Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2022 Jun;9(2):78-82.
doi: 10.1016/j.ijpam.2021.06.002. Epub 2021 Jun 22.

Liver disease in cystic fibrosis patients in a tertiary care center in Saudi Arabia

Hanaa Banjar  1 , Najlaa AbdulAziz  1 , Jumana Khader  2 , Firas Ghomraoui  3 , AbdulAziz Alansari  1 , Abdulaziz Al-Hoshan  4 , Sara AlKaf  5 , Wajeeh Aldakheel  1
Affiliations

Liver disease in cystic fibrosis patients in a tertiary care center in Saudi Arabia

Hanaa Banjar et al. Int J Pediatr Adolesc Med. 2022 Jun.

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.

Keywords: AST to platelet ratio index FIB-4, Fibrosis-4 scoring system US; CF, Cystic Fibrosis (CFTR) = Cystic Fibrosis Transmembrane Conductance Regulator Gene, Mutations.CFLD; CFTR; Cholestasis; Cystic fibrosis; Cystic fibrosis liver diseaseLD, Liver diseaseALT; Liver cirrhosis, Arabs; Liver disease; Liver function tests,INR, International normalized ratio; Ultrasound SD, Standard Deviation ALP; Ursodeoxycholic acid; alanine aminotransferaseAST, aspartate aminotransferase APRi; alkaline phosphatase GGT, gamma glutamyl transpeptidase, LFTs.

PubMed Disclaimer

References

    1. Catarino Costa P., Barreto C.C., Pereira L., Lobo M.L., Costa M.A., Gouveia Lopes A.I. Cystic fibrosis-related liver disease: a single-center experience. MDPI. 2011;3(3):21. - PMC - PubMed
    1. Banjar H., Angyalosi G. The road for survival improvement of cystic fibrosis patients in Arab countries. Int J Pediatr Adolesc Med. 2015;2(2):47–58. - PMC - PubMed
    1. Nazer H., Rahbeeni Z. Cystic fibrosis and the liver—a Saudi experience. Ann Trop Paediatr. 1994;14(3):189–194. - PubMed
    1. Bodewes F.A.J.A., van der Doef H.P.J., Houwen R.H.J., Verkade H.J. Increase of serum γ-glutamyltransferase associated with development of cirrhotic cystic fibrosis liver disease. J Pediatr Gastroenterol Nutr. 2015;61(1):113–118. - PubMed
    1. Bartlett J.R. Genetic modifiers of liver disease in cystic fibrosis. J Am Med Assoc. 2009;302(10):1076. - PMC - PubMed