Cases reported "beta-Thalassemia"

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1/18. High-risk pregnancy in beta-thalassemia major women. Report of three cases.

    Reproductive failure is common in beta-thalassemia major patients because of endocrine damage resulting from iron overload. Here 3 full-term pregnancies following spontaneous ovulation in 2 splenectomized beta-thalassemia major women are reported. The main echocardiographic parameters, such as left ventricular end-diastolic and end-systolic diameters, fractional shortening and ejection fraction, were within the normal range before pregnancy, but worsened during gestation, and 1 patient developed pre-congestive heart failure. deferoxamine therapy was continued throughout 2 pregnancies, while in the other it was stopped after 8 weeks: no abnormalities were noted in the children. Thanks to the currently applied therapies, an increased number of pregnancies may now be expected in beta-thalassemia major women: it is important to find out more about the pregnancy-related problems and their management in these patients.
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2/18. Effective treatment of beta-thalassemia intermedia during pregnancy with rHuEpo. A case report.

    The anemia of pregnancy in presence of beta-thalassemia intermedia usually aggravates pregnancy procedure. In the present study we investigated whether the administration of recombinant human erythropoietin (rHuEpo) recombinant human erythropoietin in combination with iron and folic acid may ameliorate blood indices as an alternative choice to blood transfusion.
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keywords = pregnancy
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3/18. Intravenous iron therapy for severe pregnancy anemia with high erythropoietin levels.

    BACKGROUND: To demonstrate that intravenous (IV) iron therapy rapidly can secure the physiologic correction of severe nonhemorrhagic anemia more safely than blood component therapy and recombinant erythropoietin treatment. CASE: An 18-year-old woman with beta-thalassemia in her 33rd week of gestation had a hemoglobin level of 4.8 g/dL and an erythropoietin value of 191 mU/mL. After IV iron administration, erythropoietin rapidly decreased and hemoglobin increased to 8.1 g/dL in correlation with estriol elevation. A healthy infant with normal hemoglobin and ferritin levels was delivered at 42 weeks by cesarean. CONCLUSION: Intravenous iron administration rapidly corrected severe nonhemorrhagic anemia in a pregnant patient and may produce an improvement in fetal indices. High erythropoietin levels predict a good response to iron and may obviate the need for blood transfusions and recombinant erythropoietin administration, at least until this therapy is tried.
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keywords = pregnancy, gestation
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4/18. erythropoietin use in a pregnant Jehovah's witness with anemia and beta-thalassemia: a case report.

    BACKGROUND: anemia in pregnancy is seen often because of iron deficiency and the "physiologic dilution" that occurs in the third trimester. Other causes include genetic conditions, such as sickle cell anemia and thalassemias. In cases not responding to iron therapy, patients occasionally require a blood transfusion to restore adequate circulating red blood cell mass. In patients belonging to the Jehovah's Witness sect, transfusion of blood products is not allowed, and treatment of anemia in pregnancy may require use of erythropoietin. CASE: A 26-year-old, African American woman belonging to the Jehovah's Witness sect presented with anemia associated with beta-thalassemia. Iron therapy and prenatal vitamins did not correct the anemia, and the patient became symptomatic, with fatigue and shortness of breath when walking. Therapy with synthetic erythropoietin corrected the anemia, and the patient had an otherwise-uncomplicated pregnancy and delivery. CONCLUSION: Synthetic erythropoietin has been used successfully in patients with renal failure and anemia. In pregnancy associated with renal failure and anemia, synthetic erythropoietin has been shown to be safe except for rare cases of hypertension. We treated anemia caused by beta-thalassemia in pregnancy with synthetic erythropoietin to avoid a transfusion in a Jehovah's Witness.
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ranking = 0.79744814705491
keywords = pregnancy
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5/18. Management of pregnancy in a patient with beta thalassaemia major.

