Severe Hepatic Encephalopathy Research Articles

Пространственно-ориентационные параметры узнавания изображений лиц как предикторы симптомов печеночной недостаточности у подростков

Functional assessment of higher mental functions in case of intoxication or during treatment will make it possible to identify predictors of the symptoms of hepatic encephalopathy associated with portal hypertension. The study was aimed to determine the diagnostic predictors of the emergence of the earliest symptoms of hepatic encephalopathy in adolescents with portal hypertension. The study involved 60 adolescents aged 13–17 years: 28 males, 32 females. The experimental group included 30 adolescents with the diagnosis К76.6 Portal hypertension, unspecified form. The control group included 30 adolescents with normal somatic status, who had no mental disorders, traumatic brain injuries or severe infectious diseases of the brain (based on the records of the annual check-up). The studied groups were matched by sex, age, and social status. The proprietary method, Tobii EyeX hardware and software system (GazeControl software), and Cambridge Face Memory Test for Children (CFMT-C) were used. It has been found that recognition of single face images, multiple face images, and multiple face images camouflaged with noise by adolescents with portal hypertension is associated with the greater efforts (manifested in the increased number and duration of gaze fixations), than recognition of the above by adolescents with normal somatic status. The accuracy of recognition of single face images, multiple face images, and multiple face images camouflaged with noise shown by adolescents with portal hypertension experiencing the toxic effects associated with manifestations of hepatic encephalopathy is significantly reduced compared to that shown by adolescents with normal somatic status. The results obtained can be considered as the diagnostic predictors allowing one to trace the changes in the hepatic encephalopathy severity at various stages of treatment (including after surgical intervention).

Association of low serum 25-Hydroxy vitamin D [25(OH) d] with hepatic encephalopathy in patients with decompensated liver cirrhosis

Background and study aimsThe mechanism of hepatic encephalopathy is complex and has not been conclusively established. Recent studies support lower serum 25-Hydroxy Vitamin D [25(OH) D] levels in patients with hepatic encephalopathy. This study aimed to evaluate the association between serum 25(OH) D and hepatic encephalopathy in patients with decompensated cirrhosis of liver. Patients and methodsA total of 70 cirrhosis patients (35 cases of hepatic encephalopathy and 35 patients without encephalopathy as control, mean age 53.07 ± 12.99 years, 67 % male) were recruited for this study. Assessment of the severity of cirrhosis was done by using a model for end-stage liver disease(MELD) and Child Turcotte Pugh (CTP) scores, and assessment of the severity of hepatic encephalopathy was done according to West Haven criteria. Serum 25 (OH) D level was measured by Chemiluminescent Microparticle Immuno Assay(CMIA). ResultsThe mean serum 25(OH) D level among hepatic encephalopathy patients was significantly lower in comparison to the control group without encephalopathy (18.76 ± 8.84 nmol/L vs 31.19 ± 13.9 nmol/L, P<0.0001). 91.4 % of hepatic encephalopathy patients had moderate to severe 25(OH)D deficiency as compared to 51.4 % in the control group. There was a significant correlation observed between the severity of the 25 (OH) D deficiency and the severity of liver disease (r = − 0.35, P = 0.002). No statistically significant difference in serum 25(OH) D levels was found among patients with different hepatic encephalopathy grades (P = 0.416). ConclusionA significant association was found between a low serum 25(OH) D leveland hepatic encephalopathy. It requires further large-scale multicenter studies to establish it as a risk factor and predictor of hepatic encephalopathy.

Serum Ammonia Levels Do Not Correlate With Overt Hepatic Encephalopathy Severity in Hospitalized Patients With Cirrhosis

Although ammonia is involved in the pathophysiology of hepatic encephalopathy (HE), the use of ammonia levels in clinical practice is problematic.1-3 For example, in a study of 551 patients with overt HE (OHE) receiving lactulose who had ammonia levels tested, only 60% had an increased ammonia level (defined as >72 μmol/L).2 Overall, there was no correlation observed between lactulose dose and whether ammonia levels were obtained (ie, presence/absence of increased ammonia level did not guide therapy), or between time to OHE resolution and ammonia levels.2 Additionally, there is substantial interlaboratory variability in sample handling and processing, which may affect ammonia measurements.4.

Severe hepatic encephalopathy with mechanical ventilation may inform waitlist priority in acute liver failure: A UNOS database analysis.

