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Biomedical subjects

G Vaiopoulos

Publications and source records attributed to G Vaiopoulos.

At least 55 records · Page 3Linked to original sources

Arterial calcifications in beta-thalassemia.

The purpose of this study was to define the incidence of arterial calcifications in patients with beta-thalassemia. Beta-thalassemia patients have been shown to present a high prevalence of angioid streaks and skin lesions characteristic of pseudoxanthoma elasticum (PXE). Given the fact that vascular involvement in the form of arterial calcifications is also a common manifestation of PXE, the authors investigated radiographically the presence of arterial calcifications in beta-thalassemia patients. They studied 40 patients with beta-thalassemia over 30 years of age. Forty healthy, age- and sex-matched subjects were chosen as a control group. Radiographs of the tibias were performed in order to disclose arterial calcifications. The occurrence of PXE skin lesions and of angioid streaks (AS) was also investigated. Arterial calcifications were detected in the posterior tibial artery in 22 (55%) beta-thalassemia patients and in six (15%) controls (P < 0.01 for the comparison). PXE skin lesions and AS were found in eight (20%) and 21 (52%) patients respectively. A total of 34 patients (85%) had at least one of the three lesions, namely, arterial calcifications, angioid streaks, and/or PXE-like skin lesions. Stepwise logistic regression analysis did not reveal prognostic value in independent variables such as transfusions, chelation therapy, pseudoxanthoma elasticum skin lesions and/or angioid streaks, diabetes, hemoglobin, serum ferritin, and uric acid. It was concluded that arterial calcifications are common in older beta-thalassemia patients. This finding could be a manifestation of an acquired PXE syndrome associated with beta-thalassemia, and consequently, vascular events complicating PXE should be expected in these patients.

Adult↗

Hemoglobin levels correlate with serum soluble CD23 and TNF-Rs concentrations in patients with rheumatoid arthritis.

The present study was designed to investigate the possible relationships of hemoglobin concentrations with serum levels of soluble CD23 molecules (sCD23) and soluble tumor necrosis factor receptors I and II (sTNF-RI and sTNF-RII) in rheumatoid arthritis (RA) patients. Fifty-six patients, eight males and 48 females, and 20 age and sex matched healthy volunteers were enrolled in the study. Patients were classified in two groups on the basis of disease activity: group A included 43 patients with active, and group B 13 patients with non-active RA. Serum sCD23 and sTNF-Rs levels were measured using commercially available micro-ELISA kits. It was found that patients of group A had lower hemoglobin concentrations than patients of group B or normal controls, whereas hemoglobin levels in patients of group B did not differ statistically from the controls. Patients of group A had also significantly higher serum sCD23, sTNF-RI and sTNF-RII concentrations than patients of group B or control subjects. Serum levels of all three cytokines did not differ statistically between patients of group B and normal controls. In the entire group of subjects studied, hemoglobin concentrations correlated inversely with the levels of serum sCD23, sTNF-RI and sTNF-RII, and also with the values of the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) reflecting disease activity. We concluded that anemia and elevated concentrations of sCD23, sTNF-RI and sTNF-RII in RA patients are two biological expressions of the same underlying inflammatory process, although a causal relationship between themselves cannot be excluded and needs further investigation.

Antigens, CD↗

Systemic lupus erythematosus presenting as myelofibrosis.

Myelofibrosis is not frequent in systemic lupus erythematosus (SLE). A review in the literature reveals that the co-incidence is rather rare since there are only a few papers reporting this combination. The female patient described hereby, presented with thrombocytopenia; following investigation, the diagnosis of SLE was established and bone marrow examination revealed an increase of marrow reticulin. Treatment with steroids reversed both thrombocytopenia and bone marrow fibrosis.

Adult↗

Tuberculosis of the sacroiliac joint.

A 23-year-old woman presented with low back pain of several months' duration. A tuberculous infection of the left sacroiliac joint was diagnosed by closed-needle biopsy. The clinical presentation, radiological features and outcome of this patient are discussed.

Adult↗

Trisomy 8 in a patient who responded to therapy with all-trans-retinoic acid and developed paroxysmal nocturnal haemoglobinuria.

Trisomy 8 is the most common numerical chromosomal abnormality in myelodysplastic syndromes (MDS). Paroxysmal nocturnal haemoglobinuria (PNH) is an aquired haemolytic anaemia, clonal in nature, due to somatic mutation. PNH may evolve to aplastic anaemia, to MDS or to acute myeloid leukaemia. We present a patient who had trisomy 8 mosaicism at disease presentation who received therapy with all-trans-retinoic acid, responded to therapy, and developed PNH in the course of the disease. Cytogenetics at the time of PNH diagnosis showed a normal karyotype.

