A219 EARLY PREDICTORS OF UNFAVORABLE OUTCOME IN NORTH AMERICAN INDIAN CHILDHOOD CIRRHOSIS
Notice bibliographique
Résumé
Abstract Background North American Indian Childhood Cirrhosis (NAIC) has only been described in the Cree-Ojibway First Nations of Northern Quebec. NAIC presents with transient neonatal jaundice and progresses to biliary cirrhosis often requiring liver transplantation (LT) in childhood. Only 30 patients have been described to date and risk factors associated with an earlier progression to LT have not yet been identified. Moreover, NAIC patients seem to experience more fractures than other cholestatic patients, but this has not been confirmed. Aims With this study, we aimed to identify predictors at 6 months from presentation that might suggest progression to end-stage liver disease as well as evaluate bone health in affected patients. Methods The records of all NAIC patients diagnosed between 2000–2020 were reviewed. Subjects were split into 2 groups based on whether they had undergone LT or not (No_LT) before age 18. Recorded complications included: hepatic encephalopathy (HE), variceal bleeding (VB), ascites, spontaneous bacterial peritonitis (SBP), bacteremia, and pulmonary shunts. Laboratory data (alanine aminotransferase, ALT; total bilirubin, TB) were collected at presentation and follow-up. Bone mineral density (BMD) of the lumbar spine (Z-scores) and number of fractures were compared between groups. NAIC patients were then compared to cohorts with other chronic cholestatic diseases such as biliary atresia (BA, n=24) and Alagille syndrome (AS, n=11). Results A total of 14 patients (M=9, F=5) were diagnosed with NAIC. Average age at presentation was 2.1 months (IQR 1–16.9 months), with 3 patients older than 18 months. Overall, 6 patients were transplanted (avg 8.6±1.7 years), one was listed for LT but died waiting, and 7 remained in a state of compensated cirrhosis. All complications were only observed in the LT group except for VB which also occurred in 2 patients of the No_LT group. Between presentation and 6 months, ALT and TB levels increased more in the LT vs No_LT group (p=ns). There was a greater variation of ALT/TB levels in the LT group (p=0.0047) even once the 3 patients with late referral were excluded (p=0.0381). No patient in the No_LT group had fractures, while 3 did in the LT group. BMD was lower in the LT group vs No_LT group (-2.2±1.2 vs. -1.1±1.3, p=ns). NAIC patients had lower BMD (-1.7±1.3) than those with AS (0.7±0.9, p=0.003) or BA (-0.9±1.4, p=ns) and had a higher prevalence of fractures (21.4% vs. 12.5% for BA and 18.2% for AS patients). Conclusions In patients with NAIC, variation of ALT/TB levels at 6 months from presentation may be used as an early predictor of unfavorable outcome and progression towards end-stage liver disease. Patients who evolved to LT had more complications, higher prevalence of fractures and lower BMD values. Compared to children with BA or AS, NAIC patients had poorer bone health. Funding Agencies None
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) | 0,000 | 0,000 |
| Bibliométrie | 0,001 | 0,002 |
| Études des sciences et des technologies | 0,001 | 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) | 0,003 | 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 ».