Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In this report we describe a female patient who presented with peripheral circulatory impairment. She had previously been thought to have a systemic vasculitis, but had failed to respond to corticosteroid and immunosuppressive therapy. Serologic investigations were negative. The patient was a very heavy smoker, and it became clear that her condition had many of the clinical features of Buerger's disease (thrombangiitis obliterans). The diagnosis was confirmed by angiography, and a coagulation screen showed her to have associated protein S deficiency. Her symptoms responded rapidly to cessation of smoking, warfarin therapy, and a series of intravenous prostacyclin infusions. We discuss in detail the possible pathologic mechanisms which might explain the association of protein S deficiency and thrombangiitis obliterans.
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PMID:Buerger's disease and protein S deficiency: successful treatment with prostacyclin. 880 56

We report six cases of protein S deficiency secondary to varicella. Five cases were complicated by thrombotic and vascular events, namely purpura fulminans and necrotic vasculitis, deep vein thrombosis and stroke. Two cases were associated with protein C deficiency and one case revealed a heterozygous factor XII deficiency. The underlying mechanism of this acquired protein S deficiency is unclear but could be related to a direct effect of zoster virus.
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PMID:Varicella and thrombotic complications associated with transient protein C and protein S deficiencies in children. 795 22

Little is known about the pattern of Deep Vein Thrombosis in Saudi Arabia. Over 4 year period, 62 cases with strong evidence of venous thrombosis were studied in King Abdulaziz University and King Fahad Hospitals to learn the pattern of deep vein thrombosis in Jeddah, Western Saudi Arabia. There were 32 females and 30 males. The mean age of the group was 36.0 years (range 6-90 years). One or more risk factors was/were detected in 40 patients. Among these 14 factors, age more than 50 years, obesity, vasculitis, malignancy and postpartum were the common factors encountered. In other 22 patients, no risk factor was found. However, extensive laboratory search diagnosed 9 rare disorders out of these 22 cases. Antithrombin III, protein C, protein S deficiencies in 5, 2, 1 patients, consecutively. The last patient had significantly shortened PTT. The other 13 (21.0%) patients were considered real idiopathic DVT. Extremities were involved in 54 patients compared to only 8 cases with inferior vena cava or visceral thrombosis. The upper limb was affected in only 10 patients unlike the lower limb which was more commonly affected n = 37.
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PMID:Pattern of deep venous thrombosis in Jeddah area, western Saudi Arabia. 837 13

A 27-year-old woman suffered from a sudden onset of slight paralysis of the right side of her body and the inability to express herself by speech, writing, or signs. She was admitted to the National Rehabilitation Hospital in Washington, D.C., in the US. 6 months prior to these events, she had been in a motor vehicle accident and had since experienced headaches and generalized musculoskeletal pain. The only drug she took was an oral contraceptive (OC), which she took irregularly. Health workers could not arouse her upon admission. Clinical examination revealed symptoms consistent with a left hemispheric stroke. Cerebral computed tomography and magnetic resonance imaging revealed a left temporoparietal infarct. Her free protein S was only 27% on admission and 14% 11 days after admission (normal range, 55-125%). Over the next 72 hours, her physical condition deteriorated, entailing focal motor seizures, right Babinski's sign, loss of pain reflex response on her right side, and complete paralysis of the right side of her body. The left middle cerebral artery appeared to be constricted, which physicians first believed was caused by vasculitis but later found was the result of emboli. The patient developed right femoral vein deep thrombosis. The physicians treated her initially with heparin and followed with warfarin therapy. Nevertheless, embolus. Health workers placed a filter in her inferior vena cava and continued warfarin therapy. She did not experience any more thrombotic or embolic episodes during the rest of her hospital stay. OCs reduce circulating levels of free protein S which, along with activated protein C, inhibits clotting. OCs likely reduced her already existing low levels of free protein S. Deficiency of free protein S was likely responsible for the cerebral infarction and her thrombotic and embolic episodes.
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PMID:A case of cerebral infarction in association with free protein S deficiency and oral contraceptive use. 823 70

Two girls, 22 months and 12 years of age, presented with repeated cerebral infarctions in association with primary antiphospholipid syndrome. The younger patient also suffered from protein C deficiency, while the other one had protein S and complement C4 deficiencies. All other causes of cerebral infarction were excluded; however, vasculitis remains a possibility in one patient. Both girls developed spastic tetraparesis as a sequela of the previous infarctions. The two patients were treated with aspirin and prednisone, with remission of the infarctions during the next 8 months of observation. A primary deficiency of protein C or S is proposed which would produce cerebral thrombosis with exposure of phospholipids; this thrombosis then, like antigens, would generate antibodies acting on the thrombin-thrombomodulin complex, exacerbating the thrombotic process. The association of complement C4 deficiency is an additional risk factor.
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PMID:Childhood stroke associated with protein C or S deficiency and primary antiphospholipid syndrome. 845 4

