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Biomedical subjects

C Hershko

Publications and source records attributed to C Hershko.

At least 109 records · Page 6Linked to original sources
PubMed1986

Paraprotein interference with colorimetry of phosphate in serum of some patients with multiple myeloma.

In untreated serum of three patients with multiple myeloma, concentrations of inorganic phosphate ranged from 130 to 270 mg/L as measured with a chromogenic assay based on the interaction of phosphate ion with ammonium molybdate in the presence of ferrous sulfate. There were no clinical features of hyperphosphatemia, and values for total calcium concentration in serum remained within normal limits throughout. Subsequent investigations demonstrated that this hyperphosphatemia was spurious and was caused by high concentrations of the paraprotein interfering with the chromogenic assay. Because this type of assay, adapted for automated systems, is now widely used in clinical laboratories, we call attention to this limitation to avoid confusion in clinical evaluations of patients with multiple myeloma.

Aged↗
PubMed1985

Combined use of zinc protoporphyrin (ZPP), mean corpuscular volume and haemoglobin measurements for classifying microcytic RBC disorders in children and young adults.

The diagnostic potential of the combined use of zinc-protoporphyrin (ZPP), mean corpuscular volume (MCV) and haemoglobin measurements for discriminating between iron deficiency anaemia, beta-thalassaemia minor and lead poisoning has been studied. Lead poisoning could be identified by ZPP greater than 50 micrograms/dl in the presence of normal MCV or ZPP greater than 150 micrograms/dl in the presence of microcytosis (MCV less than 80 fl) with a sensitivity of 97% and specificity 94%. Beta-thalassaemia minor was identified by the coexistence of microcytosis and ZPP less than 50 micrograms/dl with a sensitivity of 91% and specificity 79%. Iron deficiency anaemia defined by the combination of microcytosis and ZPP ranging from 50 to 150 micrograms/dl was identified with a sensitivity of 95%, but the specificity was only 51%, with many of the patients overlapping with thalassaemia minor. This problem did not exist in iron-deficiency anaemia with haemoglobin less than 10 g/dl as at that range no patients with uncomplicated thalassaemia minor have been encountered. A great advantage of the combined use of ZPP, MCV and haemoglobin for the initial screening of microcytic anaemia is its ease of performance and low cost. However, this information should only be regarded as presumptive evidence of disease, requiring subsequent confirmation by appropriate direct measurements such as transferrin saturation, serum ferritin, haemoglobin electrophoresis, or blood lead determinations.

Adolescent↗
PubMed1985

Increased leucocyte alkaline phosphatase and transcobalamin III in chronic myeloid leukaemia associated with lithium therapy.

A 38-year-old woman developed chronic myeloid leukaemia after 2 years of lithium carbonate therapy. A peculiar feature of her leukaemia, as well as of the 5 patients previously reported in whom CML has developed in the course of lithium therapy, was the unusually high degree of granulocyte maturation manifested in normal leucocyte alkaline phosphatase (LAP) score and, in 1 case, selective increase of transcobalamin III. Although a cause and effect relation between lithium therapy and CML has not yet been established, in view of the stimulatory effect of lithium on granulocyte proliferation, such treatment should be avoided in patients with established myeloproliferative disorders, or in patients at high risk of developing leukaemia.

Adult↗
PubMed1985

Atrioventricular and sinoatrial block in thyrotoxic crisis.

A 55 year old woman in thyrotoxic crisis developed atrial fibrillation, atrioventricular block, and sinoatrial block in rapid succession. All of these abnormalities resolved completely after antithyroid treatment. This course of events illustrates the profound effect of thyroid hormones on cardiac function. In view of the potential aggravation of atrioventricular conduction disturbance by beta adrenergic blocking agents, thyrotoxic patients should be carefully screened for electrocardiographic evidence of conduction disturbance before the administration of such drugs.

