Assessment of latent manganese toxicity as a prognostic factor following surgery for biliary atresia
G S Agarwal1, R Sharma, V Bhatnagar
1Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.
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 microg/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 microg/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.
Insights
Biliary atresia patients undergoing Kasai
Area of Science:
- Pediatric Surgery
- Hepatology
- Neurotoxicology
Background:
- Biliary atresia is a severe neonatal liver disease requiring surgical intervention.
- Kasai's portoenterostomy is a common surgical procedure for biliary atresia.
- Potential for latent manganese toxicity exists post-Kasai's portoenterostomy.
Purpose of the Study:
- To investigate latent manganese toxicity in biliary atresia patients.
- To assess manganese levels as a prognostic tool after Kasai's portoenterostomy.
Main Methods:
- Whole blood manganese levels were measured using atomic adsorption spectroscopy.
- Manganese levels were assessed at surgery and 6 months post-operation.
- Correlation with brain MRI findings (globus pallidus T1-weighted hyperintensity) and clinical outcomes.
Main Results:
- Elevated postoperative manganese levels were observed in some patients.
- One patient with a sharp rise in manganese showed globus pallidus hyperintensity on MRI.
- Clinical outcomes varied, with two patients developing severe jaundice and ascites; one expired.
Conclusions:
- Latent manganese toxicity is a risk in biliary atresia patients post-Kasai's portoenterostomy.
- Whole blood manganese assay and brain MRI can monitor for toxicity.
- This monitoring can aid in prognosis and prioritize liver transplant selection.