Portal vein thrombosis leading to pre-sinusoidal non-cirrhotic portal hypertension resulting in decreased synthetic function of the liver

Kevin Kline, Muhannad Al Hanayneh, Mohammad Bilal, Heather Stevenson-Lerner

Research output: Contribution to journalArticle

Abstract

Non-cirrhotic portal hypertension (NCPH), defined as elevated portal pressures in the absence of cirrhosis, is a relatively rare cause of elevated portal pressures in western countries. In NCPH decompensated liver disease is common, but complications are often mitigated by appropriate medical therapy. Liver synthetic function loss is uncommon. We present a unique case of a patient with biopsy proven NCPH, who eventually developed progressive loss of hepatic synthetic function in the setting of long standing portal hypertension. This loss of synthetic function corresponded with the interval development of incomplete septal cirrhosis (ISC), and progression of previously noted nodular regenerative hyperplasia in biopsies performed 7 years apart. Our patient’s clinical course was complicated by multiple hospitalizations for gastrointestinal hemorrhage. Patients with ISC have higher rates of bleeding varices when compared to patients with macronodular cirrhosis. While patients with NCPH typically have better overall survival and fewer bleeding complications than cirrhotic patients, this is typically attributed to the former having preserved synthetic function. It appears that the presence of ISC may be a poor prognosticator in patients with NCPH.

Original languageEnglish (US)
Pages (from-to)174-177
Number of pages4
JournalGastroenterology and Hepatology from Bed to Bench
Volume12
Issue number2
DOIs
StatePublished - Jan 1 2019

Fingerprint

Portal Hypertension
Portal Vein
Thrombosis
Fibrosis
Liver
Portal Pressure
Hemorrhage
Biopsy
Gastrointestinal Hemorrhage
Varicose Veins
Hyperplasia
Liver Diseases
Hospitalization
Survival

Keywords

  • Gastrointestinal hemorrhage
  • Pathology
  • Portal hypertension

ASJC Scopus subject areas

  • Hepatology
  • Gastroenterology

Cite this

Portal vein thrombosis leading to pre-sinusoidal non-cirrhotic portal hypertension resulting in decreased synthetic function of the liver. / Kline, Kevin; Al Hanayneh, Muhannad; Bilal, Mohammad; Stevenson-Lerner, Heather.

In: Gastroenterology and Hepatology from Bed to Bench, Vol. 12, No. 2, 01.01.2019, p. 174-177.

Research output: Contribution to journalArticle

@article{f84c6c77866240ef97a8a979f406530a,
title = "Portal vein thrombosis leading to pre-sinusoidal non-cirrhotic portal hypertension resulting in decreased synthetic function of the liver",
abstract = "Non-cirrhotic portal hypertension (NCPH), defined as elevated portal pressures in the absence of cirrhosis, is a relatively rare cause of elevated portal pressures in western countries. In NCPH decompensated liver disease is common, but complications are often mitigated by appropriate medical therapy. Liver synthetic function loss is uncommon. We present a unique case of a patient with biopsy proven NCPH, who eventually developed progressive loss of hepatic synthetic function in the setting of long standing portal hypertension. This loss of synthetic function corresponded with the interval development of incomplete septal cirrhosis (ISC), and progression of previously noted nodular regenerative hyperplasia in biopsies performed 7 years apart. Our patient’s clinical course was complicated by multiple hospitalizations for gastrointestinal hemorrhage. Patients with ISC have higher rates of bleeding varices when compared to patients with macronodular cirrhosis. While patients with NCPH typically have better overall survival and fewer bleeding complications than cirrhotic patients, this is typically attributed to the former having preserved synthetic function. It appears that the presence of ISC may be a poor prognosticator in patients with NCPH.",
keywords = "Gastrointestinal hemorrhage, Pathology, Portal hypertension",
author = "Kevin Kline and {Al Hanayneh}, Muhannad and Mohammad Bilal and Heather Stevenson-Lerner",
year = "2019",
month = "1",
day = "1",
doi = "10.22037/ghfbb.v12i2.1402",
language = "English (US)",
volume = "12",
pages = "174--177",
journal = "Gastroenterology and Hepatology from Bed to Bench",
issn = "2008-2258",
publisher = "Shahid Beheshti Medical University",
number = "2",

}

TY - JOUR

T1 - Portal vein thrombosis leading to pre-sinusoidal non-cirrhotic portal hypertension resulting in decreased synthetic function of the liver

AU - Kline, Kevin

AU - Al Hanayneh, Muhannad

AU - Bilal, Mohammad

AU - Stevenson-Lerner, Heather

PY - 2019/1/1

Y1 - 2019/1/1

N2 - Non-cirrhotic portal hypertension (NCPH), defined as elevated portal pressures in the absence of cirrhosis, is a relatively rare cause of elevated portal pressures in western countries. In NCPH decompensated liver disease is common, but complications are often mitigated by appropriate medical therapy. Liver synthetic function loss is uncommon. We present a unique case of a patient with biopsy proven NCPH, who eventually developed progressive loss of hepatic synthetic function in the setting of long standing portal hypertension. This loss of synthetic function corresponded with the interval development of incomplete septal cirrhosis (ISC), and progression of previously noted nodular regenerative hyperplasia in biopsies performed 7 years apart. Our patient’s clinical course was complicated by multiple hospitalizations for gastrointestinal hemorrhage. Patients with ISC have higher rates of bleeding varices when compared to patients with macronodular cirrhosis. While patients with NCPH typically have better overall survival and fewer bleeding complications than cirrhotic patients, this is typically attributed to the former having preserved synthetic function. It appears that the presence of ISC may be a poor prognosticator in patients with NCPH.

AB - Non-cirrhotic portal hypertension (NCPH), defined as elevated portal pressures in the absence of cirrhosis, is a relatively rare cause of elevated portal pressures in western countries. In NCPH decompensated liver disease is common, but complications are often mitigated by appropriate medical therapy. Liver synthetic function loss is uncommon. We present a unique case of a patient with biopsy proven NCPH, who eventually developed progressive loss of hepatic synthetic function in the setting of long standing portal hypertension. This loss of synthetic function corresponded with the interval development of incomplete septal cirrhosis (ISC), and progression of previously noted nodular regenerative hyperplasia in biopsies performed 7 years apart. Our patient’s clinical course was complicated by multiple hospitalizations for gastrointestinal hemorrhage. Patients with ISC have higher rates of bleeding varices when compared to patients with macronodular cirrhosis. While patients with NCPH typically have better overall survival and fewer bleeding complications than cirrhotic patients, this is typically attributed to the former having preserved synthetic function. It appears that the presence of ISC may be a poor prognosticator in patients with NCPH.

KW - Gastrointestinal hemorrhage

KW - Pathology

KW - Portal hypertension

UR - http://www.scopus.com/inward/record.url?scp=85071149544&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85071149544&partnerID=8YFLogxK

U2 - 10.22037/ghfbb.v12i2.1402

DO - 10.22037/ghfbb.v12i2.1402

M3 - Article

VL - 12

SP - 174

EP - 177

JO - Gastroenterology and Hepatology from Bed to Bench

JF - Gastroenterology and Hepatology from Bed to Bench

SN - 2008-2258

IS - 2

ER -