新生儿期起病的罕见肝窦阻塞综合征1例

王子鸣, 邓英平, 施元美, 王来栓, 孙松, 谢新宝, 赵璐, 王宏胜, 李志华

中国当代儿科杂志 ›› 2023, Vol. 25 ›› Issue (9) : 989-994.

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中国当代儿科杂志 ›› 2023, Vol. 25 ›› Issue (9) : 989-994. DOI: 10.7499/j.issn.1008-8830.2307026
多学科诊疗专栏

新生儿期起病的罕见肝窦阻塞综合征1例

  • 王子鸣1, 邓英平1, 施元美2, 王来栓1, 孙松3, 谢新宝4, 赵璐5, 王宏胜6, 李志华1
作者信息 +

A rare case of neonatal-onset hepatic sinusoidal obstruction syndrome

  • 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
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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。

Abstract

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

Key words

Hepatic sinusoidal obstruction syndrome / Omphalitis / Hepatomegaly / Ascites / Neonate

引用本文

导出引用
王子鸣, 邓英平, 施元美, 王来栓, 孙松, 谢新宝, 赵璐, 王宏胜, 李志华. 新生儿期起病的罕见肝窦阻塞综合征1例[J]. 中国当代儿科杂志. 2023, 25(9): 989-994 https://doi.org/10.7499/j.issn.1008-8830.2307026
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. A rare case of neonatal-onset hepatic sinusoidal obstruction syndrome[J]. Chinese Journal of Contemporary Pediatrics. 2023, 25(9): 989-994 https://doi.org/10.7499/j.issn.1008-8830.2307026

