Tests of organ function

The tests that are usually needed are listed in Table 4.4. Heart function is best tested by measurement of left ventricular ejection

Mri Liver With Too Much Iron

Figure 4.5 Magnetic resonance imaging T2* technique. Tissue appearances of liver and spleen: (a) normal; (b) tissue iron overload; (c) severe liver iron overload with normal cardiac iron;

(d) severe cardiac iron deposition with minimal liver iron deposition (reproduced from Anderson etal. 2001 with permission).

Figure 4.5 Magnetic resonance imaging T2* technique. Tissue appearances of liver and spleen: (a) normal; (b) tissue iron overload; (c) severe liver iron overload with normal cardiac iron;

(d) severe cardiac iron deposition with minimal liver iron deposition (reproduced from Anderson etal. 2001 with permission).

40 60

Figure 4.6 Relationships of myocardial T2* and left ventricular ejection fraction in patients with thalassaemia major and iron overload (reproduced from Anderson etal. 2001 with permission).

fraction and by tests for rhythm disturbance. Liver function assessment requires routine liver function tests as well as liver biopsy to assess liver structure and liver iron burden (histolo-gically and chemically). The endocrine system is also damaged by iron and appropriate tests are listed in Table 4.4. The anterior pituitary is particularly sensitive, with damage resulting in reduced growth and impaired sexual maturation. Direct damage to the ovaries or testes may also occur but is usually less important. Hypogonadic hypogonadism, defects of growth hormone secretion and in its receptor, and deficiency of insulin-like growth factor mainly account for growth failure; DFX may also cause this. Diabetes mellitus and pre-diabetes, due to iron deposition in the pancreatic islets, are frequent, especially in patients with genetic susceptibility. Hypothyroidism and hypo-parathyroidism are also common in poorly chelated patients. Osteoporosis is well recognized in iron-overloaded thalassaemia patients; it is due to multiple factors and is detected by bone density studies.

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