TY - JOUR
T1 - Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis
AU - on behalf of the
AU - Cystic Fibrosis Liver Disease Network (CFLD NET)
AU - Cystic Fibrosis Liver Disease Network (CFLD NET)
AU - Leung, Daniel H.
AU - Ye, Wen
AU - Molleston, Jean P.
AU - Weymann, Alexander
AU - Ling, Simon
AU - Paranjape, Shruti M.
AU - Romero, Rene
AU - Schwarzenberg, Sara Jane
AU - Palermo, Joseph
AU - Alonso, Estella M.
AU - Murray, Karen F.
AU - Marshall, Bruce C.
AU - Sherker, Averell H.
AU - Siegel, Marilyn J.
AU - Krishnamurthy, Rajesh
AU - Harned, Roger
AU - Karmazyn, Boaz
AU - Magee, John C.
AU - Narkewicz, Michael R.
AU - Alonso, Estella M.
AU - Nicholas, Jennifer L.
AU - Kaurs, Elizabeth
AU - Narkewicz, Michael R.
AU - Sokol, Ronald J.
AU - Harned, Roger
AU - Burr, Susanna
AU - Romero, Rene
AU - Freeman, Jay
AU - Alazraki, Adina
AU - Patrick, Ellen
AU - Hunter, Eric
AU - Ling, Simon C.
AU - Navarro, Oscar
AU - Ling, Julie P.
AU - Palermo, Joe J.
AU - Towbin, Alex
AU - Ferris, Andrea
AU - Denlinger, Julie
AU - Molleston, Jean P.
AU - Bozic, Molly A.
AU - Subbarao, Girish
AU - Karmazyn, Boaz
AU - Klipsch, Ann
AU - Paranjape, Shruti M.
AU - Karnsakul, Wikrom
AU - Benson, Jane E.
AU - Callahan, Karen A.
AU - Kafka, Kim
AU - Murray, Karen F.
AU - Gibson, Ron
N1 - Funding Information:
Jointly supported by the Cystic Fibrosis Foundation (NARKEW07A0 [Colorado]) and the National Institute of Diabetes and Digestive and Kidney (DK062453 and DK62456 [The University of Michigan]), the National Institutes of Health (NIH)/National Center for Advancing Translational Sciences (NCATS; UL1 TR001082 [Colorado]; UL1 TR000077 [Cincinnati], DK084536-07; UL1 TR000150 [Northwestern University]), Clinical & Translational Science Institute (CTSI UL1TR001108 [Indiana University]), Institute for Clinical & Translational Research/NCATS/NIH (UL1 TR 001079 [Johns Hopkins]), NIH/Northwestern University Clinical and Translational Sciences Institute/Institute of Translational Health Sciences (RR025014), and NIH (Clinical and Translational Science Award TR000423 [University of Washington]). Contents are the authors? sole responsibility and do not necessarily represent official NIH views. B.M. is a full time employee of the Cystic Fibrosis Foundation. The other authors declare no conflicts of interest.
PY - 2015/10/1
Y1 - 2015/10/1
N2 - Objective To investigate the relationship between abdominal ultrasound findings and demographic, historical, and clinical features in children with cystic fibrosis (CF). Study design Children age 3-12 years with CF without known cirrhosis, were enrolled in a prospective, multicenter study of ultrasound to predict hepatic fibrosis. Consensus ultrasound patterns were assigned by 3 radiologists as normal, heterogeneous, homogeneous, or cirrhosis. Data were derived from direct collection and US or Toronto CF registries. χ2 or ANOVA were used to compare variables among ultrasound groups and between normal and abnormal. Logistic regression was used to study risk factors for having abnormal ultrasound. Results Findings in 719 subjects were normal (n = 590, 82.1%), heterogeneous (64, 8.9%), homogeneous (41, 5.7%), and cirrhosis (24, 3.3%). Cirrhosis (P = .0004), homogeneous (P < .0001), and heterogeneous (P = .03) were older than normal. More males were heterogeneous (P = .001). More heterogeneous (15.0%, P = .009) and cirrhosis (25.0%, P = .005) had CF-related diabetes or impaired glucose tolerance vs normal (5.4%). Early infection with Pseudomonas aeruginosa (<2 years old) was associated with a lower risk (OR 0.42, P = .0007) of abnormal. Ursodeoxycholic acid use (OR 3.69, P < .0001) and CF-related diabetes (OR 2.21, P = .019) were associated with increased risk of abnormal. Conclusions Unsuspected cirrhosis is seen in 3.3% of young patients with CF, heterogeneous in 8.9%. Abnormal ultrasound is associated with CF-related diabetes, and early P aeruginosa is associated with normal ultrasound. Prospective assessment of these risk factors may identify potential interventional targets. Trial registration ClinicalTrials.gov: NCT01144507.
AB - Objective To investigate the relationship between abdominal ultrasound findings and demographic, historical, and clinical features in children with cystic fibrosis (CF). Study design Children age 3-12 years with CF without known cirrhosis, were enrolled in a prospective, multicenter study of ultrasound to predict hepatic fibrosis. Consensus ultrasound patterns were assigned by 3 radiologists as normal, heterogeneous, homogeneous, or cirrhosis. Data were derived from direct collection and US or Toronto CF registries. χ2 or ANOVA were used to compare variables among ultrasound groups and between normal and abnormal. Logistic regression was used to study risk factors for having abnormal ultrasound. Results Findings in 719 subjects were normal (n = 590, 82.1%), heterogeneous (64, 8.9%), homogeneous (41, 5.7%), and cirrhosis (24, 3.3%). Cirrhosis (P = .0004), homogeneous (P < .0001), and heterogeneous (P = .03) were older than normal. More males were heterogeneous (P = .001). More heterogeneous (15.0%, P = .009) and cirrhosis (25.0%, P = .005) had CF-related diabetes or impaired glucose tolerance vs normal (5.4%). Early infection with Pseudomonas aeruginosa (<2 years old) was associated with a lower risk (OR 0.42, P = .0007) of abnormal. Ursodeoxycholic acid use (OR 3.69, P < .0001) and CF-related diabetes (OR 2.21, P = .019) were associated with increased risk of abnormal. Conclusions Unsuspected cirrhosis is seen in 3.3% of young patients with CF, heterogeneous in 8.9%. Abnormal ultrasound is associated with CF-related diabetes, and early P aeruginosa is associated with normal ultrasound. Prospective assessment of these risk factors may identify potential interventional targets. Trial registration ClinicalTrials.gov: NCT01144507.
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U2 - 10.1016/j.jpeds.2015.06.062
DO - 10.1016/j.jpeds.2015.06.062
M3 - Article
C2 - 26254836
AN - SCOPUS:84938634331
VL - 167
SP - 862-868.e2
JO - Journal of Pediatrics
JF - Journal of Pediatrics
SN - 0022-3476
IS - 4
ER -