

PubMed1996

Rotenone, a mitochondrial NADH dehydrogenase inhibitor, induces cell surface expression of CD13 and CD38 and apoptosis in HL-60 cells.

We previously demonstrated that the mitochondrial NADH dehydrogenase subunit 2 (ND2) gene was overexpressed in human acute myelogenous leukemia (AML) cells. Since this finding suggested that ND2 gene expression was related to myeloid differentiation, we here investigated the effects of rotenone, a specific NADH dehydrogenase inhibitor, on HL-60 cell growth, differentiation and death. Fifty nM rotenone inhibited the growth of HL-60 cells and caused an increase in the cell population in the G(2) +M phase. In the quantitative comparison of myeloid antigen, the expression of CD13 and CD38 were relatively increased in the rotenone-treated cells. These findings suggest that the inhibition of NADH dehydrogenase changes the cell cycle and induces some specific surface antigens of HL-60 cells. On the other hand, the expression of ND2 gene remained unchanged after the rotenone treatment, suggesting the rotenone-mediated mitochondrial inhibition did not affect the mitochondrial gene expression. Five mu M rotenone strongly inhibited the cellular proliferation. Electron microscopy and an electrophoretic analysis of DNA showed that the majority of the HL-60 cells were induced into typical apoptosis within 24-48 hours. On the basis of this and other studies, we believe that mitochondrial function is directly involved in both cellular differentiation and apoptotic cell death.

ADP-ribosyl Cyclase↗

PubMed1996

Maladaptation of vascular response in frontal area of patients with orthostatic hypotension.

UNLABELLED: To clarify the relationship between abnormalities in cerebral blood flow (CBF) and in blood pressure regulation, we performed 99mTc-HMPAO brain SPECT in association with measurement of plasma norepinephrine and blood pressure in both the upright and supine positions. METHODS: We studied six patients with orthostatic hypotension and six normal patients. No patient had any significant stenosis of the carotid arteries on echocardiography-Doppler ultrasound and no abnormalities on brain MRI or CT. Changes in systolic blood pressure were monitored during the upright test, and 8-ml blood samples were taken at baseline and at 1 and 5 min after the upright position. RESULTS: Systolic blood pressure decreased by a mean of 38 +/- 10 and 9 +/- 3 mmHg in the orthostatic hypotension and normal groups, respectively. Compared with baseline values, plasma norepinephrine levels at 1 and 5 min after the upright test did not increase in the orthostatic hypotension group but did increase significantly in the normal group. In the orthostatic hypotension group, 99mTc-HMPAO brain SPECT showed postural cerebral hypoperfusion in the bilateral frontal areas, of which the mean count ratio of the frontal-to-cerebellar area between the upright and supine positions significantly changed from 0.871 +/- 0.029 to 0.942 +/- 0.067 and from 0.881 +/- 0.035 to 0.954 +/- 0.073 in the right and left areas, respectively. No postural change in CBF was evident in other areas in the orthostatic hypotension group. In the normal group, there were no such changes in CBF, blood pressure and plasma norepinephrine levels during the upright test. CONCLUSION: Postural cerebral hypoperfusion in the frontal areas in the orthostatic hypotension group might relate to maladaptation of the vascular response during the upright test.

Blood Pressure↗

PubMed1996

[Evaluation of remote effect to supratentorial areas in cerebellar infarction: a semiquantitative analysis using 99mTc-HM-PAO SPECT].

We studied 18 patients with unilateral cerebellar infarction to investigate its remote effect to the supratentorial areas with 99mTc-HM-PAO SPECT. Asymmetry indexes (AI) were calculated in the cerebellum and in seven supratentorial areas (thalamus, basal ganglia, superior and inferior frontal areas, temporal area, parietal area and occipital area). Mean AIs of supratentorial areas ranged from -0.64 +/- 2.74% to +1.04 +/- 2.03%. In cerebellar infarction of acute or subacute phase, the mean AI showed larger values (+1.13%) than that of chronic phase (+0.21%), but this difference did not reach statistical significance. There was no significant difference among supratentorial AIs grouped according to cerebellar infarction size. There was also no significant correlation among AIs of cerebellar and the supratentorial areas (r = -0.32 to +0.38). Crossed hemispheric hypoperfusion is thought to be related to the interruption of cerebellocortical tracts. We concluded that the detection of the remote effect to supratentorial areas in cerebellar infarction was difficult with 99mTc-HM-PAO SPECT.

