Evidence map›Paper›PMID 42540447›Full record

ArticleJPGN reports2026

The Brazilian Alagille syndrome study: New insights from a multicenter national cohort.

Elisa Carvalho, Gilda Porta, Irene Kazue Miura, Cristina Helena Targa Ferreira, Luciana Rodrigues Silva, Natascha Silva Sandy, Eleonora Druve Tavares Fagundes, Eliene Oliveira, Aline Falleiros Freitas, Adriana Maria Alves de Tommaso and 25 more

Abstract read
In one paragraph

Article in JPGN reports, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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

0 citing papers in PubMed.

No citing paper in PubMed yet.

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

35 authors.

Elisa CarvalhoHospital da Criança de Brasília Brasília Brazil.ORCID https://orcid.org/0000-0002-5079-5011
Gilda PortaHospital Sírio Libanês Hospital Menino Jesus Brigadeiro Brazil.
Irene Kazue MiuraHospital Sírio Libanês Hospital Menino Jesus Brigadeiro Brazil.
Cristina Helena Targa FerreiraHospital da Criança Santo Antonio - UFCSPA Porto Alegre Brazil.
Luciana Rodrigues SilvaUniversidade Federal da Bahia Salvador Brazil.
Natascha Silva SandyHospital Israelita Albert Einstein Jardim Leonor Brazil.
Eleonora Druve Tavares FagundesUniversidade Federal de Minas Gerais (UFMG) Belo Horizonte Brazil.
Eliene OliveiraHospital Sírio Libanês Hospital Menino Jesus Brigadeiro Brazil.
Aline Falleiros FreitasHospital Sírio Libanês Hospital Menino Jesus Brigadeiro Brazil.
Adriana Maria Alves de TommasoUniversidade Estadual de Campinas (Unicamp) Campinas Brazil.
Regina SawamuraFaculdade de Medicina de Ribeirão Preto da Universidade de São Paulo (USP) Ribeirão Preto Brazil.
Melina Utz MelereHospital da Criança Santo Antonio - UFCSPA Porto Alegre Brazil.
Sandra Maria Goncalves VieiraHospital das Clínicas de Porto Alegre Porto Alegre Brazil.
Renata Belém Pessoa de Melo SeixasHospital da Criança de Brasília Brasília Brazil.
Isadora de Carvalho TrevizoliHospital da Criança de Brasília Brasília Brazil.
Alexandre Rodrigues FerreiraUniversidade Federal de Minas Gerais (UFMG) Belo Horizonte Brazil.
Adriana Porta Miche HirschfeldHospital Sírio Libanês Hospital Menino Jesus Brigadeiro Brazil.
Renata Pereira Sustovich PuglieseHospital Sírio Libanês Hospital Menino Jesus Brigadeiro Brazil.
Cibele Dantas Ferreira MarquesUniversidade Federal da Bahia Salvador Brazil.
Maria Angela Bellomo BrandãoUniversidade Estadual de Campinas (Unicamp) Campinas Brazil.
Raquel Borges PintoHospital da Criança Conceição Porto Alegre Brazil.
Leticia Helena Caldas LopesUniversidade Federal de São Paulo (UNIFESP) São Paulo Brazil.
Jussara Melo de Cerqueira MaiaHospital Universitário Onofre Lopes (UFRN) Natal Brazil.
Ana Cristina Vieira de MeloHospital Universitário Onofre Lopes (UFRN) Natal Brazil.
Carolina Sores SilvaHospital da Criança Santo Antonio - UFCSPA Porto Alegre Brazil.
Luiza Salgado NaderHospital da Criança Santo Antonio - UFCSPA Porto Alegre Brazil.
Maria Tereza GuiottiUniversidade de São Paulo (USP) Ribeirão Preto Brazil.
Roberta Vacari AlcântaraUniversidade Estadual de Campinas (Unicamp) Campinas Brazil.
Leonardo ResendeHospital Universitário Maria Aparecida Pedrossian - UFMS Campo Grande Brazil.
Bruno Pereira Ribeiro RochaHospital Estadual da Criança do Rio de Janeiro Rio de Janeiro Brazil.
Nilza Maria Medeiros PerinHospital Infantil Joana de Gusmão-Florianópolis-SC Florianópolis Brazil.
Adriana Teixeira RodriguesUniversidade Federal de Minas Gerais (UFMG) Belo Horizonte Brazil.
Vera Lúcia Baggio DanesiHospital Sírio Libanês Hospital Menino Jesus Brigadeiro Brazil.
Gabriel HesselUniversidade Estadual de Campinas (Unicamp) Campinas Brazil.
Themis Reverbel SilveiraHospital da Criança Santo Antonio - UFCSPA Porto Alegre Brazil.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Objectives: To elucidate the natural history of liver disease and identify the predictors of native liver survival (NLS) in a Brazilian cohort of children with Alagille syndrome (ALGS). Methods: Multicenter retrospective cohort study of children with ALGS. Descriptive statistics summarized clinical data. Overall survival was estimated using Kaplan-Meier curves. NLS was analyzed with competing risk models. Associations between laboratory parameters and NLS were tested using Pearson's chi-square. Results: From 14 Brazilian reference centers, 120 children (52.5% male) were included, with mean age at diagnosis of 17.8 months and a median follow-up time of 88.5 months. Most presented characteristic facies (89.9%), jaundice (88.2%), pruritus (87.9%), and neonatal cholestasis (79.2%). Thirty percent underwent liver transplantation (LT) and 6.7% died. Jaundice, xanthomas, hepatomegaly, splenomegaly, and presence of excoriations were significantly associated with the need for LT or death. Direct bilirubin (DB) > 6 mg/dL, cholesterol >300 mg/dL, increased aspartate aminotransferase (AST) > 5 times the upper limit of normal (ULN), and AST-to-platelet ratio index (APRI) > 1 were associated with a significantly increased risk of LT or death, with hazard ratios of 18.2 ( Conclusions: The main clinical manifestations included characteristic facies, jaundice, and pruritus. Higher rates of LT or death were associated with jaundice, xanthomas, hepatomegaly, splenomegaly and presence of excoriations. Higher rates of NLS were associated with DB levels below 6 mg/dL, cholesterol levels below 300 mg/dL, AST levels <5x ULN, and an APRI < 1.

Indexed as

cholestasisnative liver survivalpredictors of survivalpruritus

Identifiers

PMID42540447
PMCPMC13425789

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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.