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)

Caution is called for in providing family planning counseling and contraceptive prescriptions for women with hematological disorders. Iron deficiency anemia is a common problem among women of reproductive age. During menstruation women's need for iron intake is 3 times that of men. Oral contraceptives (OCs) are an appropriate contraceptive choice for iron deficiency anemia patients since OCs are associated with reduced blood loss during menstruation. Most IUDs, and especially unmedicated and copper bearing devices, should not be used by women with iron deficiency anemia. Progestin releasing IUDs tend to increase hemoglobin and serum ferritin levels, therefore, patients with iron deficiency anemia may benefit from progestin releasing IUD insertions. Women with hemorrhagic disorders, such as hemophilia, purpuras, and platelet number and function disorders frequently experience menorrhagia. OCs are an appropriate contraceptive for many patients with these disorders. Several studies indicate that patients with hemorrhagic disorders frequently experience reduced bleeding problems when they use OCs. IUDs are contraindicated for women with hemorrhagic diseases because IUDs may increase blood loss. Women with sickle cell hemoglobinopathies need careful counseling. Pregnancy for these women entails high morbidity and mortality risks. Series data shows that pregnant women with sickle cell hemoglobinopathies have a 4%-100% risk of maternal morbidity and a 1%-35% risk of maternal mortality. The risk of maternal morbidity and mortality is equally high for women with hemoglogin sickle cell disease but somewhat lower for women with sickle cell thalassemia. Women with these diseases should be informed about the risks associated with pregnancy. These patients may want to consider sterilization. Oral and IUD contraceptives are contraindicated for patients with these disorders; the former, because it may have a thromboembolic effect, and the latter, because it is associated with high blood loss. There are some reports that progesterone protects against sickling, but more intensive studies must be undertaken before progesterone can be recommended for women with sickle cess disorders. If patients insist on using an OC, a minipill may be prescribed. Barrier methods are probably the best choice for sickle cell disorder patients.
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PMID:Patients with hematologic disorders need careful birth control counseling. 1226 20

Since sickle cell disease places women at higher risk of maternal and fetal mortality and morbidity, effective contraception is especially important. The first choice for women with sickle cell disease may be depot medroxyprogesterone acetate (DMPA). In addition to being a highly effective, reversible contraceptive, DMPA also may prevent the painful sickling crises that occur when red blood cells clog the small vessels. Use of oral contraceptives (OCs) by women with sickle cell disease remains controversial. No case-control or prospective cohort studies have examined the relationship between OCs and the formation of blood clots in women with sickle cell disease, but observational studies have failed to detect evidence of adverse effects. Norplant implants do not appear to alter the blood of women with sickle cell disease, but they do not confer the DMPA-associated benefits. Progestin-releasing IUDs, which are not associated with the increased blood loss common with copper IUDs, are another option. A study conducted in Ghana concluded that a switch from barrier methods to more reliable methods such as DMPA, OCs, and sterilization could substantially reduce pregnancy-related death and illness among women with sickle cell disease in sub-Saharan Africa.
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PMID:DMPA good choice for women with sickle cell. 1229 53

Steroids hormones modify the hematological features of homozygous sickle cell disease, including the levels of fetal hemoglobin. We used semi-quantitative RT-PCR analysis of GATA-1, GATA-2, NF-E2, and gamma-globin mRNA levels in a two-phase liquid culture system of human adult erythroid cells in order to assay the effect of progesterone upon gene expression. The levels of expression of GATA-1 and gamma-globin mRNA were significantly increased in cells treated with progesterone compared to untreated cells (1.7- to 2.0-fold). Progesterone treatment did not produce any stimulatory effect upon GATA-2 and NF-E2 mRNA expression. Differences in the synthesis of HbF protein could not be detected by flow cytometry, although we observed a small difference in mean intensity fluorescence between cells treated and cells untreated with progesterone on days 7 and 9. Using anti-transferrin receptor and anti-glycophorin A antibodies, we verified that addition of progesterone did not cause any change in erythroid proliferation and differentiation. In conclusion, it is possible that the increased expression of gamma-globin mRNA after progesterone treatment observed in this study may be related to the increased GATA-1 mRNA expression. Interactions of the steroid receptors with the basal transcriptional machinery and with transcription factors might mediate their transcriptional effects.
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PMID:Progesterone upregulates GATA-1 on erythroid progenitors cells in liquid culture. 1249 Feb 88

Primary care physicians often prescribe contraceptives to women of reproductive age with comorbidities. Novel delivery systems (e.g., contraceptive patch, contraceptive ring, single-rod implantable device) may change traditional risk and benefit profiles in women with comorbidities. Effective contraceptive counseling requires an understanding of a woman's preferences and medical history, as well as the risks, benefits, adverse effects, and contraindications of each method. Noncontraceptive benefits of combined hormonal contraceptives, such as oral contraceptive pills, include regulated menses, decreased dysmenorrhea, and diminished premenstrual dysphoric disorder. Oral contraceptive pills may be used safely in women with a range of medical conditions, including well-controlled hypertension, uncomplicated diabetes mellitus, depression, and uncomplicated valvular heart disease. However, women older than 35 years who smoke should avoid oral contraceptive pills. Contraceptives containing estrogen, which can increase thrombotic risk, should be avoided in women with a history of venous thromboembolism, stroke, cardiovascular disease, or peripheral vascular disease. Progestin-only contraceptives are recommended for women with contraindications to estrogen. Depo-Provera, a long-acting injectable contraceptive, may be preferred in women with sickle cell disease because it reduces the frequency of painful crises. Because of the interaction between antiepileptics and oral contraceptive pills, Depo-Provera may also be considered in women with epilepsy. Implanon, the single-rod implantable contraceptive device, may reduce symptoms of dysmenorrhea. Mirena, the levonorgestrel-containing intrauterine contraceptive system, is an option for women with menorrhagia, endometriosis, or chronic pelvic pain.
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PMID:Contraception choices in women with underlying medical conditions. 2176 49

Progestins induce lipid accumulation in progesterone receptor (PR)-positive breast cancer cells. We speculated that progestin-induced alterations in lipid biology confer resistance to chemotherapy. To examine the biology of lipid loaded breast cancer cells, we used a model of progestin-induced lipid synthesis. T47D (PR-positive) and MDA-MB-231 (PR-negative) cell lines were used to study progestin response. Oil red O staining of T47D cells treated with progestin showed lipid droplet formation was PR dependent, glucose dependent and reduced sensitivity to docetaxel. This protection was not observed in PR-negative MDA-MB-231 cells. Progestin treatment induced stearoyl CoA desaturase-1 (SCD-1) enzyme expression and chemical inhibition of SCD-1 diminished lipid droplets and cell viability, suggesting the importance of lipid stores in cancer cell survival. Gas chromatography/mass spectroscopy analysis of phospholipids from progestin-treated T47D cells revealed an increase in unsaturated fatty acids, with oleic acid as most abundant. Cells surviving docetaxel treatment also contained more oleic acid in phospholipids, suggesting altered membrane fluidity as a potential mechanism of chemoresistance mediated in part by SCD-1. Lastly, intact docetaxel molecules were present within progestin induced lipid droplets, suggesting a protective quenching effect of intracellular lipid droplets. Our studies suggest the metabolic adaptations produced by progestin provide novel metabolic targets for future combinatorial therapies for progestin-responsive breast cancers.
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PMID:Progestin modulates the lipid profile and sensitivity of breast cancer cells to docetaxel. 2292 95