Acquired aplastic anemia: still a serious disease

Article Abstract:

Aplastic anemia is a rare and often fatal blood disorder. It affects the bone marrow and reduces both the number of red blood cells and the amount of hemoglobin in the blood. Anemia can be inherited or it can be acquired. Acquired aplastic anemia can be caused by infection or exposure to certain chemicals or drugs, but in 70 percent of cases the cause is unknown. In some cases, the immune system (the body's natural defense system for fighting infection) may be involved, and in 50 percent of these cases treatment with drugs that suppress the immune system has proven to be beneficial. It is estimated that only 20 percent of the children with severe aplastic anemia will survive for one year without treatment. Bone marrow transplantation from a matched family donor is the preferred method of treatment, but it is estimated that only 30 percent of patients have a suitable family donor. The medical records of 38 children with acquired aplastic anemia were reviewed to evaluate treatments and outcomes of children with this condition. Nine cases were categorized as not severe, 16 as severe, and 13 as very severe. Ten of the children received bone marrow transplants; seven were from matched family donors and three were from matched unrelated donors (all three of the recipients died). Eleven children were treated with antilymphocyte globulin (a drug that suppresses the immune system), 4 with antilymphocyte globulin plus oxymetholone (an androgen or male hormone), 12 with oxymetholone, and 1 with supportive care (no drugs). The five-year survival rate was 70 percent for those receiving bone marrow transplants from family donors, 30 percent for those treated with antilymphocyte globulin, and 25 percent for those treated with oxymetholone. These findings demonstrate that the prognoses of children with acquired aplastic anemia is poor, and that new methods of treatment are needed. (Consumer Summary produced by Reliance Medical Information, Inc.)

Care and treatment, Prognosis, Bone marrow, Bone marrow transplantation, Aplastic anemia

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Kawasaki disease: recent advances

Article Abstract:

Kawasaki disease (also called mucocutaneous lymph node syndrome) was first reported in Japanese children in 1967. It is most common in children between six months and five years of age, and the cause is unknown. It causes fever, rash, and congestion, and it causes the skin to peel off of the fingers and toes. The disease affects the lymph nodes and causes inflammation in the blood vessels in the heart and other organs. In 20 to 40 percent of the cases it causes aneurysms in the coronary artery. In most cases, the aneurysms regress during the first or second year after the onset of the disease. Roughly one percent of the patients die from blood clots that become lodged in the coronary artery. Although the disease appears to spread in epidemic fashion, there is no evidence of person to person transmission of the disease, and no causative microorganisms have been identified. The disease does not respond to treatment with antibiotics. Intravenous infusions of gammaglobulin are used to reduce the risk of coronary artery aneurysms and aspirin is prescribed to prevent blood clot formation. (Consumer Summary produced by Reliance Medical Information, Inc.)

Complications and side effects, Coronary heart disease, Kawasaki disease, Kawasaki syndrome

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