Osteogenesis imperfecta (OI) is a hereditary connective tissue disorder characterized by bone fragility and Type I collagen defects. Although dentinogenesis imperfecta (DI) is a classic manifestation, patients with OI may experience significant dental deterioration even in its absence due to inherent dentinal weakness. This report describes the multidisciplinary full-mouth rehabilitation of a 18-year-old male with OI and a complex medical history including autoimmune hepatitis and seizure disorder. The patient presented with extensive caries, failure of previous restorations, multiple impacted teeth, and radiographic evidence of preeruptive intracoronal resorption (PEIR) as well as open apices in permanent second molars. Severe microstomia, joint laxity, high risk of iatrogenic jaw fracture, and limited cooperation necessitated treatment under general anesthesia. Management included vital pulp therapy to promote apexogenesis in immature teeth, stainless steel crowns for posterior rehabilitation, and composite restorations using universal adhesive systems containing 10-MDP. A conservative, non-extraction strategy was adopted to minimize mandibular fracture risk. This case highlights that dental pathology in OI may originate within bone prior to eruption and emphasizes the importance of biologically driven and fracture-conscious treatment planning in medically complex patients.

Fulminant viral hepatitis (FVH) in children is a rare but often fatal form of acute liver failure occurring in the absence of preexisting liver disease. Its exceptional incidence during otherwise common viral infections, including hepatitis A virus (HAV), hepatitis B virus (HBV), and herpes simplex virus (HSV), supports a decisive role for host susceptibility. Recent advances in human immunogenetics delineate two major, mechanistically distinct pathways to pediatric FVH. The first reflects failure of immune regulation, culminating in excessive IFN-γ-driven inflammation and immune-mediated hepatocellular necrosis. Autosomal recessive IL-18BP and IL-10RB deficiencies exemplify this mechanism, in which disruption of key regulatory checkpoints permits uncontrolled activation of cytotoxic lymphocytes and macrophage-dependent immunopathology, particularly in the context of HAV infection. The second pathway involves impaired intrinsic antiviral defense, most prominently through neutralizing autoantibodies against type I interferons, which phenocopy genetic defects of IFN-I signaling and are strongly associated with HSV-triggered FVH; in this setting, inadequate early antiviral control enables unchecked hepatic replication with extensive cytopathic damage. Finally, syndromic hyperinflammatory disorders, including familial hemophagocytic lymphohistiocytosis and X-linked lymphoproliferative disease, broaden the spectrum of immune predisposition in which fulminant hepatitis may arise. Together, these discoveries redefine pediatric FVH as an immunopathological syndrome and provide a framework for targeted genetic and serologic diagnosis and for mechanism-based interventions aimed at improving survival.

[This corrects the article DOI: 10.3389/fimmu.2026.1843865.].

Autoimmune hepatitis (AIH) with negative anti-nuclear antibody (ANA) has been reported to frequently exhibit acute clinical and histological features. We herein report a case of asymptomatic AIH with negative ANA, normal early-phase immunoglobulin G (IgG) levels, and chronic histological features. A 49-year-old asymptomatic female was found to have elevated liver enzymes during a routine health check. Liver biopsy demonstrated portal-predominant lymphoplasmacytic infiltrates with interface hepatitis, compatible with chronic AIH. Despite compatible histology, the simplified criteria yielded "unlikely" (score 3), and the revised International Autoimmune Hepatitis Group score "probable" (score 14) due to absent serology. However, the patient met the Japanese guidelines for "atypical AIH" based on histology and exclusion of other causes. Consequently, corticosteroid therapy was initiated. Notably, a rapid biochemical response and a delayed IgG elevation at treatment onset confirmed "typical AIH" by Japanese criteria, whereas the international scores remained non-definitive. This case highlights that AIH should not be excluded solely because ANA is negative and IgG levels are initially normal. Histological assessment, exclusion of alternative etiologies, monitoring IgG dynamics, and therapeutic response remain essential for establishing the diagnosis.

Drug-induced liver injury is one of the leading causes of acute liver injury and may mimic other liver diseases, including idiopathic autoimmune hepatitis. Although albendazole is generally a safe antiparasitic agent, it can be associated with rare cases of clinically significant liver injury; it can even cause a mixture of both types of injuries, or a type of hepatitis that is similar to an autoimmune disease, such as positive autoantibodies and elevated IgG, making differential diagnosis more difficult. We present a case of albendazole-induced liver injury mimicking autoimmune hepatitis in a 41-year-old woman with marked hyperbilirubinemia, positive ANA, and elevated IgG levels, in whom liver biopsy and clinical follow-up played an important role in clarifying the diagnosis.

