Salter, L., Offiah, A.C. orcid.org/0000-0001-8991-5036 and Bishop, N. orcid.org/0000-0001-7263-8546 (2018) Elevated platelet counts in a cohort of children with moderate-severe osteogenesis imperfecta suggest that inflammation is present. Archives of Disease in Childhood , 103 (8). pp. 767-781. ISSN 0003-9888
Abstract
BACKGROUND: Elevated platelet counts are observed in cancer, autoimmunity and inflammation with concurrent illness. Proinflammatory cytokines are elevated in murine osteogenesis imperfecta (OI) models. We hypothesised that platelet counts might be elevated in children with moderate-severe OI. METHODS: We reviewed the hospital records of 71 children with moderate-severe OI, treated in the Sheffield Children's Hospital's Severe, Complex and Atypical Osteogenesis Imperfecta Highly Specialised Service. Data relating platelet count (below/above average, above upper limit) to prior and concurrent events were summarised as event proportions per child. Additionally, we created platelet SD scores to assess age and time-related trends, and relationship with OI type. RESULTS: 1206 platelet counts were recorded. Platelet SD scores were right-shifted by 0.89 SD overall. 49 of 71 (69%) patients had at least one platelet count above the normal range and 246 (20.4%) of all counts were above the upper limit of normal. Of these, 101 (41%) were high despite no confounding factors being present. For the 47 children with data at age less than 2 years, 89 (30.0%) platelet counts were above the upper limit of normal and 39 (44%) had no associated confounding factor. Elevated platelet counts were recorded most often for children with new or existing vertebral fractures. CONCLUSIONS: Raised platelet counts were observed in association with new and healing vertebral fractures, but also (41%-44%) in the absence of identified proinflammatory factors or events. We speculate that these findings are evidence for a proinflammatory component to OI that could be a target for therapeutic intervention.
Metadata
Item Type: | Article |
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Authors/Creators: |
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Copyright, Publisher and Additional Information: | © 2018 BMJ Publishing Group. This is an author produced version of a paper subsequently published in Archives of Disease in Childhood. Uploaded in accordance with the publisher's self-archiving policy. |
Keywords: | bone disease; collagen disorders; musculo-skeletal |
Dates: |
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Institution: | The University of Sheffield |
Academic Units: | The University of Sheffield > Faculty of Medicine, Dentistry and Health (Sheffield) > The Medical School (Sheffield) > Division of Genomic Medicine (Sheffield) > Department of Oncology and Metabolism (Sheffield) |
Depositing User: | Symplectic Sheffield |
Date Deposited: | 19 Mar 2018 12:26 |
Last Modified: | 11 Nov 2020 09:22 |
Status: | Published |
Publisher: | BMJ Publishing Group |
Refereed: | Yes |
Identification Number: | 10.1136/archdischild-2017-313859 |
Related URLs: | |
Open Archives Initiative ID (OAI ID): | oai:eprints.whiterose.ac.uk:128666 |