Use the keywords feature with a free QxMD account.
Use Read by QxMD to access full text via your institution or open access sources.
Read also provides personalized recommendations to keep you up to date in your field.
Existing User
Sign InNew to Read
Sign Up#21
JOURNAL ARTICLE
Marlene Fabiola Escobedo-Monge, Marianela Marcos-Temprano, Joaquín Parodi-Román, María Antonieta Escobedo-Monge, Carmen Alonso-Vicente, María Carmen Torres-Hinojal, José Manuel Marugán-Miguelsanz
Cystic fibrosis (CF) is a monogenic disease with different types of mutations that mainly affect the respiratory-digestive system. Calcium (Ca), phosphorus (P), and vitamin D (Vit-D) are essential nutrients for maintaining adequate growth and development, as well as key components in crucial metabolic pathways. Proper diagnosis, treatment, and response are decisive components of precision medicine. Therefore, we conducted a cross-sectional study to evaluate Ca, P, and Vit-D levels along with health and nutritional indicators, regarding their non-skeletal functions, in a series of CF patients...
February 5, 2024: International Journal of Molecular Sciences
#22
REVIEW
Jun Wu, Shuang-Yong Cai, Xu-Liang Chen, Zhi-Tao Chen, Shao-Hua Shi
Mirizzi syndrome is a serious complication of gallstone disease. It is caused by the impacted stones in the gallbladder neck or cystic duct. One of the features of Mirizzi syndrome is severe inflammation or dense fibrosis at the Calot's triangle. In our clinical practice, bile duct, branches of right hepatic artery and right portal vein clinging to gallbladder infundibulum are often observed due to gallbladder infundibulum adhered to right hepatic hilum. The intraoperative damage of branches of right hepatic artery occurs more easily than that of bile duct, all of which are hidden pitfalls for surgeons...
January 30, 2024: Hepatobiliary & Pancreatic Diseases International: HBPD INT
#23
REVIEW
Frank Thévenod, Wing-Kee Lee
Cellular responses to toxic metals depend on metal accessibility to intracellular targets, reaching interaction sites, and the intracellular metal concentration, which is mainly determined by uptake pathways, binding/sequestration and efflux pathways. ATP-binding cassette (ABC) transporters are ubiquitous in the human body-usually in epithelia-and are responsible for the transfer of indispensable physiological substrates (e.g. lipids and heme), protection against potentially toxic substances, maintenance of fluid composition, and excretion of metabolic waste products...
February 6, 2024: Biometals: An International Journal on the Role of Metal Ions in Biology, Biochemistry, and Medicine
#24
JOURNAL ARTICLE
Frank A J A Bodewes, Alvin Jay Freeman, Alexander Weymann, Dominique Debray, Isabelle Scheers, Henkjan J Verkade, Michael R Narkewicz
The broad spectrum of hepatobiliary involvement in cystic fibrosis (CF) has been commonly referred to as cystic fibrosis liver disease (CFLD). However, differences in the definitions of CFLD have led to variations in reported prevalence, incidence rates, and standardized recommendations for diagnosis and therapies. Harmonizing the description of the spectrum of hepatobiliary involvement in all people with CF (pwCF) is deemed essential for providing a reliable account of the natural history, which in turn supports the development of meaningful clinical outcomes in patient care and research...
January 2024: Journal of Pediatric Gastroenterology and Nutrition
#25
REVIEW
Jessica A Eldredge, Mark R Oliver, Chee Y Ooi
Cystic fibrosis liver disease (CFLD) is characterised by a wide heterogenity of manifestations and severity. It represents a major cause of morbidity in people with cystic fibrosis (PwCF), which will be of increasing relevance as survival increases in the new era of cystic fibrosis care. No medical therapy currently available has evidence to treat or prevent progression of liver disease. Cystic Fibrosis Transmembrane Conductance Receptor (CFTR) modulators may be transformative on pulmonary, nutritional and quality of life, but direct effect on long term liver disease outcomes is not yet established...
December 28, 2023: Paediatric Respiratory Reviews
#26
JOURNAL ARTICLE
Teresa Fuchs, Dorothea Appelt, Helmut Ellemunter
With the worldwide spread of SARS-CoV-2 disease, people with cystic fibrosis (CF), especially solid organ transplant recipients, have quickly been identified as a risk group for severe disease. Studies have shown low antibody response to SARS-CoV-2 vaccines in recipients of solid organ transplant compared to the healthy population. Information on immune response in CF patients with solid organ transplantation is limited, especially regarding long-term efficacy. The aim of this real-world study was a long-term assessment of humoral immune response induced by three and four doses of the SARS-CoV-2 mRNA vaccine...
January 18, 2024: Vaccines
#27
JOURNAL ARTICLE
Rashmi Tresa Philpose, Abdul Aleem Mohammed, Ashrith Reddy Gowni
PURPOSE: Choledochal cysts are congenital anomalies that occur as localized cystic or fusiform dilatations of the biliary tree. Reflux and stasis of pancreatic enzymes in the biliary duct may relate to the development of intestinal metaplasia which might be an important factor related to the carcinogenesis of choledochal cyst, thus the expression of beta-catenin in the metaplastic epithelium might be associated with malignant transformation of choledochal cyst epithelium. METHODS: This study was conducted at a tertiary care pediatric center between October 2014 and March 2017...
