Systemic lupus erythematosus complicated by noncirrhotic. Etiology idiopathic 50% to 90% of children portosplenic vein inflammation umbilical sepsis umbilical catheterization neonatal peritonitis abdominal trauma. Noncirrhotic portal hypertension and pathology of the sinusoids ian r. Noncirrhotic portal hypertension portal hypertension that is not caused by cirrhosis of the liver is rare in the united states. Noncirrhotic portal hypertension ncph is a rare but potentially lifethreatening complication in patients with human immunodeficiency virus hiv. Cirrhosis is the most common cause of portal hypertension but a heterogeneous group of clinical entities, collectively referred to as noncirrhotic portal. Noncirrhotic portal hypertension ncph is a heterogeneous group of liver disorders of vascular origin, leading to portal hypertension phtn in the absence of cirrhosis. Dogs with intraabdominal portal hypertension of vascular causes or with hepatic histopathologic changes. Noninvasive predictors of highrisk varices in patients. May 03, 2017 we had a great report discussing our approach to noncirrhotic portal hypertension. The consensus statements, prepared and deliberated at length by the experts in this field, were presented at the annual meeting of the apasl. However, some anatomical limitations such as portal vein thrombosis can make tips technically impossible to perform. Idiopathic noncirrhotic portal hypertension in dogs request pdf. The natural course of ncph is largely indolent and benign with an overall better outcome than cirrhosis, as long as the.
It may arise as a result of either primary hepatic disease or the effects of systemic disorders on liver. Ncph causes can be classified according to the site of por. Noncirrhotic presinusoidal portal hypertension associated. Sep 11, 2007 the asian pacific association for the study of the liver apasl working party on portal hypertension has developed consensus guidelines on the disease profile, diagnosis, and management of noncirrhotic portal fibrosis and idiopathic portal hypertension. Liver biopsy is essential for the diagnosis of noncirrhotic portal hypertension, to exclude cirrhosis of any cause, as well as primary biliary cirrhosis, schistosomiasis and granuloma, and to assess the presence of vascular lesions, perisinusoidal fibrosis, peliosis hepatis and intrahepatic portal or hepatic venous occlusions. The disease was called noncirrhotic portal fibrosis. Surgical risk in patients with noncirrhotic portal hypertension. Genetic determinants of idiopathic noncirrhotic portal. Uptodate, electronic clinical resource tool for physicians and patients that provides information on adult primary care and internal medicine, allergy and immunology, cardiovascular medicine, emergency medicine, endocrinology and diabetes, family medicine, gastroenterology and hepatology, hematology, infectious diseases, nephrology and.
Study on noncirrhotic portal hypertension 9, and the data collected through personal communication with investigators having interest in this disease. Idiopathic noncirrhotic portal hypertension ncph is an infrequent but possibly underestimated cryptogenetic liver disease recently described in small series of hivinfected patients. Non cirrhotic portal fibrosis also constitutes a small. Noncirrhotic portal hypertension in hivinfected patients. Ncph should be considered in any patient with sle who suffers from clinical manifestations of portal hypertension in the absence of cirrhosis evidence. Portal hypertension is characterized by an increase in portal pressure 10 mmhg and could be a result of cirrhosis of the liver or of noncirrhotic diseases. Diagnosis then relies on a comprehensive understanding of the causes and clinical manifestations of this disease, as well as a careful interpretation of the liver biopsy.
Studies conducted in the 1980s or earlier found an incidence of approximately 23. Occlusive hepatic venous catheterization in the study of the normal liver, cirrhosis of the liver and noncirrhotic portal hypertension. Noncirrhotic portal hypertension concept, diagnosis and clinical management. Portosystemic shunt surgery in patients with idiopathic noncirrhotic portal hypertension. Noninvasive predictors of highrisk varices in patients with. The np plays a role in initial recognition and health maintenance of pediatric patients with portal hypertension. Noncirrhotic portal hypertension diagnosis and management. The study identifies key risk factors for poor outcome after surgery, which clinicians could use to counsel patients. Pdf noncirrhotic portal hypertension enrique molina. Pdf ncph is a heterogeneous group of liver disorders of vascular origin, leading to pht with near normal hvpg.
Request pdf idiopathic noncirrhotic portal hypertension in dogs to describe clinical signs, diagnostic findings, and outcome in dogs with idiopathic intrahepatic portal hypertension. Patients with incph have preserved liver function but may develop portal hypertension related complications. Surgical risk in patients with noncirrhotic portal. Think about noncirrhotic portal hypertension in terms of anatomical causes. Surgical management of noncirrhotic portal hypertension. Noncirrhotic portal hypertension ncph includes a spectrum of chronic liver diseases characterized by increased pressure within the portal circulation in the absence of cirrhosis. Noncirrhotic nodular transformation of the liver with portal. We evaluated the frequency of this syndrome in noncirrhotic portal hypertension 1, 3.
