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Idiopathic Non Cirrhotic Portal Hypertension and Spleno-Portal Axis Abnormalities in Patients with Severe Primary Antibody Deficiencies
Joint Authors
Pulvirenti, Federica
Pentassuglio, Ilaria
Milito, Cinzia
Valente, Michele
De Santis, Adriano
Conti, Valentina
d’Amati, Giulia
Riggio, Oliviero
Quinti, Isabella
Source
Journal of Immunology Research
Issue
Vol. 2014, Issue 2014 (31 Dec. 2014), pp.1-8, 8 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2014-03-31
Country of Publication
Egypt
No. of Pages
8
Main Subjects
Abstract EN
Background and Aim.
Portal hypertension has been reported in association with acquired and primary immune deficiencies without a comprehensive description of associated spleno-portal axis abnormalities.
Pathological mechanisms are poorly defined.
Methods.
Observational, single centre study with the aim of assessing the prevalence of spleno-portal axis abnormalities in an unselected cohort of 123 patients with primary antibody deficiencies and without known causes of liver diseases regularly followed up for a mean time of 18 ± 14 years.
A cumulative period of 1867 patients-year was analysed.
Clinical and immunological data, abdominal ultrasounds, CT scans, and endoscopy features were included in the analysis.
Results.
Twenty-five percent of patients with primary antibody deficiencies had signs of portal vein enlargement but only 4% of them had portal hypertension, with portal systemic collaterals.
Liver biopsies showed liver sinusoids congestive dilatation, endothelization, and micronodularity fulfilling the criteria for noncirrhotic portal hypertension.
Patients with portal vein enlargement had severe clinical and immunological phenotypes.
Conclusions.
In primary antibody deficient patients, infections, inflammations, splenomegaly, increased blood venous flow, and lymphocyte abnormalities contribute to establishment of liver damage possibly leading to noncirrhotic portal hypertension.
Patients with primary antibody deficiency should be considered a good model to give insight into the pathological mechanisms underlying noncirrhotic portal hypertension.
American Psychological Association (APA)
Pulvirenti, Federica& Pentassuglio, Ilaria& Milito, Cinzia& Valente, Michele& De Santis, Adriano& Conti, Valentina…[et al.]. 2014. Idiopathic Non Cirrhotic Portal Hypertension and Spleno-Portal Axis Abnormalities in Patients with Severe Primary Antibody Deficiencies. Journal of Immunology Research،Vol. 2014, no. 2014, pp.1-8.
https://search.emarefa.net/detail/BIM-1040944
Modern Language Association (MLA)
Pulvirenti, Federica…[et al.]. Idiopathic Non Cirrhotic Portal Hypertension and Spleno-Portal Axis Abnormalities in Patients with Severe Primary Antibody Deficiencies. Journal of Immunology Research No. 2014 (2014), pp.1-8.
https://search.emarefa.net/detail/BIM-1040944
American Medical Association (AMA)
Pulvirenti, Federica& Pentassuglio, Ilaria& Milito, Cinzia& Valente, Michele& De Santis, Adriano& Conti, Valentina…[et al.]. Idiopathic Non Cirrhotic Portal Hypertension and Spleno-Portal Axis Abnormalities in Patients with Severe Primary Antibody Deficiencies. Journal of Immunology Research. 2014. Vol. 2014, no. 2014, pp.1-8.
https://search.emarefa.net/detail/BIM-1040944
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1040944