参考文献

1 Fan CQ, Crawford JM. Sinusoidal obstruction syndrome (hepatic veno-occlusive disease)[J]. J Clin Exp Hepatol, 2014, 4(4): 332-346. PMID: 25755580. PMCID: PMC4298625. DOI: 10.1016/j.jceh.2014.10.002.
2 董娜, 何丹, 张海霞. 土三七致肝小静脉闭塞病临床特点及预后影响因素[J]. 临床药物治疗杂志, 2020, 18(8): 44-48. DOI: 10.3969/j.issn.1672-3384.2020.08.010.
3 Corbacioglu S, Jabbour EJ, Mohty M. Risk factors for development of and progression of hepatic veno-occlusive disease/sinusoidal obstruction syndrome[J]. Biol Blood Marrow Transplant, 2019, 25(7): 1271-1280. PMID: 30797942. DOI: 10.1016/j.bbmt.2019.02.018.
4 Corbacioglu S, Carreras E, Ansari M, et al. Diagnosis and severity criteria for sinusoidal obstruction syndrome/veno-occlusive disease in pediatric patients: a new classification from the European Society for Blood and Marrow Transplantation[J]. Bone Marrow Transplant, 2018, 53(2): 138-145. PMID: 28759025. PMCID: PMC5803572. DOI: 10.1038/bmt.2017.161.
5 Mohty M, Malard F, Abecassis M, et al. Sinusoidal obstruction syndrome/veno-occlusive disease: current situation and perspectives—a position statement from the European Society for Blood and Marrow Transplantation (EBMT)[J]. Bone Marrow Transplant, 2015, 50(6): 781-789. PMID: 25798682. PMCID: PMC4456788. DOI: 10.1038/bmt.2015.52.
6 Toksvang LN, Schmidt MS, Arup S, et al. Hepatotoxicity during 6-thioguanine treatment in inflammatory bowel disease and childhood acute lymphoblastic leukaemia: a systematic review[J]. PLoS One, 2019, 14(5): e0212157. PMID: 31125338. PMCID: PMC6534292. DOI: 10.1371/journal.pone.0212157.
7 中华医学会消化病学分会肝胆疾病协作组. 吡咯生物碱相关肝窦阻塞综合征诊断和治疗专家共识意见(2017年,南京)[J]. 临床肝胆病杂志, 2017, 33(9): 1627-1637. DOI: 10.3969/j.issn.1001-5256.2017.09.003.
8 Wang JY, Gao H. Tusanqi and hepatic sinusoidal obstruction syndrome[J]. J Dig Dis, 2014, 15(3): 105-107. PMID: 24528632. DOI: 10.1111/1751-2980.12112.
9 Stewart D, Benitz W; COMMITTEE ON FETUS AND NEWBORN. Umbilical cord care in the newborn infant[J]. Pediatrics, 2016, 138(3): e20162149. PMID: 27573092. DOI: 10.1542/peds.2016-2149.
10 Moreira-Silva SF, Frauches DO, Almeida AL, et al. Acute liver failure in children: observations in Vitória, Espírito Santo State, Brazil[J]. Rev Soc Bras Med Trop, 2002, 35(5): 483-486. PMID: 12621668. DOI: 10.1590/s0037-86822002000500010.
11 Listernick R. Liver failure in a 2-day-old infant[J]. Pediatr Ann, 2004, 33(1): 10-14. PMID: 14981864. DOI: 10.3928/0090-4481-20040101-06.
12 董永绥. 肝脾肿大的发病机制和诊断思路[J]. 中国实用儿科杂志, 2004, 19(6): 321-323. DOI: 10.3969/j.issn.1005-2224.2004.06.001.
13 Waggoner DD, Buist NR, Donnell GN. Long-term prognosis in galactosaemia: results of a survey of 350 cases[J]. J Inherit Metab Dis, 1990, 13(6): 802-818. PMID: 1706789. DOI: 10.1007/BF01800204.
14 Brady RO, Filling-Katz MR, Barton NW, et al. Niemann-Pick disease types C and D[J]. Neurol Clin, 1989, 7(1): 75-88. PMID: 2646522.
15 Hostetter MK, Levy HL, Winter HS, et al. Evidence for liver disease preceding amino acid abnormalities in hereditary tyrosinemia[J]. N Engl J Med, 1983, 308(21): 1265-1267. PMID: 6188953. DOI: 10.1056/NEJM198305263082105.
16 Heubi JE, Barbacci MB, Zimmerman HJ. Therapeutic misadventures with acetaminophen: hepatoxicity after multiple doses in children[J]. J Pediatr, 1998, 132(1): 22-27. PMID: 9469995. DOI: 10.1016/s0022-3476(98)70479-2.
17 Gyamlani GG, Parikh CR. Acetaminophen toxicity: suicidal vs. accidental[J]. Crit Care, 2002, 6(2): 155-159. PMID: 11983042. PMCID: PMC111182. DOI: 10.1186/cc1475.
18 Dignan FL, Wynn RF, Hadzic N, et al. BCSH/BSBMT guideline: diagnosis and management of veno-occlusive disease (sinusoidal obstruction syndrome) following haematopoietic stem cell transplantation[J]. Br J Haematol, 2013, 163(4): 444-457. PMID: 24102514. DOI: 10.1111/bjh.12558.
19 Orloff MJ, Orloff MS, Rambotti M. Treatment of bleeding esophagogastric varices due to extrahepatic portal hypertension: results of portal-systemic shunts during 35 years[J]. J Pediatr Surg, 1994, 29(2): 142-151. PMID: 8176584. DOI: 10.1016/0022-3468(94)90309-3.
20 Abd El-Hamid N, Taylor RM, Marinello D, et al. Aetiology and management of extrahepatic portal vein obstruction in children: King's College Hospital experience[J]. J Pediatr Gastroenterol Nutr, 2008, 47(5): 630-634. PMID: 18955865. DOI: 10.1097/MPG.0b013e31817b6eea.
21 Fraser N, Davies BW, Cusack J. Neonatal omphalitis: a review of its serious complications[J]. Acta Paediatr, 2006, 95(5): 519-522. PMID: 16825129. DOI: 10.1080/08035250600640422.
22 Zhu C, Ren X, Liu D, et al. Oxaliplatin-induced hepatic sinusoidal obstruction syndrome[J]. Toxicology, 2021, 460: 152882. PMID: 34352347. DOI: 10.1016/j.tox.2021.152882.
23 DeLeve LD, Valla DC, Garcia-Tsao G, et al. Vascular disorders of the liver[J]. Hepatology, 2009, 49(5): 1729-1764. PMID: 19399912. PMCID: PMC6697263. DOI: 10.1002/hep.22772.

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