Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis

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• 作者：Daniel H. Leung ; MD¹ ; ^{dhleung@texaschildrens.org" class="auth_mail" title="E-mail the corresponding author} ; Wen Ye ; PhD² ; Jean P. Molleston ; MD³ ; Alexander Weymann ; MD⁴ ; Simon Ling ; MD⁵ ; Shruti M. Paranjape ; MD⁶ ; Rene Romero ; MD⁷ ; Sara Jane Schwarzenberg ; MD⁸ ; Joseph Palermo ; MD⁹ ; Estella M. Alonso ; MD¹⁰ ; Karen F. Murray ; MD¹¹ ; Bruce C. Marshall ; MD¹² ; Averell H. Sherker ; MD¹³ ; Marilyn J. Siegel ; MD¹⁴ ; Rajesh Krishnamurthy ; MD¹⁵ ; Roger Harned ; MD¹⁶ ; Boaz Karmazyn ; MD¹⁷ ; John C. Magee ; MD¹⁸ ; Michael R. Narkewicz ; MD¹⁹ ; ^{michael.narkewicz@childrenscolorado.org" class="auth_mail" title="E-mail the corresponding author} ; on behalf of the Cystic Fibrosis Liver Disease Network (CFLD NET)^&lowast ; Estella M. Alonso ; MD ; Jennifer L. Nicholas ; MD ; Elizabeth Kaurs ; Michael R. Narkewicz ; MD ; Ronald J. Sokol ; MD ; Roger Harned ; MD ; Susanna Burr ; Rene Romero ; MD ; Jay Freeman ; MD ; Adina Alazraki ; MD ; Ellen Patrick ; MD ; Eric Hunter ; Simon C. Ling ; MD ; Oscar Navarro ; MD ; Julie P. Ling ; Joe J. Palermo ; MD ; Alex Towbin ; MD ; Andrea Ferris ; Julie Denlinger ; Jean P. Molleston ; MD ; Molly A. Bozic ; MD ; Girish Subbarao ; MD ; Boaz Karmazyn ; MD ; Ann Klipsch ; Shruti M. Paranjape ; MD ; Wikrom Karnsakul ; MD ; Jane E. Benson ; MD ; Karen A. Callahan ; Kim Kafka ; Karen F. Murray ; Ron Gibson ; Randolph Otto ; Alan Genatossio ; Melissa Young ; Alexander Weymann ; Marilyn J. Siegel ; Kathy Harris ; Daniel H. Leung ; Rajesh Krishnamurthy ; Jameisha Brown ; Sara Jane Schwarzenberg ; Denise Stacklie ; F. Glenn Seidel ; Edward Doo ; MD ; Averell H. Sherker ; MD ; FRCP(C) ; Sherry R. Hall ; MS ; Rebecca Torrance ; RN ; MS
• 关键词：BMI ; Body mass index ; CF ; Cystic fibrosis ; CFLD ; CF liver disease ; CFLD NET ; Cystic Fibrosis Liver Disease Network ; FEV1 ; Forced expiratory volume in 1 second ; NAFLD ; Nonalcoholic fatty liver disease ; PUSH ; Prediction by Ultrasound of the Risk of Hepatic Cirrhosis in Cystic Fibrosis
• 刊名：The Journal of Pediatrics
• 出版年：2015
• 出版时间：October 2015
• 年：2015
• 卷：167
• 期：4
• 页码：862-868.e2
• 全文大小：271 K

文摘

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. 蠂² 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.