Portal Hypertension In Adults Research Articles

TIPS and Interventional Radiology Management of Portal Hypertensive Complications in Pediatric Population

AbstractTransjugular intrahepatic portosystemic shunt (TIPS) placement is a well-established procedure for treating the sequelae of portal hypertension in adults. In pediatric patients also, TIPS is a good alternative to surgical shunt procedures because it is less invasive. However, similar or even more than in adults, TIPS creation is one of the technically more challenging procedures in children requiring special considerations regarding portal venous access and stent placement. Although data are limited to retrospective single-center experiences and meta-analysis, current findings suggest that TIPS is safe and effective in pediatric patients. If monitored and revised carefully, TIPS stents can last for a long time and may be able to serve for more than a short-term bridge to liver transplantation. This review shall provide information about the authors' procedural technique and give a short overview about the recent literature.

Liver reportalization in extrahepatic portal hypertension

The study involved literature review of history and current state of surgical treatment in extrahepatic portal hypertension in children and adults. The author analyzed Russian and foreign publications on surgical repair of hepatopetal blood flow in extrahepatic portal hypertension. The issue is a high priority in Russia, which is confirmed by the fact that the author found out the pioneer studies of Russian authors, which were published earlier than the works of foreign researchers. It was found that the role of hepatopetal blood flow restoration in extrahepatic portal hypertension in adults had been much less studied than in children. More clinical material is to be gathered to determine the feasibility of such interventions in adults.

Portal Hypertension in Adults- A Comprehensive Review

Portal Hypertension in Adults- A Comprehensive Review

Primary extrahepatic portal vein obstruction in adults: A single center experience

Extrahepatic portal venous obstruction (EHPVO) is a heterogenous disease with regards to etiology, pathogenesis, age, and geographical location. This study analyzed our experience with EHPVO in adults aged >20 years. EHPVO associated with pancreatitis and abdominal lymph node tuberculosis compressing the portal vein was excluded. Retrospective analysis of the hospital database from January 2000 to December 2009 was done. All patients with liver disease who are attending our department were also prospectively evaluated with Doppler ultrasound for the portal venous system to study the prevalence of EHPVO. Clinical, biochemical, and imaging findings; work up for thrombophilia; treatment given; and follow up were evaluated. In the retrospective analysis, primary EHPVO in adults was seen in 108/10,095 (1 %), and in the prospective analysis, it was seen in 16/2,188 (0.73 %). The main clinical presentations were abdominal pain and variceal bleed. Imaging findings included portal cavernoma, portal biliopathy, gallbladder calculi, collaterals, and ascites. The major causative factors identified were hyperhomocysteinemia, antiphospholipid antibodies, and myeloproliferative disorders, while, in a third of patients, none of these risk factors could be identified. Twelve patients were subjected to surgery, while the remaining patients were managed either by medical, endoscopic, or interventional radiological techniques. More than 2 years of follow up was available in 90 patients; two patients died due to uncontrolled bleeding, seven patients required surgery, seven patients showed deterioration in liver function, and one patient developed hepatocellular carcinoma. In the prospective study, three patients were subjected to surgery, and the others were managed medically. Primary EHPVO is an uncommon cause of portal hypertension in adults in India, and its etiological spectrum is comparable to the West.

TIMP-1/-2 and transient elastography allow non invasive diagnosis of cystic fibrosis associated liver disease

