AIM: To estimate the incidence of biliary atresia(BA) amongst neonatal cholestatic syndromes (NCS) and determine prognostic factors in BA patients who have undergone Kasai's portoenterostomy. Study design- Retrospective analysis. SETTING: Pediatric Hepatobiliary Clinic at B.J. Wadia Children's Hospital, Mumbai. METHODS AND MATERIALS: 32 patients diagnosed with BA referred to the clinic from May 2005 to July 2007 were included in the study. All patients underwent a detailed history, clinical examination and were tested for liver function tests (LFT), USG abdomen, liver biopsy, intra-operative cholangiogram and CMV tests. Patients were followed up for a period of 1 month to 7 years post operatively and complications such as cholangitis, progress to liver cell failure and cirrhosis was noted. RESULTS: Incidence of BA amongst NCS (n = 88) was 36.4%. 8 patients of BA (25%) were lost to follow up. Out of the remaining, 10 (41.7%) improved and 14 (58.3%) did not improve. The mean age of presentation was 89 + 55.8 days. 1 patient (25%) out of 4 with bile duct size of < 100 microns showed an improvement whereas 3 (37.5%) out 8 patients with bile duct size 100-200 microns showed improvement and 4 (50%) with bile duct size of > 200 microns had improvement post Kasai surgery. Those with bile duct sizes > 200 microns had better prognosis than those with sizes 100-200 microns (Odd's ratio = 1.8) and < 100 microns (Odd's ratio = 3). 12 patients (50%) were operated before 3 months of age and 50% of them responded to surgery. The remaining 12 patients were operated after 3 months of age and only 33% showed any improvement. (Odd's ratio = 2). Other parameters like SGOT (P = 0.598), SGPT (p = 0.901), total Bilirubin (p = 0.349), Direct Bilirubin (p = 0.429), Alkaline Phosphatase (p = 0.605) and GGTP (p = 0.480), cirrhosis (p = 0.417), degree of fibrosis (p = 0.384), degree of inflammation (p = 0.964) and Cholangitis (P = 0.388) had no effect on the outcome. CONCLUSION: Biliary Atresia is a common cause of NCS in India. Children with Bile duct size > 200 microns have a good prognosis. Portoenterostomy before 3 months of age has a better outcome.
AIM: To estimate the incidence of biliary atresia(BA) amongst neonatal cholestatic syndromes (NCS) and determine prognostic factors in BApatients who have undergone Kasai's portoenterostomy. Study design- Retrospective analysis. SETTING: Pediatric Hepatobiliary Clinic at B.J. Wadia Children's Hospital, Mumbai. METHODS AND MATERIALS: 32 patients diagnosed with BA referred to the clinic from May 2005 to July 2007 were included in the study. All patients underwent a detailed history, clinical examination and were tested for liver function tests (LFT), USG abdomen, liver biopsy, intra-operative cholangiogram and CMV tests. Patients were followed up for a period of 1 month to 7 years post operatively and complications such as cholangitis, progress to liver cell failure and cirrhosis was noted. RESULTS: Incidence of BA amongst NCS (n = 88) was 36.4%. 8 patients of BA (25%) were lost to follow up. Out of the remaining, 10 (41.7%) improved and 14 (58.3%) did not improve. The mean age of presentation was 89 + 55.8 days. 1 patient (25%) out of 4 with bile duct size of < 100 microns showed an improvement whereas 3 (37.5%) out 8 patients with bile duct size 100-200 microns showed improvement and 4 (50%) with bile duct size of > 200 microns had improvement post Kasai surgery. Those with bile duct sizes > 200 microns had better prognosis than those with sizes 100-200 microns (Odd's ratio = 1.8) and < 100 microns (Odd's ratio = 3). 12 patients (50%) were operated before 3 months of age and 50% of them responded to surgery. The remaining 12 patients were operated after 3 months of age and only 33% showed any improvement. (Odd's ratio = 2). Other parameters like SGOT (P = 0.598), SGPT (p = 0.901), total Bilirubin (p = 0.349), Direct Bilirubin (p = 0.429), Alkaline Phosphatase (p = 0.605) and GGTP (p = 0.480), cirrhosis (p = 0.417), degree of fibrosis (p = 0.384), degree of inflammation (p = 0.964) and Cholangitis (P = 0.388) had no effect on the outcome. CONCLUSION:Biliary Atresia is a common cause of NCS in India. Children with Bile duct size > 200 microns have a good prognosis. Portoenterostomy before 3 months of age has a better outcome.
Authors: Ana M Calinescu; Omid Madadi-Sanjani; Cara Mack; Richard A Schreiber; Riccardo Superina; Deirdre Kelly; Claus Petersen; Barbara E Wildhaber Journal: J Clin Med Date: 2022-01-19 Impact factor: 4.241