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Cystic Fibrosis-Related Diabetes Workshop: Research Priorities Spanning Disease Pathophysiology, Diagnosis, and Outcomes.
Putman, Melissa S; Norris, Andrew W; Hull, Rebecca L; Rickels, Michael R; Sussel, Lori; Blackman, Scott M; Chan, Christine L; Ode, Katie Larson; Daley, Tanicia; Stecenko, Arlene A; Moran, Antoinette; Helmick, Meagan J; Cray, Sharon; Alvarez, Jessica A; Stallings, Virginia A; Tuggle, Katherine L; Clancy, John P; Eggerman, Thomas L; Engelhardt, John F; Kelly, Andrea.
Afiliação
  • Putman MS; Division of Pediatric Endocrinology, Boston Children's Hospital, Boston, MA.
  • Norris AW; Diabetes Research Center, Massachusetts General Hospital, Boston, MA.
  • Hull RL; Department of Pediatrics, University of Iowa, Iowa City, IA.
  • Rickels MR; Fraternal Order of Eagles Diabetes Research Center, University of Iowa, Iowa City, IA.
  • Sussel L; Division of Metabolism, Endocrinology, and Nutrition, Department of Medicine, University of Washington, Seattle, WA.
  • Blackman SM; Research Service, VA Puget Sound Health Care System, Seattle, WA.
  • Chan CL; Division of Endocrinology, Diabetes, and Metabolism, Department of Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA.
  • Ode KL; Institute for Diabetes, Obesity, and Metabolism, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA.
  • Daley T; Department of Pediatrics, Barbara Davis Center for Diabetes, University of Colorado Anschutz Medical Campus, Aurora, CO.
  • Stecenko AA; Division of Pediatric Endocrinology and Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD.
  • Moran A; Department of Pediatrics, Barbara Davis Center for Diabetes, University of Colorado Anschutz Medical Campus, Aurora, CO.
  • Helmick MJ; Department of Pediatrics, University of Iowa, Iowa City, IA.
  • Cray S; Fraternal Order of Eagles Diabetes Research Center, University of Iowa, Iowa City, IA.
  • Alvarez JA; Division of Endocrinology, Department of Pediatrics, Emory University School of Medicine, Atlanta, GA.
  • Stallings VA; Children's Healthcare of Atlanta, Atlanta, GA.
  • Tuggle KL; Division of Pulmonology, Asthma, Cystic Fibrosis, and Sleep, Department of Pediatrics, Emory University, Atlanta, GA.
  • Clancy JP; Department of Pediatrics, University of Minnesota, Minneapolis, MN.
  • Eggerman TL; Cystic Fibrosis Foundation, Bethesda, MD.
  • Engelhardt JF; Cystic Fibrosis Foundation, Bethesda, MD.
  • Kelly A; Division of Endocrinology, Metabolism, and Lipids, Department of Medicine, Emory School of Medicine, Atlanta, GA.
Diabetes ; 72(6): 677-689, 2023 06 01.
Article em En | MEDLINE | ID: mdl-37125945
Cystic fibrosis (CF) is a recessive disorder arising from mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein. CFTR is expressed in numerous tissues, with high expression in the airways, small and large intestine, pancreatic and hepatobiliary ducts, and male reproductive tract. CFTR loss in these tissues disrupts regulation of salt, bicarbonate, and water balance across their epithelia, resulting in a systemic disorder with progressive organ dysfunction and damage. Pancreatic exocrine damage ultimately manifests as pancreatic exocrine insufficiency that begins as early as infancy. Pancreatic remodeling accompanies this early damage, during which abnormal glucose tolerance can be observed in toddlers. With increasing age, however, insulin secretion defects progress such that CF-related diabetes (CFRD) occurs in 20% of teens and up to half of adults with CF. The relevance of CFRD is highlighted by its association with increased morbidity, mortality, and patient burden. While clinical research on CFRD has greatly assisted in the care of individuals with CFRD, key knowledge gaps on CFRD pathogenesis remain. Furthermore, the wide use of CFTR modulators to restore CFTR activity is changing the CFRD clinical landscape and the field's understanding of CFRD pathogenesis. For these reasons, the National Institute of Diabetes and Digestive and Kidney Diseases and the Cystic Fibrosis Foundation sponsored a CFRD Scientific Workshop, 23-25 June 2021, to define knowledge gaps and needed research areas. This article describes the findings from this workshop and plots a path for CFRD research that is needed over the next decade.
Assuntos

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Intolerância à Glucose / Fibrose Cística / Diabetes Mellitus Tipo de estudo: Diagnostic_studies / Etiology_studies Limite: Adolescent / Adult / Humans / Male Idioma: En Ano de publicação: 2023 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Intolerância à Glucose / Fibrose Cística / Diabetes Mellitus Tipo de estudo: Diagnostic_studies / Etiology_studies Limite: Adolescent / Adult / Humans / Male Idioma: En Ano de publicação: 2023 Tipo de documento: Article