It is rare when systemic lupus erythematosus sle complicated by noncirrhotic portal hypertension ncph is reported, likely because it is underrecognized clinically as well as pathologically. However, the authors were not able to control confounding through multivariate modeling because of the small number of case patients. Noncirrhotic portal hypertension ncph comprises a broad group of disorders characterized by elevated portal pressures in the absence of cirrhosis. In 1965, mikkelsen et al identified a concentric thickening of the portal vein and its radicles. Noncirrhotic portal hypertension ucsf internal medicine.
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. Journal of clinical medicine article hematological indices in portal hypertension. 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. The asian pacific association for the study of the liver apasl working party on portal hypertension has developed consensus guidelines on the disease profile, diagnosis, and management of noncirrhotic portal fibrosis and idiopathic portal hypertension. Portal hypertension is a term used to describe elevated pressures in the portal venous system a major vein that leads to the liver.
Portal vein diameter and splenomegaly decreased following treatment with methylprednisolone, hydroxychloroquine and metoprolol tartrate. Idiopathic noncirrhotic 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 hypertension represents a relatively infrequent group of conditions that causes portal hypertension in the absence of cirrhosis. Cases of ncph have been reported in hivnegative individuals as result of treatment with thiopurines for leukemia or inflammatory bowel disease. Occlusive hepatic venous catheterization in the study of. T o the e ditor in 2020, kovari et al reported a strong association between exposure to didanosine ddi and noncirrhotic portal hypertension ncph, a rare condition likely to be of multifactorial etiology. This retrospective study included 328 patients with pht 239 cirrhosis and 89 ncpht. Cirrhosis is the most common cause of portal hypertension, but it can also be present in the absence of cirrhosis, a condition referred to as noncirrhotic portal hypertension. Pdf portosystemic shunt surgery in patients with idiopathic. Jan 01, 2011 t o the e ditor in 2020, kovari et al reported a strong association between exposure to didanosine ddi and noncirrhotic portal hypertension ncph, a rare condition likely to be of multifactorial etiology.
In other parts of the world it is variously named, as hepatoportal sclerosis 4, 5, noncirrhotic intrahepatic portal hypertension 6, and idiopathic noncirrhotic intrahepatic portal hypertension 7. Thus, it is suggested that in this patient, presinusoidal portal hypertension was secondary to chronic arsenical intake associated with marked intrahepatic vascular changes seen on portography. Hepatic cirrhosis, portal hypertension, and hepatic failure. Pdf noncirrhotic portal hypertension diagnosis and. Idiopathic noncirrhotic portal hypertension has been associated with immunologic disorders such as systemic lupus erythematosus or primary hypogammaglobulinemia, infections such as hiv, toxins or medications such as azathioprine and didanosine, and prothrombotic disorders leading to microthrombosis in the liver. Abstract idiopathic noncirrhotic portal hypertension ncph is an infrequent but possibly underestimated cryptogenetic liver disease recently described in small series of hivinfected patients.
Portopulmonary hypertension in a case of noncirrhotic portal fibrosis. The exposure to antiretroviral drugs, a direct role of hiv itself, microbial translocation from the gut, or a thrombophilic propensity have been suggested as. Idiopathic noncirrhotic portal hypertension incph is. Noncirrhotic portal fibrosisidiopathic portal hypertension. Noncirrhotic portal hypertension pubmed central pmc. Portal hypertension also contributes to the development of hepatic encephalopathy and hepatorenal syndrome. Extrahepatic portal vein thrombosis epvt is the second commonest cause of portal hypertension worldwide. Hj noncirrhotic portal hypertension is an increased pressure gradient in the portal system of more than 5 mm hg without the presence of cirrhosis. Schistosomiasis is one of the most common causes of noncirrhotic portal hypertension worldwide.
Summary noncirrhotic portal hypertension is an underappreciated clinical entity that can be difficult to diagnosis without a healthy suspicion. Noncirrhotic portal hypertension ncph is a heterogeneous group of vascular diseases that lead to portal hypertension ph with normal or mildly elevated hepatic venous pressure gradient and preserved liver synthetic function. 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. Noncirrhotic etiologies of portal hypertension are classified as prehepatic, hepatic, and posthepatic see causes of pediatric portal hypertension. Hepatic histopathologic changes were consistent with idiopathic noncirrhotic portal hypertension and were indistinguishable from those of dogs with surgically created portocaval anastomosis. Noncirrhotic portal hypertension ncph comprises a heterogeneous group of liver disorders causing portal hypertension without cirrhosis and carries a high risk of variceal bleeding. The etiology of our patients liver disorder re mains obscure. Pdf noncirrhotic portal hypertension ncph is a heterogeneous group of liver disorders of vascular origin, leading to portal hypertension phtn in. Demographic and clinical features, endoscopic and radiological findings, and hi.
Outcome was determined for 19 dogs released from hospital. Idiopathic noncirrhotic portal hypertension in hivinfected patients clin infect dis 2010. A very wide range of diseases can cause noncirrhotic portal hypertension. As with cirrhotic portal hypertension, most cases of noncirrhotic portal hypertension are caused by increased portal venous outflow resistance, although, rarely, an increased flow in the portal. Ascites in patients with noncirrhotic nonmalignant. The prognosis of this disease is much better than that of.
