The pathogenesis of Kotb disease involves immune mediated damage through neutrophil elastase and CD14 + activated monocytes.32 Immunohistochemical staining of the portoenterostomy liver core of infants with BA, provided evidence that there is disruption of p53 and GST Pi,35 thus disrupting the fidelity to regeneration in BA, resulting in relentless accelerated cirrhosis. The gene discussed is ELANE; the disease is Cirrhosis.