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)

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

Maternal mortality is examined from June 1980 to December 1986 at Mulago, Nsambyo, Old Kampala, Rubaga, and Mengo Hospitals in Kampala, Uganda. Clinical or immediate causes, direct and indirect, were recorded from case summary forms based on ICD9 definitions of obstetric complications. The nonabortion maternal mortality rate (NAMMR) was 2.65/1000 deliveries (580 deaths); the abortion-related maternal mortality rate (ARMMR) was 3.58/1000 abortions. The hospital maternal mortality rate was 2.0/1000 deliveries. 75% of maternal deaths of women of 28 weeks' gestation or more had delivered outside the hospital. NAMMR doubled between 1980-86, a statistically significant increase. ARMMR increases were almost significant. 75% were direct obstetric and 21% were indirect obstetric causes. 38% had clinical anemia, 29% had some sepsis, 18% had substantial bleeding, and 14% had obstructed labor. Other contributing conditions were pneumonia, ruptured uterus, laparotomy, evacuations and curettage, malaria, preeclampsia, sickle cell anemia, pulmonary embolism, malnutrition, tetanus, meningitis, prolonged labor, and hepatitis. At admission, 48% were in poor condition, 30% in good condition, and 22% in fair condition. 27% had sickle cell anemia, high blood pressure, multiple pregnancy, or malaria at admission. 64% were admitted within 24 hours after delivery, 67% 1-7 days after delivery, and 92% 7-42 days after delivery. Those in good condition were all admitted 7 days postdelivery. 41% of deaths were due to lack of drugs, 7% lack of fluids, 20% with theater problems, 14% with doctor-related factors, and 3% with midwife-related factors. Better information is needed on mortality before delivery, mortality in hospitals vs. outside, and mortality from abortion, and ectopic and hydatidiform molar pregnancies. An explanation given for the increase in maternal mortality is the decline in economic conditions. Abortion complications may be due to the concealment practiced. Causes are consistent with trends from the 1950s, 1960s, and 1970s in Uganda and developing countries in general. Availability and accessibility of gynecological and obstetric services needs great improvement. Training traditional birth attendants and obtaining rural ambulance services are also needed. Health workers lack creativity and imagination for developing country conditions; scarce resources are not the only problem.
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PMID:Incidence and causes of maternal mortality in five Kampala hospitals, 1980-1986. 176 15

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

There were 37 maternal deaths among the 109,221 livebirths registered during the period 1977-86 in Bahrain, Arabian Gulf. The maternal mortality rate was 33.9/100,000 for the 10-year study period; however, disaggregation reveals a decline in this rate from 42.3/100,000 in 1977-81 to 26.9/100,000 in 1982-86. This decline presumably reflects streamlining of the Ministry of Health's maternity services, including a central maternity hospital with all modern facilities that serves as a referral center for all of Bahrain, 2 peripheral hospitals with provision for blood transfusion and surgical deliveries, and 3 maternity units managed by fully qualified midwives. About 80% of deliveries are covered by these maternity services; only 2.5% of deliveries occur in the home. Despite this highly developed maternity care system, 18 of the maternal deaths were due to direct obstetric cause: hemorrhage, 7; pre-eclampsia and eclampsia, 5; abortion septicemia, 2; bowel perforation during cesarean section, 1; thromboembolism, 2; and amniotic fluid embolism, 1. The causes of the 19 indirect maternal deaths were: pulmonary embolism, 5; infection, 7; cardiac failure, 2; cerebrovascular accident, 2; pulmonary hypertension, 1; and uncertain, 2. Of interest is the finding that sickle cell disease was the underlying cause of maternal death in 12 of the 37 deaths in this series. Sickle cell disease was implicated in 3 of the deaths from hemorrhage, all 5 deaths from pulmonary embolism, 2 deaths from septicemia, and the 2 cases of cardiac failure. In this series, 50% of the patients with sickle cell disease had thromboembolic crises following treatment of anemia with packed cell transfusion. Blood transfusion, especially of packed cells, should be given with caution to these patients since it may precipitate vaso-occlusive crisis by increasing blood viscosity. Since sickle cell disease represents a high risk during pregnancy in this Arab population, such patients should have frequent prenatal check-ups and deliver in a well-equipped hospital.
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PMID:Maternal mortality in Bahrain with special reference to sickle cell disease. 321 81