Adult↗

PubMed1996

[Usefulness of 123I-BMIPP scanning for distinction of ischemic from nonischemic dilated cardiomyopathy].

To determine if imaging of blood flow (using 201Tl) and fatty acid (using 123-I-BMIPP) with SPECT can distinguish cardiomyopathy of coronary artery disease from nonischemic dilated cardiomyopathy, 24 patients with severe left ventricular dysfunction were evaluated. The origin of left ventricular dysfunction had been previously determined by coronary angiography to be ischemic (9 patients) or nonischemic (15 patients). Images were visually analyzed by three observers on a graded scale (score 0; normal, 1; mild uptake reduction, 2; severe uptake reduction, and 3; defect) in 20 left ventricular segments revealed higher defect score in ICM compared with NCM for 123I-BMIPP (35.5 +/- 14.4 versus 14.1 +/- 9.3, p <0.0005) and 201Tl (27.6 +/- 14.6 versus 12.1 +/- 7.4, p <0.005). The defect score/segment ratio also revealed higher value in ICM compared with NCM for 123I-BMIPP (2.25 +/- 0.52 versus 1.36 +/- 0.36, p <0.0001) and 201Tl (1.92 +/- 0.51 versus 1.24 +/- 0.42, p <0.005). Myocardium of ICM is more severely damaged than that of NCM. Thus, noninvasive SPECT imaging with 123I-BMIPP is helpful in distinguishing patients with severe left ventricular dysfunction secondary to coronary artery disease from those with nonischemic cardiomyopathy.

Adult↗

PubMed1996

[Significance of 123I-metaiodobenzylguanidine SPECT for detecting left ventricular involvement in patients with arrhythmogenic right ventricular dysplasia].

The right ventricle being primarily involved in ARVD, recent reports indicate the presence of histological and functional abnormalities in the left ventricle for some patients with ARVD. The aim of this study was to evaluate the significance of myocardial sympathetic dysfunction as an early sign of left ventricular (LV) involvement by 123I-MIBG (MIBG) SPECT and to compare the findings with those of 201TlCl (Tl) SPECT, radionuclide left ventriculography, ultrafast computed tomography (UFCT), magnetic resonance imaging (MRI) and echo-cardiography in 10 patients (pts) with ARVD. MIBG defects in LV regions were detected in 9 pts. Seven of the 9 pts showed MIBG defects in LV regions adjacent to RV. The subjects were divided into 2 groups based on left ventriculography, 5 with normal LVEF (> 55%) and 5 with reduced LVEF. In the normal LVEF group, 4 pts showed MIBG defects and 2 pts showed TI defects, and MIBG defects were larger than TI defects (ES: 14 +/- 6 vs. 5 +/- 7, p <0.05). In reduced LVEF group, all of 5 pts showed MIBG and TI defects, and MIBG defects were larger than TI defects (ES: 42 +/- 12 vs. 25 +/- 3, p <0.05). In comparison with normal LVEF group, reduced LVEF group showed larger and more severe MIBG defects (ES: 42 +/- 12 vs. 14 +/- 6, p <0.01, SS: 44 +/- 31 vs. 8 +/- 7, p <0.05). UFCT and MRI showed abnormal findings indicating LV fatty infiltration in only 3 of reduced LVEF group. Thus, MIBG showed abnormal distributions in the left ventricle with the highest frequency in all these modalities. These results suggest that MIBG SPECT provides a sensitive marker for detecting LV involvement in ARVD. Also, the extent of MIBG distribution abnormalities is helpful in assessing the severity of left ventricular involvement in patients with ARVD.

3-Iodobenzylguanidine↗

PubMed1996

Cerebral hypoperfusion in orthostatic hypotension with globally denervated myocardium.

