Evidence mapPaperPMID 36944707Full record

ArticleCalcified tissue international2023

Bone Tissue Evaluation Indicates Abnormal Mineralization in Patients with Autoimmune Polyendocrine Syndrome Type I: Report on Three Cases.

Saila Laakso, Tong Xiaoyu, Stéphane Blouin, Petra Keplinger, Ville-Valtteri Välimäki, Heikki Kröger, Outi Mäkitie, Markus A Hartmann

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Article in Calcified tissue international, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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0citing papers in PubMed
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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

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3 · Its place in the literature

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4 · The record

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5 · Who and what money

Authors and funding

8 authors at 4 institutions in 3 countries.

Saila LaaksoChildren's Hospital and Pediatric Research Center, University of Helsinki and Helsinki University Hospital, Stenbäckinkatu 9, Helsinki, Finland. saila.laakso@helsinki.fi.ORCID 0000-0002-5588-7855
Tong XiaoyuDepartment of Orthopedics, Kuopio Musculoskeletal Research Unit, University of Eastern Finland, and, Kuopio University Hospital, Kuopio, Finland.
Stéphane BlouinLudwig Boltzmann Institute of Osteology at Hanusch Hospital of OEGK and AUVA Trauma Centre Meidling, 1st Medical Department Hanusch Hospital, Vienna, Austria.
Petra KeplingerLudwig Boltzmann Institute of Osteology at Hanusch Hospital of OEGK and AUVA Trauma Centre Meidling, 1st Medical Department Hanusch Hospital, Vienna, Austria.
Ville-Valtteri VälimäkiDepartment of Orthopedics, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
Heikki KrögerDepartment of Orthopedics, Kuopio Musculoskeletal Research Unit, University of Eastern Finland, and, Kuopio University Hospital, Kuopio, Finland.
Outi MäkitieChildren's Hospital and Pediatric Research Center, University of Helsinki and Helsinki University Hospital, Stenbäckinkatu 9, Helsinki, Finland.
Markus A HartmannLudwig Boltzmann Institute of Osteology at Hanusch Hospital of OEGK and AUVA Trauma Centre Meidling, 1st Medical Department Hanusch Hospital, Vienna, Austria.
Hanusch Hospital · ATUniversity of Eastern Finland · FIUniversity of Helsinki · FIKarolinska University Hospital · SE

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Autoimmune polyendocrine syndrome type-1 (APS1) is characterized by autoimmune manifestations affecting different organs from early childhood on. Immunological abnormalities, the resulting endocrinopathies, and their treatments may compromise bone health. For the first time in APS1, we analyzed transiliac bone biopsy samples by bone histomorphometry and quantitative backscattered electron imaging in three adult patients (female P1, 38 years; male P2, 47 years; male P3, 25 years). All had biallelic mutations in the autoimmune regulator gene and in addition to endocrinopathies, also significant bone fragility. Histomorphometry showed bone volume in the lower normal range for P1 (BV/TV, - 0.98 SD) and P3 (- 1.34 SD), mainly due to reduced trabecular thickness (TbTh, - 3.63 and - 2.87 SD). In P1, osteoid surface was low (OS/BS, - 0.96 SD); active osteoblasts and double labeling were seen only on cortical bone. P3 showed a largely increased bone turnover rate (BFR/BV, + 4.53 SD) and increased mineralization lag time (Mlt, + 3.40 SD). Increased osteoid surface (OS/BS, + 2.03 and + 4.71 SD for P2 and P3) together with a large proportion of lowly mineralized bone area (Trab CaLow, + 2.22 and + 9.81 SD for P2 and P3) and focal mineralization defects were consistent with abnormal mineralization. In all patients, the density and area of osteocyte lacunae in cortical and trabecular bone were similar to healthy adults. The bone tissue characteristics were variable and included decreased trabecular thickness, increased amount of osteoid, and abnormal mineralization which are likely to contribute to bone fragility in patients with APS1.

Indexed as

Bone DensityPolyendocrinopathies, AutoimmuneAdultBone and BonesBone MatrixChild, PreschoolCortical BoneFemaleHumansMaleAutoimmune polyendocrinopathy-candidiasis-ectodermal dystrophyAutoimmune regulatorBone histomorphometryQuantitative backscattered electron imaging

Identifiers

PMID36944707
PMCPMC10198912
OpenAlexW4353020751

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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 Socratic graph.