Articles Related to Cystic Fibrosis
Elexacaftor/Tezacaftor/Ivacaftor Improves Glycemic Control in Pediatric Patients with Cystic Fibrosis-Related Diabetes
Cystic fibrosis (CF) is an inherited disorder caused by genetic mutations encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) is a CFTR modulator shown to improve lung function in certain patients with CF. We undertook this study to determine its effects on glycemic outcomes in pediatric patients with cystic fibrosis-related diabetes (CFRD). We reviewed the medical records and identified two subjects, referred to as subject A and subject B, with CFRD on insulin therapy at Duke University Hospital between 2019-2020 who were on treatment with ELX/TEZ/IVA for at least one year. The mean hgbA1C pre- and post- treatment was 5.65% (5.6-5.7) and 5.05% (5.0-5.1) respectively with a mean reduction of 0.6% (p value 0.01). Improvement in hgbA1C occurred in the absence of increased insulin requirements, subject B remained on a similar insulin regimen whereas subject A was able to come off insulin completely. In addition, improvements in BMI z-score were seen in both groups with a mean BMI z-score of -0.30 pre-treatment (-0.71-0.11) and z-score of +0.28 post-treatment (0.11-0.45).
Monotherapy versus Combination Therapy for the Treatment of Pseudomonas Aeruginosa in Cystic Fibrosis Patients
Infection with Pseudomonas aeruginosa in cystic fibrosis (CF) patients is a strong contributor to respiratory failure and associated mortality. Bacteria colonizing a cystic fibrosis lung commonly form biofilms that greatly contribute to increased antibiotic resistance and hypermutability. Antimicrobial treatment in these cases can be either through the use of a single agent (monotherapy) or through a combination of agents (combination therapy).
Editorial Board Members Related to Cystic Fibrosis
Guoshun Wang
Associate Professor
Department of Microbiology, Immunology and Parasitology
LSU Health New Orleans
United States
Department of Microbiology, Immunology and Parasitology
LSU Health New Orleans
United States
Henry Lawrence Dorkin
Associate Professor of Pediatrics
Harvard Medical School
Harvard University
United Stated
Harvard Medical School
Harvard University
United Stated
CHARLES C. MUSCOPLAT
Professor
Department of Food Science and Nutrition
University of Minnesota
United States
Department of Food Science and Nutrition
University of Minnesota
United States
Chunying Li
Assistant Professor
Department of Biochemistry and Molecular Biology
Wayne State University School of Medicine
United States
Department of Biochemistry and Molecular Biology
Wayne State University School of Medicine
United States
Guoshun Wang
Associate Professor
Departments of Microbiology and Immunology, Genetics, and Medicine
Louisiana State University Health Sciences Center
United States
Departments of Microbiology and Immunology, Genetics, and Medicine
Louisiana State University Health Sciences Center
United States
Abdelwahab Omri
Professor
Department of Chemistry and Biochemistry
Laurentian University
Canada
Department of Chemistry and Biochemistry
Laurentian University
Canada
Joseph Prandota
Emeritus Full Professor
Department of Pediatrics & Clinical Pharmacology
Faculty of Health Sciences
Wroclaw Medical University
Wroclaw
Poland
Department of Pediatrics & Clinical Pharmacology
Faculty of Health Sciences
Wroclaw Medical University
Wroclaw
Poland
Miles Weinberger
Professor
Department of Pediatrics
University of Iowa Cystic Fibrosis Center
United States
Department of Pediatrics
University of Iowa Cystic Fibrosis Center
United States