Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Invasive aspergillosis has been increasingly recognized as causing significant morbidity and mortality in immunocompromised patients but has never been diagnosed by fine-needle thyroid aspiration. A 24-year-old female with systemic lupus erythematosus presented with cough, shortness of breath, and fever of unknown origin unresponsive to broad-spectrum antibiotics. History and physical examination failed to indicate a source of infection. An 111In white blood cell scan showed thyroid localization. Physical examination revealed a multinodular goiter with a left dominant nodule. Fine-needle aspiration biopsy of a thyroid nodule revealed branching hyphae suggestive of Aspergillus sp. Despite immediate and aggressive treatment with amphotericin B and fluconazole, the patient died of overwhelming infection.
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PMID:Invasive aspergillosis diagnosed by fine-needle aspiration of the thyroid gland. 1059 62

We reported a 68-old female who was diagnosed to have systemic lupus erythematosus 18 years ago. She had been well under 5 mg of prednisolone until 1995, when she felt severe shortness of breath. Laboratory examinations disclosed severe anemia accompanying mild thrombocytopenia. Bone marrow aspiration revealed a complete absence of erythroid progenitor cells. She was diagnosed to have pure red cell aplasia (PRCA) as well as antiphospholipid syndrome. A judicious use of methylprednisolone including pulse therapy resulted in a prompt resolution of anemia as well as thrombocytopenia and the dose of corticosteroid was tapered successfully thereafter. Persistent infection of HPV B 19 in the patient with inactive SLE was considered as a main cause of PRCA because not only IgG-HPV B 19 antibody but viral DNA was demonstrated in her serum at the time of admission. The relationship between PRCA and anti-phospholipid syndrome in this patient was also noted.
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PMID:[A case of systemic lupus erythematosus with pure red cell aplasia possibly caused by persistent infection of human parvovirus B 19]. 1115 95

A 62-year-old woman with a history of pulmonary embolism and primary antiphospholipid syndrome (PAPS) with positivity for lupus anticoagulant was admitted to hospital with shortness of breath. A filter had been implanted in her inferior vena cava (IVC) 5 years previously. Emergency echocardiography revealed a lobulated, mobile echogenic mass on the tricuspid valve, and on pulmonary perfusion scintigraphy several apparently new defects were noted. Fibrinolytic therapy improved her symptoms and the pulmonary perfusion, then intravenous heparinization was continued for a further week. Repeat echocardiography performed on the 7th day of the admission showed complete disappearance of the mass, which was retrospectively diagnosed as a thrombus based on its resolution with fibrinolytic and anticoagulant therapies.
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PMID:Thrombus on the tricuspid valve in a patient with primary antiphospholipid syndrome after implantation of an inferior vena cave filter. 1195 63

A 52-year-old woman with systemic lupus erythematosus (SLE) presented with shortness of breath. Echocardiography and cardiac catheterization demonstrated a discrete left ventricular aneurysm (LVA) with normal coronary arteries. Although her heart failure symptomatically improved with medical therapy, she suffered an embolic stroke from a thrombus within the LVA. She was treated with anticoagulation and rehabilitation for 6 weeks. Reevaluation with echocardiography demonstrated persistent depressed LV function and mural thrombus within the LVA. Surgical resection of the LVA was performed with evacuation of the thrombus and local repair of the LV.
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PMID:Surgical resection of a lupus-related left ventricular aneurysm in a patient with normal coronary arteries: case report. 1576 24

Although patients undergoing cardiac surgery often present with diverse comorbidities, those with coagulation derangements are especially challenging. The present report describes the management of a patient who presented with a Factor V Leiden mutation, lupus anticoagulant, and acquired activated protein C resistance. A 42-year-old female presented with acute shortness of breath and chest pain. She was otherwise healthy 1 month prior to admission when she presented with dysfunctional uterine bleeding, resulting in the transfusion of three units of packed red blood cells. Coagulation evaluation revealed that the patient had lupus anticoagulant, factor V Leiden mutation and an activated protein C resistance. The patient presented with an acute myocardial infarction and was found to have 90% stenosis of her left main coronary artery, moderate mitral and tricuspid regurgitation, and a left ventricular ejection fraction of 25%. An emergent off-pump coronary artery bypass procedure with placement of a vein graft to the left anterior descending artery was completed. Intraoperative thrombophilia was encountered as evidenced by both an elevated thromboelastograph coagulation index (+3.6) and an acquired antithrombin-III deficiency. Postoperatively, the patient was placed on low molecular weight heparin, but developed heparin-induced thrombocytopenia and was switched to a direct thrombin inhibitor, argatroban. The following case report describes the coagulation management of this patient from the time of admission to discharge 43 days later, and the unique challenges this combination of hemostatic defects present to the clinicians.
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PMID:Coagulation management of a patient with factor V Leiden mutation, lupus anticoagulant, and activated protein C resistance: a case report. 1591 49

