Abstract
Purpose/Background: The aim of this study was to investigate latent manganese toxicity in patients with biliary atresia and to use this tool to predict the prognosis of biliary atresia patients after Kasai's portoenterostomy. Methods: 14 patients who underwent Kasai's portoenterostomy between 1993 and 2005 were included in the study. Whole blood manganese assays were performed using atomic adsorption spectroscopy (Philips PU 9200, Cambridge, UK) and verified by repeat analysis; these were done at the time of surgery and 6 months later. All blood samples were collected in an identical manner, blinded and then assayed by technicians. Whole blood manganese levels were correlated with the development of manganese deposits in the globus pallidus on T1-weighted images on magnetic resonance imaging (MRI) of the brain. Serum bilirubin of all 14 patients were noted both at the time of surgery and 6 months later. A hepatobiliary (HIDA) scan was done in all patients 6 months after Kasai's portoenterostomy. Results: The bilirubin declined in all except 2 patients after surgery. Both these patients developed ascites and severe jaundice and 1 expired one year after surgery. HIDA scan was excretory in all 14 patients. Whole blood manganese ranged from 1.26 - 27.58 µg/dl (mean 4.63 ± 7.00) at the time of surgery; the levels were normal in 11 patients and elevated in 3. The postoperative assay at 6 months after surgery demonstrated values from 1.08 - 74.46 µg/dl (mean 13.55 ± 24.17); 4 patients showed an increase from the previous value. Globus pallidus hyperintensity on T1-weighted images was noted in 1 patient on MRI; this patient had a sharp rise in the postoperative manganese level. All other MRI examinations were normal. All 14 patients were neurologically intact. Conclusions: Although the study size is small, it shows that there is a risk of latent manganese toxicity in patients with biliary atresia who have undergone Kasai's portoenterostomy. Whole blood manganese assay in conjunction with brain MRI can be used to serially monitor these patients for early detection of these complications. This tool can be used for prognosis after Kasai's portoenterostomy and can also be extremely useful in selecting patients who will need a liver transplant on a priority basis.
Key words
biliary atresia - Kasai's portoenterostomy - manganese toxicity - globus pallidus hyperintensity
References
-
1
Altman R P, Chandra R, Lilly J R.
Ongoing cirrhosis after successful portoenterostomy in infants with biliary atresia.
J Pediatr Surg.
1975;
10
685-691
-
2
Baruthio F, Guillard O, Arnaud J, Pierre F, Zawislak R.
Determination of manganese in biological materials by electrothermal atomic absorption spectrometry: a review.
Clin Chem.
1988;
34
227-234
-
3
Choi Y, Park J K, Park N H, Shin J W, Yoo C I, Lee C R, Lee H, Kim H K, Kim S R, Jung T H, Park J, Yoon C S, Kim Y.
Whole blood and red blood cell manganese reflected signal intensities of T1-weighted magnetic resonance images better than plasma manganese in liver cirrhotics.
J Occup Health.
2005;
47
68-73
-
4
Fell J M, Reynolds A P, Meadows N, Khan K, Long S G, Quaghbeur G, Taylor W J, Milla P J.
Manganese toxicity in children receiving long term parenteral nutrition.
Lancet.
1996;
347
1218-1221
-
5
Ihara H.
High signal insensity of globus pallidus on T1 weighted MRI in liver cirrhosis patients: clinical and pathological study.
Nippon Igaku Hoshasen Gakkai Zasshi.
1998;
58
479-488
-
6
Ikeda S, Yamaguchi Y, Sera Y, Ohshiro H, Uchino S, Yamashita Y, Ogawa M.
Manganese deposition in the globus pallidus in patients with biliary atresia.
Transplantation.
2000;
69
2339-2343
-
7
Klos K J, Ahlskog J E, Josephs K A, Fealey R D, Cowl C T, Kumar N.
Neurologic spectrum of chronic liver failure and basal ganglia T1 hyperintensity on magnetic resonance imaging: probable manganese neurotoxicity.
Arch Neurol.
2005;
62
1385-1390
-
8
Middlesworth W, Altman R P.
Biliary atresia.
Curr Opin Pediatr.
1997;
9
265-269
-
9
Mizoguchi N, Nishimura Y, Ono H, Sakura N.
Manganese elevations in blood of children with congenital portosystemic shunts.
Eur J Pediatr.
2001;
160
247-250
-
10
Neve J, Leclercq N.
Factors affecting determinations of manganese in serum by atomic absorption spectrometry.
Clin Chem.
1991;
37
723-728
-
11
Ohi R, Nio M, Chiba T, Endo N, Goto M, Ibrahim M.
Long term follow up after surgery for patients with biliary atresia.
J Pediatr Surg.
1990;
25
442-445
-
12
Park N H, Park J K, Choi Y, Yoo C I, Lee C R, Lee H, Kim H K, Kim S R, Jeong T H, Park J, Yoon C S, Kim Y.
Whole blood manganese correlates with high signal intensities on T1-weighted MRI in patients with liver cirrhosis.
Neurotoxicology.
2003;
24
909-915
-
13
Pleban P A, Pearson K H.
Determination of manganese in whole blood and serum.
Clin Chem.
1979;
25
1915-1918
-
14
Quaghebeur G, Taylor W J, Kingsley D P, Fell J M, Reynolds A P, Milla P J.
MRI in children receiving total parentral nutrition.
Neuroradiology.
1996;
38
680-683
-
15
Toyosaka A, Okamoto E, Okasora T, Nose K, Tomimoto Y.
Outcome of 21 patients with biliary atresia living more than 10 years.
J Pediatr Surg.
1993;
28
1498-1501
Professor V. Bhatnagar
Department of Pediatric Surgery
All India Institute of Medical Sciences
Ansari Nagar
New Delhi 110029
India
Email: veereshwarb@hotmail.com
