Enzyme deficiencies in the porphyrias.
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Biomedical subjects
Publications and source records attributed to S Kramer.
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Two consecutive studies have been performed by the Radiation Therapy Oncology Group on patients with metastatic brain tumors. Approximately 1,000 patients were entered into each trial. Treatment schedules of varying dose-time fractionations were used. Results were evaluated on the basis of improvement in general performance and neurologic function status. Survival also was recorded. Patients who received corticosteroids in conjunction with radiation therapy experienced a more rapid improvement in neurologic function than patients who did not receive steroid therapy. This was noted particularly in patients with poor neurologic function status. However, by the 4th week this difference had disappeared. Survival times were not altered by the addition of steroid therapy. Preliminary results of the second study suggest that ultra-short fractionation schedules are less effective than the longer ones used in the first study. A dose of 3,000 rads given in 10 equal fractions for a period of 2 weeks appeared to be the most satisfactory schedule for most patients with intracranial metastases.
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Oral methotrexate (MTX) was administered to a group of 48 patients with advanced head and neck tumors prior to radiation therapy in a nonrandomized manner. A second group of 75 similar patients were randomized to intravenous (i.v.) MTX plus radiation or radiation alone. Three-year survival rates by life table analysis show no significant statistical difference between i.v. MTX plus radiation or radiation alone. Those treated with oral MTX plus radiation have a statistically significant improved survival. The degree of tumor regression in the oral MTX group was correlated with survival. No similar correlation could be found in the intravenous MTX group. The use of either form of MTX correlated with a lower rate of distant metastasis.
One hundred patients with active tuberculosis were tested for tuberculin reactivity within 24 hours of their admission to the hospital. Commercial intermediate tuberculin, Tween stabilized intermediate tuberculin and the Tine test as well as a mumps antigen were applied simultaneously. False negative reactions were obtained in 28 per cent with Tine testing and in 21 per cent with Tween stabilized as well as plain tuberculin. These nonreactors were clinically identifiable as seriously ill with manifestations primarily attributable to protein depletion as a result of their illness. This is not specifically related to the effects of tuberculosis itself, but can be demonstrated in patients suffering the same sequelae of other debilitating illness. After two weeks of protein supplementation via a high calorie, high protein, hospital diet, skin reactivity was restored in the vast majority of these nonreactors. It is concluded that the lack of tuberculin reactivity on hospital admission probably results from impaired lymphocyte function in patients suffering serious protein depletion as a result of their illness; it is not attributable to deficiencies in the tuberculin test itself.
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Detailed child observational study reveals variable pathways by which the girl enters her Oedipus complex. The data of three normal girls are briefly detailed to demonstrate the path each took. The data reported do not support the generalizability of the 1925 postulate that the girl enters her Oedipus complex by way of her castration complex. It is hypothesized that the girl, as well as the boy, enters the Oedipus complex--given a favorable-enough "expectable environment"--from about two and a half years of age on, compelled by a psychobiologically determined gender-related change in her libido and inherited ego dispositions.
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The porphyrin precursor delta-aminolaevulinic acid can significantly decrease the resting membrane potential in a single fibre from the sartorius muscle of the frog Rana angolensis. This effect is dose-dependent and reversible.
The in-field long-term progressive response of bone marrow to localized fractionatedradiation to the thoracic spine was observed in weanling and adult rats. Total dose levels were 1800,3600 and 5400 rads given in daily fractions of 180 rads for 900 rads perweek, to simulate the clinical setting. Animals were sacrificed at 4 1/2, 6, 8, and 10months after irradiation, and bone marrow from within the treated area was ezamined. The juvenile marrow repopulated to higher levels of cellularity and after higher doses of radiation than the adult. The degree of marrow depletion, time of repopulation, and ultimate level of repopulation after cessation of irradiation were all dependent on the total dose. Some of the suggested clinical implications include re-evaluation of the lower doses given to children.
The porphyrin precursors beta-aminolevulinic acid (beta-ALA) and porphobilinogen (PBG) which accumulate, and are excreted in the urine in increased amounts during acute attacks of porphyria, were tested for their effects on reflex activity in the isolated hemisected spinal cords of Xenopus laevis. The two compounds were found to exert an inhibitory effect on monosynaptic ventral root responses, as well as on dorsal root responses (DRR) and dorsal root potentials (DRP). The latent period for inhibition of the monosynaptic response was longer than that for the DRR and DRP. The sensitivity of the preparations to the effect of the porphyrin precursors was subject to some seasonal variation. BETA-ALA and PBG did not effect conduction in isolated sciatic nerves at a concentration of 1 mg/ml.
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