Evidence map›Paper›PMID 27995581›Full record

ArticleJIMD reports2017

Bone Health in Classic Galactosemia: Systematic Review and Meta-Analysis.

Britt van Erven, Lindsey Welling, Sandra C van Calcar, Artemis Doulgeraki, François Eyskens, Joanna Gribben, Eileen P Treacy, Rein Vos, Susan E Waisbren, M Estela Rubio-Gozalbo and 1 more

Open access · greenAbstract read
In one paragraph

Article in JIMD reports, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.

0numbers the graph read from it
0cells of the map it votes in
12citing papers in PubMed
22.0field-weighted citation impact, top 1% 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

12 citing papers in PubMed, 30 citations in OpenAlex.

  1. Review
  2. Article
  3. Natural history of three late-diagnosed classic Galactosemia patients.Molecular genetics and metabolism reports · 2024
    Article
  4. Article
  5. Article
  6. Article
  7. Observational
  8. Article
  9. Article
  10. Bone health in patients with inborn errors of metabolism.Reviews in endocrine & metabolic disorders · 2018
    Review
  11. Article
  12. Sweet and sour: an update on classic galactosemia.Journal of inherited metabolic disease · 2017
    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

11 authors at 9 institutions in 7 countries.

Britt van ErvenDepartment of Pediatrics and Department of Clinical Genetics, Maastricht University Medical Center, PO Box 5800, 6202, Maastricht, The Netherlands.
Lindsey WellingDepartment of Pediatrics, Academic Medical Center, Emma Children's Hospital, University of Amsterdam, Amsterdam, The Netherlands.
Sandra C van CalcarDepartment of Molecular and Medical Genetics, School of Medicine, Oregon Health and Science University, Portland, Oregon, USA.
Artemis DoulgerakiDepartment of Bone and Mineral Metabolism, Institute of Child Health, Agia Sophia Children's Hospital, Athens, Greece.
François EyskensDepartment of Metabolic Disorders in Children, Antwerp University Hospital UZA, Edegem, Belgium.
Joanna GribbenNutrition & Dietetics Department, Guy's & St. Thomas' NHS Foundation Trust, London, UK.
Eileen P TreacyMater Misericordiae University Hospital, Trinity College Dublin, Dublin, Ireland.
Rein VosDepartment of Methodology and Statistics, Faculty of Health, Medicine and Life Sciences, Maastricht University, Maastricht, The Netherlands.
Susan E WaisbrenDivision of Genetics and Genomics, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.
M Estela Rubio-GozalboDepartment of Pediatrics and Department of Clinical Genetics, Maastricht University Medical Center, PO Box 5800, 6202, Maastricht, The Netherlands. estela.rubio@mumc.nl.
Annet M BoschDepartment of Pediatrics, Academic Medical Center, Emma Children's Hospital, University of Amsterdam, Amsterdam, The Netherlands.
Maastricht University · NLAcademic Medical Center · NLAntwerp University Hospital · BEChildren's Hospital Agia Sophia · GRGuy's and St Thomas' NHS Foundation Trust · GBHarvard University · USOregon Health & Science University · USUniversity College Dublin · IEUniversity of Amsterdam · NL

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

introductionPrevious studies have reported an association between classic galactosemia (CG) and decreased bone mass. The primary objective of this systematic review with meta-analysis was to determine the extent of bone mineral density (BMD) Z-score reduction. Low BMD was defined as a Z-score ≤-2 standard deviations (SD). The secondary objective was to evaluate other indicators of bone status through a descriptive analysis.

methodsSystematic search strategies were developed by an experienced clinical librarian. Selection of relevant manuscripts, risk of bias assessment, and data extraction were performed independently by two investigators.

resultsFour studies were included in the meta-analysis. BMD Z-scores in children and adults with CG measured at the lumbar spine (LBMD; 4 studies; n  = 112), total hip (HBMD; 2 studies; n = 58), and femoral neck (FBMD; 2 studies; n = 73) were assessed. Mean BMD Z-scores in the CG population were LBMD -0.70 (95% CI: -0.88, -0.52); HBMD -0.89 (95% CI: -1.14, -0.64); and FBMD -0.63 (95% CI -1.29, 0.02). Results from studies included in the descriptive analysis (n = 7) show that vitamin D levels were frequently in the low reference range, whereas serum calcium levels were within reference range.

conclusionThe mean BMD Z-score in the CG population is -0.7, which is lower than in the general population, though still within two SD of the reference mean of zero. This indicates that bone health is mildly affected in CG and that more patients, compared to the general population, are at risk for a BMD Z-score ≤-2 SD. In conclusion, clinicians should ensure appropriate preventive and therapeutic measures for CG patients.

Indexed as

Bone massBone mineral densityBone turnover markersClassic galactosemiaDXAGALT deficiencyVitamin D

Identifiers

PMID27995581
PMCPMC5585100
OpenAlexW2562021664

What OpenQuestion holds

Textmetadata
Read underepoch 390

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 OpenQuestion graph.