Evidence map›Paper›PMID 28842728›Full record

ArticleOsteoporosis international : a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA2017

Discrepancy between bone density and bone material strength index in three siblings with Camurati-Engelmann disease.

S Herrera, R Soriano, X Nogués, R Güerri-Fernandez, D Grinberg, N García-Giralt, N Martínez-Gil, S Castejón, A González-Lizarán, S Balcells and 1 more

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In one paragraph

Article in Osteoporosis international : a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed, 1 pooled it
0.9field-weighted citation impact, top 21% 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

2 citing papers in PubMed, 1 synthesis or guideline pooled it, 6 citations in OpenAlex.

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4 · The record

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5 · Who and what money

Authors and funding

11 authors at 3 institutions in 1 country.

S HerreraHospital del Mar Institute of Medical Research, Autonomous University of Barcelona and CIBERFES, Instituto Carlos III, Barcelona, Spain.ORCID 0000-0002-5057-354X
R SorianoHospital del Mar Institute of Medical Research, Autonomous University of Barcelona and CIBERFES, Instituto Carlos III, Barcelona, Spain.
X NoguésHospital del Mar Institute of Medical Research, Autonomous University of Barcelona and CIBERFES, Instituto Carlos III, Barcelona, Spain.
R Güerri-FernandezHospital del Mar Institute of Medical Research, Autonomous University of Barcelona and CIBERFES, Instituto Carlos III, Barcelona, Spain.
D GrinbergDepartment of Genetics, Microbiology and Statistics, University of Barcelona, IBUB, IDSJD, and CIBERER, Instituto Carlos III, Barcelona, Spain.
N García-GiraltHospital del Mar Institute of Medical Research, Autonomous University of Barcelona and CIBERFES, Instituto Carlos III, Barcelona, Spain.
N Martínez-GilDepartment of Genetics, Microbiology and Statistics, University of Barcelona, IBUB, IDSJD, and CIBERER, Instituto Carlos III, Barcelona, Spain.
S CastejónHospital del Mar Institute of Medical Research, Autonomous University of Barcelona and CIBERFES, Instituto Carlos III, Barcelona, Spain.
A González-LizaránHospital del Mar Institute of Medical Research, Autonomous University of Barcelona and CIBERFES, Instituto Carlos III, Barcelona, Spain.
S BalcellsDepartment of Genetics, Microbiology and Statistics, University of Barcelona, IBUB, IDSJD, and CIBERER, Instituto Carlos III, Barcelona, Spain.
A Diez-PerezHospital del Mar Institute of Medical Research, Autonomous University of Barcelona and CIBERFES, Instituto Carlos III, Barcelona, Spain. ADiez@parcdesalutmar.cat.
Universitat Autònoma de Barcelona · ESInstituto de Salud Carlos III · ESHospital Del Mar · ES

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Camurati-Engelmann (CE) is a very rare disease affecting one in every million persons worldwide. It is characterized by an enlargement of long bones. We aimed to assess bone characteristics in three siblings with different tools. Even if there was an excess of bone density, quality seemed to be deteriorated.

introductionCE disease is a rare monogenic disorder affecting approximately one in every million persons worldwide. It is mainly characterized by a progressive hyperostosis of the periosteum and endosteum of the diaphysis of long bones. Limited data are available about bone characteristics in these patients. In three siblings with CE disease, we aimed to assess bone mineral density (BMD) and trabecular bone score (TBS) by dual-energy X-ray absorptiometry (DXA) and material characteristics at tissue level using bone impact reference point indentation.

methodsClinical data were collected and a general laboratory workup was performed. At the lumbar spine and hip, BMD and TBS were measured using DXA imaging. Bone material strength index (BMSi) was measured by bone impact microindentation using an Osteoprobe instrument.

resultsAll three cases had densitometric values consistent with high bone mass (sum of Z-score at the lumbar spine and hip > 4). Hip BMD was extremely high in all three siblings at both total hip and femoral neck, while at the lumbar spine, two of them had normal values but the third again had very high BMD. TBS values were in the normal range. In contrast, BMSi measurements were at low or very low levels, compared with normal controls.

conclusionDespite strikingly increased BMD and normal microarchitecture, BMSi is affected in patients with CE. Microindentation could be an appropriate tool for assessing bone fragility in these patients. Bone disease in this group of patients requires further study to better understand the underlying regulatory mechanisms and their alterations.

Indexed as

Absorptiometry, PhotonAdultBone DensityCamurati-Engelmann SyndromeFemaleFemur NeckHip JointHumansLumbar VertebraeMaleMiddle AgedCamurati-EngelmannMicroindentationProgressive diaphyseal dysplasiaRare bone disease

Identifiers

PMID28842728
OpenAlexW2747940811

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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.