Sequelae Of Portal Hypertension Research Articles

Superior mesenteric arteriovenous fistula embolization with a vascular plug for upper gastrointestinal hemorrhage: a case report

BackgroundSuperior mesenteric arteriovenous fistula is a rare vascular anomaly often presenting with sequelae of portal hypertension, heart failure, or mesenteric ischemia. This report describes a patient with a previously unidentified superior mesenteric arteriovenous fistula who presented with variceal bleeding, thought to be the leading cause of mortality associated with this condition. Although this patient was initially referred for a transjugular intrahepatic portosystemic shunt procedure, following a thorough review of her clinical history and imaging, she instead underwent embolization of the arteriovenous fistula likely responsible for her symptoms.Case PresentationA 75-year-old woman with a past surgical history of extensive small bowel resection presented with active variceal bleeding requiring transfusions. She was referred to vascular and interventional radiology for transjugular intrahepatic portosystemic shunt procedure; however, her clinical presentation was inconsistent with cirrhosis. This prompted a further review of her imaging, which identified a superior mesenteric arteriovenous fistula as the probable etiology of her varices. This fistula was subsequently embolized with a vascular plug and follow-up upper endoscopy at 1-month demonstrated complete resolution of her varices.ConclusionsThis report highlights a potential etiology of variceal bleeding in the acutely ill patient. Through a thorough consultation, the patient described here was able to avoid a procedure with the potential to cause catastrophic consequences, and instead receive the appropriate treatment for an uncommon condition.Level of EvidenceLevel 4, Case Report.

Transjugular intrahepatic portosystemic shunt for the management of symptomatic malignant pseudocirrhosis

Pseudocirrhosis is defined by radiologic changes of the liver parenchyma secondary to metastatic disease and/or cancer treatments, and portends a high rate of morbidity and mortality from sequelae of portal hypertension. Transjugular intrahepatic portosystemic shunt (TIPS) is an effective treatment for portal hypertension; however, TIPS is relatively contraindicated in the setting of hepatic metastases. The study aims to determine the technical efficacy and clinical outcomes of patients undergoing TIPS for symptomatic pseudocirrhosis. Retrospective analysis of patients with hepatic malignancy who underwent TIPS between 2008 and 2020 at a single tertiary care center. Patients with imaging findings of pseudocirrhosis and without history of primary liver malignancy or confounding causes of cirrhosis were included. West Haven scores assessing hepatic encephalopathy were obtained from chart review. Technical success was defined as successful TIPS creation with reduction in the portosystemic gradient (PSG). Clinical success was defined as resolution of variceal bleeding and/or ascites. Nine patients (4 female/5 male), average (± SD) age 61.2±9.5 years with metastatic pseudocirrhosis were included for analysis. Primary malignancy was colorectal adenocarcinoma (n=5), neuroendocrine tumor (n=3), and malignant endothelial hemangioendothelioma (n=1). Average Model for End Stage Liver Disease (MELD-Na) score was 15.7±3.7. Technical success was 8/9 (89%) with average PSG reduced from 23.5±11.0 to 6.5±2.8 mmHg (P=0.001). Clinical success was 6/9 (67%). Two patients required TIPS revision after initial clinical success. Mild-moderate HE occurred in 6/9 patients post TIPS (67%), with a highest West Haven score of 2. Time from TIPS to death for acute variceal bleeding and ascites was 4.9±4.2 and 12±16.5 months, respectively. Cause of death was disease progression (n=5), variceal bleeding (n=1), or unavailable (n=2). TIPS in the setting of malignant pseudocirrhosis can be created safely with similar clinical outcomes to TIPS performed for benign disease. Rates of low-grade hepatic encephalopathy may be higher amongst patients undergoing TIPS for pseudocirrhosis.