January 2024: Pediatric Gastroenterology, Hepatology & Nutrition
#28
Muhammad Nabeel Shafqat, Muhammad Yousuf Y Memon, Salman Javed, Sai Gautham Kanagala, Momina Saleem
Synonymous with congenital non-obstructive saccular or fusiform intra-hepatic duct dilatation and congenital communicating cavernous ectasia of the intra-hepatic biliary tract, Caroli's syndrome (CS) is an extremely rare fibro-polycystic liver disorder characterized by ductal plate malformation and consequent peri-portal fibrosis due to segmental intra-hepatic duct dilatation. No more than 200 cases of the syndrome have been reported since 1958. CS may affect one or both lobes of the liver, but more commonly it affects the left hepatic lobe...
December 2023: Curēus
#29
Richard D Maradiaga, Mitchell L Ramsey, Stephen E Kirkby, Lindsay A Sobotka
Despite advances in treatment for cystic fibrosis (CF), liver disease remains a major contributor to morbidity and mortality for persons with CF. Therefore, liver transplantation may be considered in end-stage CF-related liver disease. We present a young patient with CF who underwent solo liver transplantation and has successfully restarted on elexacaftor/tezacaftor/ivacaftor without significant pulmonary or hepatic complications after transplant.
January 2024: ACG Case Reports Journal
#30
JOURNAL ARTICLE
Pierre-Régis Burgel, Kevin W Southern, Charlotte Addy, Alberto Battezzati, Claire Berry, Jean-Philippe Bouchara, Edwin Brokaar, Whitney Brown, Pilar Azevedo, Isabelle Durieu, Miquel Ekkelenkamp, Felicity Finlayson, Julian Forton, Johanna Gardecki, Pavla Hodkova, Gina Hong, Jacqueline Lowdon, Su Madge, Clémence Martin, Edward McKone, Anne Munck, Chee Y Ooi, Lucy Perrem, Amanda Piper, Andrew Prayle, Felix Ratjen, Margaret Rosenfeld, Don B Sanders, Carsten Schwarz, Giovanni Taccetti, Claire Wainwright, Natalie E West, Michael Wilschanski, Amanda Bevan, Carlo Castellani, Pavel Drevinek, Silvia Gartner, Andrea Gramegna, Elise Lammertyn, Eddie Edwina C Landau, Barry J Plant, Alan R Smyth, Silke van Koningsbruggen-Rietschel, Peter G Middleton
This is the third in a series of four papers updating the European Cystic Fibrosis Society (ECFS) standards for the care of people with CF. This paper focuses on recognising and addressing CF health issues. The guidance was produced with wide stakeholder engagement, including people from the CF community, using an evidence-based framework. Authors contributed sections, and summary statements which were reviewed by a Delphi consultation. Monitoring and treating airway infection, inflammation and pulmonary exacerbations remains important, despite the widespread availability of CFTR modulators and their accompanying health improvements...
January 16, 2024: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
#31
JOURNAL ARTICLE
Isobel L Ward, Chris Robertson, Utkarsh Agrawal, Lynsey Patterson, Declan T Bradley, Ting Shi, Simon de Lusignan, F D Richard Hobbs, Aziz Sheikh, Vahé Nafilyan
The emergence of the COVID-19 vaccination has been critical in changing the course of the COVID-19 pandemic. To ensure protection remains high in vulnerable groups booster vaccinations in the UK have been targeted based on age and clinical vulnerabilities. We undertook a national retrospective cohort study using data from the 2021 Census linked to electronic health records. We fitted cause-specific Cox models to examine the association between health conditions and the risk of COVID-19 death and all-other-cause death for adults aged 50-100-years in England vaccinated with a booster in autumn 2022...
January 16, 2024: Nature Communications
#32
JOURNAL ARTICLE
Jing-Yi Mou, Zhang-Wen Ma, Meng-Ying Zhang, Quan Yuan, Zi-Yi Wang, Qing-Hua Liu, Fen Li, Zhao Liu, Liang Wang
Growing evidence confirms associations between glycogen metabolic re-wiring and the development of liver cancer. Previous studies showed that glycogen structure changes abnormally in liver diseases such as cystic fibrosis, diabetes, etc. However, few studies focus on glycogen molecular structural characteristics during liver cancer development, which is worthy of further exploration. In this study, a rat model with carcinogenic liver injury induced by diethylnitrosamine (DEN) was successfully constructed, and hepatic glycogen structure was characterized...
