Idiopathic neonatal hepatitis associated with a fatal coagulopathy

Idiopathic neonatal hepatitis (INH) remains a diagnosis of exclusion in the neonate with conjugated hyperbilirubinemia. The major diagnostic challenge for the clinician is to distinguish this condition from other treatable liver disorders such as biliary atresia. Although the prognosis varies for the familial and sporadic forms of neonatal hepatitis, a poor outcome is generally distinguished by chronic and progressive liver failure. After careful review of the literature, we were unable to find a case of INH associated with a profound, irreversible coagulopathy. Herein, we present a case with the primary presenting findings of persistent prolongation of the prothrombin time (PT) and hypofibrinogenemia, resulting in a catastrophic intracranial hemorrhage and death.