Spectrum of Disease in Liver Biopsies from Patients with Neonatal Jaundice
M Westerhoff, RK Pai, CH Mziray-Andrew, L Hovan, A El-Gendi, R Azzam, J Hart. University of Chicago, Chicago; Washington University, St. Louis
Background: Liver biopsy is an essential component of the diagnostic work-up of jaundiced infants. Unfortunately, there is considerable histological overlap among the various diseases that can cause jaundice in this population.
Design: The pathology database was searched for liver biopsies performed on infants under 4 months of age. Biopsies were assessed for degree of inflammation, bile duct paucity, cholestasis, steatosis, fibrosis, and giant cell change (GCC). After histological assessment, clinical records were correlated with biopsy findings.
Results: 55 cases (61% male, average age 2.5 months) had follow-up ranging 2 - 36 months. Neonatal iron (Fe) storage disease (n=8) could be identified by the presence of marked GCC, extensive pericellular fibrosis, and Fe deposition in multinucleated hepatocytes. Alagille patients (5) exhibited duct loss but little GCC, cholestasis, or inflammation. Biliary atresia (BA) was correctly identified in all 7 cases by the presence of portal fibrosis, bile ductular proliferation with bile plugs, and cholestasis. Total parenteral nutrition cases (4) also showed these features, but also exhibited macrovesicular steatosis. Biopsies from idiopathic neonatal hepatitis (NH) (17), α-1-antitrypsin deficiency (4), progressive familial intrahepatic cholestasis (2), bile acid metabolism defect (1) and pan-hypopituitarism (PHP) (6) were histologically similar, but all PHP cases exhibited bile duct paucity. The biopsy of 1 patient with established coxsackie infection exhibited prominent portal and lobular inflammation; these findings were also seen in 3 NH cases. Chart review indicated that these 3 also had sepsis, but an infectious agent was not identified in 2. All patients with idiopathic NH recovered spontaneously. 2 with neonatal Fe storage disease expired and 2 underwent transplantation, as did 1 with bile acid metabolic defect. BA patients underwent Kasai procedure.
Conclusions: Multiple disorders cause neonatal jaundice, many of which have distinctive histological features. PHP produces typical non-specific features of NH, but in addition usually exhibits bile duct paucity, which can serve as a histological marker of this disorder. Although PHP is usually thought of as a rare disorder, it accounted for 11% of the biopsies performed to evaluate neonatal cholestasis in this series. The presence of significant portal and lobular inflammation should suggest an infectious etiology, as this feature is not typical of other disorders causing neonatal jaundice.
Category: Liver & Pancreas
Monday, March 22, 2010 1:00 PM
Poster Session II # 172, Monday Afternoon