Article
Gastroenterology & Hepatology
Omar Y. Mousa, Brian D. Juran, Bryan M. McCauley, Mette N. Vesterhus, Trine Folseraas, Coleman T. Turgeon, Ahmad H. Ali, Erik M. Schlicht, Elizabeth J. Atkinson, Chang Hu, Denise Harnois, Elizabeth J. Carey, Andrea A. Gossard, Devin Oglesbee, John E. Eaton, Nicholas F. LaRusso, Gregory J. Gores, Tom H. Karlsen, Konstantinos N. Lazaridis
Summary: In patients with PSC, alterations in plasma BA consistent with cholestasis, UDCA treatment, and IBD were observed. BA profiles were able to predict future hepatic decompensation, demonstrating the clinical potential of BA profiling for use in clinical trials.
Article
Medicine, General & Internal
Eva Steinke, Olaf Sommerburg, Simon Y. Graeber, Cornelia Joachim, Christiane Labitzke, Gyde Nissen, Isabell Ricklefs, Isa Rudolf, Matthias V. Kopp, Anna-Maria Dittrich, Marcus A. Mall, Mirjam Stahl
Summary: This study is a prospective, longitudinal cohort study aimed at determining influencing factors of early cystic fibrosis (CF) lung disease through the combined analysis of clinical information and biomaterials. The primary endpoints are the lung clearance index and magnetic resonance imaging scores, while secondary endpoints include pulmonary exacerbations, infection with pro-inflammatory pathogens, and anthropometric data.
FRONTIERS IN MEDICINE
(2023)
Article
Pharmacology & Pharmacy
Lei Zheng, Zhifeng Zhao, Jiayun Lin, Hongjie Li, Guangbo Wu, Xiaoliang Qi, Xiaolou Lou, Yongyang Bao, Haizhong Huo, Meng Luo
Summary: Telmisartan ameliorates cirrhosis-induced portal hypertension by reducing liver fibrosis, inflammation responses, angiogenesis, and vascular remodeling. KLF-4 and eNOS may be potential molecular targets for the management of cirrhosis-associated portal hypertension.
EUROPEAN JOURNAL OF PHARMACOLOGY
(2022)
Article
Radiology, Nuclear Medicine & Medical Imaging
Yun Zhang, Xiaoli Fan, Bin Song, Yifeng Liu, Yidi Chen, Tianying Zheng, Yuxin Guo, Ting Duan, Zixing Huang, Li Yang
Summary: This study explored the feasibility of using nonenhanced magnetic resonance imaging (MRI) to predict insufficient biochemical response to ursodeoxycholic acid (UDCA) in patients with primary biliary cholangitis (PBC). The results showed that a predictive model based on three pretreatment MRI features could accurately predict insufficient biochemical response to UDCA treatment in PBC patients within 12 months. Early identification of high-risk patients can facilitate the timely initiation of additional treatment.
EUROPEAN RADIOLOGY
(2023)
Article
Gastroenterology & Hepatology
Jinhang Gao, Bo Wei, Mengfei Liu, Petra Hirsova, Tejasav S. Sehrawat, Sheng Cao, Xiao Hu, Fei Xue, Usman Yaqoob, Ningling Kang, Huarui Cui, William C. K. Pomerantz, Enis Kostallari, Vijay H. Shah
Summary: Deletion of endothelial p300 can alleviate portal hypertension and liver fibrosis caused by liver injury, reducing macrophage accumulation. The regulation of inflammation may be associated with Ccl2, NF kappa B, and BRD4.
Article
Nutrition & Dietetics
Yiqing Peng, Malinda Wu, Jessica A. Alvarez, Vin Tangpricha
Summary: Cystic fibrosis-related diabetes (CFRD) affects up to half of CF patients by adulthood, primarily caused by pancreatic dysfunction. This study found that vitamin D deficiency increases the risk of developing CFRD in adults with CF, and maintaining a serum 25(OH)D concentration above 20 ng/mL may decrease this risk.
Review
Medicine, General & Internal
Michele Fiordaliso, Giuseppe Marincola, Barbara Pala, Raffaella Muraro, Mariangela Mazzone, Maria Carmela Di Marcantonio, Gabriella Mincione
Summary: Non-cirrhotic portal hypertension (NCPH) is a rare disease characterized by portal hypertension without cirrhosis. The diagnosis of NCPH is challenging and there are currently no specific tests available for confirmation. Treatment focuses on preventing complications, but there is a need for further research on the etiology and genetic background of the disease.
