Manganese elevations in blood of children with congenital portosystemic shunts

Magnetic response imaging has demonstrated increased signal intensities wit hin the basal ganglia in patients with hepatic encephalopathy; the densitie s are considered to represent manganese deposition. We measured whole blood manganese concentrations in nine children with congenital portosystemic ve nous shunts detected by screening tests for galactosaemia. Beyond 1 year of age, these patients showed significantly higher manganese concentrations t han 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 f our patients, suggesting manganese accumulation. Conclusion Children with congenital portosystemic venous shunts showed mang anese elevations in blood and magnetic response imaging changes in the basa l ganglia. These children should avoid excessive manganese intake.