A single centre experience of liver disease in adults with cystic fibrosis 1995–2006

Nash, Kathryn; Allison, Michael; McKeon, Damian; Lomas, David J.; Haworth, C.S.; Bilton, Diana; Alexander, Graeme

doi:10.1016/j.jcf.2007.10.004

Cited by 63 publications

(48 citation statements)

References 37 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Further analysis from UNOS shows good survival at 1 year and 5 years post transplant of 84% and 76% respectively (Arnon et al, 2011). This represents the largest review of outcomes following transplants for CFLD and supports the findings of previous smaller studies (Friedell et al, 2003;Melzi et al, 2006;Nash et al, 2008). However, overall survival post liver transplant for CFLD is less than that seen in non-CF patients who receive liver transplants for other indications.…”

Section: Liver Transplantationsupporting

confidence: 76%

Cystic Fibrosis Liver Disease

Low¹,

Jarad²

2012

Portal Hypertension - Causes and Complications

View full text Add to dashboard Cite

Section: Liver Transplantationsupporting

confidence: 76%

Cystic Fibrosis Liver Disease

Low¹,

Jarad²

2012

Portal Hypertension - Causes and Complications

View full text Add to dashboard Cite

“…Finally we recommend that patients are screened for early liver disease and if it is discovered they are prescribed ursodeoxycholic acid (42,43) and managed in a dedicated multi-disciplinary clinic with careful attention to nutrition and diabetes management. We have recently audited an 11-year experience of such an adult clinic in our own center and found a low incidence of progressive liver disease and no cases requiring liver transplantation (44).…”

Section: Cystic Fibrosis and Liver Transplantationmentioning

confidence: 99%

Cystic Fibrosis Liver Disease: To Transplant or Not to Transplant?

Nash

Collier

French

et al. 2008

American Journal of Transplantation

Self Cite

View full text Add to dashboard Cite

Biliary cirrhosis complicates some adults with cystic fibrosis (CF) and may require transplantation. Cardiorespiratory disease severity varies such that patients may require liver transplantation, heart/lung/liver (triple) grafts or may be too ill for any procedure. A 15-year experience of adults with CF-related liver disease referred for liver transplantation is presented with patient survival as outcome. Twelve patients were listed for triple grafting. Four died of respiratory disease after prolonged waits (4-171 weeks). Eight underwent transplantation (median wait 62 weeks); 5-year actuarial survival was 37.5%. Four died perioperatively; only one is alive at 8-years. Eighteen patients underwent liver transplant alone (median wait 7 weeks); 1-and 5-year actuarial survival rates were 100% and 69%. Three long-term survivors required further organ replacement (two heart/lung and one renal). Two others were turned down for heart/lung transplantation and four have significant renal impairment. Results for triple grafting were poor with unacceptable waiting times. Results for liver transplant alone were satisfactory, with acceptable waiting times and survival. However, further grafts were required and renal impairment was frequent. The policy of early liver transplantation for adults with CF with a view to subsequent heart/lung or renal transplantation needs assessment in the context of long-term outcome. Key words: Clinical liver transplantation, cystic fibrosisAbbreviations: ATG, anti-thymocyte globulin; BMI, body mass index; CF, cystic fibrosis; CNI, calcineurin inhibitor; FEV 1 , forced expiratory volume in 1 second; HIV, human immunodeficiency virus; IVC, inferior vena cava; MELD, model for end-stage liver disease; TIPS, transjugular intrahepatic porto-systemic shunt; UK, United Kingdom.

show abstract

“…(26) Recent reports suggest that focused care and adequate screening have contributed to maintaining clinical stability in adult CF patients with liver abnormalities. (27) Few studies have addressed the prevalence and natural history of renal disease in adults with CF. However, clinical reporting of renal abnormalities is increasing, and the determination of urinary protein excretion could prove useful in identifying CF patients at risk for progressive renal injury.…”

