Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.4.21.6 (thromboplastin)
 13,278 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An 11-year-old boy developed florid choreic movements in his right extremities after having had an episode of febrile illness. He was evaluated at our hospital where MRI disclosed a honeycomb-like low signal intensity area rimmed by a thin Gd-enhanced layer in the left putamen. Arteriography revealed the lenticulostriate arteries being segmentally narrowed and a "ground glass" staining was observed in the left putamen in late venous phase. Sydenham's chorea, that had been the initial impression, was not substantiated because of negative pharyngeal culture for streptococci, negative ASLO/ASK titers and because of lack of clinical stigmata of rheumatic fever. However, prothrombin time was prolonged, and activated partial thromboplastin time (APTT), that had been also prolonged, was not normalized by adding healthy serum, indicating the presence of lupus anticoagulant. VDRL was false positive and anticardiolipin antibodies, both IgM and IgG classes, were also detected. However, systemic lupus erythematosus was unlikely in view of negative antinuclear antibody and LE phenomenon. He deteriorated rapidly due to development of severe bilateral chorea, thereby he was unable to walk or feed himself. He received a 3-day course of mega-dose intravenous methylprednisolone, that temporarily lessened the chorea, but soon it became worse. A second course of mega-dose methylprednisolone was given, followed by daily maintenance dose of prednisolone. His chorea gradually improved in severity and after 2 months only a trace of choreic movements was detected in his hands. He has been followed at our outpatient clinic where he no longer shows chorea and the APTT has improved to nearly normal time.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of chorea as a sole presentation of primary anti-phospholipid antibody syndrome]. 181 92

Chorea has been related to the presence of antiphospholipid antibodies (a-PL) in the context of systemic lupus erythematosus (SLE). Here we report the case of a 13-year-old girl with a-PL antibodies, who had developed thrombophlebitis at the age of 11 years and chorea two years later, in the absence of clinically evident SLE. Serological tests revealed a false positive test for syphilis, a prolonged activated partial thromboplastin time, hypocomplementaemia and positive anti-DNA antibodies.
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PMID:Chorea as a manifestation of the antiphospholipid syndrome in childhood. 187 92

In a group of 10 women with circulating lupus anticoagulant 25 intrauterine deaths were previously documented in the nine multigravidae. The presence of lupus anticoagulant activity was confirmed by showing prolongation of the activated partial thromboplastin time and kaolin clotting time with failure of correction of the prolongation on incubation with normal plasma. A clinical diagnosis of systemic lupus erythematosus (SLE) was made in four women. Three had deep vein thrombosis in pregnancy, one chorea gravidarum while two had only recurrent fetal losses. All the women had positive antinuclear antibody tests and blood platelet counts less than 175 X 10(9)/l. Anti-smooth muscle antibody and VDRL tests were each positive in half the patients; anti-DNA antibody was present in two patients with clinically active SLE. In six pregnancies correction of the activated partial thromboplastin and kaolin clotting time was attempted using prednisone (40-60 mg/day); aspirin, 75 mg/day, was added. Five live infants were obtained, four by spontaneous delivery, when the restoration of the clotting abnormalities to normal was achieved. In one woman presenting with extensive deep vein thrombosis a live infant was delivered following therapeutic doses of heparin and low dose aspirin. Maternal lupus anticoagulant activity has major implications for pregnancy and should be excluded in women with a clinical suspicion of SLE, a positive antinuclear antibody test, thrombotic episodes, biologically false-positive VDRL and unexplained late or repetitive early fetal losses.
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PMID:Lupus anticoagulant in pregnancy. 642 2

A young man presented with generalized chorea as the first manifestation of probable primary antiphospholipid syndrome. He was well till 3 months before admission when he started to have involuntary, choreiform movements involving all extremities, the head and the bulbar muscles. Apart from these movements his physical examination was otherwise unremarkable. Laboratory investigations revealed mild thrombocytopenia, high partial thromboplastin time (PTT) only partially corrected by the addition of normal plasma, false positive syphilis serology, weakly positive antinuclear antibody and a high level of IgG anticardiolipin antibodies. Brain magnetic resonance imaging (MRI) showed multiple scattered small areas of high signal intensity on T2 weighted image in the area of centrum semiovale bilaterally. The patient was started on aspirin and prednisone with rapid symptomatic improvement. Despite the difficulty in proving the association between chorea and the high antiphospholipid antibodies, chorea appears in this case to be the initial symptom of primary antiphospholipid syndrome and we suggest screening for antiphospholipid antibodies in unexplained cases of chorea.
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PMID:Chorea and high antiphospholipid antibodies: probable primary antiphospholipid syndrome. 2428 89