Current concepts and management sk sarin and d kapoor department of gastroenterology, gb pant hospital, new delhi, india abstract noncirrhotic portal hypertension ncph comprises diseases having an increase in portal pressure pp due to intraheptic or prehepatic lesions, in the absence of cirrhosis. Liver disease in cystic fibrosis presents as noncirrhotic. Pathology of idiopathic noncirrhotic portal hypertension. Splenomegaly with or without anaemia has drawn the attention of a number of workers since banti. Noncirrhotic portal hypertension and portal vein thrombosis. Alcoholic, metabolic, or autoimmune liver diseases can cause portal hypertension. Idiopathic noncirrhotic portal hypertension incph is a rare disease characterized of intrahepatic portal hypertension in the absence of. Histological features of incph comprise a wide spectrum of nonspecific features, ranging from minor changes, sinusoidal.
Noncirrhotic portal hypertension in pregnancy request pdf. Pdf noncirrhotic portal fibrosisidiopathic portal hypertension. Get a printable copy pdf file of the complete article 1. Noncirrhotic portal hypertension journal of hepatology. Some have suggested an unknown infectious agent or immunologic event that leads to portal venous system fibrosis. Non cirrhotic portal hypertension ncph comprises a heterogeneous group of liver disorders causing portal hypertension without cirrhosis and carries a high risk of variceal bleeding. Idiopathic noncirrhotic portal hypertension gastroenterology. Patients can be misdiagnosed as having liver cirrhosis, but awareness of this condition presence of portal hypertension with disproportionately. Imaging and pathological features of idiopathic portal. Idiopathic portal hypertension non cirrhotic portal hypertension or banti syndrome is a term that has been given to portal hypertension occurring without hepatic cirrhosis, parasitic infection, or portal venous thrombosis. She had a previous history of recurrent episodes of abdominal pain with haematemesis and melena. Multiple splenic artery aneurysms in noncirrhotic hepatic.
Pathology of noncirrhotic portal hypertension and incomplete. Noncirrhotic portal hypertension in hivinfected individuals. Natural history of noncirrhotic portal hypertension full. Portal biliopathy in patients with noncirrhotic portal. The etiology of incph can be classified in five categories. Noncirrhotic portal hypertension and incomplete septal. Current concepts and management, journal of gastroenterology and hepatology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. These features were highly suggestive of non cirrhotic portal fibrosis ncpf.
Ncph is a heterogeneous group of liver disorders of vascular origin, leading to pht with near normal hvpg. By definition, any disorder known to cause portal hypertension in the absence of cirrhosis and any cause of chronic liver disease must be excluded to make a diagnosis of idiopathic non cirrhotic. Natural history of non cirrhotic portal fibrosis a. Longterm outcome of pediatric noncirrhotic portal fibrosis. Non cirrhotic portal hypertension authorstream presentation. Non cirrhotic portal fibrosis ncpf presents with non cirrhotic portal hypertension characterized by moderate to severe splenomegaly, evidence of varices andor collaterals, patent splenoportal axis and hepatic veins, normal liver functions, normal or nearnormal hepatic venous pressure gradient hvpg, and no evidence of cirrhosis or parenchymal injury in the liver histology. Radiological diagnosis of hepatocellular carcinoma in non. Rajender reddy, md in the western world, cirrhosis related to chronic hepatitis c and b and alcoholic cirrhosis are the most common causes for portal hyperten sion. To study the outcome of pregnancy in women with non. Idiopathic noncirrhotic intrahepatic portal hypertension. F2, combined perisinusoidal and portal periportal fibrosis. Measurement and correlation of wedged hepatic, intrahepatic, intrasplenic and intravariceal pressures in patients with cirrhosis of liver and non cirrhotic portal fibrosis.
Essentially a form of presinusoidal portal hypertension, a mechanism for this non cirrhotic portal hypertension is unclear. The aim of the present study was to evaluate complications during pregnancy and pregnancy outcome in women with non cirrhotic portal fibrosis and portal hypertension. It has been commonly described from india and japan, and is. Visceral adiposity and insulin resistance are independent. Two diseases that present only with features of pht and are common in developing countries are non. Non cirrhotic portal fibrosis is inherited in an autosomal recessive manner non cirrhotic portal fibrosis ncpf is a chronic liver disease 1 and type of non cirrhotic portal hypertension ncph. To identify potential subjects for this study, a database search of electronic patient records was performed using key words noncirrhotic portal hypertension, ncph, sarcoid, schistosomiasis, nodular regenerative hyperplasia, nrh, congenital hepatic fibrosis, portal vein thrombosis and budd. Non cirrhotic portal fibrosis is a clinical entity of uncertain etiology. Pdf the asian pacific association for the study of the liver apasl working party on. Biliary lipid composition in patients with noncirrhotic portal fibrosisa comparison with compensated cirrhosis of the liver.
