Manganese deposits in patients with biliary atresia after hepatic porto-enterostomy.
OBJECTIVE
The aim of this study was to determine if there is latent manganese toxicity in patients with biliary atresia.


METHODS
Fifteen children with biliary atresia were examined postoperatively with regard to whole-blood manganese levels using brain magnetic resonance imaging (MRI) and I-123 iodoamphetamine (IMP) per rectal portal scintigraphy.


RESULTS
Nine (60%) of the 15 had high whole-blood manganese levels (mean, 4.1 microg/dL; range, 1.2 to 9.6; normal, 0.5 to 2.5), and these 9 had hyperintense globus pallidus on T1-weighted images, with no corresponding signal change in T2 sequences. I-123 IMP per rectal portal scintigraphy was done for 13 patients to evaluate portosystemic shunt flow. 12 (92%) of these patients had an increased flow. Mean shunt ratio was estimated to be 41% (range, 0.6 to 98; normal, <5%). Encephalopathy was evident in only 1 patient.


CONCLUSIONS
Some patients with biliary atresia in the postoperative period have manganese deposits in globus pallidus on T1-weighted images and high whole-blood manganese levels, possibly caused by increased portsystemic shunt, and a latent or subclinical encephalopathy is also present.
Ikeda
S
S
Department of Pediatric Surgery, Kumamoto University Medical School, Honjo, Japan.
Sera
Y
Y
Yoshida
M
M
Ohshiro
H
H
Uchino
S
S
Oka
Y
Y
Lee
K J
KJ
Kotera
A
A
D000293:Adolescent; D000328:Adult; D001656:Biliary Atresia / Q000097:blood* / Q000150:complications / Q000473:pathology / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D005260:Female; D005917:Globus Pallidus / Q000737:chemistry / Q000473:pathology*; D006501:Hepatic Encephalopathy / Q000209:etiology; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D008345:Manganese / Q000032:analysis / Q000097:blood*; D020149:Manganese Poisoning / Q000150:complications / Q000175:diagnosis; D011171:Portoenterostomy, Hepatic
D008345:Manganese