Idiopathic portal hypertension presenting with hepatic nodular regenerative hyperplasia after splenectomy. Article information, pdf download for noncirrhotic portal fibrosis at a. Noncirrhotic portal hypertension is the most frequent cause of portal hypertension in pregnancy in our hospital. Obvious fibrosis in the portal area was found in all pathological results of iph. Biliary lipid composition in patients with noncirrhotic portal fibrosisa comparison with compensated cirrhosis of the liver. Group of diseases with phtn due to intrahepatic and extra hepatic lesions in the absence of cirrhosis. Fibrosis stage was defined by two pathologists in consensus. Idiopathic portal hypertension radiology reference article. 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. 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.
Noncirrhotic portal fibrosis at a tertiary care centre in south india v. Cystic fibrosis immunologic deficiency syndrome turner syndrome congenital hepatic fibrosis idiopathic non cirrhotic portal hypertension detailed description. Mild atrophy of the liver with a smooth outline was noted. Biliary lipid composition in patients with non cirrhotic portal fibrosis a comparison with compensated cirrhosis of the liver. Idiopathic noncirrhotic intrahepatic portal hypertension. Coagulation profile and platelet function in patients with. Ct showed massive splenomegaly with grossly dilated and tortuous splenic vein thin arrow and attenuated main portal vein thick arrow and its intrahepatic branches.
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. Non cirrhotic portal hypertension ncph has been associated with didanosine ddl exposure. Ncpf is also known as noncirrhotic intrahepatic portal hypertension nciph. 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.
Noncirrhotic intrahepatic portal hypertension nciph is characterised by an increased portal pressure with patent portal and hepatic veins, in the absence of cirrhosis. It may arise as a result of either primary hepatic disease or the effects of systemic disorders on liver. Non cirrhotic portal fibrosis current concepts medcrave online. Non cirrhotic intrahepatic portal hypertension nciph is characterised by an increased portal pressure with patent portal and hepatic veins, in the absence of cirrhosis. 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. 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. Get a printable copy pdf file of the complete article 1. 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. Various factors have been implicated in the causation of the disease. However, cfld patients typically present with pht, without cholestasis and with preserved liver function. The etiology of incph can be classified in five categories.
Noncirrhotic portal fibrosis ncpf idiopathic portal hypertension iph hepatoportal sclerosis congenital hepatic fibrosis. 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. Noncirrhotic portal fibrosis ncpf is a chronic liver disease and type of non cirrhotic portal hypertension ncph. Transient elastography fibroscan in patients with non. 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. Noncirrhotic portal fibrosis ncpf is a disease of uncertain etiology characterized by periportal fibrosis and involvement of small and medium. You can ask your doctor regarding doing physical exercise, to my opinion it. 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. Pregnancy outcome was compared in extra hepatic portal vein obstruction ehpvo and non. 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.
Fibrosis was staged on a fivepoint scale from f0 to f4 as follows. 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 hypertension ncph includes a spectrum of chronic liver diseases characterized by increased pressure within the portal circulation in the absence of cirrhosis. Obliterative portal venopathy opv, also known as idiopathic portal hypertension and hepatoportal sclerosis, is a major cause of noncirrhotic portal hypertension 1. The disease is usually non progressive, though a subset of patients may progress to progressive liver failure. Coagulation disorders commonly develop in patients with cirrhosis of the liver. Pathology of idiopathic noncirrhotic portal hypertension. 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. 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. Non cirrhotic portal fibrosis is a clinical entity of uncertain etiology. Non cirrhotic portal hypertension authorstream presentation.
This results in an increased portal venous pressure gradient in the absence of a known cause of liver disease. 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. Idiopathic portal hypertension radiology reference. We previously suggested that cfldrelated pht could be a. Essentially a form of presinusoidal portal hypertension, a mechanism for this non cirrhotic portal hypertension is unclear. Liver disease in cystic fibrosis presents as noncirrhotic. Idiopathic noncirrhotic portal hypertension incph is a rare disease characterized of intrahepatic portal hypertension in the absence of. Recent guidelines, based largely on patients with viral cirrhosis, suggest low likelihood of. Noncirrhotic portal hypertension and incomplete septal. View enhanced pdf access article on wiley online library html view download pdf for offline viewing. Visceral adiposity and insulin resistance are independent.
In order to assess the comparative efficacy and safety of endoscopic injection sclerotherapy in patients with portal hypertension of different etiology, i. Transient elastography te using fibroscan is a useful technique for evaluation of fibrosis in patients with liver disease. The two most important causes of noncirrhotic portal hypertension are noncirrhotic portal fibrosis ncpf and extrahepatic portal venous obstruction ehpvo. A 40yearold female patient, a known case of non cirrhotic hepatic fibrosis nchf, came for followup to our clinic. Ncph is a heterogeneous group of liver disorders of vascular origin, leading to pht with near normal hvpg. A case of idiopathic noncirrhotic portal hypertension in. Natural history of noncirrhotic portal hypertension full.
