Noncirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and treatment

Sarin, Shiv Kumar; Kumar, Ashish; Chawla, Yogesh; Baijal, Sanjay Saran; Dhiman, Radha K.; Jafri, Wasim; Lesmana, Laurentius A.; Mazumder, Debendranath Guha; Omata, Masao; Qureshi, Huma; Raza, Rizvi Moattar; Sahni, Peush; Sakhuja, Puja; Salih, Mohammad; Santra, Amal; Sharma, Barjesh Chander; Sharma, Praveen; Shiha, Gamal; Sollano, José D.

doi:10.1007/s12072-007-9010-9

Cited by 151 publications

(183 citation statements)

References 100 publications

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“…In our study, 3 patients (75%) were diagnosed following a sign or symptom of portal hypertension, two due to gastric bleeding esophageal varices and the other due to an episode of hepatic encephalopathy. Although these symptoms are the proper ones of the portal hypertension for other causes, it have been reported that variceal bleeding is most frequent, affecting two thirds of the patients (17,18). This explains why the diagnosis of IPH is delayed by the difficulty of diagnosing early pre-clinical stages.…”

Section: Discussionmentioning

confidence: 99%

Idiopathic portal hypertension regarding thiopurine treatment in patients with inflammatory bowel disease

Ferrer¹,

Herrera²,

Moya³

et al. 2015

Rev Esp Enferm Dig

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Introduction:The possibility of developing idiopathic portal hypertension has been described with thiopurine treatment despite compromises the prognosis of these patients, the fact its true prevalence is unknown.Material and methods: A cross-sectional study was conducted in a cohort of inflammatory bowel disease (IBD) patients followed at our unit, to determine the prevalence of diagnosis of idiopathic portal hypertension (IPH) and its relationship with thiopurine treatment.Results: At the time of the analysis, 927/1,419 patients were under treatment with thiopurine drugs (65%). A total of 4 patients with IBD type Crohn's disease with idiopathic portal hypertension probably related to the thiopurine treatment were identified (incidence of 4.3 cases per 1,000). Seventy-five percent of patients started with signs or symptoms of portal hypertension. Only one patient was asymptomatic but the diagnosis of IPH because of isolated thrombocytopenia is suspected. However, note that all patients had thrombocytopenia previously. Abdominal ultrasound with fibroscan, hepatic vein catheterization and liver biopsy were performed on all of them as part of the etiology of portal hypertension. In the abdominal ultrasound, indirect portal hypertension data were observed in all patients (as splenomegaly) cirrhosis was also ruled out. The fibroscan data showed significant liver fibrosis (F2-F3).Conclusion: Idiopathic portal hypertension following thiopurine treatment in IBD patients is a rare occurrence, but it must be borne in mind in the differential diagnosis for early diagnosis, especially in patients undergoing thiopurine treatment over a long period. The presence of thrombocytopenia is often the only predictor of its development in the preclinical stage.

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Section: Discussionmentioning

confidence: 99%

Idiopathic portal hypertension regarding thiopurine treatment in patients with inflammatory bowel disease

Ferrer¹,

Herrera²,

Moya³

et al. 2015

Rev Esp Enferm Dig

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“…12 Differences in socioeconomic status, living conditions, average lifespan, and ethnic background may be responsible for the higher occurrence in lower socioeconomic classes, though this is probably related to poor sanitation and hygiene and poor access to healthcare. 13 The incidence of NCPF has probably declined over the past two decades along with a reduction in the incidence of extrahepatic portal vein obstruction (EHPVO).…”

Section: Epidemiologymentioning

confidence: 99%

“…Over the years, the improvement in the living standards and the improvement in sanitation and hygiene, also confirmed by the reduced incidence of other diarrheal illnesses, may play a role in the reducing incidence of NCPF and EHPVO. 12 Perinatal practices have also changed resulting in reducing the frequency of umbilical sepsis in India. 12 …”

Section: Epidemiologymentioning

confidence: 99%

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Noncirrhotic Portal Hypertension

