Evidence map›Paper›PMID 39523291›Full record

SynthesisPediatric nephrology (Berlin, Germany)2025

Inborn errors of metabolism in neonates and pediatrics on varying dialysis modalities: a systematic review and meta-analysis.

Manan Raina, Kush Doshi, Archana Myneni, Abhishek Tibrewal, Matthew Gillen, Jieji Hu, Timothy E Bunchman

Abstract readMeta-AnalysisSystematic Review
In one paragraph

Synthesis in Pediatric nephrology (Berlin, Germany), 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.

0numbers the graph read from it
0cells of the map it votes in
3citing 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

3 citing papers in PubMed.

  1. Article
  2. Article
  3. 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

7 authors.

Manan RainaHawken High School, Cleveland, OH, USA.
Kush DoshiAkron Nephrology Associates/Cleveland Clinic Akron General Medical Center, Akron, OH, USA.ORCID http://orcid.org/0000-0002-6243-4918
Archana MyneniAkron Nephrology Associates/Cleveland Clinic Akron General Medical Center, Akron, OH, USA.
Abhishek TibrewalAkron Nephrology Associates/Cleveland Clinic Akron General Medical Center, Akron, OH, USA.
Matthew GillenDivision of Neonatal-Perinatal Medicine, Department of Pediatrics, Emory University School of Medicine, Atlanta, GA, USA.
Jieji HuCollege of Medicine, Northeast Ohio Medical University, Rootstown, OH, USA.
Timothy E BunchmanDepartment of Pediatric Nephrology & Transplantation, Children's Hospital of Richmond at the Virginia Commonwealth University, 1000 E Broad St, PO Box 980498, Richmond, VA, 23298, USA. Timothy.bunchman@vcuhealth.org.

Funding

Atlanta Network for Training In KUH Scientific Research (ATLANTIS)TL1DK136047 · NIDDK · EMORY UNIVERSITY · PI Vivien Andrea Sheehan · 2022 to 2026
$3.0M
NIDDK NIH HHS TL1 DK136047
6 · The paper itself

Abstract

backgroundSome inborn errors of metabolism (IEMs) resulting in aberrations to blood leucine and ammonia levels are commonly treated with kidney replacement therapy (KRT). Children with IEMs require prompt treatment, as delayed treatment results in increased neurological and developmental morbidity.

objectivesOur systematic review in neonates and pediatrics evaluates survival rates and reductions in ammonia and leucine levels across different KRT modalities (continuous KRT (CKRT), hemodialysis (HD), peritoneal dialysis (PD)). DATA SOURCES: A literature search was conducted through PubMed, Web of Science, and Embase databases for articles including survival rate and toxic metabolite clearance data in pediatric patients with IEM undergoing KRT. STUDY ELIGIBILITY CRITERIA: Cross-sectional, prospective, and retrospective studies with survival rates reported in patients with IEM with an intervention of CKRT, PD, or HD were included. Studies with patients receiving unclear or multiple KRT modalities were excluded. STUDY APPRAISAL AND SYNTHESIS

methodsAnalysis variables included efficacy outcomes [% reduction in ammonia (RIA) from pre- to post-dialysis and time to 50% RIA] and mortality. The Newcastle Ottawa Risk of Bias quality assessment was used to assess bias. All statistical analyses were performed with MedCalc Statistical Software version 19.2.6.

resultsA total of 37 studies (n = 642) were included. The pooled proportion (95% CI) of mortality on CKRT was 24.84% (20.93-29.08), PD was 34.42% (26.24-43.33), and HD 34.14% (24.19-45.23). A lower trend of pooled (95% CI) time to 50% RIA was observed with CKRT [6.5 (5.1-7.8)] vs. PD [14.4 (13.3-15.5)]. A higher mortality was observed with greater plasma ammonia level before CKRT (31.94% for ≥ 1000 µmol/L vs. 15.04% for < 1000 µmol/L). CONCLUSIONS AND IMPLICATIONS OF KEY

findingsDespite the limitations in sample size, trends emerged suggesting that CKRT may be associated with lower mortality rates compared to HD or PD, with potential benefits including prevention of rebound hyperammonemia and improved hemodynamic control. While HD showed a trend towards faster achievement of 50% RIA, all modalities demonstrated comparable efficacy in reducing ammonia and leucine levels. PROSPERO REGISTRATION: CRD42023418842.

Indexed as

Metabolism, Inborn ErrorsRenal DialysisRenal Replacement TherapyAmmoniaChildChild, PreschoolHumansInfantInfant, NewbornPeritoneal DialysisSurvival RateTreatment OutcomeAmmoniaCKRTIEMNeonatesPediatrics

Identifiers

PMID39523291
PMCPMC12116838

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.