AA amyloidosis and pyrin gene mutations.
Explore the source record for details and available documents.
Aged↗
Biomedical subjects
G Vaiopoulos
Publications and source records attributed to G Vaiopoulos.
Clinical features of juvenile Adamantiades-Behçet's disease in Greece.
Adamantiades-Behçet's disease (A-BD) is a chronic relapsing vasculitis of unknown etiology. This disease is relatively rare in children and only recently have series of patients been reported. The objective of this study was to describe the clinical features of the disease in juvenile patients, and to compare them with adult cases and with those juveniles reported in the literature.
Adolescent↗
Onset signs, clinical course, prognosis, treatment and outcome of adult patients with Adamantiades-Behçet's disease in Greece.
OBJECTIVES: To examine the onset signs, clinical course, prognosis, treatment and outcome of Adamantiades-Behçet's disease in adult Greek patients during a 10-year follow-up period, and to compare the results with those of other series reported. METHODS: The studied population consisted of 82 adult patients (54 male and 28 female) of Greek origin. Patients filled out a standard questionnaire and were followed up regularly. The findings during the follow-up of these patients were compared with those of other series. RESULTS: The most frequent onset sign was oral aphthae, both in our patients and in those of other published series. Eye involvement, joint involvement, genital ulcers and skin lesions followed in frequency. During a 10-year follow-up differences were observed between men and women, with arthritis being more frequent in females compared to males and with an overall more severe course in males. Eye involvement occurred earlier than neurological and vascular manifestations. A few differences were found in the clinical parameters in comparison to previously reported ethnic series. HLA-B51 positive patients presented an odds ratio of 9.5, the activity index was 5.17 +/- 2.56 and the mean severity score 5.96 +/- 2.32. Early treatment led to improvement of the disease. CONCLUSION: The pattern of Adamantiades-Behçet's disease in adult Greek patients provides major similarities when compared to patterns in various other national groups.
Adult↗
Late onset of long-lasting fever as a sole complication of treatment with anti-TNFalpha.
Explore the source record for details and available documents.
Aged↗
Defective mitogen-induced cellular cytotoxicity in myelodysplastic syndromes. Recovery after alpha-interferon administration.
Peripheral blood mitogen - induced cellular cytotoxicity (MICC) and natural killer- cell cytotoxicity (NKCC) were assessed in 25 patients with myelodysplastic syndromes (MDS). Both MICC and NKCC were examined under the same experimental conditions using the 18 hr chromium release assay, except that cultures for MICC were stimulated in vitro by the addition of phytohemagglutinin (PHA). Patients' MICC was found significantly reduced, in relation to controls (p less than 0.001), but significantly higher than patients' NKCC (p less than 0.001). Furthermore, patients CD3+ cells and CD4+ cells, as well as the CD4+/CD8+ ratio, were significantly decreased (p less than 0.01, p less than 0.001 and p less than 0.001, respectively), while CD8+ cells and CD16+ cells were within normal limits. No relationship was noted between patients' MICC and total lymphocyte count or any lymphocyte subpopulation. In eleven patients who were subsequently subjected to a-interferon (a-IFN) administration, MICC values were found within normal range one month after the cessation of alpha-IFN, while NKCC values were significantly increased (p less than 0.01), but they still remained below the lower limit of the control (p less than 0.001). Percentages of CD3+, CD4+ and CD8+ cells, as well as the CD4+/CD8+ ratio, did not change after alpha-IFN, but the absolute numbers of CD3+ cells and CD8+ cells were significantly reduced. A statistically significant rise was noted in CD16+ cells. Post- IFN rises in MICC did not correlate with lymphocyte subpopulations. The findings indicate that MDS patients display very low MICC, which can be restored by alpha-IFN administration. The cause of this disturbance and the mechanism of its restoration by alpha-IFN remain unclear.
Aged↗
Asplenia in systemic lupus erythematosus: a simple coincidence?
Two patients with asplenia and systemic lupus erythematosus are described. Both patients presented with unusual symptoms of the disease. The possible association of asplenia, presumably congenital, and systemic lupus erythematosus is discussed.
Antibodies, Antinuclear↗
Multiple myeloma associated with autoimmune hemolytic anemia.
A rare case of multiple myeloma associated with severe Coombs-positive hemolytic anemia is described. A 60-year-old woman was hospitalized for acute hemolysis due to an IgG warm autoantibody with pan-agglutinin specificity. Serum and urine electrophoresis revealed the presence of a monoclonal IgGk protein and a BJk protein, respectively. Bone marrow aspirates showed diffuse infiltration with plasma cells, and skeletal survey revealed lytic lesions in the skull and diffuse osteoporosis. Treatment with prednisone, and subsequently with melphalan, cyclophosphamide and vincristine resulted in hematological improvement within two weeks. A reduction of paraprotein below 50% of the initial levels was found after six months of therapy.
Anemia, Hemolytic, Autoimmune↗
Impaired release of natural killer cytotoxic factor(s) by peripheral blood lymphocytes in patients with chronic LGL-proliferative disease.