S2381 Evaluating Platelet Quantity in the Presence of Essential Thrombocythaemia With Cirrhosis

INTRODUCTION: Thrombocytopenia is normally a sequela of decompensated cirrhosis. Essential thrombocythaemia (ET), by contrast, is a myeloproliferative disorder involving an elevated platelet count that can manifest as thromboses of microvasculature. The rare concurrence of these two conditions is challenging for providers in that a normal platelet count may not be a proper indication of an adequate synthetic liver function, as a baseline value is unknown. CASE DESCRIPTION/METHODS: 66-year-old female with a history of ET and untreated chronic hepatitis B and C viral infections, who presented with intermittent left upper quadrant pain. On physical exam, the patient’s vital signs were within normal limits, and abdomen was non-tender on palpation. A complete metabolic panel was significant for elevated aminotransferases, with AST 78, ALT 78, and an alkaline phosphatase of 164. Additionally, a complete blood count was significant for thrombocytosis to 879 and hemoglobin of 10.6. Magnetic resonance imaging of the abdomen showed evidence of cirrhosis with sequelae of portal hypertension, including splenomegaly and upper abdominal varices, which were also seen on computed tomography of abdomen. She was diagnosed with new-onset cirrhosis secondary to chronic untreated viral hepatitis. Thrombopoietin (TPO) levels were not assessed as platelet count was markedly elevated. The patient was continued on her home hydroxyurea for continued management of ET. DISCUSSION: Platelet count is an important measure for patients with cirrhosis. Calculation like the AST to Platelet Ratio Index (APRI) score is used to predict disease severity. Assessing platelet production as an indicator of synthetic function in cirrhosis becomes difficult when treating patients with known ET. Prior cases have suggested that the thrombocytosis in ET may be attributed to an abnormality of the normal feedback inhibition responsible for platelet turnover. TPO levels are generally normal in ET and are notably reduced in decompensated cirrhosis. Providers, therefore, should consider direct measurement of TPO levels in patients with normal platelet counts who are diagnosed with ET and cirrhosis in order to assess whether synthetic liver function is impacted by liver disease. Furthermore, patients with cirrhosis have an increased risk of portal vein thrombosis (PVT); given that patients with both cirrhosis and ET may have an even higher risk of PVT, providers may consider prophylactically anticoagulating these patients to prevent such a complication.

Portal Venous Interventions after Liver Transplant

AbstractLiver transplant (LT) recipients are a challenging patient population when presenting with graft dysfunction or sequelae of portal hypertension. When no medical causes for graft dysfunction are found, a hemodynamically significant vascular lesion is suspected. Although rare, a variety of diseases may arise involving the portal system, which inhibit flow and graft function. Herein, we present a review of portal venous interventions performed in the LT setting, with focus on imaging diagnosis, endovascular treatment techniques, and outcomes. Entities addressed include portal venous stenosis, portal venous thrombosis, and recurrent fibrosis/cirrhosis with sequelae of portal hypertension.

The role of surgical shunts in the treatment of pediatric portal hypertension

BackgroundPortal diversion by surgical shunt plays a major role in the treatment of medically refractory portal hypertension. We evaluate our center’s experience with surgical shunts for the treatment of pediatric portal hypertension. MethodsAll patients who underwent surgical shunt at a single institution from 2008 to 2017 were reviewed. The primary outcome was intervention-free shunt patency. ResultsIn this study, 34 pediatric patients underwent portal shunt creation. The median age was 7.7 years (interquartile range 4.3–12.0). Twenty-nine patients (85%) had prehepatic portal hypertension and 5 patients (15%) had intrahepatic portal hypertension. The primary manifestations of portal hypertension were esophageal varices (97%) and gastrointestinal bleeding (77%). Eighteen patients (53%) underwent meso-Rex bypass, 10 patients (29%) underwent splenorenal shunt, and 6 patients (18%) underwent mesocaval shunt. Outcomes were notable for minimal wound complications (9%), rebleeding events (12%), and mortality (3%). In the postoperative setting, 10 patients (29%) experienced a shunt complication (occlusion or stenosis), 4 of which occurred in the early postoperative period and required urgent intervention. The 1-year and 5-year “primary patency” patency rates were 71% and 66%, respectively. ConclusionChildren suffer significant morbidity from the sequelae of portal hypertension. Our experience reinforces the feasibility of surgical shunts as an effective treatment option associated with low rates of morbidity and mortality.

