Combination of gamma-glutamyl transferase and liver stiffness measurement for biliary atresia screening at different ages: a retrospective analysis of 282 infants.

BACKGROUND: This study aims to explore the diagnostic accuracy of the combination of gamma-glutamyl transferase (GGT) and liver stiffness measurement (LSM) for biliary atresia (BA) screening at different ages. METHODS: Our retrospective study involved 282 infants under the age of 120 days with jaundice who were admitted into Beijing Children's Hospital between January 2016 to December 2018. The GGT and LSM levels of infants were obtained. A parallel test was used, and ROC curve was created to obtain cutoff values of GGT and LSM for BA infants at different ages. RESULTS: Of the 282 infants, 135 were diagnosed with BA and 147 were non-BA infants. In all age groups (A: ≤60 days; B: 61-90 days; C: 91-120 days), the LSM and GGT levels of the BA group were significantly higher than that of the non-BA group, P < 0.05. The cutoff value of GGT and LSM to diagnosis BA was 191.2 U/L, 213.2 U/L, 281.5 U/L and 7.5 kPa, 10.0 kPa, 11.0 kPa in groups A, B and C, respectively. The parallel test was used to determine a sensitivity of 97.3, 98.1 and 100% in group A, B and C when either GGT or LSM levels were met in BA infants. The sensitivities of parallel testing for group A and B were higher than LSM or GGT used alone. CONCLUSIONS: Cutoff values of GGT and LSM to screen BA increased with age. Parallel testing of GGT and LSM in infants who are younger than 90 days old can decrease the rate of BA misdiagnosis.

Acquired right diaphragmatic hernia following pediatric living donor orthotopic liver transplantation.

ADH following OLT is a rare entity. Herein, we report a case of Alagille syndrome who developed ADH secondary to OLT, and possible etiological causes are discussed in light of the literature.

Diagnostic Laparoscopy in Infantile Cholestatic Jaundice

When jaundice persists for more than 14 days postnatally, the early diagnosis of surgical jaundice is important for the prognosis in extrahepatic biliary atresia after draining procedure. The role of diagnostic laparoscopy to differenctiate medical causes of jaundice from biliary atresia is evaluated in this report. Four patients with prolonged jaundice have been included in this study. When the gallbladder was not visualized we proceeded to laparotomy. In patients with enlarged gallbladder visualized at laparoscopy, laparoscopic guided cholangiogram was performed, and laparoscopic liver biopsy was done for those who had a patent biliary tree. Two patients had small atretic gallbladder and underwent a Kasai hepato-portoenterostomy. One patients showed a patent gallbladder and common bile duct with atresia of the common hepatic and intrahepatic ducts, and they underwent a Kasai hepatic-portoenterostomy. One patient showed an enlarged gallbladder and laparoscopic-guided cholangiogram were normal. Laparoscopic liver biopsy was performed. There were no complications. Laparoscopy wth laparoscopic-guided cholangiogram may be a valuable method in accurate and earlier diagnosis in an infant with prolonged jaundice.

The Usefulness of MRCP in the Evaluation of Pancreaticobiliary Diseases in Children

PURPOSE: Magnetic resonance cholangiopancreatography(MRCP) is a noninvasive method for imaging the pancreaticobiliary tree. The aim of this study was to evalute the usefulness of MRCP for the diagnosis of pancreaticobiliary diseases in children. METHODS: From October 1996 to May 2001, 67 patients with obstructive jaundice and three patients with chronic recurrent pancreatitis were evaluated with abdominal ultrasonography and MRCP. The final diagnosis was based on the operative and pathologic findings with biopsy specimen including clinical and laboratory findings. RESULTS: A total of 70 patients, consisting of 31 males and 39 females, with a mean age of 2.6+/-3.3 years were studied. The final diagnosis was biliary atresia in 25, neonatal cholestasis in 18, choledochal cyst without anomalous pancreatobiliary duct union(APBDU) in nine, choledochal cyst with APBDU in seven, cholestatic hepatitis in five, chronic recurrent pancreatitis in three, sclerosing cholangitis in two, and secondary biliary cirrhosis in one case. The overall diagnostic accuracy of abdominal ultrasonography was 75.7% and that of MRCP was 97.1%. The sensitivity and specificity of MRCP were 100% and 98% for biliary atresia, 87.5% and 100% for choledochal cyst with APBDU, 100% and 100% for choledochal cyst without APBDU, sclerosing cholangitis and chronic recurrent pancreatitis, respectively. CONCLUSION: MRCP is a fast, non-invasive and reliable method for diagnosing pancreaticobiliary diseases in children and will be the standard diagnostic procedure in the future.

The Usefulness of Magnetic Resonance Cholangiography in the Diagnosis of Biliary Atresia

PURPOSE: To evaluate the usefulness of magnetic resonance cholangiography(MRC) for the diagnosis of biliary atresia in infantile cholestatic jaundice. METHODS: Fifty consecutive infants with cholestatic jaundice underwent single-shot MRC for 3 years. The radiologic diagnosis of non-biliary atresia with MRC was based on visualization of the common bile duct and common hepatic duct. The diagnosis of biliary atresia was based on non-visualization of either the common bile duct or common hepatic duct. The final diagnosis of biliary atresia or non-biliary atresia was made with operations or clinical follow-up until jaundice resolved. RESULTS: MRC could clearly visualized the gallbladder, cystic duct, common hepatic duct, common bile duct, both intrahepatic ducts and second order intrahepatic ducts in small neonates and infants. MRC had accuracy of 98%, sensitivity of 100% and specificity of 96% for diagnosis of biliary atresia as the cause of infantile cholestatic jaundice. CONCLUSIONS: MRC is a very reliable noninvasive imaging study for diagnosis of biliary atresia in infants with cholestatic jaundice.