In sub-Saharan Africa, canine babesiosis, the most significant tick-borne disease in dogs, is primarily caused by the highly virulent intra-erythrocytic protozoan Babesia rossi, transmitted by ixodid ticks. The disease presents in two forms: uncomplicated and complicated. The former is characterized by hemolysis, while the latter involves severe systemic inflammation and multiple organ dysfunction, leading to high morbidity and mortality. Building on recent evidence of severe immunoinflammatory disruption in the spleen, lung, and bone marrow, this study examines liver histopathology in fatal Babesia rossi infection to determine whether the frequent hyperbilirubinemia and icterus of complicated babesiosis reflect primary hepatic injury or secondary systemic effects.

Vanishing bile duct syndrome is a rare complication of classical Hodgkin's lymphoma (cHL) which may present with significant cholestasis and hepatic dysfunction. Management is primarily focused on chemotherapy and symptomatic management of cholestatic liver failure. We describe a case of a man in his 60s with relapsed cHL with significant cholestatic liver injury who was treated with brentuximab vedotin complicated by renal failure. The complex decision-making surrounding chemotherapeutic choice with the presence of multiorgan dysfunction is discussed with a review of literature describing regimens in similar clinical circumstances.

Ursodeoxycholic acid (UDCA) is a hydrophilic, non-toxic bile acid that represents the gold standard in the treatment of various cholestatic and hepatobiliary diseases. Its therapeutic benefit lies in its complex pleiotropic mechanism of action, including cytoprotection of hepatocytes and cholangiocytes, stimulation of hepatobiliary secretion, antiapoptotic effects, and immunomodulatory properties. This article comprehensively summarizes the pharmacological characteristics of UDCA and its rational clinical use in key indications such as primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), dissolution of cholesterol gallstones, reactive gastritis due to duodenogastric reflux, and intrahepatic cholestasis of pregnancy (ICP). Furthermore, it focuses on the safety profile of UDCA, including its excellent tolerability, dose-dependent diarrhea, and confirmed safety for use during pregnancy and lactation, making it a crucial drug in hepatology.

Autoimmune pericarditis is commonly idiopathic but may represent the initial manifestation of systemic autoimmune disease. Hepatic autoimmune disorders rarely present with primary cardiac involvement, and recognition of extrahepatic presentations is essential for early diagnosis and prevention of organ damage. Autoimmune hepatitis-primary biliary cholangitis (AIH-PBC) overlap syndrome is an uncommon entity characterized by combined hepatocellular and cholestatic immune-mediated injury. A previously healthy young woman presented with progressive pleuritic chest pain and dyspnea and was found to have a large pericardial effusion causing hemodynamic compromise requiring surgical drainage. Pericardial pathology demonstrated acute and chronic fibrinous pericarditis. Initial evaluation revealed elevated inflammatory markers and mild transaminitis with positive autoimmune serologies including antinuclear antibody and anti-smooth muscle antibody. Following clinical improvement, she developed recurrent pericarditis accompanied by worsening liver enzyme abnormalities. Liver biopsy demonstrated chronic active hepatitis with bile duct injury and bridging fibrosis, consistent with autoimmune hepatitis-primary biliary cholangitis overlap syndrome. Cardiac magnetic resonance imaging confirmed active pericardial inflammation without myocarditis. Immunosuppressive therapy with corticosteroids followed by azathioprine resulted in clinical stabilization. This case highlights autoimmune pericarditis as the presenting manifestation of AIH-PBC overlap syndrome and underscores the importance of evaluating unexplained pericarditis for systemic autoimmune disease. Early recognition of cardio-hepatic autoimmune overlap allows timely immunosuppressive therapy and may prevent progression to advanced hepatic fibrosis.

Progressive familial intrahepatic cholestasis type 3 (PFIC-3), caused by pathogenic variants in the ABCB4 gene, is a rare inherited cholestatic liver disorder that often presents later in childhood. In some patients, hematologic manifestations may dominate the clinical picture and delay recognition of the underlying liver disease. We report a 4-year-old girl referred for evaluation of anemia, hepatosplenomegaly, and recurrent bleeding. Initial investigation focused on primary hematologic causes. However, imaging revealed portal hypertension with esophageal varices, and genetic testing identified a homozygous pathogenic ABCB4 variant, confirming the diagnosis of PFIC-3. This case highlights how chronic cholestatic liver disease may masquerade as a primary hematologic disorder and emphasizes the importance of considering systemic causes in children presenting with anemia, hepatosplenomegaly, and bleeding manifestations. Early recognition of underlying liver disease is essential to facilitate appropriate multidisciplinary management and prevent potentially life-threatening complications.

