ArticleDigestive diseases and sciences2011
Idiopathic noncirrhotic intrahepatic portal hypertension is associated with sustained ADAMTS13 Deficiency.
Article in Digestive diseases and sciences, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed.
- Non-cirrhotic intrahepatic portal hypertension: Is the gun loaded?Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology · 2024Article
- Porto-sinusoidal vascular disease: a new definition of an old clinical entity.Clinical and experimental hepatology · 2023Review
- Targeting von Willebrand factor in liver diseases: A novel therapeutic strategy?Journal of thrombosis and haemostasis : JTH · 2021Review
- What makes non-cirrhotic portal hypertension a common disease in India? Analysis for environmental factors.The Indian journal of medical research · 2019Review
- Duodenal variceal bleeding secondary to idiopathic portal hypertension treated with transjugular intra-hepatic porto-systemic shunt plus embolization: A case report.World journal of clinical cases · 2018Article
- Idiopathic portal hypertension and extrahepatic portal venous obstruction.Hepatology international · 2018Review
- ADAMTS13 missense variants associated with defective activity and secretion of ADAMTS13 in a patient with non-cirrhotic portal hypertension.Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology · 2017Article
- Plasma von Willebrand factor levels predict in-hospital survival in patients with acute-on-chronic liver failure.Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology · 2016Article
- Arsenicosis, possibly from contaminated groundwater, associated with noncirrhotic intrahepatic portal hypertension.Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology · 2016Article
- Orthotopic liver transplantation (OLTx) in non-cirrhotic portal hypertension secondary to ADAMTS13 deficiency.Przeglad gastroenterologiczny · 2016Article
- Hepatobiliary quiz-12 (2014).Journal of clinical and experimental hepatology · 2014Article
- Investigation into celiac disease in Indian patients with portal hypertension.Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology · 2014Article
- Idiopathic Non-Cirrhotic Intrahepatic Portal Hypertension (NCIPH)-Newer Insights into Pathogenesis and Emerging Newer Treatment Options.Journal of clinical and experimental hepatology · 2014Review
- ADAMTS13 deficiency, despite well-compensated liver functions in patients with noncirrhotic portal hypertension.Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology · 2014Article
- Noncirrhotic intrahepatic portal hypertension: towards a unifying definition and etiology.Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology · 2014Article
- Acute liver injury induced by low dose dimethylnitrosamine alters mediators of hepatic vascular flow.Toxicology reports · 2014Article
- A study of aetiology of portal hypertension in adults (including the elderly) at a tertiary centre in southern India.The Indian journal of medical research · 2013Article
- Use of serum vitamin B12 level as a marker to differentiate idiopathic noncirrhotic intrahepatic portal hypertension from cryptogenic cirrhosis.Digestive diseases and sciences · 2013Article
- Von Willebrand Factor as a Biomarker for Liver Disease - An Update.Journal of clinical and experimental hepatologyReview
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7 authors.
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Abstract
backgroundADAMTS13 deficiency leading to excess ultralarge von Willebrand factor (VWF) multimers and platelet clumping is typically found in thrombotic thrombocytopenic purpura (a type of thrombotic microangiopathy). Idiopathic noncirrhotic intrahepatic portal hypertension (NCIPH) is a microangiopathy of portal venules associated with significant thrombocytopenia and predisposing gut disorders.
aimTo determine whether the portal microangiopathy in NCIPH is associated with ADAMTS13 deficiency.
methodsPlasma levels of ADAMTS13, anti-ADAMTS13 antibodies, and VWF were compared between cases (NCIPH patients) and controls (with chronic liver diseases of other etiology) matched for severity of liver dysfunction. Eighteen NCIPH patients [median (range) MELD score 12 (7-25)] and 25 controls [MELD score 11 (4-26)] were studied.
resultsADAMTS13 activity was reduced in all 18 NCIPH patients and significantly lower than controls (median, IQR: 12.5%, 5-25% and 59.0%, 44-84%, respectively, P<0.0001) [normal range for plasma ADAMTS13 activity (55-160%)]. ADAMTS13 activity was <5% in 5/18 NCIPH patients (28%) and 0/25 controls (P=0.009). ADAMTS13 antigen levels were also decreased. Sustained low ADAMTS13 levels were seen in four NCIPH patients over 6 weeks to 11 months (highest ADAMTS13 level in each patient: <5%, 6%, 6%, and 25%), despite two patients having MELD score 12. Although nine cases had low titer anti-ADAMTS13 antibodies, there was no significant difference between cases and controls. Abnormally large VWF multimers were observed in 4/11 NCIPH patients (36%) and in 0/22 controls (P=0.008).
conclusionsSustained deficiency of ADAMTS13 appears characteristic of NCIPH, irrespective of severity of liver disease.
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