Seshadri, R., Colebatch, J. H., and Fisher, R. (1974). Archives of Disease in Childhood, 49, 195. Urinary iron excretion in thalassaemia after desferrioxamine administration. Urinary iron excretion induced with desferrioxamine (DFA) was estimated in 26 children with thalassaemia major. Four separate 24-hour urine collections were made-during a baseline period, after intramuscular injection of DFA 1 g, during blood transfusion with DFA 0 5 g/unit transfused blood, and during the post-transfusion period. Urinary iron, plasma iron, and total iron-binding capacity were estimated by atomic absorption spectrophotometry. Urinary iron excretion in the 24 hours after 1 g DFA ranged from 1 1 to 23 mg/M2 surface area compared with 0 * 1 to 1 * 6 mg/M2 during the baseline period. A positive correlation was obtained between DFA-induced urinary iron excretion and the amount of blood transfused, plasma iron level, and size of the spleen. Splenectomized patients excreted less iron after DFA than those who were not splenectomized. DFA-induced urinary iron was measured before and after splenectomy in 6 patients. In 5 of the 6 patients a drop in iron excretion was observed and the analysis of the 6 pairs of results indicated that splenectomy produced a decrease in DFA-induced urinary iron excretion.These findings indicate that the enlarged spleen in thalassaemia is a major source of iron chelated by DFA. It is suggested that treatment with DFA in the presence of a large spleen may be ineffective in removing excess iron from the myocardium and liver.The survival of children with thalassaemia major depends on repeated blood transfusions. Marked iron deposition develops in various vital organs, and if this is untreated it leads to a severe degree of morbidity and finally death, commonly during the second decade of life. Hence, assessment of the degree of iron overload is important in the prognosis and management of children with thalassaemia major. In recent years desferrioxamine (DFA), a specific and effective chelator of iron, has been used for the assessment of iron stores (Ploem et al., 1966;Hedenberg, 1969 size. They also suggest that splenectomy diminishes urinary iron excretion, which indicates that the spleen plays a major role in iron chelation by DFA in thalassaemia major.
PatientsIn 1972-73, 26 patients with thalassaemia major in the Royal Children's Hospital, Melbourne, were studied. There were 13 boys and 13 girls, their ages at the time of the study ranging from 2 to 21 years with a median of 10 years. The 3 oldest patients, aged 15, 21, and 21 *ears, will be discussed separately because in addition to splenectomy they had been receiving DFA daily for 5 to 8 years before the present study was carried out. This prolonged chelation therapy may have altered significantly the degree of iron overload compared with the remaining 23 patients. Those 23 patients, except for the ones who had received less than 50 units of blood, had clinical evidence of haemosiderosis such as skin pigmentation, cardiomegaly, and liver enlargeme#t...