Evidence map›Paper›PMID 29073271›Full record

ArticlePloS one2017

Lower incidence of fracture after IV bisphosphonates in girls with Rett syndrome and severe bone fragility.

Anne-Sophie Lambert, Anya Rothenbuhler, Perrine Charles, Sylvie Brailly-Tabard, Séverine Trabado, Elisabeth Célestin, Emmanuel Durand, Isabelle Fontaine, Lotfi Miladi, Philippe Wicart and 2 more

Open access · goldAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in PloS one, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
9citing papers in PubMed, 1 pooled it
1.1field-weighted citation impact, top 18% 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

9 citing papers in PubMed, 1 synthesis or guideline pooled it, 14 citations in OpenAlex.

  1. Pooled it
  2. Review
  3. Article
  4. The Management of Bone Defects in Rett Syndrome.Calcified tissue international · 2025
    Review
  5. Review
  6. Article
  7. Article
  8. Altered Bone Status in Rett Syndrome.Life (Basel, Switzerland) · 2021
    Review
  9. 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

12 authors at 5 institutions in 1 country.

Anne-Sophie LambertAPHP, Department of pediatric endocrinology and diabetology for children, Bicêtre Paris-Sud, Le Kremlin Bicêtre, France.ORCID http://orcid.org/0000-0002-4232-3882
Anya RothenbuhlerAPHP, Department of pediatric endocrinology and diabetology for children, Bicêtre Paris-Sud, Le Kremlin Bicêtre, France.
Perrine CharlesAPHP, Department of Genetics, Reference Centre for Intellectual Disabilities, Pitié-Salpêtrière, Paris, France.
Sylvie Brailly-TabardAPHP, Department of Genetics and Hormonology, Bicêtre Paris-Sud, Le Kremlin Bicêtre, France.
Séverine TrabadoAPHP, Department of Genetics and Hormonology, Bicêtre Paris-Sud, Le Kremlin Bicêtre, France.
Elisabeth CélestinAPHP, Rett Center, Necker-Enfants Malades, Paris, France.
Emmanuel DurandAPHP, IR4M Unit, CNRS-Université Paris-Saclay, Orsay, France.
Isabelle FontaineIMP Marie-Auxiliatrice, Draveil, France.
Lotfi MiladiAPHP, Department of Pediatric Orthopedics, Necker-Enfants Malades, School of Medicine, University Paris Descartes Sorbonne Paris Cité, Paris, France.
Philippe WicartAPHP, Department of Pediatric Orthopedics, Necker-Enfants Malades, School of Medicine, University Paris Descartes Sorbonne Paris Cité, Paris, France.
Nadia Bahi-BuissonAPHP, Rett Center, Necker-Enfants Malades, Paris, France.
Agnès LinglartAPHP, Department of pediatric endocrinology and diabetology for children, Bicêtre Paris-Sud, Le Kremlin Bicêtre, France.
Assistance Publique – Hôpitaux de Paris · FRDélégation Paris 5 · FRCentre National de la Recherche Scientifique · FRInstitut Necker Enfants Malades · FRSorbonne Université · FR

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundClassic Rett Syndrome (RS) is a disabling condition mainly caused by MECP2 mutations. Girls with RS are at risk of developing bone fragility and fractures at a young age which results in pain and may seriously impair quality of life.

objectiveTo retrospectively assess the safety and efficacy of IV bisphosphonates on fracture, bone mineral density (BMD) and bone markers in RS girls with bone fragility.

methodsRS girls received either IV pamidronate (n = 19) or IV zoledronate (n = 1) for 2 years.

resultsOf 20 patients studied (age: 12.5 years [6; 39]), 14 were non-ambulatory. The incidence of fracture decreased from 37 fractures in 20 patients, to 1 fracture during or after treatment (follow-up: 3.1 years [1.5; 5]). The spine BMD Z-score improved from -3.2 [-5.6; -0.1] to -2.2 [-3.8; 0.0], p = 0.0006. Most parents reported decreases in chronic pain and 2 patients started to walk. Urinary calcium excretion decreased from 0.7 [0.18; 1.5] to 0.2 [0.03; 0.67] mM/mM of creatinine (p = 0.0001). Pamidronate was well tolerated.

conclusionRS girls should be screened for impaired bone mineralization and preventive measures should be taken. In girls experiencing fractures, IV bisphosphonates constitute a beneficial adjuvant treatment to diminish the risk of fracture and restore bone density.

Indexed as

AdolescentAdultBone DensityBone Density Conservation AgentsCalciumChildCreatinineDiphosphonatesFemaleFractures, BoneHumansImidazolesIncidencePamidronateRett SyndromeYoung AdultBone Density Conservation AgentsCalciumCreatinineDiphosphonatesImidazolesPamidronateZoledronic Acid

Identifiers

PMID29073271
PMCPMC5658100
OpenAlexW2766457702

What OpenQuestion holds

Textmetadata
LicenceCC BY
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.