Non cirrhotic portal hypertension ncph is a common cause of variceal bleed in developing countries. Ct showed massive splenomegaly with grossly dilated and tortuous splenic vein thin arrow and attenuated main portal vein thick arrow and its intrahepatic branches. Natural history of non cirrhotic portal fibrosis a tropical. Anatomy and physiology the portal vein is formed by the union of the superior mesenteric vein and the splenic vein just posterior to the head of the pancreas at about the level of the second lumbar vertebra enters the liver at the porta hepatis in two main branches, one to each lobe. Non cirrhotic portal hypertension diagnosis and management. Pregnancy outcome was compared in extra hepatic portal vein obstruction ehpvo and non. Apr 12, 2018 idiopathic non cirrhotic portal hypertension is an underrecognized vascular liver disease of unknown etiology, characterized by clinical signs of portal hypertension in the absence of cirrhosis. Noncirrhotic portal fibrosis idiopathic portal hypertension. Noncirrhotic portal hypertension in pregnancy sciencedirect.
In the absence of a portal vein thrombosis, such low hvpg can only be explained by a presinusoidal type of portal hypertension 3 x 3 khanna, r. Noninvasive predictors of highrisk varices in patients with. Transient elastography te using fibroscan is a useful technique for evaluation of fibrosis in patients with liver disease. Normal portal tract up to 30% of small portal tracts may lack portal vein branches. Also, in the chronic arsenic exposure model, there is increased hydroxyproline and collagen without significant hepatic fibrosis 26. Various factors have been implicated in the causation of the disease. It may arise as a result of either primary hepatic disease or the effects of systemic disorders on liver. Pdf noncirrhotic portal hypertension enrique molina. Incomplete septal cirrhosis and nodular regenerative hyperplasia have recently been shown to be part of the spectrum of the disease. The two most important causes of noncirrhotic portal hypertension are noncirrhotic portal fibrosis ncpf and extrahepatic portal venous obstruction ehpvo. Coagulation disorders commonly develop in patients with cirrhosis of the liver. We previously suggested that cfldrelated pht could be a.
Noncirrhotic portal hypertension diagnosis and management. Jul 27, 2010 visceral adiposity and insulin resistance are independent predictors of the presence of noncirrhotic nafldrelated portal hypertension. May 30, 2015 idiopathic non cirrhotic portal hypertension incph is a rare disease characterized of intrahepatic portal hypertension in the absence of cirrhosis or other causes of liver disease and splanchnic venous thrombosis. Non cirrhotic portal fibrosis idiopathic portal hypertension. Non cirrhotic portal fibrosis ncpf variously called as idiopathic pht iph, hepatoportal sclerosis and obliterative venopathy, is a disorder of unknown etiology, clinically characterized by features of pht. Mar, 2020 non cirrhotic portal fibrosis ncpf presents with non cirrhotic portal hypertension characterized by moderate to severe splenomegaly, evidence of varices andor collaterals, patent splenoportal axis and hepatic veins, normal liver functions, normal or nearnormal hepatic venous pressure gradient hvpg, and no evidence of cirrhosis or parenchymal injury in the liver histology. Cystic fibrosis immunologic deficiency syndrome turner syndrome congenital hepatic fibrosis idiopathic non cirrhotic portal hypertension detailed description. An ill understood and controversial liver disease, second only to cirrhosis in presenting with features of significant and sustained portal hypertension, has been known for many years under a variety of names such as banti syndrome, tropical splenomegaly, non cirrhotic portal fibrosis ncpf, idiopathic portal hypertension iph, hepatoportal. We aimed to determine the number of individuals with ncph within our cohort and define their characteristics. Idiopathic portal hypertension radiology reference. Coagulation profile and platelet function in patients with.
The disease is usually non progressive, though a subset of patients may progress to progressive liver failure. Bunch of grapes appearance in noncirrhotic portal fibrosis. Article information, pdf download for noncirrhotic portal fibrosis at a. View enhanced pdf access article on wiley online library html view download pdf for offline viewing. It is a distinct variety of portal hypertension which exists in adults between 25 to 35 years, and is characterized by splenomegaly, anemia, episodes of bleeding from gastrointestinal varices, hepatic encephalopathy and absence of ascites. Idiopathic non cirrhotic portal hypertension incph is a diagnosis of exclusion with increased portal venous pressure without cirrhosis, hepatoportal flow obstruction, splanchnic venous thrombosis, and other causes of liver disease. Fibrosis was staged on a fivepoint scale from f0 to f4 as follows. Group of diseases with phtn due to intrahepatic and extra hepatic lesions in the absence of cirrhosis. Idiopathic non cirrhotic portal hypertension incph is a rare disease characterized of intrahepatic portal hypertension in the absence of cirrhosis or other causes of liver disease and splanchnic.
