| Autor: |
Anton-Păduraru DT; Department of Mother and Child Medicine, 'Grigore T. Popa' University of Medicine and Pharmacy, 700115 Iaṣi, Romania.; 'Sf. Maria' Children Emergency Hospital, 700309 Iasi, Romania., Azoicăi AN; Department of Mother and Child Medicine, 'Grigore T. Popa' University of Medicine and Pharmacy, 700115 Iaṣi, Romania.; 'Sf. Maria' Children Emergency Hospital, 700309 Iasi, Romania., Trofin F; Department of Preventive Medicine and Interdisciplinarity-Microbiology, 'Grigore T. Popa' University of Medicine and Pharmacy, 700115 Iaṣi, Romania., Murgu AM; Department of Mother and Child Medicine, 'Grigore T. Popa' University of Medicine and Pharmacy, 700115 Iaṣi, Romania.; 'Sf. Maria' Children Emergency Hospital, 700309 Iasi, Romania., Mîndru DE; Department of Mother and Child Medicine, 'Grigore T. Popa' University of Medicine and Pharmacy, 700115 Iaṣi, Romania.; 'Sf. Maria' Children Emergency Hospital, 700309 Iasi, Romania., Bocec AS; Department of Mother and Child Medicine, 'Grigore T. Popa' University of Medicine and Pharmacy, 700115 Iaṣi, Romania., Halițchi COI; Department of Mother and Child Medicine, 'Grigore T. Popa' University of Medicine and Pharmacy, 700115 Iaṣi, Romania., Zota GR; Department of Pharmacology, Clinical Pharmacology and Algesiology, 'Grigore T. Popa' University of Medicine and Pharmacy, 700115 Iasi, Romania., Păduraru D; 'Dr. C. I. Parhon' Clinical Hospital, 700503 Iaṣi, Romania., Nastase EV; Department of Internal Medicine II-Infectious Diseases, 'Grigore T. Popa' University of Medicine and Pharmacy, 700115 Iasi, Romania.; Clinical Hospital of Infectious Diseases 'Sf. Parascheva', 700116 Iasi, Romania. |
| Abstrakt: |
Cystic fibrosis (CF) is a multifaceted disorder predominantly investigated for its pulmonary manifestations, yet patients with CF also exhibit a spectrum of extrapulmonary manifestations, notably those involving the hepatobiliary system. The latter constitutes the third leading cause of morbidity and mortality in individuals with CF. Cystic fibrosis-related liver disease (CFLD), with an escalating prevalence, manifests diverse clinical presentations ranging from hepatomegaly to cirrhosis and hepatopulmonary syndrome. Consequently, early detection and appropriate management are imperative for sustaining the health and influencing the quality of life of CF patients afflicted with CFLD. This review aims to consolidate existing knowledge by providing a comprehensive overview of hepatobiliary manifestations associated with CF. It delineates the clinical hepatobiliary manifestations, diagnostic methodologies, incorporating minimally invasive markers, and therapeutic approaches, encompassing the impact of novel CFTR modulators on CFLD. Given the exigency of early diagnosis and the intricate management of CFLD, a multidisciplinary team approach is essential to optimize care and enhance the quality of life for this subset of patients. In conclusion, recognizing CF as more than solely a pulmonary ailment, the authors underscore the imperative for further clinical investigations to establish a more robust evidence base for CFLD management within the continuum of this chronic disease. |
