Kupffer Cell Hyaline Globules in Children With Autoimmune Hepatitis

• Published in: Journal of clinical and experimental hepatology Vol. 12; no. 1; pp. 20 - 28
• Main Authors: Khedr, Mohammed A., Adawy, Nermin M., Salim, Tahany A., Salem, Menan E., Ghazy, Ramy M., Elharoun, Ahmed S., Sultan, Mervat M., Ehsan, Nermine A.
• Format: Journal Article
• Language: English
• Published: India Elsevier B.V 01.01.2022; Elsevier
• Subjects: autoimmune hepatitis; hepatitis C virus infection; hyaline globules; Kupffer cells; liver histopathology; Original; Wilson’s disease; PAS; ALP; Kupffer cells; hyaline globules; ALT; liver histopathology; WD; PCLD; IAIHG; autoimmune hepatitis; AMA; KCs; AIH; AST; H&E; SMA; LKMA; hepatitis C virus infection; Wilson’s disease; WBCs; ANA; GGT; HCV; KCHGs; antiLC1; PAS-D; WD, Wilson’s disease; ANA, Antinuclear antibody; PCLD, Pediatric chronic liver disease; IAIHG, International Autoimmune Hepatitis Group; H&E, Hematoxylin and eosin; PAS, Periodic Acid Schiff; SMA, Smooth muscle antibody; antiLC1, Antiliver cytosol type 1 antibody; AIH, Autoimmune hepatitis; AMA, Antimitochondrial antibody; HCV, Hepatitis C virus; LKMA, Liver-kidney microsome 1 antibody; WBCs, White blood cell count; GGT, Gamma-glutamyl transpeptidase; ALP, Alkaline phosphatase; KCHGs, Kupffer cell hyaline globules; ALT, Alanine aminotransferase; PAS-D, Periodic acid–Schiff–Diastase; AST, Aspartate aminotransferase; KCs, Kupffer cells
• ISSN: 0973-6883; 2213-3453
• DOI: 10.1016/j.jceh.2021.04.013

Hyaline globules (HGs) in the cytoplasm of Kupffer cells (KCs) have been appraised for being a typical feature of autoimmune hepatitis (AIH). This study aimed to determine how useful Kupffer cell hyaline globules (KCHGs) are in diagnosing AIH vs. other causes of pediatric chronic liver diseases (PCLDs). This retrospective study recruited 124 children; 58 with AIH, 50 with chronic hepatitis C virus (HCV) infection, and 16 with Wilson’s disease (WD). Two pathologists retrieved paraffin blocks of liver biopsies and prepared new cut sections for Periodic acid–Schiff–Diastase (PAS-D) stain. They independently examined liver biopsies before starting treatment. Two pediatricians reviewed medical records for demographic, clinical, laboratory, and serological findings. Females represented 48.6% of the studied children with a median age of 5.8 (4.9) years. Pathologists identified KCHGs in 67.24%, 12.5%, and 6.0% of AIH, WD, and HCV affected children respectively, P < 0.001. A significantly higher proportion of seropositive than seronegative AIH patients had KCHGs (77.5% vs. 50.0%), (P < 0.05). In multivariate analysis, KCHGs and prolonged prothrombin time were the only significant predictors that differentiate between AIH and the other studied PCLDs. The odds ratio of having AIH increased 68 times if KCHGs were seen. Among children with AIH, the presence of KCHGs was associated with higher median levels of direct bilirubin 2.2 (1.3) vs. 1.2 (2.2), and immunoglobulin G 3.2 (1.9) vs. 2.0 (1.7), (P < 0.05), but not to histopathological findings or hepatic fibrosis and activity. KCHGs are key indicators that can differentiate between AIH and other PCLDs, and between seropositive and seronegative AIH.