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ABSTRACT: Background
Internationally, Cystic fibrosis-associated liver disease (CFLD) is considered the third leading cause of death, following lung disease and transplantation complications.Aims
To identify the prevalence of CFLD in cystic fibrosis (CF) patients.Methodology
A retrospective chart review for all patients with CF liver disease from a tertiary care center.Result
A total of 341 CF patients were included. The mean age at the diagnosis of liver disease is 13.5 (7.6) years.The first elevated ALT was reported in 190/341 patients (56%), elevated AST in 124 patients (36%), elevated alkaline phosphatase (ALP) in 166 patients (49.1%), elevated GGT in 57 patients (23%), and elevated bilirubin in 24 patients (7%). There was an improvement of the liver enzyme values during the follow-up period, P-value = (<0.05).Ultrasound liver assessments were performed in 258/341 patients (75.7%). One hundred and twelve patients (43%) had abnormal findings. In 14 patients (5.4%), assessment exhibited advanced liver disease (liver cirrhosis and periportal fibrosis). Out of 190 patients, who were given ursodeoxycholic acid for elevated liver enzymes, 180 (94.7%) exhibited improvement. One patient underwent liver transplant at the age of 12. Four patients were submitted for liver biopsy; periportal fibrosis was observed in 4 patients (1.6%), and liver cirrhosis by ultrasound (US) in 10 patients (4%).Conclusion
Patients with CF should be screened early for liver enzymes, and should undergo the US study to detect liver disease at early stages and to prevent its progression.
SUBMITTER: Banjar H
PROVIDER: S-EPMC9152554 | biostudies-literature | 2022 Jun
REPOSITORIES: biostudies-literature
Banjar Hanaa H AbdulAziz Najlaa N Khader Jumana J Ghomraoui Firas F Alansari AbdulAziz A Al-Hoshan Abdulaziz A AlKaf Sara S Aldakheel Wajeeh W
International journal of pediatrics & adolescent medicine 20210622 2
<h4>Background</h4>Internationally, Cystic fibrosis-associated liver disease (CFLD) is considered the third leading cause of death, following lung disease and transplantation complications.<h4>Aims</h4>To identify the prevalence of CFLD in cystic fibrosis (CF) patients.<h4>Methodology</h4>A retrospective chart review for all patients with CF liver disease from a tertiary care center.<h4>Result</h4>A total of 341 CF patients were included. The mean age at the diagnosis of liver disease is 13.5 (7 ...[more]