Evaluation of Bleeding Symptoms in Children with An Inherited Mucocutaneous Bleeding Disorder.
Notice bibliographique
Résumé
Abstract Abstract 1292 Poster Board I-314 Introduction Accurate assessment of bleeding symptoms is an essential step in the diagnosis of an inherited mucocutaneous bleeding disorder (IMBD). This is difficult in childhood when symptoms such as bruising and epistaxis are frequently noted in normal children. In addition, children may not have been exposed to hemostatic challenges such assurgery, dental extraction and menarche. Knowledge of the spectrum of presenting features of an IMBD may aid diagnosis. The MCMDM-1 VWD Bleeding Questionnaire, which uses a symptom-specific grade of -1 to 4 (Tosetto et al., J Thromb Haemost 2006:4;766), was recently modified for use in a pediatric population by inclusion of questions related to pediatric-specific bleeding symptoms. The Pediatric Bleeding Questionnaire (PBQ) has been validated in the assessment of bleeding symptoms in children and a score of 2 or greater predicts a diagnosis of VWD (Bowman et al., J Thromb Haemost 2009; doi:10.1111/j.1538-7836.2009.03499.x). This present study applied the PBQ to a cohort of children with a prior diagnosis of an IMBD in order to evaluate the prevalence and severity of specific bleeding symptoms. Patients and Methods The PBQ was used to determine bleeding score for 123 children with a prior diagnosis of VWD or a platelet function disorder (PFD) at The Hospital for Sick Children, Toronto, or Kingston General Hospital, Kingston, by interview of parents/children. A clinically-significant bleeding symptom was defined as one severe enough to require consultation with a healthcare professional, medical/surgical intervention or blood transfusion. Results 100 children had a diagnosis of VWD and 23, a PFD (Glanzmann thrombasthenia: 4; dense granule deficiency: 9; MYH9-related disease: 3; Noonan syndrome: 2; Ehlers-Danlos syndrome: 2; undefined PFD: 3). 65 were male and 58 female with a median age of 10.7 years (range: 0.6-18). Mean bleeding score was 8.3 for the entire cohort (range: 0-29) and varied between diagnostic groups (definite type 1 VWD: 9.5 (range: 2-18); possible type 1 VWD: 3.0 (range: 0-15); type 2 VWD: 11.8 (range: 3-17); type 3 VWD: 14.6 (range: 4-29); PFD: 9.5 (range: 1-20). The prevalence of each of the bleeding symptoms is shown in Table 1. The most frequent clinically-significant bleeding symptoms in the cohort were: menorrhagia, 57% (of menstruating females); epistaxis, 36%; prolonged bleeding from minor wounds, 35%; and bleeding after tooth extraction, 30%. The most frequent clinically-significant bleeding symptoms did not vary greatly between each of the diagnostic groups but oral cavity bleeding, hemarthrosis, gastrointestinal bleeding and hematoma were more frequent in children with type 3 VWD. All symptoms were less frequent in children with possible type 1 VWD. Menorrhagia was a prevalent symptom in menstruating females in all diagnostic groups. Clinically-significant pediatric-specific bleeding symptoms were seen in 20% of children. The most frequent were: post-circumcision bleeding, 35% (of circumcised males); cephalohematoma, 7%; macroscopic hematuria, 4%; and umbilical stump bleeding, 3%. These symptoms occurred most frequently in children with type 3 VWD, type 2 VWD or PFD. Conclusions Children with an IMBD frequently report menorrhagia, epistaxis, prolonged bleeding from minor wounds and bleeding after tooth extraction. Pediatric-specific bleeding symptoms also occur. There are differences in prevalence of symptoms between subtypes of VWD and PFDs. This information can assist the evaluation of children with bleeding symptoms. 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 | 0,001 | 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 | 0,000 |
| 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 ».