International Journal of Clinical Research
International Journal of Clinical Research. 2024; 8: (11) ; 10.12208/j.ijcr.20240449 .
总浏览量: 108
1 大理大学临床医学院 云南大理
2 大理大学第二附属医院消化内科 云南昆明
*通讯作者: 杨涓,单位: 大理大学第二附属医院消化内科 云南昆明;
NCPH的核心特征是门静脉系统压力升高,常见并发症包括脾肿大、食管胃底静脉曲张及腹水等。NCPH与妊娠关联是一种罕见的情况,而NCPH合并HELLP 综合征则更为少见。在妊娠期间,由于血容量和心输出量的增加,血流动力学发生显著变化,这些变化可能加剧门静脉高压,导致静脉曲张破裂出血的风险上升。特别是对于NCPH患者,这种风险可能进一步增加。HELLP综合征的出现,特征为溶血、肝酶升高和血小板减少,进一步加剧了多系统损害,从而显著提高了围产期并发症和死亡风险。由于缺乏更广泛的研究和标准的临床实践指南,其诊疗和管理对消化科、肝病科、产科医生都具有挑战性。在此分享1例在NCPH基础上发生部分性HELLP综合征的病例,加强医生对此类疾病的认识及重视。
The core feature of Non-Cirrhotic Portal Hypertension (NCPH) is elevated pressure in the portal venous system, with common complications including splenomegaly, esophageal and gastric varices, and ascites. The association of NCPH with pregnancy is a rare occurrence, and the combination of NCPH with HELLP syndrome is even less common. Hemodynamic changes during pregnancy, the worsening of portal hypertension, and multi-system damage caused by HELLP syndrome collectively exacerbate the patient's condition and significantly increase the risk of perinatal complications and mortality. Due to the lack of broader research and standardized clinical practice guidelines, the diagnosis and management of this condition pose challenges for gastroenterologists, hepatologists, and obstetricians. Here, we share a case of partial HELLP syndrome occurring on the basis of NCPH to enhance physicians' awareness and attention to such diseases.
[1] B L T A, D G T C .Portal Hypertension: Pathogenesis and Diagnosis - ScienceDirect[J].Clinics in Liver Disease, 2019, 23( 4):573-587.
[2] 何福亮,马琳,李悦榕等.非肝硬化性门脉高压的临床诊断[J].实用肝脏病杂志,2022,25(01):1-4.
[3] 周慧,冯晓宁,任浩,等. 非肝硬化门脉高压患者临床特点分析.实用肝脏病杂志. 2021,24(3): 415-418.
[4] 杨柳,邹丽.HELLP综合征诊治的最新认识[J].中华产科急救电子杂志, 2021, 10(3):5.
[5] Gibbens J, Spencer S K, Solis L ,et al.Fas Ligand neutralization attenuates hypertension, endothelin-1 and placental inflammation in an animal model of HELLP Syndrome[J]. AJP Regulatory Integrative and Comparative Physiology, 2020, 319(2).
[6] Aggarwal N, Negi N , Aggarwal A ,et al.Pregnancy with Portal Hypertension[J].Journal of Clinical and Experimental Hepatology, 2014.
[7] Ali A A A , Eldin I B .Outcome of pregnancy in women with splenomegaly[J].BMC Pregnancy and Childbirth, 2023, 23(1).
[8] Morton A , Laurie J , Hill J .Portal hypertension in pregnancy – Concealed perils[J]. Obstetric Medicine, 2018, 13(3): 1753495X1880146.
[9] Giri S , Sahoo S .Pregnancy in Patients with Non-cirrhotic Portal Hypertension: A Literature Review[J].Revista Brasileira de Ginecologia e Obstetrícia / RBGO Gynecology and Obstetrics[2024-09-22].
[10] Gala A R , Surapaneni T , Aziz N ,et al.A Review of Outcomes in Pregnant Women with Portal Hypertension[J].Springer India, 2018(6).
[11] Keepanasseril A , Gupta A , Ramesh D ,et al.Maternal–fetal outcome in pregnancies complicated with non-cirrhotic portal hypertension: experience from a Tertiary Centre in South India[J].Hepatology International, 2020, 14(2).
[12] Ali A A A , Eldin I B .Outcome of pregnancy in women with splenomegaly[J].BMC Pregnancy and Childbirth, 2023, 23(1).
[13] Early ML, Eke AC, Gemmill A, Lanzkron S, Pecker LH. Comparisons of Severe Maternal Morbidity and Other Adverse Pregnancy Outcomes in Pregnant People With Sickle Cell Disease vs Anemia. JAMA Netw Open. 2023;6(2): e2254545.