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Query: UMLS:C0038454 (stroke)
 147,016 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Annual age-specific incidence rates of Streptococcus pneumoniae or Haemophilus influenzae bacterial septicemia in sickle cell anemia (SS) were determined for the years of 1957 through 1989. Forty-nine patients had 64 episodes of septicemia among a population of 786 SS patients observed for 8,138 person-years. Peak frequency of infection occurred between 1968-1971 and 1975-1981 with a conspicuous absence of episodes in 1972, 1973, 1982-1984, and 1986-1987, thus demonstrating cycles of high and low attack rates. The annual age-specific incidence rate of septicemia varied from 64.5 (1965) to 421.1 (1980) per 1,000 person-years for those under 2 years of age and never exceeded 10.2 per 1,000 in those over 4 years of age. Following the introduction of pneumococcal polyvalent vaccine in 1978, incidence of infection decreased in SS children greater than 2 years of age. No modification of the risk of infection was observed in immunized children less than 2 years of age. During these three decades, there has been a ten-fold increase in the number of SS adults over 20 years of age. The relative risk of chronic sickle complications comparing the survivors of septicemia to the non-infected patients was: subsequent death 1.76, retinopathy 4.06, avascular necrosis 1.95, symptomatic cholelithiasis 1.33, stroke 1.30, and priapism 1.26. These data suggest that prognosis for lifetime severe SS is initially manifested as an increased risk of septicemia during childhood.
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PMID:Polysaccharide encapsulated bacterial infection in sickle cell anemia: a thirty year epidemiologic experience. 154 14

There is wide variation in the clinical manifestations of sickle cell disease (SCD) from one affected individual to another. Many investigators have sought to discern parameters that would explain this variability. In the present studies we have attempted to correlate the frequency of painful events and the extent of end organ failure in SCD with rheologic properties of packed suspensions of sickle cells, using a magneto-acoustic ball microrheometer developed in our laboratory. Using this device we have measured the steady-state viscosity, and the viscous and elastic moduli of cell suspensions in 16 individuals with hemoglobin SS disease who were untransfused and in their steady state. The rheologic parameters were then correlated with clinical parameters. The clinical parameters measured were emergency department visits, hospitalizations, hemoglobin, reticulocyte count, age, and end organ failure (nephropathy, avascular necrosis of bone, stroke, retinopathy, resting hypoxemia after acute chest syndrome(s), leg ulcer, and priapism with impotence). The P value for the correlation between the steady state viscosity and end organ failure was .001 with a correlation coefficient (R value) of .73. The P value for the correlation between the viscous modulus of viscosity and end organ failure was .00006 with an R value of .83. The P value for the correlation between the elastic modulus of viscosity and end organ failure was .0006 with an R value of .76. However, there was no significant correlation between any component of packed cell rheology and emergency department visits or hospitalizations for pain.
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PMID:Relationship of clinical severity to packed cell rheology in sickle cell anemia. 182 65

Much of the morbidity and mortality in sickle cell disease (SCD) is caused by tissue ischemia and infarction resulting from vascular occlusion. Research in this area has been dominated by the hypothesis that vascular occlusion in SCD is due primarily to microvascular obstruction by sickle erythrocytes (SS RBC), yet there is no direct evidence that microvascular occlusion is responsible for any of the vasocclusive complications of SCD. In this paper an alternate hypothesis is proposed: that thrombotic occlusion of larger arteries and veins is an important factor in many of the vasocclusive complications of SCD. Large-vessel cerebral arterial disease (intimal hyperplasia with superimposed thrombosis) has clearly been established as the most important cause of stroke in SCD, and considerable evidence suggests that pulmonary arterial thrombosis/embolism is a major cause of pulmonary infarction and hypertension. The involvement of large-vessel thrombosis in painful crisis, aseptic necrosis of bone, priapism, leg ulcers, retinopathy, and miscarriage has not been adequately investigated. Large-vessel occlusion in SCD is probably a consequence of the abnormal adhesive and procoagulant properties of SS RBC, which produce endothelial damage, secondary intimal proliferation, and thrombosis. Techniques currently used to treat large-vessel occlusion in other disorders (antiplatelet and anticoagulant agents, thrombolytic therapy, angioplasty, endarterectomy, and vascular bypass surgery) should be considered in sickle cell subjects with large-vessel occlusion, especially in the cerebral vasculature.
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PMID:Large-vessel occlusion in sickle cell disease: pathogenesis, clinical consequences, and therapeutic implications. 189 Sep 82

