Review
Medicine, General & Internal
Fernanda Iafusco, Giovanna Maione, Francesco Maria Rosanio, Enza Mozzillo, Adriana Franzese, Nadia Tinto
Summary: Cystic fibrosis, caused by mutations in the CFTR gene, is the most common autosomal recessive disease in the Caucasian population. Cystic fibrosis-related diabetes (CFRD) is a common nonpulmonary complication, with a prevalence that increases with age and is influenced by various factors. Genetic factors, including CFTR genotype, play a role in the development of CFRD.
Article
Endocrinology & Metabolism
Kevin J. Scully, Jordan S. Sherwood, Kimberly Martin, Melanie Ruazol, Peter Marchetti, Mary Larkin, Hui Zheng, Gregory S. Sawicki, Ahmet Uluer, Isabel Neuringer, Lael M. Yonker, Leonard Sicilian, Deborah J. Wexler, Melissa S. Putman
Summary: This study investigated the correlation between continuous glucose monitoring (CGM) measures and clinical outcomes in adults with cystic fibrosis (CF), as well as the relationship between hemoglobin A1c (HbA1c) and CGM-derived average glucose (AG). The findings suggest that CGM measures of hyperglycemia and glycemic variability are superior to HbA1c in distinguishing those with and without cystic fibrosis-related diabetes (CFRD), with CGM-derived AG strongly correlated with HbA1c in this population. Future research is needed to explore the potential of using CGM as a diagnostic and screening tool for CFRD.
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM
(2022)
Article
Respiratory System
Helen K. Chadwick, Janice Abbott, Margaret Anne Hurley, Louise Dye, Clare L. Lawton, Michael W. Mansfield, Daniel Peckham
Summary: This study investigated the impact of cystic fibrosis-related diabetes (CFRD) on cognitive function. The results found that individuals with cystic fibrosis demonstrated deficits in verbal and spatial memory, processing speed, and cognitive flexibility. Furthermore, individuals with CFRD had additional difficulties with higher-level processes known as 'executive function'. These cognitive impairments may interfere with self-care and everyday task performance.
JOURNAL OF CYSTIC FIBROSIS
(2022)
Article
Respiratory System
Helen K. Chadwick, Janice Abbott, Margaret Anne Hurley, Louise Dye, Clare L. Lawton, Michael W. Mansfield, Daniel Peckham
Summary: This study investigated the impact of cystic fibrosis-related diabetes (CFRD) on cognitive function. The results showed that individuals with CFRD demonstrated deficits in aspects of verbal and spatial memory, processing speed, and cognitive flexibility. They also experienced higher-level difficulties with executive function. These cognitive impairments may interfere with self-care and the efficient performance of everyday tasks.
JOURNAL OF CYSTIC FIBROSIS
(2022)
Review
Pediatrics
Bernadette Prentice, Michael Nicholson, Grace Y. Lam
Summary: Cystic fibrosis-related diabetes (CFRD) is a common complication of CF that increases with age. Poor glycemic control negatively affects lung function and weight, leading to higher risk of pulmonary exacerbations. The emergence of highly effective modulator therapies (HEMT) has improved the lives of CF patients, but their impact on CFRD is not well understood. This review examines the pathophysiology of CFRD, summarizes available evidence on HEMT impact, and highlights the research needs in this field.
PAEDIATRIC RESPIRATORY REVIEWS
(2023)
Article
Respiratory System
Anna Semaniakou, Frederic Chappe, Younes Anini, Valerie Chappe
Summary: Investigation revealed decreased VIP levels and reduced innervation in the pancreas of CF mice, leading to decreased insulin secretion, increased glucagon production, and elevated random blood glucose levels. Therefore, VIP deficiency may contribute to the development of CFRD.
JOURNAL OF CYSTIC FIBROSIS
(2021)
Article
Endocrinology & Metabolism
Zahrae Sandouk, Farah Khan, Swapnil Khare, Antoinette Moran
Summary: CFRD is a common complication in cystic fibrosis patients, with specific diabetes complications impacting prognosis significantly. Patients may experience abnormal glucose tolerance related to insulin insufficiency even before the clinical diagnosis of CFRD. The negative impact of CFRD on prognosis is thought to be mainly due to protein catabolism, decreased lean body mass, and an inflammatory state related to intermittent high blood sugar levels.