Budd-Chiari syndrome (BCS) is a rare hepatic vascular disorder frequently associated with prothrombotic conditions such as myeloproliferative neoplasms. Pregnancy and assisted reproductive technologies further increase thrombotic and portal-hypertensive risk. We report a successful in vitro fertilization-conceived pregnancy in a 35-year-old woman with chronic BCS secondary to JAK2-positive polycythemia vera. The pregnancy was complicated by ovarian hyperstimulation syndrome and severe intrahepatic cholestasis of pregnancy. Progressive portal hypertension with worsening esophageal varices prompted planned preterm delivery at 34 weeks' gestation. A multidisciplinary team performed cesarean delivery under epidural anesthesia with prophylactic perioperative terlipressin administration and thromboprophylaxis. Maternal and neonatal outcomes were favorable. With careful multidisciplinary management, pregnancy in women with stable BCS is feasible.

Low-phospholipid-associated cholelithiasis (LPAC) syndrome is a rare ABCB4-related cholestatic disorder characterized by recurrent biliary symptoms and intrahepatic lithiasis. A 55-year-old woman with recurrent intrahepatic hepatolithiasis underwent multiple endoscopic retrograde cholangiopancreatographies with lithotripsy and stenting without durable stone clearance, ultimately requiring right hepatic lobectomy. Postoperative genetic testing identified a heterozygous ABCB4 variant, confirming LPAC syndrome, and ursodeoxycholic acid therapy was initiated with subsequent clinical stability. This case highlights an atypical late-onset LPAC presentation and diagnostic challenges when imaging findings are subtle. LPAC should be considered in recurrent intrahepatic stones regardless of age.

Vanishing bile duct syndrome (VBDS) is a rare cholangiopathy that may follow drug-induced liver injury and can progress to liver failure. No established pharmacological treatment is currently available. We present a case of a 65-year-old man diagnosed with histologically confirmed and progressive symptomatic VBDS, probably sustained by drug-induced liver injury, who was successfully treated with a 4-week course of off-label ileal bile acid transporter inhibitor treatment of the management of pruritus. To our knowledge, this is among the first reports of successful ileal bile acid transporter inhibitor use in VBDS. This case highlights the potential role of bile acid modulation in managing severe symptomatic VBDS and underscores the importance of a proactive, multidisciplinary approach to optimize outcomes in complex cholangiopathies.

Undifferentiated carcinoma with osteoclast-like giant cells of the pancreas is an extremely rare malignancy with nonspecific radiological features, often requiring histological confirmation. We report the case of a 57-year-old woman presenting with abdominal pain and cholestasis, in whom imaging revealed a pancreatic head mass. After an initial nondiagnostic endoscopic ultrasound sampling, endoscopic ultrasound-guided fine-needle biopsy performed at our tertiary referral center allowed definitive diagnosis by identifying characteristic osteoclast-like giant cells and undifferentiated neoplastic cells with a specific immunohistochemical profile. This case highlights the key role of endoscopic ultrasound-guided fine-needle biopsy in the preoperative diagnosis of undifferentiated carcinoma with osteoclast-like giant cells.

Arthrogryposis, Renal dysfunction and Cholestasis (ARC) syndrome is a rare inherited disorder caused by defects in the VPS33B trafficking protein, leading to impaired bile flow, progressive liver disease and early death. No effective treatments are currently available. Gene therapy offers a potential approach by restoring the missing VPS33B protein in liver cells. Here, we show that liver-targeted lentiviral gene therapy safely and effectively rescues key features of ARC syndrome in a mouse model following in vivo administration by intravenous injection, combined with transient liver macrophage depletion. To assess the treatment efficacy, disease severity is exacerbated by 0.25% cholic acid diet feeds. A liver-specific vector shows a favourable safety profile over a ubiquitous vector. Mice receiving the safe liver-specific vector display improved survival, growth and liver function, reduced fibrosis and bile canaliculi restoration. These findings support targeted gene therapy as a promising treatment for ARC syndrome and related early-onset liver diseases.