SUN-749 Denosumab In Combination With Burosumab For A Case Of X-linked Hypophosphatemia Associated With Postmenopausal Osteoporosis
Bibliographic record
Abstract
Abstract R.A. Alnajimi: None. A.A. Khan: Other; Self; Amgen Inc, Research Fund. Denosumab in Combination With Burosumab for a Case of X-Linked Hypophosphatemia Associated With Postmenopausal Osteoporosis Background X-linked hypophosphatemia (XLH) is a rare inherited metabolic disorder with multisystem manifestations. This progressive disease is characterized by hypophosphatemia due to excess serum levels of fibroblast growth factor 23 (FGF23) resulting in renal phosphate wasting and reduced 1,25-dihydroxyvitamin D synthesis. Denosumab is effective in reducing fractures in patients with postmenopausal osteoporosis. We report a case of coexisting osteoporosis in a patient with XLH and management with denosumab. Clinical Case A 55-years old female presented with a diagnosis of XLH at 5 years of age. She had presented with bowing of legs. No history of fractures, dental abscesses, osteoarthritis, enthesopathy or renal disease. She had been treated with conventional therapy (phosphate salts and active vitamin D) with intermittent interruption of phosphate between ages 64 and 69, leading to high ALP consistent with osteomalacia. At age 59 years, the patient was diagnosed with hypertension and hyperparathyroidism secondary to chronic kidney disease. PTH remained elevated despite reductions in active vitamin D dose and phosphate. phosphate levels were between 0.59-1.29 mmol/L (RR: 0.8-1.45). At age 64 years, the BMD at total hip site was 0.420 g/cm2 corresponding to T-score -3.0. At the age of 67 years, she developed hypercalcemia with elevations of ionized calcium and PTH, ALP remained high. Calcium creatinine clearance ratio was low at 0.009 attributed to stage 3a CKD. Burosumab was initiated at this stage, the mean PO4 levels remained in the low range at 0.63-0.79 mmol/L with mean TmP/GFR of 0.44-0.78 (RR: 0.96 -1.44). The patient was offered parathyroidectomy but she declined and she also declined cinacalcet to improve parathyroid function. At 69-years of age, she agreed to start 60 mg denosumab 6 monthly for coexisting postmenopausal osteoporosis. She had elevations in ALP ,PTH , persistent hypophosphatemia and renal phosphate leakage. Subsequent evaluation at 70-years of age revealed that with Denosumab and Burosumab, normal ALP and ionized Calcium were restored, despite low PO4 at 0.60 mmol/L in association with hyperparathyroidism as PTH was elevated at 29.4 pmol/l . The BMD at the total hip site showed improvement, measuring 0.852 g/cm2, corresponding to a T-score of -1.2 Conclusion To our knowledge, this is the first case demonstrating the potential role of denosumab in short-term treatment of coexisting postmenopausal osteoporosis in an XLH patient as evidenced by improved BMD. However, the long-term efficacy of denosumab needs to be determined. Sunday, June 2, 2024
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 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 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.001 | 0.000 |
| Science and technology studies | 0.001 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.002 | 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".