Patients with acute liver failure (ALF) awaiting liver transplantation (LT) may develop multiorgan failure, but organ failure does not impact waitlist prioritization. The aim of this study was to examine the impact of organ failure on waitlist mortality risk and post LT outcomes in patients with ALF. We studied adults waitlisted for ALF in the United Network for Organ Sharing (UNOS) database (2002-2019). Organ failures were defined using a previously described Chronic Liver Failure modified sequential organ failure score assessment adapted to UNOS data. Regression analyses of the primary endpoints, 30-day waitlist mortality (Competing risk), and post-LT mortality (Cox-proportional hazards), were performed. Latent class analysis (LCA) was used to determine the organ failures most closely associated with 30-day waitlist mortality. About 3212 adults with ALF were waitlisted, for hepatotoxicity (41%), viral (12%) and unspecified (36%) etiologies. The median number of organ failures was three (interquartile range 1-3). Having ≥3 organ failures (vs. ≤2) was associated with a sub hazard ratio (HR) of 2.7 (95%CI 2.2-3.4)) and a HR of 1.5 (95%CI 1.1-2.5)) for waitlist and post-LT mortality, respectively. LCA identified neurologic and respiratory failure as most impactful on 30-day waitlist mortality. The odds ratios for both organ failures (vs. neither) were higher for mortality 4.5 (95% CI 3.4-5.9) and lower for delisting for spontaneous survival .5 (95%CI .4-.7) and LT .6 (95%CI .5-.7). Cumulative organ failure, especially neurologic and respiratory failure, significantly impacts waitlist and post-LT mortality in patients with ALF and may inform risk-prioritized allocation of organs.

Evaluating clinical outcomes and prognosis in patients with cirrhosis and portal hypertension: a retrospective observational cohort study

ObjectiveCirrhosis describes the end-stage of chronic liver disease. Irreversible changes in the liver cause portal hypertension, which can progress to serious complications and death. Only a few studies with small...

Regional cerebral blood flow in dogs with congenital extrahepatic portosystemic shunts before surgery and six months after successful closure

Previous studies both in humans and dogs with chronic liver diseases have shown that regional cerebral brain flow (rCBF) is altered. The current study aimed to assess abnormalities in rCBF in dogs with congenital extrahepatic portosystemic shunts (cEHPSS), both at diagnosis and after successful surgical attenuation. Furthermore, the influence of age at diagnosis, severity of hepatic encephalopathy (HE) and type of cEHPSS on rCBF were explored as a base for future research.Single photon emission computed tomography (SPECT) with 99mtechnetium-hexamethylpropylene amine oxime tracer was performed before surgical attenuation and six months postoperatively.Twenty-four dogs with cEHPSS had SPECT at time of diagnosis and 13 dogs with a confirmed closed cEHPSS had a second SPECT six months postoperatively. At diagnosis, dogs with cEHPSS had an altered rCBF distribution compared to healthy dogs. This altered rCBF distribution seemed to be most apparent in dogs ≥ one year and in dogs with overt HE at diagnosis. Six months postoperatively, only the rCBF distribution in the subcortical region decreased compared to pre-operatively.In conclusion, all dogs with cEHPSS had altered rCBF which did not seem to normalize completely six months after successful surgical attenuation. Dogs diagnosed at an older age seemed to have more distinct abnormalities in rCBF compared to younger dogs.

TCD assessment in fulminant hepatic failure: Improvements in cerebral autoregulation after liver transplantation

Introduction and ObjectivesAcute liver failure, also known as fulminant hepatic failure (FHF), includes a spectrum of clinical entities characterized by acute liver injury, severe hepatocellular dysfunction and hepatic encephalopathy. The objective of this study was to assess cerebral autoregulation (CA) in 25 patients (19 female) with FHF and to follow up with seventeen of these patients before and after liver transplantation. Patients and MethodsThe mean age was 33.8 years (range 14–56, SD 13.1 years). Cerebral hemodynamics was assessed by transcranial Doppler (TCD) bilateral recordings of cerebral blood velocity (CBv) in the middle cerebral arteries (MCA). ResultsCA was assessed based on the static CA index (SCAI), reflecting the effects of a 20–30 mmHg increase in mean arterial blood pressure on CBv induced with norepinephrine infusion. SCAI was estimated at four time points: pretransplant and on the 1st, 2nd and 3rd posttransplant days, showing a significant difference between pre- and posttransplant SCAI (p = 0.005). SCAI peaked on the third posttransplant day (p = 0.006). Categorical analysis of SCAI showed that for most patients, CA was reestablished on the second day posttransplant (SCAI > 0.6). ConclusionsThese results suggest that CA impairment pretransplant and on the 1st day posttransplant was re-established at 48–72 h after transplantation. These findings can help to improve the management of this patient group during these specific phases, thereby avoiding neurological complications, such as brain swelling and intracranial hypertension.

