Abstract | AIM: METHODS: Between 1994 and 2006, 71 infants with BA were referred. Four died from uncorrectable congenital heart disease/cardiorespiratory failure without undergoing portoenterostomy, 7 underwent primary liver transplantation (3 referred > or = 19 weeks of age), and 60 underwent portoenterostomy at a median of 51 (10-104) days. Of these, 55 (92%) had type 3 BA and 6 had the BA splenic malformation syndrome. Fifty (83%) received the following adjuvant therapy beginning on postoperative day 5: oral dexamethasone 0.3 mg/kg bd for 5 days, 0.2 mg/kg bd for 5 days, and 0.1 mg/kg bd for 5 days together with oral ursodeoxycholic acid 5 mg/kg bd and phenobarbitone 5 mg/kg nocte, both of which were continued for 1 year. All infants received routine perioperative prophylactic antibiotics. RESULTS: CONCLUSION: In this series, adjuvant postoperative treatment with a short course of oral dexamethasone and longer-term ursodeoxycholic acid significantly improved the outcome after Kasai portoenterostomy.
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Authors | Mark D Stringer, Suzanne M Davison, Sanjay R Rajwal, Patricia McClean |
Journal | Journal of pediatric surgery
(J Pediatr Surg)
Vol. 42
Issue 8
Pg. 1324-8
(Aug 2007)
ISSN: 1531-5037 [Electronic] United States |
PMID | 17706489
(Publication Type: Journal Article, Multicenter Study, Randomized Controlled Trial)
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Chemical References |
- Cholagogues and Choleretics
- Glucocorticoids
- Ursodeoxycholic Acid
- Dexamethasone
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Topics |
- Biliary Atresia
(drug therapy, surgery)
- Chemotherapy, Adjuvant
- Cholagogues and Choleretics
(therapeutic use)
- Dexamethasone
(therapeutic use)
- Double-Blind Method
- Glucocorticoids
(therapeutic use)
- Humans
- Infant
- Portoenterostomy, Hepatic
- Survival Analysis
- Ursodeoxycholic Acid
(therapeutic use)
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