Evidence map›Paper›PMID 36768808›Full record

ArticleInternational journal of molecular sciences2023

Fibrosis-Related Gene Profiling in Liver Biopsies of PiZZ α1-Antitrypsin Children with Different Clinical Courses.

Jan C Kamp, Naomi N Kappe, Carlos Fernández Moro, Jan Fuge, Mark P Kuehnel, Sabine Wrenger, Tobias Welte, Bart van Hoek, Danny D Jonigk, Padmini P S J Khedoe and 5 more

Abstract read
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In one paragraph

Article in International journal of molecular sciences, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing 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

1 citing paper in PubMed.

  1. Article
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

15 authors.

Jan C KampDepartment of Respiratory Medicine, Hannover Medical School, 30625 Hannover, Germany.ORCID 0000-0002-5002-409X
Naomi N KappeDepartment of Pulmonology, Leiden University Medical Center, Member of European Reference Network Lung, Section Alpha-1-Antitrypsin Deficiency, 2333 ZA Leiden, The Netherlands.
Carlos Fernández MoroDepartment of Laboratory Medicine, Division of Pathology, Karolinska Institutet, 171 77 Stockholm, Sweden.
Jan FugeDepartment of Respiratory Medicine, Hannover Medical School, 30625 Hannover, Germany.ORCID 0000-0003-2926-3608
Mark P KuehnelBiomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center of Lung Research (DZL), 30625 Hannover, Germany.
Sabine WrengerDepartment of Respiratory Medicine, Hannover Medical School, 30625 Hannover, Germany.ORCID 0000-0002-9733-6162
Tobias WelteDepartment of Respiratory Medicine, Hannover Medical School, 30625 Hannover, Germany.
Bart van HoekDepartment of Gastroenterology and Hepatology, Leiden University Medical Center, 2333 ZA Leiden, The Netherlands.ORCID 0000-0001-6527-764X
Danny D JonigkBiomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center of Lung Research (DZL), 30625 Hannover, Germany.ORCID 0000-0002-5251-2281
Padmini P S J KhedoeDepartment of Pulmonology, Leiden University Medical Center, Member of European Reference Network Lung, Section Alpha-1-Antitrypsin Deficiency, 2333 ZA Leiden, The Netherlands.ORCID 0000-0002-0798-014X
Pavel StrnadMedical Clinic III, Gastroenterology, Metabolic Diseases and Intensive Care, University Hospital RWTH Aachen, Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN RARE LIVER), 52074 Aachen, Germany.ORCID 0000-0002-7122-6379
Mikael BjörnstedtDepartment of Laboratory Medicine, Division of Pathology, Karolinska Institutet, 171 77 Stockholm, Sweden.ORCID 0000-0003-2831-3837
Jan StolkDepartment of Pulmonology, Leiden University Medical Center, Member of European Reference Network Lung, Section Alpha-1-Antitrypsin Deficiency, 2333 ZA Leiden, The Netherlands.
Sabina JanciauskieneDepartment of Respiratory Medicine, Hannover Medical School, 30625 Hannover, Germany.
Antal NemethDepartment of Laboratory Medicine, Division of Pathology, Karolinska Institutet, 171 77 Stockholm, Sweden.

Funding

Deutsche Forschungsgemeinschaft STR1095/6-1NCATS NIH HHS L40 TR001095Stichting Marcel Brus Fonds RSIN 860974376
6 · The paper itself

Abstract

PiZZ (Glu342Lys) α1-antitrypsin deficiency (AATD) is characterized by intrahepatic AAT polymerization and is a risk factor for liver disease development in children. The majority of PiZZ children are disease free, hence this mutation alone is not sufficient to cause the disease. We investigated Z-AAT polymers and the expression of fibrosis-related genes in liver tissues of PiZZ children with different clinical courses. Liver biopsies obtained during 1979-2010 at the Department of Paediatrics, Karolinska University Hospital, Sweden, were subjected to histological re-evaluation, immunohistochemistry and NanoString-based transcriptome profiling using a panel of 760 fibrosis plus 8 bile acid-related genes. Subjects were divided into three groups based on clinical outcomes: NCH (neonatal cholestasis, favourable outcome,

Indexed as

alpha 1-Antitrypsin DeficiencyCholestasisBiopsyChildDisease ProgressionHumansInfant, NewbornLipidsLiverLiver CirrhosisLipidscholestasiscirrhosislipid metabolismlivermRNA expressionnCounter Fibrosis PanelPiZZ deficiencytranscriptome profilingZ-AAT polymers

Identifiers

PMID36768808
PMCPMC9916468

What Socratic holds

Textmetadata
LicenceCC BY
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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 Socratic graph.