Hospitalization Rate and Regional Differences in Comprehensive Care in Transfused Patients with Sickle Cell Disease Compared to Thalassemia: A Report from the Multi-Center Study of Iron Overload.
Notice bibliographique
Résumé
Abstract Red blood cell transfusions are often used to prevent the complications of thalassaemia (Thal) and sickle cell disease (SCD), though chronic therapy frequently results in iron overload-related organ injury. The Multi-Center Study of Iron Overload is a prospective, natural history study conducted to compare the comorbidities of iron overload in Thal with SCD. This report examines differences in morbidity and comprehensive care by disease and geographic region. 584 subjects aged 12 years and over, were screened for inclusion from 30 clinical hematological centers in the US, Canada and the UK. Socioeconomic and clinical data from subjects with iron overload (LIC of ≥ 10 mg/g dry wt by biopsy) and either Thal (N=142, 54% Male) or Tx-SCD (N=199, 43% Male) were collected. A group of non-iron overloaded SCD (NonTx-SCD N=64; 50% Male) were also enrolled. Tx-SCD were hospitalized more frequently (4.1± 3.8 times/year) compared to Thal (1.8± 1.7) or NonTx-SCD (2.1± 1.7; p<0.001) subjects, though average length of stay was similar (Tx-SCD: 7.0± 5.3 days; Thal: 5.7± 4.6; NonTx-SCD: 5.3± 3.8, p=0.16). Within the Tx-SCD group, adult subjects were 2.4 times more likely to be hospitalized in the previous 12 months compared to pediatric subjects (p=0.004, 95%CI 1.3–4.4). Roughly half of the SCD hospitalizations were for pain, whereas subjects with Thal were hospitalized for a variety of reasons, most often secondary to infection. Subjects with Thal were more likely to have routine iron assessments (LIC by biopsy: 66% vs. 37%), and screening for iron related organ injury such as thyroid function (TSH: 85% vs. 33%) and cardiac function (ECHO: 75% vs. 54%; EKG: 60% vs. 41%) compared to Tx-SCD (all p<0.001). Subjects with Thal had 3.4 times higher odds of having a recent liver biopsy compared to Tx-SCD (p<0.001; 95%CI: 2.2–5.3). Pediatric Tx-SCD subjects were more likely to have a biopsy compared to adults (46.5 vs. 23.5; p <0.001), however this likelihood was not related to serum ferritin level, duration of transfusion or chelation. Combining Thal and Tx-SCD groups, liver biopsy was more commonly performed at international vs. U.S. centers, (p<0.001), however the size of the clinical center was unrelated. Whereas, both regional and center size differences were observed in ECHO and TSH testing (p<0.01). A total of 26 subjects have died or exited from the study due to significant medical events. The mortality among adult Tx-SCD subjects was 2.8 fold greater than that of Thal (p=0.017). These data suggest that despite significant morbidity and iron burden, Tx-SCD subjects are monitored on a less frequent basis for iron related organ damage compared to Thal subjects. Differences were also observed in standards of care by region and center size. This disparity in care requires attention and guidelines for the assessment and management of Tx-SCD subjects should be developed and supported until the effect of iron overload in this population is better understood.
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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) | 0,000 | 0,000 |
| Bibliométrie | 0,001 | 0,001 |
| Études des sciences et des technologies | 0,000 | 0,000 |
| Communication savante | 0,001 | 0,000 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,000 | 0,000 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,001 | 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 ».