Intravascular Ultrasound in the Creation of Transjugular Intrahepatic Portosystemic Shunts: Review of the Literature and Future Directions

Transjugular intrahepatic portosystemic shunt (TIPS) placement is a well-established interventional radiology procedure for treating sequelae of portal hypertension involving shunting portal blood flow to the hepatic vein bypassing the liver through a stent graft. Creating a TIPS can be a complex and radiation-intensive procedure due to the technically challenging step of cannulating the portal vein from the hepatic vein. As this is the most unpredictable step in TIPS creation, multiple techniques have been described for visualizing the portal vein to facilitate its access. The use of intravascular ultrasound (IVUS) has gained significant interest over the last few years due to reports of improved procedural metrics such as shortened procedural time and radiation dose. The purpose of this article is to review the literature and detail technical advances utilizing IVUS in TIPS creation. Intravascular ultrasound has been shown to be a safe and reproducible means of real-time image guidance for TIPS creation. The use of IVUS has been associated with improved procedural metrics including a decreased number of intrahepatic needle passes, shorter portal vein access times, decreased contrast usage, and a decrease in radiation dose. Due to technical advances and real-time portal vein visualization using IVUS, TIPS creation at our institution has dramatically changed. Our institutional practice has nearly completed shifted to the sole use of IVUS for portal vein access in TIPS creation due to significantly improved procedural metrics including radiation dose and contrast usage.

Transjugular intrahepatic portosystemic shunt as a bridge to liver transplant: Current state and future directions.

Liver transplantation is one of the mainstays of treatment for liver failure due to severe chronic liver disease. Bridging therapies, such as placement of a transjugular intrahepatic portosystemic shunt (TIPS), are frequently employed to control complications of portal hypertension such as ascites, hydrothorax, and variceal bleeding, and thereby reduce morbidity in patients awaiting transplant. There is no significant difference seen in either graft survival or patient survival between those receiving TIPS pre-transplant and those who do not, although those receiving TIPS placement on average have a longer waiting time on the transplant waitlist. Locoregional therapies, such as thermal ablation or chemoembolization, can be efficacious in patients with HCC and pre-existing TIPS; however there is a risk for increased adverse events in patients receiving these therapies who have TIPS compared to those who do not. In summary, TIPS is a safe, effective treatment that can be used to ameliorate the complications that are sequelae of portal hypertension. While it does not appear to improve survival post-transplant, TIPS placement pre-transplant may increase survival time to transplant, thus improving overall survival as well as quality of life.

Acute Portal Vein Thrombosis

AbstractAcute portal vein thrombosis (PVT) is characterized by thrombus formation within the portal vein or its branches. This thrombus formation leads to a nonspecific clinical presentation, which emphasizes the need for imaging to establish the diagnosis. Prompt diagnosis is vital, because if left untreated, acute PVT can lead to bowel infarction or the clinical sequelae of portal hypertension. Currently, guidelines recommend anticoagulation as first line therapy for patients with acute PVT with endovascular management reserved for those who fail anticoagulation or present with signs of bowel ischemia. However, many patients treated with anticoagulation will develop portal vein cavernoma and long term sequalea of portal hypertension, which suggests the need for earlier endovascular intervention in select patients. This article will discuss the risk factors for development of acute PVT, the clinical presentation and diagnosis of the disease, and the pharmacologic, endovascular, and surgical therapy options.

Factors affecting mortality and resource use for hospitalized patients with cirrhosis: A population-based study.

Hospitalizations for advanced liver disease are costly and associated with significant mortality. This population-based study aimed to evaluate factors associated with in-hospital mortality and resource use for the management of hospitalized patients with cirrhosis.Mortality records and resource utilization for 52,027 patients hospitalized with cirrhosis and/or complications of portal hypertension (ascites, hepatic encephalopathy, variceal bleeding, spontaneous bacterial peritonitis, or hepatorenal syndrome) were extracted from a nationally representative sample of Thai inpatients covered by Universal Coverage Scheme during 2009 to 2013.The rate of dying in the hospital increased steadily by 12% from 9.6% in 2009 to 10.8% in 2013 (P < .001). Complications of portal hypertension were independently associated with increased in-hospital mortality except for ascites. The highest independent risk for hospital death was seen with hepatorenal syndrome (odds ratio [OR], 5.04; 95% confidence interval [CI], 4.38–5.79). Mortality rate remained high in patients with infection, particularly septicemia (OR, 4.26; 95% CI, 4.0–4.54) and pneumonia (OR, 2.44; 95% CI, 2.18–2.73). Receiving upper endoscopy (OR, 0.29; 95% CI, 0.27–0.32) and paracentesis (OR, 0.93; 95% CI, 0.87–1.00) were associated with improved patient survival. The inflation-adjusted national annual costs (P = .06) and total hospital days (P = .07) for cirrhosis showed a trend toward increasing during the 5-year period. Renal dysfunction, infection, and sequelae of portal hypertension except for ascites were independently associated with increased resource utilization.Renal dysfunction, infection, and portal hypertension-related complications are the main factors affecting in-hospital mortality and resource utilization for hospitalized patients with cirrhosis. The early intervention for modifiable factors is an important step toward improving hospital outcomes.

