Shah, F.T.; (2007) The relationship between non transferrin bound iron and iron overload in thalassaemia and sickle syndromes. Doctoral thesis , University of London.

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Abstract

Iron overload is a major cause of morbidity and subsequent mortality in patients with thalassaemia major, its effects in thalassaemia intermedia and sickle cell anaemia are however less well known. The presence of non transferrin bound iron is well described in adult thalassaemia patients but it is unclear as to when NTBI appears and what relationship it has to ineffective erythropoiesis and end organ damage. Data is presented on children with thalassaemia from a five-year prospective study showing that NTBI is present early in thalassaemia syndromes and this is probably due to ineffective erythropoiesis. In addition results from this study show that there is no relationship between markers of oxidative damage and NTBI in early childhood. Following this a comparison of adult patients with sickle cell anaemia and thalassaemia is undertaken looking at NTBI and cardiac iron burdens assessed by MRI. The thalassaemia patients at high liver iron burdens have a significant risk of cardiac iron loading and when patients with sickle cell anaemia and thalassaemia major are matched for liver iron it is seen that cardiac iron loading is not seen in sickle patients and this may be because NTBI is lower in this group. In the last chapter data is presented showing that serum pro-hepcidin is down regulated by NTBI, anaemia and erythropoietin in thalassaemia but not sickle syndromes. There is no clear relationship between pro-hepcidin and liver iron but hepcidin mRNA is down regulated by iron burden supporting the important role of this protein in iron regulation.

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