TY - JOUR
T1 - Multisystem imaging findings of cystic fibrosis in adults
T2 - Recognizing typical and atypical patterns of disease
AU - Averill, Sarah
AU - Lubner, Meghan G.
AU - Menias, Christine O.
AU - Bhalla, Sanjeev
AU - Mellnick, Vincent M.
AU - Kennedy, Tabassum A.
AU - Pickhardt, Perry J.
N1 - Publisher Copyright:
© American Roentgen Ray Society.
PY - 2017/7
Y1 - 2017/7
N2 - OBJECTIVE. There is an expanding and increasingly heterogeneous population of adult patients with cystic fibrosis (CF). Although CF is usually diagnosed in children with progressive multisystem involvement, up to 7% of CF cases are currently diagnosed de novo in adults with subtle manifestations distinct from the typical features of classic CF. The purpose of this article is to present the wide spectrum of CF in adults, including both classic and nonclassic variants, with an emphasis on the nonclassic imaging findings. CONCLUSION. Recurrent pancreatitis, chronic sinusitis, and congenital bilateral absence of the vas deferens (CBAVD) are several of the ways in which CF is identified in adult patients with relatively rare mutations and with overall milder manifestations. It is important for radiologists to recognize the wide spectrum of CF to optimally monitor disease progression and response to therapeutic interventions in distinct adult patient populations.
AB - OBJECTIVE. There is an expanding and increasingly heterogeneous population of adult patients with cystic fibrosis (CF). Although CF is usually diagnosed in children with progressive multisystem involvement, up to 7% of CF cases are currently diagnosed de novo in adults with subtle manifestations distinct from the typical features of classic CF. The purpose of this article is to present the wide spectrum of CF in adults, including both classic and nonclassic variants, with an emphasis on the nonclassic imaging findings. CONCLUSION. Recurrent pancreatitis, chronic sinusitis, and congenital bilateral absence of the vas deferens (CBAVD) are several of the ways in which CF is identified in adult patients with relatively rare mutations and with overall milder manifestations. It is important for radiologists to recognize the wide spectrum of CF to optimally monitor disease progression and response to therapeutic interventions in distinct adult patient populations.
KW - Adults
KW - Cystic fibrosis
KW - Multisystem
UR - http://www.scopus.com/inward/record.url?scp=85021052185&partnerID=8YFLogxK
U2 - 10.2214/AJR.16.17462
DO - 10.2214/AJR.16.17462
M3 - Review article
C2 - 28639921
AN - SCOPUS:85021052185
SN - 0361-803X
VL - 209
SP - 3
EP - 18
JO - American Journal of Roentgenology
JF - American Journal of Roentgenology
IS - 1
ER -