Colonoscopy with polypectomy is associated with a low rate of complications in patients with cirrhosis.

Background and study aims: Cirrhotic patients are at a theoretically increased risk of bleeding. The safety of polypectomy in cirrhosis is poorly defined. Patients and methods: We performed a retrospective review of patients with cirrhosis who underwent colonoscopic polypectomy at a tertiary-care hospital. Patient characteristics and polyp data were collected. Development of complications including immediate bleeding, delayed bleeding, hospitalization, blood transfusion, perforation, and death were recorded to 30-day follow-up. Clinical characteristics between bleeders and non-bleeders were compared, and predictors of bleeding were determined. Results: A total of 307 colonoscopies with 638 polypectomies were identified. Immediate bleeding occurred in 7.5 % (95 % CI 4.6 % – 10.4 %) and delayed bleeding occurred in 0.3 % (95 % CI 0.0 % – 0.9 %) of colonoscopies. All cases of immediate bleeding were controlled endoscopically and none resulted in serious complication. The rate of hospitalization was 0.7 % (95 % CI 0.0 % – 1.6 %) and repeat colonoscopy 0.3 % (95 % CI 0.0 % – 0.9 %); no cases of perforation, blood transfusion, or death occurred. Lower platelet count, higher INR, presence of ascites, and presence of esophageal varices were associated with increased risk of bleeding. Use of electrocautery was associated with a lower risk of immediate bleeding. There was no significant difference between bleeding and non-bleeding polyps with regard to size, morphology, and histology. Conclusions: Colonoscopy with polypectomy appears safe in patients with cirrhosis. There is a low risk of major complications. The risk of immediate bleeding appears higher than an average risk population; however, most bleeding is self-limited or can be controlled endoscopically. Bleeding tends to occur with more advanced liver disease. Both the sequelae of portal hypertension and coagulation abnormalities are predictive of bleeding.

PWE-046 Comparison of Transient Elastography, Thrombocytopenia and Splenomegaly in Risk Assessment for The Presence of Oesophageal Varices: Abstract PWE-046 Table 1

Introduction The usage of transient elastography (TE) as a predictor of oesophageal varices has been widely studied. The formation of varices is the sequelae of portal hypertension (PH) which also manifest as splenomegaly leading to thrombocytopenia. We aim to establish the utility of TE in conjunction with PH in predicting varices. Methods Patients who underwent TE with the FibroScan® between June 2010 and January 2016 in our trust were identified. Only patients with a liver stiffness measurement (LSM) of >11.5 kPa which is indicative of cirrhosis and had undergone an endoscopy were included. Thrombocytopenia was defined as a platelet count of 25 kPa has been regarded as the optimum cut-off value for predicting varices and is used in this study. Results 2,643 patients underwent TE during the time period. 372 patients had a LSM of >11.5 kPa and of these patients, 157 patients underwent a gastroscopy. 41 patients (26.1%) had evidence of varices on gastroscopy. The sensitivity and specificity of TE in various combinations of PH in predicting varices are outlined in the table below. In total 46 out of our 157 patients with cirrhosis had TE Conclusion In our cohort of patients, a cut-off value of >25 kPa on TE is a better predictor of varices (sensitivity 83%) than thrombocytopenia or splenomegaly. Assessment of LSM using TE in conjunction with thrombocytopenia and splenomegaly improves sensitivity further to 97%. Limiting screening endoscopy in patients with liver cirrhosis to those with any of those 3 criteria: TE > 25 kPA, thrombocytopenia or splenomegaly, may reduce demand for endoscopy in that group of patients by 30%. Disclosure of Interest None Declared