Article
Endocrinology & Metabolism
Xiu-Ping Bai, Wen-Jin Du, Hua-Bing Xing, Guo-Hua Yang, Rui Bai
Summary: In this study, rats with cirrhosis induced by bile duct ligation (BDL) were used to investigate the effect of ursodeoxycholic acid (UDCA) on glucose levels. The results showed that UDCA could improve liver fibrosis, increase islet area, decrease glucose levels, and regulate the expression of genes related to bile acid synthesis and the TGR5 gene in the intestine.
DIABETOLOGY & METABOLIC SYNDROME
(2023)
Article
Microbiology
Barry Linnane, Aaron M. Walsh, Calum J. Walsh, Fiona Crispie, Orla O'Sullivan, Paul D. Cotter, Michael McDermott, Julie Renwick, Paul McNally
Summary: The study thoroughly characterized the lower airway microbiome in pre-school children with CF, finding that while the CF lung microbiome is broadly similar to that in non-CF children in terms of alpha diversity at an early age, several key genera were significantly differentially abundant between the CF and non-CF groups. CF diagnosis explained only a small amount of variation between the microbiomes, and there were distinct patterns of microbial abundance changes with age in CF and non-CF populations.
Article
Health Care Sciences & Services
Jakub Gazda, Sylvia Drazilova, Martin Janicko, Ivica Grgurevic, Tajana Filipec Kanizaj, Tomas Koller, Beatrica Bodorovska, Tonci Bozin, Maja Mijic, Zrinka Rob, Ivana Mikolasevic, Anita Madir, Branislav Kucinsky, Peter Jarcuska
Summary: Several pretreatment laboratory parameters, including total bilirubin, ALT, AST, ALP, total cholesterol, APRI, as well as pretreatment albumin and ALT/ALP ratio, are associated with treatment response in patients with primary biliary cholangitis. Treatment response after 6 months of therapy significantly predicts treatment response after 12 months of ursodeoxycholic acid therapy. Additionally, treatment responses at 6 and 12 months decrease the risk of liver decompensation episodes in these patients.
JOURNAL OF PERSONALIZED MEDICINE
(2021)
Article
Gastroenterology & Hepatology
Jong-In Chang, Jung Hee Kim, Dong Hyun Sinn, Ju-Yeon Cho, Kwang Min Kim, Joo Hyun Oh, Yewan Park, Won Sohn, Myung Ji Goh, Wonseok Kang, Geum-Youn Gwak, Yong-Han Paik, Moon Seok Choi, Joon Hyeok Lee, Kwang Cheol Koh, Seung-Woon Paik
Summary: The study validated the effectiveness of the UDCA response score (URS) in predicting the response to UDCA treatment in Asian patients with primary biliary cholangitis (PBC). URS can be used to predict the treatment response and clinical outcome in patients with PBC.
Review
Medicine, General & Internal
Sasan Sakiani, Theo Heller, Christopher Koh
Summary: Treatment options for portal hypertension are limited and mainly focus on reducing hyperdynamic circulation and treating hypervolemia. Newer and more effective treatment options are needed to improve survival and quality of life.
FRONTIERS IN MEDICINE
(2022)
Article
Medicine, General & Internal
Zhifeng Zhao, Chihao Zhang, Jiayun Lin, Lei Zheng, Hongjie Li, Xiaoliang Qi, Haizhong Huo, Xiaolou Lou, Bruce D. Hammock, Sung Hee Hwang, Yongyang Bao, Meng Luo
Summary: PTUPB alleviated liver fibrosis and portal hypertension by inhibiting fibrotic deposition, inflammation, angiogenesis, sinusoidal, and SMA remodeling, potentially mediated through the downregulation of sEH/COX-2/TGF-beta.
FRONTIERS IN MEDICINE
(2021)
Article
Biochemistry & Molecular Biology
Iris Pinheiro, Aurora Barbera, Imma Raurell, Federico Estrella, Marcel de Leeuw, Selin Bolca, Davide Gottardi, Nigel Horscroft, Sam Possemiers, Maria Teresa Salcedo, Joan Genesca, Maria Martell, Salvador Augustin
Summary: The present study demonstrated that oral administration of a specific bacterial consortium improved disease outcomes in two rodent models of non-alcoholic fatty liver disease. The consortium was found to effectively replace and enrich the gut microbiome, leading to improvements in endothelial function, fibrosis, and disease progression.
