https://pubmed.ncbi.nlm.nih.gov/39675556/ cystic fibrosis

Gastroenterol Hepatol. 2024 Dec 13:502321.

doi: 10.1016/j.gastrohep.2024.502321.Online ahead of print.

Assessment of cystic fibrosis related liver disease in a pediatric cohort

[Article in English, Spanish]

S Vicente-Santamaría 1, M E Torres-Guerrero 2, M García-González 3, A Tabares-González 4, C Gascón-Galindo 1, C M López-Cárdenes 1, E Blitz-Castro 1, A Morales-Tirado 1, M I Mota Goitia 5, J R Gutiérrez Martínez 6, C Tutau Gómez 7, R García Romero 8, E Salcedo Lobato 9, L Peña Quintana 10, A Reyes Domínguez 10, E Torcuato Rubio 11, P Ortiz Pérez 11, A E Fernández-Lorenzo 12, A Moreno Álvarez 12, Alfonso Solar Boga 12, H Romero Rey 12, M Álvarez Beltrán 13, E Masip Simo 14, D González Jiménez 6; CF and Pancreas Working Group of the Spanish Society of Pediatric Gastroenterology, Hepatology and Nutrition (SEGHNP)

Affiliations expand
PMID: 39675556

DOI: 10.1016/j.gastrohep.2024.502321
Abstract

Background: Cystic fibrosis (CF) is an autosomal recessive, chronic, potentially lethal genetic disease. CF manifestations are due to mutations in the CF transmembrane receptor transporter (CFTR) gene which codes for a protein (CFTR) that acts as an anion transporter, mainly chlorine, at epithelial cells where it is expressed. Cystic fibrosis related liver disease (CFRLD) includes a spectrum of hepatobiliary manifestations whose diagnosis and follow-up remains a challenge.

Methods: Cross-sectional, descriptive study from 10 Spanish Cystic fibrosis Units. Clinical and biochemical data obtained. Patients categorized into 3 groups according to liver involvement based on ESPGHAN 2017 criteria. Liver stiffness assessed by transient elastography (TE) and findings from abdominal ultrasound recorded. Statistics performed using SPSS v25.0.
Results: We obtained hepatic TE data from 155 pediatric CF patients. Forty-four classified as CFRLD, 38 (86%) had CFRLD without cirrhosis and 6 (14%) had cirrhosis. Fourteen patients without CFRLD (12%) had ultrasound abnormalities. Mean liver elastography value (kPa) was 4.7 (3.5-5.3) in non-CFRLD and 6.09 (4.4-6.7) in CFRLD (p=0.01;Tstudent [T]).

Conclusions: CFRLD is common in children with CF. Transient elastography is a useful method for diagnosis and follow-up, as higher values of TE are found in patients with CFRLD.