Su1516 Nationwide Increase in Hospitalization and Mortality of Cirrhotic Patients With Sequelae of Portal Hypertension in Thailand: A Population-Based Study

Su1516 Nationwide Increase in Hospitalization and Mortality of Cirrhotic Patients With Sequelae of Portal Hypertension in Thailand: A Population-Based Study

Hepatocellular Carcinoma: therapeutic options 2015

The incidence of hepatocellular carcinoma (HCC), a common neoplasm, is rising and the prognosis is poor. Many factors have to be taken into account when deciding on the best mode of therapy, like tumor size and number, liver function, sequelae of portal hypertension or other comorbidities. These factors are reflected in the Barcelona Clinic Liver Cancer (BCLC) classification. Resection, radiofrequency ablation (RFA) and liver transplantation can be seen as curative therapies for the early and localized HCC. For the intermediate state of the HCC, there are other therapeutic modalities in therapy available: transarterial chemoembolization (TACE), selective internal radiation therapy (SIRT, rarer occasions), off label: stereotactic body radiation therapy (SBRT). At the moment, Sorafenib is the only option in treating advanced stages of HCC. Alternative treatment strategies, like e.g. immunological therapies, are being investigated.

Vascular Artifact Mimicking Thrombosis on MR Imaging Using Ferumoxytol as a Contrast Agent in Abdominal Vascular Assessment

PurposeTo describe an artifact that mimics thrombosis when assessing abdominal vasculature on magnetic resonance (MR) imaging using ferumoxytol in patients with contraindications to gadolinium-based contrast agents and to evaluate factors that may contribute to this artifact. Materials and MethodsThree radiologists in consensus retrospectively evaluated 61 abdominal MR imaging examinations using ferumoxytol as an intravenous contrast agent for the presence of an observed artifact that can mimic thrombosis. Patient demographics and contrast agent bolus concentrations were compared with an unpaired Wilcoxon signed rank test. ResultsAn artifact mimicking thrombosis was observed in 30 of 61 examinations, all on the arterial phase sequences. In examinations with this artifact, the average concentration of administered ferumoxytol was greater than in examinations where the artifact was not observed (P < .01). Several additional vascular findings were observed, including portal vein thrombosis (n = 2) and aneurysm (n = 1), renal vein thrombosis (n = 2), abdominal aortic aneurysm (n = 1), abdominal and iliac artery dissection (n = 3), and sequelae of portal hypertension (n = 8). ConclusionsAlthough MR imaging using ferumoxytol as an intravenous contrast agent can be useful in detecting abdominal vascular abnormalities, an artifact mimicking vascular thrombosis was observed in nearly half of the examinations.

Clinical manifestations of autosomal recessive polycystic kidney disease (ARPKD): kidney-related and non-kidney-related phenotypes

Autosomal recessive polycystic kidney disease (ARPKD), although less frequent than the dominant form, is a common, inherited ciliopathy of childhood that is caused by mutations in the PKHD1-gene on chromosome 6. The characteristic dilatation of the renal collecting ducts starts in utero and can present at any stage from infancy to adulthood. Renal insufficiency may already begin in utero and may lead to early abortion or oligohydramnios and lung hypoplasia in the newborn. However, there are also affected children who have no evidence of renal dysfunction in utero and who are born with normal renal function. Up to 30 % of patients die in the perinatal period, and those surviving the neonatal period reach end stage renal disease (ESRD) in infancy, early childhood or adolescence. In contrast, some affected patients have been diagnosed as adults with renal function ranging from normal to moderate renal insufficiency to ESRD. The clinical spectrum of ARPKD is broader than previously recognized. While bilateral renal enlargement with microcystic dilatation is the predominant clinical feature, arterial hypertension, intrahepatic biliary dysgenesis remain important manifestations that affect approximately 45 % of infants. All patients with ARPKD develop clinical findings of congenital hepatic fibrosis (CHF); however, non-obstructive dilation of the intrahepatic bile ducts in the liver (Caroli's disease) is seen at the histological level in only a subset of patients. Cholangitis and variceal bleeding, sequelae of portal hypertension, are life-threatening complications that may occur more often in advanced cases of liver disease. In this review we focus on common and uncommon kidney-related and non-kidney-related phenotypes. Clinical management of ARPKD patients should include consideration of potential problems related to these manifestations.

