Fewtrell M S, Noble-Jamieson G, Revell S, Valente J, Friend P, Johnston P, Rasmussen A, Jamieson N, Calne R Y, Barnes N D
Department of Paediatrics, Addenbrooke's Hospital, Cambridge.
Arch Dis Child. 1994 Jun;70(6):501-4. doi: 10.1136/adc.70.6.501.
One hundred and seventy three children, including 93 with biliary atresia, received liver grafts at Addenbrooke's Hospital between 1983 and 1993. Of these, only seven developed cyanosis due to intrapulmonary shunting as a complication of their liver disease, and all seven of these had the biliary atresia/polysplenia syndrome. Intrapulmonary shunting was confirmed by a radioisotope scan in four children. Only one child with the syndrome did not have cyanosis when undergoing transplantation. Seven of the eight children are alive 6-54 months after transplantation, with normal pulmonary and hepatic function. Cyanosis recurred in one child who developed chronic rejection with liver failure.
(a) there is a strong association between the biliary atresia/polysplenia syndrome and cyanosis due to intrapulmonary shunting; (b) intrapulmonary shunting is fully reversible after successful liver transplantation; and (c) cyanosis, once present, is progressive, and these children should be considered for liver transplantation as soon as it occurs.
1983年至1993年间，173名儿童在阿登布鲁克医院接受了肝脏移植，其中93名患有胆道闭锁。在这些儿童中，只有7名因肺内分流作为其肝脏疾病的并发症而出现发绀，并且这7名儿童均患有胆道闭锁/多脾综合征。4名儿童通过放射性同位素扫描确诊为肺内分流。患有该综合征的儿童中只有1名在接受移植时没有发绀。8名儿童中有7名在移植后6至54个月存活，肺和肝功能正常。1名发生慢性排斥反应并伴有肝衰竭的儿童再次出现发绀。
（a）胆道闭锁/多脾综合征与因肺内分流导致的发绀之间存在密切关联；（b）成功进行肝脏移植后，肺内分流可完全逆转；（c）一旦出现发绀，病情会进展，这些儿童一旦出现发绀就应考虑进行肝脏移植。
