Evidence map›Paper›PMID 32061406›Full record

Observational studyThe Journal of pediatrics2020

Heterogeneous Liver on Research Ultrasound Identifies Children with Cystic Fibrosis at High Risk of Advanced Liver Disease: Interim Results of a Prospective Observational Case-Controlled Study.

Marilyn J Siegel, A Jay Freeman, Wen Ye, Joseph J Palermo, Jean P Molleston, Shruti M Paranjape, Janis Stoll, Daniel H Leung, Prakash Masand, Boaz Karmazyn and 16 more

Registry-linked trialOpen access · greenAbstract readMulticenter StudyObservational Study
In one paragraph

Observational study in The Journal of pediatrics, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT01144507 (Prediction by Ultrasound of the Risk of Hepatic Cirrhosis in Cystic Fibrosis), which is not on this map. Cited by 9 papers.

0numbers the graph read from it
0cells of the map it votes in
9citing papers in PubMed
3.5field-weighted citation impact, top 6% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

NCT01144507 completednot on this map

Prediction by Ultrasound of the Risk of Hepatic Cirrhosis in Cystic Fibrosis (PUSH)

TypeobservationalSponsorNational Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)Ran2010 to 2023Enrolled774ConditionsCystic Fibrosis, Pancreatic InsufficiencyArmsAbdominal Ultrasound, Sample collection procedures
3 · Its place in the literature

Who cites it

9 citing papers in PubMed, 28 citations in OpenAlex.

  1. Trial
  2. Biomarkers for liver disease in urea cycle disorders.Molecular genetics and metabolism · 2021
    Trial
  3. Review
  4. Article
  5. Heterogeneous liver on research ultrasound identifies children with cystic fibrosis at high risk of advanced liver disease.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2023
    Observational
  6. Cystic fibrosis related liver disease and endocrine considerations.Journal of clinical & translational endocrinology · 2022
    Article
  7. Article
  8. Review
  9. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

26 authors at 15 institutions in 2 countries.

Marilyn J SiegelMallinckrodt Institute of Radiology, Washington University School of Medicine, St Louis, MO.
A Jay FreemanDivision of Pediatric Gastroenterology, Hepatology and Nutrition, Emory University School of Medicine, Atlanta, GA.
Wen YeDepartment of Biostatistics, University of Michigan Medical School, Ann Arbor, MI.
Joseph J PalermoDivision of Pediatric Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH.
Jean P MollestonPediatric Gastroenterology, Hepatology and Nutrition, Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, IN.
Shruti M ParanjapeDivision of Pediatric Pulmonology, John Hopkins School of Medicine, Baltimore, MD.
Janis StollDivision of Gastroenterology and Nutrition, Washington University School of Medicine, St Louis, MO.
Daniel H LeungDivision of Gastroenterology, Hepatology and Nutrition, Texas Children's Hospital, Houston, TX.
Prakash MasandDivision of Radiology, Texas Children's Hospital, Houston, TX.
Boaz KarmazynPediatric Radiology, Riley Hospital for Children, Indianapolis, IN.
Roger HarnedDivision of Pediatric Radiology, Children's Hospital Colorado and University of Colorado School of Medicine, Aurora, CO.
Simon C LingDivision of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada.
Oscar M NavarroDepartment of Medical Imaging, University of Toronto, Toronto, Ontario, Canada; Department of Diagnostic Imaging, The Hospital for Sick Children, Toronto, Ontario, Canada.
Wikrom KarnsakulDivision of Pediatric Gastroenterology, Hepatology and Nutrition, John Hopkins School of Medicine, Baltimore, MD.
Adina AlazrakiDepartment of Radiology, Emory University School of Medicine and Children's Healthcare of Atlanta, Egleston, Atlanta, GA.
Sarah Jane SchwarzenbergPediatric Gastroenterology, University of Minnesota Masonic Children's Hospital, Minneapolis, MN.
Frank Glen SeidelPediatric Radiology, Lucile Packard Children's Hospital, Stanford, CA.
Alex TowbinDepartment of Radiology, Cincinnati Children's Hospital Medical Center and Department of Radiology, University of Cincinnati College of Medicine, Cincinnati, OH.
Estella M AlonsoDivision of Pediatric Gastroenterology, Hepatology and Nutrition, Ann & Robert H. Lurie Children's Hospital, Chicago, IL.
Jennifer L NicholasMallinckrodt Institute of Radiology, Washington University School of Medicine, St Louis, MO.
Karen F MurrayDivision of Gastroenterology and Hepatology, University of Washington and Seattle Children's Hospital, Seattle, WA.
Randolph K OttoDepartment of Radiology, Seattle Children's Hospital, Seattle, WA.
Averell H SherkerLiver Diseases Branch, NIDDK, NIH, Bethesda, MD.
John C MageeDepartment of Surgery, University of Michigan Medical School, Ann Arbor, MI.
Michael R NarkewiczDigestive Health Institute, Children's Hospital Colorado and Section of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO. Electronic address: michael.narkewicz@childrenscolorado.org.
CFLD Network
Children's Hospital Colorado · USCincinnati Children's Hospital Medical Center · USEmory University · USJohns Hopkins University · USMallinckrodt (United States) · USRiley Hospital for Children · USSeattle Children's Hospital · USTexas Children's Hospital · USUniversity of Michigan · USUniversity of Toronto · CALucile Packard Children's Hospital · USLurie Children's Hospital · USNational Institutes of Health · USUniversity of Minnesota Children's Hospital · USWashington University in St. Louis · US

