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

Sickle cell anemia, a congenital hemolytic type of anemia due to a genetic defect in the beta chain of the globin molecule can cause severe disease. During pregnancy, the risk for preeclampsia and deep venous thrombosis is increased in patients with sickle cell anemia. Occlusion of placenta blood vessels with rigid deformed erythrocytes can cause repeated miscarriages and intra-uterine fetal death. Repeated blood transfusions can prevent these complications by reducing the concentration of abnormal hemoglobin S. We report on the evolution of five pregnancies in three patients with sickle cell anemia who received multiple blood transfusions during gestation, and discuss advantages and risks involved in the care of such cases.
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PMID:[Sickle cell anemia and pregnancy: considerations on systematic prophylactic transfusion]. 1131 68

Barrier methods of contraception and natural family planning may pose unacceptable risks of unintended pregnancy for women with medical conditions in which pregnancy could be dangerous. Although more effective at preventing pregnancy, hormonal methods may affect the course of a chronic disease. The table that comprises this article outlines contraceptive choices and contraindications for women with the following diseases: breast cancer; endometrial, ovarian, and cervical cancer; deep venous thrombosis/pulmonary embolism; hypertension (past, moderate, or severe); diabetes (with and without vascular disease); liver disease; epilepsy; headache; and sickle cell disease.
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PMID:Chronic diseases and contraceptive use. 1229 56

The work assessed the performance of the Kendall SCD Response intermittent pneumatic compression system for deep vein thrombosis prophylaxis, which claimed to set its cycle according to the blood flow characteristics of individual patient limbs. A series of tests measured the system response in various situations, including application to the limbs of healthy volunteers, and to false limbs. Practical experimentation and theoretical analysis were used to investigate influences on the system functioning other than blood flow. The system tested did not seem to perform as claimed, being unable to distinguish between real and fake limbs. The intervals between compressions were set to times unrealistic for venous refill, with temperature changes in the cuff the greatest influence on performance. Combining the functions of compression and the measurement of the effects of compression in the same air bladder makes temperature artefacts unavoidable and can cause significant errors in the inter-compression interval.
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PMID:Analysis of the operation of the SCD Response intermittent compression system. 1235 Feb 77

The WizAir-DVT is a miniature, lightweight (690 g), battery-operated and mobile intermittent pneumatic compression device (ICD), which enables continuous intraoperative use and immediate patient mobilization postoperatively. We compared its efficacy with a commonly used ICD, the Kendall SCD. Peak femoral vein flow velocity was measured in 20 apparently healthy volunteers at rest and with each device: we found no significant differences between them. A second prospective, randomized, clinical trial was used to compare the efficiency of the device in preventing deep venous thrombosis (DVT) after joint replacement in 50 patients (n=25/group). None developed DVT. Doppler ultrasonography revealed no significant differences. The WizAir-DVT antithrombotic compression device is as safe and effective as the Kendall SCD.
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PMID:A miniature and mobile intermittent pneumatic compression device for the prevention of deep-vein thrombosis after joint replacement. 1551 91

Here we describe an 8-year old male child with homozygous sickle cell disease who presented with left parietal skull bone infarction and, during his stay in hospital, developed a right femoral deep vein thrombosis (DVT), both uncommon complications of the disease. He initially presented with severe headache and generalised tenderness of the calvarium, which did not respond to simple analgesics. Scalp swelling in and around the left frontal (including left orbit) and parietal regions developed 24 h after presentation. The differential diagnosis included incipient stroke, acute sickle bone crisis and osteomyelitis, with a possible complication of epidural haematoma, or orbital compression syndrome. An initial exchange blood transfusion did not lead to appreciable reduction in opiate requirements. Significant symptomatic relief was attained only after a second exchange transfusion. The DVT developed at the site of catheterisation (right femoral vein), and this was treated with maximal doses of enoxaparin followed by warfarin. The child is now well and off anti-coagulants. In this article we present a review of the literature and discuss possible mechanisms of these complications in our patient.
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PMID:Skull bone infarctive crisis and deep vein thrombosis in homozygous sickle cell disease- case report and review of the literature. 1745

Intermittent pneumatic compression (IPC) devices are used to aid circulation in the lower limbs as a means of preventing deep vein thrombosis--a condition that can be dangerous and even fatal if it results in a pulmonary embolism. To be effective, IPC devices need to be safe, easy to use, and comfortable. Our Evaluation tells you which products meet these qualifications and how you can select one that best suits your needs. We tested and rated the following products: the VenaFlow, supplied by Aircast/DJO; the Flowtron Excel and Flowtron Universal, supplied by Huntleigh Healthcare; the PlexiPulse and Pulse SC, supplied by Kinetic Concepts Inc. (KCI); the A-V Impulse System, SCD Express, and SCD Response, supplied by Tyco Healthcare/Kendall.
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PMID:Intermittent pneumatic compression devices. 1769 75

