Obliterative Portal Venopathy Without Cirrhosis Is Prevalent in Pediatric Cystic Fibrosis Liver Disease With Portal Hypertension

Wu, Hao; Vu, Megan; Dhingra, Sadhna; Ackah, Ruth; Goss, John A.; Rana, Abbas; Quintanilla, Norma; Patel, Kalyani R.; Leung, Daniel H.

doi:10.1016/j.cgh.2018.10.046

Cited by 53 publications

(57 citation statements)

References 7 publications

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“…Indeed, liver disease arising in patients with CF is a rare indication for LT. (26) The pathogenesis of portal hypertension in CF liver disease has recently been reappraised. Although some patients develop portal hypertension as a result of increased bile viscosity leading to bile duct obstruction, inflammation, proliferation, focal biliary fibrosis, and, ultimately, biliary cirrhosis, recent series (27)(28)(29) have shown that a significant proportion of patients develop noncirrhotic portal hypertension because of obstructive portal venopathy, which is characterized by narrowing, thrombosis, or nonvisibility of the intrahepatic portal vein branches. These patients present with extreme portal hypertensive manifestations characterized by an increased portal vein caliper measurement, large portosystemic collaterals, and severe splenomegaly.…”

Section: Discussionmentioning

confidence: 99%

Vascular Remodeling of Visceral Arteries Following Interruption of the Splenic Artery During Liver Transplantation

et al. 2019

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Splenic artery (SA) ligation can be performed during liver transplantation (LT) to avoid portal hyperperfusion, which is involved in the pathogenesis of both small‐for‐size and SA syndrome. The SA can also be used as an inflow for arterial reconstruction. Exceptionally, SA interruption or agenesis has been associated with positive remodeling of collateral arteries supplying the spleen via the left gastric artery (LGA), short gastric vessels, and the gastroepiploic arcade (GEA), with subsequent severe upper gastrointestinal (GI) bleeding. To determine incidence, magnitude, predictors, and clinical implications of vascular remodeling after SA interruption during LT, we identified 465 patients transplanted in the period 2007‐2017 who had the SA ligated or interrupted at LT. Among them, 88 had a computed tomography angiography suitable for evaluation of vascular remodeling after LT. The presence of prominent gastric arterial collaterals and the increase in LGA and GEA diameter were evaluated on 2‐dimensional axial images and multiplanar reconstructions. Of the 88 patients, 28 (31.8%), 32 (36.4%), and 22 (25.0%) developed gastric collateralization graded as mild, moderate, or severe. Of the patients for whom comparison with pre‐LT imaging was possible (n = 54), 51 (94.4%) presented a median 37% and 55% increase in LGA and GEA diameter, respectively. Severe gastric collateralization was associated with lower body mass index (odds ratio, 0.84; 95% confidence interval [CI], 0.71‐0.98; P = 0.03), whereas a GEA caliper measurement increase was positively correlated with Model for End‐Stage Liver Disease score (r2 = 0.12; 95% CI, 0.65‐4.15; P = 0.008). Out of 465 patients, 2 (0.43%) had severe episodes of arterial upper GI bleeding, possibly exacerbated by vascular remodeling. In conclusion, vascular remodeling after SA interruption during LT is frequent and can aggravate GI bleeding during follow‐up.

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Section: Discussionmentioning

confidence: 99%

Vascular Remodeling of Visceral Arteries Following Interruption of the Splenic Artery During Liver Transplantation

et al. 2019

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“…However, it needs to be taken into account that portal hypertension in patients with CFLD often occurs in the absence of liver cirrhosis and the presence of preserved liver function [ 29 ]. Non-cirrhotic portal hypertension in CFLD was found to be associated with portal branch venopathy in small case series [ 30 , 31 ]. In these patients, transjugular intrahepatic portosystemic shut can be a therapeutic option.…”

Section: General Treatment Recommendations For Cfldmentioning

confidence: 99%

Current Treatment Options for Cystic Fibrosis-Related Liver Disease

Staufer

2020

IJMS

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Cystic Fibrosis-related liver disease (CFLD) has become a leading cause of morbidity and mortality in patients with Cystic Fibrosis (CF), and affects children and adults. The understanding of the pathogenesis of CFLD is key in order to develop efficacious treatments. However, it remains complex, and has not been clarified to the last. The search for a drug might be additionally complicated due to the diverse clinical picture and lack of a unified definition of CFLD. Although ursodeoxycholic acid has been used for decades, its efficacy in CFLD is controversial, and the potential of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulators and targeted gene therapy in CFLD needs to be defined in the near future. This review focuses on the current knowledge on treatment strategies for CFLD based on pathomechanistic viewpoints.

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“…This is quite different from findings with a cohort with CFLD, among whom the APRI correlated with extent of liver fibrosis, but patients with F3-F4 fibrosis had an APRI of only 0.75 (13). Similarly, Aqul et al found that median APRI was One contributor to the apparent discrepancy between CFLD phenotype and LS values may be the findings of several recent studies that demonstrate the importance of non-cirrhotic PHTN among patients with severe CLFD (44,45). In a series of 17 pediatric CFLD patients who underwent liver transplant for PHTN, nodular regenerative hyperplasia (NRH) without cirrhosis was seen in 94% of explants (44).…”

Section: Apri = Aspartate Aminotransferase To Platelet Ratio Index; Lmentioning

confidence: 75%

Role of transient elastography and APRI in the assessment of pediatric cystic fibrosis liver disease

Woolfson

Schreiber

Raveendran

et al. 2021

CanLivJ

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Background: Diagnosis and monitoring of cystic fibrosis liver disease (CFLD) is challenging. Transient elastography (TE) is a rapid, non-invasive method for assessing liver fibrosis. Its role in detecting fibrosis in CFLD has only begun to be explored. The aspartate aminotransferase to platelet ratio index (APRI) has been validated as a predictor of hepatic fibrosis in other chronic liver diseases. The purpose of this study was to assess the utility of APRI and TE in identifying liver fibrosis in pediatric CF patients. Methods: Patients aged 2–18 years were recruited from the British Columbia Children’s Hospital CF clinic. Patients were determined to have CFLD using standard criteria. Charts were reviewed, and each patient underwent TE. Results: Of the 55 patients included in the study (50.9% male, mean age 11.6 y), 22 (40%) had CFLD. All mean liver enzymes were higher in the CFLD group, notably alanine transaminase ( p = 0.031). Mean liver stiffness (LS) and APRI were also higher in the CFLD group (LS: 5.9 versus 4.5 kPa, p = 0.015; APRI: 0.40 versus 0.32, p = 0.119). Linear regression showed a mild positive association between the two ( r2 = 0.386). Conclusions: TE values were higher among CFLD patients and correlated with APRI values, suggesting that these tools may have clinical applications for identifying and following this population. Further research is needed on a larger scale to determine the relative value and clinical utility of TE and APRI among patients with CFLD.

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Obliterative Portal Venopathy Without Cirrhosis Is Prevalent in Pediatric Cystic Fibrosis Liver Disease With Portal Hypertension

Cited by 53 publications

References 7 publications

Vascular Remodeling of Visceral Arteries Following Interruption of the Splenic Artery During Liver Transplantation

Vascular Remodeling of Visceral Arteries Following Interruption of the Splenic Artery During Liver Transplantation

Current Treatment Options for Cystic Fibrosis-Related Liver Disease

Role of transient elastography and APRI in the assessment of pediatric cystic fibrosis liver disease

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