Section: Resultsmentioning

confidence: 99%

Proteinuria in cystic fibrosis: a possible correlation between genotype and renal phenotype

Cemlyn-Jones

Gamboa

2009

J. bras. pneumol.

View full text Add to dashboard Cite

Objective: To assess proteinuria in patients with cystic fibrosis (CF), and to correlate proteinuria with genotype, CF-related diabetes and disease severity. Methods: A prospective study was carried out over a six-month period and involving 22 CF patients. After the collection and analysis of 24-h urine samples, the patients were divided into two subgroups: protein excretion < 150 mg/day (low-proteinuria); and protein excretion ≥ 150 mg/day (highproteinuria). Patient charts were reviewed to obtain data on genotype and CF-related diabetes. Disease severity was assessed based on acute exacerbations in the last six months and FEV 1 measured during the study period. To assess the correlation between genotype and proteinuria, the two main mutations (ΔF508 and R334W) were evaluated. Due to the existence of genotype ΔF508/R334W, two categories were created to enable statistical analysis, ΔF508 being evaluated in category 1 and R334W being evaluated in category 2. Results: The ΔF508 mutation tended to be associated with normal protein excretion: 100% of the low-proteinuria subgroup patients were considered ΔF508 in category 1, compared with 86.7% in category 2. Protein excretion tended to be higher in patients with the R334W mutation: 60.0% of the high-proteinuria subgroup patients were considered R334W in category 1, compared with 80.0% in category 2 (p = 0.009 and p = 0.014, respectively). No significant association was found for any of the other variables. Conclusions: The results suggest that genotype is associated with renal phenotype, depending on the mechanism by which the genotype alters the function of the cystic fibrosis transmembrane conductance regulator gene.Keywords: Proteinuria; Cystic fibrosis; Genotype. ResumoObjetivo: Avaliar a proteinúria em pacientes com fibrose cística (FC) e correlacioná-la com o genótipo, com a diabetes relacionada à FC e com a gravidade da doença. Métodos: Estudo prospectivo realizado num período de seis meses com 22 pacientes com FC. Efetuada proteinúria de 24 h com a divisão dos pacientes em dois subgrupos: proteinúria < 150 mg/dia (proteinúria-baixa); e proteinúria ≥ 150 mg/dia (proteinúria-alta). Revisamos os prontuá-rios clínicos para a coleta de informações sobre o genótipo e a presença de diabetes relacionada à FC. A gravidade da doença foi avaliada pelas exacerbações agudas no último semestre e pelo VEF 1 durante o período de estudo. Para avaliar a correlação entre genótipo e proteinúria, consideraram-se as duas principais mutações, ΔF508 e R334W. Dada a existência do genótipo ΔF508/R334W, foram criadas duas categorias para se proceder à avaliação estatística, sendo esse genótipo considerado ΔF508 na categoria 1 e R334W na categoria 2. Resultados: A mutação ΔF508 se associou com valores normais de proteinúria: 100% dos pacientes do subgrupo proteinúria-baixa foram considerados ΔF508 na categoria 1, comparados a 86,7% na categoria 2. Em pacientes com a mutação R334W, os valores de proteinuria foram mais elevados: 60,0% dos pacientes do subgrupo proteinúria-alta for...

show abstract

A single centre experience of liver disease in adults with cystic fibrosis 1995–2006

Abstract: Liver disease was common in adults with CF but disease progression was rare. Thus liver disease detected and closely monitored in adults appeared to have a milder course than childhood CF. Splenomegaly, unrelated to portal hypertension may be a consequence of CF.

Cited by 63 publications

References 37 publications

Cystic Fibrosis Liver Disease

Cystic Fibrosis Liver Disease

Cystic Fibrosis Liver Disease: To Transplant or Not to Transplant?

Proteinuria in cystic fibrosis: a possible correlation between genotype and renal phenotype

Contact Info

Product

Resources

About