Funding

Childhood Liver Disease Research Network Data Coordinating CenterU01DK062456 · NIDDK · UNIVERSITY OF MICHIGAN AT ANN ARBOR · PI MAGEE, JOHN C, MERION, ROBERT M · 2002 to 2019
$36.8M
Childhood Liver Disease Research Network Data Coordinating CenterU24DK062456 · NIDDK · UNIVERSITY OF MICHIGAN AT ANN ARBOR · PI JOHN C MAGEE · 2019 to 2026
$18.9M
Etiology and Treatment of Biliary Atresia and INHU01DK062453 · NIDDK · UNIVERSITY OF COLORADO DENVER · PI RONALD J. SOKOL · 2002 to 2026
$13.5M
Continuation of ChiLDReN, the Childhood Liver Disease Research Network: Indiana UniversityU01DK084536 · NIDDK · INDIANA UNIVERSITY INDIANAPOLIS · PI MOLLESTON, JEAN PAPPAS · 2009 to 2023
$5.9M
Clinical Center for ChiLDREN: Pathogenesis, Biomarkers, and Antifibrotic TherapyU01DK103149 · NIDDK · BAYLOR COLLEGE OF MEDICINE · PI BENJAMIN L SHNEIDER · 2014 to 2026
$4.8M
NIDDK NIH HHS U01 DK062453NIDDK NIH HHS U01 DK062456NIDDK NIH HHS U01 DK084536NIDDK NIH HHS U01 DK103149NIDDK NIH HHS U24 DK062456
6 · The paper itself

Abstract

objectiveTo assess if a heterogeneous pattern on research liver ultrasound examination can identify children at risk for advanced cystic fibrosis (CF) liver disease. STUDY

designPlanned 4-year interim analysis of a 9-year multicenter, case-controlled cohort study (Prospective Study of Ultrasound to Predict Hepatic Cirrhosis in CF). Children with pancreatic insufficient CF aged 3-12 years without known cirrhosis, Burkholderia species infection, or short bowel syndrome underwent a screening research ultrasound examination. Participants with a heterogeneous liver ultrasound pattern were matched (by age, Pseudomonas infection status, and center) 1:2 with participants with a normal pattern. Clinical status and laboratory data were obtained annually and research ultrasound examinations biannually. The primary end point was the development of a nodular research ultrasound pattern, a surrogate for advanced CF liver disease.

resultsThere were 722 participants who underwent screening research ultrasound examination, of which 65 were heterogeneous liver ultrasound pattern and 592 normal liver ultrasound pattern. The final cohort included 55 participants with a heterogeneous liver ultrasound pattern and 116 participants with a normal liver ultrasound pattern. All participants with at least 1 follow-up research ultrasound were included. There were no differences in age or sex between groups at entry. Alanine aminotransferase (42 ± 22 U/L vs 32 ± 19 U/L; P = .0033), gamma glutamyl transpeptidase (36 ± 34 U/L vs 15 ± 8 U/L; P < .001), and aspartate aminotransferase to platelet ratio index (0.7 ± 0.5 vs 0.4 ± 0.2; P < .0001) were higher in participants with a heterogeneous liver ultrasound pattern compared with participants with a normal liver ultrasound pattern. Participants with a heterogeneous liver ultrasound pattern had a 9.1-fold increased incidence (95% CI, 2.7-30.8; P = .0004) of nodular pattern vs a normal liver ultrasound pattern (23% in heterogeneous liver ultrasound pattern vs 2.6% in normal liver ultrasound pattern).

conclusionsResearch liver ultrasound examinations can identify children with CF at increased risk for developing advanced CF liver disease.

Indexed as

Case-Control StudiesChildChild, PreschoolCystic FibrosisFemaleHumansLiverLiver DiseasesMaleProspective StudiesRisk AssessmentUltrasonographycirrhosiscystic fibrosis liver disease

Identifiers

PMID32061406
PMCPMC7096278
OpenAlexW3006074781

What OpenQuestion holds

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Registered trials

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.