Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis

on behalf of the, Cystic Fibrosis Liver Disease Network (CFLD NET), Cystic Fibrosis Liver Disease Network (CFLD NET)

Research output: Contribution to journal › Article

Abstract

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. χ(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 (

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.

Original languageEnglish (US)
Pages (from-to)862-868
Number of pages7
JournalJournal of Pediatrics
Volume167
Issue number4
DOIs
StatePublished - Oct 1 2015

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Cystic Fibrosis
Fibrosis
Glucose Intolerance
Pseudomonas aeruginosa
Multicenter Studies
Registries
Analysis of Variance
Logistic Models
Demography
Prospective Studies
Liver
Infection

ASJC Scopus subject areas

  • Medicine(all)

Cite this

on behalf of the, Cystic Fibrosis Liver Disease Network (CFLD NET), & Cystic Fibrosis Liver Disease Network (CFLD NET) (2015). Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis. Journal of Pediatrics, 167(4), 862-868. https://doi.org/10.1016/j.jpeds.2015.06.062

Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis. / on behalf of the; Cystic Fibrosis Liver Disease Network (CFLD NET); Cystic Fibrosis Liver Disease Network (CFLD NET).

In: Journal of Pediatrics, Vol. 167, No. 4, 01.10.2015, p. 862-868.

Research output: Contribution to journal › Article

on behalf of the, Cystic Fibrosis Liver Disease Network (CFLD NET) & Cystic Fibrosis Liver Disease Network (CFLD NET) 2015, 'Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis', Journal of Pediatrics, vol. 167, no. 4, pp. 862-868. https://doi.org/10.1016/j.jpeds.2015.06.062
on behalf of the, Cystic Fibrosis Liver Disease Network (CFLD NET), Cystic Fibrosis Liver Disease Network (CFLD NET). Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis. Journal of Pediatrics. 2015 Oct 1;167(4):862-868. https://doi.org/10.1016/j.jpeds.2015.06.062
on behalf of the ; Cystic Fibrosis Liver Disease Network (CFLD NET) ; Cystic Fibrosis Liver Disease Network (CFLD NET). / Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis. In: Journal of Pediatrics. 2015 ; Vol. 167, No. 4. pp. 862-868.
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title = "Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis",
abstract = "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. χ(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 (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.",
author = "{on behalf of the} and {Cystic Fibrosis Liver Disease Network (CFLD NET)} and {Cystic Fibrosis Liver Disease Network (CFLD NET)} and Leung, {Daniel H.} and Wen Ye and Molleston, {Jean P.} and Alexander Weymann and Simon Ling and Paranjape, {Shruti M} and Rene Romero and Schwarzenberg, {Sara Jane} and Joseph Palermo and Alonso, {Estella M.} and Murray, {Karen F.} and Bruce Marshall and Sherker, {Averell H.} and Siegel, {Marilyn J.} and Rajesh Krishnamurthy and Roger Harned and Boaz Karmazyn and Magee, {John C.} and Narkewicz, {Michael R.} and Alonso, {Estella M.} and Nicholas, {Jennifer L.} and Elizabeth Kaurs and Narkewicz, {Michael R.} and Sokol, {Ronald J.} and Roger Harned and Susanna Burr and Rene Romero and Jay Freeman and Adina Alazraki and Ellen Patrick and Eric Hunter and Ling, {Simon C.} and Oscar Navarro and Ling, {Julie P.} and Palermo, {Joe J.} and Alex Towbin and Andrea Ferris and Julie Denlinger and Molleston, {Jean P.} and Bozic, {Molly A.} and Girish Subbarao and Boaz Karmazyn and Ann Klipsch and Paranjape, {Shruti M.} and Karnsakul, {Wikrom W} and Benson, {Jane E.} and Callahan, {Karen A.} and Kim Kafka and Murray, {Karen F.} and Ron Gibson",
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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

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 W

AU - Benson, Jane E.

AU - Callahan, Karen A.

AU - Kafka, Kim

AU - Murray, Karen F.

AU - Gibson, Ron

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. χ(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 (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. χ(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 (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

EP - 868

JO - Journal of Pediatrics

JF - Journal of Pediatrics

SN - 0022-3476

IS - 4

ER -

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