Following Kasai portoenterostomy for biliary atresia(BA),long-term survival in children is associated with chronic liver cirrhosis leading to hepatopulmonary syndrome(HPS)and portopulmonary hypertension(Po PH).This fu...Following Kasai portoenterostomy for biliary atresia(BA),long-term survival in children is associated with chronic liver cirrhosis leading to hepatopulmonary syndrome(HPS)and portopulmonary hypertension(Po PH).This further causes a diverse array of respiratory and circulatory symptoms.The incidence of these complications in children with BA is higher than that seen in other chronic liver diseases.With a faster progression,BA is life-threatening in some severe cases.Moreover,HPS and Po PH may coexist in the same child.Enhancing our understanding of HPS/Po PH,making an early diagnosis,rational selection of medication,and appropriate timing of liver transplantation underscore the need for multidisciplinary collaboration.展开更多
文摘Following Kasai portoenterostomy for biliary atresia(BA),long-term survival in children is associated with chronic liver cirrhosis leading to hepatopulmonary syndrome(HPS)and portopulmonary hypertension(Po PH).This further causes a diverse array of respiratory and circulatory symptoms.The incidence of these complications in children with BA is higher than that seen in other chronic liver diseases.With a faster progression,BA is life-threatening in some severe cases.Moreover,HPS and Po PH may coexist in the same child.Enhancing our understanding of HPS/Po PH,making an early diagnosis,rational selection of medication,and appropriate timing of liver transplantation underscore the need for multidisciplinary collaboration.
