Chronic renal failure in sickle cell disease: risk factors, clinical course, and mortality

Article Abstract:

Sickle cell anemia is an inherited disease that predominantly affects blacks. It is characterized by abnormalities in the shape of the red blood cells, which cause them to lodge in blood vessels throughout the body, resulting in various painful complications. As medical progress has permitted those with sickle cell disease to live well into adulthood, late complications of the disease are being seen more frequently than in the past. One of these complications is kidney or renal failure, attributed to interruption of renal blood flow by the abnormal, sickle-shaped blood cells. A group of 36 patients who developed kidney failure due to sickle cell disease were studied. The average age for kidney dysfunction in patients with sickle cell disease is 23 years; those with a milder variant known as sickle C disease develop kidney problems at an average age of 50 years. Warning signs of impending renal failure in sickle cell disease include worsening of the anemia or low red blood cell counts, protein in the urine, blood in the urine, and high blood pressure. Since most patients with sickle cell disease have lower blood pressure than the black population as a whole, a rise in blood pressure in sickle cell patients may be a particularly sensitive warning of future kidney disease. Once kidney failure occurs, many of the complications associated with sickle cell disease show an increased incidence. Early death is far more common in those sickle cell patients who develop kidney failure. Prompt attention to the warning signs of kidney failure in sickle cell patients might lead to opportunities for early intervention, and an increase in the lifespan of those sickle cell patients whose kidneys fail. (Consumer Summary produced by Reliance Medical Information, Inc.)

Diagnosis, Risk factors, Physiological aspects, Chronic kidney failure, Acute renal failure, Acute kidney failure

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Myonecrosis and myofibrosis as complication of sickle cell anemia

Article Abstract:

Patients with sickle cell disease constantly run the risk of a sickle cell crisis. During such a crisis, the normally round, indented red blood cells take on bizarre shapes (that is, 'sickle') and can no longer fit through tiny blood vessels. This deprives tissues of oxygen, a condition called ischemia. If ischemia persists, an infarction results as the tissue cannot recover from the physiological insult. Tissue damage of this sort may affect any organ of the body. However, complications of the muscles resulting from sickle cell anemia are less frequent than complications involving other organs, and muscle infarction resulting from sickle cell disease may on occasion be misdiagnosed as the more common bone infarction. Four cases are presented which illustrate characteristics of muscle damage that can occur in patients with sickle cell anemia. During sickle cell crises, the patients also developed swelling and tenderness of some muscles in the arms and legs. In two cases, muscle biopsy confirmed the presence of myonecrosis, or the dying of muscle cells, which is believed to be a direct consequence of infarction. Muscle then becomes fibrotic, that is, fibrous scar tissue forms, taking the place of the dying muscle tissue. While the acute symptoms resolved slowly over a period of several weeks, the patients experienced chronic symptoms including atrophy, in which the muscle mass wastes away, induration, in which the muscle is hardened, and contractures, in which the muscles resist being stretched. (Consumer Summary produced by Reliance Medical Information, Inc.)

Causes of, Muscles, Atrophy, Muscular, Muscular atrophy, Necrosis, Myonecrosis

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Antithrombin III concentrate for treatment of chronic leg ulcers in sickle cell-beta thalassemia: a pilot study

Article Abstract:

Beta thalassemia is an inherited blood disorder that is prevalent in the Mediterranean and Southeast Asia. It is characterized by anemia (a decrease in the numbers of normal red blood cells), which results from the abnormal production of the beta chain portion of hemoglobin (the oxygen-carrying pigment of red blood cells). Sickle cell anemia is another inherited anemia in which normal red blood cell numbers are decreased and abnormal sickle-shaped red blood cells are present. These hereditary blood disorders are often associated with the formation of long-term leg ulcers, although the mechanism of the disease process is not known. Patients with beta-thalassemia or sickle cell anemia have an increased risk of thrombosis or blood clot formation, which may contribute to the development of leg ulcers. Hence, antithrombotic agents may be useful in treating leg ulcers in patients with sickle cell-beta thalassemia. The effectiveness of the antithrombotic agents, calcium heparin and human antithrombin concentrate, was assessed in 10 patients with sickle cell-beta thalassemia and long-term leg ulcers. Laboratory data showed that the patients lacked antithrombin III, and the administration of this factor combined with heparin improved the leg ulcers. Thus, human antithrombin III concentrate is effective in treating leg ulcers in patients with acquired antithrombin deficiency associated with sickle cell-beta thalassemia. (Consumer Summary produced by Reliance Medical Information, Inc.)

Health aspects, Evaluation, Leg, Leg ulcer, Thalassemia, Antithrombins

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