Partial splenectomy in sickle cell syndromes

Article Abstract:

Sickle cell disease is a genetic disorder characterized by the presence of red blood cells having a sickle or crescent shape. This disorder results from the production of an abnormal hemoglobin, the oxygen-carrying pigment of red blood cells. Sickle cell disease may be complicated by disorders of the spleen, which functions to produce certain blood cells, store blood, and remove bacteria, foreign matter, and immature red blood cells from the blood. Acute splenic sequestration is characterized by decreased levels of hemoglobin, increased activity of the bone marrow to compensate for declining function of the spleen, and an enlarged spleen. This complication is a major cause of death and disability among children with sickle cell disease younger than five years of age. Hypersplenism, another complication, is characterized by an enlarged spleen, decreased hemoglobin levels, increased numbers of bone marrow cells known as reticulocytes, and decreased numbers of platelets, which are cells involved in blood clotting. Patients with hypersplenism rely on transfusions and require routine hospitalization. Splenectomy, the surgical removal of the spleen, serves as an alternative treatment for hypersplenism to avoid the risks of transfusions. Partial splenectomy, or removal of only a portion of the spleen, may be performed to maintain some of the spleen's protective function in fighting infection. The benefits of partial splenectomy were assessed in 12 children with forms of sickle cell disease. Acute splenic sequestration was diagnosed in one case, hypersplenism in five cases, and both complications in six cases. Surgery was uncomplicated in 11 cases and resulted in less need for transfusion and hospitalization, no recurrence of splenic disorders and severe infection, and improved hemoglobin levels and numbers of platelets and white blood cells. The benefits of partial splenectomy are comparable to those of total splenectomy for treating complications of the spleen associated with sickle cell disease in children. (Consumer Summary produced by Reliance Medical Information, Inc.)

Evaluation, Sickle cell anemia, Lymphatic diseases

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Partial splenectomy in homozygous beta thalassaemia

Article Abstract:

Hypersplenism (increased hemolytic action of the spleen) is a common complication of homozygous beta-thalassemia, one of a group of inherited disorders of hemoglobin metabolism in which there is a decrease in the synthesis of a particular globin chain without concomitant change in the chain structure. Splenectomy reduces the transfusion requirements and also the iron overload; it also exposes the children to postoperative infection, however. As an alternative, partial splenectomy was performed on 30 thalassemic patients: 24 with thalassemia major (TM) and 6 with thalassemia intermedia (TI); the diagnosis based on transfusion requirements. Five patients were treated with a high transfusion regimen before and after surgery. The remainder received a lower transfusion rate. The surgical procedures were uneventful for all of the patients. The surgery left intact on average the approximate equivalent of one half of the normal spleen. The TI patients improved, having reduced blood requirements. Nine of the TM patients had a recurrence of hypersplenism, and required a total splenectomy. The blood transfusion regimen is an important consideration in establishing a treatment program. No relapses occurred in patients with the high-transfusion regimen, but did occur in those on the low-transfusion regimen. (Consumer Summary produced by Reliance Medical Information, Inc.)

Health aspects, Blood transfusion

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Intradermal hepatitis B vaccination in thalassaemia

Article Abstract:

Thalassemia is a hereditary form of anemia, characterized by decreased production of hemoglobin, the substance in red blood cells that transports oxygen. The treatment of thalassemia often requires multiple blood transfusions, and consequently, these patients are at high risk for contracting hepatitis B (a blood-borne viral illness that affects the liver). Although hepatitis B vaccine is available, patients with thalassemia often require repeated immunization to maintain immunity against hepatitis B. For 56 children, previously immunized against hepatitis B, a booster vaccination was given intradermally, that is, injected into the deeper layers of the skin. The antibodies produced by the patient were assayed two weeks after the booster shot, and inoculations were repeated at two week intervals until the measured antibodies reached the desired level. The procedure produced effective levels of antibodies rapidly, and required a small dose of vaccine. It is suggested that intradermal injection may be the preferred site for inoculating children with thalassemia against hepatitis B. Although in some instances the intradermal route of injection is not particularly effective for adult patients, this study has demonstrated very successful intradermal immunization of children. (Consumer Summary produced by Reliance Medical Information, Inc.)

Methods, Prevention, Risk factors, Vaccination, Hepatitis B

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