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Medical treatment of osteogenesis imperfecta

2000· article· en· W1981020841 on OpenAlexaff
Horacio Plotkin, Francis H. Glorieux

Bibliographic record

VenueDrug Development Research · 2000
Typearticle
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicConnective tissue disorders research
Canadian institutionsShriners Hospitals for Children - CanadaMcGill UniversityMontreal Children's Hospital
Fundersnot available
KeywordsOsteogenesis imperfectaMedicineAdverse effectDeformityBone mineralIncidence (geometry)PediatricsSurgeryOsteoporosisInternal medicine

Abstract

fetched live from OpenAlex

Osteogenesis imperfecta (OI) comprises a group of disorders principally affecting type I collagen which result in increased bone fragility. Children with severe OI suffer recurrent fractures, resulting in severe deformity and growth stunting in many cases, with loss of independent ambulation by the teenage years in over 50% of cases. Recently, cyclical intravenous treatment with pamidronate has proven of benefit to children with severe forms of OI. Bone mineral density (BMD) and physical activity increased markedly in these patients and fracture rate decreased. In our experience with more than 160 children with OI treated with pamidronate, BMD increases dramatically and the incidence of radiologically confirmed fractures falls. The treatment does not alter fracture healing, growth rate, or growth plate appearances. Dependence on mobility aids is reduced and all subjects able to communicate reported substantial relief of chronic pain and fatigue. No adverse side effects were noted apart from the well-known acute phase reaction during the first infusion cycle. In our group of patients, pamidronate did not have a detrimental effect on growth. It is unclear at present how long pamidronate treatment should be continued, and which is the optimal treatment schedule and dosage. There were no significant side effects after 7 years of treatment, but longer surveillance is warranted. New bisphosphonates are under investigation to compare their effects to those of pamidronate. Although this form of therapy does not address the basic abnormalities that underlie the OI syndromes, it allows, for the first time, to significantly alter the natural course of the disease and improve patients' clinical status and quality of life. Drug Dev. Res. 49:141–145, 2000. © 2000 Wiley-Liss, Inc.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame machine prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.

metaresearch head score (Codex)0.000
metaresearch head score (Gemma)0.001
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Not applicable · Consensus signal: none
GenreCandidate signal: Empirical · Consensus signal: none
Teacher disagreement score0.005
Threshold uncertainty score0.015

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0000.001
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0010.000
Science and technology studies0.0010.000
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.001
Insufficient payload (model declined to judge)0.0050.001

Machine scores (provisional)

The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.

Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.

Opus teacher head0.036
GPT teacher head0.374
Teacher spread0.338 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designNot applicable
Domainnot available
GenreEmpirical

How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".

Quick stats

Citations2
Published2000
Admission routes1
Has abstractyes

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