BackgroundCystic fibrosis (CF) associated liver disease develops in approximately 30% of CF patients. However, routine sensitive diagnostic tools are lacking. AimsWe aimed to compare the value of transient elastography and experimental fibrosis markers for the detection of liver disease in CF patients. Methods145 CF patients (75 children, 70 adults) were prospectively studied and received transient elastography. CF liver disease was diagnosed according to recent guidelines. Serum concentrations of YKL-40, HA, PIIIP, MMP-9, TIMP-1 and TIMP-2 were determined by ELISA. ResultsTransient elastography was increased in adults and children with CF hepatopathy compared to those without and exhibited a high diagnostic accuracy for CF liver disease. In adults with portal hypertension, elastography was further enhanced. TIMP-2 was elevated in adults with CF hepatopathy associated portal hypertension and exhibited a high diagnostic accuracy for portal hypertension in adults and for CF hepatopathy in children. TIMP-1 had a high diagnostic accuracy for CF hepatopathy in adults. Diagnostic sensitivities were increased when elastography and respective biomarkers were combined for the detection of CF hepatopathy and portal hypertension. ConclusionsTIMP-1 and TIMP-2 represent powerful biomarkers for CF associated liver disease and portal hypertension. Their determination may confirm and improve the diagnostic accuracy of transient elastography.

TIPS stent migration into the heart with 6-year follow-up

The transjugular intrahepatic portosystemic shunt (TIPS) is widely used for the treatment of portal hypertension in adults, but no studies have defined the best approach to treat portal hypertension in pediatric patients. Pediatric use of TIPS is rare even in large centers of adult practice. The migration of stents has also been reported as a complication in adults. There is no standard way to treat this type of complication, and it is not always clear whether immediate removal or watchful waiting is safer for the patient. We report the case of an 11-year-old patient who underwent urgent TIPS implantation due to variceal bleeding, after unsuccessful sclerotherapy. During the procedure, due to the deep impact of the stent, a second, telescopic, stent was inserted. The portal pressure decreased, no further bleeding occurred, and the patient was listed for transplantation. Three weeks later a routine chest X-ray discovered the migration of the second stent into the right ventricle. No interventional radiological removal or open heart surgery was available for the transplant waiting list patient. The patient underwent uneventful combined liver-kidney transplantation. During the 6-year follow-up period the child had no signs of hemodynamic instability, and his somatic and mental development were appropriate. To our knowledge this case is the first publication on a heart-impacted TIPS stent in a child. The watchful waiting was justified by uneventful combined liver-kidney transplantation and long-term follow-up. This case also underlines the need for best practice guidelines in pediatric portal hypertension.

Multidetector CT findings of splenic artery aneurysm in children with chronic liver disease

Splenic artery aneurysm (SAA) is a well-known complication of chronic liver disease and portal hypertension in adults. The incidence of SAA in children undergoing selective hepatic angiography prior to liver transplantation is reported as 4%, but there are few systematic studies. To investigate the SAAs detected by multidetector CT angiography (MDCTA) among children with chronic liver disease. A total of 124 children (71 girls, 53 boys; mean age 118 months; age range 5 days to 204 months) with chronic liver disease underwent MDCTA to display the vascular anatomy and any vascular complications during the pretransplantation period. Of these children, 23 also underwent coeliac angiography. The digital subtraction angiography (DSA) and MDCTA findings were compared. SAAs were detected in 13 children (10.4%); none was detectable by US. All patients had more than one aneurysm; ten patients had more than three. In all except one patient, the SAAs were located only in the intraparenchymal branches of the splenic artery; in one patient they were located in the intraparenchymal segment and in the distal third of the splenic artery. The mean size of the aneurysms was 6.5 mm (range 2.5-18 mm). All patients with aneurysms had splenomegaly and vascular collaterals. Nine of the children with SAAs had portal vein pathologies (two occlusions, two stenoses, five dilatations). A statistically significant difference existed with regard to the size of spleen (P < 0.05) and patient age (P < 0.05) between children with SAAs and children without SAAs. There was an increased risk of SAAs in patients with portal vein pathologies. In 19 patients without SAAs on MDCTA, no SAAs were seen on DSA. It is likely that the incidence of SAA in children with chronic liver disease will increase with improved survival of children with long-standing portal hypertension and chronic liver disease. MDCTA with multiplanar reconstruction is a noninvasive and effective means of imaging paediatric patients with SAAs, especially during the peritransplantation period, which is considered to be a time of significant risk for SAA rupture in this patient population.

