HLA antigens in polycythemia vera.
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Biomedical subjects
Publications and source records attributed to D Benjamin.
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Cryoglobulinemia with peripheral cryoglobulinemic neuropathy is reported in a patient with Gaucher's disease. To the best of our knowledge, a similar relationship has not been previously described. The question whether the cryoglobulinemic neuropathy in this patient is related to Gaucher's disease, or is a mere coincidence, is discussed.
A patient with chronic myelocytic leukemia in whom a clinical and hematological remission occurred after a single course of busulfan is described. The remission lasted for a period of 13 years and during that lapse of time, she gave birth to a healthy child. Afterwards, acute myeloblastic leukemia was diagnosed. Following treatment with rubidomycin and arabinoside C another remission was achieved, lasting already for 18 months.
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A family with genetic hypouricemia due to isolated renal tubular abnormality in urate handling is reported. Urinary urate excretion was decreased by 61% following administration of pyrazinamide, and increased by 25% following administration of probenecid. The response to these drug suggest genotype heterogeneity of renal hypouricemia in man.
Lymphocytes were obtained from a lymph node biopsy from a patient with chronic lymphocytic leukemia in whom B and T lymphocytes were present in equal percentages in the peripheral blood. The lymphocytes were examined for rosette formation with mouse and sheep erythrocytes in order to determine whether the leukemic process was of the B or T cell type.
Hyperlipemia was found in 46 of 118 polycythemia vera patients and in 20 of a control group of 115 healthy subjects. Myocardial infarction in the polycythemia vera group occurred in 14 of the 46 hyperlipemic and in 17 of the remaining 72 nonhyperlipemic patients. Repeated phlebotomies may induce hyperlipemia; therefore, this form of treatment may be potentially dangerous in polycythemia vera patients who are already hyperlipemic.
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A 37-year-old female was found to have hypouricemia (1.1-1.9 mg%) with markedly increased uric acid clearance (24.7039.5 ml/min). Uric acid excretion was only slightly affected by pyrazinamide, a drug which suppresses renal tubular uric acid secretion, and by probenecid, a drug which inhibits tubular uric acid reabsorption. The attenuated response in this subject to both drugs suggest a renal tubular defect in the proximal high capacity-high affinity uric acid reabsorption mechanism. No other renal tubular or metabolic abnormalities were detected. A survey of the family-three sisters and two brothers, revealed two similarly affected sisters. The abnormality described in this family is defined as familial renal hypouricemia due to an isolated renal tubular defect with attenuated response of uric acid clearance to probenecid and pyrazinamide.
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A series of amino acid reagents was tested on the glucose-6-P dependent D, and independent I forms of glycogen synthase (UDPG: glycogen alpha-4-glucosyltransferase, EC 2.4.I. II) from rabbit skeletal muscle, at two levels of purification. Whereas blocking of aliphatic hydroxyl groups did not result in any inhibition of the enzyme(s), blocking of aromatic hydroxyl groups resulted in a gradual and complete inhibition. Under the stated assay conditions both forms of the enzyme were similarly affected in terms of activity, but the tyrosines of the D form were found to react more readily chemically. Tyrosine appears to be "essential" for catalysis. No desensitization to the allosteric modulator glucose-6-P was detected.
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Hyperlipidemia was reported among 46 of 118 polycythemia vera patients as compared to a control group of 115 healthy subjects in whom hyperlipidemia was reported in 20. Among the 46 hyperlipidemic patients with polycythemia vera 14 had a myocardial infarction, while among the other 72 nonhyperlipidemic polycythemia vera patients myocardial infarction occurred amont 17. Since repeated phlebotomies may induce hyperlipidemia, this form of treatment of polycythemia vera may be potentially dangerous in already hyperlipidemic polycythemia vera patients.
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