A 57-yr-old woman had frequent syncope when rising from a seated position. Her blood pressure fell from 140/80 mmHg to 60-70/40 mmHg while changing positions. Iodine-123-metaiodobenzylguanidine ([123I]MIBG) did not accumulate in the heart, whereas 201Tl-Cl (201Tl) did. Raise-up 99mTc-hexamethyl-propyleneamine oxime (99mTc-HMPAO) brain SPECT revealed decreased activity in the bilateral frontal areas, and subsequent supine 99mTc-HMPAO brain SPECT revealed filling in these areas, indicating that the cerebral blood flow (CBF) was transiently decreased in the frontal areas more than others in a standing position. The plasma norepinephrine (NE) level of this patient was normal during supine rest, but when she stood up, failure to increase the plasma level of NE uncovered a sympathetic nervous dysfunction. The CBF abnormality in patients with orthostatic hypotension may be due to a "functional" hemodynamic mechanism that induces orthostatic stress. This patient had transient hypoperfusion in the frontal areas when standing, without organic cerebral arterial stenosis. Only CBF in the frontal areas revealed relative hypoperfusion. These regions might be highly susceptible to a change in blood flow. The causes of orthostatic hypotension of this patient were autonomic failure with a disturbance of the sympathetic nerve endings, which was revealed by 99mTc-HMPAO brain SPECT and cardiac [123l]MIBG imaging.

3-Iodobenzylguanidine↗

PubMed1995

HLA DRB4 0101-restricted immunodominant T cell autoepitope of pyruvate dehydrogenase complex in primary biliary cirrhosis: evidence of molecular mimicry in human autoimmune diseases.

We established six T cell clones specific for pyruvate dehydrogenase complex (PDC)-E2 peptides from four different patients with primary biliary cirrhosis using 33 different peptides of 17-20 amino acid residues corresponding to human PDC-E2 as stimulating antigens. The minimal T cell epitopes of these six T cell clones were all mapped to the same region of the PDC-E2 peptide 163-176 (GDLLAEIETDKATI), which corresponds to the inner lipoyl domain of PDC-E2. The HLA restriction molecules for this epitope were all identified as HLA DRB4 0101. The common essential amino acids of this epitope for these T cell clones were E, D, and K at positions 170, 172, and 173, respectively; other crucial amino acids for this epitope differed in each T cell clone. In addition, the alanine-substituted peptides at positions 170 and 173, but not 172, inhibited the proliferation of all T cell clones induced by the original peptide of human PDC-E2 163-176, indicating that amino acid D at position 172 is a critical MHC-binding site for all T cell clones tested. Interestingly, all T cell clones reacted to PDC-E2 peptide 36-49 (GDLIAEVETDKATV), which corresponds to the outer lipoyl domain of human PDC-E2. Furthermore, one T cell clone cross-reacted with exogenous antigens such as Escherichia coli PDC-E2 peptide 31-44/134-147/235-248 (EQSLITVEGDKASM), which has an EXDK sequence. This is a definite demonstration of the presence of molecular mimicry at the T cell clonal level in human autoimmune diseases. It is also considered possible to design peptide-specific immunotherapy based on the findings of T cell autoepitopes in primary biliary cirrhosis.

Adult↗

PubMed1995

Stability and pharmacokinetic studies of a new immunosuppressant, mycophenolate mofetil (RS-61443), in rats.

Mycophenolate mofetil (MPM), a new immunosuppressant, is a morpholinoethyl ester of mycophenolic acid (MPA). The enzymatic and non-enzymatic hydrolysis was studied in an artificial digestive fluid, rat plasma, and tissue homogenates. MPM was chemically stable in the artificial digestive fluid. In rat tissue homogenates and plasma, MPM was rapidly hydrolysed to MPA. The conversion rate of MPM to MPA in various rat tissue homogenates was in the order of liver > kidney > plasma > small-intestine epithelial cells. After the intravenous injection of MPM at 16.7 mg kg-1, the terminal elimination half-life, t1/2 beta, was 4.74 +/- 0.33 (mean +/- SD)h, and the area under the plasma concentration versus time curve, AUC, was 48.78 +/- 6.01 micrograms h mL-1. After intraduodenal (ID) administration of MPM at 16.7 mg kg-1, t1/2 beta was 3.92 +/- 1.05 h, and the AUC was 38.08 +/- 8.30 micrograms h mL-1. The systemic availability of MPA after ID MPM dosing was 1.52 times higher than that after ID administration of MPA. This result supports the usefulness of MPM as an oral prodrug of MPA as a new oral immunosuppressant.

