Recent approaches to the treatment of sickle cell anemia
Article Abstract:
Sickle cell anemia is an inherited blood disorder in which the red blood cells take on a sickled shape, rather than their normal round shape. When these sickled cells become caught in capillaries in sufficient numbers, sickle cell crisis occurs, with breakup of the red cells, severe pain, and an increased susceptibility to infection. Occlusions of the circulatory system by clumps of these cells can cause permanent damage to the spleen, strokes, and compromise blood supply to virtually any organ, with significant damage. Recent evidence demonstrates that the mechanism of this sickling is the aggregation of groups of hemoglobin S molecules, which are the oxygen-carrying molecules in red blood cells that have the sickling abnormality. Certain other inherited disorders of hemoglobin are actually helpful to sickle cell patients, because they decrease the amount of the dangerously abnormal hemoglobin S, and increase the amount of other abnormal hemoglobins that do not cause such severe damage. Treatment of sickle cell disease involves careful monitoring for the particular infections to which these patients are most prone, and giving the vitamin folic acid, which is needed when there is increased turnover of red blood cells (as in sickle cell anemia). Large volumes of fluids and appropriate doses of narcotics are necessary in the treatment of acute sickle cell crisis. Studies are ongoing to find other strategies to treat the disease, such as the use of hydroxyurea, normally used as chemotherapy for cancer, which increases the level of fetal hemoglobin (hemoglobin F) in sickle cell patients, thereby effectively decreasing the level of hemoglobin S. Some studies are examining the use of human erythropoietin, a protein that promotes synthesis of hemoglobin F. Preliminary successes with these treatments have led the way to their being used in treatment of beta thalassemia, another inherited blood disorder, which is frequently fatal at a young age. Greater understanding of sickle cell disease and its treatments may provide benefits in other hemoglobin abnormalities. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: JAMA, The Journal of the American Medical Association
Subject: Health
ISSN: 0098-7484
Year: 1991
User Contributions:
Comment about this article or add new information about this topic:
Innovative ways to fight cancer dominate 1989 AMA Houston science news conference
Article Abstract:
The American Medical Association's Eighth Annual Science Reporters Conference convened in Houston, TX for a three-day meeting. Areas of interest included presentations on the development of anti-cancer vaccines, immunization, anti-herpes vaccines, pediatric AIDS (acquired immunodeficiency syndrome), and areas of genetic engineering. Also presented was research dealing with DNA fingerprinting and its use in forensic medicine and genetic therapy for cancer. New work on the encapsulation of anti-cancer drugs within a fatty envelope, which enables a higher dose to be delivered with reduced side-effects, was also discussed. Genetic markers that can signal the presence of hereditary diseases have also been developed, and consequently during the last year the gene responsible for cystic fibrosis (CF), the most common lethal genetic disease in the United States, has been identified. Although an estimated 10 million people carry the genetic trait for CF, few are aware of it. Several papers on the relationship between genetics and schizophrenia were also presented. A study of schizophrenia in 62 pairs of identical twins in which one or both of the twins had been hospitalized for this disease shows that the risk of having a schizophrenic child is 16.8 percent for the non-affected and 17.4 percent for a normal twin. The prevention of hypertension and cancer were also discussed. Problems and concerns about the quality, priority and distribution of research funds were touched on by a number of participants.
Publication Name: JAMA, The Journal of the American Medical Association
Subject: Health
ISSN: 0098-7484
Year: 1989
User Contributions:
Comment about this article or add new information about this topic:
Life-threatening sepsis associated with adjuvant doxorubicin plus docetaxel for intermediate-risk breast cancer
Article Abstract:
The adverse events associated with adjuvant chemotherapy for breast cancer, which led to premature termination of a clinical trial, are described. Findings suggest that the doxorubicin-docetaxel combination should not be considered as an alternative to the doxorubicin-cyclophosphamide regimen outside carefully designed studies that include primary prophylaxis for febrile neutropenia.
Publication Name: JAMA, The Journal of the American Medical Association
Subject: Health
ISSN: 0098-7484
Year: 2005
User Contributions:
Comment about this article or add new information about this topic:
- Abstracts: Problems in transfusion therapy. Treatment of severe malaria in the United States with a continuous infusion of quinidine gluconate and exchange transfusion
- Abstracts: Transforming growth factor beta as a predictor of liver and lung fibrosis after autologous bone marrow transplantation for advanced breast cancer
- Abstracts: Ranitidine in the treatment of non-steroidal anti-inflammatory drug associated gastric and duodenal ulcers. Effect of calcitonin on gastric emptying in patients with an active duodenal ulcer
- Abstracts: Radiation therapy after breast augmentation or reconstruction in early or recurrent breast cancer. Psychosocial adjustment after mastectomy and breast-conserving treatment
- Abstracts: A multicentre study of coagulation and haemostatic variables during oral contraception: variations with four formulations