Summary. In this non‐randomized prospective study, liver and spleen iron concentrations were monitored annually over a 4‐year period by non‐invasive Superconducting Quantum Interference Device biomagnetometry in 54 β‐thalassaemia major patients (age, 7–22 years) receiving treatment with deferiprone (75 mg/kg/d). Median liver iron concentrations increased significantly from 1456 to 2029 and 2449 µg/gliver at baseline, after 2·0 and 3·2 years respectively. Another group of 51 thalassaemic patients (aged 4–34 years) who received desferrioxamine s.c. for 1·9 years increased their liver iron concentration from 1076 to 1260 µg/gliver. Taking into account the increase of the daily iron input from transfusions of 3·6 mg/d, caused by weight gain in 67% of the patients treated with deferiprone, a larger total body iron elimination rate was achieved after 2 years than at baseline. A negative ferritin change was observed in 51% of the patients. In 15 non‐splenectomized patients, liver iron significantly increased from 1260 to 1937 µg/gliver (P < 0·01), but serum ferritin remained stable at 2100 µg/l, as did the spleen iron concentration at 1200 µg/gspleen. A two‐compartment model may predict an average chelation efficacy for desferrioxamine and deferiprone, with a saturation effect of the latter, for a certain chelation and transfusion regimen by a single liver iron quantification.
It is concluded from the present investigation that the two scoring methods represent good diagnostic tools with high agreement rates in critical ill ICU patients.
To investigate the applicability of noninvasive Superconducting Quantum Interference Device (SQUID) biomagnetic liver susceptometry and its limitations in thalassemic children , 23 patients with-thalassemia major and other iron loading anemias (age: 4-16 years) and 16 age-related normal children were studied. Liver iron concentrations ranged from 600 to 11,000 µg/g liver for thalassemic patients and from 60 to 340 µg/g liver for normal patients. Measuring the respective organ volumes by sonography, liver and spleen iron stores, accounting for 80% of total body iron stores, were estimated. Nonliver contributions from the lung or intestine to the measured SQUID signals in the small-sized patients were not observed. Moreover, livers in thalassemia were found to be enlarged by 18% per 1,000 µg/g (r = 0.75, P < 10 −3). Serum ferritin values correlate significantly with iron stores (r = 0.64, P < 10 −3), but predict iron stores only within large error intervals of 4,000 µg/g liver. Analyzing the experimental data from biomagnetometry and from related transfusion and chelation treatment data within the framework of a two-compartment model, we were able to derive additional information on total body iron elimination and chelation therapy efficacy. The exponential decline of iron stores allows forecast of steady-state conditions of the final iron load for a particular transfusion and chelation therapy regi-men. Am. J. Hematol. 60:289-299, 1999.
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