A 23-year-old woman was admitted with progressive shortness of breath. Echocardiography showed a large volume of pericardial effusion, which indicated cardiac tamponade. Yellowish and puriform fluid with increased white blood cell count (neutrophil dominant) was aspirated, but antibiotics were ineffective. Further examination revealed the presence of positive anti ds-DNA antibody, anti SS-A antibody and anti Sm antibody, resulting in a diagnosis of systemic lupus erythematosus. Her condition was smoothly improved by predonisolone administration. Cardiac tamponade is a rare initial manifestation of systemic lupus erythematosus.
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PMID:Systemic lupus erythematosus initially manifesting as acute pericarditis complicating with cardiac tamponade : a case report. 1755 93

A 70-year-old woman complained of mild shortness of breath. Laboratory findings revealed pancytopenia, positive lupus anticoagulant and severe hypocomplementemia without anti-nuclear or anti-DNA antibodies. After the failure of prednisolone treatment, an acquired C1-esterase inhibitor (C1-INH) deficiency was diagnosed. There were no episodes of angioedema or deep vein thrombosis. Three months later, extreme splenomegaly was detected. Lymph node biopsy suggested splenic marginal zone B-cell lymphoma. Acquired C1-INH deficiency due to a lymphoproliferative disorder should be considered as a possible diagnosis for patients with severe hypocomplementemia.
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PMID:Acquired C1-esterase inhibitor deficiency and positive lupus anticoagulant accompanied by splenic marginal zone B-cell lymphoma. 1788 23

Antiphospholipid syndrome is an autoimmune disease characterized pathophysiologically by the presence of antiphospholipid antibodies and > or =1 clinical manifestation, the most common being venous or arterial thrombosis. We describe the case of a 40-year-old male with unexplained severe pulmonary arterial hypertension with a seven-day history of progressive shortness of breath, hemoptysis, chest discomfort and bilateral pedal edema. Electrocardiographic, echocardiographic and imaging studies showed changes consistent with chronic thromboembolic pulmonary hypertension. Further work-up showed positive anticardiolipin antibodies and lupus anticoagulant with negative features for lupus with negative primary thrombophilic studies as well. The patient was managed adequately with oral anticoagulation with improvement of his clinical status and referred to a tertiary care center to be screened for pulmonary thromboendarterectomy. For patients meeting surgical selection criteria, pulmonary thromboendarterectomy has demonstrated positive outcomes with respect to survival, functionality and quality of life. We discuss the pathophysiology and treatment as well as novel therapies in nonsurgical candidates with chronic thromboembolic pulmonary hypertension in the setting of primary antiphospholipid syndrome.
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PMID:Chronic thromboembolic pulmonary hypertension as an uncommon presentation of primary antiphospholipid syndrome. 1859 78

Mast cell tryptase can be an indicator of type I hypersensitivity reaction and thus may serve as a surrogate marker of anaphylaxis. A 34-year-old white male patient presented with a history of systemic lupus erythematosus. Shortly after administration of cefazolin for dialysis, he developed pruritus and shortness of breath. He expired an hour later. Autopsy excluded anatomic causes of death. There was an elevated postmortem mast cell tryptase level, 29.2 ng/mL. For mast cell tryptase level to be useful, the patient must survive long enough after exposure to an allergen for mast cells to release this enzyme. A credible allergen must be identified. In this case such, mast cell tryptase could establish anaphylaxis as the cause of death. The case suggests that in a patient with autoimmune disease, it may be prudent to test for immune reaction to a drug before administering it a second time via pinprick or other method.
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PMID:Mast cell tryptase in a case of anaphylaxis due to repeat antibiotic exposure. 1951 97

Chronic thromboembolic pulmonary hypertension (CTEPH) caused by intraluminal thrombus organization and fibrous stenosis or complete obliteration of pulmonary arteries, is a not rare but life-threatening complication of acute pulmonary embolism. The prognosis of medically treated patients with CTEPH is poor and worsens as pulmonary hypertension exacerbates. We describe the case of a 43-years old with a history of progressive shortness of breath, hemoptysis, chest discomfort and syncope. Echocardiographic and imaging studies showed changes consistent with chronic thromboembolic pulmonary hypertension. Further work-up showed only moderate increase of homocysteine level with negative features for lupus and others primary thrombophilic disease. The patient was managed adequately with thrombolytic and inotropic therapy; oral anticoagulation was started with improvement of his clinical status and was screened for pulmonary thrombo endarterectomy, but he refused. The case presented despite its evolution 'temporarily' positive perhaps related to the reduction of hemodynamic overload through bronchial arteries, reiterates the importance of early surgical intervention, before it establishes the hypertensive vasculopathy. Abnormal pulmonary function at rest and after exercise stress test associated to non invasive echocardiographic measurements are an excellent tool to identify the bad prognosis patients in CTEPH. We discuss the pathophysiology and conclude that in selected cases, pulmonary thromboendarterectomy is the best therapy, but only if executed early.
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PMID:Chronic thromboembolic pulmonary hypertension: take care to a "favourable" apparently evolution. A case report. 2206 57


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