JOURNAL OF CLINICAL AND TRANSLATIONAL ENDOCRINOLOGY
(2021)
Article
Pediatrics
Francesco Maria Rosanio, Enza Mozzillo, Chiara Cimbalo, Alberto Casertano, Angela Sepe, Valeria Raia, Adriana Franzese, Antonella Tosco
Summary: This case firstly highlights the contribution of insulin glargine in preserving the patient from further clinical deterioration due to prediabetes in the years before the pandemic, and secondly underscores the negative impact of the COVID-19 lockdown on the clinical course of a chronic disease like CF.
ITALIAN JOURNAL OF PEDIATRICS
(2021)
Review
Endocrinology & Metabolism
Sarah S. Malik, Diksha Padmanabhan, Rebecca L. Hull-Meichle
Summary: Cystic fibrosis related diabetes is a common complication in patients with cystic fibrosis, but the mechanisms behind its occurrence are not well understood. Cystic fibrosis affects the pancreatic/islet microenvironment, leading to the loss of beta cell function and pathological changes. Further research can help develop better treatment methods.
FRONTIERS IN ENDOCRINOLOGY
(2023)
Article
Critical Care Medicine
Heather D. Green, Andrew M. Jones
Summary: Cystic fibrosis is a disease characterized by chronic airway infection and progressive decline in respiratory function. Recent changes in the landscape of airway infection in CF have resulted in an increased prevalence of atypical gram-negative bacterial infections. The clinical relevance and treatment strategies for many of these lower prevalence organisms are still not well understood.
Review
Critical Care Medicine
Gemma E. Stanford, Kavita Dave, Nicholas J. Simmonds
Summary: Pulmonary exacerbations in cystic fibrosis patients are significant events associated with declining lung function, reduced quality of life, hospitalizations, and decreased survival. With the increasing adult CF population globally, the management of PExs needs to evolve to reflect changing demographics and improve outcomes. Studies are underway to address unmet needs and optimize treatment strategies for PExs in adults with CF.
Review
Medicine, Research & Experimental
Shijing Jia, Jennifer L. Taylor-Cousar
Summary: Cystic fibrosis (CF) is a genetic disease that affects multiple organ systems and can lead to various complications. Traditional treatments focused on managing the symptoms of each affected system. However, the development of modulator therapies targeted at specific genetic mutations has significantly improved the lives and prognosis of CF patients.
ANNUAL REVIEW OF MEDICINE
(2023)
Article
Pediatrics
Raphael Enaud, Eric Frison, Sophie Missonnier, Aude Fischer, Victor de Ledinghen, Paul Perez, Stephanie Bui, Michael Fayon, Jean-Francois Chateil, Thierry Lamireau
Summary: This study evaluated the repeatability and reproducibility of TE and pSWE VTQ in children with CF, finding both techniques to be reliable for assessing liver fibrosis. The high ICC values obtained suggest that TE and pSWE VTQ can be used effectively in the follow-up of CF patients, depending on their availability in CF centers.
PEDIATRIC RESEARCH
(2022)
Review
Pharmacology & Pharmacy
Yizi Wang, Bin Ma, Wenya Li, Peiwen Li
Summary: Triple combination therapy for cystic fibrosis patients achieves better clinical results and comparable adverse events compared to the control group.
FRONTIERS IN PHARMACOLOGY
(2022)
Article
Obstetrics & Gynecology
Rita F. Padoan, Serena Quattrucci, Annalisa Amato, Marco Salvatore, Donatello Salvatore, Giuseppe Campagna
Summary: The study found that preconceptional respiratory function of women with cystic fibrosis (CF) is associated with the duration of pregnancy and birthweight of newborns, and cesarean deliveries are common among young CF women with normal respiratory function.
ACTA OBSTETRICIA ET GYNECOLOGICA SCANDINAVICA
(2021)