Pediatric patients with nonalcoholic fatty liver disease (NAFLD) undergo workup to exclude underlying diseases. For many patients, this may include screening for Wilson’s disease with a ceruloplasmin level. In a recent study, lower ceruloplasmin in 100 pediatric patients with NAFLD were associated with more severe NAFLD (JPGN 2013; 56: 370-75).
All patients had measurements of copper, iron, ceruloplasmin, transferrin ferroxidase activity, and ferritin; these assays were from archival serum samples from a cohort with biopsy-proven NAFLD. These patients had undergone testing for other etiologies of liver disease.
The authors were trying to determine if oxidative stress and its association with iron or copper may be playing a role in the severity of NAFLD. Those with lower severity NAFLD score (< 5) had a mean ceruloplasmin of 36.4 mg/dL (standard deviation 5.3); in contrast, those with higher severity NAFLD score (≥5) who had a mean ceruloplasmin of 28.1 mg/dL (standard deviation 7.2). That is, there was an inverse association between ceruloplasmin levels and the severity of NAFLD score. Lower ceruloplasmin was associated with increased inflammation, more ballooning histology, and more steatosis.
Key point:
Lower ceruloplasmin (<28.6 mg/dL) had a 92% specificity and 76% sensitivity for identifying more severe NAFLD. Thus, even a borderline-low ceruloplasmin that does not suggest Wilson’s disease may be useful in discriminating children more likely to need a liver biopsy.
Related blog entry:
gutsandgrowth 