Association Of Electroencephalogram Patterns With Ammonia Levels In Hepatic Encephalopathy Patients

Objective: To find out the association between electroencephalogram (EEG) and hepatic encephalopathy.&#x0D; Methodology: This cross-sectional study included 100 patients (with the age of52.5±6.09years for males and 51.7 ± 6.10 years for females) of reported hepatic encephalopathy, visiting the medical department (indoor and OPD) at Federal Government Polyclinic Hospital, Islamabad. The study was conducted from January 2020 to May 2021. Patients who had known epileptic and structural brain lesions or strokes were excluded from the study. Statistical analysis was done using GraphPad Prism software. The significance of data (p-value or R2 value) was calculated through a two-tailed test or correlation coefficient.&#x0D; Results: All the patients in hepatic encephalopathy grade IV reported abnormal EEG representing triphasic waves and flattening of EEG pattern. There was no correlation observed between age, gender and hepatic encephalopathy grades. However, a significant correlation (R2= 0.9032) was observed between serum ammonia levels and hepatic encephalopathy grades. Elevated serum ammonia levels depicted the severity of hepatic encephalopathy. Overall, the percentage of patients with abnormal EEG increased with increasing grade of hepatic encephalopathy. It was quite intriguing to note that EEG, being the common method to diagnose hepatic encephalopathy grades, is not dependent on patients’ socio-economic status.&#x0D; Conclusion: Data concluded that serum ammonia levels are well associated with the progression of hepatic encephalopathy. Moreover, the EEG patter provides the appropriate information about the neurological abnormalities associated with the severity of hepatic encephalopathy. Hence, serum ammonia levels and EEG both should accurately be used as indicators for diagnosis and monitoring the response to the treatment of various grades of hepatic encephalopathy. Data warrant further investigations to get a better insight into hepatic encephalopathy's relationship with EEG patterns through the inclusion of molecular parameters.

Educational Nursing Program Implementation: It’s Effect on Hepatic Encephalopathy Severity among Patients with Liver Cirrhosis

Educational Nursing Program Implementation: It’s Effect on Hepatic Encephalopathy Severity among Patients with Liver Cirrhosis

Hypokalemia as a responsible factor related with the severity of hepatic encephalopathy: a wide multination cross-sectional study.

After taking approval from the hospital ethical review committee, a total of 5000 patients with hepatic encephalopathy were recruited by consecutive sampling. They were interviewed, examined and investigated for serum potassium levels and other precipitating factors of hepatic encephalopathy. Total of 5000 patients including 3070 (61.4%) males and 1930 (38.6%) females, aging 13 years and above were studied. The frequency of hypokalemia was 78% (3900 patients). Relating the serum potassium level with the severity of hepatic encephalopathy, 1200 (60%) out of 2000 patients with serum potassium below 2.5mEq/l were in grade 4 (40%) and 800 out of 2000 were in grade 3 encephalopathy. On the other hand, only 700 patients (6.4%) out 1100 with serum potassium above 3.4mEq/l were in grade 4 encephalopathy. Hypokalemia is a frequent finding in patients with hepatic encephalopathy and found to be directly related to its severity.

WED-383 - Serum ammonia levels do not correlate with overt hepatic encephalopathy severity and time to resolution in hospitalized patients with cirrhosis

WED-383 - Serum ammonia levels do not correlate with overt hepatic encephalopathy severity and time to resolution in hospitalized patients with cirrhosis

The role of albumin replacement therapy in the treatment of hepatic encephalopathy in patients with liver cirrhosis