Erratum

Alimentary Pharmacology & TherapeuticsVolume 27, Issue 6 p. 528-529 Free Access Erratum This article corrects the following: Review article: the management of non-cirrhotic non-malignant portal vein thrombosis and concurrent portal hypertension in adults V. M. C. W. SPAANDER, H. R. VAN BUUREN, H. L. A. JANSSEN, Volume 26Issue s2Alimentary Pharmacology & Therapeutics pages: 203-209 First Published online: December 7, 2007 First published: 20 February 2008 https://doi.org/10.1111/j.1365-2036.2008.03608_4.xAboutSectionsPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat In Ref.4, the data in Table 2 was published incorrectly. The corrected Table 2 is printed below. Table 2. Treatment recommendations of EPVT21 Treatment Indication Thrombotic obstruction Anticoagulants Acute EPVT Chronic EPVT if a prothrombotic disorder is present Thrombolysis and thrombectomy Not recommended (high complication rate) Variceal bleeding Endoscopic treatment Active variceal bleeding Secondary prophylaxis for rebleeding Vasoactive agents Can be considered in patients with active variceal bleeding β-Adrenergic blockade Not yet recommended (insufficient evidence) TIPS If endoscopic treatment fails and if technically applicable (little evidence) Surgical shunt If endoscopic treatment fails Portal biliopathy Sphincterotomy, balloon dilatation, endoprosthesis Jaundice/cholangitis/biliary stenosis/bile duct stones Portosystemic shunt Persistent biliary obstruction despite endoscopic treatment Hepaticojejunostomy Persistent biliary obstruction despite a portosystemic shunt EPVT, extrahepatic portal vein thrombosis; TIPS, transjugular intrahepatic portosystemic shunt. We apologize for this error. References 1 Irving PM, Gearry RB, Sparrow MP, Gibson PR. Review article: appropriate use of corticosteroids in Crohn’s disease. Aliment Pharmacol Ther 2007; 26: 313– 29. 2 Campisi G, Di Liberto C, Iacono G, et al. Oral pathology in untreated coelic disease. Aliment Pharmacol Ther 2007; 26: 1529– 36. 3 Manolakopoulos S, Bethanis S, Koutsounas S, et al. Long-term therapy with adefovir dipivoxil in hepatitis B e antigen-negative patients developing resistance to lamivudine. Aliment Pharmacol Ther 2008; 27: 266– 73. 4 Spaander VM, Van Buuren HR, Janssen HL. Review article: the management of non-cirrhotic non-malignant portal vein thrombosis and concurrent portal hypertension in adults. Aliment Pharmacol Ther 2007; 26 (Suppl. 2): 203– 9. Volume27, Issue6March 2008Pages 528-529 ReferencesRelatedInformation

