TY - JOUR
T1 - Cystic fibrosis related diabetes (CFRD) prognosis
AU - Sandouk, Zahrae
AU - Khan, Farah
AU - Khare, Swapnil
AU - Moran, Antoinette
N1 - Publisher Copyright:
© 2021 The Authors
PY - 2021/12
Y1 - 2021/12
N2 - Cystic fibrosis related diabetes (CFRD) occurs in at least 40–50% of adults with CF. With other forms of diabetes, microvascular and macrovascular disease are the major causes of morbidity and mortality. Macrovascular disease is rare in CF. While microvascular disease does occur in this population, there are CF-specific diabetes complications that have a more important impact on prognosis. The additional diagnosis of diabetes in CF is associated with decreased lung function, poor nutritional status, and an overall increase in mortality from lung disease. These negative findings start even before the clinical diagnosis of CFRD, during the period when patients experience abnormal glucose tolerance related to insulin insufficiency. The main mechanisms by which CFRD negatively affects prognosis are thought to be a combination of 1) protein catabolism, decreased lean body mass and undernutrition resulting from insulin insufficiency, and 2) an increased pro-inflammatory and pro-infectious state related to intermittent hyperglycemia. With the introduction of CFTR modulators, the care of CF patients has been revolutionized and many aspects of CF health such as BMI and lung function are improving. The impact of these drugs on the adverse prognosis related to the diagnosis of diabetes in CF, as well as the potential to delay or prevent onset of CFRD remain to be determined.
AB - Cystic fibrosis related diabetes (CFRD) occurs in at least 40–50% of adults with CF. With other forms of diabetes, microvascular and macrovascular disease are the major causes of morbidity and mortality. Macrovascular disease is rare in CF. While microvascular disease does occur in this population, there are CF-specific diabetes complications that have a more important impact on prognosis. The additional diagnosis of diabetes in CF is associated with decreased lung function, poor nutritional status, and an overall increase in mortality from lung disease. These negative findings start even before the clinical diagnosis of CFRD, during the period when patients experience abnormal glucose tolerance related to insulin insufficiency. The main mechanisms by which CFRD negatively affects prognosis are thought to be a combination of 1) protein catabolism, decreased lean body mass and undernutrition resulting from insulin insufficiency, and 2) an increased pro-inflammatory and pro-infectious state related to intermittent hyperglycemia. With the introduction of CFTR modulators, the care of CF patients has been revolutionized and many aspects of CF health such as BMI and lung function are improving. The impact of these drugs on the adverse prognosis related to the diagnosis of diabetes in CF, as well as the potential to delay or prevent onset of CFRD remain to be determined.
KW - CFTR modulators
KW - Cystic fibrosis
KW - Cystic fibrosis related diabetes
KW - Macrovascular complications
KW - Microvascular complications
KW - Prognosis
KW - Transplant prognosis
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U2 - 10.1016/j.jcte.2021.100278
DO - 10.1016/j.jcte.2021.100278
M3 - Article
C2 - 34926166
AN - SCOPUS:85120648141
SN - 2214-6237
VL - 26
JO - Journal of Clinical and Translational Endocrinology
JF - Journal of Clinical and Translational Endocrinology
M1 - 100278
ER -