1/5. Clinical and physiologic studies of two siblings with prekallikrein (Fletcher factor) deficiency.Two siblings with hereditary Fletcher factor (prekallikrein) deficiency were studied for alterations of fibrinolysis, platelet function, skin inflammatory responses, permeability factor (PF/dil) formation and leukocyte chemotaxis. In vivo stimulation of fibrinolytic activity was normal; the bleeding time and platelet functions (adhesivity, aggregation, release reaction) were also normal. Both immediate (wheal-flare reaction to histamine, bradykinin, prostaglandin E1, physical agents) and delayed sensitivity skin test reactions were within normal limits. Migration of subjects' leukocytes to attractants in skin windows and in Boyden-type chambers was the same as that of control leukocytes. serum complement components were essentially normal. One subject's leukocytes showed normal tissue factor production on stimulation by endotoxin, although prekallikrein deficiency did impair the endotoxin-stimulated generation of serum procoagulant activity. PF/dil caused increased vessel permeability in human skin; in vitro generation of PF/dil required both the Hageman factor and prekallikrein. The Fletcher factor-deficient subjects responded in a normal manner to PF/dil. Based on the Fletcher factor-coagulation assay, the biologic half-disappearance time of prekallikrein (after the transfusion of normal plasma in one of the subjects) was estimated at 35 hours. Therefore, these studies suggest that severe prekallikrein (Fletcher factor) deficiency in man is not associated with any clinically significant impairment in hemostasis, fibrinolysis, inflammatory responses or leukocyte function.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/5. A case of head and neck kaposiform hemangioendothelioma simulating a malignancy on imaging.Kaposiform hemangioendothelioma (KH) is an endothelial-derived spindle cell neoplasm often associated with kasabach-merritt syndrome. Most cases arise in infancy and childhood and are soft-tissue tumors. The tumor displays an appearance between capillary hemangioma and Kaposi's sarcoma. We report a case of KH in a 1-year-old girl involving a mass that showed abnormal enhancement of soft tissue superficial to the right temporal bone with partial destruction of the temporal bone, the temporomandibular joint, mandibular condyle, and occipital bone. The physical finding of a discolored mass led clinicians to consider a hemangiomatous lesion, whereas the radiological picture suggested a more aggressive diagnosis of rhabdomyosarcoma and aggressive fibromatosis.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/5. Acute anterior myocardial infarction as first manifestation of acute myeloid leukemia.A 42-year-old man was admitted with heavy retrosternal pain lasting 30 min. electrocardiography showed typical signs of acute anterior myocardial infarction. The patient reported only attacks of coughing for a couple of days, and no serious diseases. The physical examination was normal. Laboratory tests showed a white blood cell count of 45/nl, platelet count of 58/nl, and hemoglobin of 14.4 g/dl. Blood chemistry showed elevated lactic dehydrogenase (413 U/l) but no elevation in creatine phosphokinase or glutamic-oxaloacetic transaminase. Therefore no thrombolysis was administered, but coronary angiography was performed. This showed a long-distance, subtotal thrombotic occlusion of the left anterior descending artery. After percutaneous transluminal coronary angioplasty and implantation of serial stents a normal perfusion of the artery was observed. The patient's blood and bone marrow films revealed acute myeloid leukemia FAB M2. Various conditions can cause a myocardial infarction in leukemias. We discuss the clinical management and the possible reasons for a subtotal thrombotic occlusion of the coronary artery.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/5. Cerebrovascular disease and antiphospholipid antibodies in systemic lupus erythematosus, lupus-like disease, and the primary antiphospholipid syndrome.PURPOSE AND patients AND methods: Antiphospholipid antibodies, lupus anticoagulant antibodies to cardiolipin, and a false-positive result on testing for syphilis have been linked to thrombotic vascular occlusions, particularly in patients with systemic lupus erythematosus (SLE) or lupus-like disease, i.e., patients not fulfilling four American Rheumatism association criteria for the classification of SLE. The clinical and serologic features of 35 patients with cerebrovascular disease (strokes/transient ischemic attacks) who demonstrated antibodies to phospholipids are presented. Complete histories were obtained from all 35 patients, and all underwent routine physical examinations, radiography, electrocardiography, computed tomographic brain scanning, and immunologic studies. Psychometric tests were performed in nine patients. RESULTS: The strokes were often multiple and were followed by multi-infarct dementia in nine patients. Of particular interest were 10 patients in whom the presence of antiphospholipid antibodies was the major and often the sole immunologic disturbance present. Several of these patients were antinuclear antibody-negative, and the antinuclear antibodies, when present, were usually of a low titer (1:40 to 1:160). These patients conform to a group classified as having a primary antiphospholipid syndrome. CONCLUSION: Antiphospholipid antibodies are strongly associated with cerebrovascular occlusions in patients with SLE as well as in those with lupus-like disease and the primary antiphospholipid syndrome. All patients with any of these conditions who present with vascular events should be screened for these antibodies, as their occurrence may have a bearing on future therapy.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
5/5. Making the diagnosis when the patient has 'blue toes'.Painful "blue toes" describes a physical sign whose meaning may be discovered through a carefully taken history, physical, and laboratory evaluation. Accurate diagnosis is important, because therapy for one specific condition may be contraindicated for another. When embolism is suspected, ultrasound examination of the heart, aorta, and periphery may be useful prior to angiography in order to avoid exacerbation of cholesterol crystal embolization. Transesophageal echocardiography or MRI may be needed to exclude a thoracic aortic source. The differential diagnosis can be divided into three categories: emboli from the cardiac and arterial system, acquired hypercoagulability disorders, and syndromes that lead to peripheral vascular pathology. A clinical approach to the evaluation of patients is presented, with case reports.- - - - - - - - - - ranking = 2keywords = physical (Clic here for more details about this article) |