RESUMO
We describe the management and clinical outcome of pregnancies among 100 Greek Cypriot women with thalassaemia: 88 with thalassaemia major and 12 with thalassaemia intermedia. A total of 152 successful pregnancies and 161 deliveries were included. All patients had endocrine assessment and frequent ferritin measurements. Multiple successful pregnancies included 7 twins and 1 triple pregnancy. Pregnant thalassaemics required significantly larger amount of total blood transfusion during pregnancy. There was a statistically significant increase in the ferritin levels during pregnancy, and levels remained significantly higher after pregnancy. Most pregnancies resulted in delivery of full-term healthy babies, and obstetric complications were rare, although some problems were encountered.
Assuntos
Transfusão de Sangue/estatística & dados numéricos , Cuidado Pós-Natal/organização & administração , Complicações Hematológicas na Gravidez/terapia , Resultado da Gravidez/epidemiologia , Cuidado Pré-Natal/organização & administração , Talassemia/terapia , Assistência ao Convalescente , Parto Obstétrico/métodos , Parto Obstétrico/estatística & dados numéricos , Feminino , Ferritinas/sangue , Aconselhamento Genético , Grécia/epidemiologia , Necessidades e Demandas de Serviços de Saúde , Humanos , Monitorização Fisiológica , Guias de Prática Clínica como Assunto , Gravidez , Complicações Hematológicas na Gravidez/sangue , Complicações Hematológicas na Gravidez/diagnóstico , Complicações Hematológicas na Gravidez/epidemiologia , Estudos Retrospectivos , Estatísticas não Paramétricas , Talassemia/sangue , Talassemia/diagnóstico , Talassemia/epidemiologiaRESUMO
We describe the management and clinical outcome of pregnancies among 100 Greek Cypriot women with thalassaemia: 88 with thalassaemia major and 12 with thalassaemia intermedia. A total of 152 successful pregnancies and 161 deliveries were included. All patients had endocrine assessment and frequent ferritin measurements. Multiple successful pregnancies included 7 twins and 1 triple pregnancy. Pregnant thalassaemics required significantly larger amount of total blood transfusion during pregnancy. There was a statistically significant increase in the ferritin levels during pregnancy, and levels remained significantly higher after pregnancy. Most pregnancies resulted in delivery of full-term healthy babies, and obstetric complications were rare, although some problems were encountered
Assuntos
Talassemia , Mulheres , Transfusão de Sangue , Ferritinas , Estudos Retrospectivos , Hormônios , Testes de Função Cardíaca , Resultado da GravidezRESUMO
Patients with beta-thalassaemia major frequently suffer from hypersiderosis which leads to hemochromatosis of major organs such as the heart and liver. Little information exists about the ultrastructural pathology of the human heart in beta-thalassaemia patients. Five Cypriot patients with elevated blood ferritin and intractable heart failure were investigated. Cardiac biopsies from these patients were studied by light and electron microscopy, as well as by X-ray microanalysis. Ultrastructural examination revealed the presence of disrupted myocytes showing loss of myofibers, dense nuclei, and a variable number of pleomorphic electron dense granules. These cytoplasmic granules or siderosomes consisted of iron-containing particles as confirmed by X-ray microanalysis. It is likely that the ultrastructural changes observed in myocytes of patients with beta-thalassaemia are largely due to iron deposition.