In all 4 patients, hvpg was 49 mm hg, clearly below the cutoff for clinically significant pht. Oct 27, 2016 non cirrhotic portal fibrosis ncpf is a clinical entity of uncertain etiology that is typically characterized by periportal fibrosis due to involvement of small and medium branches of the portal vein, resulting in the portal hypertension 1. This results in an increased portal venous pressure gradient in the absence of a known cause of liver disease. However, cfld patients typically present with pht, without cholestasis and with preserved liver function. Pdf portal hypertension occurs commonly in patients with cirrhosis and rarely in those without cirrhosis of liver. A 40yearold female patient, a known case of non cirrhotic hepatic fibrosis nchf, came for followup to our clinic. Noncirrhotic portal fibrosis at a tertiary care centre in south india v. Noninvasive predictors of highrisk varices in patients. Idiopathic noncirrhotic portal hypertension incph is a rare disease characterized by of intrahepatic portal hypertension in the absence of cirrhosis, other causes of liver disease and splanchnic venous thrombosis 17.
Noncirrhotic portal hypertension ncph includes a spectrum of chronic liver diseases characterized by increased pressure within the portal circulation in the absence of cirrhosis. Amati2 1department of clinical medicine, center for the diagnosis and treatment of portal hypertension, 2department of radiological, oncological, and pathological sciences, sapienza university of rome, rome, italy. Noncirrhotic intrahepatic portal hypertension nciph is characterised by an increased portal pressure with patent portal and hepatic veins, in the absence of cirrhosis. Backgroundnoncirrhotic portal fibrosis ncpf is one of the important. Noncirrhotic portal fibrosis ncpf is a disease of uncertain etiology characterized by periportal fibrosis and involvement of small and medium. Obliterative portal venopathy opv, also known as idiopathic portal hypertension and hepatoportal sclerosis, is a major cause of noncirrhotic portal hypertension 1. Noncirrhotic portal fibrosis ncpf idiopathic portal hypertension iph hepatoportal sclerosis congenital hepatic fibrosis. Mar 21, 2018 non cirrhotic portal hypertension ncph comprises a group of diseases that are characterized by increased portal pressure in the absence of cirrhosis of the liver. Idiopathic non cirrhotic portal hypertension is an underrecognized vascular liver disease of unknown etiology, characterized by clinical signs of portal hypertension in the absence of cirrhosis.
Ncpf is also known as noncirrhotic intrahepatic portal hypertension nciph. In order to assess the comparative efficacy and safety of endoscopic injection sclerotherapy in patients with portal hypertension of different etiology, i. Although many liver diseases may be associated with portal hypertension in the absence of advanced fibrosis, the term ncph is most widely used to describe a spectrum of changes resulting. A case of idiopathic noncirrhotic portal hypertension in. The two most important causes of non cirrhotic portal hypertension are noncirrhotic portal fibrosis ncpf and extrahepatic portal venous obstruction ehpvo. Non cirrhotic intrahepatic portal hypertension nciph is characterised by an increased portal pressure with patent portal and hepatic veins, in the absence of cirrhosis.
Mild atrophy of the liver with a smooth outline was noted. Idiopathic portal hypertension presenting with hepatic nodular regenerative hyperplasia after splenectomy. In three patients, calcifications in the larger portal vein. Non cirrhotic portal fibrosis current concepts medcrave online. Fibrosis stage was defined by two pathologists in consensus. Non cirrhotic portal hypertension ncph comprises a group of diseases that are characterized by increased portal pressure in the absence of cirrhosis of the liver. Phlebothrombosis as a common pathogenic denominator in noncirrhotic portal. Noncirrhotic portal hypertension ncph encompasses a group of diseases of varied etiology. Idiopathic portal hypertension radiology reference article. Noncirrhotic portal fibrosis ncpf is a chronic liver disease and type of non cirrhotic portal hypertension ncph. Cystic fibrosis related liver disease cfld is allegedly caused by small bile duct obstruction leading to focal biliary fibrosis and eventually to cirrhosis with portal hypertension pht. Obvious fibrosis in the portal area was found in all pathological results of iph. Oo noncirrhotic portal hypertension enrique molina, md, and k.
Noncirrhotic portal hypertension is the most frequent cause of portal hypertension in pregnancy in our hospital. A retrospective analysis of 50 pregnancies in 27 women with ncph was carried out. Outcome of injection sclerotherapy using absolute alcohol in. You can ask your doctor regarding doing physical exercise, to my opinion it. A wide spectrum of clinical and morphologic changes in 32 autopsy cases of noncirrhotic portal fibrosis have been described. Biliary lipid composition in patients with non cirrhotic portal fibrosis a comparison with compensated cirrhosis of the liver. A case of idiopathic noncirrhotic portal hypertension in a. Non cirrhotic portal hypertension ncph has been associated with didanosine ddl exposure.
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