摘要
Background:Medical literature on the prevalence of genetic liver disease is lacking.In this study,we investigated the inhospital healthcare and economic burden from genetic causes of non-alcoholic chronic liver disease(NACLD)and nonalcoholic 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 improvemedical treatments and the quality of life for patients at risk for liver deterioration.
背景:有关遗传性肝病流行情况的医学文献比较缺乏。本研究中,我们调查了美国遗传性非酒精性慢性肝病(NACLD)和非酒精性肝硬化(NALC)患者的住院治疗情况和经济负担。方法:数据来源于美国国家住院患者样本(NIS)数据库,选取2002-2014年间NACLD和NALC患者(代码为ICD9)中有关遗传性疾病的资料,包括1型抗胰蛋白酶缺乏症(A1ATd)、囊性纤维化(CF)、肝豆状核变性(WD)、遗传性血友病(HHC)、糖原贮积病(GSD)和芳香族氨基酸代谢紊乱病(DAAAM)。结果:研究期间共有19,332例与上述6种遗传性疾病相关的NACLD,其中NALC 14,368例。住院费用共计10.9亿美元。790例患者住院期间死亡,955例患者接受了肝移植手术。8,983例(62.52%)NALC住院患者患者A1ATd,其次是WD(2,375例,16.53%)、CF(1,532例,10.66%)和HHC(923例,6.42%)。住院患者病死率最高的是HHC患者,接受肝移植频率最高的是DAAAM患者。结论:遗传性肝疾病的住院患者数量持续上升。随着研究经费的增多和专项研究的开展,我们能够最终提高这些存在肝脏恶化风险的肝病患者的治疗效果和生活质量。
