Background: Extrahepatic biliary atresia (EHBA) causes a rare obstructive cholestasis in infants. Kasai portoenterostomy if done before the third month of life may relieve obstruction. Genetic predisposition has been implicated in EHBA etiopathogenesis with rs17095355 polymorphism having the strongest association. We determined the association between rs17095355 and EHBA susceptibility of Filipino children, and described the outcome in each genotype among timely operated patients. Methods: Thirty-four histologically diagnosed EHBA patients and thirty-three age- and sex-matched controls were recruited. Genomic DNA was extracted from peripheral blood and subjected to PCR and direct sequencing. Success of surgery among patients operated before 90 days of life was assessed by jaundice clearance six months post-surgery and native liver survival two and five years post-surgery. Results: There was no significant difference among individuals carrying T and C alleles in developing EHBA (OR:1.36; 95%CI:0.65–2.86). Jaundice persisted post-operatively in 75%, 33% and 27% of Kasai-operated homozygous T (T/T), homozygous C (C/C) and heterozygous (C/T) patients, respectively. Fifty percent of Kasai-operated C/C and C/T patients retained their native liver whereas all Kasai-operated T/T patients required liver transplantation two years post-surgery. Conclusion There is insufficient evidence to associate rs17095355 in EHBA development among Filipinos. Further investigation is warranted to elucidate genetic mechanisms in EHBA etiopathogenesis.
Biliary Atresia ; Polymorphism, Single Nucleotide ; Portoenterostomy, Hepatic

A 6(1/2)-year-old girl developed recurrent cholangitis following hepatic portoenterostomy for biliary atresia. Computed tomogram showed an ovoid cyst (4.5 x 4.0 cm in size) in the left hepatic lobe and another tubular dilatation (2.0 x 0.8 cm in size) in the right hepatic lobe. Percutaneous transhepatic cholangio-drainage (PTCD) with cystogram showed an ovoid cyst in the left hepatic lobe (Tsuchida type A), measuring 6.6 x 5.0 cmin size. She became afebrile and anicteric with aid of PTCD and parenteral antibiotics. However she continued to drain 45-150 cc of bile per day via the tube for over 2 weeks. Then she successfully underwent intrahepatic cystojejunostomy with guidance of intraoperative ultrasonography. This case illustrates relapsing cholangitis caused by Tsuchida type A intrahepatic cyst, which was successfully managed with PTCD followed by internal drainage procedure.
Anti-Bacterial Agents ; Bile ; Biliary Atresia* ; Cholangitis ; Dilatation ; Drainage ; Female ; Humans ; Portoenterostomy, Hepatic ; Ultrasonography

The diagnosis of biliary atresia (BA) is mainly based on clinical manifestations, screening, and related biochemistry tests. In recent years, the development of blood biomarkers and the improvement in ultrasound examination have made it possible for BA to be diagnosed at a younger age. In particular, matrix metalloproteinase-7 shows high sensitivity and specificity and has a higher diagnostic efficiency than existing biochemical parameters, thereby holding a promise for clinical application. Sound touch elastography can increase the diagnostic efficiency for BA in terms of diagnosis and prognostic evaluation. Surgery is still the only method for the treatment of BA at present, with the preferred surgical treatment regimen of Kasai portoenterostomy combined with pharmacotherapies for alleviating infection and inflammation, and the patients who fail Kasai portoenterostomy or have liver dysfunction may require liver transplantation to save their lives. Therefore, the current research on BA should focus on the biomarkers for early diagnosis, specifically targeted drugs, and drugs for preventing progressive liver fibrosis. This article reviews the current diagnosis and treatment methods for BA and discusses the potential research directions.
Humans ; Biliary Atresia/therapy* ; Portoenterostomy, Hepatic/methods* ; Liver Transplantation/methods* ; Prognosis ; Biomarkers

Bile Duct Diseases ; Biliary Atresia/surgery* ; Cysts ; Humans ; Infant ; Liver Failure ; Portoenterostomy, Hepatic ; Retrospective Studies