Sickle cell disease (SCD) is associated with an increased incidence of deep vein thrombosis and pulmonary embolism which contributes to the morbidity and mortality associated with it. The predisposition to thrombosis has been attributed to the different blood components including platelets, the coagulation system and recently damaged red cells. This article discusses the possible role of reticulocytes in promoting thrombus formation in individuals with SCD and suggests a similar pathogenetic mechanism in thromboembolism associated with other hemolytic states.
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PMID:The possible role of reticulocytes in sickle cell disease associated thromboembolism. 1853 70

The diagnosis of deep vein thrombosis (DVT) is unusual in patients with sickle cell disease (SCD). Despite the incidence of cerebral thrombosis in SCD patients due to vasooclusion, thrombotic manifestations in peripheral vessels are rare. Patients with homozygous SCD present a variety of renal abnormalities as a result of sickle crisis. In this group of patients, kidney transplantation has been performed in the renal endstage patients; with graft survival rates similar to that of the general transplant population at 1 year. However, patients with SCD experience an augmentation in the frequency of painful crises in the first year after transplantation, which has been attributed to concurrent elevations in hematocrit and plasma viscosity. Despite etiology, renal transplant patients are at increased risk for the development of thromboembolic events such as deep vein thrombosis and renovascular thrombosis after allograft procedure. These events can be due to a prothrombotic state generated by the use of immunosuppressive agents. Although other factors such as acquired or inherited disorders of the clotting system may increase the risk of thrombosis. Here, we report a case of a renal transplant patient with sickle cell disease who presented recurrent episodes of DVT and increase painful sickle episodes after kidney transplantation.
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PMID:Thrombosis after kidney transplantation. 1952 94

Pulmonary embolism (PE) and deep vein thrombosis (DVT) are associated with considerable morbidity and mortality, mostly, in case of PE for its lack of sensitivity of its early detection. For as much as twenty-five percent of PE patients the primary clinical appearance is unexpected death. While PE is one of the most avertable causes of hospital associated deaths, its diagnostics can be extremely difficult. Newly increased interest in an inherited thrombophilic states has been provoked by the discovery of several common inherited abnormalities, i.e. the prothrombin (PT) gene G20210A, Factor V Leiden (FVL) mutation (Arg506Gln), hyperhomocystenemia and homocysteiuria, Wein-Penzing defect, Sticky Platelet Syndrome (SPS), Quebec platelet disorder (QPD) and Sickle Cell Disease (SCD). PE incidence rates increase exponentially with age for both men and women, as they might harbor more than one thrombophilic state. Although the impact of genetic factors on PE is to some extent documented with lacking taxonomy, its genetic testing as its prevention strategy fall short.In this review thrombophilic states are divided into inherited or acquired, and only the inherited and newly documented are more closely followed. Factors are further grouped based on its thrombophilic taxonomy into; inherited defects of coagulation, inherited defects of fibrinolysis, inherited defects of enzymatic pathway in relation to development of VTE and PE and inherited defects of platelets in relation to PE. It was beyond the scope of this review to follow all inherited and newly recognized factors and its association to VTE and PE; however the overall taxonomy makes this review clinically valuable i.e. in relation to genetic testing as PE prevention.
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PMID:Inherited trombophilic states and pulmonary embolism. 2177 60

We report a case of a high-performance athlete with hemoglobin SC who presented with asymmetric calf soreness after an intense calf workout. By ultrasonography, he was diagnosed with a deep vein thrombosis (DVT) of his right calf. Subsequently he presented with a number of sequelae of sickle cell disease: acute chest syndrome, avascular necrosis of the hips, and chronic kidney disease. The case is instructive as an example of DVT after exercise of the lower extremities, which has not been documented well. The case also illustrates a number of health sequelae of sickle cell disease that mimic more common musculoskeletal complaints. Sports medicine providers will have to consider these uncommon but profound diagnostic entities when caring for athletes with sickle cell disease. The case further highlights how research can inform the clinical decisions and policies aimed at reducing the risk of life-threatening and lifelong sequelae of sickle cell disease in athletes.
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PMID:Case report: lower extremity deep vein thrombosis following an intense calf workout. 2314 14


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