.
Microcytic Anemia
These are associated with an inability to produce hemoglobin. Hemoglobin
consists of iron inserted into the prtoporphyrin ring complex to form heme
which in turn is inserted into the globin chain. Hence these anemias
are seen in:
iron deficiency - absence of iron
chronic disease - iron unavailable
thalassemia - inability to produce globin chains
sideroblastic anemia- inability to produce heme
........................................ ...........................
THALASSEMIA
Thalassemia is a disease which was originally widespreaded in the Mediterranean Basin, South-East Asia and various countries in equatorial Africa. However, following its persistent migration over planet it is today verifiable in almost all regions of the globe.
The term "Thalassemia" implies a genetic disorder of an extremely heterogenous group which is characterised by a reduced or erroneous production of haemoglobin, the respiratory pigment contained in the red cells. The probability of a child being born affected by Thalassemia Major - the most serious form of this genetic disorder also known as "ß-Thalassemia or Cooleys Disease" - is a 25% chance if the parents are carriers, that is to say if both possess one of the two genes for erroneously coded haemoglobin in their cromosomic make-up. It is estimed that in Italy only, the number of patients affected by Thalassemia Major flucuates between 5,000 and 8,000 individuals.
The genetic defect which characterises Thalassemia Major is intrinsic to the haemopoietics cells, the progenitor cells of the elements which appear in the blood (red and white cells, blood plates) which are present in bone marrow. If up until now correct transfusion therapy has been the only available treatment for patients affected by Thalassemia Major, it is today possible to cure the disease by way of performing bone marrow transplant using a compatible donor and in this way replacing diseased cells whit healty ones.
http://www.abanet.it/fondazioneberloni/ing/talassem.htm
........................................ ..................
Sickle cell anemia and S-thalassemia in Sicilian children
HbS is endemic in Sicily area for and this anomaly has been described in Sicilians and in people of Sicilian ancestry (3). For thousands of years the Mediterranean basin has been the crossroad for trade, races, ideas, and art. The geographical position of Sicily at the center of the Mediterranean made it a natural stopover on these journeys. Phoenicians, Greeks, Carthaginians, Byzantines, Saracens, Normans, Spaniards, Arabs, Jews, and mercenaries from allover the world came to Sicily in large numbers to settle. In contrast with the past, there has been almost no immigration during the last few centuries. The genetic structure of the Sicilians is clearly not due to recent additions. The consensus is that the gene was introduced into Sicily and Southern Italy from Northern Africa through the trans-Saharan trade routes, or, alternatively, by means of the Greek colonisation, although the introduction of the gene into Sicily during the Muslim invasion cannot be excluded
Bookmarks