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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The most important side effects of oral contraceptives (OCs) and their incidence, together with advice and monitoring of the patient at risk, are pointed out. There is a mild increase in blood pressure in longterm contraceptive use caused by increased angiotensinogen production by the liver. It is significant only for women with a history of familial hypertension, diabetes mellitus, or pre-eclampsia. Smoking increases this risk. Urinary tract infections are 25-50% more frequent in pill users. Glucose tolerance is slightly decreased. Contraceptives' diabetogenic effect is higher in women with hereditary tendency for diabetes, latent diabetes, and/or obesity. They are contraindicated in latent diabetes. Findings are contradictory in their effects on cholesterol and triglyceride serum level, but the pill is contraindicated in lipid metabolism disorders. There is an increased incidence in cholecystitis and cholelithiasis in pill-users (70-80 additional cases/100,000 user years). Liver diseases, intrahepatic cholestasis, occur rarely and benign liver tumors have not conclusively been proved to be caused by the pill. A variety of laboratory findings have been related to contraceptive use and drug interactions occur with barbiturates, rifampicin, hydantoin, and phenylbutazone. Blood coagulation is increased, partially by increased production of various blood coagulation factors; but more importantly, by a decreased synthesis of antithrombin III, a natural protective mechanism against intravascular coagulation. This increases thrombosis risk. Risk doubles with simultaneous cigarette smoking. Various epidemiological studies indicate a 5-10 fold increase in thromboembolism and thrombophlebitis, deep vein thrombosis, and pulmonary embolism. There is a correlation between contraceptive use and cerebrovascular disorders and myocardial infarction. This risk increases with age and years of pill use. The pill is contraindicated with symptoms of thrombophlebitis and thromboembolism, sickle cell anemia, proposed surgery, and longterm immobilization. Overall risk factors are not too high. Recommendations for rational pill use related to age are given and further contraindications are mentioned.
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PMID:[Adverse effects of oral contraceptives]. 55 52

A 25-year-old gravid woman, homozygous for sickle cell anemia, with a history of recent deep venous thrombosis, was examined using Tc-99m labeled red blood cell venography for recurrent thrombosis. Although negative for thrombus, the study presented an unusual incidental finding: the patient's peripheral bone marrow was hyperemic in a distribution consistent with peripheral red bone marrow expansion. Such a pattern has not been documented before using this technique. This report supports other literature that has demonstrated hyperemia of peripheral red bone marrow in other hemolytic anemias. This finding may ultimately define an additional role of scintigraphy in assessing the pathophysiologic status of the sickle cell patient.
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PMID:Hyperemic peripheral red marrow in a patient with sickle cell anemia demonstrated on Tc-99m labeled red blood cell venography. 203 25

The haemostatic balance can basically be described as the equilibrium between fibrin formation (coagulation) and fibrin lysis (fibrinolysis). The status of this balance may therefore be reflected by the products of these two processes. Until recently, the tests for assessment of fibrin(ogen) degradation products were performed in serum since they were based on polyclonal antibodies, which cross-react with fibrinogen. However, the use of serum introduces many artefacts so the utility of these serum tests is limited. New assays have now become available, which can be divided into quantitative enzyme immunoassays (EIAs) and semi-quantitative latex agglutination assays. The new assays can be carried out in plasma since they use highly specific monoclonal antibodies, the majority of which do not cross-react with fibrinogen. This makes it possible to avoid the serum artefacts. Furthermore, these plasma assays can discriminate between degradation products of fibrin and those of fibrinogen (FbDPs and FgDPs, respectively). The possible clinical utility of the new assays is discussed on the basis of literature data on the following clinical states: deep venous thrombosis (DVT) and pulmonary embolism, liver disease and liver transplantation, sickle cell disease, renal diseases, pregnancy and preeclampsia, disseminated intravascular coagulation (DIC), malignancy, coronary artery disease and thrombolytic therapy. Fibrinolysis appears to be accompanied by fibrinogenolysis. Detection of fibrin(ogen) derivatives may be used to rule out DVT and to monitor efficacy of anticoagulant treatment for DVT or DIC, and reflects severity of renal disease but not renal function. High levels of FgDPs were found during orthotopic liver transplantation and thrombolytic therapy. Fibrin(ogen) degradation products cannot be used to predict reperfusion following thrombolytic therapy. The fibrinolytic system remained active during normal and complicated pregnancy and in patients with malignancies. The new assays provide valuable information on fibrin(ogen)olysis in several diseases. More information on the haemostatic balance may be obtained by using these new assays for fibrin(ogen)olysis products in combination with assays for coagulation products.
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PMID:Monoclonal antibody-based plasma assays for fibrin(ogen) and derivatives, and their clinical relevance. 210 91

