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
BONAFEDE, R (2021). Hereditary Haematological Disorders In The Greek Population Of Cape Town. Afribary. Retrieved from https://tracking.afribary.com/works/hereditary-haematological-disorders-in-the-greek-population-of-cape-town
BONAFEDE, R.P. "Hereditary Haematological Disorders In The Greek Population Of Cape Town" Afribary. Afribary, 15 May. 2021, https://tracking.afribary.com/works/hereditary-haematological-disorders-in-the-greek-population-of-cape-town. Accessed 21 Nov. 2024.
BONAFEDE, R.P. . "Hereditary Haematological Disorders In The Greek Population Of Cape Town". Afribary, Afribary, 15 May. 2021. Web. 21 Nov. 2024. < https://tracking.afribary.com/works/hereditary-haematological-disorders-in-the-greek-population-of-cape-town >.
BONAFEDE, R.P. . "Hereditary Haematological Disorders In The Greek Population Of Cape Town" Afribary (2021). Accessed November 21, 2024. https://tracking.afribary.com/works/hereditary-haematological-disorders-in-the-greek-population-of-cape-town