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THE ROLE OF SOME PROTEIN BIOMARKERS IN THE EARLY IDENTIFICATION OF THE RISK OF CYSTIC FIBROSIS ASSOCIATED LIVER DISEASE

ELENA-SIMONA MOICEANU 1,2, IUSTINA VIOLETA STAN 3,4*, SIMONA ELENA MOȘESCU 5, VIORELA GABRIELA NIȚESCU 2,3, MARIA IACOBESCU 6, XIMENA MARIA MUREȘAN 6,7, CRISTINA-ADELA IUGA 6,7, ANDREEA LESCAIE 2,3, ANDREI-ALEXANDRU MUNTEAN 3,8, COSTIN-ȘTEFAN CARACOTI 3,8, GEORGIANA CRISTIANA SULIȚĂ 9, ALEXANDRA BRATU 4, CORINA-ELENA DELIA 4,10, GEANINA-MIRELA TOMA 4, DAN LUCIAN DUMITRAȘCU 11

1 “Iuliu Hațieganu” University of Medicine and Pharmacy, 8 Victor Babeș Street, 400347, Cluj-Napoca, Romania
2 Paediatric Poison Centre, “Grigore Alexandrescu” Clinical Emergency Hospital for Children, 30-32 Iancu de Hunedoara Road, 011743, Bucharest, Romania
3 “Carol Davila” University of Medicine and Pharmacy, 37 Dionisie Lupu Street, 020021, Bucharest, Romania
4 “Alessandrescu-Rusescu” National Institute for Mother and Child Health, 120 Lacul Tei Boulevard, 020395, Bucharest, Romania
5 Department of Paediatrics, “Grigore Alexandrescu” Clinical Emergency Hospital for Children, 30-32 Iancu de Hunedoara Road, 011743, Bucharest, Romania
6 Institute of Medical Research and Life Sciences – MEDFUTURE, “Iuliu Hațieganu” University of Medicine and Pharmacy, 4-6 Louis Pasteur Street, 400349, Cluj-Napoca, Romania
7 Drug Analysis, Department Pharmacy 3, Faculty of Pharmacy, “Iuliu Hațieganu” University of Medicine and Pharmacy, 6 Louis Pasteur Street, 400349, Cluj-Napoca, Romania
8 “Cantacuzino” National Military Medical Institute for Research and Development, 103 Splaiul Independenței, 050096, Bucharest, Romania
9 Laboratory Department, “Grigore Alexandrescu” Clinical Emergency Hospital for Children, 30-32 Iancu de Hunedoara Road, 011743, Bucharest, Romania
10 Faculty of Biology, University of Bucharest, 91-95 Splaiul Independenței, 050095, Bucharest, Romania
11 2nd Department of Internal Medicine, “Iuliu Hațieganu” University of Medicine and Pharmacy, 400006, Cluj-Napoca, Romania

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The study aimed to evaluate the presence of three protein biomarkers in the serum of patients with cystic fibrosis (CF) and correlate these markers with the degree of hepatic impairment. We conducted a cross-sectional observational study that included 36 paediatric patients with CF, equally divided into two groups (a Case group - with hepatic involvement and a Control group), recruited between November 2023 and March 2024, in accordance with the new guidelines of the CF Foundation. The following biomarkers were measured in the serum of these patients: ubiquitin carboxyl-terminal hydrolase L1 (UCHL1), Endoglin/CD105 and Tissue Inhibitor of Metalloproteinases-4 (TIMP-4). Serum TIMP-4 levels were significantly elevated in patients with hepatic involvement, and this finding correlated with the calculated cystic fibrosis scores. No significant differences were observed in the levels of Endoglin and UCHL1 between the two groups. TIMP-4 is a non-invasive, rapid and cost-effective biomarker associated with the early onset of hepatic lesions.