Table 1 Patient Demographics
Heart | 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 | 10 |
|---|---|---|---|---|---|---|---|---|---|---|
Race | White | White | White | White | White | White | White | White | White | Asian |
Origin | Italy | France | Italy | Portugal | Italy | Italy | Italy | Italy | Italy | Thailand |
Sex | Male | Female | Male | Female | Female | Male | Male | Male | Female | Male |
Death/Transplant Year | Death 2004 | Death 2005 | Death 1964 | Death 1972 | Death 1990 | Tx 1995 | Tx 1997 | Tx 1999 | Tx 2000 | Death 2005 |
Cause of death/Transplant | Stroke | Heart failure | Heart failure | Heart failure | Heart failure | Heart failure | Heart failure | Heart failure | Heart failure | Heart failure |
LV iron by spectrometry (mg/g dw) | 0.38 ± 0.13 | 8.2 ±1.4 | 9.5 ±1.9 | 25.9 ±10.3 | 7.7 ±1.5 | 3.6 ±0.8 | 3.4 ±0.5 | 5.9 ±1.0 | 8.8 ±1.9 | 5.6 ±1.4 |
LV T2* (ms) | 44.4 ± 5.3 | 4.7 ±0.6 | 3.7 ±0.6 | 2.0 ±0.4 | 3.6 ±0.5 | 8.0 ±1.0 | 7.7 ±1.2 | 4.4 ±0.6 | 3.9 ±0.6 | 5.8 ±1.4 |
Age at death/transplant (years old) | 46 | 62 | 10 | 15 | 20 | 23 | 24 | 21 | 31 | 24 |
Diagnosis | TM | SA | TM | TM | TM | TM | TM | TM | TM | TM |
Age at diagnosis (years) | 2 | 32 | 4 | 0.25 | 0 | 1 | 0 | 0 | 5 | 2 |
Height (cm) | 173 | 160 | 140 | 140 | 156 | 170 | 164 | 157 | 151 | 167 |
Weight (kg) | 63 | 51 | 33 | 30 | 51 | 54 | 61 | 57 | 57 | 50 |
Ferritin (ng/mL) | 100 | >4000 | na | na | 975 | 954 | 2531 | 820 | 155 | >4000 |
Mean 1Â yr Haemoglobin (g/dL) | 12.2 | 9.5 | 7.9 | na | 9.2 | 8.5 | 9.5 | 10.2 | 10.3 | 9.2 |
Age commenced transfusions (years) | 2 | 22 | 0.3 | 3 | 2 | 1 | 2 | 1 | 5 | 2 |
Units of blood in year before death/transplant | 36 | 50 | 17 | na | 24 | 24 | 24 | 25 | 24 | 30 |
Total estimated units transfused | 1584 | 2000 | 170 | 288 | 432 | 765 | 528 | 500 | 624 | 660 |
Age commenced chelation (years old) | 6 | 42 | No chelation | No chelation | 6 | 10 | 6 | 4 | 7 | 10 |
Chelation in year before death/transplant | DFO, DFP | DFO | None | None | DFO | DFO | DFO | DFO | DFO | DFO, DFP |
Hepatitis C | Yes | No | na | na | No | Yes | No | No | Yes | Yes |
Diabetes | Yes | Yes | na | Yes | na | No | Yes | Yes | Yes | Yes |
Splenectomy | Yes | No | Yes | Yes | Yes | Yes | Yes | Yes | Yes | Yes |
Osteoporosis | Yes | No | na | Yes | No | No | No | No | No | Yes |
Hypogonadism | Yes | No | Yes | Yes | No | No | No | Yes | No | na |
Hypothyroid | Yes | No | Yes | Yes | No | No | No | Yes | No | na |
Cardiac drugs | Diur Dig ACEI | ACEI | Dig | Dig | na | none | Dig Ami | CCB | Diur Dig | None |
Other cardiac conditions | AF | AF | none | none | AF | none | AF | AF | none | none |