[Pyogenic sacroiliitis: study of seven cases].
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Biomedical subjects
Publications and source records attributed to F Cardellach.
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Molecular, kinetic, and functional studies were carried out on erythrocytes and leukocytes in a Spanish male with G6PD deficiency, congenital nonspherocytic hemolytic anemia (CNSHA), and increased susceptibility to infections. G6PD activity was absent in patient's red cells and was about 2% of normal in leukocytes. Molecular studies using standard methods (WHO, 1967) showed G6PD in the patient to have a slightly fast electrophoretic mobility at pH 8.0 with otherwise normal properties (heat stability at 46 degrees C, apparent affinity for substrates, optimum pH, and utilization of substrate analogues). Other tests showed the patient's granulocytes to engulf latex particles normally, but to have impaired reduction of nitroblue tetrazolium and ferricytochrome-c as well as reduced iodination. Chemotaxis and random migration of the patient's granulocytes were normal as were myeloperoxidase, leukocyte alkaline phosphatase (LAP), and ultrastructural features. The molecular characteristics of G6PD in the patient differed from those of all previously reported variants associated with CNSHA, so the present variant was provisionally called G6PD Barcelona to distinguish it from other G6PD variants previously described. Possible mechanisms for the severe deficiency of G6PD in erythrocytes and granulocytes was investigated by studies on the immunologic specific activity of the mutant enzyme.
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The case histories of 1200 patients admitted to our hospital over a 20 month period were reviewed to determine the degree, frequency and cause of dissociated cholestasis as a biological syndrome. Patients were divided into two groups: group I with 80 cases, included all patients whose gamma-GT levels were more than 30 mU/ml and serum-bilirubin less than 1.2 mg/ml, with alkaline phosphatase levels between 90-180 mU/ml. Group II included those with alkaline phosphatase levels higher than 180 mU/ml (57 cases). All over incidence of dissociated cholestasis was 13.82%. Main causes in group I were infectious diseases, mainly pneumonias and urinary infections and congestive cardiac failure. In group II, neoplasias such as Hodgkin's disease and epithelial metastases and obstructions of the biliary tract such as vesicular or choledocal litiasis were the main causes. Transaminase levels underwent variable increases according to the different entities, without there being any difference between the two groups. The physiopathology as well as the anatomopathological aspects which could originate the syndrome are discussed.
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The hemophagocytosis or the process of ingestion of blood cells by phagocytes and macrophages of the mononuclear phagocytic system (MFS), is a phenomenon that rarely could be observed in the morphological examination of the bone marrow aspirate. Occasionally it is present in certain pathologic conditions such as, malignant histiocitosis, autimmune hemolytic anemia, or some chronic inflammatory diseases. The capacity of ingestion is not an exlusive property of the phagocytes and macrophages of the MFS, so that different neoplastic cells can show it too. This study analyzes the presence of hemophagocytosis by neoplastic cells in a total of 552 bone marrow aspirates corresponding to a series of 130 patients with acute leukemia and 422 patients with diverse solid tumors. In the group of patients with acute leukemia, hemophagocytosis by blastic cells was observed in five cases with acute monocytic leukemia. In the other group with solid tumors, hemophagocytosis was present in three patients with oat-cell lung carcinomas and diffuse bone marrow metastases. The interest of the evaluation of hemophagocytosis by neoplastic cells in the morphological examination of the bone marrow is stressed, as well as it possible value in the cytological diagnosis of acute monocytic leukemia. However, in these circumstances a sdiffuse metastases by solid tumors should be always discarded, since their cytomorphological characteristics are in most cases superimposed to those of the leukemic bone marrow infiltrate.