Trauma patients are at risk for thromboembolic complications, but effective methods of prophylaxis have not been established for this heterogenous population. In this prospective trial, 400 trauma patients were assigned to one of three groups, depending upon their injuries, and randomized within each group to a treatment mode: Group I: sequential gradient pneumatic leg compression (SCD), low-dose subcutaneous heparin (H), or control (C); Group II: H or C; Group III: SCD or C. Venous duplex ultrasound examinations were performed on admission and weekly thereafter. Of the 251 patients who completed the study, 15 (6%) developed lower extremity venous thrombosis and two additional patients developed pulmonary embolism (one fatal). Significant risk factors associated with the development of thromboembolism included immobilization > 3 days, age 30 years or older, and the presence of pelvic or lower extremity fractures. In patients with neurotrauma who cannot receive heparin (Group III), the SCD was more effective than control in preventing DVT (p = 0.057). Neither H nor SCD appeared to offer protection for the other groups of trauma patients, but surveillance with ultrasound examinations allowed for prompt recognition and treatment of occult deep vein thrombosis.
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PMID:Prevention of venous thromboembolism in trauma patients. 808 13

There is a lack of information on the use of contraceptives among women with sickle cell disease in the United Kingdom, and 150 babies are born with sickle cell disease every year. This study examines the questionnaire responses of 156 women who had sickle cell disease and were known to the hematology departments and sickle cell centers in north London. 42 of the women had sickle cell hemoglobin C disease; 12 women had sickle thalassemia disease, and 102 had homozygous sickle cell disease. 149 were sexually active. The mean age was 28.4 years. 100 of the women had 207 pregnancies of which 133 were unplanned. 25 women with 31 pregnancies miscarried. 31 women (39 pregnancies) terminated the pregnancies for social or medical reasons. 4 (3 with homozygous sickle cell disease and 1 with sickle cell thalassemia) of the 67 women, who used combined oral contraceptives, reported increased crises, and 2 women, with homozygous sickle cell disease, reported deep vein thrombosis. 54 of the 148 sexually active patients had been counseled not to get pregnant. Only 3 women said this advice influenced their plans. There were only 44 women (28%) who adequately understood the inheritance patterns of sickle cell disease although 94 (60%) knew about the possibility of prenatal diagnosis. Many factors affect decision- making including a poor obstetric history and rejection of abortion on moral grounds. 45% have used oral contraceptives at one time or another and 19% used the intrauterine device. This is similar to use patterns in North America. The medical community may to blame for the inadequate use of contraceptives, because of not knowing which contraceptives to recommend in these cases. There are no contraindications to prevent sickle-cell women from using oral contraceptives.
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PMID:Contraceptives, counselling, and pregnancy in women with sickle cell disease. 840 Sep 60

Deep venous thrombosis and subsequent pulmonary embolism due to venous pooling/stasis commonly occur in patients during hip and/or knee arthroplasty (i.e., replacement). This problem may be alleviated by using techniques to promote lower limb blood flow. Electrical stimulation-induced contractions have been shown to activate the skeletal muscle pump, promote limb blood flow, and may be effective for reducing venous pooling/stasis and edema. Therefore, electrical stimulation may reduce the incidence of deep venous thrombosis (DVT) and pulmonary embolism (PE) during and following surgery. The overall goal of this project was to evaluate the clinical efficacy of sequential electrical stimulation-induced leg muscle contractions on the venous blood flow during surgery. The degree of venous pooling/stasis was monitored via electrical impedance changes in the thorax. The changes in the patient's central hemodynamics were then calculated. Thirty patients were recruited and randomly assigned to either a control group (n = 15, mean age = 66.4 +/- 7.3) or experimental group (n = 15, age = 60.7 +/- 9.7). Both groups received the standard medical treatment for prevention of DVT (i.e., coumadin, heparin, etc.) and compression stockings (TED, Kendall). The control group used the sequential compression device (SCD + TED) and the experimental group used electrical stimulation (ES + TED). Electrical stimulation was applied via surface electrodes to the lower-limb muscles (tibialis anterior and gastrocnemius) and upper limb muscles (quadriceps femoris and hamstrings). These muscles contracted sequentially, using an eight-channel electrical stimulator. Four seconds of calf (contraction/compression) were followed by 7-s of calf and thigh (contraction/compression) interspersed by 60-s rest period during both electrical stimulation or sequential compression device. This cycle continued throughout the surgery (60-75 min) for both groups. At 15 min intervals, venous return was monitored by impedance cardiograph. Physiologic responses including ventricular stroke volume (SV), cardiac output (CO), heart rate (HR), total peripheral resistance (TPR), as well as mean arterial pressure (MAP) were monitored. These responses were statistically analyzed and compared throughout the surgery within each group and between the two groups. The results show stroke volume and cardiac output to be higher throughout surgery in the electrical stimulation group as compared with the sequential compression device group. The heart rate was consistently lower during electrical stimulation for both groups. Total peripheral resistance did not change in the electrical stimulation group; but increased in the sequential compression device group. The data suggest that continuous electrical stimulation-induced contractions could improve lower leg circulation by eliciting the physiologic muscle pump. This will lead to improved venous circulation and reduction of blood stasis during total hip and/or knee surgery. This technique may offer greater protection against DVT and PE during surgery than the commonly used sequential compression device.
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PMID:Electrical stimulation-induced contraction to reduce blood stasis during arthroplasty. 908 86

