Progressive Pseudorheumatoid Dysplasia: Functional impact of unilateral total hip replacement of three cases.
Bibliographic record
Abstract
Background : Progressive pseudorheumatoid dysplasia (PPD) is a rare genetic disorder of cartilage homeostasis. By its multiple osteoarthropathies, it leads to severe physical disability affecting several functions. Prosthetic joint replacement can be adopted to handle end-stage arthropathy, although, to our knowledge, there have been a few reports concerning functional outcomes after joint replacement.The purpose of this work is to describe the functional impact after unilateral total hip arthroplasty (THA) of three sisters with PPD. Case report : Three sisters aged 28, 25 and 31 years old, had symmetric mixed polyarthralgia starting at the age of 10 years with articular deformation and stiffness of the cervical and lumbar spine, major and minor joints including significant functional consequences that limit their daily activities. Laboratory tests revealed no anomalies. Standard radiographs demonstrate epimetaphyseal enlargement without erosive lesions. The three sisters received unilateral THA followed by rehabilitation sessions at the Department of Physical and Rehabilitation Medicine. Range of motion has been improved as well as the function index Postel-Merle of Aubigné and the Western Ontario and McMaster Universities Arthritis Index without overall functional progress. Conclusion : PDD usually causes a numerous joint stiffness that may be a source of substantial functional limitations. Therefore, prosthetic joint replacement might be the only way to rebuild a functional joint. In this observation, although the three patients benefited from a unilateral total hip replacement combined with rehabilitation exercises, which hold an important place in therapeutic management; the functional result was not very satisfactory due to several associated factors, hence the importance of early diagnosis.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot 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.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.002 |
| Meta-epidemiology (narrow) | 0.002 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.002 | 0.001 |
| Science and technology studies | 0.002 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.002 |
| Research integrity | 0.003 | 0.001 |
| Insufficient payload (model declined to judge) | 0.002 | 0.000 |
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.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
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".