Adolescent↗

Mixed cryoglobulinemia in Greece. Primary disorders in 10 cases.

Mixed cryoglobulinemia (MC) is a systemic disorder whose pathogenesis is based on the presence of serum cryoglobulins. The purpose of this study was to evaluate on a prospective basis patients presenting with MC with regard to the clinical manifestations and the underlying disorders. We present ten patients with MC, who were diagnosed and followed up during a one year period in the Division of autoimmune rheumatic diseases (Clinical Department of Pathophysiology). MC was associated with hepatitis C virus (HCV) infection in two cases, with hepatitis B virus (HBV) infection in six, one patient had both HCV and evidence of HBV infection, while the remaining three patients fulfilled European classification criteria for diagnosis of Sjögren's syndrome (SS). In all ten cases, the presence of an underlying factor was identified, being either viral or autoimmune. It is concluded therefore that all patients presenting with MC should be completely evaluated for a hepatitis virus infection or an autoimmune or lymphoproliferative disorder. Furthermore, since the initiation of the process of MC is triggered by many factors, research should be directed towards the identification of the underlying common denominator.

Adult↗

Family history as a risk factor for rheumatoid arthritis: a case-control study.

OBJECTIVE: To investigate the risk of rheumatoid arthritis (RA) in the first degree relatives and to investigate whether the sex of the parent influences the pattern of inheritance. METHODS: An interview based case-control study, with subjects serially matched for age and sex. We analyzed 126 cases (hospital cases) and 94 controls (derived from the same hospitals), who gave information for family history of RA. Data concerning RA history among siblings and parents were computerized and analyzed univariately and multivariately. RESULTS: The odds ratio (OR) for developing RA is 4.4 (p < 0.001) if a first degree relative reported having the disease and 5.4 (p < 0.01) if a female first degree relative reported having the disease. For females the OR is 7.0 (p < 0.01) if the first degree relative is female. When the analysis was restricted to parents only, it was found that mothers with RA predispose their daughters and sons to develop RA more (OR = 8.6, p < 0.01, for daughters and 4.8, p < 0.05, for both sexes) than fathers (OR = 1.1 and 1.9, respectively). CONCLUSION: This case-control study confirms the familial clustering of RA and suggests that mothers confer susceptibility to RA on their offspring more often than fathers.

Adult↗

Downregulation of interleukin-2 and apha-chain interleukin-2 receptor biosynthesis by cisplatin in human peripheral lymphocytes.

To further investigate the mechanisms by which the antineoplastic agent cisplatin interferes with immune function, we studied its effect on the biosynthesis of interleukin-2 (IL-2) and its alpha-chain receptor (IL-2Ralpha). Normal human peripheral blood lymphocytes (PBL) were activated in vitro with phytohemagglutinin (PHA), and anti-CD3 antibody in the presence of various concentrations of cisplatin. Purified T cells were also cultured with anti-CD3 antibody and costimulated by CD80 (B7-1, B7/BB1)-transfected P815 mastocytoma cells in the presence of cisplatin. Tritiated thymidine incorporation assays, an enzyme-linked immunosorbent assay for soluble IL-2Ralpha determination, and RNA dot-blot analysis and hybridization with IL-2- and IL-2Ralpha-specific probes were used. PHA-induced and anti-CD3 antibody-induced proliferation of PBL were significantly inhibited by cisplatin at concentrations attainable in vivo. This inhibition was not due to direct cell death as shown by the absence of trypan blue uptake in the presence of high concentrations of cisplatin. Therapeutic concentrations of cisplatin (1 microgram/ml) also inhibited the IL-2-dependent proliferation of purified T cells, mediated via the CD28-CD80 costimulatory pathway. In addition, the amount of soluble IL-2Ralpha released in the T cell culture supernatants was decreased by cisplatin in a dose-dependent fashion, suggesting that inhibition of cell proliferation was associated with a parallel decrease in IL-2Ralpha production. These effects correlated with a specific cisplatin-induced downregulation of both IL-2 and IL-2Ralpha messenger RNA accumulation in PHA-stimulated PBL that was dependent on the concentration of the drug. These findings suggest that the immunomodulatory effects of cisplatin may result in part from its capacity to directly downregulate the IL-2/IL-2R system in activated lymphocytes.

