SUN-749 Denosumab In Combination With Burosumab For A Case Of X-linked Hypophosphatemia Associated With Postmenopausal Osteoporosis
Notice bibliographique
Résumé
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
Récupéré en direct depuis OpenAlex et désinversé. Les résumés ne sont pas conservés dans cette base de données : les index inversés représentent 8,6 Go des 9,3 Go de texte de la base, et le serveur dispose de 13 Go libres.
Comment cette classification a été obtenuedéplier
Prédiction machine sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Le volet Gemma est une étiquette directe du modèle pour chaque travail de la base, lue sur la notice réduite au titre. Le volet Codex est un classifieur appris des 10 348 étiquettes directes de Codex et calibré sur les taux pondérés de l'échantillon; les champs sans appui suffisant ne portent aucun appel Codex. Le mode candidate est l'union des deux volets; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont pas des étiquettes humaines.
Scores du classifieur distillé par catégorie (deux têtes)
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,000 | 0,001 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,001 | 0,001 |
| Bibliométrie | 0,001 | 0,000 |
| Études des sciences et des technologies | 0,001 | 0,000 |
| Communication savante | 0,000 | 0,000 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,002 | 0,001 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,002 | 0,000 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule source (Gemma direct ou Codex distillé), pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».