Rajekar¹,

Vasishta²,

Chawla³

et al. 2011

Journal of Clinical and Experimental Hepatology

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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. When portal hypertension occurs in the absence of liver cirrhosis, noncirrhotic portal hypertension (NCPH) must be considered. The prognosis of this disease is much better than that of cirrhosis. Noncirrhotic diseases are the common cause of portal hypertension in developing countries, especially in Asia. NCPH is a heterogeneous group of diseases that is due to intrahepatic or extrahepatic etiologies. In general, the lesions in NCPH are vascular in nature and can be classified based on the site of resistance to blood flow. In most cases, these disorders can be explained

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“…In contrast, NCPF is characterized by huge splenomegaly (average weight 1,500 g), predominantly affects people of the lower socioeconomic strata in the fourth decade of life, has a patent splenoportal axis with dilated portal vein and splenic vein with large perisplenic, retroperitoneal collaterals, and these patients commonly develop esophagogastric varices with a high incidence of variceal bleeding [12][13][14][15][16]. Ultrastructurally, the liver has obliteration of the portal venules and increased periportal fibrosis in the absence of liver regeneration [12,13].…”

Section: Natural History Of Phtmentioning

confidence: 99%

Current Role of Surgery in Portal Hypertension

Pal

2011

Indian J Surg

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Treatment for portal hypertension (PHT) has evolved from surgery being the only option during the 1970s to the wide range of options currently available. Surgery has not vanished from the therapeutic armamentarium, but its role has changed and is constantly evolving. The present review primarily focuses on the role of surgery in tackling patients with PHT and varices with regard to the Indian scenario and also looks at its relevance, given the availability of a host of other therapeutic options.Keywords Portal hypertension . Portosystemic shunt . Surgery . Variceal haemorrhage Pathophysiology of PHTFor understanding how surgery can help modify the natural course in patients with PHT, it is necessary to understand its pathophysiology and the natural history of varices in such patients. PHT is usually the consequence of increased resistance to portal blood flow coupled with increased blood flow through the splanchnic circulation because of a hyperdynamic circulatory state that exists both in cirrhotics and to some extent in noncirrhotic etiologies (large congested splenomegaly) as well. Traditionally, at the ultrastructural level, PHT has been classified into presinusoidal, sinusoidal, and postsinusoidal. It is for the sinusoidal and postsinusoidal blocks that there is an accurate but invasive way of monitoring the portal pressure by using the method to estimate the hepatic venous pressure gradient (HVPG) in the vascular/hemodynamic lab [1][2][3]. In cirrhotic patients, as about a third to half of them will actually have PHT; HVPG >5 mmHg indicates PHT. PHT becomes clinically significant when the HVPG is ≥10 mmHg [1][2][3]. This is the pressure threshold when PHT leads to the formation of esophagogastric varices, retroperitoneal and periportal collaterals, hypersplenism, low serum albuminascitic albumin gradient ascites, spontaneous bacterial peritonitis, etc. Varices rupture and bleed only when the HVPG >12 mmHg [1][2][3]. Natural History of PHTIt has been estimated that varices are present in about 30-40% of compensated cirrhotics and nearly two-thirds of the decompensated patients. In cirrhotic patients without varices on the first endoscopy, the annual incidence of new varices is between 5% and 10% [1,4]. Once developed, the progression of varices from small to large depends on the deterioration of liver function and continued hepatic insult (virus-, alcohol-, metabolic-, drug-induced, etc.). The overall annual incidence of bleeding is about 4% and this increases to 15% per year in those with large varices, red signs, and poor Child's status [1,[4][5][6]. About a third of the cirrhotic patients with varices will bleed and the mortality following each episode of bleeding is about 20% (maybe higher) in experienced tertiary health centers. Early mortality (within 6 weeks) following an acute episode of variceal bleeding depends on the etiology of PHT, the severity of underlying liver disease (Child's C, model for end-stage liver disease [MELD] >18), actively bleeding varices, bleeding gastric varices (GV...

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Noncirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and treatment

Cited by 151 publications

References 100 publications

Idiopathic portal hypertension regarding thiopurine treatment in patients with inflammatory bowel disease

Idiopathic portal hypertension regarding thiopurine treatment in patients with inflammatory bowel disease

Noncirrhotic Portal Hypertension

Current Role of Surgery in Portal Hypertension

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