[Bone marrow aplasia and liver damage caused by methimazole].
We present a case of 52 years old woman with cutaneous lupus erythematosus and methimazole induced bone marrow aplasia with agranulocytosis and liver damage.
Anemia, Aplastic↗
Biomedical subjects
J Musiał
Publications and source records attributed to J Musiał.
[Three episodes of acute multiorgan failure in a woman with secondary antiphospholipid syndrome].
We report the case of a 43-year-old woman with systemic lupus erythematosus who survived three episodes of catastrophic antiphospholipid syndrome. During the first episode symptoms involved predominantly the central nervous system, whilst during the second episode of multiorgan failure, the cardiovascular system, lungs and kidneys were particularly affected. Twenty months later, the patient experienced an acute exacerbation of chronic renal failure and later, died of massive pulmonary embolism. The characteristic findings of antiphospholipid syndrome included persistently high titers of IgG anticardiolipin antibodies, positive lupus anticoagulant, and microcytic anaemia with a distinct haemolytic component.
Adult↗
[Sneddon syndrome--case report].
We report a case of Sneddon syndrome in a 39-year-old woman who developed recurrent cerebral ischaemic events associated with livedo racemosa. We describe clinical and radiological features of this rare vasculopathy and laboratory findings essential for differential diagnosis.
Adult↗
Aspirin delays thrombin generation in vitro through interaction with platelet phospholipids.
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Anti-Inflammatory Agents, Non-Steroidal↗
Thrombin generation markers and coronary heart disease risk factors in a Polish population sample.
Thrombosis plays a major role in the development of atherosclerosis and its acute vascular complications. Epidemiological studies have shown that elevated levels of plasma fibrinogen are associated with an increased risk of coronary heart disease (CHD). It is not clear whether this association is linked to hemostatic functions of fibrinogen which serves as a substrate for thrombin. Generation of thrombin in vivo can be evaluated by measurement of its specific markers in plasma, i.e. thrombin-antithrombin III complex (TAT) and prothrombin fragment 1 + 2 (F1 + 2). We determined plasma levels of TAT and F1 + 2 in a population sample of southeastern Poland and evaluated relations of these markers with plasma fibrinogen, factor VII coagulant activity (FVIIc), and other known CHD risk factors. The population studied consisted of 215 men and 251 women, aged 43-75 years. Final analysis was performed on 195 men and 222 women. The distribution of plasma TAT and F1 + 2 concentrations were highly skewed with the higher median values for women than for men. Log values of TAT correlated with log values of F1 + 2 in men (r = 0.27, p < 0.01) and in women (r = 0.15, p < 0.05). In the regression analysis both markers were positively related to age in women but not in men. After adjustment to age there was a positive relation between TAT and fibrinogen in both sexes. In women, but not in men, F1 + 2 showed a positive association with FVIIc. Total plasma cholesterol was negatively related to TAT in women only. There was no association between thrombin generation markers and plasma triglycerides, HDL-cholesterol, LDL-cholesterol, blood pressure, cigarette smoking and body mass index (BMI). The association of plasma fibrinogen and FVIIc with thrombin generation markers points to an important role of the hemostatic system in the pathogenesis of atherosclerosis and coronary heart disease in humans.
Adult↗
Thrombin generation measured ex vivo following microvascular injury is increased in SLE patients with antiphospholipid-protein antibodies.
Antiphospholipid-protein antibodies (APA) include lupus-type anticoagulant (LA) and antibodies recognizing complexes of anionic phospholipids (e.g. cardiolipin) and proteins (e.g. prothrombin and beta2-glycoprotein I). The presence of APA is associated with an increased risk of both arterial and venous thrombosis. However, the pathogenic mechanism leading to thrombosis in patients with APA remains unclear. We studied 32 patients with systemic lupus erythematosus (SLE) who were divided into two groups depending on the presence (n = 19) or absence (n = 13) of APA. Healthy volunteers (n = 12) matched by age and sex served as controls. In all subjects LA and IgG class anticardiolipin antibodies (ACA) were determined. Thrombin generation was monitored ex vivo measuring fibrinopeptide A (FPA) and prothrombin fragment F1 + 2 (F1 + 2) in blood emerging from a skin microvasculature injury, collected at 30 second intervals. In subjects with antiphospholipid antibodies mean FPA and F1 + 2 concentrations were significantly higher at most blood sampling times than in controls. In some SLE patients with APA the process of thrombin generation was clearly disturbed and very high concentrations of fibrinopeptide A were detected already in the first samples collected. Two minutes after skin incision SLE patients without APA produced slightly more FPA, but not F1 + 2, as compared to healthy subjects. Mathematical model applied to analyze the thrombin generation kinetics revealed that APA patients generated significantly greater amounts of thrombin than healthy controls (p = 0.02 for either marker). In contrast, in the same patients generation of thrombin in recalcified plasma in vitro was delayed pointing to the role of endothelium in the phenomenon studied. In summary, these data show for the first time that in SLE patients with antiphospholipid-protein antibodies thrombin generation after small blood vessel injury is markedly increased. Enhanced thrombin generation might explain thrombotic tendency observed in these patients.
