Pandey Snehavardhan, Sood Vikrant, Khanna Rajeev, Lal Bikrant B, Sood Arun Kumar, Kabra S K, Alam Seema
Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, 110 070, India.
Department of Cardiology, Institute of Liver and Biliary Sciences, New Delhi, India.
Indian J Gastroenterol. 2020 Feb;39(1):66-74. doi: 10.1007/s12664-020-01015-0. Epub 2020 Mar 18.
Limited pediatric literature is available regarding hepatopulmonary syndrome (HPS) especially in subjects with biliary atresia (BA) despite its proven prognostic significance. Thus, we aimed to study the natural history, risk factors, and outcome of HPS in BA and other chronic liver disease (CLD) subjects.
All children (BA and other non-BA CLDs) older than 6 months of age were included in the study. HPS was diagnosed on the basis of standard international criteria. Also, fractional exhaled nitric oxide (FeNO) was measured at baseline.
During the study period from January 2017 to December 2018, there were 42 children in BA and 62 in the CLD group. The overall prevalence of HPS was 42.3%: 57.1% in the BA group and 32.2% in the CLD group. Median age at HPS diagnosis was 14.4 months and 90 months in the BA and non-BA CLD groups, respectively. By the end of study period, the prevalence of HPS in the BA group further increased to 73.8% at 0.7% per month. Lower serum albumin (p < 0.05) in BA and higher splenic Z scores (p 0.013) in other CLDs were found to be significant risk factors for HPS. FeNO measurement did not reach diagnostic significance.
Prevalence of HPS is higher and also develops at an earlier age in the BA group compared to other CLDs. Also, risk of HPS development increases with increasing disease duration in BA. Lower serum albumin in BA and higher splenic Z scores in other CLDs may predict risk for HPS development.
尽管肝肺综合征（HPS）已被证实具有预后意义，但关于小儿肝肺综合征的文献有限，尤其是在胆道闭锁（BA）患者中。因此，我们旨在研究BA及其他慢性肝病（CLD）患者中HPS的自然病史、危险因素及预后。
本研究纳入所有年龄大于6个月的儿童（BA及其他非BA的CLD患者）。HPS根据标准国际标准进行诊断。此外，在基线时测量呼出一氧化氮分数（FeNO）。
在2017年1月至2018年12月的研究期间，BA组有42例儿童，CLD组有62例。HPS的总体患病率为42.3%：BA组为57.1%，CLD组为32.2%。BA组和非BA的CLD组HPS诊断时的中位年龄分别为14.4个月和90个月。到研究期结束时，BA组HPS的患病率以每月0.7%的速度进一步升至73.8%。发现BA患者较低的血清白蛋白（p<0.05）和其他CLD患者较高的脾脏Z评分（p=0.013）是HPS的重要危险因素。FeNO测量未达到诊断意义。
与其他CLD相比，BA组HPS的患病率更高且发病年龄更早。此外，BA患者中HPS的发病风险随病程延长而增加。BA患者较低的血清白蛋白和其他CLD患者较高的脾脏Z评分可能预示HPS的发病风险。
