Assessment of Progression Markers for Cirrhosis in Cystic Fibrosis Patients

Main Article Content

Gabriela Riter Martins de Matos Franciane Pavloski Kühl Guilherme Cecchetti Carolina Chaves Hahnemann Gustavo Lenci Marques Jean Rodrigo Tafarel

Abstract

Cystic Fibrosis is an autosomal dominant disease that affects the CFTR gene, which is responsible for producing a protein that regulates the transport of chloride ions across the cell membrane. This alteration results in obstruction in the body's ducts and channels, primarily affecting the pulmonary system. Additionally, other systems can be affected. Cystic Fibrosis-related liver disease is a common complication that can lead to steatosis, fibrosis, and multifocal biliary cirrhosis; however, there are no well-established diagnostic criteria yet. The present study was conducted at the Cystic Fibrosis Outpatient Clinic of the Hospital de Clínicas, Federal University of Paraná, examining 98 medical records of patients with cystic fibrosis for at least 10 years. Patients with hepatitis B, hepatitis C, alcohol consumption, or pregnancy were excluded. The objective was to determine the best predictor for the outcome of cirrhosis, and for this purpose, demographic parameters, laboratory tests, and imaging were collected. The statistical analysis was performed using SPSS® software, with quantitative variables described as mean ± standard deviation and categorical variables presented as the number of observations and percentage. ROC curves were adjusted to determine the best predictor, using the Youden index to establish the optimal cutoff point and the corresponding area under the curve to determine the model's efficacy. The study included 80 patients, and 10 of them (12.5%) were diagnosed with Cystic Fibrosis-related cirrhosis. The average age of the cirrhosis group was 21.6 years, with a predominance of males. The most accurate parameter for predisposition to cirrhosis was the APRI score, with a cutoff value of 0.27. Univariate analysis showed that gender, hepatic changes on ultrasound, splenomegaly, increased portal vein diameter, presence of vomiting, levels of alanine transaminase, aspartate transaminase, platelet count, and FIB-4 score were significantly associated with an APRI score value greater than 0.27. This study suggests the hypothesis that the APRI score is the most sensitive and specific tool for defining cirrhosis in patients with cystic fibrosis.

Article Details

How to Cite
DE MATOS, Gabriela Riter Martins et al. Assessment of Progression Markers for Cirrhosis in Cystic Fibrosis Patients. Medical Research Archives, [S.l.], v. 12, n. 3, mar. 2024. ISSN 2375-1924. Available at: <https://esmed.org/MRA/mra/article/view/5203>. Date accessed: 13 apr. 2024. doi: https://doi.org/10.18103/mra.v12i3.5203.
Section
Research Articles

References

1. Sakiani S, Kleiner D, Heller T. Hepatic manifestations of cystic fibrosis. Clin Liver Dis. 2019 May; 23 (2): 263-277. doi: 10.1016/j.cld.2018.12.008.
2. Castellani C, Assael BM. Cystic fibrosis: a clinical view. Cell Mol Life Sci, 2017 Jan; 74 (1):129-140. doi: 10.1007/s00018-016-2393-9.
3. Van De Peppel IP, Bertolini A, Jonker JW, Bodewes FAJA, Verkade HJ. Diagnosis, follow-up and treatment of cystic fibrosis-related liver disease. Curr Opin Pulm Med. 2017 Nov; 23 (6): 562-569.
doi: 10.1097/MCP.0000000000000428.
4. Ringshausen FC, Hellmuth T, Dittrich AM. Evidence-based treatment of cystic fibrosis, Internist (Berl). 2020 Dec; 61 (12):1212-1229. doi: 10.1007/s00108-020-00896-9.
5. Leung DH, Khan M, Minard CG, Guffey D, Ramm LE, Clouston AD, et al. Aspartate aminotransferase to platelet ratio and fibrosis-4 as biomarkers in biopsy-validated pediatric cystic fibrosis liver disease. Hepatology, 2015 Nov; 62 (5):1576-83.
doi: 10.1002/hep.28016.
6. Kitson MT, Kemp WW, Iser DM, Paul E, Wilson JW, Roberts SK. Utility of transient elastography in the non-invasive evaluation of cystic fibrosis liver disease. Liver Int. 2013 May; 33 (5): 698-705. doi: 10.1111/liv.12113.
7. Efrati O, Barak A, Modan-Moses D, Augarten A, Vilozni D, Katznelson D, Szeinberg A, et al. Liver cirrhosis and portal hypertension in cystic fibrosis. Eur J Gastroenterol Hepatol. 2003 Oct; 15 (10): 1073-1078. doi: 10.1097/00042737-200310000-00002.
8. Boelle PY, Debray D, Guillot L, Clement A, Corvol H. Cystic fibrosis liver disease: outcomes and risk factors in a large cohort of french patients. Hepatology. 2019 Apr; 69 (4): 1648-1656. doi: 10.1002/hep.30148.
9. Fagundes EDT, Roquete MLV, Penna FJ, Reis FJC, Goulart EMA, Duque C. Risk factors for cystic fibrosis related liver disease. Jornal De Pediatria, 2005; 81 (6): 478-484. doi.0021-7557/05/81-06/478.
10. Flass T, Narkewicz MR. Cirrhosis and other liver disease in cystic fibrosis. J Cyst Fibros. 2013 Mar; 12 (2): 116-124.
doi: 10.1016/j.jcf.2012.11.010.
11. Amaral ISA, Dias MPF, Fernandes CCR, Moia LJMP, Miranda ECBM, Demachki S. Evaluation of apri test as liver fibrosis marker. Rev Para Med 2007 Dec; 21 (4). URL: http://scielo.iec.gov.br/pdf/rpm/v21n4/v21n4a02.pdf
12. Vendrusculo FM, Donadio MVF, Pinto LA. Achievements related to the survival of patients with cystic fibrosis in Brazil. J Bras Pneumol. 2021; 47 (2): e20210140.
doi 10.36416/1806-3756/E/20210140.