Medical and Surgical Management for Heavy Menstrual Bleeding in Women with Ehlers-Danlos Syndrome
Notice bibliographique
Résumé
Background Ehlers-Danlos syndrome (EDS) is a heterogenous group of inherited collagen disorders associated with bleeding diathesis. While easy bruising is a diagnostic criterion in many EDS subtypes, it is not a formal diagnostic criterion in the most common subtype, hypermobile EDS (hEDS). As such, EDS diagnosis may be delayed in patients with bleeding of unknown cause. While heavy menstrual bleeding (HMB) is present in up to 60-76% of patients with EDS, there is scarce data on management. Existing studies have primarily focused on hormonal therapies in pediatric and adolescent populations. Objectives To describe the rates of gynecological bleeding and evaluate medical and surgical management of HMB in women with EDS, compared with age-matched controls with von Willebrand disease (VWD). Methods This retrospective cohort study included women (≥18 years) followed by the Northern Alberta Bleeding Disorders Program with a diagnosis of EDS (2000-2023). The cohort was matched to women with VWD by age (± 2 years). Those with other bleeding disorders were excluded. We examined quality of care measures including time from bleeding symptom to EDS diagnosis and surgical management for HMB prior to EDS diagnosis. We compared rates of gynecological bleeding, focusing on medical and surgical management for HMB in EDS patients compared with matched controls. Results We included 42 women with EDS and 42 women with VWD (type 1 VWD in 17 [71%], type 2 in 6 [25%], unspecified in 1 [4%]); median age was 38 years. Median ISTH-BAT bleeding scores were similar in EDS (9, IQR 9-10) and VWD cohorts (8, IQR 5-10). EDS subtypes included: hEDS in 29 (69%), unspecified in 8 (19%), and vascular EDS in 5 (12%). Almost half (19; 45%) had medical genetics consultation for EDS. Most common comorbidities in the EDS cohort included psychiatric disorders (18; 43%), hypertension (11; 26%), dyslipidemia (6; 14%), diabetes (6; 14%), and venous thromboembolism (5; 12%). Women experienced delayed diagnosis despite a high proportion (48% of EDS and 33% of VWD cohort) with known family history of bleeding disorder prior to hematology consult. EDS patients were diagnosed at an older age than VWD (median 33 vs 26 years, P=0.04), and experienced a median of 4 bleeding events prior to diagnosis (vs 3 in VWD, P=0.13). Twelve (29%) EDS patients experienced severe bleeding requiring Emergency Department (ED) visit and 6 (14%) required surgical hemostasis prior to diagnosis. Thirty-seven (88%) women with EDS experienced HMB, including 21 (50%) with severe HMB requiring ED visit, hospitalization, transfusions, or surgical hemostasis. Thirteen (31%) experienced postpartum hemorrhage, 6 (14%) hemorrhagic ovarian cysts, and 4 (10%) antepartum hemorrhage. Despite the high prevalence of gynecological bleeding, only 13 (31%) received gynecology consultation. There was no significant difference in the rates of gynecological bleeding between EDS and VWD. Most common HMB management included tranexamic acid (TXA, 29/37 with HMB; 78%), desmopressin (DDAVP, 21; 57%), oral contraceptive pills (OCP, 18; 49%) and intrauterine system (IUS, 16; 43%). Seven (19%) women did not receive hormonal therapy for HMB. Surgical management included hysterectomy (11; 30%), endometrial ablation (4; 11%), and dilation and curettage (3; 8%). Most hysterectomies/ablations (78%) were performed prior to EDS diagnosis. Those who received TXA had a significantly lower rate of ID than those who did not at last follow-up (21% vs 62%, P=0.01); DDAVP recipients trended towards lower rate of ID (19% vs 48%, P=0.10). Medical and surgical therapy rates were comparable among the 36 VWD patients with HMB: TXA (28/36; 78%), DDAVP (21; 58%), OCP (17; 46%), hysterectomy (7; 19%), and endometrial ablation (3; 8%), except for a trend towards lower IUS use (7; 19%, P=0.05). Nearly half (43%) underwent hysterectomies/ablations prior to VWD diagnosis. Conclusions Women with EDS and bleeding diathesis experienced delayed diagnosis despite high prevalence of known family history and gynecological bleeding. Gynecology consultation, hormonal therapies and TXA for HMB all have room for improvement. Delayed diagnosis and suboptimal medical therapy may contribute to higher rates of surgical hemostasis for HMB in EDS compared to age-matched VWD patients. Better patient and clinician education, and multidisciplinary care may improve timely recognition and management of gynecological bleeding in EDS.
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 | 0,001 |
| 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) | 0,001 | 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 ».