Corrigenda

Alimentary Pharmacology & TherapeuticsVolume 27, Issue 6 p. 528-529 Free Access Corrigenda This article corrects the following: Review article: appropriate use of corticosteroids in Crohn’s disease P. M. IRVING, R. B. GEARRY, M. P. SPARROW, P. R. GIBSON, Volume 26Issue 3Alimentary Pharmacology & Therapeutics pages: 313-329 First Published online: May 25, 2007 First published: 20 February 2008 https://doi.org/10.1111/j.1365-2036.2008.03608_1.xAboutSectionsPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat In Ref.1, Figure 1 contained an error in which Finland was shown in yellow colour (availability of infliximab, 0–25%), where it should have been shown in blue colour (availability of infliximab, 76–100%). The corrected Figure 1 is printed below. Figure 1Open in figure viewerPowerPoint Gastroenterologists’ perceptions of availability of infliximab in their own countries. As the information was not made available by the manufacturer or distributor of infliximab, gastroenterologists around the world were contacted and asked to estimate what proportion of their country’s population had access to infliximab to induce remission for active Crohn’s disease. African gastroenterologists provided data for more than one country. References 1 Irving PM, Gearry RB, Sparrow MP, Gibson PR. Review article: appropriate use of corticosteroids in Crohn’s disease. Aliment Pharmacol Ther 2007; 26: 313– 29. 2 Campisi G, Di Liberto C, Iacono G, et al. Oral pathology in untreated coelic disease. Aliment Pharmacol Ther 2007; 26: 1529– 36. 3 Manolakopoulos S, Bethanis S, Koutsounas S, et al. Long-term therapy with adefovir dipivoxil in hepatitis B e antigen-negative patients developing resistance to lamivudine. Aliment Pharmacol Ther 2008; 27: 266– 73. 4 Spaander VM, Van Buuren HR, Janssen HL. Review article: the management of non-cirrhotic non-malignant portal vein thrombosis and concurrent portal hypertension in adults. Aliment Pharmacol Ther 2007; 26 (Suppl. 2): 203– 9. Volume27, Issue6March 2008Pages 528-529 FiguresReferencesRelatedInformation

Corrigenda

Alimentary Pharmacology & TherapeuticsVolume 27, Issue 6 p. 528-529 Free Access Corrigenda This article corrects the following: Review article: appropriate use of corticosteroids in Crohn’s disease P. M. IRVING, R. B. GEARRY, M. P. SPARROW, P. R. GIBSON, Volume 26Issue 3Alimentary Pharmacology & Therapeutics pages: 313-329 First Published online: May 25, 2007 First published: 20 February 2008 https://doi.org/10.1111/j.1365-2036.2008.03608_1.xAboutSectionsPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat In Ref.1, Figure 1 contained an error in which Finland was shown in yellow colour (availability of infliximab, 0–25%), where it should have been shown in blue colour (availability of infliximab, 76–100%). The corrected Figure 1 is printed below. Figure 1Open in figure viewerPowerPoint Gastroenterologists’ perceptions of availability of infliximab in their own countries. As the information was not made available by the manufacturer or distributor of infliximab, gastroenterologists around the world were contacted and asked to estimate what proportion of their country’s population had access to infliximab to induce remission for active Crohn’s disease. African gastroenterologists provided data for more than one country. References 1 Irving PM, Gearry RB, Sparrow MP, Gibson PR. Review article: appropriate use of corticosteroids in Crohn’s disease. Aliment Pharmacol Ther 2007; 26: 313– 29. 2 Campisi G, Di Liberto C, Iacono G, et al. Oral pathology in untreated coelic disease. Aliment Pharmacol Ther 2007; 26: 1529– 36. 3 Manolakopoulos S, Bethanis S, Koutsounas S, et al. Long-term therapy with adefovir dipivoxil in hepatitis B e antigen-negative patients developing resistance to lamivudine. Aliment Pharmacol Ther 2008; 27: 266– 73. 4 Spaander VM, Van Buuren HR, Janssen HL. Review article: the management of non-cirrhotic non-malignant portal vein thrombosis and concurrent portal hypertension in adults. Aliment Pharmacol Ther 2007; 26 (Suppl. 2): 203– 9. Volume27, Issue6March 2008Pages 528-529 FiguresReferencesRelatedInformation

Review article: the management of non‐cirrhotic non‐malignant portal vein thrombosis and concurrent portal hypertension in adults

