Manganese elevations in blood of children with congenital portosystemic shunts

Magnetic response imaging has demonstrated increased signal intensities within the basal ganglia in patients with hepatic encephalopathy; the densities are considered to represent manganese deposition. We measured whole blood manganese concentrations in nine children with congenital portosystemic venous shunts detected by screening tests for galactosaemia. Beyond 1 year of age, these patients showed significantly higher manganese concentrations than controls (2.40 +/- 0.43 versus 1.48 +/- 0.38 mug/dl; P = 0.0001). Four of the nine patients were studied by magnetic response imaging. T1-weighted images showed increased signal intensities in the basal ganglia of those four patients, suggesting manganese accumulation. Conclusion Children with congenital portosystemic venous shunts showed manganese elevations in blood and magnetic response imaging changes in the basal ganglia. These children should avoid excessive manganese intake.