Causes and outcomes of the acute chest syndrome in sickle cell disease

Article Abstract:

Infection is probably a common cause of acute chest syndrome. This syndrome is characterized by chest pain, cough, wheezing, rapid breathing and fluid in the lungs in a patient with sickle cell disease. In a study of 671 episodes of acute chest syndrome in patients with sickle cell disease, 249 were associated with an infectious organism. The most common organisms were Chlamydia pneumoniae, Mycoplasma pneumoniae, and respiratory syncytial virus. Infection was implicated in half of the deaths. Most patients recovered following aggressive treatment, including artificial ventilation when necessary.

Prognosis, Lung diseases

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Alloimmunization in sickle cell anemia and transfusion of racially unmatched blood

Article Abstract:

Sickle cell anemia is a hereditary form of anemia (a condition in which there is insufficient hemoglobin, the molecule that carries oxygen in the blood) associated with severe symptoms, such as joint pain, blood clotting, fever, and weakness. The disease affects a significant number of black Americans. Such patients almost always receive transfusions of red blood cells and many develop an immune response to the transfused blood cells (alloimmunization). Alloimmunization may result from racial differences between donors and recipients. This was explored by studying 158 black patients with sickle cell anemia, 107 of whom had received transfusions. The remaining 51 patients had not been transfused. A third group was composed of 19 nonblack patients with other forms of chronic anemia who had undergone transfusion. Patients' records and transfusion histories were reviewed, and blood donors voluntarily identified their racial backgrounds. Results indicated that alloantibodies (antibodies to 'foreign' proteins in blood) were present in 30 percent of the 107 patients, and in none of the 51 patients who had not undergone transfusion. In addition, only one of the 19 nonblack patients had developed alloantibodies, in spite of having received far more transfusions than the sickle cell patients. The alloantibodies produced by the sickle cell patients were characterized and found to be mainly against the K, E, C, and Jkb antigens (molecules associated with red blood cells). The most likely explanation for the development of alloantibodies among transfused sickle cell patients is racial, resulting in the transfer of blood cells that elicit an immune response in the recipient. In general, blood donors are white, a situation reflecting the lack of participation by blacks in blood banking programs. Paradoxically, sickle cell patients who are alloimmunized now require 20 percent of all units of blood shipped by the American Red Cross in the rare-donor category. Patients with sickle cell disease who undergo transfusion should be matched with donors who have compatible blood. This requires the active recruitment of black people as blood donors. (Consumer Summary produced by Reliance Medical Information, Inc.)

Social aspects, Testing, Genetic aspects, Immune response, Blood transfusion, Blacks, Blood donors, Blood donation, Blood banks, Black race

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Sickle cell disease as a cause of osteonecrosis of the femoral head

Article Abstract:

Sickle cell disease, an inherited disorder of hemoglobin, is associated with a variety of complications, including skeletal disease. Osteonecrosis (death of bone tissue) of the femoral head (the upper part of the thighbone) is one such complication. It is not confined to those with hemoglobin SS (sickle cell anemia), but includes those with disorders associated with heterozygosity for two hemoglobin genes (such as hemoglobin SC disease or hemoglobin S-B0-thalassemia). To learn more concerning osteonecrosis in patients with sickle cell disease, a study was begun at 23 medical centers in 1978 of patients over five years of age with sickle cell disease. Osteonecrosis was diagnosed in 253 of 2,590 patients (9.8 percent) when the study began. This was most prevalent among patients with the SB0 genotype (S-beta thalassemia 0) who had an incidence of 13.1 percent. The rates of osteonecrosis for other genotypes were 10.2 percent for the hemoglobin SS genotype; 8.8 percent for the SC genotype; and 5.8 percent for the SB+ genotype. Patients who were homozygous for alpha-thalassemia had a prevalence of osteonecrosis of 21.2 percent, while those with the SS genotype without the alpha-thalassemia gene had a prevalence of 8.7 percent. Slightly more than half of all patients developed disease in both hips. Risk factors for osteonecrosis of the femoral head included more frequent painful vasoocclusive crises (a complication of sickle cell disease in which tiny clots block small blood vessels) and a higher hematocrit (the volume of erythrocytes in a blood sample after centrifugation). Hip surgery had been performed on 44 of the 253 patients with osteonecrosis when the study began, and 27 more underwent surgery during the study period. Five of these operations needed to be repeated several months later. Osteonecrosis of the femoral head is common among patients with sickle cell disease and is even present in children as young as five. Its overall prevalence is approximately 10 percent. Hip joint replacement is not as successful in these patients as in those who suffer from arthritis. (Consumer Summary produced by Reliance Medical Information, Inc.)

Bones, Causes of, Physiological aspects, Joint diseases, Osteonecrosis

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