Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.4.21.69 (APC)
 16,337 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Tissue plasminogen activator (t-PA) and its inhibitor (PAI) were assessed in venous blood drawn before and after venous occlusion (bvo, avo) for 33 patients with Raynaud's phenomenon (RP), 14 with primary RP (PRP), 9 with suspected secondary RP (SSRP), and 10 with definite collagen disease and secondary RP (SRP). There were significant differences in PAI values avo between PRP (and controls), SSRP, and SRP. PAI activity decreased significantly avo only in controls and in PRP, and there was significant t-PA antigen elevation avo in the same groups. In addition, since PAI is neutralized by activated protein C (PC), both PC antigen and PC activity were assessed avo and bvo. PC Ag remained unchanged in all groups, with PC activity significantly lower than controls in SRP and SSRP. Finally the authors looked for interference of anticardiolipin antibodies (ACA) and lupus-like anticoagulant (LAC) with the PC system in collagen disease-associated RP. Specific IgG ACA were found in only 1 patient with SRP. In conclusion, there is an endothelial derangement, involving t-PA release and PAI, in SSRP and SRP patients. The reduced PC activity in these latter groups appears to be due to increased PAI influence rather than to ACA/LAC.
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PMID:t-PA, PAI, and protein C before and after vascular occlusion of the upper limb in patients with Raynaud's phenomenon. 190 68

In 15 patients with systemic sclerosis (SS) and 8 patients suffering from silicosis and/or silica dust exposure-associated scleroderma (SAS), various parameters of endothelial cell and platelet function and of blood coagulation and fibrinolysis were studied. In 9 of the 23 patients the values for the von Willebrand factor antigen were increased, and the same applied to the endothelin levels in 8 of 23 patients. Protein C, protein S, anti-thrombin III and tissue plasminogen activator (before and after 10 min venous occlusion) were normal. The plasminogen activator inhibitor, however, was increased in 5 patients. Increased levels of platelet factor 4 and of beta-thromboglobulin were found in 20 patients, while the ADP- and epinephrine-induced platelet aggregation was reduced in 5 patients. No individual patient was found to have a general disturbance of all parameters. The deviations in the parameters of endothelial cell and platelet function and of blood coagulation and fibrinolysis proved to be rather inconsistent. This suggests different functional stages in dependence on the various influential factors. There was no close correlation either with the severity of Raynaud's phenomenon or with the type of SS. In addition, there were no basic differences between SS and SAS. The disturbances occurred with similar frequency and in similar proportions in both disease groups.
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PMID:[Vascular function parameters in idiopathic and quartz-induced progressive scleroderma]. 827 90

Necrosis of the digits is a rare complication of warfarin therapy of obscure pathogenesis. We report a 61-year-old woman with a 12-month history of Raynaud's phenomenon who developed multiple digital necrosis following aortic valve replacement with mechanical prosthesis for aortic insufficiency caused by nonbacterial thrombotic endocarditis. Exacerbation of Raynaud's phenomenon occurred during the postoperative period, with daily episodes of ischemia of the fingers and toes that improved with local warming. However, coincident with the occurrence of immune heparin-induced thrombocytopenia, and while undergoing routine warfarin anticoagulation because of the mechanical valve prosthesis, the patient abruptly developed progression of digital ischemia to multiple digital necrosis on postoperative day 8, at the time the international normalized ratio reached its peak value of 4.3. All limb pulses were readily palpable, and vascular imaging studies showed thrombosis only in the superficial femoral and popliteal veins of the right leg. Coagulation studies showed greatly elevated levels of thrombin-antithrombin complexes and prothrombin fragment F1.2 levels, consistent with uncontrolled thrombin generation. After vitamin K administration, no abnormalities of the protein C anticoagulant pathway were identified, consistent with previous studies of other patients with warfarin-induced necrosis complicating heparin-induced thrombocytopenia. Subsequently, the patient was shown to have metastatic breast adenocarcinoma, which explained the patient's initial presentation with nonbacterial thrombotic endocarditis. This patient case suggests that multiple digital gangrene can result from the interaction of various localizing and systemic factors, including compromised microvascular blood flow (Raynaud's phenomenon), increased thrombin generation (heparin-induced thrombocytopenia, adenocarcinoma), and warfarin-induced failure of the protein C natural anticoagulant pathway.
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PMID:Warfarin-associated multiple digital necrosis complicating heparin-induced thrombocytopenia and Raynaud's phenomenon after aortic valve replacement for adenocarcinoma-associated thrombotic endocarditis. 1469 34

Hepatitis A virus is rarely associated with extrahepatic complications. A child presented with a history suggesting Raynaud's phenomenon and severe digital gangrene, as a complication of hepatitis A infection. Coagulation study results (protein C, protein S, antithrombin III, and activated protein C resistance) were all normal except for anticardiolipin antibodies, which were present on initial presentation and resolved later. Antinuclear antibodies, rheumatoid factor, lupus anticoagulant, antineutrophilic cytoplasmic antibodies, and Venereal Disease Research Laboratory test were all negative. Cryoglobulins were not detected. C3 and C4 levels were normal. Blood urea nitrogen and creatinine levels were normal as well. The patient was treated with aspirin and diclofenac, and improved slowly with complete resolution of gangrene and symptoms after 3 months. She remains asymptomatic 4 years later. Transient antiphospholipid syndrome or isolated Raynaud's phenomenon may be added to the list of extrahepatic complications of hepatitis A.
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PMID:Isolated digital gangrene complicating hepatitis a infection in a child. 1704 69