Antineoplastic Agents↗

Adamantiadis-Behçet's disease in sisters.

Two sisters with documented Adamantiadis-Behçet's disease of different severity, associated with HLA-B51, are described. Although the overall incidence of this complex disease is not different between sexes, only 4 female siblings, versus 14 male, that fulfill the 1990's International Study Group for Behçet's Disease criteria have been reported in the literature.

Behcet Syndrome↗

Development of plasma cell tumors during treatment of multiple myeloma.

Plasma cell tumors (plasmacytomas-PCT) of the bone, or extramedullary PCT, may be diagnosed in patients with or without the diagnostic criteria for systemic multiple myeloma (MM). The reason for the local development of these tumors is not clear. Recent reports emphasize the contribution of CT and MRI in the detection of bone lesions and their expansion into the soft tissues. We report the development of PCT in nine patients with MM under maintenance treatment with alpha-IFN, of whom six had no evidence of systemic relapse and three had indications of early relapse. The PCT were located in the pelvis (4), thoracic (3), cervical (1), and lumbar (2) spine and in 8/9 cases were not demonstrable on plain X-rays. These observations suggest that frequent screening with advanced imaging techniques may detect local disease expansion in asymptomatic patients. Early application of radiochemotherapy may improve prognosis.

Adult↗

Retinal toxicity in long term hydroxychloroquine treatment.

OBJECTIVE: To report clinical experience from patients with rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) who were receiving recommended doses of hydroxychloroquine for more than six years, and were monitored for evidence of hydroxychloroquine related retinopathy every six months. METHODS: A prospective (and continuing) evaluation was made of the potential retinal toxicity of hydroxychloroquine in a cohort of 360 Greek patients followed for RA and SLE, 58 of whom have received long term treatment ( > six years). Fundoscopy, colour vision tests, dark adaptation tests, visual field testing, automated perimetry, and electroretinogram were performed every six months. RESULTS: Among 58 patients receiving hydroxychloroquine for more than six years, two relatively young women (3.5%), one treated for RA and the other treated for SLE, developed characteristic hydroxychloroquine related toxic retinal lesions after cumulative doses of 700 g (6.5 years) and 730 g (8 years) of hydroxychloroquine, respectively. Bilateral visual acuity was 6/6 and 6/7.5, respectively; both patients had normal colour perception. Despite an early diagnosis and cessation of treatment, permanent visual field paracentral scotomata in both patients, and persisting lesions in fluorescein angiography in the patient with SLE, were observed at 4.5 and 3 years of follow up, respectively. No other specific cases of hydroxychloroquine related retinopathy have to date been identified in the remaining 302 patients. CONCLUSION: Cases of irreversible, hydroxychloroquine related retinopathy in patients who did not receive overdoses have not been reported previously. The present observations in two relatively young patients should raise our concern regarding the long term usage of an increasingly popular medication in rheumatology practice.

Adult↗

Primary Sjögren's syndrome complicated by hemolytic uremic syndrome.

We describe a case of primary Sjögren's syndrome complicated by hemolytic uremic syndrome (HUS), possibly the first report of this association. The immunohistochemical findings suggest that immune complexes may have contributed to the endothelial injury and the pathogenesis of HUS in this case.

Female↗

Correction of anaemia and thrombocytopenia in a case of adult type I osteopetrosis with recombinant human erythropoietin (rHuEPO).

A case of adult osteopetrosis Type I was diagnosed in a 22-year-old female. She presented for investigation of anaemia with 'myelophthisic' characteristics and extramedullary haemopoiesis which was resistant to haematinics, nandrolone and low-dose corticosteroids. She became progressively transfusion-dependent with gradually worsening thrombocytopenia. She was successfully treated with recombinant erythropoietin. Anaemia as well as thrombocytopenia were corrected. There appeared to be a synergistic action of erythropoietin with steroids.

Adult↗

Histological and functional changes in the salivary glands in thalassaemia major.

Oral salivary secretion and oral histology in 15 multi-transfused adult thalassaemia homozygotes was studied. Iron stores of minor salivary glands were also histochemically determined. Salivary secretion in multi-transfused thalassaemia patients was subclinically decreased, but no clear correlation was evident between salivary secretion impairment and salivary gland destruction by iron deposition, lymphocyte infiltration and fibrosis. The underlying mechanism remains to be determined.

Adolescent↗