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O Shalev

Publications and source records attributed to O Shalev.

At least 37 records · Page 2Linked to original sources

GPI Mount Scopus--a variant of glucosephosphate isomerase deficiency.

Glucosephosphate isomerase (GPI) deficiency is an unusual cause of hereditary nonspherocytic hemolytic anemia. The disease, inherited as an autosomal recessive disorder, is most often manifested by symptoms and signs of chronic hemolysis, ameliorated by splenectomy. We recently diagnosed GPI deficiency in a 23-year-old Ashkenazi Jewish man who displayed the typical clinical course of this disorder. The biophysical characteristics of the GPI variant are slow electrophoretic mobility, presence of only one of the two bands normally present, and extreme thermolability. To the best of our knowledge, this is the first report of GPI deficiency in a patient of Jewish descent, and we propose to designate this enzyme variant "GPI Mount Scopus".

Adult↗

Posttransfusional hemolysis in recipients of glucose-6-phosphate dehydrogenase-deficient erythrocytes.

To test the hypothesis that transfusion of blood donated by individuals with glucose-6-phosphate dehydrogenase (G6PD) deficiency may result in a hemolytic reaction, we conducted a prospective longitudinal study in which 10 patients transfused with 1 unit of G6PD-deficient and 1 unit of normal red blood cells (RBC) were compared with 10 patients transfused with 2 units of age-matched normal RBC. We found that 24 h after transfusion serum bilirubin (mumol/l) in the recipients of G6PD-deficient RBC was significantly higher than in the recipients of normal RBC (36 +/- 14 vs. 18 +/- 5, respectively, p > 0.004). A parallel increase was found in the serum lactate dehydrogenase (LDH; IU/l) between the two groups (378 +/- 151 vs. 264 +/- 56, p < 0.001). The difference in serum bilirubin (26 +/- 10 vs. 15 +/- 5, p < 0.03) was still noted 48 h after transfusion, with only a marginal difference (p < 0.08) in LDH. We conclude that an immediate posttransfusional hemolytic reaction can occur in recipients of G6PD-deficient RBC and therefore suggest that the differential diagnosis of posttransfusional hemolysis, particularly in populations where G6PD deficiency is prevalent, includes transfusion of erythrocytes from G6PD-deficient donors.

Glucosephosphate Dehydrogenase Deficiency↗

Envenomation by Echis coloratus (Mid-East saw-scaled viper): a review of the literature and indications for treatment.

Envenomation by the snake Echis coloratus causes a local swelling and hemostatic failure. Most cases recover uneventfully, however about one-third of the victims bleed or develop anemia, and one known death due to renal failure has been reported. Uncontrolled observations suggest that treatment by a specific antivenom reduces the duration of the hemostatic failure. Still the management of victims of E coloratus remains uncertain. Some authors advocate antivenom treatment for all patients, while others recommend its use only in the event of complications. We review reported data on the effect of the venom in vitro, in laboratory animals and in humans, and reexamine alternative treatment strategies by applying a revised version of a published decision model. The probability of bleeding and the efficacy of antivenom treatment were the main determinants in the choice between antivenom treatment and expectant management of victims of E. coloratus. Assuming a therapeutic efficacy of 32%, the decision model favored antivenom treatment when the risk of bleeding exceeded 7.5%. The estimated risk of bleeding exceeds this threshold in patients who present with either proteinuria, a blood urea of > 7 mmol/l, a platelet count of < 100,000/microliters, or a hemoglobin level of < 13 g/dl. In patients who had been exposed to antiserum in the past, or in whom the annual probability of future envenomation exceeds 0.9%, antivenom treatment was preferred only when bleeding was certain. Errors in our estimates of the efficacy of antivenom treatment, of the mortality after a bleeding event and of the risk of anaphylaxis after a repeated exposure to antiserum may have affected our conclusions. Nonetheless, they are consistent with presently available information and, pending more reliable estimates, may be considered as guidelines for treatment.

Animals↗

Primaquine-induced superoxide production by beta-thalassemic red blood cells.

Primaquine, a prooxidant antimalarial drug, incubated with human red blood cells (RBC) induced marked superoxide generation in the cells as detected by exogenous cytochrome c reduction. In the presence of primaquine, beta-thalassemic RBC produced significantly more superoxide than normal RBC, thus reflecting the vulnerability of beta-thalassemic cells to oxidative stress.