Ten homozygous sickle cell patients, aged from 4 to 20 years, underwent a periodic red blood cells transfusion program. Indications were = one stroke or more in 4 cases, repeated severe painful crisis in 2 cases, and for others = chronic anaemia induced by hypersplenism, priapism, chronic leg ulceration, dwarfism with delayed sexual maturation. Transfusion program lasted from 6 months to 6 years. In 7 cases, a phlebotomy preceded the transfusion by the same venipuncture in order to reduce the inevitable iron overload progression. Clinical benefit was clear in the following cases: lack of painful crisis with return to school, leg ulceration healing, growth and weight increase, onset of puberty. Benefit is more difficult to assess in the other observations and, particularly, in stroke. No red cell antibodies were found. An HIV contamination occurred in 3 children. All transfused patients during a period exceeding one year developed iron overload, needing a daily chelation by intramuscular deferoxamine in two of them.
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PMID:[Long-term blood transfusion in sickle-cell anemia]. 361 65

Hyposexuality is a common problem in stroke patients. Some stroke patients, however, may present with hypersexuality. We report three stroke patients who demonstrated hypersexuality and deviant sexual behavior after stroke. Abnormal sexual behavior was noted by members of the stroke rehabilitation team while the patients were in hospital. Details of pre- and poststroke sexual activity were obtained from patients and their spouses. All three patients had temporal lobe lesions on computerized tomography, and all had a history of poststroke seizure activity. The 53-year-old man reported poststroke increase in libido and coital frequency with a tendency towards priapism. The two women (47 and 55 years old) exhibited deviant sexual behavior, mood changes, and hyperphagia. The exact cause of these behavior changes is unknown but they may be related to temporal lobe involvement. Hypersexuality has previously been reported in animals and in patients with temporal lobe seizures.
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PMID:Hypersexuality in stroke. 371

The indications and management of blood transfusion in the haemoglobinopathies have been reviewed. The sickle cell diseases that require transfusion support are sickle cell anaemia, sickle haemoglobin-C and -D diseases and sickle beta-thalassaemia. Homozygous beta-thalassaemia (Cooley's anaemia) is the major problem among the thalassaemias. The pathophysiology of the sickle cell disorders is largely based on the secondary effects of increased blood viscosity, whereas in the thalassaemias the defect is ineffective haematopoiesis. In the former the major problems occur as manifestations of vaso-occlusive crises with disseminated bone and abdominal pain, priapism, stroke and leg ulcers. Bone infarction and aseptic necrosis occur but the widespread bone changes, underdevelopment and haemochromatosis that complicate the thalassaemia are not prominent. Transfusion therapy in the sickle cell diseases is mainly episodic and is guided by the frequency of crises and the severity of vaso-occlusive complications. Partial exchange transfusion and the maintenance of haemoglobin A concentrations at 40 to 50 per cent is frequently indicated. In the thalassaemias, maintenance of haemoglobin levels is essential for normal growth and development. The problem of haemochromatosis is very serious. With hypertransfusion regimens the haemoglobin and haemotocrit are maintained above 12-13 g/dl and 35 per cent. The resulting benefit appears to be reduced blood volume, less iron turnover, and less intestinal iron absorption. The splenomegaly in these disorders is frequently associated with hypersplenism requiring well-timed splenectomy. Chronic and intensive chelation is necessary to prevent the ravages of iron overload. The availability of automated equipment for in vivo and ex vivo blood cell separation has brought new possibilities for improving the management of these haemoglobinopathies. It is feasible, but not as yet practical, to offer transfusions of neocytes (red cells with a mean age of 30 days) which have a 50 per cent longer survival than routine red cell preparations (mean age of 60 days). Neocytes can be prepared ex vivo from fresh routine blood donations using blood cell separator devices. The result is reduced transfusion requirements. A more recent suggestion for using the new technology is to remove the patient's oldest and most abnormal corpuscles on the basis of buoyant density and replacing them with neocytes . Thus the short-lived abnormal red cells would be removed before they could unload their iron. With automation it is possible to perform these procedures on an outpatient basis.
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PMID:Transfusion support for haemoglobinopathies. 637 80

