caa a22 4500 445796413 CHVBK 20180317145126.0 cr unu---uuuuu 170323e20110801xx s 000 0 eng 10.1007/s10620-011-1729-4 doi (NATIONALLICENCE)springer-10.1007/s10620-011-1729-4 Idiopathic Noncirrhotic Intrahepatic Portal Hypertension is Associated with Sustained ADAMTS13 Deficiency [Elektronische Daten] [Ian Mackie, C. Eapen, Desley Neil, Andrew Lawrie, Andrew Chitolie, Jean Shaw, Elwyn Elias] Background: ADAMTS13 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. Aim: To determine whether the portal microangiopathy in NCIPH is associated with ADAMTS13 deficiency. Methods: Plasma 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. Results: ADAMTS13 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 6weeks to 11months (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). Conclusions: Sustained deficiency of ADAMTS13 appears characteristic of NCIPH, irrespective of severity of liver disease. Springer Science+Business Media, LLC, 2011 ADAMTS13 deficiency nationallicence Portal venopathy nationallicence Noncirrhotic intrahepatic portal hypertension nationallicence Enteropathies nationallicence NCIPH : Idiopathic noncirrhotic intrahepatic portal hypertension nationallicence ADAMTS13 : A disintegrin and metalloprotease with a thrombospondin type 1 motif, member 13 nationallicence VWF : von Willebrand factor nationallicence MELD : Model for end-stage liver disease nationallicence TTP : Thrombotic thrombocytopenic purpura nationallicence Mackie Ian Haemostasis Research Unit, Haematology Department, University College London, London, UK aut Eapen C. Liver Unit, University Hospitals Birmingham NHS Foundation Trust, B15 2QT, Edgbaston, Birmingham, UK aut Neil Desley Department of Histopathology, University of Birmingham, Birmingham, UK aut Lawrie Andrew Haemostasis Research Unit, Haematology Department, University College London, London, UK aut Chitolie Andrew Haemostasis Research Unit, Haematology Department, University College London, London, UK aut Shaw Jean Liver Unit, University Hospitals Birmingham NHS Foundation Trust, B15 2QT, Edgbaston, Birmingham, UK aut Elias Elwyn Liver Unit, University Hospitals Birmingham NHS Foundation Trust, B15 2QT, Edgbaston, Birmingham, UK aut Digestive Diseases and Sciences Springer US; http://www.springer-ny.com 56/8(2011-08-01), 2456-2465 0163-2116 56:8<2456 2011 56 10620 https://doi.org/10.1007/s10620-011-1729-4 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s10620-011-1729-4 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Mackie Ian Haemostasis Research Unit, Haematology Department, University College London, London, UK aut NATIONALLICENCE 700 1- Eapen C. Liver Unit, University Hospitals Birmingham NHS Foundation Trust, B15 2QT, Edgbaston, Birmingham, UK aut NATIONALLICENCE 700 1- Neil Desley Department of Histopathology, University of Birmingham, Birmingham, UK aut NATIONALLICENCE 700 1- Lawrie Andrew Haemostasis Research Unit, Haematology Department, University College London, London, UK aut NATIONALLICENCE 700 1- Chitolie Andrew Haemostasis Research Unit, Haematology Department, University College London, London, UK aut NATIONALLICENCE 700 1- Shaw Jean Liver Unit, University Hospitals Birmingham NHS Foundation Trust, B15 2QT, Edgbaston, Birmingham, UK aut NATIONALLICENCE 700 1- Elias Elwyn Liver Unit, University Hospitals Birmingham NHS Foundation Trust, B15 2QT, Edgbaston, Birmingham, UK aut NATIONALLICENCE 773 0- Digestive Diseases and Sciences Springer US; http://www.springer-ny.com 56/8(2011-08-01), 2456-2465 0163-2116 56:8<2456 2011 56 10620 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer