Hepatobiliary disease in children and adolescents with cystic fibrosis

Nascimento, Fernanda de S.; Sena, Nelson A.; Ferreira, Tatiane; Silva, Luciana Rodrígues; Souza, Edna Lúcia

doi:10.1016/j.jped.2017.07.006

Cited by 11 publications

(3 citation statements)

References 16 publications

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“…This raises the question of whether earlier antibiotic exposure, earlier infection or longer states of inflammation modify the risk of developing fibrosis or portal hypertension via mechanisms such as nodular regenerative hyperplasia 38 . In a prior large multi‐center study, it was reported that earlier P. aeruginosa infection may be protective from development of abnormal liver ultrasound patterns among children with CF, 39 making our observations of particular interest.…”

Section: Discussionmentioning

confidence: 87%

Risk factors for more rapid progression of severe liver fibrosis in children with cystic fibrosis‐related liver disease: A multi‐center study validated by liver biopsy

Sakhuja

Staples

Minard

et al. 2023

Liver International

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Background and Aims: Early identification of risk factors for the development of severe fibrosis in children with cystic fibrosis-related liver disease (CFLD) is crucial as promising therapies emerge. Methods:This multi-center cohort study of children with a priori defined CFLD from 1999 to 2016, was designed to evaluate the clinical utility of CF-specific characteristics and liver biomarkers assessed years prior to liver biopsy-proven CFLD to predict risk of developing severe fibrosis (F3-4) over time. Fibrosis was staged by Metavir classification. Results:The overall study cohort of 42 patients (F0-2 (n = 22) and F3-4 (n = 20)) was 57% male (n = 24) with median age of 7.6 years at baseline visit versus 10.3 years at biopsy. Median FEV 1 % predicted was lower in F3-4 participants at baseline versus F0-2 (59% vs. 85%; p = .002), while baseline FIB-4, APRI and GGT were higher in F3-4.Median splits for FIB-4 (≥.13), APRI (≥.36), GPR (≥.09), GGT (≥25.5), and FEV 1 % (<64%) were associated with more rapid progression to F3-4 (p < .01 for all). Using a combination of change/year in FIB-4, APRI, and GPR to predict F3-4, the AUROC was .81 (95% CI, .66, .96; p < .0001). For up to 5.8 years prior, thresholds for GPR were met 6.5-fold more rapidly, and those for APRI and FIB-4 were met 2.5-fold more rapidly, in those who progressed to F3-4 than those that did not. Conclusions:This study suggests mild-moderate pulmonary dysfunction and higher liver biomarker indices at baseline may be associated with faster progression of CFLD.

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Section: Discussionmentioning

confidence: 87%

Risk factors for more rapid progression of severe liver fibrosis in children with cystic fibrosis‐related liver disease: A multi‐center study validated by liver biopsy

Sakhuja

Staples

Minard

et al. 2023

Liver International

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“…The frequency of hepatic involvement observed in this study (62.3%) was greater than that described by Salvatore et al (5.7% in children) [ 28 ]. In a Brazilian study, hepatobiliary disease was diagnosed in 16.4% of the patients, and it occurred as an initial manifestation of CF in 55.6% of these cases [ 29 ]. This variation in prevalence may be justified by the difference in the methods used for the diagnosis of liver disease associated with CF.…”

Section: Discussionmentioning

confidence: 99%

Cystic Fibrosis: Clinical Phenotypes in Children and Adolescents

Santos

Nogueira

et al. 2018

Pediatr Gastroenterol Hepatol Nutr

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PurposeThe objective of this study was to describe the clinical phenotypes of children and adolescents with cystic fibrosis (CF); and to assess the role of pancreatic insufficiency and neonatal screening in diagnosis.MethodsA cross-sectional study was conducted, which included 77 patients attending a reference center of CF between 2014 and 2016. Epidemiological data, anthropometric measurements, and the presence of pulmonary, pancreatic, gastrointestinal and hepatobiliary manifestations were evaluated based on clinical data and complementary examinations.ResultsOf the 77 patients, 51.9% were male, with a median age of 147 months (7.0–297.0 months), and the majority showed adequate nutritional status. The most common phenotype was pulmonary (92.2%), followed by pancreatic (87.0%), with pancreatic insufficiency in most cases. Gastrointestinal manifestation occurred in 46.8%, with constipation being the more common factor. Hepatobiliary disease occurred in 62.3% of patients. The group with pancreatic insufficiency was diagnosed earlier (5.0 months) when compared to the group with sufficiency (84.0 months) (p=0.01). The age of diagnosis was reduced following implementation of neonatal screening protocols for CF (6.0 months before vs. 3.0 months after, p=0.02).ConclusionThe pulmonary phenotype was the most common, although extrapulmonary manifestations were frequent and clinically relevant, and should mandate early detection and treatment. Neonatal screening for CF led to earlier diagnosis in patients with pancreatic failure, and therefore, should be adopted universally.

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“…At this level, proliferation of bile ducts and chronic inflammation, associated with fibrosis, occurs. Focal bile duct fibrosis is a characteristic feature of CF and is being discussed in cases of prolonged neonatal jaundice [48]. The macroscopic examination reveals an abundant mucoid material in the lumen of the intra- and extrahepatic bile ducts, as well as in the gallbladder’s lumen, where calculi can also appear.…”

Section: Pain In Cfmentioning

confidence: 99%

Current Practices and Potential Nanotechnology Perspectives for Pain Related to Cystic Fibrosis

Trandafir

Leon

Frăsinariu

et al. 2019

JCM

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Pain is a complex, multidimensional process that negatively affects physical and mental functioning, clinical outcomes, quality of life, and productivity for cystic fibrosis (CF) patients. CF is an inherited multi-system disease that requires a complete approach in order to evaluate, monitor and treat patients. The landscape in CF care has changed significantly, with currently more adult patients than children worldwide. Despite the great advances in supportive care and in our understanding regarding its pathophysiology, there are still numerous aspects of CF pain that are not fully explained. This review aims to provide a critical overview of CF pain research that focuses on pain assessment, prevalence, characteristics, clinical association and the impact of pain in children and adults, along with innovative nanotechnology perspectives for CF management. Specifically, the paper evaluates the pain symptoms associated with CF and examines the relationship between pain symptoms and disease severity. The particularities of gastrointestinal, abdominal, musculoskeletal, pulmonary and chest pain, as well as pain associated with medical procedures are investigated in patients with CF. Disease-related pain is common for patients with CF, suggesting that pain assessment should be a routine part of their clinical care. A summary of the use of nanotechnology in CF and CF-related pain is also given. Further research is clearly needed to better understand the sources of pain and how to improve patients’ quality of life.

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Hepatobiliary disease in children and adolescents with cystic fibrosis

Cited by 11 publications

References 16 publications

Risk factors for more rapid progression of severe liver fibrosis in children with cystic fibrosis‐related liver disease: A multi‐center study validated by liver biopsy

Risk factors for more rapid progression of severe liver fibrosis in children with cystic fibrosis‐related liver disease: A multi‐center study validated by liver biopsy

Cystic Fibrosis: Clinical Phenotypes in Children and Adolescents

Current Practices and Potential Nanotechnology Perspectives for Pain Related to Cystic Fibrosis

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