PURPOSE: The surgical results for congenital extrahepatic biliary atresia have been improved since Kasai first described hepatic portoenterostomy(Kasai operation) in 1959. This study was conducted to evaluate the postoperative prognosis of congenital extrahepatic biliary atresia after hepatic portoenterostomy. METHODS: This clinical study was done by reviewing the medical records of 30 patients who have undergone hepatic portoenterostomy at the Severance Hospital, Yonsei University College of Medicine between January 1984 to June 1994 due to congenital extrahepatic biliary atresia. RESULTS: 1) The ages of the 30 patients ranged from 45 to 242 days and the mean age at operation was 90.8 days. Seventeen patients were male and 13 were female, and the male to female ratio was 1.3:1. 2) Of the total 30 patients, 9 cases(30%) had died, 12 cases(40%) were still being followed up, and 9 cases(30%) never returned for during follow-up. The calculated survival rate of more than 3 years was 53%. 3) Jaundice was improved in 8 patients(27%) after portoenterostomy, but the remaining 22 patients(73%) showed no improvement. The two year survival rate of the jaundice-free group was 100%, compared with 42% in the jaundice-persistent group, which showed a statistically significant difference (p<0.05). 4) Hepatic portoenterostomy was performed in 7 patients(23%) before the age of 60 days, and done in the remaining 23 patients(77%) after 60 days. The two year survival rate of the former group was 54%, compared with 70% in the latter group, which did not show a statistically significant difference. 5) Portal hypertension was complicated in 17 patients(57%), but not in the remaining 13 patients(43%). The two year survival rate of the former group was 54%, compared with 100% in the latter group, which showed a statistically significant difference between the two groups (p<0.05). 6) Cholangitis was complicated in 15 patients(50%), but not in the remaining 15 patients(50%). The two year survival rate of the former group was 53%, compared with 81% in the latter group, which did not show a statistically significant difference. CONCLUSIONS: The 3 year survival rate in patients who performed hepatic portoenterostomy due to congenital extrahepatic biliary atresia was 53%. The persistent jaundice and the development of portal hypertension after surgery were important determinants of long term survival of exteahepatic biliary atresia in this study.
Biliary Atresia* ; Cholangitis ; Female ; Follow-Up Studies ; Humans ; Hypertension, Portal ; Jaundice ; Male ; Medical Records ; Portoenterostomy, Hepatic* ; Prognosis* ; Survival Rate

PURPOSE: Whereas the Kasai portoenterostomy (KPE) is an accepted first line of surgery for bile drainage in infants with biliary atresia, its long-term effectiveness is not clear because its etiology and pathogenesis remains unknown. This study was aimed to investigate the late complications occurring in long-term survivors and the current status of living patients who survived over 10 years after KPE. METHODS: A retrospective analysis of the medical records of 32 patients who underwent KPE from 1990 to 2000 was done. We analyzed 10-year survival rates with the Kaplan-Meier method and the current status of the long-term survivors. RESULTS: The overall 10-year survival rate by Kaplan-Meier method after KPE was 76.2%. Eight (25%) patients had died, including 4 who were transplanted. Nineteen (59.4%) patients survived over 10 years. Among them, 6 (31.6%) patients had portal hypertension, and 5 (26.3%) had episodes of cholangitis. Two had intrahepatic cyst and 2 had intestinal obstruction. Six (31.6%) patients have been well without any complications. CONCLUSION: The long-term survival rate of biliary atresia is slightly improving. However, two thirds of patients suffer from various complications. One-third of survivors go on without any complication. As biliary atresia is known as a progressive inflammatory disease, careful life-long follow- up is needed in long-term survivals after KPE.
Bile ; Biliary Atresia ; Cholangitis ; Drainage ; Humans ; Hypertension, Portal ; Infant ; Intestinal Obstruction ; Medical Records ; Portoenterostomy, Hepatic ; Retrospective Studies ; Survival Rate ; Survivors ; Transplants

