Gonadal function after allogenic bone marrow transplantation for thalassaemia

Article Abstract:

Thalassemia consists of a group of hereditary anemias. It tends to occur in populations of Mediterranean and Southeast Asian descent, and results from the abnormal production of hemoglobin, the oxygen-carrying pigment of red blood cells. Thalassemia major is a severe form of the disease which develops in childhood. It is characterized by fatigue, severe anemia, enlargement of the heart, slight jaundice (yellowing of the skin), leg ulcers, and the formation of gallstones. Increased activity of the bone marrow may result in thickening of the skull and projection of the cheekbones. Generally, the younger the person is at the time of diagnosis, the worse the outcome. Thalassemia can be treated by bone marrow transplantation. Prior to bone marrow transplant, however, anticancer agents are administered to destroy the patient's bone marrow and suppress the immune system (and thereby reduce graft rejection). The effects of chemotherapy on endocrine function have been researched in adolescents and adults, but there is limited information about the unfavorable side effects of chemotherapy on children's endocrine function, specifically gonadal function (the ovaries in females and testes in males). The gonadal function of thalassemic children, who typically experience some endocrine dysfunction, has not been studied in relation to bone marrow transplantation. Pituitary gonadal function (the release of gonadal hormones from the pituitary gland) was assessed in 30 prepubertal thalassemic patients (15 females and 15 males; average age 12.9 years) who received a bone marrow transplant. The patients had been treated with the drugs busulfan and cyclophosphamide prior to transplantation, and pituitary gonadal function was assessed 0.7 and 5.1 years later. Gonadotrophin levels were increased, indicating gonadal damage, in 80 percent of girls. Most boys had reduced gonadotrophin and testosterone responses to activators of release, possibly due to iron overload and/or cytotoxic (cell-killing) drugs. These findings demonstrate that young thalassemic patients treated with chemotherapy for bone marrow transplantation should be followed-up for a long period of time to determine their need for hormone replacement therapy. (Consumer Summary produced by Reliance Medical Information, Inc.)

Evaluation, Complications and side effects, Physiological aspects, Bone marrow, Bone marrow transplantation, Gonads, Organ transplantation, Pediatric surgery, Transplantation of organs, tissues, etc. in children

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Social integration of the older thalassaemic patient

Article Abstract:

Beta thalassemia is a chronic genetic disorder of the blood that is characterized by severe hemolytic anemia, the rupture of red blood cells leading to a decreased capacity of the blood to carry oxygen. This disorder results from the abnormal production of beta chains, which are a portion of the oxygen-carrying pigment of red blood cells called hemoglobin. Patients with thalassemia require frequent blood transfusions for the anemia. More extensive therapy including agents to remove iron, hospitalization, and regular medical supervision may be needed to treat complications of the disease. Thalassemia was previously considered to be a fatal disease, but survival has improved due to advances in treatment. The psychological element is important in survival with thalassemia, and psychosocial support plays a significant role in the management of this disease. Psychological and social problems may arise from complications associated with treatment, and interference with employment and education of the thalassemic patient. The social integration of adolescent and young adult thalassemic patients was assessed. The subjects were 171 persons from Greece and 112 individuals from Italy, who were patients of established specialist centers for the care of thalassemic patients. These facilities offered psychosocial support to their patients, who showed good social integration and a favorable self-image. These findings suggest that psychosocial support may be beneficial in achieving social integration for thalassemic patients. (Consumer Summary produced by Reliance Medical Information, Inc.)

Psychological aspects, Social aspects

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Microcytosis, iron deficiency, and thalassaemia in preschool children

Article Abstract:

Microcytosis, a decrease in the size of red blood cells, affects Asian children more often than European children of the same age. This may be caused by a lack of iron in the diet or a condition known as thalassaemia, in which there is an aberration of the oxygen-carrying blood protein hemoglobin. Children with microcytosis are often treated with iron supplements to counteract their low iron levels. In a recent study, 204 Asian children and 88 European children attending community infant welfare clinics were screened for abnormalities of their red blood cells. Thirty-seven percent of Asian children and 12 percent of European children were found to have very small red blood cells. Twenty-one percent of the Asian children with microcytosis were found to have thalassaemia and 66 percent were found to have a dietary lack of iron. Tests that measured the ability of the blood to bind iron were more effective in diagnosing dietary iron deficiency than other tests. These results indicate that a large number of children with microcytosis may be given unnecessary iron treatments. Children who do not respond to iron supplements should be suspected of having thalassaemia or of not taking their medication. It is important that blood be monitored often to determine the effectiveness of iron supplements. (Consumer Summary produced by Reliance Medical Information, Inc.)

Diagnosis, Drug therapy, Iron deficiency anemia

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