Patients with iCCA exhibit gut microbiota shifts marked by opportunistic pathogen enrichment (e.g., Veillonella, Klebsiella), with pathogen-derived LPS activating TLR4 on cholangiocytes and KCs to induce NF-κB-driven IL-6/TNF-α production, amplifying biliary injury and cholestasis (21). Here, TLR4 is linked to infantile convulsions and choreoathetosis.