Humans ; Infant ; Biliary Atresia/diagnosis* ; Choledochal Cyst/diagnosis* ; RNA, Circular ; Diagnosis, Differential

Humans ; Biliary Atresia

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

Objective: To compare the clinical efficacy of split liver transplantation (SLT) and living donor liver transplantation(LDLT) in the treatment of children with biliary atresia. Methods: The clinical data of 64 children with biliary atresia who underwent SLT and 44 children who underwent LDLT from June 2017 to May 2022 at Liver Surgery & Liver Transplantation Center,the Third Affiliated Hospital of Sun Yat-sen University were retrospectively analyzed. Among the children who received SLT, there were 40 males and 24 females. The median age at transplantation was 8 months (range:4 to 168 months). Among the patients who received LDLT, there were 24 males and 20 females. The age at transplantation ranged from 4 to 24 months,with a median age of 7 months. Sixty-four children with biliary atresia were divided into two groups according to the SLT operation time: 32 cases in the early SLT group(June 2017 to January 2019) and 32 cases in the technically mature SLT group (February 2019 to May 2022). Rank sum test or t test was used to compare the recovery of liver function between the LDLT group and the SLT group,and between the early SLT group and the technically mature SLT group. The incidence of postoperative complications was compared by χ² test or Fisher exact probability method. Kaplan-Meier method and Log-rank test were used for survival analysis. Results: The cold ischemia time(M (IQR)) (218 (65) minutes), intraoperative blood loss(175 (100) ml) and graft-to-recipient body weight ratio (3.0±0.7) in the LDLT group were lower than those in the SLT group(500 (130) minutes, 200 (250) ml, 3.4±0.8) (Z=-8.064,Z=-2.969, t=-2.048, all P<0.05). The cold ischemia time(457(158)minutes) and total hospital stay ((37.4±22.4)days) in the technically mature SLT group were lower than those in the early SLT group(510(60)minutes, (53.0±39.0)days).The differences were statistically significant (Z=-2.132, t=1.934, both P<0.05).The liver function indexes of LDLT group and SLT group showed unimodal changes within 1 week after operation. The peak values of ALT, AST, prothrombin time, activeated partial thromboplasting time, international normalized ratio, fibrinogen and creatinine all appeared at 1 day after operation, and the peak value of prothrombin activity appeared at 3 days after operation. All indicators returned to normal at 7 days after operation. The 1-,2-,and 3-year overall survival rates were 95.5% in LDLT group and 93.5% in the technically mature SLT group, and the difference was not statistically significant. The 1-,2-,and 3-year overall survival rates were 90.2% in the early SLT group and 93.5% in the technically mature SLT group, and there was no significant difference between the two groups(P>0.05). The main complications of the early SLT group were surgery-related complications(28.1%,9/32), and the main complications of the technically mature SLT group were non-surgery-related complications(21.9%,7/32). There were 5 deaths in the SLT group,including 4 in the early SLT group and 1 in the technically mature SLT group. Conclusion: The survival rate of SLT in the treatment of biliary atresia is comparable to that of LDLT.
Adolescent ; Biliary Atresia/surgery* ; Child ; Child, Preschool ; Creatinine ; Female ; Fibrinogen ; Humans ; Infant ; Liver Transplantation/methods* ; Living Donors ; Male ; Postoperative Complications/epidemiology* ; Prothrombin ; Retrospective Studies ; Treatment Outcome

Objective. To determine factors predictive of obstructive neonatal cholestasis among Filipino infants and to describe their outcome.

Methods. Jaundiced infants within the first eight weeks of life with liver biopsy were included. Excluded were cholestasis secondary to metabolic or infective causes. Retrospective chart review (2009-2012) and prospective recruitment of patients (2013) were done. A final diagnosis of non-obstructive or obstructive neonatal cholestasis was made on clinical, biochemical, ultrasonographic, and histologic findings, using histology and/or operative cholangiogram as the gold standard. The outcome was assessed on the 6th and 12th months from diagnosis. The crude odds ratio for obstructive jaundice was computed. Multiple logistic regression on significant variables (p-value <0.05) was done.

Results. Two hundred sixty-three (263) patients were included: 161 with non-obstructive and 102 with obstructive cause. Mean age at first consult was higher in those with obstruction. On logistic regression, females (OR:2.3), absence of a family history of idiopathic neonatal hepatitis (OR:4), and persistently pale/acholic stools (OR:13) were predictive of obstruction. 85% of patients with a non-obstructive cause are alive and well, while 80% of patients with obstruction have died.

Conclusion. Among jaundiced infants females, the absence of a family history of idiopathic neonatal hepatitis and persistently pale yellow/acholic stools were predictive of obstruction. The outcome was poor in patients with obstructive jaundice.