Markers of endothelial cell activation were measured in 28 patients presenting with various forms of limited or focal type cutaneous vasculitis. Plasma levels of tissue plasminogen activator antigen (t-PA:Ag), plasminogen activator inhibitor type 1 antigen (PAI-1:Ag) and PAI-1 activity, fibrin plate, von Willebrand factor antigen (vWF:Ag), tissue factor (TF) and soluble thrombomodulin (sTM) were measured. In comparison with the control group (n = 20) there was a significant increase in t-PA:Ag, vWF:Ag and TF (P < 0.05, Mann-Whitney U-test) in the cutaneous vasculitis group. This study confirms that measurable degrees of endothelial activation occur in cutaneous vasculitis. Cutaneous vasculitis includes a diverse group of clinical conditions, which are associated with inflammatory changes in cutaneous blood vessels with local fibrin deposition. The aetiology and pathogenesis of the majority of these entities remain unknown. Causative mediators are thought to include immune complexes, anti-endothelial cell antibodies, cytotoxic lymphocytes and viruses. Histologically, immune complexes and complement are frequently detected on the vessel wall, and serologically anti-endothelial antibodies are often detected in patients with vasculitis and in systemic lupus erythematosus (SLE) which correlate with the severity of cutaneous vasculitis, arthritis and nephritis. Lymphocyte-mediated toxicity to endothelial cells has been reported in a small number of patients with giant cell arteritis and Takayasu's arteritis. The vascular endothelium plays a central part in the control of haemostasis. Under physiological conditions endothelial cells present an anticoagulant surface to blood constituents, partially due to surface expression of heparan sulphate and thrombomodulin (TM). Heparan sulphate binds antithrombin III (ATIII), thereby accelerating inactivation of intrinsic coagulation enzymes. Thrombomodulin is an endothelial cell surface glycoprotein which promotes anticoagulation by forming a complex with thrombin which then activates protein C. Activated protein C together with a cofactor, protein S, inactivates FVa and FVIIIa. von Willebrand factor (vWF) is synthesized by endothelial cells, stored in Weibel-Palade bodies and released into the circulation upon endothelial stimulation. vWF mediates the binding of platelets to the subendothelium and is the carrier molecule for FVIIIC. The endothelium controls fibrinolysis by producing t-PA and its inhibitor PAI-1. Inflammatory cytokines such as interleukin-1 (IL-1) and tumour necrosis factor (TNF) activate endothelial cells, causing a shift from an antithrombotic to prothrombotic state, including expression of tissue factor, increased synthesis of PAI-1 and decreased expression of TM. Fibrin deposition and intravascular thrombosis are seen in cutaneous vasculitis syndromes, suggesting local endothelial cell activation. The aim of this pilot study was to assess whether perturbation of the endothelium in cutaneous vasculitis could be detected in the patients' plasma samples. If so, further studies to assess any correlation in levels of these markers with disease activity might prove useful in the future.
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PMID:Endothelial cell activation in cutaneous vasculitis. 868 65

Cerebral infarction before the age of 45 years accounts for 4-6% of all strokes. The etiology remains unexplained in a significant proportion of patients even after extensive investigations. The reported risk factors of this age group are cardiopathies, hypertension, smoking, hypercholesterolemia, reduction of anticoagulant proteins, hypercoagulable states, antiphospholipid antibodies primary syndrome, antiphospholipid antibodies secondary syndrome, some hemoglobinopathies, hyperviscosity syndromes, vasculitis, collagen vascular diseases, fibromuscular dysplasia, arterial dissections, migraine, myopathy encephalopathy lactic acidosis stroke like episodes, homocystinuria, familial amyloid angiopathy, microangiopathy with retinopathy encephalopathy and deafness, systemic lupus erythematosus, use of cocaine, traumas or manipulations of neck, AIDS. From 1/1/94 to 04/30/95 we observed 19 patients with cerebral infarctions and 9 patients with transitory ischemic attacks in young people. The aim of our study was to apply a diagnostic protocol by sequential tests of first level and second level. According to this protocol we found that the more common risk factors were ischemic cardiopathy, hypertension, smoking and hypercholesterolemia. Moreover we observed other independent risk factors, although less frequent, like the antiphospholipid antibodies, neurolupus, AIDS, deficit of protein S.
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PMID:[The application of a new diagnostic protocol for stroke in the young]. 876 46

The clinical spectrum of retroviruses is expanding rapidly. Human T-cell lymphotropic virus type I (HTLV-I) was the first retrovirus to be described, and its role had been established in adult T-cell leukemia/lymphoma and tropical spastic paraparesis. We report the case of a 35-year-old woman with HTLV-I and the unusual combination of cutaneous T-cell lymphoma, tropical spastic paraparesis, cerebral vasculitis, and protein S deficiency. We discuss the relationship of all her diseases to HTLV-I.
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PMID:Cutaneous T-cell lymphoma, tropical spastic paraparesis, cerebral vasculitis, and protein S deficiency in a patient with HTLV-I. 886 96

Calciphylaxis is a rare syndrome mostly affecting patients with secondary hyperparathyroidism and in some cases with functional protein C or protein S deficiency. Skin lesions begin as superficial painful patches that progress to deep necrotic lesions. The findings are often misdiagnosed as livedo vasculitis and the prognosis is poor. Histopathologically, calcification in the media of small arteries and arterioles with intimal hyperplasia is seen. It is unclear if this morphologic hallmark is pathogenetic. Therapeutically, the calcium-phosphate product should be lowered pharmacologically by an intensified and modified dialysis treatment and parathyroidectomy.
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PMID:[Calciphylaxis of the skin as a sequela of terminal kidney failure. Report and discussion of 3 cases]. 1041 32

A 28-year old female patient presented with unilateral dropped visual acuity and a central scotoma. Fundus examination and a fluorescein angiogram were suggestive for central retinal vein prethrombosis. Further tests revealed an isolated protein S deficiency for which oral anticoagulation therapy was initiated. Six months later she presented with new symptoms of a right retrobulbar pain on ocular movements. The retinal vessels had normalized with improved visual acuity. Re-evaluation of the fluorescein angiogram taken six months earlier showed an image compatible with unilateral vasculitis. During this six month period persisting high levels of IgG and IgM antibodies against Toxoplasma were observed while no signs of other active infection could be found. This report illustrates that in cases of prethrombosis with vasculitis in a young patient, a full medical examination and a thorough investigation of infectious diseases are warranted.
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PMID:Unusual retinal vasculitis in a patient with protein S deficiency and systemic toxoplasmosis: a case report. 1134 18


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