Non-cirrhotic portal hypertension (obliterative portal venopathy) is the predominant form of chronic liver disease in cystic fibrosis

ONeill, John P; Clifford, Cathal; Nolan, Niamh; McCormick, Peter

doi:10.1007/s11845-022-03153-0

Cited by 3 publications

(2 citation statements)

References 20 publications

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“…has attempted to answer this question by comparing explanted livers from adults with CFLD and two other classic cholestatic diseases (primary biliary cirrhosis [PBC] and PSC). 39 The study found differences in rates of cirrhosis (0/9 patients with CFLD vs. 11/14 in PBC/PSC group) and NRH (8/9 in CFLD vs. 0/14 in PBC/PSC group). These findings suggest a venous etiology of damage in CFLD that is distinct from other cholestatic liver disease.…”

Section: Pathogenesis Considerationsmentioning

confidence: 77%

“…The question remains whether the portal vein is affected by its close proximity to the damaged and inflamed biliary system or from the inflow of inflammatory products and mediators from the intestines. A recent study from Ireland by Oneill et al has attempted to answer this question by comparing explanted livers from adults with CFLD and two other classic cholestatic diseases (primary biliary cirrhosis [PBC] and PSC) 39 . The study found differences in rates of cirrhosis (0/9 patients with CFLD vs. 11/14 in PBC/PSC group) and NRH (8/9 in CFLD vs. 0/14 in PBC/PSC group).…”

Section: Pathogenesis Of Non‐cirrhotic Portal Hypertension and Oblite...mentioning

confidence: 99%

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Pathophysiology of Cystic Fibrosis Liver Disease

Kasper,

Assis

2024

Pediatric Pulmonology

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Hepatobiliary complications of Cystic Fibrosis (CF) constitute a significant burden for persons with CF of all ages, with advanced CF liver disease in particular representing a leading cause of mortality. The causes of the heterogeneity of clinical manifestations, ranging from steatosis to focal biliary cholestasis and biliary strictures, are poorly understood and likely reflect a variety of environmental and disease‐modifying factors in the setting of underlying CFTR mutations. This review summarizes the current understanding of the pathophysiology of hepatobiliary manifestations of CF, and discusses emerging disease models and therapeutic approaches that hold promise to impact this important yet incompletely addressed aspect of CF care.

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Section: Pathogenesis Considerationsmentioning

confidence: 77%

Section: Pathogenesis Of Non‐cirrhotic Portal Hypertension and Oblite...mentioning

confidence: 99%

Pathophysiology of Cystic Fibrosis Liver Disease

Kasper,

Assis

2024

Pediatric Pulmonology

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Management of liver disease and portal hypertension in cystic fibrosis: a review

Colombo,

Lanfranchi,

Tosetti

et al. 2024

Expert Review of Respiratory Medicine

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Single‐cell RNA sequencing of cystic fibrosis liver disease explants reveals endothelial complement activation

Declercq,

Treps,

Geldhof

et al. 2024

Liver International

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Background & AimsCystic fibrosis (CF) is considered a multisystemic disorder in which CF‐associated liver disease (CFLD) is the third most common cause of mortality. Currently, no effective treatment is available for CFLD because its pathophysiology is still unclear. Interestingly, CFLD exhibits identical vascular characteristics as non‐cirrhotic portal hypertension, recently classified as porto‐sinusoidal vascular disorders (PSVD).MethodsSince endothelial cells (ECs) are an important component in PSVD, we performed single‐cell RNA sequencing (scRNA‐seq) on four explant livers from CFLD patients to identify differential endothelial characteristics which could contribute to the disease. We comprehensively characterized the endothelial compartment and compared it with publicly available scRNA‐seq datasets from cirrhotic and healthy livers. Key gene signatures were validated ex vivo on patient tissues.ResultsWe found that ECs from CF liver explants are more closely related to healthy than cirrhotic patients. In CF patients we also discovered a distinct population of liver sinusoidal ECs—coined CF LSECs—upregulating genes involved in the complement cascade and coagulation. Finally, our immunostainings further validated the predominant periportal location of CF LSECs.ConclusionsOur work showed novel aspects of human liver ECs at the single‐cell level thereby supporting endothelial involvement in CFLD, and reinforcing the hypothesis that ECs could be a driver of PSVD. Therefore, considering the vascular compartment in CF and CFLD may help developing new therapeutic approaches for these diseases.

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Non-cirrhotic portal hypertension (obliterative portal venopathy) is the predominant form of chronic liver disease in cystic fibrosis

Cited by 3 publications

References 20 publications

Pathophysiology of Cystic Fibrosis Liver Disease

Pathophysiology of Cystic Fibrosis Liver Disease

Management of liver disease and portal hypertension in cystic fibrosis: a review

Single‐cell RNA sequencing of cystic fibrosis liver disease explants reveals endothelial complement activation

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