Congenital Hepatic Fibrosis and Autosomal Recessive Polycystic Kidney Disease

The published natural history of congenital hepatic fibrosis (CHF) was examined to inform clinical decision making in autosomal recessive polycystic kidney disease (ARPKD). A systematic literature search of the data on CHF, ARPKD, Caroli disease, Caroli syndrome, and type V choledochal cyst was performed to extract data related to portal hypertension, infection, malignancy, mortality, and transplantation. Information related to 1230 patients with CHF was extracted from 155 articles. Median and mean age at diagnosis were 2 and 11.2 years, respectively. Median and mean time followed after diagnosis were 5.0 and 7.5 years, respectively (range 0-38 years). Sequelae of portal hypertension (n = 409), cholangitis (n = 152), and malignancy (n = 21) were noted. The nature of the portal hypertension was similar to that in other pediatric conditions (164 with varices, 74 bleeding varices, 81 underwent portosystemic shunting). Documented cholangitis was fatal in 3 of 23 children who were infected after renal transplantation. Twenty-one patients developed hepatobiliary cancer, with the majority having cholangiocarcinoma (n = 19). Cholangiocarcinoma (CCA) was predominant in individuals older than 40 years with either Caroli syndrome or isolated CHF, not ARPKD (median and mean age at CCA diagnosis were 70.3 and 60.1 years, respectively; range 33-75 years). There was a relative paucity of data on combined liver-kidney transplantation. Clinical decision making in ARPKD should reflect an understanding of the potential issues emanating from CHF. Accepted pediatric specific approaches to portal hypertension are warranted but must take into consideration the stage of renal insufficiency and potential plans for renal transplantation. Cholangitis is a major issue and necessitates anticipatory guidance and awareness. CCA, although a dreaded complication, does not appear to be a major issue during childhood. The indications for liver and combined liver-kidney transplantation are controversial and warrant further analysis.

Hepatocellular Carcinoma Masquerading As A Bleeding Gastric Ulcer

Purpose: To report an atypical presentation of hepatocellular carcinoma (HCC), mimicking a bleeding stomach ulcer, controlled by transcatheter arterial embolization (TAE). Clinical vignette: 72 year old African American male with a history of alcohol abuse presents with hematemesis and anemia. Endoscopy revealed an extrinsically compressed stomach with outlet obstruction and a small bleeding ulcer with a protruding central lesion (Figure 1). Endoscopic hemostasis was unsuccessful. Angiography was performed revealing prominent neoplastic vascularity fed by the left gastric artery (LGA), at the site of the obstructed gastric air column, corresponding to the endoscopic site of bleeding (Figure 2). TAE of the LGA was performed achieving hemostasis. A dynamic computed tomography of the abdomen with intravenous contrast showed a large 16 cm mass in the left dome of the liver that was fused to the stomach. Alfa fetoprotein level was 227,308 ng/ml, thus establishing the diagnosis of HCC. No recurrence of bleeding occurred during the hospital stay and the hemoglobin remained stable. Discussion: HCC is the twelfth most common cancer in the United States. Bleeding from the upper gastrointestinal tract in patients with HCC can occur as a sequelae of portal hypertension. Locoregional invasion of the stomach leading to upper gastrointestinal hemorrhage has been rarely reported in the literature, with diagnosis and hemostasis achieved by surgical approach (Table 1). Our case illustrates the efficacy of TAE in controlling HCC tumor bleeding resulting from gastric wall invasion. This method should be attempted as first line approach given the high surgical risk and the poor prognosis associated with locally advanced HCC.FigureFigure

Human extrahepatic portal vein obstruction correlates with decreased factor VII and protein C transcription but increased hepatocyte proliferation

