Table 2

Patient details: fulminant hepatic failure

Patient no Age at diagnosis (y), sex Non-caeruloplasmin copper (μg/l)2-154 Caeruloplasmin2-150 (g/l) Liver copper2-151 (μg/g) Radiocopper study Kayser-Fleischer rings Urine copper2-152 (μg/24 h) Histology
2311, F4620.08380PresentCirrhosis
2412, FLow2-153 1100PresentCirrhosis
2515, F9250.151847Present6437Cirrhosis
2615, FLow2-153 420AbsentCirrhosis
2716, F17430.21767Present9375Cirrhosis
2818, M5000.09600Present844Cirrhosis
2944, F13300.14717AbsentCirrhosis
3054, F3250.181526Present2268Cirrhosis/CAH
  • 2-150 Normal caeruloplasmin 0.2–0.45 g/l;

  • 2-151 normal liver copper 15–60 μg/g dry weight liver;

  • 2-152 normal urine copper less than 100 μg/24 h;

  • 2-153 caeruloplasmin quantitated in optical density units;

  • 2-154 normal non-caeruloplasmin copper less than 375 μg/l, could not be quantitated in two patients as caeruloplasmin measured in OD units.

  • C/W WD: radiocopper study consistent with the diagnosis of Wilson's disease; CAH, chronic active hepatitis; FHF, fulminant hepatic failure.