Additional Laboratory Evaluation
Slit-lamp examination of our patient revealed Kayser-Fleischer rings. This is shown in Figure 3.
She also had an MRI of the liver. Figure 4 is a non-contrast-enhanced, T1-weighted magnetic resonance image showing no focal masses in the liver and splenomegaly with a probable splenic infarct. Figure 5 is a contrast-enhanced, early-phase ,T1-weighted image showing marked heterogeneity of the liver, suggestive of fibrosis. Figure 6 is a contrast-enhanced, delayed-phase, T1-weighted image showing absence of enhancing focal hepatic lesions. Figure 7 is a T2-weighted image showing the darker density of liver relative to spleen, suggestive of metallic deposition.
Results of copper studies were as follows: ceruloplasmin, 19 mg/dL (normal range, 20-35 mg/dL); serum copper, 59 mcg/L (normal range, 700-1500 mcg/L); urinary copper, 16 mcg/day (normal range, < 30 mcg/day).
The patient underwent liver biopsy. Figure 8 shows a low-power view with a portal tract infiltrated by inflammatory cells that "spill" across the limiting plate into the liver lobule. There is also bile duct proliferation. Figure 9 shows a higher-power view demonstrating steatosis, ballooning degeneration, and hepatocellular necrosis. Figure 10 is a higher-power view showing dead and dying hepatocytes.
Collectively, these slides demonstrate histologic evidence of severe active chronic hepatitis. The copper stain was negative for copper.
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