Purpose A 3-year-old girl developed extrahepatic portal vein obstruction (EHPVO) after a liver transplant. She had sequelae of portal hypertension that required another transplantation. The circumstances allowed for comparison of liver-dependent coagulation factor production between the second donor liver and the explanted liver with EHPVO. Methods Liver samples from the explanted first graft and the second transplant were obtained. Fresh tissue was used to perform reverse transcription–polymerase chain reaction with primers against factors V, VII, as well as VIII, protein C, and paraffin-embedded sections for hepatocyte proliferation using Ki-67 antibody as well as for apoptosis using TUNEL assay. Results The transcription of factor VII and that of protein C were decreased in the explant as compared with the newly transplanted liver (factor VII, 77% of the donor; protein C, 88% of the donor). The transcription of factor V and that of factor VIII were unchanged. The explant had a greater percentage of proliferating hepatocytes than the new organ (0.85% ± 0.75% vs 0.11% ± 0.21%). The percentage of apoptotic cells was similar between the 2 livers (0.09% ± 0.13% vs 0.09% ± 0.13%). Conclusions Idiopathic EHPVO is associated with a reduction in liver-dependent coagulation factor transcription and an increase in hepatocyte proliferation. Portal blood flow deprivation alters hepatic homeostasis and initiates mechanisms that attempt to restore liver-dependent coagulation factors.

Nationwide Increase in Hospitalizations and Hepatitis C Among Inpatients With Cirrhosis and Sequelae of Portal Hypertension

Advanced liver disease and complications of portal hypertension are common indications for hospitalization. Our objectives were to characterize longitudinal trends in incidence, characteristics, and outcomes of patients hospitalized with complications of portal hypertension using a nationally representative data set. Admissions for complications of portal hypertension (hepatic encephalopathy, ascites, or variceal bleed) were identified from the Nationwide Inpatient Sample between 1998 and 2003 using International Classification of Diseases, 9th Revision, Clinical Modification diagnosis codes. International Classification of Diseases, 9th Revision, Clinical Modification procedural codes were used to identify liver transplantation and portosystemic shunt procedures. National estimates for incidence of hospitalization over time, in-hospital mortality, and hospital charges accounted for survey design. Hospitalization rates increased significantly by 5% annually between 1998 and 2003, particularly in the Northeast and the South. The prevalence of hepatitis C-related advanced liver disease among these hospitalized patients increased from 12.9% to 23.7%, and in those with HCV and concurrent alcohol-related disease the rate increased from 5.6% to 11.2%. US population-based in-hospital mortality increased modestly from 1.9 to 2.1 per 100,000 (P<.001), with hepatorenal syndrome as the strongest predictor of death (odds ratio, 9.5; 95% confidence interval, 8.4-10.7). The inflation-adjusted total economic burden of decompensated cirrhosis increased from $1.15 billion to $2.1 billion during the 6-year period (P<.003). A significant increase in the incidence of hospitalization for complicated portal hypertension between 1998 and 2003 and a growing burden of hepatitis C-related disease have profound economic impact and underscore the need for interventions to prevent progression to advanced liver disease.

Treatment of Pediatric Cholestatic Liver Disease.

Cholestatic liver disease affects a small percentage of children, but therapy results in millions of healthcare dollars being spent each year. Close monitoring of nutritional status, pruritus, and complications from portal hypertension should improve the patient's quality of life and survival without liver transplantation. Other comorbid conditions, such as cardiac or renal disease, must also be integrated into the care plan and will affect the overall prognosis of the patient. Portal hypertension leads to ascites, variceal hemorrhage, and infection, which can result in significant mortality if not promptly recognized and treated. Surgical shunts are being used less because the expertise to perform endoscopic sclerotherapy and endoscopic band ligation is available at most medical centers. However, many cholestatic diseases, including biliary atresia, progressive familial intrahepatic cholestasis (PFIC) I, II, and III, and Alagille syndrome, may still require liver transplantation (Table 1). The cost of this procedure can exceed several hundred thousand dollars per patient, without including the annual costs of immunosuppressant therapy and medical monitoring. Meticulous medical management of nutrition and the sequelae of portal hypertension may prolong survival and reduce the potential morbidity and mortality of liver transplantation.