Introduction. It is often observed that decompensated liver cirrhosis is accompanied by hepatic encephalopathy. The role of violation of the structural and functional properties of albumin in the development of this pathology is currently not defined.Aim. Was to evaluate the effect of albumin replacement therapy on the clinical manifestations of hepatic encephalopathy and the severity of structural and functional changes in serum albumin.Materials and methods. The study included 28 adult patients with decompensated cirrhosis and hepatic encephalopathy divided into two comparable groups. In addition to the standard examination, the properties of the albumin molecule were evaluated using the EPR test. The dynamics of indicators was assessed in the groups of standard treatment and standard treatment + albumin. Mathematical processing was carried out in the StatTech v. 3.1.4 (developer Stattech LLC, Russia).Results and discussion. It was revealed that patients with decompensated liver cirrhosis have serious disturbances in the albumin configuration, which lead to a decrease in its functional activity. The ability to bind, transport and detoxify were impaired in the vast majority of patients. The native conformation of the molecule was abnormal in all patients. Against the background of albumin replacement therapy, these disorders are able to recover to normal values. Along with this, we found that albumin replacement therapy reduced the severity of hepatic encephalopathy to a greater extent than standard treatment.Conclusion. Violation of the structural and functional albumin properties is associated with the progression of hepatic encephalopathy in patients with liver cirrhosis. Transfusions of highly concentrated human albumin lead to the normalization of the molecule properties and the encephalopathy regression.

Severe Acute Liver Dysfunction Induces Delayed Hepatocyte Swelling and Cytoplasmic Vacuolization, and Delayed Cortical Neuronal Cell Death.

Liver dysfunction is the main cause of hepatic encephalopathy. However, histopathological changes in the brain associated with hepatic encephalopathy remain unclear. Therefore, we investigated pathological changes in the liver and brain using an acute hepatic encephalopathy mouse model. After administering ammonium acetate, a transient increase in the blood ammonia level was observed, which returned to normal levels after 24 h. Consciousness and motor levels also returned to normal. It was revealed that hepatocyte swelling, and cytoplasmic vacuolization progressed over time in the liver tissue. Blood biochemistry also suggested hepatocyte dysfunction. In the brain, histopathological changes, such as perivascular astrocyte swelling, were observed 3 h after ammonium acetate administration. Abnormalities in neuronal organelles, especially mitochondria and rough endoplasmic reticulum, were also observed. Additionally, neuronal cell death was observed 24 h post-ammonia treatment when blood ammonia levels had returned to normal. Activation of reactive microglia and increased expression of inducible nitric oxide synthase (iNOS) were also observed seven days after a transient increase in blood ammonia. These results suggest that delayed neuronal atrophy could be iNOS-mediated cell death due to activation of reactive microglia. The findings also suggest that severe acute hepatic encephalopathy causes continued delayed brain cytotoxicity even after consciousness recovery.

Clinical heterogeneity of hyperornithinemia-hyperammonemia-homocitrullinuria syndrome in thirteen palestinian patients and report of a novel variant in the SLC25A15 gene.

Background: Hyperornithinemia-Hyperammonemia-Homocitrullinuria (HHH) syndrome, is a rare autosomal recessive disorder characterized by impaired ornithine transport across the inner mitochondrial membrane. HHH is caused by biallelic disease-causing variants in the SLC25A15 gene. The clinical presentation of HHH is highly variable ranging from severe neonatal encephalopathy and hepatic failure to a milder form with corresponding learning difficulties. Methods: In this study, data from thirteen patients with HHH syndrome, diagnosed between the age of 1week-29years at two tertiary care centers in Palestine, is presented. The clinical, biochemical, and molecular data are reviewed. Results: Analysis of the SLC25A15 gene sequence revealed a novel homozygous frameshift deletion in exon 5, NM_014252.4:c.552-555delTTTC; p (Phe185SerfsTer8) in nine patients. The remaining four patients had a recurrent homozygous frameshift variant; NM_014252.4:c.446delG, (p.Ser149ThrfsTer45). The major acute clinical presentation found was encephalopathy and liver dysfunction. Nervous system involvement was common, progressive, and presented with signs of upper motor neuron disease as well as variable degrees of cognitive impairment. One patient had an initial presentation in adulthood with acute encephalopathy that responded well to treatment. There was no clear genotype-phenotype correlation. Conclusion: Our results confirm the marked clinical heterogeneity of HHH including severe neonatal presentation, hepatic failure, and progressive pyramidal tract dysfunction in all age groups. The disease progression was variable, even in patients with the same genetic variant, and in patients with severe neonatal-onset hepatic encephalopathy. We report a novel pathogenic variant in the SLC25A15 gene, further expanding the molecular spectrum of the disease.

Suppressed serological vitamin A in patients with liver cirrhosis is associated with impaired liver function and clinical detoriation.