    beta thalassaemia is one of the world's most wide-spread monogenetic disorders. Advances in the management of beta thalassaemia major by extensive blood transfusions and chelation therapy have improved survival of patients into adult life. Due to the prolonged life expectancy and improvements in quality of life, pregnancy has now become an important issue for patients and clinicians. We report a case of a pregnant patient with beta thalassaemia major who underwent a successful caesarean section under spinal anaesthesia. The multidisciplinary approach to management of beta thalassaemia major and pregnancy is discussed.
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keywords = pregnancy
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6/18. hemoglobin e-Saskatoon and pregnancy: report of two cases.

    hemoglobin e-Saskatoon (beta22-Glu-Lys) is found worldwide but is extremely rarely. Two cases of pregnant women who carried the abnormal hemoglobin and the various problems that arise from it are reported. A discussion of the combinations with other abnormal hemoglobin is also presented.
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keywords = pregnancy
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7/18. Fatal splenic rupture in a pregnant woman with hemoglobin c/beta-thalassemia and myeloid metaplasia.

    splenic rupture with intraperitoneal hemorrhage is a fatal condition that is rarely encountered during the third trimester of pregnancy; its pathogenetic mechanisms and causes are largely unknown. We report a case of splenic rupture in a pregnant woman that caused the death of the mother and child. The patient was a carrier of double heterozygosis for hemoglobin c/beta-thalassemia. spleen and liver enlargement due to extramedullary hematopoiesis was found at autopsy. Our data suggest that rare and hidden hematologic disorders should be considered as possible causes of splenic enlargement and rupture during pregnancy.
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ranking = 0.31897925882196
keywords = pregnancy
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8/18. Management of pregnancy when maternal blood has a very high level of fetal haemoglobin.

    fetal blood normally has a higher oxygen affinity than maternal blood because of the predominance of haemoglobin (Hb) F in the former and of Hb A in the latter; this predominance facilitates the transfer of oxygen from maternal to fetal blood. We report two patients who had exclusively or predominantly Hb F in their blood and were managed differently. When patient 1 became pregnant she had regular exchange blood transfusions in order to reduce her Hb F from 80% to below 50%; patient 2, who had 100% Hb F, was not transfused before, during or after her pregnancy. Each patient delivered a normal healthy baby. We conclude that the differential oxygen affinity produced by the combination of Hb A in the maternal blood and Hb F in the fetal blood is not indispensable to ensure an oxygen supply adequate for normal fetal development and growth.
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keywords = pregnancy
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9/18. Transfusion-dependent homozygous beta-thalassaemia major: successful twin pregnancy following in-vitro fertilization and tubal embryo transfer.

    Homozygous beta-thalassaemia (thalassaemia major) is a severe, transfusion-dependent anaemia that also causes infertility due to endocrine impairment. Very few pregnancies are reported among such patients and there is only one report in the literature referring to a pregnancy achieved with ovulation induction and intra-uterine insemination. We report here the first successful twin pregnancy following in-vitro fertilization and tubal embryo transfer in a transfusion-dependent homozygous beta-thalassaemic woman with an oligoasthenozoospermic partner. Prior to ovarian stimulation, desferrioxamine was discontinued due to potential fetotoxicity. Pre-gestational transfusional and chelating therapies were resumed after delivery. In such patients, ovulation induction and assisted reproductive techniques appear crucial in achieving pregnancy with concurrent haematological balance without desferrioxamine administration.
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ranking = 1.159489629411
keywords = pregnancy, gestation
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10/18. beta-thalassemia syndromes and pregnancy.

    The literature relating to homozygous beta-thalassemia and the problems these patients have during their pregnancy is reviewed and another case report of a twin pregnancy in a homozygous beta-thalassemia individual is included. The majority of cases described refer to the intermedia clinical form of the hematological problem because patients with thalassemia major generally have poor sexual development. Although the reported cases had a greater frequency of delayed development, only one woman suffered from infertility and required ovulation induction agents. The thalassemia pregnant mother faces deleterious consequences resulting from chronic anemia and in nonsplenectomized patients there remains the risk of the onset of a hypersplenic crisis. The problems of splenectomized patients during pregnancy is also discussed. The chronic anemia of thalassemic patients predisposes to a poor fetal outcome with greater fetal loss, preterm labor, and intrauterine growth retardation.
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keywords = pregnancy
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