POLYSPLENIA WITH PULMONARY ARTERIOVENOUS-MALFORMATIONS
POLYSPLENIA WITH PULMONARY ARTERIOVENOUS-MALFORMATIONS
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DOI：
10.1007/bf00796995
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发表时间：
1993-03-01
影响因子：
1.6
通讯作者：
ARMSTRONG, BE
中科院分区：
文献类型：
--
作者：
PAPAGIANNIS, J;KANTER, RJ;ARMSTRONG, BE
1例多脾综合征、心绞痛、左房异构、大血管关系正常且无心内分流的患者发生进行性紫绀和杵状变。肺动静脉畸形（PAVMs）通过血管造影诊断，并通过肺活检证实。上级肠系膜动脉造影显示肝内门静脉分支发育不全和门体分流。PAVM的可能病因进行了讨论。
A patient with polysplenia syndrome, dextrocardia, left atrial isomerism, normal great vessel relationships, and no intracardiac shunts developed progressive cyanosis and clubbing. Pulmonary arteriovenous malformations (PAVMs) were diagnosed by angiography and confirmed by lung biopsy. Superior mesenteric arteriogram revealed hypoplasia of the intrahepatic portal vein branches and a portosystemic shunt. The possible etiologies of PAVMs are discussed.