Cystic fibrosis is an autosomal progressive disease affecting the lung, pancreas, and liver. Some patients develop end-stage respiratory and liver failure. For such patients, combined lung-liver transplantation remains the only therapeutic option. In this article we present the first simultaneous lung-liver transplantation in Poland, as well as in Central and Eastern Europe, with detailed clinical history, surgical aspects, and postoperative course.

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.transproceed.2022.02.023DOI Listing

Publication Analysis

Top Keywords

cystic fibrosis
8
lung-liver transplantation
8
poland simultaneous
4
simultaneous liver-lung
4
liver-lung transplantation
4
transplantation liver-first
4
liver-first approach
4
approach recipient
4
recipient cystic
4
fibrosis case
4

Similar Publications

Cystic Fibrosis (CF) is a life-threatening hereditary disease resulting from mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene that encodes a chloride channel essential for ion transport in epithelial cells. Mutations in CFTR, notably the prevalent F508del mutation, impair chloride transport, severely affecting the respiratory system and leading to recurrent infections. Recent therapeutic advancements include CFTR modulators such as ETI, a combination of two correctors (Elexacaftor and Tezacaftor) and a potentiator (Ivacaftor), that can improve CFTR function in patients with the F508del mutation.

View Article and Find Full Text PDF

The Uptake of the Influenza Vaccine in Patients With Cystic Fibrosis: A Retrospective Study.

J Paediatr Child Health

January 2025

Paediatric Respiratory and Sleep Department, Women's and Children's Hospital, Adelaide, South Australia, Australia.

Background: Children with cystic fibrosis are more likely to become severely unwell with influenza-associated illness compared to children without chronic lung disease. The provision of accessible influenza vaccinations is essential in the prevention of infection.

Objectives: To describe the prevalence of the influenza vaccine uptake in children with cystic fibrosis from 2016 to 2020 at a single tertiary paediatric hospital site and determine if the COVID pandemic of 2020 and the introduction of telehealth encounters affected the vaccine uptake.

View Article and Find Full Text PDF

Cystic Fibrosis (CF) is a life-shortening autosomal recessive disease caused by mutations in the CFTR gene, resulting in functional impairment of the encoded ion channel. F508del mutation, a trinucleotide deletion, is the most frequent cause of CF affecting approximately 80% of persons with cystic fibrosis (pwCFs). Even though current pharmacological treatments alleviate the F508del-CF disease symptoms there is no definitive cure.

View Article and Find Full Text PDF

Therapeutic Potential of Ketogenic Interventions for Autosomal-Dominant Polycystic Kidney Disease: A Systematic Review.

Nutrients

December 2024

Centre for Diabetes, Obesity and Endocrinology Research (CDOER), Westmead Institute for Medical Research, Westmead, Sydney, NSW 2145, Australia.

Background: Recent findings have highlighted that abnormal energy metabolism is a key feature of autosomal-dominant polycystic kidney disease (ADPKD). Emerging evidence suggests that nutritional ketosis could offer therapeutic benefits, including potentially slowing or even reversing disease progression. This systematic review aims to synthesise the literature on ketogenic interventions to evaluate the impact in ADPKD.

View Article and Find Full Text PDF

Whole-Exome Sequencing: Discovering Genetic Causes of Granulomatous Mastitis.

Int J Mol Sci

January 2025

Department of Molecular Medicine, Aziz Sancar Institute of Experimental Medicine, Istanbul University, 34093 Istanbul, Türkiye.

Granulomatous mastitis (GM) is a rare, benign, but chronic and recurrent inflammatory breast disease that significantly impacts physical and psychological well-being. It often presents symptoms such as pain, swelling, and discharge, leading to diagnostic confusion with malignancy. The etiology of GM remains unclear, though autoimmune and multifactorial components are suspected.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!