Liver, Pancreas and Biliary TractHepatic encephalopathy in patients with non-cirrhotic portal hypertension: Description, prevalence and risk factors
Introduction
Hepatic encephalopathy, at least in the western world, is mainly associated to liver cirrhosis (type C-HE; C from cirrhosis) and is considered a consequence of both the liver damage and the presence of portal-systemic shunts. HE has also been described in animals and in patients with congenital portal-systemic shunts [1], [2] (Type B-HE; B from bypass), suggesting that the presence of a large portal-systemic shunt, by reducing the detoxification of gut derived toxins, may induce alterations in the central nervous system even in the absence of significant liver damage. In the western world, portal-hypertension not due to cirrhosis is an uncommon condition, mainly caused by chronic portal vein thrombosis (PVT). More recently, a disorder characterized by portal hypertension not associated to liver cirrhosis or chronic PVT [3], [4], [5] has been better defined [6], [7] and named as idiopathic non-cirrhotic portal hypertension (INCPH). Patients with non-cirrhotic portal hypertension (NCPH), as well as cirrhotic patients, may develop collaterals and, in some cases, large spontaneous portal-systemic shunts, which may predispose the patients to HE. In liver cirrhosis the relationship between large portal-systemic shunts and HE is supported by a number of clinical observations such as the high incidence of HE after surgical [8] or radiological [9] porto-caval anastomosis, the relationship between the diameter of the shunt and the severity and recurrence of HE episodes and the amelioration of the neurological symptoms after the reduction of the shunt calibre in patients submitted to transjugular intrahepatic portosystemic shunt (TIPS). Moreover, in cirrhotic patients the presence of large portal-systemic shunts have been related to a severe and persistent form of HE, poorly responsive to medical treatment [10], which can be ameliorated by the closure of the shunt [11], [12], [13].
The aims of the present study were to describe the prevalence of the different grades of cognitive impairment (overt HE and minimal/covert HE) in 51 adult Italian patients with NCPH (35 with chronic PVT and 16 with INCPH) and to identify the risk factors for its development, focusing on the role of large portal-systemic shunts.
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Patients and methods
Among 90 patients affected by NCPH seen and followed up in our Centre for the Study of Portal Hypertension, fifty-one patients available for HE assessment during the study period (from October 2014 to June 2015) were included in the study. Thirty-five were affected by chronic portal vein thrombosis (PVT) and 16 by idiopathic non-cirrhotic portal hypertension (INCPH), according to the diagnostic criteria suggested in the EASL Clinical Practice Guidelines on Vascular diseases of the liver [14].
Results
The demographic, clinical and biochemical features obtained in the patients at entry are summarized in Table 1. Age was significantly higher in cirrhotic patients than in patients with NCPH (p < 0.001). Albumin levels were significantly higher (p = 0.008) in chronic PVT group than in the other two, although the mean values were in range of normality in all the three groups.
At the evaluation, signs of portal hypertension (oesophageal varices, variceal bleeding, ascites) were equally present in the
Discussion
In the present study we analyzed the prevalence and the description of the cognitive impairment (overt and minimal HE) detectable in a group of patients with non-cirrhotic portal hypertension which included patients with chronic portal vein thrombosis or with idiopathic non-cirrhotic portal hypertension. A control group of compensated cirrhotic patients (Child-Pugh Class A) was also studied in order to compare the prevalence of cognitive alterations occurring in patients with or without a
Conflict of interest
None declared.
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