Cirrhosis versus noncirrhotic portal hypertension abdurrahman sahin 1, id, hakan artas 2 id, nurettin tunc 1, mehmet yalniz 1 and ibrahim halil bahcecioglu 1 1 division of gastroenterology, department of internal medicine, firat university school of medicine, 23200 elazig, turkey. Noncirrhotic portal hypertension ncph has been recently reported as a liver disease in human immunodeficiency virus hivinfected patients under antiretroviral therapy art. The aim of the study is to investigate the differences in hi between cirrhotic subjects and subjects who have noncirrhotic pht ncpht. Portal hypertension pht leads to several alterations on hematological indices hi. Portal hypertension is defined by a pathologic increase in the pressure of the portal venous system. Association of noncirrhotic portal hypertension in hivinfected persons and antiretroviral therapy with didanosine. Wanless department of pathology dalhousie university halifax, canada. Epidemology in the west 5% to 10% developing countries 15% to 20% of all cases of portal hypertension, in children, ehpvo accounts for 80% to 90% of cases of portal hypertension etiology. Idiopathic noncirrhotic portal hypertension gastroenterology. Full text idiopathic noncirrhotic portal hypertension. Noncirrhotic portal hypertension associated with didanosine. In general, portal hypertension secondary to a noncirrhotic etiology has a better prognosis than portal hypertension caused by liver cirrhosis because of maintained synthetic function in the former.
Apr 29, 2019 these complication and mortality rates after abdominal surgery in patients with noncirrhotic portal hypertension are similar to those reported in patients with cirrhosis and significantly higher than rates in the general population. Transjugular intrahepatic portosystemic shunt tips creation can be very beneficial to decrease high portal pressure and its consequent dreadful complications, such as variceal hemorrhage. Idiopathic noncirrhotic portal hypertension in dogs. Portal hypertension may be caused by intrinsic liver disease, obstruction, or structural changes that result in increased portal venous flow or increased hepatic resistance. Natural history of noncirrhotic portal hypertension full. Here, we describe a case of a 72yearold female patient who previously had a rouxeny. Idiopathic noncirrhotic portal hypertension incph is relatively uncommon and defined as portal hypertension in the absence of cirrhosis and portal and hepatic vein abnormalities. Combination of nonexclusive mechanisms has been described. Love, sammy top pearls schistosomiasis is one of the most common causes of noncirrhotic portal.
The etiology of incph can be classified in five categories. Twentyfive consecutive patients 16 men with biopsyproven noncirrhotic portal hypertension 24 with noncirrhotic portal fibrosis and 1 with portal vein thrombosis without cardiopulmonary disease were screened by air contrastenhanced echocardiography. When portal hypertension occurs in the absence of liver cirrhosis, noncirrhotic portal hypertension ncph must be considered. Histological features of incph comprise a wide spectrum of nonspecific features, ranging from minor changes, sinusoidal dilatation, phlebosclerosis and portal. Noncirrhotic hyperammonaemic encephalopathy laish 2011. Histological features of incph comprise a wide spectrum of nonspecific features, ranging from minor changes, sinusoidal. Professor of pathology, icahn school of medicine at mount sinai new york, new york overview. Cureus treatment of noncirrhotic portal hypertension. Jun 21, 2011 liver biopsy is essential for the diagnosis of noncirrhotic portal hypertension, to exclude cirrhosis of any cause, as well as primary biliary cirrhosis, schistosomiasis and granuloma, and to assess the presence of vascular lesions, perisinusoidal fibrosis, peliosis hepatis and intrahepatic portal or hepatic venous occlusions. Idiopathic noncirrhotic portal hypertension journal of rare. Portal hypertension with or without cirrhosis plays an essential role in this association but the exact mechanism is yet to be defined. The complications from ncph are similar to that of cirrhosis induced portal hypertension which includes the development of gastrointestinal varices, portal. Mar 21, 2018 noncirrhotic portal hypertension ncph comprises a group of diseases that are characterized by increased portal pressure in the absence of cirrhosis of the liver.
Idiopathic noncirrhotic portal hypertension incph is characterized by the elevation of portal venous pressure with no. It occurs when blood flow in the major vein in the liver the portal vein slows down. Noncirrhotic portal hypertension and pathology of the sinusoids. In the western world, terms such as hepatoportal sclerosis, idiopathic portal hypertension, incomplete septal cirrhosis, and nodular regenerative. However, 3 months later, the patient died of upper gastrointestinal bleeding and respiratory failure. Noncirrhotic portal hypertension europe pmc article. We had a great report discussing our approach to noncirrhotic portal hypertension. The major causes are extra hepatic portal vein obstruction ehpvo and congenital hepatic fibrosis chf. The patient was diagnosed with active sle complicated by noncirrhotic portal hypertension ncph without liver histopathology, due to the patients refusal for liver biopsy. Thank you, polly, for presenting the case of a young man with portal hypertension without a definitive diagnosis of cirrhosis who presented with aki and profound anemia. From the department of medicine, duke university school of medicine durham, n. Flow chart showing how the articles were selected and excluded.
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