Animals↗

PubMed1995

Establishment and structural analysis of human mAb to the E2 component of the 2-oxoglutarate dehydrogenase complex generated from a patient with primary biliary cirrhosis.

We established one Epstein-Barr virus-transformed B cell hybrid clone producing human mAb of the IgG class to the 2-oxoglutarate dehydrogenase complex (OGDC) for the first time from the peripheral B lymphocytes of a patient with primary biliary cirrhosis (PBC). This mAb, designated mAbM37GO37, specifically bound to OGDC and its dissociation constant with OGDC was calculated to be 3.70 x 10(-10) mol/l. mAbM37GO37 stained murine stomach/kidney cryostat sections in a typical immunofluorescence pattern of antimitochondrial antibody (AMA). Western blotting analysis revealed that mAbM37GO37 reacted with an E2 component of OGDC but not with other components of OGDC nor pyruvate dehydrogenase complex (PDC). Furthermore, mAbM37GO37 completely inhibited the enzymatic activity of OGDC. In order to determine the structure and genetic origin of anti-OGDC autoantibody, we cloned and sequenced the Ig heavy and light chain variable regions of mAbM37GO37. This mAb used the VHIII family member, V3-7, and the V kappa IV family member. The amino acid difference between the expressed V genes of this mAb and respective putative germline genes was concentrated within the complementarity determining regions (CDR) rather than the framework regions (FR). The R:S mutation ratio was high in the CDR and low in the FR. These features suggested that the immune response to OGDC is similar to that to exogenous antigen, and that the heavy and light chain variable regions of the anti-OGDC antibody undergo somatic hypermutation through antigen-driven clonal selection. This human mAb to OGDC, which was established for the first time from a patient with PBC and characterized at the molecular level, would be a valuable tool to study the B cell autoepitopes of OGDC, to clone as yet undetermined full length cDNA encoding OGDC and to dissect the autoimmune response to mitochondrial antigens in PBC.

Adult↗

PubMed1995

I-123 iodoamphetamine lung scanning in patients with ventilation-perfusion mismatching.

I-123 IMP is a nonparticulate agent and becomes trapped by endothelial membranes in the pulmonary capillaries. Using I-123 IMP, the authors studied six patients with ventilation-perfusion mismatch. Three of six patients had pulmonary thromboembolism, and three had pulmonary hypertension. In comparison with conventional perfusion lung scanning using Tc-99m MAA, defect sizes visualized by I-123 IMP were smaller in all patients. I-123 iodoamphetamine is a useful agent for assessing the perfusion of pulmonary arterial microvasculature which Tc-99m MAA fails to penetrate.

Amphetamines↗

PubMed1995

Inferior vena cava occlusion with pulmonary embolism because of complications due to ruptured abdominal aneurysm demonstrated by radionuclide venography.

A 66-year-old man with an abdominal aortic aneurysm confirmed by CT had bilateral swelling of the lower extremities with pain radiating to the back. Radionuclide venography and pulmonary scintigraphy demonstrated occlusion of the inferior vena cava and multiple pulmonary emboli, with a hot spot in the liver. Surgery revealed a ruptured abdominal aortic aneurysm that occluded the inferior vena cava, fistula formation, and extensive thrombosis of the inferior vena cava proximal to the occlusion site. Radionuclide venography was useful in detecting venous obstruction and the collateral formation represented by the hot spot in the liver as complications of the ruptured abdominal aortic aneurysm, and in assessing the improvement of pulmonary embolism by medical therapy.

Aged↗

PubMed1995

Usefulness of dipyridamole-thallium imaging in 257 patients with atherosclerotic vascular disease.

