Evaluation of the Severity and Pattern of Epistaxis In Children with a Mucocutaneous Bleeding Disorder and In Healthy Children Using a Standardized Questionnaire
Notice bibliographique
Résumé
Abstract Abstract 713 Epistaxis is a common symptom in children with mucocutaneous bleeding disorders such as von Willebrand disease (VWD) or a platelet function disorder (PFD), but also occurs in healthy children. Determining the features of epistaxis that suggest underlying hemostatic defects would help distinguish which children with epistaxis should undergo laboratory testing to screen for an inherited bleeding disorder. This is particularly significant in young children who may not yet have been exposed to a hemostatic challenge. However, there is no standardized approach to determine which children presenting with epistaxis should undergo testing. The aim of this study was to describe the severity and pattern of epistaxis in children with VWD or a PFD vs. healthy children using the PBQ, a standardized, validated Pediatric Bleeding Questionnaire (Bowman et al, J Thromb Haemost 2009:7;1418). PBQ epistaxis scores were assigned depending on clinical severity of the most severe episode (0: no epistaxis or trivial, ≤5/yr; 1: >5/yr or >10 min duration; 2: consultation with a healthcare professional; 3: packing, cauterization or antifibrinolytics; 4: blood transfusion, replacement therapy or desmopressin); scores of ≥2 were considered clinically significant. Epistaxis scores and features (frequency, duration, onset (spontaneous or not), site (uni- or bilateral), presence of seasonal correlation, whether cessation required medical intervention) were retrospectively reviewed in 107 questionnaires administered to patients/parents of patients with a known diagnosis of VWD (type 1, n=60 [definite, with abnormal laboratory results at least twice + a significant bleeding history, n=37; possible, with abnormal laboratory results at least once ± a significant bleeding history, n=23]; type 2, n=8; type 3, n=16) or a PFD (Glanzmann thrombasthenia, dense granule disorder, MYH9-related disorder, Noonan syndrome, Ehlers-Danlos syndrome, unspecified; n=23) and in 163 questionnaires administered to healthy children (Bowman et al, J Thromb Haemost 2009:7;1418; Biss et al, J Thromb Haemost 2010:8;950; Biss et al, J Thromb Haemost 2010:8;1416). Epistaxis was present in 66/107 patients (62%) (definite type 1 VWD: 62%; possible type 1 VWD: 48%; type 2 VWD: 38%; type 3 VWD: 75%; PFD: 74%) with a median age of 12 yrs (range: 0.6–18.3 yrs), and in 56/163 healthy children (34%), with a median age of 11.5 yrs (range: 1.5–17.0 yrs). 59% of the 66 patients with epistaxis were males, as were 50% of the 56 controls with epistaxis. The mean PBQ epistaxis score in these 66 patients with VWD or a PFD was greater, 2.1 (range: 0–4), than in the 56 controls, 0.4 (range: 0–2) (P<0.0001). The mean PBQ epistaxis score in patients varied according to underlying diagnosis and was greater in those with type 3 VWD (3.1) than in the patients with definite type 1 VWD (2.2), possible type 1 VWD (0.8), type 2 VWD (2.0) or a PFD (2.1) (P<0.05). There was no effect of age or gender on PBQ epistaxis scores within patient and control groups. 38/66 patients (58%) had a clinically significant score compared to only 5/56 (9%) of controls (P<0.0001). Epistaxis frequency (>5 episodes/yr) was greater in patients than controls, 44/66 (67%) vs. 11/56 (20%) (P<0.0001). More patients (41/66; 62%) had epistaxis lasting >10 min compared with controls (5/56; 9%) (P<0.0001). Most patients and controls reported spontaneous onset of epistaxis − 57/66 (86%) and 44/56 (79%), respectively (P=0.2316). However, a greater number of patients bled from both nostrils (51/66; 77%) than controls (15/56; 27%) (P<0.0001). Seasonal correlation of epistaxis occurred less frequently in the patient group; 14/66 patients (21%) had epistaxis only during 1 or 2 specified seasons of the year while 24/56 controls (43%) had seasonal correlation of epistaxis (P=0.0219). More patients (38/66; 58%) required medical intervention for cessation of epistaxis than controls (5/56; 9%) (P<0.0001). In conclusion, the standardized PBQ is useful in the assessment of epistaxis pattern and severity in children with a mucocutaneous bleeding disorder – VWD or a PFD. The severity score is significantly greater in patients vs. controls. Features of epistaxis suggestive of an underlying bleeding disorder include: epistaxis frequency >5 episodes/yr; duration >10 min; epistaxis occurring from both nostrils; lack of seasonal correlation; and need for medical intervention for cessation. 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 | 0,003 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,000 | 0,001 |
| Bibliométrie | 0,001 | 0,001 |
| Études des sciences et des technologies | 0,000 | 0,000 |
| Communication savante | 0,000 | 0,001 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,000 | 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 ».