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

PURPOSE: To assess the feasibility of the use of spin-echo echo-planar imaging (SE-EPI) magnetic resonance elastography (MRE) in livers of children and young adults. MATERIALS AND METHODS: Patients (≤ 20 years old) who underwent 3T SE-EPI MRE were included retrospectively. Subjects were divided into three groups according to the purpose of the liver MRI: suspicion of fatty liver or focal fat deposition in the liver (FAT group), liver fibrosis after receiving a Kasai operation from biliary atresia (BA group), and hepatic iron deposition after receiving chemotherapy or transfusions (IRON group). Technical failure of MRE was defined when a stiffness map showed no pixel value with a confidence index higher than 95%, and the patients were divided as success and failure groups accordingly. Clinical findings including age, gender, weight, height, and body mass index and magnetic resonance imaging results including proton density fat fraction (PDFF), T2*, and MRE values were assessed. Factors affecting failure of MRE were evaluated and the image quality in wave propagation image and stiffness map was evaluated using the appropriate scores. RESULTS: Among total 240 patients (median 15 years, 211 patients in the FAT, 21 patients in the BA, and 8 patients in the IRON groups), technical failure was noted in six patients in the IRON group (6/8 patients, 75%), while there were no failures noted in the FAT and BA groups. These six patients had T2* values ranging from 0.9 to 3.8 ms. The image quality scores were not significantly different between the FAT and BA groups (P > 0.999), while the scores were significantly lower in the IRON group (P < 0.001). CONCLUSION: The 3T SE-EPI MRE in children and young adults had a high technical success rate. The technical failure was occurred in children with decreased T2* value (≤ 3.8 ms) from iron deposition.
Biliary Atresia ; Body Mass Index ; Child ; Drug Therapy ; Echo-Planar Imaging ; Elasticity Imaging Techniques ; Fatty Liver ; Humans ; Iron ; Liver Cirrhosis ; Liver ; Magnetic Resonance Imaging ; Protons ; Retrospective Studies ; Young Adult

We present the case of a patient with biliary and duodenal atresia who showed false-negative hepatobiliary scintigraphy results. The patient was born at 37 weeks and 2 days of gestation. Her mother had undergone amnioreduction after detection of a double-bubble ultrasound sign in the fetal abdomen. At 2 days of age, total serum bilirubin level was elevated. On hepatobiliary scintigraphy 4 days later, the gallbladder was visualized from 30 min and it showed duodeno-gastric reflux at 240 min. After 24 h, the radiotracer was almost washed out in the hepatic parenchyma, but there was retention in the gastroduodenal junction. Because the biliary to duodenal transit was visible, biliary atresia seemed unlikely. Abdominal ultrasonography at 7 days of age showed a small dysmorphic gallbladder, but triangular cord sign was not definite. Magnetic resonance cholangiography revealed atretic gallbladder. Although cystic and common bile ducts were visible, the proximal common hepatic bile duct was not visible. The next day, serum total bilirubin levels remained elevated (17.1 mg/dl) with direct bilirubin level of 1.2 mg/dl. Kasai portoenterostomy with duodeno-duodenostomy was performed at 10 days of age. Histopathological evaluation showed a fibrous obliteration of the common bile duct, consistent with that of biliary atresia.
Abdomen ; Bile Ducts ; Biliary Atresia ; Bilirubin ; Cholangiography ; Common Bile Duct ; Duodenogastric Reflux ; Gallbladder ; Humans ; Infant, Newborn ; Jaundice, Neonatal ; Mothers ; Pregnancy ; Radionuclide Imaging ; Ultrasonography

OBJECTIVE: To study the value of serum gamma-glutamyl transpeptidase (GGT) combined with direct bilirubin (DB) in the diagnosis of biliary atresia. METHODS: A total of 667 infants with cholestasis who were hospitalized and treated from July 2010 to December 2018 were enrolled as subjects. According to the results of intraoperative cholangiography and follow-up, they were divided into biliary atresia group with 234 infants and cholestasis group with 433 infants. The two groups were compared in terms of age of onset, sex, and serum levels of total bilirubin (TB), DB, alanine aminotransferase (ALT), aspartate aminotransferase (AST), total bile acid (TBA), and GGT. A receiver operating characteristic (ROC) curve analysis was performed for indices with statistical significance, and the area under the ROC curve (AUC) and the optimal cut-off value for diagnosis were calculated. RESULTS: The biliary atresia group had a significantly younger age of onset than the cholestasis group (P<0.001). There were no significant differences in sex, ALT, and AST between the two groups (P>0.05), while the biliary atresia group had significantly higher serum levels of TB, DB, TBA, and GGT than the cholestasis group (P<0.05). GGT combined with DB had the highest AUC of 0.892 (95% confidence interval: 0.868-0.916) in the diagnosis of biliary atresia. At the optimal cut-off values of 324.0 U/L for GGT and 115.1 μmmol/L for DB, GGT combined with DB had a sensitivity of 79.8% and a specificity of 83.2% in the diagnosis of biliary atresia. CONCLUSIONS GGT combined with DB has high sensitivity and specificity in the diagnosis of biliary atresia and can be used as an effective indicator for diagnosis of biliary atresia in infants.
Alanine Transaminase ; Aspartate Aminotransferases ; Biliary Atresia ; diagnosis ; Bilirubin ; Humans ; Infant ; gamma-Glutamyltransferase ; blood

Pediatric hepatobiliary imaging is important for evaluation of not only congenital or structural disease but also metabolic or diffuse parenchymal disease and tumors. A variety of ultrasonography and magnetic resonance imaging (MRI) techniques can be used for these assessments. In ultrasonography, conventional ultrasound imaging as well as vascular imaging, elastography, and contrast-enhanced ultrasonography can be used, while in MRI, fat quantification, T2/T2* mapping, diffusion-weighted imaging, magnetic resonance elastography, and dynamic contrast-enhanced MRI can be performed. These techniques may be helpful for evaluation of biliary atresia, hepatic fibrosis, nonalcoholic fatty liver disease, sinusoidal obstruction syndrome, and hepatic masses in children. In this review, we discuss each tool in the context of management of hepatobiliary disease in children, and cover various imaging techniques in the context of the relevant physics and their clinical applications for patient care.
Biliary Atresia ; Child ; Elasticity Imaging Techniques ; Fibrosis ; Hepatic Veno-Occlusive Disease ; Humans ; Liver ; Magnetic Resonance Imaging ; Non-alcoholic Fatty Liver Disease ; Patient Care ; Ultrasonography