BackgroundThe liver is of critical importance for the homeostasis of metabolic and immunomodulatory properties as well as the storage of vitamins, especially vitamin A. In this prospective analysis, the incidence of serological vitamin A deficiency and the association with disease severity as well as clinical complications in patients with liver cirrhosis were investigated.MethodFrom May 2017 to May 2018, 159 patients with primarily alcohol-associated and non-alcoholic steatohepatitis (NASH)-associated preexisting liver cirrhosis were prospectively enrolled and vitamin A status was collected. Clinical complications and infections were followed and recorded over a period of 1-year follow-up. Selected findings were validated in an independent cohort of 44 patients.ResultsAt study inclusion, 77% of patients showed decreased serological vitamin A. Suppressed vitamin A was more common in alcoholic (52 vs. 8%) and NASH-associated liver cirrhosis (16 vs. 9%) than in viral-associated liver cirrhosis. MELD score as well as Child-Pugh score were significantly associated with suppressed vitamin A (P < 0.001). The association between the degree of vitamin A suppression and liver function was confirmed in univariate and multivariate regression analysis. After 1 year of follow-up, 57 patients died and 21 patients received a liver transplant. In addition, low vitamin A levels were more commonly observed in patients with severe ascites (P = 0.001), hepatic encephalopathy (P = 0.002) and hepatorenal syndromes (P = 0.008). In addition, patients with reduced vitamin A showed an increased incidence of infections (P = 0.02), especially respiratory infections (P = 0.04).Conclusion:Suppressed serological Vitamin A is common in patients with liver cirrhosis and is associated with liver function. Clinical complications and infections are more frequent in patients with liver cirrhosis and vitamin A suppression.

Sex is associated with differences in oxidative stress and susceptibility to severe hepatic encephalopathy in bile-duct ligated rats.

Hepatic encephalopathy (HE) is a debilitating neurological complication of chronic liver disease (CLD). Hyperammonemia plays an important role in HE's pathogenesis, acting synergistically with systemic oxidative stress. During CLD, muscle plays a compensatory role in detoxifying ammonia, and therefore muscle loss leads to an increase in the risk of developing HE. With most animal studies involving males, sex's impact on the development of CLD and associated complications such as HE and muscle loss remains unknown. Therefore, we aimed to identify the impact of sex on CLD, HE, and muscle mass loss in a rodent model of CLD. Liver injury markers, hyperammonemia, oxidative stress, muscle mass, and ammonia clearance were measured in female and male bile-duct ligated (BDL) rats. In addition, covert HE was assessed in females while ammonia-precipitated severe HE was assessed in female and male BDL rats, and male BDL rats treated with allopurinol (100 mg/kg), an antioxidant (xanthine oxidase inhibitor). Female BDL developed CLD and HE (impaired motor coordination and night activity) compared to respective SHAM. Hyperammonemia and muscle ammonia clearance were similar between female and male BDL. However, only female BDL rats did not develop muscle loss, brain edema, and short-term memory impairment (vs. female SHAM) and systemic oxidative stress and decreased albumin levels (vs. male BDL). Furthermore, both female BDL and allopurinol-treated male BDL rats were protected against ammonia-induced overt HE. In conclusion, female and male BDL rats develop distinct features of CLD and HE, with systemic oxidative stress playing a pivotal role in the susceptibility to ammonia-precipitated overt HE.

Effects of PCSK-9 Inhibition by Alirocumab Treatments on Biliary Cirrhotic Rats.

Hyperlipidemia and oxidative stress with elevated oxidized low-density lipoprotein (ox-LDL) exacerbate hepatic inflammation and fibrosis. The plasma level of low-density lipoprotein (LDL) is controlled by proprotein convertase subtilisin/kexin 9 (PCSK9). Alirocumab is a monoclonal antibody that decreases LDL via inhibiting PCSK9 function. Apart from lipid-lowering effects, alirocumab exerts anti-inflammation, anti-angiogenesis and anti-oxidant effects. This study aims to investigate the impact of alirocumab treatment on common bile duct ligation (BDL)-induced biliary cirrhotic rats. After a 4-week treatment of alirocumab, the hemodynamic data, blood biochemistry, ox-LDL level, oxidative stress markers, severity of hepatic encephalopathy and abnormal angiogenesis of BDL rats were measured and compared to the control group. BDL rats presented cirrhotic pictures and elevated ammonia, total cholesterol, LDL and ox-LDL levels compared to the control group. Alirocumab decreased plasma levels of total cholesterol, LDL, and oxidative stress markers; however, it did not affect the hemodynamics, liver and renal biochemistry, and the plasma levels of ammonia and ox-LDL. The motor activities, portal-systemic collaterals and mesenteric vascular density were not significantly different between alirocumab-treated and control groups. In addition, it did not affect hepatic inflammation, intrahepatic angiogenesis, liver fibrosis and free cholesterol accumulation in the liver of BDL rats. In conclusion, PCSK9 inhibition by alirocumab treatment ameliorates hyperlipidemia and systemic oxidative stress in biliary cirrhotic rats. However, it does not affect the plasma level of ox-LDL, intrahepatic inflammation and fibrosis. In addition, PCSK9 inhibition has a neutral effect on abnormal angiogenesis and hepatic encephalopathy in biliary cirrhotic rats.