Erythrocytes↗

Risk indicators after envenomation in humans by Echis coloratus (mid-east saw scaled viper).

To determine the frequency, severity and predictors of bleeding and azotemia after envenomation in humans by Echis coloratus, a retrospective survey of 68 cases in Israel between 1970 and 1989 was carried out. We used univariate and multivariate analyses of clinical variables on admission for the outcome variables of bleeding, hemoglobin and platelet levels, and blood urea. Within hours or days after envenomation, a major bleeding episode occurred in 18% of the victims, a drop in hemoglobin to 10 g/dliter or less in 14%, and an increase in blood urea to 9 mmole/liter or more in 15%. These complications correlated with time interval between envenomation and hospital admission, and the following admission variables: degree of bleeding, hemoglobin level, platelet and white blood cell counts, blood urea and proteinuria. Complications were unlikely in patients who were presented with all of the following: a hemoglobin level of 13 g/dliter or more, a platelet count of 100,000/mm3 or more, a blood urea level of 7 mmole/liter or less, no proteinuria and no bleeding. Treatment on admission with a specific monovalent antiserum was associated with a shorter duration of hemostatic failure and a reduced incidence of anemia and thrombopenia. Infusion of fresh frozen plasma on admission did not appear to be effective in preventing complications.

Adult↗

Falciparum malaria-induced hypoglycaemia in a diabetic patient.

We report a patient with diabetes mellitus who suffered severe falciparum malaria complicated by profound and persistent hypoglycaemia. The hypoglycaemia evolved before therapy with quinine was begun and resolved with eradication of the parasitaemia. The patient reverted to her baseline hyperglycaemia despite continuation of quinine. This case illustrates the critical role of falciparum malaria in the pathogenesis of malaria-associated hypoglycaemia, rather than quinine-mediated mechanisms. Anticipation of hypoglycaemia in falciparum malaria and its vigorous treatment may improve the poor prognosis associated with this complication.

Blood Glucose↗

[Viper envenomation by Echis coloratus].

Clinical and therapeutic experience with 24 cases of envenomation by Echis coloratus, the Mideast saw-scaled viper, is reported. These cases were seen between 1979-1989 at this hospital (Mt. Scopus). A clinical classification is proposed, based on severity of the bleeding diathesis and platelet count at presentation. It is suggested as a predictor of clinical outcome and as a guide to whether antiserum should be administered.

Antivenins↗

Multiple brain abscesses caused by Streptococcus bovis.

A case of multiple brain abscesses caused by Streptococcus bovis in a 28-year-old man is described. Infected bronchiectasis is presumed to be the source of infection. Streptococcus bovis infection is a rare cause of brain abscess. The aetiology and manifestations of this infection are discussed.

Adult↗

Cimetidine and ranitidine may not cross-react to cause thrombocytopenia.

We report the case of a patient with peptic ulcer disease who developed cimetidine-induced thrombocytopenia. The thrombocytopenia resolved spontaneously following discontinuation of cimetidine, and did not recur with ranitidine. We concluded that cimetidine and ranitidine do not necessarily cross-react to induce thrombocytopenia. Thus the exclusion of both H2-blockers in the event that one has caused thrombocytopenia may not be justified.

Aged↗

Long-term, low-dose aspirin is safe in glucose-6-phosphate dehydrogenase deficiency.

Forty-four patients with Mediterranean-type glucose-6-phosphate dehydrogenase (G-6-PD) deficiency receiving long-term, low-dose aspirin were monitored over three months for evidence of hemolysis. Complete blood count, reticulocyte count and serum bilirubin were normal in all patients before treatment and upon periodic retesting. We conclude that there is sufficient current evidence to remove the hemolytic stigma of aspirin in G-6-PD deficiency, thereby establishing its safety for long-term therapy in this condition.

Adult↗

Decreased erythrocyte (Ca2+ + Mg2+)-ATPase activity in hemodialyzed uremic patients.

