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Query: UNIPROT:Q9UL83 (She)
 55,362 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a 21-year-old woman in whom chorea was associated with antiphospholipid antibodies. In August 1986, she developed involuntary movement which started in the right hand but subsided spontaneously. In September 1988, she again developed right-sided involuntary movements which started in the right hand but rapidly progressed to involve the whole of the right side. In September 1990, she was admitted to our hospital for investigation of choreiform movements, because her involuntary movements had progressed to involve all four extremities. She had no family or past history of chorea, psychiatric, rheumatological or vascular disease. On admission, she had difficulty in speaking and swallowing due to choreiform movements of her mouth and tongue. Her gait was unsteady. On walking she had wild gyrations of the arms. Choreiform movements of all four extremities, neck, face, mouth and tongue were present at rest, more marked on the right side. There was no other neurological deficits. She had none of the classical features of SLE. She had none of the complications commonly associated with antiphospholipid antibody syndrome (APS) (i.e., recurrent spontaneous abortion, thrombosis and thrombocytopenia). Laboratory tests revealed that antinuclear antibody was present. Cardiolipin antibody (VDRL) was positive but specific tests for syphilis were negative. Anticardiolipin antibodies were present. All coagulation studies have failed to reveal lupus anticoagulant. Brain CT, MRI, 123IMP-SPECT and cerebral angiography were normal. Associated with her chorea, she had the serological but not the clinical features of APS. We suggest that antiphospholipid antibodies should be looked for in all unexplained cases of chorea, even when the associated clinical signs of APS are absent.
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PMID:[Chorea associated with antiphospholipid antibodies]. 130 Feb 73

A 55-year-old woman with a lupus like disease, associated with the lupus anticoagulant, was admitted because of facial edema. Her facial swelling was previously attributed to the steroids she had been taking and they were tapered without improvement. Laboratory tests revealed high titers of anticardiolipin antibodies. Computerized tomography of her chest and Doppler ultrasound examination of her neck veins demonstrated a thrombus in the superior vena cava. She was treated with heparin and was maintained with longterm warfarin therapy with uneventful followup. Superior vena cava obstruction should be added to the long list of thromboembolic complications of the antiphospholipid syndrome.
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PMID:Superior vena cava occlusion in a patient with antiphospholipid antibody syndrome. 157 77

A 23-year-old woman, who had nonbullous congenital ichthyosiform erythroderma since her childhood, was diagnosed as nephrotic syndrome caused by systemic lupus erythematosus (SLE). She was pregnant but experienced fetal loss at the age of 25. Although 10 mg/day of oral prednisolone was administered, low levels of serum complement, proteinuria, thrombocytopenia (6.0 x 10(4)/mm3) and biological false positive for STS continued. When she was 27 years old, right hemichorea developed. She was admitted to our hospital at the age of 28 because of low levels of serum complement, high titers of anti ds-DNA antibody, profuse proteinuria, gingival bleeding and thrombocytopenia (1.5 x 10(4)/mm3). The nephrotic syndrome gradually improved after 1 g/day of methylprednisolone for 2 days and the oral prednisolone dosage was then increased up to 40 mg/day, and was tapered to 10 mg/day. Epileptic attack (minor seizure) occurred at the age of 29. Continuous low levels of serum complement and high titers of anti ds-DNA antibody were improved by adding 50 mg/day of cyclophosphamide. However, high levels of beta 2 GPI dependent anticardiolipin antibody and lupus anticoagulant activity were observed throughout the study. Our patient was a very rare case of congenital ichthyosis with typical antiphospholipid antibody syndrome and SLE. A few cases of acquired ichthyosis associated with SLE has been reported, and ichthyosis developed only in active stage of SLE. However, our patient's ichthyosiform lesions were not changed throughout the course.
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PMID:[Systemic lupus erythematosus and antiphospholipid antibody syndrome in a patient with congenital ichthyosiform erythroderma]. 748 67

This case involves a 41-year-old woman with SLE. The patient began having symptoms of arthralgia in 1978 and developed fever, pleuritis and lupus psychosis in 1986. Laboratory exams showed positive antinuclear-antibody, LE-cell phenomenon, hypocomplementemia and lupus anticoagulant. Echo cardiography demonstrated mitral regurgitation and stenosis. She was treated with 50 mg of prednisolone and these manifestations subsided. In 1989, she developed dyspnea on exertion and echo cardiography revealed severe mitral stenosis. Pulmonary infarction was detected by MAA lung scintigraphy. At this time, she was diagnosed as SLE associated with antiphospholipid syndrome (APS). A mitral valvular replacement operation was performed in 1991. Pathological studies of mitral valve demonstrated Libman Sacks endocarditis. APS is known occasionally to complicate with left-sided valvular diseases, mitral stenosis is quite rare in both SLE and APS. This patient reveals a rare case of SLE associated with APS and mitral stenosis. It is suggested that this patient developed mitral stenosis with Libman Sacks endocarditis, associated with the presence of antibody against phospholipids.
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PMID:[A case of SLE associated with antiphospholipid syndrome and mitral stenosis]. 755 44

We presented the coexistence of the severe aortic insufficiency and the systemic lupus erythematosus with antiphospholipid syndrome in 33-years old woman. She was qualified for the operation of the prosthesis aortic valve replacement after she was treated with steroids. During the operation, the heart infarct of the inferior wall had been observed, but finally in the postoperation period the heart efficiency improvement was observed. We have discussed same theories and clinical experiences of lupus erythematosus with antiphospholipid syndrome and clinical sequels.
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PMID:[Coexistence of severe aortic insufficiency and systemic lupus erythematosis with antiphospholipid syndrome--case report]. 771 57

