ArticleIndian journal of gastroenterology : official journal of the Indian Society of Gastroenterology2014
ADAMTS13 deficiency, despite well-compensated liver functions in patients with noncirrhotic portal hypertension.
Article in Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT04267406 (Investigation of Changes in ADAMTS-13 and Von Willebrand Factor Levels and Activities in Children With Cirrhosis and/or Portal Hypertension), which is not on this map. Cited by 12 papers.
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Investigation of Changes in ADAMTS-13 and Von Willebrand Factor Levels and Activities in Children With Cirrhosis and/or Portal Hypertension
Who cites it
12 citing papers in PubMed, 24 citations in OpenAlex.
- 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
- 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
- Potential danger of isolated platelet transfusion in patients with dengue infection.The Indian journal of medical research · 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
- 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
- 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
- Von Willebrand Factor as a Biomarker for Liver Disease - An Update.Journal of clinical and experimental hepatologyReview
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Authors and funding
9 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundWe have reported A disintegrin and metalloprotease with thrombospondin type 1 motif, member 13 (ADAMTS13) deficiency in noncirrhotic intrahepatic portal hypertension (NCIPH) patients of European origin with preserved liver function. We aimed to study ADAMTS13-von Willebrand factor (vWF) imbalance in Indian patients with NCIPH.
methodsTwenty-nine cases with NCIPH [22 males; 29 years (13-58); Child's A, 23; B, 6], 22 disease controls with cryptogenic chronic liver disease [15 males; 46 years (18-74); Child's A, 9; B, 9; C, 4] and 17 healthy controls [14 males; 32 years (27-45)] were enrolled in the study. We measured ADAMTS13 antigen and activity (by collagen binding assay (CBA) and by fluorescence resonance energy transfer [FRET] assay), and vWF antigen levels in plasma of study patients.
resultsADAMTS13 activity by CBA in NCIPH patients (32 %, 5 % to 100 %; median, range; p-value <0.001) and disease controls (36 %, 5 % to 144 %; p = 0.001) was significantly lower than in healthy controls (87 %; 60 % to 148 %). ADAMTS13 antigen and activity by FRET assay were also lower in cases and disease controls. ADAMTS13 activity (by CBA) to antigen ratio was lower in NCIPH and disease controls than in healthy controls. Of 29 NCIPH patients, 3 (all in Child's A) had severe ADAMTS13 deficiency (<10 % ADAMTS13 activity), and 8 (Child's A, 7; B, 1) had moderate ADAMTS13 deficiency (10 % to 25 % activity). Conversely, vWF antigen and vWF:ADAMTS13 ratio were higher in patients with NCIPH and in disease controls than in healthy controls.
conclusionsThis study validates the finding of ADAMTS13 deficiency in NCIPH despite preserved liver functions in an Indian population suggesting its involvement in pathogenesis of NCIPH.
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