新生儿期起病的罕见肝窦阻塞综合征1例  

A rare case of neonatal-onset hepatic sinusoidal obstruction syndrome

作　　者：王子鸣 邓英平 施元美[2] 王来栓 孙松[3] 谢新宝[4] 赵璐 王宏胜 李志华 WANG Zi-Ming;DENG Ying-Ping;SHI Yuan-Mei;WANG Lai-Shuan;SUN Song;XIE Xin-Bao;ZHAO Lu;WANG Hong Sheng;LI Zhi-Hua(Department of Neonatology,Children's Hospital of Fudan University/National Children's Medical Center/National Health Commission Key Laboratory of Neonatal Diseases,Shanghai 201102,China)

机构地区：[1]国家儿童医学中心/复旦大学附属儿科医院新生儿科/国家卫生健康委员会新生儿疾病重点实验室,上海201102 [2]泰兴市人民医院儿科,江苏泰州225499 [3]国家儿童医学中心/复旦大学附属儿科医院新生儿外科,上海201102 [4]国家儿童医学中心/复旦大学附属儿科医院肝病科,上海201102 [5]国家儿童医学中心/复旦大学附属儿科医院心内科,上海201102 [6]国家儿童医学中心/复旦大学附属儿科医院血液科,上海201102

出　　处：《中国当代儿科杂志》2023年第9期989-994,共6页Chinese Journal of Contemporary Pediatrics

摘　　要：患儿,男,1个月14 d,因腹胀2周,加重3 d入院。患儿生后曾有脐炎病史,体格检查示腹胀明显、腹壁静脉显露、肝脾触诊不满意,移动性浊音阳性。肝脏转氨酶轻度增高,肝脏超声及CT均提示门静脉肝内段及肝脏左中右静脉肝内段不显影、管腔闭塞、周边纤维增生,临床诊断为脐炎引发的肝窦阻塞综合征。入院后12 d患儿出现大量血性腹水,继发低血容量性休克、呼吸衰竭,家属放弃治疗后临床死亡。该文对新生儿期起病的肝窦阻塞综合征的诊断思路及多学科诊疗进行重点描述,并总结肝大、腹水的鉴别思路。A male infant,aged 1 month and 14 days,was admitted to the hospital due to abdominal distension lasting for 2 weeks and worsening for 3 days.The infant had a history of omphalitis.Physical examination revealed severe abdominal distension,prominent abdominal wall veins,hepatosplenomegaly,and massive ascites.There was a slight elevation in liver transaminase levels.Liver ultrasound and CT scans demonstrated the absence of visualization of the intrahepatic segment of the portal vein and the left,middle,and right veins of the liver,indicating occlusion of these vessels,along with surrounding fibrous hyperplasia.The clinical diagnosis was hepatic sinusoidal obstruction syndrome resulting from omphalitis.A large amount of bloody ascites developed after 12 days of hospitalization,resulting in hypovolemic shock and respiratory failure.The infant passed away following the family's decision to discontinue treatment.This article focuses on the diagnostic approach and multidisciplinary management of neonatal-onset hepatic sinusoidal obstruction syndrome,as well as provides insights into the differential diagnosis of hepatomegaly and ascites.

关 键 词：肝窦阻塞综合征 脐炎 肝大 腹水 新生儿 

分 类 号：R722.1[医药卫生—儿科]