BACKGROUND: This report describes the clinicopathologic findings of six hepatic masses that developed after Kasai hepatic portoenterostomy (HPE) in six patients with longstanding biliary atresia (BA). METHODS: Hepatic masses were found in six of 55 pediatric patients who underwent liver transplantation for BA after Kasai HPE from 1997 to 2009. Clinicopathologic analysis was performed and immunohistochemical staining was carried out for CD34, smooth muscle actin (SMA) and cytokeratin 7. RESULTS: Of the six hepatic masses, two were diagnosed as focal nodular hyperplasia (FNH)-like lesions, two were large regenerative nodules (LRN), one was a mesenchymal hamartoma (MH) and one was a cholangiocarcinoma. The immunohistochemical staining findings for SMA and CD34 were more prominent for the FNH-like nodules than for the cirrhotic background liver. Dysplastic biliary epithelium arising from intestinal metaplasia was found in the cholangiocarcinoma. CONCLUSIONS: Our findings suggest that FNH-like lesions, LRNs and MH are the results of vascular hemodynamic changes after Kasai HPE and that cholangiocarcinoma is due to recurrent cholangitis after BA. All the lesions in this series must be included in the differential diagnosis of a newly formed hepatic mass in patients after portoenterostomy.
Actins ; Biliary Atresia ; Child ; Cholangiocarcinoma ; Cholangitis ; Diagnosis, Differential ; Epithelium ; Focal Nodular Hyperplasia ; Hamartoma ; Hemodynamics ; Humans ; Keratins ; Liver ; Liver Transplantation ; Metaplasia ; Muscle, Smooth ; Portoenterostomy, Hepatic

BACKGROUND: Biliary atresia is an extrahepatic progressive obliterate cholangiopathy that occurs in infants. Kasai procedure, a surgical method that can help re-establish bile flow from the liver into the intestine, is its first line treatment. Since infants with biliary atresia already have advanced hepatic dysfunction, all kinds of schemes should be considered to minimize further liver damage during surgery. The objective of this study was to compare the postoperative hepatic functions between the two commonly used inhalational anesthetics in infants undergoing the Kasai procedure (i.e., desflurane and sevoflurane). METHODS: This prospective, randomized, double-blind, single-center, and parallel group study included 40 children undergoing Kasai procedure. They were randomly allocated to Group S (sevoflurane) or Group D (desflurane). All the patients were anesthetized with designated anesthetic agent with the end-tidal concentration of about 0.8–1 minimum alveolar concentration. Postoperative hepatic functions were assessed by aspartate aminotransferase (AST), alanine aminotransferase (ALT), albumin, prothrombin time, and total bilirubin. RESULTS: A total of 38 patients were selected for the study. In both groups, AST, ALT were increased in magnitude to the peak on postoperative day 0 and decreased to preoperative value at postoperative day 3. There were no significant differences between the groups in any laboratory results related to liver function. CONCLUSIONS: Sevoflurane and desflurane, inhalation anesthetics for maintaining anesthesia used in infants undergoing the Kasai procedure, did not show any difference in preserving postoperative hepatic function.
Alanine Transaminase ; Anesthesia ; Anesthetics ; Anesthetics, Inhalation ; Aspartate Aminotransferases ; Bile ; Biliary Atresia ; Bilirubin ; Child ; Humans ; Infant ; Intestines ; Liver ; Liver Function Tests ; Methods ; Portoenterostomy, Hepatic ; Prospective Studies ; Prothrombin Time

PURPOSE: The purpose of this study was to define the role of cyclooxygenase-2 inhibitors (COX-2i) in reducing hepatic fibrosis in pediatric patients with chronic liver disease. MATERIALS AND METHODS: From September 2009 to September 2010, patients over 2 years old who visited our outpatient clinic for follow-up to manage their chronic liver disease after Kasai portoenterostomy for biliary atresia, were included in this study. Volunteers were assigned to the study or control groups, according to their preference. A COX-2i was given to only the study group after obtaining consent. The degree of hepatic fibrosis (liver stiffness score, LSS) was prospectively measured using FibroScan, and liver function was examined using serum analysis before and after treatment. After 1 year, changes in LSSs and liver function were compared between the two groups. RESULTS: Twenty-five patients (18 females and 7 males) were enrolled in the study group. The control group included 44 patients (26 females and 18 males). After 1 year, the least square mean values for the LSSs were significantly decreased by 3.91±0.98 kPa (p=0.004) only in the study group. Serum total bilirubin did not decrease significantly in either group. CONCLUSION: COX-2i treatment improved the LSS in patients with chronic liver disease after Kasai portoenterostomy for biliary atresia.
Biliary Atresia/complications/enzymology/*surgery ; Child ; Child, Preschool ; Chronic Disease ; Cyclooxygenase 2 Inhibitors/*therapeutic use ; Female ; Humans ; Liver Cirrhosis/etiology/pathology/*prevention & control ; Male ; *Portoenterostomy, Hepatic ; Thiazines/*therapeutic use ; Thiazoles/*therapeutic use