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Introduction: The objective was to compare safety of pediatric percutaneous liver biopsy (PLB) performed by fellows or staff physicians., Methods: Outcomes of 212 PLB completed by first-year pediatric gastroenterology fellows or by staff physicians over 8 years were analyzed and compared., Results: Approximately 81.5% of the biopsies were completed by trainees. No significant differences were found between groups (fellows vs staff) regarding number of punctures (median of 1.7 for both), nonrepresentative biopsies (4.2% vs 2.6%), and hemoglobin drop (median of 0.7 vs 0.5 g/L)., Discussion: Complications of pediatric PLB are uncommon and did not differ among physicians with different training levels.

Objective: To describe a case of cholesteryl ester storage disease (CESD) and discuss the importance of liver biopsy for diagnosis., Case Description: A female patient, aged two years and ten months, presented with an increased abdominal volume following hepatomegaly for four months. Abdominal ultrasound demonstrated hepatomegaly and hepatic steatosis. Laboratory tests showed elevated liver serum enzymes and dyslipidemia. Liver biopsy was consistent with CESD., Comments: Although measuring enzyme activity is the gold standard for CESD diagnosis, liver biopsy is very helpful when investigating suspected cases of CESD, particularly upon other differential diagnoses to be considered.

Biliary atresia (BA) is classically described at the neonatal age. However, rare cases of BA in older infants have also been reported. We report four cases of late-onset BA in infants older than 4 weeks (3 males, 1 female), and describe the diagnostic and management difficulties. One of the cases had a late-onset (29 weeks) presentation with a successful surgical procedure. We highlight the importance of this unusual differential diagnosis in infants with cholestatic syndrome, who may benefit from Kasai surgery, regardless of age.

Aim: Paracoccidioidomycosis is a systemic mycosis that is endemic to certain countries in Latin America. This study aimed to describe the histological features of liver involvement in patients with paracoccidioidomycosis aged <16 years of age who were treated between 1980 and 2010, with a diagnosis that was confirmed by detection of the fungus by pathological examination., Methods and Results: Liver tissue was obtained from one necropsy and 12 biopsies. Throughout 2007, biopsies were taken from patients with persistent jaundice or portal hypertension, after which biopsies became indicated due to elevated aminotransferase and low albumin levels. Using haematoxylin and eosin (H&E), Masson's trichrome and immunohistochemical (CK7 and CK19) staining, we noted degenerative alterations in bile duct cells and inflammatory injury to the bile ducts in 10 biopsies. Using immunohistochemistry for CK7 and CK19, we observed ductal proliferation in all 12 samples., Conclusions: Bile duct injuries by inflammatory cells might explain the predominant increase in canalicular enzymes; immunohistochemistry is more sensitive in demonstrating ductular reactions and might show changes that are not apparent on H&E staining., (© 2013 John Wiley & Sons Ltd.)

Background: Biliary atresia (BA) is the most severe hepatic disorder in newborns and its etiopathogenesis remains unknown. Viral involvement has been proposed, including the human cytomegalovirus (HCMV). The aims of the study were to use the polymerase chain reaction (PCR) to screen the liver tissue of infants with extrahepatic cholestasis for HCMV and to correlate the results with serological antibodies against HCMV and histological findings., Methods: A retrospective study in a tertiary care setting included 35 patients (31 BA, 1 BA associated with a choledochal cyst, 2 congenital stenosis of the distal common bile duct and 1 hepatic cyst). HCMV serology was determined by ELISA. Liver and porta hepatis were examined histologically. Liver samples from infants and a control group were screened for HCMV DNA., Results: Twelve patients had HCMV negative serology, 9 were positive for IgG antibodies and 14 were positive for IgG and IgM. Nine liver and seven porta hepatis samples were positive for HCMV DNA but none of the control group were positive (general frequency of positivity was 34.3%-12/35). There was no correlation between HCMV positivity by PCR and the histological findings. The accuracy of serology for detecting HCMV antibodies was low., Conclusion: These results indicate an elevated frequency of HCMV in pediatric patients with extrahepatic neonatal cholestasis. They also show the low accuracy of serological tests for detecting active HCMV infection and the lack of correlation between HCMV positivity by PCR and the histopathological changes.

Neonatal cholestasis still presents a diagnostic challenge, both from the anatomic and etiologic point of view. Distinguishing intrahepatic from extrahepatic causes of cholestasis is of paramount importance since the latter may be treated by surgery but prognosis depends on the age at which operation is performed. Many tests have been proposed to help in differentiating these two entities, among which liver biopsy is the most frequently employed, and ultrasonography one of the most recently included. Our purpose is to present our experience with these two methods in the differential diagnosis of intra and extrahepatic causes of cholestasis. From January 1989 to July 1993, 35 patients with neonatal cholestasis were evaluated through a protocol which included liver biopsy and ultrasonography. The latter was performed after a 4 hour fast and was considered indicative of extrahepatic cholestasis when the gallbladder was not visualized, was hypoplastic or non-functioning, or if a cystic structure was seen at the extrahepatic biliary tree. Sensitivity, specificity, and accuracy were determined for these two tests. Seventeen patients were found to have extrahepatic cholestasis (all with biliary atresia) and 18 intrahepatic cholestasis, on the basis of clinical evolution or operative findings. Sensitivity was 100% for ultrasonography and 76% for liver biopsy in diagnosing extrahepatic cholestasis. Accuracy was 83% for ultrasonography and 86% for biopsy, rising to 96% when both tests were considered together. Based on these findings we strongly recommend ultrasonography with definite criteria as the initial investigation tool in the management of neonatal cholestasis, associated with liver biopsy.