We evaluated the usefulness of dipyridamole-thallium imaging for the detection of ischaemic heart disease in 257 patients with atherosclerotic vascular disease (80 patients with arteriosclerosis obliterans, 81 patients with aneurysm of the abdominal aorta, 60 patients with aneurysm of the thoracic aorta and 36 patients with dissecting aortic aneurysm). Clinical evidence of ischaemic heart disease was found in 69 of 257 (27%) patients, including 32 patients with arteriosclerosis obliterans, 23 with aneurysm of the abdominal aorta, 9 with aneurysm of the thoracic aorta and 5 with dissecting aortic aneurysm. Dipyridamole-thallium imaging identified myocardial ischaemia in 49 of 69 (71%) patients with clinical evidence of ischaemic heart disease. Dipyridamole-thallium imaging showed positive results in 67 of 81 (83%) patients with aneurysm of the abdominal aorta. In patients with no clinical evidence of ischaemic heart disease, the results of dipyridamole-thallium imaging were positive in 39 of 188 (21%) patients. Dipyridamole-thallium imaging was positive in 90 of the 257 (35%) patients as a whole. When we combined the patients with positive dipyridamole-thallium imaging with those with negative dipyridamole-thallium imaging but who had clinical evidence of ischaemic heart disease, 42% of all patients had evidence of ischaemic heart disease. Our findings suggest that atherosclerotic vascular disease is strongly associated with ischaemic heart disease and that dipyridamole-thallium imaging is useful for the detection of ischaemic heart disease.

Aged↗

PubMed1995

Scintigraphic assessment of silent myocardial ischaemia after early infarction using myocardial SPET imaging with 201Tl and 123I-MIBG.

To test the hypothesis that myocardial sympathetic denervation reflects silent myocardial ischaemia early after infarction, 12 patients with myocardial infarction but without post-infarction angina pectoris underwent single photon emission tomography (SPET) at rest with 201Tl and 123I-metaiodobenzylguanidine (MIBG) shortly after and 3 months after infarction. Short-axis SPET images at the basal, mid-ventricular and apical portions of the left ventricle were selected, and each short-axis image was divided into eight segments. Tracer uptake in each of the 24 segments was scored using a 4-point scale. The total score in each segment was calculated as the defect score for each image, and the difference between the total defect score for the 201Tl and 123I-MIBG images was calculated as the delta defect score. All 12 patients underwent exercise stress 201Tl scintigraphy 1 month after infarction, and they were divided into two groups: those patients with (Group A, n = 7) and those patients without (Group B, n = 5) transient perfusion defects in the peri-infarcted region without chest pain. For the 123I-MIBG defect score, a marked reduction at 3 months was observed in Group A (24 +/- 12 vs 13 +/- 6; P < 0.01), whereas the defect score remained unchanged in Group B (25 +/- 7 vs 23 +/- 8; N.S.). The delta defect score was significantly reduced in Group A (10 +/- 5 vs 6 +/- 4; P < 0.05), whereas it remained unchanged in Group B. The 123I-MIBG defect score early after infarction was higher than the exercise-induced 201Tl defect score (24 +/- 12 vs 20 +/- 9; P < 0.01), whereas at 3 months post-infarction it was lower than the exercise-induced 201Tl defect score (13 +/- 6 vs 20 +/- 9; P < 0.05). Moreover, effort chest pain during daily activities was noted in 5 of the 7 (71%) patients in Group A within 3 months post-infarction. The results of this study suggest that viable but denervated myocardium (mismatched 123I-MIBG defects) is present in peri-infarcted regions, and that myocardial sensory nervous disturbance, which may co-exist with sympathetic nervous denervation, may induce silent myocardial ischaemia in patients with myocardial infarction.

3-Iodobenzylguanidine↗

PubMed1995

Perfusion lung scanning before and after percutaneous transvenous mitral commissurotomy--early estimation of lung congestion relief.