[This corrects the article DOI: 10.3389/fimmu.2025.1510770.].

Exosomes are extracellular membrane vesicles with a diameter of 30-150 nm secreted by various cell types into the extracellular environment and regulating many vital physiological processes, including signaling, immune activation, proliferation, differentiation, and apoptosis. Numerous studies conducted in recent years have demonstrated the key role of exosomes in the development of various gastrointestinal diseases, including cancer, allowing them to be considered as potentially promising diagnostic biomarkers. Furthermore, due to their inherent biocompatibility, ability to overcome biological barriers, low toxicity, and immunogenicity, exosomes can be used for targeted drug delivery in the treatment of patients with gastrointestinal diseases. This review article systematizes and presents information on exosomes, their functions, extraction and detection methods, and examines the main exosomal markers, which demonstrate significant potential for the diagnosis and treatment of various gastrointestinal diseases.

Immune dysregulation in pediatric acute liver failure (PALF) is a distinct phenomenon that has garnered interest with respect to disease outcomes and targeted therapies. Some patients with PALF have an "indeterminate" (iPALF) etiology ranging from acute severe hepatitis to fulminant liver failure. Recent evidence from iPALF demonstrates that a large subset suffers from a unified, immune-mediated disorder. This immune-mediated PALF has been defined by the presence of dense T-cell infiltrates on liver biopsy and is often referred to as T-cell PALF (TC-PALF). TC-PALF has common features with other inflammatory liver diseases, including autoimmune hepatitis, hemophagocytic lymphohistiocytosis, and macrophage activation syndrome, while also demonstrating distinct pathologic features that contribute to liver injury in PALF. In this review, the spectrum of disease, comparisons between young and aged liver immune microenvironments, and the current literature evaluating the immune system during PALF are summarized. Relationships between TC-PALF, other inflammatory liver diseases, and contemporary studies that associate specific immune subsets with this pathology are also reviewed. These studies use precision "omic" technologies to investigate tissue and blood samples in TC-PALF and have opened new lines of investigation into potential genetic, immunologic, and environmental risk factors for disease. Together, recent data suggest that immune dysregulation is a central feature of TC-PALF, and facets of disease offer potential biomarker identification to aid in the clinical management of TC-PALF.

Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease treated with glucocorticoids (GCs). Long-term GCs therapy may increase the risk of hepatic steatosis. This study aimed to evaluate the impact of prolonged GCs therapy on hepatic steatosis in patients with AIH.

Autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome is rare and incompletely defined. Its association with renal disease is less frequently reported. Systemic disorders such as systemic lupus erythematosus, immunoglobulin G4-related disease, light chain disorders, amyloidosis, and pediatric autoimmune polyendocrine syndromes may cause concurrent hepatic and renal involvement; however, renal disease with liver pathology in the absence of systemic illness is exceedingly uncommon. We describe a case of autoimmune hepatitis/primary sclerosing cholangitis overlap complicated by nephrotic syndrome due to minimal change disease with tubulointerstitial involvement. No systemic disease or drug-induced cause was identified. Renal function fully recovered with treatment while liver disease persisted.

Sexual dimorphism, which is defined as systematic differences between male and female individuals not limited to characteristics related to reproduction, has been recognized in many organ systems and is emerging as an important component of hepatic function in health and disease. Oestrogen is considered the predominant driver of sexual dimorphism in the liver. Other molecular mechanisms independent of oestrogen including epigenetic regulation and immune function contribute to differences between sexes as do other as yet unknown mechanisms. Clinically, hepatic sexual dimorphisms manifest in many ways. For example, women have a lower incidence of many liver disorders including metabolic-dysfunction-associated steatotic liver disease and liver cancer but are more susceptible to benign neoplasms and autoimmune liver diseases such as primary biliary cholangitis. Better understanding the diverse contributors to hepatic sexual dimorphism, including sociodemographic factors, could deepen our understanding of liver biology and aid in sex-specific clinical management and disease prevention. In this Review, we describe the molecular mechanisms of sexual dimorphisms in liver physiology, including oestrogen signalling, genes encoded on sex chromosomes, epigenetic effects on metabolism and immune function. We also describe hepatic sexual dimorphisms in liver pathophysiology, including in metabolic-dysfunction-associated steatotic liver disease, autoimmune diseases, viral hepatitis and liver cancer.