A retrospective analysis of 50 pregnancies in 27 women with ncph was carried out. 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. May 30, 2015 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. Measurement and correlation of wedged hepatic, intrahepatic, intrasplenic and intravariceal pressures in patients with cirrhosis of liver and non cirrhotic portal fibrosis. Noncirrhotic portal hypertension in pregnancy request pdf. Pdf portal hypertension occurs commonly in patients with cirrhosis and rarely in those without cirrhosis of liver. 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. We aimed to determine the number of individuals with ncph within our cohort and define their characteristics. Alcoholic, metabolic, or autoimmune liver diseases can cause portal hypertension. 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.
Noncirrhotic portal fibrosis is inherited in an autosomal recessive manner. Bunch of grapes appearance in noncirrhotic portal fibrosis. In all 4 patients, hvpg was 49 mm hg, clearly below the cutoff for clinically significant pht. Of the 30 pregnant patients with portal hypertension in pregnancy during the years 19831998, 83. Pathology of noncirrhotic portal hypertension and incomplete. Pdf noncirrhotic portal hypertension enrique molina. 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. Noncirrhotic portal hypertension in pregnancy sciencedirect. 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. She had a previous history of recurrent episodes of abdominal pain with haematemesis and melena.
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. Radiological diagnosis of hepatocellular carcinoma in non. Backgroundnoncirrhotic portal fibrosis ncpf is one of the important. Multiple splenic artery aneurysms in noncirrhotic hepatic. 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. Noncirrhotic portal fibrosis idiopathic portal hypertension.
In three patients, calcifications in the larger portal vein. A wide spectrum of clinical and morphologic changes in 32 autopsy cases of noncirrhotic portal fibrosis have been described. Noncirrhotic portal hypertension diagnosis and management. 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. Noncirrhotic portal hypertension journal of hepatology. Pdf noncirrhotic portal fibrosisidiopathic portal hypertension.
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. It has been commonly described from india and japan, and is. Non cirrhotic portal hypertension ncph is a common cause of variceal bleed in developing countries. Dec 21, 2001 coagulation disorders commonly develop in patients with cirrhosis of the liver. Splenomegaly with or without anaemia has drawn the attention of a number of workers since banti. F2, combined perisinusoidal and portal periportal fibrosis. Noninvasive predictors of highrisk varices in patients with. The natural course of ncph is largely indolent and benign with an overall better outcome than cirrhosis, as long as the.
Radiological diagnosis of hepatocellular carcinoma in non cirrhotic patients hepatoma research is an open access journal and focuses on all topics related to hepatoma. 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. Non cirrhotic portal fibrosis ncpf is characterized by perivenular fibrosis of the small and medium branches of the portal vein resulting in portal hypertension pht while the liver structure and function remain normal. 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. Longterm outcome of pediatric noncirrhotic portal fibrosis. Also, in the chronic arsenic exposure model, there is increased hydroxyproline and collagen without significant hepatic fibrosis 26. 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. To study the outcome of pregnancy in women with non.
Natural history of non cirrhotic portal fibrosis a tropical. Jul 27, 2010 visceral adiposity and insulin resistance are independent predictors of the presence of noncirrhotic nafldrelated portal hypertension. Normal portal tract up to 30% of small portal tracts may lack portal vein branches. Oo noncirrhotic portal hypertension enrique molina, md, and k. Noncirrhotic portal hypertension and portal vein thrombosis. These features were highly suggestive of non cirrhotic portal fibrosis ncpf. The two most important causes of non cirrhotic portal hypertension are noncirrhotic portal fibrosis ncpf and extrahepatic portal venous obstruction ehpvo. Pdf the asian pacific association for the study of the liver apasl working party on. Incomplete septal cirrhosis and nodular regenerative hyperplasia have recently been shown to be part of the spectrum of the disease.
Portal biliopathy in patients with noncirrhotic portal. Non cirrhotic portal hypertension diagnosis and management. Histological features of incph comprise a wide spectrum of nonspecific features, ranging from minor changes, sinusoidal. Noncirrhotic portal hypertension in hivinfected individuals. Patients can be misdiagnosed as having liver cirrhosis, but awareness of this condition presence of portal hypertension with disproportionately.
Some have suggested an unknown infectious agent or immunologic event that leads to portal venous system fibrosis. Idiopathic noncirrhotic portal hypertension gastroenterology. Non cirrhotic portal fibrosis idiopathic portal hypertension. Noncirrhotic portal hypertension ncph encompasses a group of diseases of varied etiology. Noninvasive predictors of highrisk varices in patients.