Percutaneous transvenous mitral commissurotomy (PTMC) has recently been used to treat mitral stenosis. The aim of this study was to evaluate the usefulness of radionuclide perfusion lung scanning in assessing the effect of PTMC on the relief of lung congestion. We studied 30 patients (7 males and 23 females, mean age 55 years). Perfusion lung scannings were performed within 1 week before and after PTMC. We calculated the ratio of activity in the upper quarter to that in the lower quarter of the right lung (U/L) as an index of lung congestion. After PTMC, the mean mitral valve area increased from 1.1 +/- 0.3 to 1.9 +/- 0.4 cm2, the mean left atrial pressure decreased from 14.8 +/- 6.3 to 9.1 +/- 3.5 mmHg, the mean pulmonary artery pressure decreased from 22.7 +/- 8.6 to 17.4 +/- 6.3 mmHg, and the U/L ratio decreased significantly from 0.89 +/- 0.40 to 0.68 +/- 0.24 (p < 0.0001). The U/L ratio showed greater improvement (4.5%) in patients whose NYHA class improved (n = 19) than in those whose NYHA class did not improve after PTMC. The U/L ratio was closely related to mitral valve area, and left atrial and pulmonary artery pressures. The change in the U/L ration before and after PTMC also reflected symptomatic improvement. In conclusion, U/L ratios obtained from perfusion lung scannings before and after PTMC reflect mitral valve area and pressures, and can be used to assess lung congestion relief after PTMC.

Adult↗

PubMed1995

[A case of hemophagocytic syndrome manifesting adult Still's disease and acute hepatitis].

We report a 20 year-old woman with hemophagocytic syndrome. In February 1993, she developed high fever, arthralgia, salmon-like pink eruption, leukocytosis and splenomegaly. She was diagnosed as adult Still's disease and successfully treated with intravenous immunoglobulin and oral prednisolone. In September 1993, she was re-admitted to our hospital complaining of general fatigue and low grade fever and treated with oral prednisolone at a daily dose of 15 mg. On October 2, 1993, she suddenly developed high fever and salmon-like pink eruption on her leg followed by the marked increase of serum transaminase and LDH levels (GOT 3,270 IU/l, GPT 1,880 IU/l, LDH 5,480 IU/l) on October 7. Since hepatic failure progressed, we started methylprednisolone pulse therapy and plasmapheresis. However, because of the progression of pancytopenia caused by hemophagocytosis, the treatment with VP-16 was initiated. However, she died of DIC on November 2, 1993. Autopsy revealed submassive necrosis of the hepatocytes with moderate infiltration of histiocytes. She was retrospectively diagnosed as hemophagocytic syndrome whose manifestations are very similar to those in adult Still's disease and acute viral hepatitis.

Acute Disease↗

PubMed1995

Peripheral B lymphocyte repertoire to mitochondrial antigen in primary biliary cirrhosis--positive correlation between the disease activity and the frequency of circulating B lymphocytes specific for pyruvate dehydrogenase complex.

B lymphocytes committed to the production of IgG antibodies (Abs) to mitochondrial antigen such as pyruvate dehydrogenase complex(PDC) were quantitated in the peripheral blood of patients with primary biliary cirrhosis(PBC) using Epstein-Barr virus as a polyclonal activator of human B lymphocytes. B lymphocytes committed to the production of IgG Abs to PDC were found in high frequency in patients with PBC(0.54 +/- 0.16%, mean value +/- SE, of total IgG-producing B lymphocytes) in contrast to type C chronic hepatitis and healthy subjects in which they were less than 0.01%. The frequency of these B lymphocytes specific for PDC increased in parallel to the progression of the Scheuer's stage from I to II (stage I: 0.35 +/- 0.23%, stage II: 1.04 +/- 0.32%), but decreased with further progression to stage IV (stage III: 0.39 +/- 0.21%, stage IV: 0.07 +/- 0.06%). In addition, B lymphocytes specific for PDC decreased in the peripheral blood during the administration of cyclosporin A; this was accompanied by an improvement of lymphocyte infiltration severity in the liver. These data indicate that B lymphocytes specific for PDC are present in the peripheral blood of patients with PBC and their frequency reflects the degree of the lymphocyte infiltration in the liver.

Adult↗