Correlation of Serum Sodium Level with Severity of Hepatic Encephalopathy

Objective: To determine correlation of serum sodium levels with severity of hepatic encephalopathy (HE). Study Design: A Cross-sectional study. Place and Duration of the Study: Department of Medicine, Mardan Medical Complex, Mardan from 1st October 2020 to 30th April 2021. Methodology: A total of 170 patients of both genders with liver cirrhosis presenting with HE aged 35-65 years were included. Under aseptic conditions, blood was taken dispatched to institutional laboratory for serum sodium levels. Severity of hyponatremia was compared with study variables along with grades of HE. Post stratification chi square test was applied and p&lt;0.05 was considered statistically significant. Results: Out of a total of 170 patients, 140 (82.4%) we male. Overall, mean age was 55.0±3.87 years while 130 (76.5%) patients were aged between 51 to 65 years. Grading of HE revealed most of the patients, 73 (42.9%) to be having grade-III. Grading of hyponatremia revealed that 101(59.4%) patients had mild hyponatremia, 50 (29.4%) moderate hyponatremia while 19 (11.2%) had profound hyponatremia. Age distribution was found to have significant association with severity of hyponatremia (p&lt;0.0001). Significant correlation between grades of HE and severity of hyponatremia was noted as 15 (78.9%) out of 19 profound hyponatremia cases belonged to HE grade-III whereas all 50 patients of moderate hyponatremia belong to HE grade III and IV (p&lt;0.0001). Conclusion: Hyponatremia was observed to be a very common abnormality among patients of liver cirrhosis presenting with hepatic encephalopathy. Significant correlation of hyponatremia was found with respect to HE grades as severity of hyponatremia increased with severity of HE grades. Higher age groups had significant association with higher degree of hyponatremia. Keywords: Hepatic encephalopathy, hyponatremia, liver cirrhosis, serum sodium.

A Case of Surgical Repair for End-Stage Tricuspid Regurgitation with Severe Liver Dysfunction and Hepatic Encephalopathy

A Case of Surgical Repair for End-Stage Tricuspid Regurgitation with Severe Liver Dysfunction and Hepatic Encephalopathy

Nodular Liver in Portosinusoidal Vascular Disease: A Case of Mistaken Identity

Background Portosinusoidal vascular disease (PSVD) has been proposed as a unifying term for vascular abnormalities of the small portal veins and sinusoids that occur in the noncirrhotic liver. This term supersedes other designations including incomplete septal cirrhosis, nodular regenerative hyperplasia, hepatoportal sclerosis, and obliterative portal venopathy. Most patients have noncirrhotic portal hypertension, but the latter is not required to establish a diagnosis of PSVD. Diagnosis requires a liver biopsy of adequate size (≥20 mm long) in the presence of accompanying clinical signs of noncirrhotic portal hypertension and/or histological changes of PSVD. We report a case of severe PSVD that caused severe liver atrophy and required transplantation for intractable hepatic encephalopathy. The explanted liver showed numerous focal nodular hyperplasia-like nodules. Case Report A 57-year-old man with neurofibromatosis type 2 and diabetes mellitus type 2 controlled with insulin developed ascites and signs of portal hypertension. He was presumed to have cirrhosis due to nonalcoholic fatty liver disease and underwent liver transplantation for intractable portal hypertension and severe hepatic encephalopathy. The explanted liver had a nodular appearance; however, histological sections showed no bridging fibrosis. Thin, incomplete curvilinear fibrous septa were seen emanating from portal tracts in a patchy distribution. Our case illustrates that PSVD may cause severe hepatic atrophy and lead to architectural abnormalities that may radiologically mimic the parenchymal nodularity of cirrhosis.