新生儿布加综合征1例报告并文献复习
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摘要
目的探讨新生儿布加综合征的临床表现、诊断及治疗。方法回顾1例布加综合征新生儿的临床资料,并复习相关文献。结果患儿,女,21天。胎龄39周,顺产,出生体质量3 150 g。临床表现为腹胀、肝脾肿大、腹水、反复低蛋白血症、血小板计数低下,类似败血症表现。影像学检查提示下腔静脉狭窄段长约24 mm,最狭窄处仅1.59 mm,诊断为布加综合征。放弃治疗后死亡。结论新生儿布加综合征较为罕见,易误诊,病死率高。
Objective To explore the clinical manifestation, diagnosis and treatment of Budd-Chiari syndrome in neonates. Method The clinical data of Budd-Chiari syndrome in a neonate were retrospectively reviewed and relevant literature was reviewed. Results The 21-day-old girl was born through vaginal delivery with gestational age of 39 weeks and birth weight of 3150 g. Her clinical manifestations included abdominal distention, hepatosplenomegaly, ascites, repeated hypoproteinemia and low platelet count, similar to sepsis. Imaging examination indicated hepatic segment stenosis of the inferior vena cava(the stenosis segment was about 24 mm in length and 1.59 mm in diameter at the narrowest place). The girl was diagnosed with BuddChiari syndrome and died after abandoning treatment. Conclusion Neonatal Budd-Chiari syndrome is rare, easily misdiagnosed and has a high mortality.
Objective To explore the clinical manifestation, diagnosis and treatment of Budd-Chiari syndrome in neonates. Method The clinical data of Budd-Chiari syndrome in a neonate were retrospectively reviewed and relevant literature was reviewed. Results The 21-day-old girl was born through vaginal delivery with gestational age of 39 weeks and birth weight of 3150 g. Her clinical manifestations included abdominal distention, hepatosplenomegaly, ascites, repeated hypoproteinemia and low platelet count, similar to sepsis. Imaging examination indicated hepatic segment stenosis of the inferior vena cava(the stenosis segment was about 24 mm in length and 1.59 mm in diameter at the narrowest place). The girl was diagnosed with BuddChiari syndrome and died after abandoning treatment. Conclusion Neonatal Budd-Chiari syndrome is rare, easily misdiagnosed and has a high mortality.
引文
[1]蒋立韦,朱力平,候海燕.布-加综合征误诊26例分析[J].中国误诊学杂志,2011,11(3):637.
[2]周蓓华,邵肖梅,张旭东,等.新生儿Budd-Chiari综合征二例[J].中华围产医学杂志,1998,1(1):38.
[3]Parker RG.Occlusion of the hepatic viens in man[J].Medicine(Baltimore),1959,38:369-402.
[4]Janssen HL,Garcia-Pagan JC,Elias E,et al.Budd-Chiari syndrome:a review by an expert panel[J].J Hepatol,2003,38(3):364-371.
[5]Ludwig J,Hashimoto E,Mcgill DB,et al.Classification of hepatic venous outflow obstruction:ambiguous terminology of the Budd-Chiari syndrome[J].Mayo Clin Proc,1990,65(1):51-55.
[6]Qi X,De Stefano V,Wang J,et al.Prevalence of inherited antithrombin,protein C,and protein S deficiencies in portal vein system thrombosis and Budd-Chiari syndrome:a systematic review and meta-analysis of observational studies[J].J Gastroenterol Hepatol,2013,28(3):432-442.
[7]Wang H,Sun G,Zhang P,et al.JAK2 V617F mutation and46/1 haplotype in Chinese Budd-Chiari syndrome patients[J].J Gastroenterol Hepatol,2014,29(1):208-214.
[8]Vella DC.Primary Budd-Chiari syndrome[J].J Hepatol,2009,50(1):195-203.
[9]Zhang W,Qi X,Zhang X,et al.Budd-Chiari syndrome in China:a systematic analysis of epidemiological features based on the Chinese literature survey[J].Gastroenterol Res Pract,2015,2015:738548.
[10]Hadengue A,Poliquin M,Vilgrain V,et al.The changing s c e n e o f h e p a t i c v e i n t h r o m b o s i s:r e c o g n i t i o n o f asymptomatic cases[J].Gastroenterology,1994,106(4):1042-1047.
[11]中华医学会放射学分会介入学组.布加综合征介入诊疗规范的专家共识[J].中华放射学杂志,2010,44(4):345-349.
[12]European Association for the Study of the Liver.EASLclinical practice guidelines:vascular diseases of the liver[J].J Hepatol,2016,64(1):179-202.
[13]Simonetto DA,Yang HY,Yin M,et al.Chronic passive venous congestion drives hepatic fibrogenesis via sinusoidal thrombosis and mechanical forces[J].Hepatology,2015,61(2):648-659.
[14]党晓卫,李路豪.我国布加综合征的治疗现状[J].中华普通外科学,2013,7(6):423-426.