Erythrocyte (Ca2+ + Mg2+)-ATPase activity and calcium content were determined in 15 uremic-hemodialyzed patients and 15 normal controls. A decrease in the activity of the enzyme (mean +/- SD = 65 +/- 7 vs. 79 +/- 12 mumol Pi/g Hb/h, p less than 0.001) and a parallel increase in the calcium content (17.2 +/- 6.4 vs. 5.1 +/- 4.2 mumol/L RBC, p less than 0.05) were found in the patients' erythrocytes when compared with those of the controls. It is proposed that malfunction of the calcium pump in hemodialyzed uremic patients is pathophysiologically significant in the accumulation of intracellular calcium. The increased intracellular calcium found in other tissues in uremia may be the result of the systemic malfunction of (Ca2+ + Mg2+)-ATPase in this disorder.

Adult↗

Hereditary spherocytosis associated with deletion of human erythrocyte ankyrin gene on chromosome 8.

Hereditary spherocytosis (HS) is one of the most common hereditary haemolytic anaemias. HS red cells from both autosound dominant and recessive variants are spectrin-deficient, which correlates with the severity of the disease. Some patients with recessive HS have a mutation in the spectrin alpha-2 domain (S.L.M. et al., unpublished observations), and a few dominant HS patients have an unstable beta-spectrin that is easily oxidized, which damages the protein 4.1 binding site and weakens spectrin-actin interactions. In most patients, however, the cause of spectrin deficiency is unknown. The alpha- and beta-spectrin loci are on chromosomes 1 and 14 respectively. The only other genetic locus for HS is SPH2, on the short arm of chromosome 8 (8p11). This does not correspond to any of the known loci of genes for red cell membrane proteins including protein 4.1 (1p36.2-p34), the anion exchange protein (AE1, band 3; 17q21-qter), glycophorin C (2q14-q21), and beta-actin (7pter-q22). Human erythrocyte ankyrin, which links beta-spectrin to the anion exchange protein, has recently been cloned. We now show that the ankyrin gene maps to chromosome 8p11.2, and that one copy is missing from DNA of two unrelated children with severe HS and heterozygous deletions of chromosome 8 (del(8)(p11-p21.1)). Affected red cells are also ankyrin-deficient. The data suggest that defects or deficiency or ankyrin are responsible for HS at the SPH2 locus.

Ankyrins↗

The origin of sickle cell alleles in Israel.

Molecular genetic studies were undertaken to determine the source of chromosomes carrying the sickle cell allele in Israeli patients. Analysis of restriction fragment length polymorphism (RFLP) patterns (haplotypes) along the beta-globin gene cluster was performed on 31 sickle chromosomes obtained from 10 unrelated families living in Israel. One is a Caucasian Jewish family, recently found to be carrying the sickle allele, and the other 9 are Arab families of various communities. The Jewish family, previously noted not to carry African red blood cell markers, was discovered to have the most common African haplotype of the beta-globin gene cluster, Benin. Similarly, 8 of the Arab families were also found to carry the Benin haplotype, whereas the ninth has the CAR (Central African Republic or Bantu) haplotype. The results suggest that sickle alleles in Israel originated in Africa, probably in two different regions, and migrated north into Arab and Jewish populations.

Africa↗

Treatment of envenomation by Echis coloratus (mid-east saw scaled viper): a decision tree.

Envenomation by Echis coloratus causes a transient hemostatic failure. Systemic symptoms, hypotension and evident bleeding are rare, with only one reported fatality. In this paper, we examine the decision to treat victims of Echis coloratus by a specific horse antiserum. The decision model considers the mortality of treated and untreated envenomation, and the side effects of antiserum treatment: fatal anaphylaxis, serum sickness and increased risk of death after a possible repeated exposure to horse antiserum in the future. The results of the analysis are not sensitive to variations in the probability of side effects of antiserum treatment. They are sensitive to variations in the risk of bleeding after envenomation, in the degree of reduction of this risk by antiserum treatment and in the risk of dying after an event of bleeding. Prompt administration of antiserum appears to be the treatment of choice if it reduces the risk of bleeding from 23.6% to 20.3% and if 1.6% or more of the bleeding events are fatal. We conclude that presently available data support antiserum treatment of victims of Echis coloratus who present with hemostatic failure, even though the advantage imparted by this treatment appears to be small.

Adult↗