Adult↗
[The role of magnesium in the pathogenesis and therapy of bronchial asthma].
Magnesium is the fourth most abundant metal found in the body. It plays a crucial role in numerous biological processes. It is a natural calcium antagonist. New experimental data suggest that Mg+2 influences a variety of lung structures. Intracellular Mg+2 is thought to modulate smooth muscle contractions and it is known to have a direct effect on calcium uptake, resulting in smooth muscle relaxation. Magnesium has been forgotten cation from the therapeutical point of view, but now several clinical reports point to the salutary actions of Mg+2 in various lung diseases. Many reports suggests that magnesium sulfate and aspartate has certainly a role as an adjunct to traditional therapy in asthma and asthma-like conditions and have been helpful in the treatment of acute exacerbations of asthma.
Asthma↗
[Ehlers-Danlos syndrome type VII--case report].
We described a 30-year old man with Ehlers-Danlos syndrome type VI, manifested by marked kyphoscoliosis and severe myopia, who was admitted to our department because of chest pain. Abnormalities of stature and joint system along with eye changes, occurred starting from the birth and they aggravated gradually. Signs of increased fragility of the skin blood vessels appeared during childhood. At the age of 26 years, the patient suffered from a spontaneous rupture of the right brachial artery. Spirometry revealed marked restrictive ventilatory insufficiency, which at present constitutes the predominant health risk for the patient.
Adult↗
[Immunologic tests in patients with idiopathic uveitis--preliminary reports].
AIM: This study aimed to evaluate the immune system function in patients with uveitis of unknown aetiology. METHODS: The clinical material comprised 19 patients with endogenous uveitis. In all cases the following immunological tests were performed: serum immunoglobulins A, G, M, total IgE, circulating immune complexes, complement components C3c and C4-all determined by laser nephelometry; antinuclear antibodies assessed with indirect immunofluorescence method using HEp-2 cell lines; and antineutrophil cytoplasmic antibodies using indirect immunofluorescence test. In 4 cases lupus anticoagulant was measured with APTT and dRVVT assays. RESULTS: Among our 19 examined patients immunological abnormalities were found in 12 cases. Changes in immunoglobulin concentrations were found in 8 cases. In 4 patients abnormalities of the complement system were observed. Antinuclear antibodies with speckled pattern in indirect immunofluorescence were present in 7 cases. CONCLUSION: In a proportion of patients with endogenous uveitis mild immunological abnormalities were present, suggesting an autoimmune background of the disease. Studies of the immunological profile can therefore help in better evaluation of the patients. It remains to be determined whether the observed immunological alterations are of any importance in the pathogenesis of the studied disease.
Adolescent↗
[Prevalence of thrombosis in secondary antiphospholipid-protein syndrome].
