Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Isolabeling segments were found in the distal region of the long arm of Y chromosomes derived from human leukocytes grown through two replication cycles in medium containing BrdU and stained by the FPG technique. Three main types of Y chromosome staining patterns were demonstrated: I-Y chromosome with typical SCD, II-Y chromosome with weakly stained distal regions of long arms (isolabeling segments), III-Y chromosome with both terminal regions displaying SCD interrupted by one isolabeled segment. The existence of different types of Y chromosome staining patterns was explained on the basis of the previously described hypothesis of unequal distribution of thymine residues between two DNA polynucleotide chains in the distal part of the long arms of human Y chromosomes.
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PMID:Isolabeling of the long arm of the human Y chromosome demonstrated by the FPG technique. 8 34

The direct staining of BrdU-substituted Chinese hamster chromosomes in a Na2HPO4-Giemsa solution without any pretreatments resulted in a B-dark type SCD istituted (TB) chromatids stained light. Detailed examinations of the staining process suggested that the Na2HPO4 solution acts to collapse chromosomes whereas the Giemsa dye works to reconstruct the collapsed chromosomes, and that during the reconstruction process preferential binding of the Giemsa dye to the BB-chromatids occurs to produce the B-dark SCD. It was revealed that not only the time but the temperature at which chromosome preparations are kept prior to use considerably affect the occurrence of SCD.
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PMID:Sister chromatid differential staining by direct staining in Na2HPO4-Giemsa solution and the mechanism involved. 9 41

The injury-vasospasm hypothesis of IHD was discussed in relation to coronary artery autoregulation and the anoxic-feedback mechanism. Observations in the recent literature, not usually attributed to spasm, were examined in light of this phenomenon. This includes reperfusion models of experimental AMI, the association of AMI with myocarditis, and findings in AMI and SCD as necrotic microlesions, prodromata, and epicardial arterial plaque rupture and hemorrhage. The disparity between the severity of coronary disease and the occurrence of the various types of IHD suggest that atherosclerosis itself does not precipitate attacks of chest pain. It was emphasized that plaque rupture due to spasm might help induce CAT. With exercise, the possible importance of the autoregulatory system was explored in the prevention and induction of AMI and SCD, and the improvement of AP. The role of spasm in IHD should be defined.
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PMID:The injury-vasospasm hypothesis of ischemic heart disease, revisited. 33 91

Cell adhesion and spreading were studied on sulphonated polystyrene dishes in serum-free saline (Mn, Na, Cl, buffer) i.e., without an intervening protein layer. Spreading as a function of surface charge density, SCD, peaked around 2-10 negative charges per square nanometer, corresponding to a monomolecular layer of sulphonate ions. At optimal SCD, macrophages, BHK-C13 and whole mouse embryo secondary cells all showed considerable spreading, even in monovalent saline-more so than on a conventional tissue-culture surface. But outside this narrow range of SCD, or on protein-coated surfaces, the divalent cation was indispensable. The biphasic effect of sulphonation on cell adhesion is consistent with the theory that a substratum need not be biochemically specific, provided it is physiochemically polar, rigid and dense. According to this theory, polystyrene of sub-optimal SCD would not be sufficiently polar, while supra-optimal sulphonation would produce a hydrogel surface, lacking in local rigidity and density, due to osmotic swelling. The principle of polymer exclusion, by a surface hydrogel layer, is also consistent with observations on the inhibitory effects of adsorbed proteins-viz., albumin, collagen, serum and cellular exudate, respectively-contrasted with the ready attachment of cells to a bare, optimally charged substratum, in this minimal in vitro system.
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PMID:Sulphonated polystyrene as an optimal substratum for the adhesion and spreading of mesenchymal cells in monovalent and divalent saline solutions. 40 1

A critical issue in the planning of better health care delivery for developing countries is the training and education of health personnel. The Center for Educational Development in Health (CEDH) has field tested its text, Systematic Course Design for the Health Fields, a manual on the application of competency-based curriculum development techniques. A competency-based approach to curriculum design derives instructional objectives, content, and evaluation from the on-the-job responsibilities for the health worker being trained. A field test was conducted in order to determine whether the systematic course design method was applicable in developing countries. For the purpose of the field test, 10 national field directors from Cameroon, Colombia, Honduras, Lebanon, Malaysia, Nepal, and Vietnam trained by CEDH in Boston taught teacher training workshops based on the Systematic Course Design model of a range of health professionals in their own countries. Evaluation of the teacher training workshops was conducted during and immediately following instruction and 1 year later. 167 men and women representing a range of 29 health professions, participated in the workshops. Participants designed 132 courses based on the SCD model and taught 54 courses to about 2000 students. In Colombia, Honduras, Malayasia, and Lebanon, the national field directors applied the method to a range of activities. These field directors developed and taught 11 additional teacher training workshops on SCD to 140 health professionals, and they provided consultation to 12 other health professional schools and governmental agencies. Subject to the limitations imposed by the data, it does seem that a systematic course design method can be applied in the developing world to train health professions teachers. A range of health professionals used the method to design and teach a variety of courses for their own instructional purposes. Further research on the long-range effect of the competency-based training program is required.
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PMID:Competency-based training of health professions teachers in seven developing countries. 44 99

