Categories: Liver/biliary system phene

Links to possible relevant human trait(s) and/or gene(s) in OMIM: 600643 (trait)

Mendelian trait/disorder: unknown

Disease-related: yes

Species-specific description: Caliendo et al. (2012) report biliary tract abnormalities resembling biliary cystoadenoma in a 3-month-old white African lion. “Peribiliary cysts and biliary cystoadenomas have been reported occasionally in lions. Typically, it has been suggested that these lesions are congenital in origin, but presentation has normally been in aged animals (13-22years)…” (Compiled by Natalia Denisse Ramos Reyes 7/11/2022)

Clinical features: Caliendo et al. (2012): "A captive 3-mo-old white African lion (Panthera leo) presented with clinical signs of acute pain and a distended abdomen. Despite emergency treatment, the lion died a few hours after presentation."

Pathology: Caliendo et al. (2012): “Postmortem examination revealed gross changes in the liver, spleen, and lungs and an anomalous cystic structure in the bile duct. Histologic examination identified severe generalized multifocal to coalescent necrotizing and neutrophilic hepatitis, neutrophilic splenitis, and mild interstitial pneumonia, consistent with bacterial septicemia. ... Histology of the cystic bile duct nodular lesion demonstrated a polycystic aggregation of torturous tubular structures resembling hyperplastic bile ducts, embedded withing fibrous tissues interspersed with small blood vessels and occasional clusters of lymphocytes, and plasma cells. The ducts varied in diameter, but each was lined by tall cuboidal epithelia in simple or pseudostratified arrays, with papilliform invaginations into the lumen.”