BACKGROUND: Chronic LGL-proliferative disease (LGL-PD) is a clonal expansion of cells with large granular lymphocyte (LGL) morphology. In most cases, proliferating cells express both suppressor/cytolytic T-cell and natural killer (NK) cell surface markers, but other cell phenotypes may be observed. LGL-PD lymphocytes have been found to lack or show very low natural killer cell activity (NKa). The aim of the present paper is to investigate the underlying mechanisms responsible for impaired NKa in a homogeneous group of five selected LGL-PD patients with a CD3+, CD8+, CD57+ cell phenotype. RESULTS: In all patients, the expanded cell population expressed very low NKa against K562 cell targets, but this increased significantly with recombinant human interleukin-2 (rhIL-2) and phytohemagglutinin (PHA) activation. Recombinant human alpha-interferon (rhIFN-alpha) had no significant effect on NKa. Cells displayed normal tumor cell binding capacity but failed to release sufficient amounts of functionally active natural killer cytotoxic factor(s) (NKCFs) upon interaction with the NK-sensitive K562 cells targets. However, they did release soluble cytolytic molecules against K562 cells upon activation with PHA. CONCLUSIONS: Our findings provide evidence that the defective NKa in LGL-PD patients with the aforementioned phenotype is probably due, at least in part, to the inability of expanded lymphocytes to release NKCFs upon interaction with NK-sensitive cell targets. Since recognition of target cells by patient lymphocytes is not disturbed and the cells are capable of producing NKCFs upon activation with PHA, it is probable that the cause of this abnormality is located at the level of the activation signal provided by the stimulatory target cells. Studies in subcellular level are certainly needed for a more precise determination of the underlying defect.
Adult↗
Asymptomatic pericarditis in Adamantiadis-Behçet's disease.
Pericardial involvement in Adamantiadis-Behçet's disease (ABD) is uncommon. A 14-year-old boy presented with fever of unknown origin, as the initial and only manifestation of his disease. Other features were leukopenia, abdominal lymphadenopathy and asymptomatic pericarditis, which is a very rare finding of the disease. A review of pericarditis is presented.
Adolescent↗
Concurrent development of thrombotic thrombocytopenic purpura and systemic lupus erythematosus in a male patient.
Explore the source record for details and available documents.
Adult↗
Plasma renin activity as a marker of renovascular injury in patients with rheumatoid arthritis.
OBJECTIVES: To assess whether plasma renin activity (PRA) in patients with rheumatoid arthritis (RA) and evidence of renal involvement (microhematuria) can serve as potential marker of renovascular injury. METHODS: PRA was measured at rest and following exercise. All nonsteroidal antiinflammatory drugs or other medications that might affect renin release were stopped at least ten days prior to PRA measurements. PRA was correlated with the number of dysmorphic erythrocytes present in the urine sediment as indicators of glomerular capillary injury (microhematuria). RESULTS: All patients with RA had a higher mean PRA than controls. Moreover, all patients with RA in whom microhematuria was present had a higher PRA than those without microhematuria. Simple and multiple regression analysis revealed a significant correlation between: a) PRA and rheumatoid factor levels; b) rheumatoid factor levels and the number of erythrocytes in the urine sediment; and c) PRA levels and the number of erythrocytes in the urine sediment. CONCLUSIONS: The observations indicate that increased PRA may occur in normotensive patients with RA and no clinical or biochemical evidence of renal involvement. This may reflect activation of the renin-angiotensin system. The positive correlation between enhanced PRA, rheumatoid factor levels and microhematuria in RA patients may indicate inflammatory injury of the glomerular microvasculature involving the juxtaglomerular apparatus.
Adult↗
Parathyroidectomy in the treatment of secondary hyperparathyroidism in chronic renal failure.
During the period 1983-1995, 200 chronic renal failure patients (115 males and 85 females) were parathyroidectomized for hyperparathyroidism in our Department. In all of them, the presenting clinical symptoms, physical signs, biochemical and radiological tests were typically those of hyperparathyroidism. One hundred ninety patients were operated for the first time whereas 10 were re-operated due to relapse of the disease; 3 of these cases were primary hyperparathyroidism, 182 secondary and 5 tertiary. All three primary hyperparathyroidism cases underwent removal of the adenoma; in the group of secondary hyperparathyroidism, 50 underwent removal of all the parathyroid glands found, 25 underwent total parathyroidectomy with forearm or deltoid autograft and 60 subtotal parathyroidectomy whereas in 39 and 8 patients only 3 and 2 parathyroid glands were found respectively. In the group of tertiary hyperparathyroidism, we removed only the hyperplastic gland detected as the operative detection of the rest was not possible. Ten cases were re-operated for removal of the remaining glands. No complications were noted postoperatively, apart from severe hypocalcemia in 20 cases, treated successfully by Calcium and Vitamin D administration. The highest relapse rate was noted among the 8 patients with only the 2 parathyroid glands removed. It seems that total or subtotal parathyroidectomy represents the most successful methods for surgical treatment of hyperparathyroidism complicating chronic renal failure.
Female↗