Evidence map›Paper›PMID 38896081›Full record

ArticleHepatology communications2024

Liver disease and transplantation in telomere biology disorders: An international multicenter cohort.

YunZu Michele Wang, Batul Kaj-Carbaidwala, Adam Lane, Suneet Agarwal, Fabian Beier, Alison Bertuch, Kristin A Borovsky, Steven K Brennan, Rodrigo T Calado, Luiz Fernando B Catto and 24 more

Abstract readMulticenter Study
PubMed Full text at PMC Publisher
In one paragraph

Article in Hepatology communications, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 8 papers.

0numbers the graph read from it
0cells of the map it votes in
8citing papers in PubMed
–field-weighted citation impact
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

8 citing papers in PubMed.

  1. Article
  2. Genetic testing in liver diseases: Clinical applications.JHEP reports : innovation in hepatology · 2026
    Review
  3. Article
  4. Review
  5. Article
  6. Observational
  7. Review
  8. 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

34 authors.

YunZu Michele WangDivision of Bone Marrow Transplantation and Immune Deficiency, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
Batul Kaj-CarbaidwalaDepartment of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Lurie Children's Hospital, Chicago, Illinois, USA.
Adam LaneDivision of Bone Marrow Transplantation and Immune Deficiency, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
Suneet AgarwalDepartment of Pediatrics, Division of Hematology/Oncology, Boston Children's Hospital, Boston, Massachusetts, USA.
Fabian BeierDepartment of Hematology and Oncology, University Klinik Aachen, Aachen, Germany.
Alison BertuchDepartment of Pediatric Hematology-Oncology, Texas Children's Hospital, Houston, Texas, USA.
Kristin A BorovskyDepartment of Gastroenterology, Hepatology, and Nutrition, Texas Children's Hospital, Houston, Texas, USA.
Steven K BrennanDepartment of Pediatrics, Division of Allergy and Pulmonary Medicine, Washington University in St. Louis, St. Louis, Missouri, USA.
Rodrigo T CaladoDepartment of Hematology and Oncology, University of Sao Paulo, Sao Paulo, Brazil.
Luiz Fernando B CattoDepartment of Hematology and Oncology, University of Sao Paulo, Sao Paulo, Brazil.
Carlo DufourHematology Unit. IRCCS Istituto Giannina Gaslini, Genoa, Italy.
Christen L EbensDivision of Pediatric Blood and Marrow Transplant & Cellular Therapy, University of Minnesota, Minneapolis, MN, USA.
Francesca FioreddaDepartment of Hematology, Giannina Gaslini Institute, Genoa, Italy.
Neelam GiriDepartment of Pediatrics, Clinical Genetics Branch, National Cancer Institute, Bethesda, Maryland, USA.
Nicholas GloudeDivision of Pediatric Hematology/Oncology, Department of Pediatrics, Rady Children's Hospital San Diego, San Diego, California, USA.
Frederick GoldmanDivision of Pediatric Hematology and Oncology, Department of Pediatrics, University of Alabama Birmingham, Birmingham, Alabama, USA.
Paula M HertelDepartment of Gastroenterology, Hepatology, and Nutrition, Texas Children's Hospital, Houston, Texas, USA.
Ryan HimesDivision of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Ochsner Health, New Orleans, Louisiana, USA.
Sioban B KeelDepartment of Hematology, University of Washington, Seattle, Washington, USA.
Divya T KouraDivision of Hematology-Oncology and Bone Marrow Transplantation, Department of Medicine, University of California, San Diego, San Diego, California, USA.
Christian P KratzDepartment of Pediatric Hematology and Oncology, Hannover Medical School, Hannover, Germany.
Sakil KulkarniDepartment of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Washington University in St. Louis, St. Louis, Missouri, USA.
Iris LiouDivision of Gastroenterology, Department of Medicine, University of Washington, Seattle, Washington, USA.
Taizo A NakanoCenter for Cancer and Blood Disorders, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, Colorado, USA.
Silvia NastasioDepartment of Gastroenterology/Hepatology, Boston Children's Hospital, Boston, Massachusetts, USA.
Marena R NiewischDepartment of Hematology, Giannina Gaslini Institute, Genoa, Italy.
Daniel D PenriceDepartment of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA.
Ghadir S SasaSarah Cannon Transplant and Cellular Therapy Network, San Antonio, Texas, USA.
Sharon A SavageDepartment of Hematology, Giannina Gaslini Institute, Genoa, Italy.
Douglas A SimonettoDepartment of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA.
David S ZieglerSchool of Clinical Medicine, UNSW Medicine & Health, UNSW Sydney, Kensington, NSW, Australia.
Alexander G MiethkeDepartment of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA.
Kasiani C MyersDivision of Bone Marrow Transplantation and Immune Deficiency, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
Clinical Care Consortium for Telomere-associated Ailments (CCCTAA)

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundPatients with telomere biology disorders (TBD) develop hepatic disease, including hepatitis, cirrhosis, and hepatopulmonary syndrome. No specific treatment exists for TBD-related liver disease, and the role of liver transplantation (LT) remains controversial. Our study objectives were to describe the clinical characteristics, management, and outcomes in patients with TBD-related liver disease, and their LT outcomes.

methodsData from 83 patients with TBD-associated liver disease were obtained from 17 participating centers in the Clinical Care Consortium of Telomere-Associated Ailments and by self-report for our retrospective, multicenter, international cohort study.

resultsGroup A ("Advanced") included 40 patients with advanced liver disease. Of these, 20 underwent LT (Group AT). Group M ("Mild") included 43 patients not warranting LT evaluation, none of whom were felt to be medically unfit for liver transplantation. Supplemental oxygen requirement, pulmonary arteriovenous malformation, hepatopulmonary syndrome, and higher bilirubin and international normalized ratio values were associated with Group A. Other demographics, clinical manifestations, and laboratory findings were similar between groups. Six group A patients were declined for LT; 3 died on the waitlist. Median follow-up post-LT was 2.9 years (range 0.6-13.2 y). One-year survival post-LT was 73%. Median survival post-LT has not been reached. Group AT patients had improved survival by age compared to all nontransplant patients (log-rank test p = 0.02). Of 14 patients with pretransplant hypoxemia, 8 (57%) had improved oxygenation after transplant.

conclusionsLT recipients with TBD do not exhibit excessive posttransplant mortality, and LT improved respiratory status in 57%. A TBD diagnosis should not exclude LT consideration.

Indexed as

Liver TransplantationAdolescentAdultChildChild, PreschoolFemaleHumansLiver DiseasesMaleMiddle AgedRetrospective StudiesTelomereTreatment OutcomeYoung Adult

Identifiers

PMID38896081
PMCPMC11186813

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Registered trials

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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.