☆ 4.7 Article

Features of Severe Liver Disease With Portal Hypertension in Patients With Cystic Fibrosis

CLINICAL GASTROENTEROLOGY AND HEPATOLOGY (2016)

Journal

CLINICAL GASTROENTEROLOGY AND HEPATOLOGY

Volume 14, Issue 8, Pages 1207-+

Publisher

ELSEVIER SCIENCE INC

DOI: 10.1016/j.cgh.2016.03.041

Keywords

CFLD; ALT; Phenotype; Portal Pressure; INR

Funding

Cystic Fibrosis Foundation [STONEB12I0, KNOWLE00A0]
National Institutes of Health [DK066368, HL068890]
Agence Nationale de la Recherche [R09186DS]
Direction Generale de la Sante
Vaincre La Mucoviscidose
Association Agir et Informer Contre la Mucoviscidose
CF Canada
Genome Canada
Ontario Research Fund
Lloyd Harris Carr Foundation

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Abstract

BACKGROUND & AIMS: Liver disease is the third leading cause of death in patients with cystic fibrosis (CF), but features of patients with CF, severe liver disease, and portal hypertension have not been characterized fully. METHODS: We performed a retrospective analysis of data from 561 patients with CF (63% male, 99% with pancreatic insufficiency), liver disease (hepatic parenchymal abnormalities consistent with cirrhosis, confirmed by imaging), and portal hypertension (esophageal varices, portosystemic collaterals, or splenomegaly), with no alternate causes of liver disease. All patients were enrolled in the Genetic Modifier Study of Severe CF Liver Disease at 76 international centers, from January 1999 through July 2013. RESULTS: Male patients were diagnosed with liver disease at a younger age than female patients (10 vs 11 y; P=.01). Splenomegaly was observed in 99% of patients and varices in 71%. Levels of liver enzymes were near normal in most patients. Thrombocytopenia affected 70% of patients and was more severe in patients with varices (88 x 10(9)/L vs 145 x 10(9)/L; P<.0001). Ninety-one patients received liver transplants (16%), at a median age of 13.9 years. Compared with patients who did not receive liver transplants, patients who received liver transplants had lower platelet counts (78 x 10(9)/L vs 113 x 10(9)/L; P<.0001), higher international normalized ratios (P<.0001), and lower levels of albumin (P=.0002). The aminotransferase to platelet ratio index (APRi) and fibrosis index based on 4 factor (FIB-4) values were higher than the diagnostic thresholds for CF liver disease in 96% and in 90% of patients, respectively. Patients who received liver transplants or who had varices had higher APRi and FIB-4 values than patients who did not. CONCLUSIONS: In patients with CF, severe liver disease develops early in childhood (approximately 10 years of age), and is more common in boys than in girls. Patients with varices and those who receive liver transplants have more abnormal platelet counts and APRi and FIB-4 scores.

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Circulating microbiome in patients with portal hypertension

Rolandas Gedgaudas, Jasmohan S. Bajaj, Jurgita Skieceviciene, Greta Varkalaite, Gabija Jurkeviciute, Sigita Gelman, Irena Valantiene, Romanas Zykus, Andrius Pranculis, Corinna Bang, Andre Franke, Christoph Schramm, Juozas Kupcinskas

Summary: This study explored the circulating bacterial DNA signatures, inflammatory cytokines, and gut permeability markers in different blood compartments of patients with cirrhosis and portal hypertension (PH). The results showed that the circulating plasma microbiome profiles in patients with cirrhosis were distinct from those of the controls and were associated with PH severity, liver function, and inflammation biomarkers. However, the composition of the circulating microbial profile failed to predict the severity of PH.

GUT MICROBES (2022)