Dept. of Clinical Medicine, Center for the Diagnosis and Treatment of Portal Hypertension, Rome, Italy.
Dept. of Clinical Medicine, Center for the Diagnosis and Treatment of Portal Hypertension, Rome, Italy.
Dig Liver Dis. 2018 Aug;50(8):839-844. doi: 10.1016/j.dld.2018.01.132. Epub 2018 Jan 31.
The knowledge of natural history of patients with portal hypertension (PH) not due to cirrhosis is less well known than that of cirrhotic patients.
To describe the clinical presentation and the outcomes of 89 patients with non-cirrhotic PH (25 with non-cirrhotic portal hypertension, INCPH, and 64 with chronic portal vein thrombosis, PVT) in comparison with 77 patients with Child A cirrhosis.
The patients were submitted to a standardized clinical, laboratory, ultrasonographic and endoscopic follow-up. Variceal progression, incidence of variceal bleeding, portal vein thrombosis, ascites and survival were recorded.
At presentation, the prevalence of varices, variceal bleeding and ascites was similar in the 3 groups. During follow-up, the rate of progression to varices at risk of bleeding (p < 0.0001) and the incidence of first variceal bleeding (p = 0.02) were significantly higher in non-cirrhotic then in cirrhotic patients. A PVT developed in 32% of INCPH patients and in 18% of cirrhotics (p = 0.02).
In the patients with non-cirrhotic PH variceal progression is more rapid and bleeding more frequent than in cirrhotics. Patients with INCPH are particularly prompt to develop PVT. This observational study suggests that the management of patients with non-cirrhotic PH should take into consideration the natural history of portal hypertension in these patients and cannot be simply derived by the observation of cirrhotic patients.
非肝硬化性门静脉高压(PH)患者的自然病史知识不如肝硬化患者那么了解。
描述 89 例非肝硬化性 PH 患者(25 例非肝硬化性门静脉高压,INCPH,64 例慢性门静脉血栓形成,PVT)的临床表现和结局,并与 77 例 Child A 级肝硬化患者进行比较。
患者接受了标准化的临床、实验室、超声和内镜随访。记录了静脉曲张进展、静脉曲张出血、门静脉血栓形成、腹水和生存率。
在初次就诊时,3 组患者的静脉曲张、静脉曲张出血和腹水的发生率相似。在随访期间,非肝硬化患者进展为有出血风险的静脉曲张(p<0.0001)和首次静脉曲张出血(p=0.02)的发生率明显高于肝硬化患者。INCPH 患者中有 32%发生了 PVT,而肝硬化患者中有 18%发生了 PVT(p=0.02)。
在非肝硬化性 PH 患者中,静脉曲张进展更快,出血更频繁,而非肝硬化性 PH 患者比肝硬化患者更容易发生 PVT。INCPH 患者尤其容易发生 PVT。这项观察性研究表明,非肝硬化性 PH 患者的管理应考虑到这些患者门静脉高压的自然病史,而不能简单地从肝硬化患者的观察中推断。