Successful Management of Pregnancy with Severe Von Willebrand Disease in a Jehovah's Witness with Recombinant Von Willebrand Factor
Notice bibliographique
Résumé
Abstract Background: Von Willebrand disease (vWD) is the most common inherited bleeding disorder with a reported prevalence of 1% in epidemiological studies and symptomatic prevalence of 1 in 10,000. Pregnancy in vWD is associated with increased bleeding risk particularly postpartum hemorrhage. Treatment options include desmopressin acetate (DDAVP), plasma derived factor concentrates and antifibrinolytic agents. Human Recombinant von Willebrand factor (vWF) (Vonvendi®) has been approved in the United States for on demand treatment and perioperative management of adults with vWD. It has been shown to maintain sustained levels of VWF activity but requires co-administration with FVIII to achieve adequate FVIII levels. Recombinant VWF is an option for patients who refuse blood for religious reasons. Case: Here we describe a 39 year old patient in her third pregnancy who is a Jehovah's Witness. Consent was obtained from the patient for this report. She was initially diagnosed with von Willebrand`s disease at the age of six, when she had hematuria. At the age of 11, with menarche, she had significant menorrhagia resulting in symptomatic anemia, a reduction in her hemoglobin concentration to 44 g/L, requiring uterine artery embolization. She was placed on an oral contraceptive pill for menorrhagia. She used DDAVP for trauma induced injury. Her first pregnancy resulted in spontaneous abortion. She required a D&C and DDAVP was used. Prior to delivery of the second pregnancy DDAVP was used but she had postpartum hemorrhage, requiring additional dosing of DDAVP and uterine artery embolization. In the current pregnancy, aPTT was 36.4 seconds with normal PT and platelet count and blood group O. At 15 weeks' gestational age, VWF antigen (ACL TOP 700 -IL HemosIL) was 0.11 units/ml (normal range for blood group O 0.45-1.5 unit/ml), ristocetin cofactor (ACL TOP 700 -IL HemosIL), 0.09 units/ml (normal range 0.48-2.0 units/ml for blood group O), and FVIII level (Sysmex CS5100 -Dade Actin FS) 0.09 units/ml (normal range 0.58-1.9 units/ml). Factor levels at 23 weeks' gestation 1 hour following DDAVP were FVIII 1.05 units/ml, VWF antigen 0.5 units/ mL, and ristocetin cofactor 0.52 units/ml. She was willing to accept recombinant factor concentrates only. Results: She had an elective admission for induction of labor at 37 weeks but proceeded to Cesarean Section due to non-progression of labor. Her PTT on admission was 40 seconds with VWF antigen of 0.11 units/ml, VWF activity < 0.07 units/ml and FVIII 0.13 units/ml. The Table describes her levels following the administration of recombinant vWF. She was administered Vonvendi® on a planned dose of 40 IU/kg Xynta® and rFVIII 30 IU/kg to increase factor levels to more than 50%; 60-min following infusions vWF : RCo was 0.64 U/ml with FVIII 0.79 U/ml. The Cesarean section was performed under spinal anesthesia without complications. Tranexamic acid was used intravenously before the delivery of the neonate and continued for first two days and then changed to oral dose. Target levels were achieved over the 5 days after delivery with the current regimen. She experienced a transient hypersensitivity reaction with urticaria and dyspepsia after the third dose of Vonvendi®. She did not have significant bleeding. Her hemoglobin concentration remained stable at 120 g/L throughout her inpatient stay. The neonate did not have bleeding with delivery but was found to have VWF antigen of 0.11 U /ml, VWF activity of 0.07 U/ml and FVIII of 0.16 U/ml. Genetic analysis of the mutation associated with her vWD is in progress. Conclusion: The use of rvWF and rVIII resulted in adequate hemostasis peripartum. Further prospective data are required to reaffirm the safety and dosing of rvWF for peripartum management of patients with vWD who require intervention. Disclosures Kazi: Shire: Other: Vonvendi was provided by Shire.
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 | 0,002 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,000 | 0,001 |
| Bibliométrie | 0,001 | 0,000 |
| Études des sciences et des technologies | 0,001 | 0,000 |
| Communication savante | 0,001 | 0,001 |
| Science ouverte | 0,000 | 0,001 |
| Intégrité de la recherche | 0,001 | 0,002 |
| 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 ».