The period of adolescence in the patient with sickle cell disease seems to be a period of relative calm, medically speaking. The current 5-year prospective Cooperative Study of the Clinical Course of Sickle Cell Disease should demonstrate the actual spectrum of disease in this age group. The recent literature documents with significant relationship between retardation of growth and sexual maturation in the child with sickle cell disease, an effect which seems independent of the severity of the disease. Several other major problems in the adolescent are discussed, including psychological disturbances, leg ulcers, aseptic necrosis, pulmonary disease, priapism, stroke, cholelithiasis, and birth control. A retrospective series of 76 cases of sickle cell disease is briefly presented and the complication occurring during the second decade of life are reviewed.
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PMID:Problems and complications in the adolescent with sickle cell disease. 709 75

A review of hospital admissions during 80 months revealed only 8 patients with episodes of priapism of approximately 400 pediatric male patients with sickle cell disease. The patients, who ranged in age from 5 to 19 years, underwent a 99mtechnetium penile scan, and 4 had a low and 4 had a high flow scan. Three cases resolved with hydration alone. Five patients received exchange transfusion of whom 3 subsequently underwent shunt procedures. One patient with a 5-year history of recurrent stuttering episodes was placed on transfusion therapy for 6 months and stuttering episodes have not recurred. One patient had a cerebrovascular accident 1 day after hospital discharge and another had priapism while on chronic transfusion therapy for a cerebrovascular accident. Each postpubertal patient had a severe clinical course; 1 had temporary impotence for 3 months and another had impotence at 2 weeks but was lost to followup. While 99mtechnetium penile scans may help clarify the severity of vascular stagnation, in our small group they were not helpful in predicting clinical course.
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PMID:Priapism in children with sickle cell disease. 760 95

Management of transfusion therapy in sickle cell disease patients with acute complications is often made difficult because of confusing indications, a variety of methods, disparate goals, and varying needs for maintenance transfusion. In priapism, acute chest syndrome, many major surgical procedures, toxemia of pregnancy, and cerebrovascular accidents, the target hemoglobin A level should be made as close to 100% as possible by mechanized red blood cell exchange. If mechanized exchange is unavailable, manual exchange should be instituted. Hemoglobin A should be maintained at greater than 60% to 70% by periodic simple transfusion until patients are fully recovered. Stroke patients should undergo maintenance transfusions for at least 3 years and perhaps 5 to 12 years. Physicians and patients should be aware of the transfusion-related risks of hepatitis and HIV infection. Alloimmunization and iron overload should be minimized in patients requiring frequent transfusions and chelation therapy should be utilized for iron overload.
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PMID:Transfusion therapy in sickle cell disease patients: methods and acute indications. 812 Apr 39

The venoms from 3 snakes have been shown to induce defibrinogenation: ancrod from the venom of Calloselasma rhodostoma (formerly known as Agkistrodon rhodostoma), batroxobin from the venom of Bothrops atrox moojeni, and crotalase from the venom of Crotalus adamanteus. The purified fractions of ancrod, batroxobin, and crotalase possess coagulant, proteolytic and esterolytic properties, although their primary mechanism of action is a proteolytic effect on circulating fibrinogen. Ancrod cleaves only the A-fibrinopeptides, but not the B-fibrinopeptides, from fibrinogen; this contrasts with thrombin, batroxobin and crotalase, which cleave both fibrinopeptides A and B. Within minutes of administration of ancrod or batroxobin, there is a significant reduction in plasma fibrinogen levels, and these remain exceedingly low with repeated administration (once or twice daily). The rapid fall in plasma fibrinogen levels is accompanied by a slightly delayed but marked rise in the level of fibrinogen-fibrin degradation products. Plasminogen levels are decreased and blood viscosity is reduced, but formed elements in the circulating blood remain unaltered. Ancrod and batroxobin have been investigated in patients with stroke, deep-vein thrombosis, myocardial infarction, peripheral arterial thrombosis, priapism, and sickle-cell crisis; crotalase has not been administered to humans. However, results have been difficult to interpret, and additional well designed trials are needed to better define the optimum role of ancrod and batroxobin in the management of these conditions. Overall, treatment is well tolerated and serious adverse events are infrequent. In the coagulation laboratory, ancrod, batroxobin and crotalase may be used as reagents to perform coagulation studies on specimens of blood that contain heparin. These venom fractions can be substituted for thrombin in performing the thrombin time and in removing fibrinogen from plasma for accurate determination of fibrinogen-fibrin degradation products.
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PMID:Defibrinogenating enzymes. 936 Aug 49


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