In this study 21 adults with severe form of sickle cell disease (SCD; sickle cell anaemia, n = 15; Hb S/beta degree-thal, n = 6) were treated with hydroxyurea (HU) to assess the effectiveness of the drug in managing SCD. The individual dose was selected for each patient. The dose selection was based on the HU clearance study. Thereafter, the patients received daily doses of 15-20 mg/kg body weight. An evaluation data form was filled out at the monthly visit. The severity index (SI) of the disease was determined and haematological parameters including red cell indices, platelet counts, reticulocyte counts, irreversibly sickled cells, red cell deformability, Hb F, Hb F cells, total and direct bilirubin levels were measured prior to treatment, at follow-up intervals during treatment and after cessation of treatment. The trial period lasted 3 months. Statistically significant improvement was observed in the clinical presentation, haematological and biochemical parameters. Hb F level and F cells showed a significant increase in most patients, but to a variable degree. A major resultant effect was an increase in mean cell volume. Our experience shows that HU can be used for the treatment of severe forms of SCD with no major side effects, provided that the doses are monitored and that laboratory investigations are regularly undertaken.
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PMID:Hydroxyurea for the treatment of sickle cell disease. 129 5

A new computer system for genetic counseling in reciprocal translocations is described. This system, namely RCPc (RCP counseling) is a knowledge base extracted from a data base called SCD (Structural Chromosome Data) which contains 1376 families carrying reciprocal translocations. RCPc gives key information for each translocation which allows an evaluation of the risk of unbalance at birth and a prediction of the characteristics of potential unbalances. This information could provide a useful basis for deciding whether a prenatal diagnosis is required and if so, the preferred sampling method i.e. amniocentesis or chorionic villus sampling (CVS).
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PMID:Human reciprocal translocations: a new computer system for genetic counseling. 129 14

According to the FAB classification, a patient (case 1) could not be diagnosed as MDS-RA, although she had clinical features of MDS, as compared with another patient (case 2) who was diagnosed as RAS and had abnormal karyotype (20q- and 5q-) of bone marrow (BM) cells. BM cells of the two patients were SCD (sister chromatid differentiation) negative. Rearrangement of c-erbB and c-erbA was found in the genome of the BM cells in both patients, when southern blot hybridization was performed with probe v-erbB+A. Therefore, case 1 could be diagnosed as preleukemia. During a period about 3 years of treatment with the drug stanozolol in case 1 there was good effect and successful reversion was obtained. She had then normal hematologic and cytogenetic patterns of BM and PB and the rearrangement of c-erbB of BM cells also disappeared. She has worked for two years since then. The mechanism of effective treatment and successful reversion was discussed briefly. Probe v-erbB was shown to be useful in investigation of gene diagnosis of preleukemia or MDS (shown elsewhere).
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PMID:[Gene diagnosis and successful reversion in a patient with preleukemia]. 130 46

In mice, DMPS (250 mg/kg, i.v.) combined with diazepam (1.25 mg/kg, i.p.) could increase LD50 of p. o. SCD 5.3 times. DMPS (62.5 mg/kg, i.v.) antagonized completely the respiratory depression and neuromuscular blockade caused by SCD(7.5 mg/kg, i.v.) in rabbits. SCD (15 mg/kg, i.v.) caused tremor, tonic convulsion and the abnormal paroxysmal discharges in EEG in rabbits. DMPS (0.5 mg/kg, i.c.v) could not eliminate the abnormal paroxysmal discharges in EEG of rabbits. DMPS (62.5 mg/kg, i.v.) combined with diazepam (5 mg/kg, i.v.) completely and rapidly antagonize these toxic symptoms and the abnormal changes in EEG.
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PMID:[Antidotal effects of 2,3-dimercaptopropane-1-sulfonate sodium (DMPS) and combined with diazepam on acute poisoning caused by sodium ammonium dimethyl-2-propano-1,3-dithiosulfate monohydrate (SCD)]. 133 78

B lymphocytes from the spleens of normal (BALB/c) and autoimmune (MRL/lpr) strains of mice express the SCD-2 form of stearoyl-CoA desaturase as opposed to the SCD-1 form of the gene which is expressed in liver. However, whereas BALB/c T cells did not express SCD-1 or SCD-2, both BALB/c thymocytes and MRL/lpr T cells expressed SCD-2, suggesting a developmental down-regulation of SCD-2 within the T cell lineage. Northern analyses also revealed the expression of SCD-2 in the T cell lines BW5147, CTLL-2 and HT-2 and in BCL1, a B cell line. SCD-1 expression was not detected in any of the lymphoid cells tested. Finally, we show that SCD-2 gene expression is inhibited by arachidonic acid (20:4). These results demonstrate the complexity of SCD-2 regulation in lymphoid cells.
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PMID:Stearoyl-CoA desaturase gene expression in lymphocytes. 135 72


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