Electrocardiography↗
PubMed1985

Acute hemolytic anemia caused by severe hypophosphatemia in diabetic ketoacidosis.

Hypophosphatemia in diabetic ketoacidosis is well recognized, but is believed to be usually of moderate severity. We describe 2 patients in whom acute hemolytic anemia secondary to severe (0.19-0.35 mmol/l) hypophosphatemia has developed 1-2 days following treatment for diabetic ketoacidosis. Our experience indicates that severe hypophosphatemia requiring phosphate supplementation does occur in diabetic patients, and calls for increased awareness for the clinical and laboratory manifestations of this complication of diabetic ketoacidosis.

Acidosis↗
PubMed1985

Chronic cutaneous polyarteritis nodosa simulating recurrent thrombophlebitis.

A 30-year-old man who had 12 repeated hospital admissions within a period of 7 years for localized pain and swelling of the extremities is described. Excision biopsy of several subcutaneous lesions revealed histologic changes typical of polyarteritis nodosa (PN). Systemic disease was excluded because of a normal testicular biopsy, negative abdominal angiogram, and the lack of evidence for renal or other visceral involvement. Response to steroid therapy was excellent. Cutaneous PN is a distinct subset of polyarteritis with a chronic course and excellent prognosis. The present case underlines the need for a greater awareness, and for considering early biopsy of suspected lesions in patients with multiple episodes of apparent superficial thrombophlebitis.

Adult↗
PubMed1985

Multivariate analysis of prognostic factors in chronic lymphocytic leukemia.

Prognostic factors in a group of 90 patients with chronic lymphocytic leukemia were studied by methods of survival analysis. The relationship between survival and a set of demographic, clinical and laboratory variables, and identification of subsets of variables that are associated with survival, was tested by multivariate analysis, which is based upon Cox proportional hazards regression models in a stepwise procedure. Six variables showed significant correlation with survival: lymph node enlargement, splenomegaly, hepatomegaly, increased percentage (greater than 80%) of lymphocytes, hyperuricemia, and anemia. Stepwise analysis showed that the number of coexistent risk factors was a better predictor of survival than any single risk marker (P less than 0.001). Median survival of patients with 0 or 1 risk marker was 120 months; with 2 or 3, 96 months; with 4, 36 months; and with 5 or 6, only 24 months. Comparison of staging by number of risk markers with staging of the same patients by the Rai system showed a significant trend of decreasing survival with increasing number of risk markers within the same Rai stage. Staging by the number of coexistent risk markers is a simple and readily available method, which may complement existing methods to provide a more accurate assessment of prognosis in patients with chronic lymphocytic leukemia.

Adult↗
PubMed1985

Heart cells in culture: a model of myocardial iron overload and chelation.

The effect of iron loading and chelation was studied in heart cell cultures obtained from newborn rats. Radioactive iron uptake per 2 X 10(6) cells/24 hr was 3.8% for 59Fe-transferrin, 15.8% for 59Fe-ferric ammonium citrate (FeAC) at 20 micrograms Fe/ml in 20% serum, and 37.1% for 59FeAC at 20 micrograms Fe/ml in serum-free medium. About one third of the cellular radioactive iron was in ferritin and the rest in an insoluble lysosomal fraction. Iron uptake was almost completely inhibited by reducing the incubation temperature from 37 degrees C to 10 degrees C. Intracellular concentrations of malonyldialdehyde (MDA) were doubled after 15 minutes of iron loading and reached maximal concentrations at 3 hours. Conversely, iron mobilization by deferoxamine at concentrations ranging from 0.025 mmol/L to 0.3 mmol/L resulted in normalization of cellular MDA concentrations, in direct proportion to the amounts of iron removed. These findings indicate that cultured myocardial cells are able to assimilate large amounts of nontransferrin iron and that iron uptake and mobilization are associated with striking changes in lipid peroxidation as manifested by the respective increase and decrease in cellular MDA concentrations.