The prevalence of the prothrombin gene variant (allele 20.210 A), factor V Leiden mutation, and homozygosity for transition 677C-->T in the methylenetetrahydrofolate reductase (MTHFR) gene was determined among patients with sickle cell disease (SCD). The group included 73 patients with median age of 32.3 years with a diagnosis of sickle cell anemia in 53 patients, hemoglobinopathy SC in 16 patients, and four with S/beta(0) thalassemia. Vascular complications such as ischemic stroke or deep vein thrombosis were diagnosed in nine patients. Heterozygosity for the prothrombin gene variant or factor V Leiden mutation was identified in four patients. However, only one patient, who developed ischemic stroke, was identified as a carrier of factor V Leiden mutation. None of the patients presented homozygosity for the thermolabile variant of the MTHFR. These data suggest a low clinical impact of inherited hypercoagulability risk factors in developing thrombosis, occlusive stroke, or mortality data among patients with SCD in Brazil.
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PMID:Prothrombin mutant, factor V Leiden, and thermolabile variant of methylenetetrahydrofolate reductase among patients with sickle cell disease in Brazil. 972 76

We performed a crossover study to evaluate the haemodynamic effect of active dorsal to plantar flexion and seven pneumatic compression devices in ten patients who had a total knee arthroplasty. Using the Acuson 128XP/10 duplex ultrasound unit with a 5MHz linear array probe, we assessed the augmentation of peak venous velocity and venous volume above and below the junction of the greater saphenous and common femoral veins in order to study both the deep and superficial venous systems. The pneumatic compression devices evaluated included two foot pumps (A-V Impulse System and PlexiPulse Foot), a foot-calf pump (PlexiPulse Foot-Calf), a calf pump (VenaFlow System) and three calf-thigh pumps (SCD System, Flowtron DVT and Jobst Athrombic Pump). The devices differed in a number of ways, including the length and location of the sleeve and bladder, the frequency and duration of activation, the rate of pressure rise, and the maximum pressure achieved. A randomisation table was used to determine the order of the test conditions for each patient. The enhancement of peak venous velocity occurred primarily in the deep venous system below the level of the saphenofemoral junction. The increases in peak venous velocity were as follows: active dorsal to plantar flexion 175%; foot pumps, A-V Impulse System 29% and PlexiPulse 65%; foot-calf pump, PlexiPulse, 221%; calf pump, VenaFlow, 302% and calf-thigh pumps, Flowtron DVT 87%, SCD System 116% and Jobst Athrombic Pump 263%. All the devices augmented venous volume, the greatest effect being seen with those incorporating calf compression. The increases in ml/min were found in the deep venous system as follows: foot pumps, A-V Impulse System 9.6 and PlexiPulse Foot 16.7; foot-calf pump, PlexiPulse, 38.1; calf pump, VenaFlow, 26.2; calf-thigh pumps, Flowtron DVT 61.5, SCD System 34.7 and Jobst Athrombic Pump 82.3. Active dorsal to plantar flexion generated 8.5 ml for a single calf contraction.
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PMID:Venous haemodynamics after total knee arthroplasty: evaluation of active dorsal to plantar flexion and several mechanical compression devices. 985 3

Vaso-occlusive crisis is the most common cause of morbidity in patients with sickle cell anemia (SCA). Central nervous system involvement that leads to hemiplegia is the most frequent neurological complication in those patients. Peripheral deep venous thromboembolism was not reported in SCA patients. Activated protein C resistance is associated with an increased risk of thrombophilia. The authors report an SCA patient with recurrent cerebrovascular accident and deep venous thrombosis. Activated protein C resistance due to factor V Leiden heterozygous and heterozygocity for the methylenetetrahydrofolate reductase were diagnosed and suspected to be the risk factors that contribute to the development of the deep vein thrombosis in this SCA patient.
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PMID:Venous thromboembolism, factor V Leiden, and methylenetetrahydrofolate reductase in a sickle cell anemia patient. 1050 25

Hypercoagulability is frequently seen in the trauma patients. Debate continues over the best method of prophylaxis, diagnosis, and treatment for the trauma patient. From experience with orthopedic and general surgery patients, much has been learned about prophylaxis and diagnosis, and as treatment protocols have been taken from internal medicine literature. Universal guidelines relating specifically to the trauma patient have not, however, been established. Overall, most of the literature suggests using LMWH for the prophylaxis of trauma patients. When LMWH is contraindicated, SCD should be used, with AVFP as a second choice. Surveillance screening for DVT remains controversial, but surveillance before transfer to extended care facilities has proven beneficial. Finally, when DVT is diagnosed, treatment should be initiated as soon as possible and should be continued until the DVT has resolved. Long-term anticoagulation therapy or use of caval filters may be necessary to prevent the morbidity of PE or thrombophlebitic syndrome.
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PMID:Thrombosis risk in the trauma patient. Prevention and treatment. 1080 64


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