How does thalassemia affect the skeletal system Essay

How does thalassemia affect the skeletal system - Essay Example Thalassemia is a hereditary disorder characterized by a diminished or a malfunction of the production of hemoglobin; a blood protein that transports oxygen to the tissues, which results from the mutation of the manufacture of hemoglobin primarily because of the abnormality of the bones and the enlargement of the spleen. Anemia is a good example that portrays severe cases of Thalassemia; bone and growth deformities, defects and hemosiderosis (Barber & Stringer, 2011). The most common Thalassemias are alpha-Thalassemia and beta-Thalassemia whose frequency of occurrence is around 1.7% worldwide. Alpha-Thalassemia comes from the abnormal production of the ÃŽ ±-globin chain while ÃŽ ²-Thalassemia is from the ÃŽ ²-globin chain (Barber & Stringer, 2011). Alpha-Thalassemia affects mainly people from Africa and South-East Asia while beta-Thalassemia is prevalent among people from the Mediterranean, Africa, and South-East Asia. The predominance of ÃŽ ²-Thalassemia is higher in areas where malaria is or was once an endemic. Severe changes occur on the calvarium due to porotic hyperostosis as shown in figure 1 below. This manifests extensively on the frontal portion including the inferior portion, the parietals and the squamous of the occipital through porosis (Lagia et al., 2007). Due to Thalassemia, patients often experience extensive pitting in the posterior portion of the skull, which includes the parietal and occipital bones as shown in figure 3. There is also mild pitting of the frontal bone and the orbital roof as demonstrated in figure 2. For this reason, the cranial bones become thicker than usual, wavering from typical 4.5mm to a maximum of 10mm in thickness (Filon et al., 1995). The expanded diploe can be seen on the external surface, which becomes eroded over time. The trabeculae of the diploe become increasingly reduced in number, accompanied by the thickening and radial arrangement of the trabeculae that are left;