Evidence map›Paper›PMID 31553814›Full record

ArticleHepatology (Baltimore, Md.)2020

Crigler-Najjar Syndrome Type 1: Pathophysiology, Natural History, and Therapeutic Frontier.

Kevin A Strauss, Charles E Ahlfors, Kyle Soltys, George V Mazareigos, Millie Young, Lauren E Bowser, Michael D Fox, James E Squires, Patrick McKiernan, Karlla W Brigatti and 3 more

Open access · greenAbstract read
In one paragraph

Article in Hepatology (Baltimore, Md.), 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.

0numbers the graph read from it
0cells of the map it votes in
20citing papers in PubMed
4.0field-weighted citation impact, top 7% 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.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

20 citing papers in PubMed, 49 citations in OpenAlex.

  1. Article
  2. Article
  3. Article
  4. [Expert consensus on the diagnosis and therapy of inherited hyperbilirubinemia (version 2025)].Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology · 2025
    Article
  5. Clinical development of therapeutic mRNA applications.Molecular therapy : the journal of the American Society of Gene Therapy · 2025
    Review
  6. Article
  7. Review
  8. The Multifaceted Role of Bilirubin in Liver Disease: A Literature Review.Journal of clinical and translational hepatology · 2024
    Review
  9. Therapeutic Options for Crigler-Najjar Syndrome: A Scoping Review.International journal of molecular sciences · 2024
    Article
  10. [Correlation between the mutation spectrum of the UGT1A1 gene and clinical phenotype in patients with inherited hyperunconjugated bilirubinemia].Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology · 2024
    Article
  11. Article
  12. Article
  13. Review
  14. Article
  15. Article
  16. Review
  17. Review
  18. Article
  19. Living Related Liver Transplantation for Metabolic Liver Diseases in Children.Journal of pediatric gastroenterology and nutrition · 2021
    Article
  20. 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

13 authors at 5 institutions in 1 country.

Kevin A StraussClinic for Special Children, Strasburg, PA.
Charles E AhlforsDepartment of Pediatrics, Stanford University School of Medicine, Stanford, CA.
Kyle SoltysDepartment of Surgery, Division of Pediatric Transplantation, Hillman Center for Pediatric Transplantation, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA.
George V MazareigosDepartment of Surgery, Division of Pediatric Transplantation, Hillman Center for Pediatric Transplantation, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA.
Millie YoungClinic for Special Children, Strasburg, PA.
Lauren E BowserClinic for Special Children, Strasburg, PA.
Michael D FoxClinic for Special Children, Strasburg, PA.
James E SquiresDivision of Gastroenterology and Hepatology, Department of Pediatrics, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA.
Patrick McKiernanDivision of Pediatric Gastroenterology, Hepatology and Nutrition, UPMC Children's Hospital of Pittsburgh and Pittsburgh Liver Research Center, Pittsburgh, PA.
Karlla W BrigattiClinic for Special Children, Strasburg, PA.
Erik G PuffenbergerClinic for Special Children, Strasburg, PA.
Vincent J CarsonClinic for Special Children, Strasburg, PA.
Hendrik J VremanDivision of Neonatal and Developmental Medicine, Department of Pediatrics, Stanford University School of Medicine, Stanford, CA.
Clinic for Special Children · USChildren's Hospital of Pittsburgh · USAlfred I. duPont Hospital for Children · USStanford Medicine · USStanford University · US

Funding

Pittsburgh Liver Research CenterP30DK120531 · NIDDK · UNIVERSITY OF PITTSBURGH AT PITTSBURGH · PI Gavin E Arteel · 2019 to 2026
$10.9M
NIDDK NIH HHS P30 DK120531
6 · The paper itself

Abstract

BACKGROUND AND

aimsWe describe the pathophysiology, treatment, and outcome of Crigler-Najjar type 1 syndrome (CN1) in 28 UGT1A1 c.222C>A homozygotes followed for 520 aggregate patient-years. APPROACH AND

resultsUnbound ("free") bilirubin (B

conclusionSeven decades after its discovery, CN1 remains a morbid and potentially fatal disorder.

Indexed as

BilirubinBrain DiseasesCrigler-Najjar SyndromeLiver CirrhosisAdolescentFemaleGlucuronosyltransferaseHomozygoteHumansInfant, NewbornKaplan-Meier EstimateLiver TransplantationMalePhototherapyRisk AssessmentSerum AlbuminBilirubinGlucuronosyltransferaseSerum AlbuminUGT1A1 Enzyme

Identifiers

PMID31553814
PMCPMC7909716
OpenAlexW2975259672

What OpenQuestion holds

Textmetadata
LicenceTDM
Read underepoch 390

Registered trials

None linked

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.