Article
Multidisciplinary Sciences
Peder Berg, Jesper Frank Andersen, Mads Vaarby Sorensen, Tobias Wang, Hans Malte, Jens Leipziger
Summary: The lungs and kidneys play a crucial role in regulating the body's acid-base balance. In cystic fibrosis, impaired renal excretion of HCO3- leads to metabolic alkalosis, which can contribute to reduced lung function by suppressing ventilatory drive.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2022)
Article
Respiratory System
Nicholas J. Simmonds, C. Kors van der Ent, Carla Colombo, Nils Kinnman, Cynthia DeSouza, Teja Thorat, Marci L. Chew, Keval Chandarana, Carlo Castellani
Summary: This study investigated the long-term effectiveness of ivacaftor on clinical outcomes and healthcare resource utilization in people with cystic fibrosis. The results showed significant improvements in lung function, nutritional status, and respiratory infections after ivacaftor treatment, along with a decrease in hospitalizations and antibiotic use.
JOURNAL OF CYSTIC FIBROSIS
(2023)
Article
Rehabilitation
Simone Gambazza, Federica Carta, Federico Ambrogi, Giacomo Bassotti, Anna Brivio, Maria Russo, Carla Colombo
Summary: This study aimed to investigate the clinical significance of the 6-minute walk test (6MWT) in candidates for lung transplantation (LTx) with cystic fibrosis (CF) for lung allocation score (LAS) calculation. The results showed a significant association between walked distance and the hazard ratio (HR) of LTx or death, but when used as an LAS calculation indicator, it does not provide useful information. Longitudinal trajectories of walked distance may provide complementary information about prognosis in individuals with CF.
DISABILITY AND REHABILITATION
(2023)
Article
Respiratory System
Daniela Dolce, Laura Claut, Carla Colombo, Antonella Tosco, Alice Castaldo, Rita Padoan, Silviana Timpano, Benedetta Fabrizzi, Paolo Bonomi, Giovanni Taccetti, Vito Terlizzi
Summary: This study assessed the clinical data, management, and outcomes of Italian infants with CF screen-positive inconclusive diagnosis (CRMS/CFSPID) and Pseudomonas aeruginosa (Pa) in their airways. The results showed that Pa detection in asymptomatic CRMS/CFSPID infants is common but tends to clear spontaneously. Further studies are needed to determine if Pa isolation can predict disease progression.
JOURNAL OF CYSTIC FIBROSIS
(2023)
Article
Biochemistry & Molecular Biology
Marilena Pariano, Matteo Puccetti, Claudia Stincardini, Valerio Napolioni, Leonardo Gatticchi, Roberta Galarini, Giorgia Renga, Carolina Barola, Marina M. Bellet, Fiorella D'Onofrio, Emilia Nunzi, Andrea Bartoli, Cinzia Antognelli, Lisa Cariani, Maria Russo, Luigi Porcaro, Carla Colombo, Fabio Majo, Vincenzina Lucidi, Enza Montemitro, Ersilia Fiscarelli, Helmut Ellemunter, Cornelia Lass-Florl, Maurizio Ricci, Claudio Costantini, Stefano Giovagnoli, Luigina Romani
Summary: Hypoxia contributes to ineffective airway inflammation in cystic fibrosis and inhibiting hypoxia-inducible factor 1 alpha (HIF-1 alpha) could impair essential immune functions. This study explores the interaction between hypoxia and the aryl hydrocarbon receptor (AhR) and demonstrates that AhR agonism can prevent hypoxia-induced inflammation and improve lung function in CF.
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY
(2023)
Article
Gastroenterology & Hepatology
Laura Zazzeron, Gianfranco Alicandro, Valeria Dacco, Chiara Lanfranchi, Anna Bulfamante, Calogero Sathya Sciarrabba, Fabiola Corti, Carla Colombo
Summary: This study found that the use of proton pump inhibitors (PPI) as adjuvant therapy in people with cystic fibrosis (pwCF) may increase the risk of respiratory infection and does not improve nutritional status.
DIGESTIVE AND LIVER DISEASE
(2023)
Article
Endocrinology & Metabolism
Simone De Leo, Matteo Trevisan, Carla Colombo, Claudia Moneta, Noemi Giancola, Laura Fugazzola
Summary: This retrospective cohort study aimed to investigate the incidence, characteristics, and management of hypocalcemia in patients with advanced thyroid cancer treated with lenvatinib. The study found a higher incidence of hypocalcemia (24%) compared to the registration trial (6.9%). The hypocalcemia in this cohort was caused by both PTH-dependent and PTH-independent mechanisms.