Antiphospholipid-protein syndrome (APS) comprises venous and arterial thrombosis, spontaneous abortion and thrombocytopenia in patients with antiphospholipid-protein antibodies (APA). Such antibodies are detected by immunoenzymatic (ELISA) methods (e.g. anticardiolipin antibodies-ACL) or coagulation assays (lupus anticoagulant-LA). APS in patients showing other symptoms of autoimmune disease is called secondary antiphospholipid-protein syndrome. The aim of the study was to find relation between history of thrombosis and APA in a group of patients with lupus erythematosus and lupus-like disease. Lupus anticoagulant was detected by a three step procedure using phospholipid dependent clotting assays and anticardiolipin antibodies were measured by ELISA. We studied 95 subjects (91 women, 4 men) suffering from lupus erythematosus (67 patients) and lupus-like-disease (28 patients). Lupus anticoagulant was found in 26, anticardiolipin antibodies IgG in 34 and IgM in 27 subjects. In a retrospective study 40 thrombotic events were detected in 36 patients; deep vein thrombosis in 19, pulmonary embolism in 7, ischaemic CNS events in 13 and myocardial infarction in one. Thrombosis was present more often in subjects with LA (61%) and ACL IgG (52%) than in subjects without these antibodies (24%) (p = 0.004 and 0.015, respectively). ACL IgM antibodies were not related to thrombotic episodes. The ACL IgG antibodies and LA are helpful in identifying subjects at risk factors of venous and arterial thrombosis among patients suffering from lupus erythematosus and lupus-like disease.
Adolescent↗
[Evan's syndrome with antiphospholipid-protein antibodies].
This is a case report of a 34 years old man with Evans syndrome associated with antiphospholipid-protein antibodies. They include lupus anticoagulant and antibodies against cardiolipin, prothrombin and beta 2-glycoprotein I, detected by ELISA. No thrombotic events were observed. The presence of several antibodies directed against surface cell membrane structures in Evans syndrome suggests a common pathogenetic mechanism.
Adult↗
[Laboratory diagnosis of lupus anticoagulant].
Lupus anticoagulant (LA) prolongs clotting times in vitro, but in vivo leads to an increased risk of thromboembolic complications. LA is detected in many patients with lupus erythematosus but also in subjects without any autoimmune disease (primary antiphospholipid syndrome). We determined LA in 133 patients, mostly with lupus erythematosus and other autoimmune diseases. Three screening and two confirmatory tests based on different mechanisms of clotting system activation were used. LA was found in 34 patients. DRVVT was the most useful test in detecting LA while kaolin clotting time was the least sensitive. Anticardiolipin antibodies (ACL) of IgG class were found in 41 patients studied (31%). They were most often detected in patient positive for LA (64%). ACL IgM were not associated with prolonged clotting times. Laboratory diagnosis of antiphospholipid syndrome is easy when the presence of LA is confirmed by two independent tests and in addition ACL would be detected. In other cases, when only single tests are positive, it is suggested to repeat diagnostic procedure at least once.
Adolescent↗
Interferon-gamma in the treatment of atopic dermatitis: influence on T-cell activation.
Atopic dermatitis (AD) is a chronic relapsing skin disease characterized by various immunologic abnormalities. Its pathogenesis remains obscure and its treatment difficult. We have studied the efficacy of systemic recombinant human interferon-gamma (rhIFN-gamma) treatment (0.05 mg/m2 sc on 3 consecutive days, during 4 weeks) in 10 patients with severe AD. Marked clinical improvement was observed starting from the third week of treatment. Erythema, dryness, and lichenification were the most responsive symptoms. Serum immunoglobulin E and IgG4 levels did not change during treatment. Blood eosinophil count decreased only transiently at the end of the first and second series of injections (days 4 and 11; P = 0.02). Patients with AD showed an increase in CD25-positive cells (11.0% vs 4.88%; P = 0.0001) as compared to 10 age-matched healthy controls. Moreover, in parallel with clinical improvement, a distinct decrease in CD25-positive lymphocytes was observed on days 32 and 50 (P = 0.002 and P = 0.006, respectively). We suggest that in AD the beneficial effect of rhIFN-gamma might be related to the inhibition of excessive T-cell activation, perhaps of the subpopulations, producing interleukin (IL)-4 and IL-5.
Adolescent↗
Autoimmune vasculitis preceding aspirin-induced asthma.
Two female patients have been described in whom peripheral vasculitis with cold sensitivity preceded by about 5 years typical symptoms of aspirin-induced asthma. Vasculitis was immunologically mediated and took the form of either limited cutaneous scleroderma or perniosis. Antinuclear autoantibodies of SS-B specificity were present in the serum. Challenge with aspirin provoked asthma and distinct blood circulation disturbance in the hands. It is speculated that in these patients vasculitis and asthma were causally linked, and leukotrienes mediated bronchial and vascular reactions that occurred simultaneously following aspirin administration.
Adult↗
[High levels of cholesterol and lipoprotein (A) in serum decreases the inhibitory effect of aspirin on generation of thrombin].
