Renal Dysfunction in Thalassemia.
Notice bibliographique
Résumé
Abstract Abstract 2008 Poster Board I-1030 BACKGROUND: Renal function is generally thought to be normal in patients with thalassemia, but it has not been studied in detail. There are recent reports of renal tubular defects in both thalassemia intermedia and major. The cause is unclear, but both iron toxicity and iron chelation therapy have been suggested. The study of renal function in thalassemia is timely, because the new oral iron chelator, deferasirox, may increase creatinine concentration and cause proteinuria. Pre-existing, thalassemia-related renal function abnormalities could be a risk factor for deferasirox-related nephropathy. Therefore, it is critical to know how thalassemia affects the kidney. We aimed to determine the prevalence of renal function abnormalities in patients with thalassemia before deferasirox was widely clinically available and to evaluate associations between renal abnormalities and genetic factors, environmental factors, and transfusion regimens. METHODS: We studied patients who participated in the Low Bone Mass Cross-sectional Observational Study (LBMCOS) of the NIH Thalassemia Clinical Research Network. We included all LBMCOS patients (≥6 years of age) with a creatinine clearance (CCr) and calcium to creatinine ratio (Ca:Cr) measured by 24-hour urine collection. We only included patients whose urinary creatinine excretion rate was appropriate for age and gender, indicating a complete urine collection. Notably, none of these patients was treated with deferasirox; nearly all were treated with deferoxamine. We used multivariable models to determine significant predictors of CCr and Ca:Cr. Potential predictors included age, gender, diagnosis, transfusion status, serum transferrin receptor concentration, serum ferritin, and the presence of a variety of clinical complications. RESULTS: This cohort included 216 patients [51.4% female, mean age 12.6 years (range 6.1 – 75.4)]. 188 had beta thalassemia, 14 had Hb H disease or Hb H-Constant Spring, 13 had Hb E-beta-thalassemia, and 1 had homozygous alpha thalassemia major. 180 subjects were regularly transfused (≥8/yr) and 36 were not. Among all patients, 7.8% had a low CCr, 71.3% had a normal CCr, and 20.8% had a high CCr (adjusted for age, gender and body surface area). Regularly transfused patients had significantly lower mean CCr than those not regularly transfused [143.8 ± 54.3 (mean ± SD) mL/min/1.73 m2 vs. 173.4 ± 65; P=0.004]. Among all patients, 71.3% had a normal Ca:Cr, whereas 28.7% had a high Ca:Cr. Regularly transfused patients had significantly higher mean Ca:Cr than those not regularly transfused (0.18 ± 0.10 vs. 0.10 ± 0.08; P<0.0001). Multivariable models showed that the only significant predictors of CCr were regular transfusion status [Odds Ratio (OR) 0.39, 0.17-0.88] and serum transferrin receptor concentration (OR 1.03, 1.01-1.05). Likewise, the only significant predictors of Ca:Cr were regular transfusion status (OR 3.72, 1.24-11.2) and serum transferrin receptor concentration (OR 0.98, 0.95-0.99). CONCLUSIONS: Renal hyperfiltration is common in thalassemia patients, especially those who are not regularly transfused. Hyperfiltration could be the consequence of chronic anemia. Regularly transfused thalassemia patients have a lower CCr and are more likely to have hypercalciuria than patients who are not regularly transfused. This transfusion-related effect could represent iron-mediated tubular and glomerular injury. Awareness of underlying renal dysfunction in thalassemia patients could inform decisions about the use and monitoring of potentially nephrotoxic agents, such as deferasirox. Further studies are needed to better understand renal function in patients with thalassemia. Disclosures: No relevant conflicts of interest to declare.
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 | 0,001 | 0,002 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,000 | 0,000 |
| Bibliométrie | 0,000 | 0,001 |
| Études des sciences et des technologies | 0,000 | 0,000 |
| Communication savante | 0,000 | 0,000 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,000 | 0,001 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,004 | 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 ».