Article
Respiratory System
Carla Colombo, Grant A. Ramm, Anders Lindblad, Fabiola Corti, Luigi Porcaro, Federico Alghisi, Irina Asherova, Helen Evans, Nataliya Kashirskaya, Elena Kondratyeva, Peter J. Lewindon, Isabelle de Monestrol, Mark Oliver, Chee Y. Ooi, Rita Padoan, Sahana Shankar, Gianfranco Alicandro
Summary: Cystic-fibrosis-related liver disease (CFLD) is a variable phenotype of CF. CFTR modulator therapies, elexacaftor, tezacaftor, and ivacaftor (ETI), and ivacaftor (IVA), are available for CF patients. Genetic eligibility evaluation showed that 13% of patients without CFLD and 11% of patients with severe CFLD are not eligible for ETI or IVA therapy. Ineligible patients cannot benefit from these new treatments.
JOURNAL OF CYSTIC FIBROSIS
(2023)
Article
Respiratory System
Francesco Milo, Fabiana Ciciriello, Federico Alghisi, Paola Tabarini
Summary: This study investigated the lived experiences of CF patients who are not eligible for ETI. The findings revealed that these patients experience disappointment, but also have some hope, and they require mental health monitoring and integrated care to help them overcome their disappointment and improve coping.
JOURNAL OF CYSTIC FIBROSIS
(2023)
Article
Pediatrics
Amin J. Roberts, Alicia Lim, Jonathan R. Bishop, Ed Gane, Elizaveta Rakhmanova, William Wong, Helen M. Evans
Summary: This study investigated the occurrence of atopy/allergy in solid organ transplant recipients, focusing on paediatric liver transplant patients in New Zealand. The results showed a significantly higher prevalence of atopy/allergy in paediatric liver transplant recipients compared to adult liver and paediatric kidney transplant recipients. The most common allergies were eczema and IgE-mediated food allergy.
JOURNAL OF PAEDIATRICS AND CHILD HEALTH
(2023)
Article
Pediatrics
Vivek Rajasekaran, Craig McCaffer, Jonathan Bishop, Graeme Van Der Meer, Edward C. C. Toll, Helen Evans
Summary: Late airway complications following pediatric liver transplantation, as a result of immunosuppression, are rarely reported. Two cases of airway obstruction due to supraglottic pathology were described, including infiltrative eosinophilic laryngitis and post-transplant lymphoproliferative disease. Maintaining a high index of suspicion and prompt diagnosis and management are crucial in these cases.
PEDIATRIC TRANSPLANTATION
(2023)
Article
Endocrinology & Metabolism
Simone De Leo, Matteo Trevisan, Claudia Moneta, Carla Colombo
Summary: Tyrosine kinase inhibitors (TKIs) have shown improved outcomes for various tumors, but they can also cause adverse events and endocrine-related toxicities. The most common endocrinopathies caused by TKIs are thyroid dysfunction and hypothyroidism, with potential mechanisms including dysfunction of the thyroid gland and hormone metabolism impairment. TKIs can also affect other glands, leading to adrenal insufficiency, growth retardation, hypogonadism, and fertility impairment. TKIs can also disrupt bone metabolism and cause metabolic alterations, such as hypoglycemia and hyperglycemia. These endocrine-related adverse events should be managed by endocrinologists with replacement therapy or symptomatic treatment.
ANNALES D ENDOCRINOLOGIE
(2023)
Editorial Material
Gastroenterology & Hepatology
Darrell H. G. Crawford, Grant A. Ramm, Kim R. Bridle, Amanda J. Nicoll, Martin B. Delatycki, John K. Olynyk
HEPATOLOGY INTERNATIONAL
(2023)
Article
Gastroenterology & Hepatology
Shruti Sakhuja, Heather M. Staples, Charles G. Minard, Louise E. Ramm, Peter J. Lewindon, Grant A. Ramm, Daniel H. Leung
Summary: This study aimed to evaluate the clinical utility of lung function and liver biomarkers assessed years prior to liver biopsy-proven CFLD in predicting the risk of severe fibrosis development. The findings suggest that mild-moderate pulmonary dysfunction and higher liver biomarker indices at baseline may be associated with faster progression of CFLD.
LIVER INTERNATIONAL
(2023)
Article
Health Care Sciences & Services
Andrea Foppiani, Fabiana Ciciriello, Arianna Bisogno, Silvia Bricchi, Carla Colombo, Federico Alghisi, Vincenzina Lucidi, Maria Ausilia Catena, Mariacristina Lucanto, Andrea Mari, Giorgio Bedogni, Alberto Battezzati
Summary: This study aimed to describe the distribution and provide reference quartiles of OGTT-related variables in the Italian cystic fibrosis population. The results showed the deterioration of glucose tolerance and insulin secretion with age according to sex and pancreatic insufficiency, highlighting a deviation from linearity for patients 35 years of age.
JOURNAL OF PERSONALIZED MEDICINE
(2023)