Extrahepatic portal vein thrombosis is an important cause of non-cirrhotic portal hypertension. To provide an update on recent advances in the aetiology and management of acute and chronic non-cirrhotic non-malignant extrahepatic portal vein thrombosis. A PubMed search was performed to identify relevant literature using search terms including 'portal vein thrombosis', 'variceal bleeding' and 'portal biliopathy'. Myeloproliferative disease is the most common risk factor in patients with non-cirrhotic non-malignant extrahepatic portal vein thrombosis. Anticoagulation therapy for at least 3 months is indicated in patients with acute extrahepatic portal vein thrombosis. However, in patients with extrahepatic portal vein thrombosis due to a prothrombotic disorder, permanent anticoagulation therapy can be considered. The most important complication of extrahepatic portal vein thrombosis is oesophagogastric variceal bleeding. Endoscopic treatment is the first-line treatment for variceal bleeding. In several of the patients with extrahepatic portal vein thrombosis biliopathy changes on endoscopic retrograde cholangiography (ERCP) have been reported. Dependent on the persistence of the biliary obstruction, treatment can vary from ERCP to hepaticojejunostomy. Prothrombotic disorders are the major causes of non-cirrhotic, non-malignant extrahepatic portal vein thrombosis and anticoagulation therapy is warranted in these patients. The prognosis of patients with non-cirrhotic, non-malignant extrahepatic portal vein thrombosis is good, and is not determined by portal hypertension complications but mainly by the underlying cause of thrombosis.

Complications of portal hypertension in adults: a French consensus

Portal hypertension is responsible for different severe complications such as variceal bleeding, ascites and pulmonary disorders. Different pharmacological and endoscopic treatments have been proposed but some recommendations may differ. The conclusions of a French consensus conference on the prevention and treatments of the main complications of portal hypertension in adults are reported.

A man with type III glycogenosis associated with cirrhosis and portal hypertension

Type III glycogenosis, an inherited disorder of glycogen metabolism that results from reduced or absent activity of the enzyme amylo-1,6-glycosidase (debranching enzyme), has not been frequently associated with cirrhosis and portal hypertension in adults. An adult Caucasian man with well-documented type IIIa glycogenosis, who presented with a variceal hemorrhage secondary to hepatic cirrhosis, is described here. No other cause of cirrhosis was found.

Portasystemic shunting procedures for portal hypertension: Twenty-six year experience in adults with cirrhosis of the liver

1. 1. The results of the 404 cases of portal hypertension in adults with portal cirrhosis treated by portasystemic shunts are reviewed. 2. 2. The over-all operative mortality was 12 per cent. 3. 3. The five year survival rate was 58 per cent. 4. 4. Incidence of moderate and severe encephalopathy combined was 34 per cent in the follow-up period. 5. 5. Control of portasystemic encephalopathy is still imperfect but colon bypass offers hope when conservative therapy has failed. 6. 6. The incidence of shunt failure ranged between 5 and 50 per cent depending on the type of shunt performed. 7. 7. The most important factor in determining the clinical results of any shunting procedure is the underlying liver function status.

The potential hazard of pregnancy in extrahepatic portal hypertension. Report of two cases.

PORTAL hypertension may result from either intrahepatic or extrahepatic obstruction of the portal venous system. Intrahepatic narrowing of the portal venous radicles occurs in cirrhosis of the liver, and this disease accounts for the great majority of cases of portal hypertension in adults. Extrahepatic thrombosis or cavernous transformation of the portal vein is a less common cause of portal hypertension in adults, but is the cause in almost half of the patients who are children. This type of portal hypertension has been treated by various surgical procedures, the two most effective being portalsystemic shunt operations5or direct ligation of the varices.1,4Shunt operations decompress the portal venous system, while the direct ligation of varices occludes the dangerous area of collateral flow. We have had the unique opportunity at the Cleveland Clinic to follow the progress of 14 patients with extrahepatic portal hypertension who underwent transthoracic, transesophageal ligation of

Portal hypertension. Pathogenesis, management and prognosis.

Portal hypertension in adults is most frequently a result of nutritional or postnecrotic cirrhosis. Portal thrombosis is the most common cause in children. Portal-systemic venous shunts are the only means available to lower portal pressure permanently and to prevent variceal hemorrhage. Operative mortality and long-term survival are directly related to residual hepatic reserve. Criteria for assessment of hepatic reserve and experience with portacaval shunts are presented.