Circulating anticoagulant in patients with Gaucher's disease?
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Biomedical subjects
Publications and source records attributed to M Shaklai.
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Migration inhibition factor (MIF) activity, expressed as a migration index, was studied in the sera of 48 chronic lymphatic leukemia (CLL) patients and 48 healthy controls. MIF activity was detected in the sera of 50% of the CLL patients. The medical condition of patients in advanced clinical stages (III and IV) and with detectable MIF activity was more stable (after 18-mo follow-up) than was that of the patients in advanced stages but without detectable MIF activity. No relationship was found between the clinical stage of the disease, absolute lymphocyte count, and MIF activity.
An acute myocardial infarction was observed in a 62-year-old patient with hemophilia A, as well as myxedema, hypertension, obesity, hypercholesterolemia and angina pectoris. The occurrence of myocardial infarction in hemophiliacs is rare, and, to the best of our knowledge, this patient represents the fourth documented case in the literature.
Significantly higher density of intramembranous particles (IMP) was noted in the endothelial plasma membrane facing the blood-front compared to the tissue-front. The difference may be related to the function of the vascular endothelium as an active barrier between the blood and the tissue.
A case of myeloschisis in a six weeks old human embryo is reported. The 39-year-old mother was treated by busulfan before and during the early stages of gestation for chronic lymphatic leukemia. Additional histological findings consisted in a decrease of mesenchymal elements together with a somitic disorganisation in the affected area of the embryo. In spite of the well known experience that this drug is well tolerated by leukemic pregnant women, a possible interference of busulfan with the oocyte differentiation in this elderly primipara is proposed.
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This study is an ultrastructural analysis of haemopoiesis in W/Wv anaemic mice in whom the anaemia is caused by a defect in the haemopoietic stem cell. Cellularity of marrow is normal but there is an increase in the proportion of less mature cells suggesting a delay in maturation. In addition, heterochromatin constitutes a higher proportion than normal of the nucleus in most stages of maturation suggesting a defect in DNA activation. The findings suggest that in this strain not only the differentiation of the haemopoietic stem cell but also the maturation of the committed cell is defective.
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This study is an ultrastructural analysis of hemopoiesis in S1/S1d anemic mice. The anemia in this strain is caused by a defect in hemopoietic microenvironment which fails to support hemopoiesis. Marrow cellularity is reduced and vascular channels are dilated. Coverage of sinus endothelium by adventitial cells is nearly twice as in normal mice, suggesting a reduced rate of cell traffic into the circulation. Central macrophages are small with little or no interdigitation with erythroid cells and no crystalloid inclusions as seen in normal littermates. The findings suggest that a fundamental abnormality in the central macrophage may be responsible for the defect in hemopoietic microenvironment.
Membrane characteristics of cultured endothelial cells derived from the superior vena cava of the rabbit were studied in freeze-fracture replicas. Gap junctions were identified, but tight junctions were not seen. In addition to pinocytic vesicles, fenestrations of plasma membrane which are not normally seen in the endothelium of vena cava in situ were found. This finding may be explained by the absence of platelets in the culture medium and is consistent with the current concept of endothelial-platelet interaction.
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A method for obtaining a uniform, dense precipitate of lanthanum nitrate to delineate extracellular space is described. Improvement of the previous technique is achieved by phosphate precipitation of lanthanum in the tissue carried out at low temperature. This method has been successfully applied to bone marrow.
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Transatrial membranotomy was performed in a patient with membranous obstruction of the hepatic segment of the inferior vena cava, manifesting as longstanding varicosity and edema of the legs, chronic type of Budd-Chiari syndrome, liver cirrhosis and hypersplenism. The course in this patient and similar ones reported in the literature suggests a conservative approach.
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