National trends and outcomes of genetically inherited non-alcoholic chronic liver disease in the USA: estimates from the National Inpatient Sample (NIS) database

BACKGROUND: Medical literature on the prevalence of genetic liver disease is lacking. In this study, we investigated the in-hospital healthcare and economic burden from genetic causes of non-alcoholic chronic liver disease (NACLD) and non-alcoholic liver cirrhosis (NALC) in the USA. METHODS: Data were abstracted from the National Inpatient Sample database between 2002 and 2014 using ICD9 codes for patients discharged with NACLD and NALC secondary to genetic diseases including alpha-1 antitrypsin deficiency (A1ATd), cystic fibrosis (CF), Wilson disease (WD), hereditary hemochromatosis (HHC), glycogen storage disease, and disorders of aromatic amino-acid metabolism (DAAAM). RESULTS: Throughout the study period, there were 19,332 discharges for NACLD associated with the six genetic diseases including 14,368 for NALC. There were $1.09 billion in hospital charges, 790 in-hospital deaths, and 955 liver transplants performed. Overall, A1ATd was associated with 8,983 (62.52%) hospitalizations for NALC followed by WD, CF, and HHC. The highest in-hospital mortality was seen with HHC. The greatest frequency of liver transplants was seen with DAAAM. CONCLUSION: The number of hospitalizations for genetic liver diseases continues to increase. With increased funding and directed research efforts, we can aim to improve medical treatments and the quality of life for patients at risk for liver deterioration.