title: Idiopathic non cirrhotic portal hypertension and spleno-portal axis abnormalities in patients with severe primary antibody deficiencies.
abstract: OBJECTIVE
  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.
authors.affiliation: Department of Molecular Medicine, Sapienza University of Rome, Viale dell'Università 37, 00186 Rome, Italy.
authors.forename: Federica
authors.initials: F
authors.lastname: Pulvirenti
authors.affiliation: Gastroenterology, Department of Clinical Medicine, Sapienza University of Rome, Rome, Italy.
authors.forename: Ilaria
authors.initials: I
authors.lastname: Pentassuglio
authors.affiliation: Department of Molecular Medicine, Sapienza University of Rome, Viale dell'Università 37, 00186 Rome, Italy.
authors.forename: Cinzia
authors.initials: C
authors.lastname: Milito
authors.affiliation: Department of Experimental Medicine and Pathology, Sapienza University of Rome, Rome, Italy.
authors.forename: Michele
authors.initials: M
authors.lastname: Valente
authors.affiliation: Gastroenterology, Department of Clinical Medicine, Sapienza University of Rome, Rome, Italy.
authors.forename: Adriano
authors.initials: A
authors.lastname: De Santis
authors.affiliation: Department of Molecular Medicine, Sapienza University of Rome, Viale dell'Università 37, 00186 Rome, Italy.
authors.forename: Valentina
authors.initials: V
authors.lastname: Conti
authors.affiliation: Department of Experimental Medicine and Pathology, Sapienza University of Rome, Rome, Italy.
authors.forename: Giulia
authors.initials: G
authors.lastname: d'Amati
authors.affiliation: Gastroenterology, Department of Clinical Medicine, Sapienza University of Rome, Rome, Italy.
authors.forename: Oliviero
authors.initials: O
authors.lastname: Riggio
authors.affiliation: Department of Molecular Medicine, Sapienza University of Rome, Viale dell'Università 37, 00186 Rome, Italy.
authors.forename: Isabella
authors.initials: I
authors.lastname: Quinti
mesh_terms: D000328:Adult; D000361:Agammaglobulinemia / Q000150:complications / Q000276:immunology / Q000473:pathology*; D017074:Common Variable Immunodeficiency / Q000150:complications / Q000276:immunology / Q000473:pathology*; D005260:Female; D040181:Genetic Diseases, X-Linked / Q000150:complications / Q000276:immunology / Q000473:pathology*; D006503:Hepatic Veins / Q000276:immunology / Q000473:pathology; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications / Q000276:immunology / Q000473:pathology*; D008099:Liver / Q000098:blood supply / Q000276:immunology / Q000473:pathology; D008103:Liver Cirrhosis / Q000150:complications / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D010198:Pancytopenia / Q000150:complications / Q000276:immunology / Q000473:pathology*; D011169:Portal Vein / Q000276:immunology / Q000473:pathology; D011446:Prospective Studies; D013154:Spleen / Q000098:blood supply / Q000276:immunology / Q000473:pathology; D013163:Splenomegaly / Q000150:complications / Q000276:immunology / Q000473:pathology*; D000094724:Idiopathic Noncirrhotic Portal Hypertension
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