Anti-phospholipid syndrome, originally called anticardiolipin syndrome, is characterized by the presence of anti-phospholipid antibodies and a marked tendency to both arterial and venous thrombosis. The little information available on the implications of this syndrome for anesthesia derive from the recent description of the disease. We describe 2 patients, each with 1 of the 2 forms of antiphospholipid syndrome that have been described to date, and each needing surgery for a different reason. The first was a 24-year-old woman who was admitted to the hospital with diarrhea, fever and metrorrhagia in her fifth month of pregnancy. Blood tests revealed a weakly positive title of anti-cardiolipin antibodies. Steroid and antiplatelet therapy was begun. Delivery was at 35 weeks by elective cesarean with epidural anesthesia due to oligoamnios. The second patient was 52-year-old woman with a history of 13 miscarriages, cerebrovascular accident and deep venous thrombosis. She had been diagnosed as having systemic lupus erythematosus with anti-phospholipid syndrome and was receiving corticoid and antiplatelet therapy. She had been admitted on 2 occasions for epistaxis, purpura in the lower extremities and severe thrombocytopenia. The last condition did not respond well to immunosuppressant therapy and a splenectomy was therefore performed with the patient under general anesthesia. In both cases recovery was good in spite of the serious complications of anesthetic management.
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PMID:[Anesthetic implications in antiphospholipid syndrome. 2 clinical cases]. 779 18

A young Caucasian woman developed bilateral deep vein thromboses (DVT) while taking the oral contraceptive (OC) in 1984. After a 9-month period of longterm anticoagulation therapy with warfarin, she experienced recurrent DVT 2 weeks following discontinuation of anticoagulation. She was then maintained with warfarin for the following 6 years. Elevated antibodies to cardiolipin (IgG aCL) were first detected in 1988. In 1990, warfarin was switched to aspirin and low molecular weight heparin as she wished to become pregnant. At 24 weeks she developed eclampsia and labor was induced. During the first postpartum week she experienced loin pain, associated with rapidly rising creatinine levels and features of a nephrotic syndrome. An abdominal ultrasound showed bilateral renal vein thrombosis with enlarged kidneys. She was treated with continuous ambulatory peritoneal dialysis (CAPD) for 4 months and her renal function is now recovering. Our patient is another example of the primary antiphospholipid syndrome.
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PMID:Postpartum bilateral renal vein thrombosis in the primary antiphospholipid syndrome. 796 89

Thrombotic thrombocytopenic purpura (TTP) is a rare syndrome of unknown etiology and has a high mortality rate. TTP is characterized by a pentad of clinical findings, including microangiopathic hemolytic anemia, thrombocytopenia, renal abnormalities, neurologic signs and fever. The pathological feature of TTP consists of disseminated microvascular platelet thrombi. We describe a case of TTP with primary anti-phospholipid syndrome. A 27-year-old woman developed TTP in her second trimester of pregnancy. She presented with classical symptoms of TTP with compatible renal biopsy findings. Although four articles of SLE criteria (1982 ARA) were fulfilled, three of them were considered to be derived from multiple thrombosis except for a positive antinuclear antibody. Positive antiphospholipid antibodies (lupus anticoagulant and anticardiolipin antibody) with SLE-like findings suggested the diagnosis of antiphospholipid antibody syndrome rather than SLE. Although TTP has been described in patients with SLE and they can share common clinical and pathological features, the relationship between these two diseases is controversial. Many theories have been proposed to explain the nature and cause of intravascular platelet aggregation in TTP. But the pathogenesis of TTP is still unclear. This case suggests an important causal relationship between antiphospholipid antibodies and TTP during pregnancy. In TTP patients who also have SLE or SLE-like features, the antiphospholipid antibodies may have a role in the development of multiple microthrombosis.
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PMID:[Thrombotic thrombocytopenic purpura (TTP) observed in a patient with primary antiphospholipid antibody syndrome]. 786 89

The case of a 35 years old female patient is here reported. Her previous medical records included repeated miscarriages and ischaemic ulcers in the right leg. She developed thrombosis at the left common femoral artery with serum antiphospholipid antibodies. The patient was treated with anticoagulants and two months later developed multiorganic failure. She was successfully managed with plasmapheresis, high dose prednisone, and anticoagulants. The clinical picture was considered to be a catastrophic antiphospholipid syndrome.
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PMID:[Catastrophic antiphospholipid syndrome. Presentation of a case]. 787 64

An 18-years-old female was admitted to our hospital because of severe edema, that had developed within one week, and massive proteinuria. She has been given treatment for mild proteinuria and Raynaud's phenomenon. Before the edema developed, she complained of recurrent attack of migraine associated with scintillating scotoma. At admission, she was suffering from butterfly erythema, hydropericardium and ascites. The results of tests for antinuclear antibodies and ENA antibodies were negative, but those for BFP and IgG-anticardiolipin antibody were positive, and the platelet and complement levels were low. Renal biopsy revealed typical mesangiolysis without any sign of hyperplastic nephritis. Electron microscopy demonstrated the deposition of microfibrillar structures in the mesangial areas. She was diagnosed as having SLE-antiphospholipid antibody syndrome. After the administration of prednisolone 40mg/day, the edema, Raynaud's sign and proteinuria disappeared. Migraine was improved by the additional use of warfarin, and the platelet count was normalized. These findings suggest that in this case, acute nephrotic symptom developed due to hemangioendothelial injury caused by anti-phospholipid antibody.
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PMID:[A case of anti-phospholipid antibody syndrome associated with typical mesangiolysis]. 796 76


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