Evidence map›Paper›PMID 34398413›Full record

ArticleIndian journal of pediatrics2022

Liver Transplantation: A Safe and Definitive Alternative to Lifelong Nitisinone for Tyrosinemia Type 1.

Jagadeesh Menon, Naresh Shanmugam, Joseph J Valamparampil, Abdul Hakeem, Mukul Vij, Anil Jalan, Mettu Srinivas Reddy, Mohamed Rela

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Article in Indian journal of pediatrics, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.

0numbers the graph read from it
0cells of the map it votes in
5citing papers in PubMed
1.3field-weighted citation impact, top 23% 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

5 citing papers in PubMed, 12 citations in OpenAlex.

  1. Tyrosinemia presenting as rickets.Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology · 2026
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4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

8 authors at 1 institution in 2 countries.

Jagadeesh MenonDepartment of Pediatric Gastroenterology & Hepatology, Dr Rela Institute & Medical Centre, Bharat Institute of Higher Education and Research, Chennai, Tamil Nadu, 600044, India.
Naresh ShanmugamDepartment of Pediatric Gastroenterology & Hepatology, Dr Rela Institute & Medical Centre, Bharat Institute of Higher Education and Research, Chennai, Tamil Nadu, 600044, India. drnareshps@gmail.com.
Joseph J ValamparampilDepartment of Pediatric Gastroenterology & Hepatology, Dr Rela Institute & Medical Centre, Bharat Institute of Higher Education and Research, Chennai, Tamil Nadu, 600044, India.
Abdul HakeemDepartment of Hepatobiliary surgery & liver transplantation, Dr Rela Institute & Medical Centre, Bharat Institute of Higher Education and Research, Chennai, Tamil Nadu, India.
Mukul VijDepartment of Histopathology, Dr Rela Institute & Medical Centre, Bharat Institute of Higher Education and Research, Chennai, Tamil Nadu, India.
Anil JalanDepartment of Pediatric Genetics, NIRMAN, Mumbai, Maharashtra, India.
Mettu Srinivas ReddyDepartment of Hepatobiliary surgery & liver transplantation, Dr Rela Institute & Medical Centre, Bharat Institute of Higher Education and Research, Chennai, Tamil Nadu, India.
Mohamed RelaDepartment of Hepatobiliary surgery & liver transplantation, Dr Rela Institute & Medical Centre, Bharat Institute of Higher Education and Research, Chennai, Tamil Nadu, India.
Bharath University · IN

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectivesTo report the experience of liver transplantation (LT) for tyrosinemia type 1 (TT-1).

methodsClinical data of children with TT-1 who underwent living donor LT between July 2009 and May 2020 were retrospectively analyzed. Data included pre-LT nitisinone therapy, graft type, post-LT complications, HCC incidence, and graft/patient survival.

resultsNine children were diagnosed with TT-1 at a median age of 12 mo (6-54 mo). Nitisinone was started in 6 patients at a median age of 15 mo (6-42 mo), but all had frequent interruption of therapy due to logistics with drug procurement including its cost. Median age at transplantation was 5 y (2-11 y). Explant liver showed HCC in 5 patients (55% of total cohort). The graft and patient survival are 100% with median follow-up of 58 mo (24-84 mo).

conclusionLT is curative for TT-1 and excellent results can be obtained in experienced centers. This is especially favorable in countries with limited resources where the cost of medical therapy is highly prohibitive, with lifelong diet restrictions and unclear long-term risk of HCC.

Indexed as

Carcinoma, HepatocellularLiver NeoplasmsLiver TransplantationTyrosinemiasChildCyclohexanonesHumansNitrobenzoatesRetrospective StudiesTreatment OutcomeCyclohexanonesnitisinoneNitrobenzoatesHepatocellular carcinomaImmunosuppressionLiver transplantationNitisinonePatient survivalTyrosinemia type 1

Identifiers

PMID34398413
OpenAlexW3193410933

What OpenQuestion holds

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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.