[15]Seijo S,Plessier A,Hoekstra J,et al.Good long-term outcome of Budd-Chiari syndrome with a step-wise management[J].Hepatology,2013,57(5):1962-1968.
[16]Tripathi D,Sunderraj L,Vemala V,et al.Long-term outcomes following percutaneous hepatic vein recanalization for BuddChiari syndrome[J].Liver Int,2017,37(1):111-120.
[17]Tripathi D,Macnicholas R,Kothari C,et al.Good clinical outcomes following transjugular intrahepatic portosystemic stent-shunts in Budd-Chiari syndrome[J].Aliment Pharmacol Ther,2014,39(8):864-872.
[18]Fagiuoli S,Bruno R,Debernardi Venon W,et al.Consensus conference on TIPS management:techniques,indications,contraindications[J].Dig Liver Dis,2017,49(2):121-137.
[19]Wang Q,Lv Y,Bai M,et al.Eight millimetre covered TIPSdoes not compromise shunt function but reduces hepatic encephalopathy in preventing variceal rebleeding[J].JHepatol,2017,67(3):508-516.
[20]Segev DL,Nguyen GC,Locke JE,et al.Twenty years of liver transplantation for Budd-Chiari syndrome:a national registry analysis[J].Liver Transpl,2007,13(9):1285-1294.
[2]周蓓华,邵肖梅,张旭东,等.新生儿Budd-Chiari综合征二例[J].中华围产医学杂志,1998,1(1):38.
[3]Parker RG.Occlusion of the hepatic viens in man[J].Medicine(Baltimore),1959,38:369-402.
[4]Janssen HL,Garcia-Pagan JC,Elias E,et al.Budd-Chiari syndrome:a review by an expert panel[J].J Hepatol,2003,38(3):364-371.
[5]Ludwig J,Hashimoto E,Mcgill DB,et al.Classification of hepatic venous outflow obstruction:ambiguous terminology of the Budd-Chiari syndrome[J].Mayo Clin Proc,1990,65(1):51-55.
[6]Qi X,De Stefano V,Wang J,et al.Prevalence of inherited antithrombin,protein C,and protein S deficiencies in portal vein system thrombosis and Budd-Chiari syndrome:a systematic review and meta-analysis of observational studies[J].J Gastroenterol Hepatol,2013,28(3):432-442.
[7]Wang H,Sun G,Zhang P,et al.JAK2 V617F mutation and46/1 haplotype in Chinese Budd-Chiari syndrome patients[J].J Gastroenterol Hepatol,2014,29(1):208-214.
[8]Vella DC.Primary Budd-Chiari syndrome[J].J Hepatol,2009,50(1):195-203.
[9]Zhang W,Qi X,Zhang X,et al.Budd-Chiari syndrome in China:a systematic analysis of epidemiological features based on the Chinese literature survey[J].Gastroenterol Res Pract,2015,2015:738548.
[10]Hadengue A,Poliquin M,Vilgrain V,et al.The changing s c e n e o f h e p a t i c v e i n t h r o m b o s i s:r e c o g n i t i o n o f asymptomatic cases[J].Gastroenterology,1994,106(4):1042-1047.
[11]中华医学会放射学分会介入学组.布加综合征介入诊疗规范的专家共识[J].中华放射学杂志,2010,44(4):345-349.
[12]European Association for the Study of the Liver.EASLclinical practice guidelines:vascular diseases of the liver[J].J Hepatol,2016,64(1):179-202.
[13]Simonetto DA,Yang HY,Yin M,et al.Chronic passive venous congestion drives hepatic fibrogenesis via sinusoidal thrombosis and mechanical forces[J].Hepatology,2015,61(2):648-659.
[14]党晓卫,李路豪.我国布加综合征的治疗现状[J].中华普通外科学,2013,7(6):423-426.
[15]Seijo S,Plessier A,Hoekstra J,et al.Good long-term outcome of Budd-Chiari syndrome with a step-wise management[J].Hepatology,2013,57(5):1962-1968.
[16]Tripathi D,Sunderraj L,Vemala V,et al.Long-term outcomes following percutaneous hepatic vein recanalization for BuddChiari syndrome[J].Liver Int,2017,37(1):111-120.
[17]Tripathi D,Macnicholas R,Kothari C,et al.Good clinical outcomes following transjugular intrahepatic portosystemic stent-shunts in Budd-Chiari syndrome[J].Aliment Pharmacol Ther,2014,39(8):864-872.
[18]Fagiuoli S,Bruno R,Debernardi Venon W,et al.Consensus conference on TIPS management:techniques,indications,contraindications[J].Dig Liver Dis,2017,49(2):121-137.
[19]Wang Q,Lv Y,Bai M,et al.Eight millimetre covered TIPSdoes not compromise shunt function but reduces hepatic encephalopathy in preventing variceal rebleeding[J].JHepatol,2017,67(3):508-516.
[20]Segev DL,Nguyen GC,Locke JE,et al.Twenty years of liver transplantation for Budd-Chiari syndrome:a national registry analysis[J].Liver Transpl,2007,13(9):1285-1294.