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

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

Pulmonary disease, including thromboembolic problems, accounts for a large portion of the morbidity of sickle cell disease. Chronic transfusion therapy is now a part of long-term treatment of sickle cell patients with stroke and chest syndrome. The resultant iron overload must be treated with chelation therapy using deferoxamine. Poor compliance with subcutaneous chelation therapy has necessitated intravenous deferoxamine treatment. We describe two patients with sickle cell disease on such a regimen, who became hypoxic as a result of pulmonary thromboembolism, secondary to venous thrombophlebitis. The thrombophlebitis and subsequent pulmonary embolism probably reflect the hypercoagulable state seen in sickle cell and are not due to the deferoxamine therapy.
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PMID:Pulmonary embolism developing in patients with sickle cell disease on hypertransfusion and IV deferoxamine chelation therapy. 938 84

The pulmonary complications remain the prime cause of morbidity and mortality in sickle cell disease. The pathogenetic mechanisms consists both of an alteration of the rheological properties of the blood, the existence of a hypercoagulability state and above all specific interactions between the abnormal sickle cells and the vascular endothelium and a dysregulation of the vascular reactivity in which nitrous oxide intervenes. The acute chest syndrome (ACS) is characterised by chest pain with dyspnoea and recent radiological abnormalities and it is an acute lung complication whose problem is one of aetiology. The infectious pneumonias are rarely documented. On the other hand, alveolar hypoventilation linked to infarcts of the thoracic ribs, thoracoabdominal trauma, subdiaphragmatic pain, the administration of analgesics causing respiratory depression, obesity or sleep disturbance are frequent causes of ACS. Bronchoalveolar lavage has revealed a frequency of fat emboli following infarcts in the long bones. Pulmonary emboli is rarely a cause. Pulmonary thrombosis is a serious complication, the diagnosis is difficult and is seen in a predisposed clinical setting. The treatment of ACS rests on controlled hydration and antibiotic therapy, oxygen therapy and controlled analgesic therapy. The indications for blood transfusion and for exchange transfusion merits a better evaluation. In the long term patients with sickle cell disease present with a failure of normal thoracopulmonary growth with a restrictive ventilatory defect and progressive diminution in the transfer factor of carbon monoxide with age. A history of ACS favours chronic lung disease. Pulmonary arterial hypertension is less frequent.
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PMID:[The sickle cell anemia lung from childhood to adulthood]. 960 86

Medicolegal (coroner's) autopsies are an important source of epidemiological data. A large proportion of them comprise sudden natural deaths and an analysis of such cases has never been undertaken at the University Hospital of the West Indies, the only teaching hospital in Jamaica. In a retrospective study, 841 cases of sudden natural deaths comprising 51.3% of the medicolegal autopsies conducted over the 15-year period, January 1983 to December 1997, were analyzed. There were 459 males and 382 females (M:F ratio = 1.2:1); 35 patients (4.1%) were less than 1 year of age, and the mean age of the remainder was 53.7+/-21.8 years. The peak age group was the seventh decade accounting for 21.9% of cases. The most common causes of death were cerebrovascular accidents (13.6%), pneumonia (9.4%), pulmonary embolism (7.4%), ischaemic heart disease (7.0%) and diabetes mellitus (6.1%). These findings contrasted with those from developed countries in which ischaemic heart disease is the commonest cause of sudden death. Hypertension was associated with the majority of cases of cerebrovascular accident and congestive cardiac failure (78.1 and 61.9%, respectively). Sickle cell disease represented one of the 10 most common causes of death accounting for 2.5% of cases. Documentation of autopsy-based data such as these is important in the planning of medical services in a developing country.
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PMID:Causes of sudden natural death in Jamaica: a medicolegal (coroner's) autopsy study from the University Hospital of the West Indies. 1224 80

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


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