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Biomedical subjects

Y Shoenfeld

Publications and source records attributed to Y Shoenfeld.

At least 19 recordsLinked to original sources

A new player in the antiphospholipid syndrome: the beta 2 glycoprotein I cofactor.

The study of antiphospholipid (aPL) antibodies has been greatly developed in recent years and conclusive evidence now exists concerning the correlation between aPL and clinical signs such as thrombosis, thrombocytopenia, abortion, and fetal loss. Several hypotheses have been put forward concerning the pathogenic mechanism of aPL, but none has received final confirmation from experimental data. Many studies have been devoted to characterizing the antigens recognized by the different aPL autoantibodies and to a cofactor involved in the binding of autoantibodies and phospholipids; this cofactor has been identified as an apolipoprotein, the beta 2 glycoprotein I (beta 2GPI) or APO-H. Direct evidence now exists which suggests that both the beta 2GPI and the phospholipid comprise the epitope to which aPL are directed. On the other hand anti-beta 2GPI antibodies have been identified in sera of patients suffering from SLE and primary Antiphospholipid Syndrome. beta 2GPI is normally present in human plasma/serum and possesses numerous inhibitory functions in multiple coagulation pathways. The amino acid sequence of beta 2GPI has been identified and found to consist of five repeating units that belong to the complement control protein (CCP) superfamily. This development of knowledge related to aPL has followed three steps respectively: 1. the standardization of the techniques of detection: 2. identification of the clinical signs related to the autoantibodies: and finally 3. the discovery of a new player, the beta 2GPI cofactor.

Animals

Plasma cell dyscrasia. Analysis of 423 patients.

Present clinical and laboratory diagnostic criteria permit a more accurate diagnosis and closer follow-up of patients with plasma cell dyscrasias. A ten-year follow-up of a group of 423 patients showed that the indications for and the adjustment of treatment are more precise when these criteria are summarized into profiles based on each diagnostic category. M components may be an indication of the presence of another sometimes nonreticular malignant neoplasm. The improvement of the specificity and sensitivity of immunologic methods sheds additional light on mechanisms controlling the synthesis of homogeneous antibodies such as prevalence of IgM-K in mixed cryoglobulinemia and lambda-light chains in IgD myeloma, excretion of lambda-Bence Jones proteins in amyloidosis, and greater IgG-subclass restriction in multiple myeloma as compared with benign monoclonal gammopathy. The activation of additional clones (biclonal gammopathies) was found in 3% of our patients.

Adult

The effect of exposure to heat on intraocular pressure.

During exposure to heat there is an increase of the intraocular pressure in nonacclimatized persons. This increase is not found after heat acclimatization has been carried out. The increase of the intraocular pressure in the nonacclimatized persons can be attributed to the increase of the body temperature due to insufficient sweating.

Acclimatization

Depressed PHA transformation and acute leukemia in macroglobulinemia.

Two out of fifty-three patients with macroglobulinemia developed acute leukemia following chemotherapy. The Phytohemagglutinin (PHA) Transformation Index performed prior to the appearance of acute leukemia was found to be markedly depressed in these two patients in comparison with ten other patients with macroglobulinemia. In addition, a clone with monosomy 7 was detected in one of the currently reported patients when the leukemic process became apparent. The cytogenetic analysis was normal in the same patient 48 months earlier, when macroglobulinemia was diagnosed. The low PHA Transformation Index and the chromosomal hypodiploidy are of interest and their clinical significance merits further investigation.

Adolescent

HLA antigens in genetic neutropenia of Yemenite Jews.

The HLA antigenic system was studied in Yemenite Jews with genetic neutropenia. No deviation in antigen frequency was observed when compared with matched controls from the same ethnic group. HLA antigen frequency and common haplotypes of the Yemenite Jews were found entirely different from those of the African blacks, known to have the same anomaly.

Adult

Pulmonary alveolar proteinosis associated with fanconi's anemia.

A 16-year old patient with Fanconi's anemia developed pre-leukemia and pulmonary infiltration which was found upon autopsy to be pulmonary alveolar proteinosis. The question whether the pulmonary alveolar proteinosis was due to the marked leukopenia and an eventual defective phagocytic ability of the leukocytes is raised.

Adolescent

Heat intolerance in former heatstroke patients.

Nine young men who had suffered from heatstroke on previous occasions (heat-intolerant subjects) and 10 young volunteers (control subjects) were examined to determine their physiologic responses to exercise in temperate (23 degrees C) and hot environments (40 degrees C). The tests included an orthostatic test, work loads of 40 W and 80 W, and oxygen consumption (Vo2) determination. Although all the control subjects completed the exercise under severe heat load (3 h), none of the heat-intolerant subjects succeeded in completing this test due to high rectal temperatures and high heart rates. Sweat rates were similar in both groups, with Vo2 slightly higher in the control subjects. Orthostatic responses were similar in each group. The results suggest that inefficient thermoregulation, possibly due to decreased heat conductance from core to periphery, contributes to heat intolerance in former heatstroke patients.

Adult

Systemic lupus erythematosus associated with mixed cryoglobulinaemia and circulating anticoagulant.

We describe a 30-year-old woman with systemic lupus erythematosus (SLE), in whom the only evidence of the disease, during the 10 years before diagnosis was established, was an accelerated erythrocyte sedimentation rate and a positive Wassermann test. Her disease was characterized by the presence of an anticoagulant without indication of bleeding, mixed cryoglobulinaemia, steroid-resistant renal damage, and persistent false-positive tests for syphilis. The relationship between these results and the presence of anticoagulant, mixed cryoglobulinaemia and renal damage in patients with SLE are discussed.

Adult

Cold-induced profuse sweating on back and chest. A new genetic entity?

Two sisters whose parents shared a grandfather had cold-induced sweating. Since childhood they had sweated profusely from the back and chest when exposed to environmental temperatures of 18 degrees to 7 degrees C. They had additional abnormalities--e.g., high palate and inability fully to extend the elbows--which neither their parents nor their sibs shared. The cold-induced sweating, which could not be stopped by a beta-adrenergic blocking agent, was abolished by postganglionic blockade with atropine sulphate. This indicates the possibility of a peripheral mechanism.

Administration, Oral