Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis

Daniel H. Leung, Wen Ye, Jean P. Molleston, Alexander Weymann, Simon Ling, Shruti M. Paranjape, Rene Romero, Sara J ane Schwarzenberg, Joseph Palermo, Estella M. Alonso, Karen F. Murray, Bruce C. Marshall, Averell H. Sherker, Marilyn J. Siegel, Rajesh Krishnamurthy, Roger Harned, Boaz Karmazyn, John C. Magee, Michael R. Narkewicz

Research output: Contribution to journalArticle

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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

Fingerprint

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

PubMed: MeSH publication types

  • Journal Article
  • Multicenter Study
  • Observational Study
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

Cite this

Leung, D. H., Ye, W., Molleston, J. P., Weymann, A., Ling, S., Paranjape, S. M., ... Narkewicz, M. R. (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. / Leung, Daniel H.; Ye, Wen; Molleston, Jean P.; Weymann, Alexander; Ling, Simon; Paranjape, Shruti M.; Romero, Rene; Schwarzenberg, Sara J ane; Palermo, Joseph; Alonso, Estella M.; Murray, Karen F.; Marshall, Bruce C.; Sherker, Averell H.; Siegel, Marilyn J.; Krishnamurthy, Rajesh; Harned, Roger; Karmazyn, Boaz; Magee, John C.; Narkewicz, Michael R.

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

Research output: Contribution to journalArticle

Leung, DH, Ye, W, Molleston, JP, Weymann, A, Ling, S, Paranjape, SM, Romero, R, Schwarzenberg, SJA, Palermo, J, Alonso, EM, Murray, KF, Marshall, BC, Sherker, AH, Siegel, MJ, Krishnamurthy, R, Harned, R, Karmazyn, B, Magee, JC & Narkewicz, MR 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
Leung DH, Ye W, Molleston JP, Weymann A, Ling S, Paranjape SM et al. 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
Leung, Daniel H. ; Ye, Wen ; Molleston, Jean P. ; Weymann, Alexander ; Ling, Simon ; Paranjape, Shruti M. ; Romero, Rene ; Schwarzenberg, Sara J ane ; Palermo, Joseph ; Alonso, Estella M. ; Murray, Karen F. ; Marshall, Bruce C. ; Sherker, Averell H. ; Siegel, Marilyn J. ; Krishnamurthy, Rajesh ; Harned, Roger ; Karmazyn, Boaz ; Magee, John C. ; Narkewicz, Michael R. / 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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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 = "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 J ane} and Joseph Palermo and Alonso, {Estella M.} and Murray, {Karen F.} and Marshall, {Bruce C.} 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.}",
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T1 - Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis

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 J ane

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.

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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