Animals↗
PubMed1985

Identification of community flour mills as the source of lead poisoning in West Bank Arabs.

Following the discovery of severe lead poisoning in members of several households in a West Bank village, studies were carried out to establish the magnitude of the problem in the community and to identify the source of lead poisoning. Forty-three patients with Centers for Disease Control risk group IV lead poisoning were identified and treated in three villages within a radius of about 10 km of each other. The prevalence of increased lead burden among 563 schoolchildren aged 10 to 18 years was 19% for Centers for Disease Control risk groups I and II and 11% for groups III and IV. A survey of potential sources excluded all items, except for locally ground flour, which was heavily contaminated in all affected households. Examination of community flour mills revealed that, in contrast to unprocessed grain, freshly ground flour contained large amounts of lead originating from lead fillings employed to fasten the housing of the driveshafts to the millstones. Systematic screening of 146 community stone mills in 92 West Bank villages showed significant lead contamination of flour in 33 mills (23%). In all cases, the source of lead contamination was identical. As methods of milling in the area are similar, a prompt investigation of this potential source of lead poisoning in other near-Eastern countries is indicated.

Adolescent↗
PubMed1985

Use of danazol in the management of chronic idiopathic thrombocytopenic purpura.

Danazol, an impeded androgen, was used for treating three patients with chronic idiopathic thrombocytopenic purpura (ITP) refractory to corticosteroid therapy. Sustained remission was achieved in one of the three cases. A temporary response was obtained in the second patient. In the last case, platelet count improved, but the patient died of severe interstitial pneumonitis. These observations confirm recent reports on the efficacy of danazol in the management of ITP and indicate that danazol treatment improves platelet counts in the majority of patients with refractory ITP, and in some cases may obviate the need for splenectomy.

Adolescent↗
PubMed1984

Familial hemophagocytic lymphohistiocytosis in Israel. II. Pathologic findings.

Presented are the pathologic findings in familial hemophagocytic lymphohistiocytosis (FHLH), based on observation of 11 cases from four affected families. The outstanding morphologic feature was a multiorgan involvement with lymphohistiocytic cellular infiltrates. The organs most frequently affected were the bone marrow and lymph nodes and, less frequently, the liver and the brain. The histiocytes in the infiltrates were usually bland and displayed prominent hemophagocytosis. The differential diagnosis of FHLH and the difficulty in distinguishing it from malignant histiocytosis is discussed. In view of the focal distribution of lesions, it is suggested that histopathologic diagnosis of FHLH be made only after evaluation of combined findings in several organs and consideration of the patient's family history as well as the result of the clinical and laboratory investigations.

Bone Marrow↗
PubMed1984

Familial hemophagocytic lymphohistiocytosis (FHLH) in Israel. I. Description of 11 patients of Iranian-Iraqi origin and review of the literature.

Eleven patients with familial hemophagocytic lymphohistiocytosis (FHLH) are described. They all belonged to four Jewish families of Iranian and Iraqi origin. Parental consanguinity was found in three families. The age of onset of disease ranged from 6 weeks to 36 months. All patients had fever, wasting, and enlargement of the liver and spleen. In addition, lymph-node enlargement and neurologic complications were common. The most consistent laboratory findings were pancytopenia, atypical lymphomonocytoid cells in the peripheral blood, abnormal liver function test results, and increased cerebrospinal fluid protein. The course was fatal in all patients. Nine of the 11 patients died within 2 weeks to 3 months of presentation, and 2 patients achieved temporary remissions but died of disease within 8 and 24 months, respectively. Response to antibiotic therapy or to the administration of corticosteroids and cytotoxic drugs was unimpressive. Pancytopenia complicated by sepsis or bleeding, hepatic failure, or encephalopathy were the terminal events. This report draws attention to the existence of FHLH in Jews of Iranian-Iraqi origin in whom parental consanguinity is very common.

Child, Preschool↗