Hereditary haematological disorders in the Greek population of Cape Town

 

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dc.contributor.author Bonafede, R P en_ZA
dc.date.accessioned 2015-09-14T08:06:55Z
dc.date.available 2015-09-14T08:06:55Z
dc.date.issued 1979 en_ZA
dc.identifier.citation Bonafede, R. 1979. Hereditary haematological disorders in the Greek population of Cape Town. University of Cape Town. en_ZA
dc.identifier.uri http://hdl.handle.net/11427/13847
dc.description.abstract It is a recognized fact that most well defined population groups have their own typical pattern of inherited disorders. The genetic conditions most commonly found in Greek persons are the thalassaemias, glucose-6-dehydrogenase (G-6-PD) deficiency and, to a lesser extent, certain of the haemoglobinopathies. The clinical and socio-economic consequences of these disorders are significant. In the homozygous state, alpha-thalassaemia is incompatible with life, while thalassaemia major (homozygous beta-thalassaemia) results in a severe anaemia with death usually occurring in the second or third decades. Treatment to prolong the life of these patients is very costly. Alpha- and betathalassaemia, when heterozygous, may result in a mild anaemia or be asymptomatic. G-6-PD deficiency results in attacks of haemolysis on ingestion of certain medical preparations and is of far less importance than the thalassaemias. Haemoglobin Sis the commonest haemoglobinopathy occurring in Greeks and results in a severe clinical condition when homozygous, as with the thalassaemias. However, the gene frequency is far less than that of the thalassaemias. The high prevalence of G-6-PD deficiency and haemoglobin Shave been demonstrated to be due to the selective advantage they confer against malaria. This same mechanism probably applies to the thalassaemias but has not been proven. Thus, these disorders have been demonstrated to occur more frequently in low-lying areas and places where malaria was endemic in the past. Population movement has made this situation less clearcut in recent times. Cape Town has a Greek population numbering approximately 5000 persons. As the thalassaemias, particularly, cause a notable public health problem in Greece, it could be expected that the position would be similar, but on a smaller scale in Cape Town. For these reasons it was decided to undertake a study in Cape Town to ascertain exactly what the position was and what recommendations to make concerning management and prevention. en_ZA
dc.language.iso eng en_ZA
dc.subject.other Medicine en_ZA
dc.title Hereditary haematological disorders in the Greek population of Cape Town en_ZA
dc.type Doctoral Thesis
uct.type.publication Research en_ZA
uct.type.resource Thesis en_ZA
dc.publisher.institution University of Cape Town
dc.publisher.faculty Faculty of Health Sciences en_ZA
dc.publisher.department Department of Medicine en_ZA
dc.type.qualificationlevel Doctoral
dc.type.qualificationname MD en_ZA
uct.type.filetype Text
uct.type.filetype Image
dc.identifier.apacitation Bonafede, R. P. (1979). <i>Hereditary haematological disorders in the Greek population of Cape Town</i>. (Thesis). University of Cape Town ,Faculty of Health Sciences ,Department of Medicine. Retrieved from http://hdl.handle.net/11427/13847 en_ZA
dc.identifier.chicagocitation Bonafede, R P. <i>"Hereditary haematological disorders in the Greek population of Cape Town."</i> Thesis., University of Cape Town ,Faculty of Health Sciences ,Department of Medicine, 1979. http://hdl.handle.net/11427/13847 en_ZA
dc.identifier.vancouvercitation Bonafede RP. Hereditary haematological disorders in the Greek population of Cape Town. [Thesis]. University of Cape Town ,Faculty of Health Sciences ,Department of Medicine, 1979 [cited yyyy month dd]. Available from: http://hdl.handle.net/11427/13847 en_ZA
dc.identifier.ris TY - Thesis / Dissertation AU - Bonafede, R P AB - It is a recognized fact that most well defined population groups have their own typical pattern of inherited disorders. The genetic conditions most commonly found in Greek persons are the thalassaemias, glucose-6-dehydrogenase (G-6-PD) deficiency and, to a lesser extent, certain of the haemoglobinopathies. The clinical and socio-economic consequences of these disorders are significant. In the homozygous state, alpha-thalassaemia is incompatible with life, while thalassaemia major (homozygous beta-thalassaemia) results in a severe anaemia with death usually occurring in the second or third decades. Treatment to prolong the life of these patients is very costly. Alpha- and betathalassaemia, when heterozygous, may result in a mild anaemia or be asymptomatic. G-6-PD deficiency results in attacks of haemolysis on ingestion of certain medical preparations and is of far less importance than the thalassaemias. Haemoglobin Sis the commonest haemoglobinopathy occurring in Greeks and results in a severe clinical condition when homozygous, as with the thalassaemias. However, the gene frequency is far less than that of the thalassaemias. The high prevalence of G-6-PD deficiency and haemoglobin Shave been demonstrated to be due to the selective advantage they confer against malaria. This same mechanism probably applies to the thalassaemias but has not been proven. Thus, these disorders have been demonstrated to occur more frequently in low-lying areas and places where malaria was endemic in the past. Population movement has made this situation less clearcut in recent times. Cape Town has a Greek population numbering approximately 5000 persons. As the thalassaemias, particularly, cause a notable public health problem in Greece, it could be expected that the position would be similar, but on a smaller scale in Cape Town. For these reasons it was decided to undertake a study in Cape Town to ascertain exactly what the position was and what recommendations to make concerning management and prevention. DA - 1979 DB - OpenUCT DP - University of Cape Town LK - https://open.uct.ac.za PB - University of Cape Town PY - 1979 T1 - Hereditary haematological disorders in the Greek population of Cape Town TI - Hereditary haematological disorders in the Greek population of Cape Town UR - http://hdl.handle.net/11427/13847 ER - en_ZA


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