Aspirin (ASA) is widely used in the treatment of cardiovascular diseases. Recently, we have found that aspirin decreases not only platelet aggregation but also thrombin generation. This effect, however, was seen only in certain subjects. Therefore we decided to examine influence of a single dose of aspirin (500 mg) on thrombin generation in healthy volunteers. Thrombin genesis was assessed by serial measurements of fibrinopeptide A concentration in blood emerging from standardised forearm skin incisions. Aspirin reduced thrombin generation in persons with normal serum level of lipids. This effect was lost, however, in subjects with high level of cholesterol and lipoprotein (a)--well known risk factors of ischaemic heart disease. While the mechanism by which aspirin affects thrombin generation remains to be elucidated, our data indicate that hypercholesterolemic subjects might benefit less than others from preventive aspirin treatment.
Adult↗
The mechanism of anti-thrombotic, thrombolytic and fibrinolytic actions of camonagrel--a new thromboxane synthase inhibitor.
So far pharmacological consequences of inhibition of thromboxane A2 (TXA2) synthase by imidazole derivatives (e.g., camonagrel or dazoxiben) were linked to suppression of platelet activity. Here we report that in patients with peripheral atherosclerosis or in cats with extracorporeal thrombogenesis treatment with camonagrel is associated with activation of fibrinolysis or thrombolysis. These phenomena seem to be related to the camonagrel-induced shift in metabolism of prostaglandin endoperoxides from TXA2 to prostacyclin (PGI2), although in an in vitro model the involvement of the L-arginine/nitric oxide pathway cannot be excluded. In cats camonagrel (10 mg/kg i.v.) produced not only a fall in TXB2 but also a rise in 6-keto-PGF1 alpha and no change in cyclic-GMP plasma levels. This points to PGI2 rather than to nitric oxide as an in vivo mediator of camonagrel-induced thrombolysis. The crucial role of endogenous PGI2 in the thrombolytic response to camonagrel in cats was evidenced by its blockade following pretreatment of animals with a megadose of aspirin (50 mg/kg i.v.) and lack of any effect on pretreatment with L-NAME (100 micrograms/kg/min, i.v.). Obviously TXA2 synthase inhibitors (e.g., camonagrel) and cyclo-oxygenase inhibitors (e.g., aspirin) antagonize each other in their anti-thrombotic actions and must not be administered at the same time. Furthermore, in patients camonagrel (800 mg orally) suppressed TXA2 generation by 99.5% and doubled the plasma level of 6-keto-PGF1 alpha.(ABSTRACT TRUNCATED AT 250 WORDS)
Animals↗
[Human recombinant interferon gamma in the treatment of atopic dermatitis].
Atopic dermatitis (AD) is a chronic relapsing skin disease characterized by various immunologic abnormalities. We have studied the efficacy of recombinant human interferon gamma (rhINF-gamma) administered subcutaneously at a dose of 0.05 mg/m2 in ten patients with severe AD. Patients were treated for 4 weeks. They have shown marked clinical improvement starting from the third week of treatment. The efficacy of the drug varied, with erythema, dryness and lichenification being the most responsive symptoms. There was no change in serum immunoglobulin E and IgG4 levels. Whole blood eosinophil count decreased only transiently and was accompanied by a tendency to lower values of serum eosinophil cationic protein. Patient with AD showed an increased expression of a T-cell surface activation marker CD 25 as compared to healthy controls. Moreover, clinical improvement was roughly paralleled by the decrease in this T-cell activation marker. We conclude that rhINF-gamma is a novel efficacious therapeutic approach in severe AD. We suggest that its primary action might be related to the inhibition of T-cell activation.
Adolescent↗
[Lipid metabolism abnormalities and atherosclerosis in patients on maintenance hemodialysis].
Cardiovascular disease is the leading cause of death in maintenance hemodialyzed patients with end-stage chronic renal failure. Lipid abnormalities have been identified as significant risk factors for the development of premature atherosclerosis in such patients. These abnormalities include significant elevations of serum triglycerides and triglyceride-rich lipoproteins (VLDL, IDL, remnant particles), Ip(a), decrease in LDL and HDL, accompanied by changes in apolipoprotein content. The article reviews quantitative and qualitative changes in lipoprotein composition and their possible pathogenesis. The role of such changes in atherogenesis is also discussed.
Arteriosclerosis↗