Critical Care of the Adult Patient With Cystic Fibrosis

Chest: The Journal of Circulation, Respiration and Related Systems

A. Whitney Brown^1¹  Christopher S. King^1² 

[1] Adult Cystic Fibrosis Center, Division of Pulmonary and Critical Care Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC^2;Adult Cystic Fibrosis Program, Inova Fairfax Hospital, Falls Church, VA^1

关键词: cystic fibrosis;    ECMO and cystic fibrosis;    intensive care;    lung transplant;    mechanical ventilation;    APE;    acute pulmonary exacerbation;    ARF;    acute respiratory failure;    BAE;    bronchial artery embolization;    CF;    cystic fibrosis;    CFRD;    CF-related diabetes mellitus;    CFTR;    CF transmembrane conductance regulator;    DIOS;    distal intestinal obstruction syndrome;    ECMO;    extracorporeal membrane oxygenation;    HHHFNC;    heated-humidity high-flow nasal cannula;    HTS;    hypertonic saline;    IMV;    invasive mechanical ventilation;    MRSA;    methicillin-resistant Staphylococcus aureus;    NIV;    noninvasive ventilation;    PEEP;    positive end-expiratory pressure;    PERT;    pancreatic enzyme replacement therapy;   

DOI  :  10.1016/j.chest.2018.07.025

学科分类：呼吸医学

来源: American College of Chest Physicians

【 摘 要 】

Cystic fibrosis (CF) is a multiorgan, genetic disease resulting from dysfunction of the CF transmembrane conductance regulator. The primary clinical manifestations are bronchiectasis, chronic pulmonary infections with recurrent exacerbations, and pancreatic insufficiency. Advances in CF care have led to improved survival, with more than one-half of those affected being adults. As a result, adult pulmonary and critical care physicians increasingly will be involved in the care of these patients. Patients with CF are at risk for numerous conditions that require ICU admission, including respiratory failure, massive hemoptysis, pneumothorax, hepatic failure, and bowel obstruction. Multiple aspects of the care of patients with CF benefit from specialized knowledge, including pancreatic enzyme replacement and nutritional support; airway clearance modalities; treatment of multiply resistant, polymicrobial infections, and unique drug metabolism. In extreme cases, patients may benefit from advanced therapies, including extracorporeal support and organ transplant. Optimal care of patients with CF requires a multidisciplinary care team that includes respiratory therapists, dieticians, social workers, psychologists, pharmacists, and physicians who have expertise in the treatment of this complex, multisystem disorder.

【 授权许可】

CC BY