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      TALASEMIA ASOCIADA A EMBARAZO

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          Abstract

          La Talasemia es un desorden congénito hemolítico causado por una deficiencia parcial o completa de la síntesis de las cadenas alfa o beta de las globinas de la hemoglobina. Se manifiesta en una amplia gama de cuadros clínicos que van desde la muerte intrauterina hasta la microcitosis asintomática sin anemia. El depósito de hierro constituye la complicación más importante de la talasemia y su mayor preocupación en el manejo. Existen escasos reportes de esta condición asociada a embarazo, se sabe poco acerca de su manejo y de las complicaciones que puede tener en el embarazo. Presentamos una paciente portadora de Talasemia, en la cual se manejó su embarazo en nuestro servicio

          Translated abstract

          Thalassemia is a hemolytic congenital disorder caused by a partial or complete of the alpha or beta chain synthesis deficiency of the globins of the hemoglobin. It is presented in an ample range of clinical pictures that go from the intrauterine death to the asymptomatic microcytosis without anemia. The iron deposit constitutes the most important complications of Thalassemia and the major problem in handling. Exists few reports of this condition in pregnancy, it is known little about its handling and of the complications that can have in the pregnancy. We presented a patient carrier of Thalassemia, with pregnancy, assisted in our department

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          Most cited references63

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          Iron-chelating therapy and the treatment of thalassemia.

          Iron-chelating therapy with deferoxamine in patients with thalassemia major has dramatically altered the prognosis of this previously fatal disease. The successes achieved with deferoxamine, as well as the limitations of this treatment, have stimulated the design of alternative strategies of iron-chelating therapy, including orally active iron chelators. The development of the most promising of these, deferiprone, has progressed rapidly over the last 5 years; data from several trials have provided direct and supportive evidence for its short-term efficacy. At the same time, the toxicity of this agent mandates a careful evaluation of the balance between risk and benefit of deferiprone in patients with thalassemia, in most of whom long-term deferoxamine is safe and efficacious therapy.
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            Survival in medically treated patients with homozygous beta-thalassemia.

            The prognosis of patients with homozygous beta-thalassemia (thalassemia major) has been improved by transfusion and iron-chelation therapy. We analyzed outcome and prognostic factors among patients receiving transfusions and chelation therapy who had reached the age at which iron-induced cardiac disease, the most common cause of death, usually occurs. Using the duration of life without the need for either inotropic or antiarrhythmic drugs as a measure of survival without cardiac disease, we studied 97 patients born before 1976 who were treated with regular transfusions and chelation therapy. We used Cox proportional-hazards analysis to assess the effect of prognostic factors and life-table analysis to estimate freedom from cardiac disease over time. Of the 97 patients, 59 (61 percent) had no cardiac disease; 36 (37 percent) had cardiac disease, and 18 of them had died. Univariate analysis demonstrated that factors affecting cardiac disease-free survival were age at the start of chelation therapy (P < 0.001), the natural log of the serum ferritin concentration before chelation therapy began (P = 0.01), the mean ferritin concentration (P < 0.001), and the proportion of ferritin measurements exceeding 2500 ng per milliliter (P < 0.001). With stepwise Cox modeling, only the proportion of ferritin measurements exceeding 2500 ng per milliliter affected cardiac disease-free survival (P < 0.001). Patients in whom less than 33 percent of the serum ferritin values exceeded 2500 ng per milliliter had estimated rates of survival without cardiac disease of 100 percent after 10 years of chelation therapy and 91 percent after 15 years. The prognosis for survival without cardiac disease is excellent for patients with thalassemia major who receive regular transfusions and whose serum ferritin concentrations remain below 2500 ng per milliliter with chelation therapy.
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              Venous thromboembolism and hypercoagulability in splenectomized patients with thalassaemia intermedia.

              Thromboembolic phenomena have been described in patients with thalassaemia intermedia and major, although there are relatively few epidemiological data on the overall frequency of these complications. To obtain more insight into the risk and mechanism of venous thromboembolism in thalassaemia, the aims of this study were: (i) to establish retrospectively the prevalence of thromboembolic events in a large group of adults with thalassaemia intermedia and major during a follow up period of 10 years; (ii) to measure in subgroups of these patients sensitive markers of activation of coagulation and fibrinolysis enzymes; and (iii) to look for possible procoagulant mechanisms. A high prevalence of thromboembolic events was found, particularly in splenectomized patients with thalassaemia intermedia (29%). These patients had high plasma levels of markers of coagulation and fibrinolysis activation. Furthermore, thalassaemic red cells and erythroid precursors from splenectomized patients with thalassaemia intermedia had an enhanced capacity to generate thrombin. To evaluate the role of splenectomy per se on procoagulant activity, we evaluated the capacity to form thrombin in healthy individuals who had been splenectomized for trauma. They produced the same amount of thrombin as non-splenectomized controls. In conclusion, the results of this study show the existence of a hypercoagulable state in splenectomized patients with thalassaemia intermedia and that their red and erythroid cells are capable of acting as activated platelets in thrombin generation.
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                Author and article information

                Journal
                rchog
                Revista chilena de obstetricia y ginecología
                Rev. chil. obstet. ginecol.
                Sociedad Chilena de Obstetricia y Ginecología (Santiago, , Chile )
                0048-766X
                0717-7526
                2003
                : 68
                : 2
                : 124-128
                Affiliations
                [01] orgnameUniversidad de Antofagasta orgdiv1Hospital Regional de Antofagasta orgdiv2Servicio de Ginecología y Obstetricia Chile
                Article
                S0717-75262003000200006 S0717-7526(03)06800206
                10.4067/S0717-75262003000200006
                8b84ce5a-55e0-418e-9616-8ea855771765

                This work is licensed under a Creative Commons Attribution 4.0 International License.

                History
                : December 2002
                : May 2003
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 39, Pages: 5
                Product

                SciELO Chile

                Categories
                Caso Clínico

                Thalassemia,Talasemia y embarazo,pregnancy
                Thalassemia, Talasemia y embarazo, pregnancy

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