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Horm Mol Biol Clin Investig ; 39(2)2019 Jul 13.
Artigo em Inglês | MEDLINE | ID: mdl-31301670

RESUMO

Beta-thalassemia major is a subtype component of hemoglobinopathies; autosomal recessive disorders complicated with anemia that affect at least 50,000 babies each year. It contributes to problems in reproductive entities such as infertility due to iron deposition in the endocrine organs, which leads to malfunction of the hypothalamus-pituitary axis. Due to this, there have been very few pregnancies discovered and reported with this type of condition as they usually required an ovulation-induction agent with assisted reproductive technique to achieved pregnancy. We report a successful spontaneous pregnancy in a woman with beta-thalassemia major who underwent splenectomy with lifelong transfusion-dependence complicated with myocardial siderosis and osteoporosis. The close monitoring and regular blood transfusion are a core of successful support to this type of pregnancy. The unintentional consumption of Fosamax, hydroxyurea and deferiprone (Ferriprox) up till 20 weeks of gestation did not show any adverse effects on fetal well-being. As expected, this pregnancy ended with the preterm delivery via cesarean section due to intrauterine growth restriction with oligohydramnios, and currently, this child is thriving. We concluded that pregnancy is not a contraindication in beta-thalassemia major; complex individual care is needed to achieve a safe outcome for the mother.


Assuntos
Cardiomiopatias/etiologia , Homozigoto , Osteoporose/etiologia , Siderose/etiologia , Globinas beta/genética , Talassemia beta/complicações , Transfusão de Sangue , Cardiomiopatias/diagnóstico , Cardiomiopatias/tratamento farmacológico , Deferiprona/administração & dosagem , Deferiprona/uso terapêutico , Feminino , Humanos , Quelantes de Ferro/administração & dosagem , Quelantes de Ferro/uso terapêutico , Osteoporose/tratamento farmacológico , Gravidez , Complicações na Gravidez , Resultado da Gravidez , Siderose/diagnóstico , Siderose/tratamento farmacológico , Esplenectomia/métodos , Adulto Jovem , Talassemia beta/diagnóstico , Talassemia beta/etiologia
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