January 14, 2024: International Journal of Biological Macromolecules
#33
JOURNAL ARTICLE
Michael Wilschanski, Anne Munck, Estefania Carrion, Marco Cipolli, Sarah Collins, Carla Colombo, Dimitri Declercq, Elpis Hatziagorou, Jessie Hulst, Daina Kalnins, Christina N Katsagoni, Jochen G Mainz, Carmen Ribes-Koninckx, Chris Smith, Thomas Smith, Stephanie Van Biervliet, Michael Chourdakis
BACKGROUND: Nutritional status is paramount in Cystic Fibrosis (CF) and is directly correlated with morbidity and mortality. The first ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with CF were published in 2016. An update to these guidelines is presented. METHODS: The study was developed by an international multidisciplinary working group in accordance with officially accepted standards. Literature since 2016 was reviewed, PICO questions were discussed and the GRADE system was utilized...
February 2024: Clinical Nutrition
#34
JOURNAL ARTICLE
Aziz Anil Tan, Duygu Demirtas, Hayriye Hizarcioglu-Gulsen, Jale Karakaya, Emel Isiyel, Hasan Ozen, Berna Oguz, Mithat Haliloglu, H Nursun Ozcan
BACKGROUND: Liver involvement is an important cause of morbidity and mortality in patients with cystic fibrosis (CF). While liver biopsy is the gold standard for demonstrating involvement, its invasiveness prompts a search for noninvasive alternatives. OBJECTIVE: To evaluate liver involvement in pediatric patients with CF (versus healthy controls) using magnetic resonance (MR) elastography/spectroscopy and to correlate the imaging findings with clinical/laboratory characteristics...
December 22, 2023: Pediatric Radiology
#35
REVIEW
Kathrin Burgmaier, Ilse J Broekaert, Max C Liebau
Autosomal recessive polycystic kidney disease (ARPKD) is the rare and usually early-onset form of polycystic kidney disease with a typical clinical presentation of enlarged cystic kidneys and liver involvement with congenital hepatic fibrosis or Caroli syndrome. ARPKD remains a clinical challenge in pediatrics, frequently requiring continuous and long-term multidisciplinary treatment. In this review, we aim to give an overview over clinical aspects of ARPKD and recent developments in our understanding of disease progression, risk patterns, and treatment of ARPKD...
September 2023: Adv Kidney Dis Health
#36
Li-Jie Zhang, Xing-Yu Liu, Teng-Fei Chen, Zhong-Ya Xu, Han-Jun Yin
BACKGROUND: Abernethy malformation, also known as congenital extrahepatic portosystemic shunt, is an uncommon malformation resulting from aberrant development of the portal venous system. Cystic fibrosis (CF) is an autosomal recessive genetic disease caused by mutations in the CFTR gene. It mainly affects the exocrine glands of the respiratory, digestive and reproductive systems. It is considered extremely rare in the Asian population. We present a clinical case involving a pediatric patient of Asian descent who was diagnosed with Abernethy malformation and CF...
November 16, 2023: World Journal of Clinical Cases
#37
JOURNAL ARTICLE
Jennifer A Scott, Andrew M Jones, Elliot Jokl, Timothy Gordon-Walker, Peter J Barry, Neil A Hanley, Karen Piper Hanley, Varinder S Athwal
BACKGROUND & AIMS: Cystic Fibrosis related liver disease (CFLD) is the 3rd largest cause of death in Cystic Fibrosis (CF). As advances in pulmonary therapies have increased life-expectancy, CFLD has become more prevalent. Current guidelines may underdiagnose liver fibrosis, particularly in its early stages. Newer modalities for the assessment of fibrosis may provide a more accurate assessment. FibroScan is validated in assessing fibrosis for several aetiologies including alcohol and fatty liver, the CFLD cohort have an entirely different phenotype so the cut off values are not transferrable...
November 2023: Heliyon
#38
JOURNAL ARTICLE
Steffie E M Vonk, Rianne Lub, Els J M Weersink, Ulrich Beuers, Ron A A Mathôt, E Marleen Kemper, Josje Altenburg
PURPOSE: Cystic fibrosis (CF) is a monogenetic disease caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein and affecting multiple organs, including the lungs and liver. Almost 90% of people affected carry at least 1 Phe508del CFTR mutation. Medical treatment with the CFTR-modulating drug elexacaftor-tezacaftor-ivacaftor (ETI) has been proven to be efficacious in carriers of at least 1 Phe508del CFTR mutation. Use of ETI in patients with CF (pwCF) and liver cirrhosis is still controversial...
February 2024: Clinical Therapeutics
#39
JOURNAL ARTICLE
Amel El-Faramawy, Samya Z Nasr, Mohamed M S Al Emam, Heba M H Fahmy
BACKGROUND: Cystic fibrosis (CF) is an autosomal recessive disease. It affects multiple organ systems, including the liver, leading to CF-related liver disease (CFLD). It was noted that CFLD in Egyptian children with CF is more common than in non-Egyptian people with CF (pwCF). This study aimed to determine the incidence of CFLD and the potential risk factors for developing CFLD in Egyptian children. The correlation between CFLD and the various genotypes prevalent in Egyptian CF children will be discussed...
December 1, 2023: Pediatric Pulmonology
#40
JOURNAL ARTICLE
Y T Kan, W F Wu, Y F Yang
No abstract text is available yet for this article.
October 20, 2023: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
Make the most of Read.
Download the mobile app.
Download the mobile app.
Fetching more papers... 
