Adequate menstrual suppression in adolescents with inherited bleeding disorders often requires multiple treatment changes: Retrospective cohort study of a multidisciplinary clinic.

Heavy menstrual bleeding (HMB) is often the presenting symptom for females with inherited bleeding disorders (IBD). Multidisciplinary clinics leverage the expertise of hematologists and women's health specialists. This study characterizes the complexity of HMB management for adolescents with IBDs from a large multidisciplinary clinic. Adolescents often required multiple different menstrual suppression treatments, with only about 20% achieving acceptable suppression with their first treatment. Adolescents switched therapy most often for uncontrolled bleeding, followed by adverse effects, and patient preference. Given the difficulty in achieving adequate menstrual suppression, multidisciplinary clinics offer necessary expertise in accomplishing bleeding control with minimal adverse effects.

Procedural outcomes in children with mild type 1 von Willebrand disease.

Background: In patients with mild type 1 von Willebrand disease (VWD), treatment guidelines suggest individualization of surgical management. However, these conditional recommendations are based on very low-certainty evidence due to limited data on surgical outcomes in this population. Objectives: To characterize procedural bleeding prophylaxis strategies and outcomes in children with mild type 1 VWD. Methods: This is a retrospective cohort study that included patients aged between 0 and 21 years with mild type 1 VWD (defined as von Willebrand factor antigen and/or an activity of 30-50 IU/dL) who underwent a procedure from July 1, 2017, to July 1, 2022. Demographic, surgical, medication, and bleeding data were collected by manual chart review. Results: A total of 161 procedures were performed in 108 patients. The population was primarily female (75%), White (77.8%), and non-Hispanic (79.6%). Median age was 15.8 years (IQR, 8.2-17.6). Fifty-nine surgeries were classified as major, 66 as minor, and 36 as dental. For most procedures, patients received only antifibrinolytics for bleeding prophylaxis (n = 128, 79.5%); desmopressin was used in 17 (10.6%) procedures, and von Willebrand factor concentrate was used in 12 (7.5%) procedures. Bleeding complications occurred in 8 (5.0%) procedures: these included 1 major, 4 clinically relevant nonmajor, and 3 minor bleeding events. No patient required blood transfusion or an additional procedure to achieve hemostasis. Most bleeding complications were seen following intrauterine device (IUD) placement (5/8). Nearly 30% of patients who underwent IUD placement reported bleeding. Conclusion: Pediatric patients with mild type 1 VWD can safely undergo procedures using a tailored approach. Bleeding complications were uncommon, with the majority following IUD placement.

Dental habits and oral health in children and adolescents with bleeding disorders: A single-institution cross-sectional study.

INTRODUCTION: Oral health is an important component of care at haemophilia treatment centres (HTCs). Correlations between oral health and inflammation suggest that proper oral health may improve joint health. AIM: To evaluate the dental habits, needs, and oral health status of paediatric patients with bleeding disorders, and identify predicters of poor oral health. METHODS: From May 2016 to October 2017, consecutive paediatric HTC patients completed a 14-question survey and were examined by dental professionals. Descriptive analyses, chi-square tests and logistic regression models identified characteristics associated with four main dental outcomes. RESULTS: Evaluations from 226 consecutive patients (age 1-20 years) were included. Diagnoses included haemophilia A and B (64%), von Willebrand disease (25%) and other bleeding disorders (13%). Nearly half of patients reported not brushing their teeth twice a day (44%). One-quarter of patients did not currently have a dentist (27%), and 15% reported specific challenges with access to dental care. Oral screening demonstrated significant pathology: 89% of patients had plaque accumulation, 37% had gingivitis and 8% had lesions suggestive of dental caries. Multivariate analysis revealed that having a primary caregiver with active decay was associated with significantly higher rates of suspicious lesions (OR 4.34, CI 1.41-13.35) and gingival erythema (OR 3.44, CI 1.63-7.25) and lower rates of twice daily teeth brushing (OR .17, CI .08-.37). CONCLUSION: Children with bleeding disorders commonly have significant dental pathology and report obstacles to dental care, posing the potential risk for morbidity. Primary caregiver dental health is strongly associated with dental pathology in children.

Elevated von Willebrand factor levels during heavy menstrual bleeding episodes limit the diagnostic utility for von Willebrand disease.

BACKGROUND: Heavy menstrual bleeding (HMB) is often the first bleeding symptom for female individuals with inherited bleeding disorders. Guidelines recommend performing the hemostatic evaluation at HMB presentation. Von Willebrand factor (VWF) levels increase with stress, making it unclear if VWF studies during acute bleeding are beneficial in diagnosing von Willebrand disease (VWD). OBJECTIVES: To determine the utility of testing for VWD during acute HMB. PATIENTS/METHODS: This retrospective cohort study evaluated VWF levels of individuals presenting to the emergency department (ED) with HMB from January 1, 2017, to December 31, 2018, after prospective implementation of a clinical practice guideline recommending hemostatic evaluation in the ED. We compared VWF and factor VIII (FVIII) levels between acute presentation and follow-up visit after bleeding resolution. We compared the diagnostic accuracy of initial and follow-up labs. RESULTS: During the study period, 221 individuals were seen in the ED for acute HMB, and 39 had VWD testing at both time points. Median FVIII and VWF levels were higher during acute bleeding than at follow-up. The difference in VWF levels between visits was negligible when initial FVIII value was normal. Overall incidence of VWD was 7.5%; 69% of those with VWD had low VWF levels during acute HMB. CONCLUSION: VWD testing during acute HMB detects the majority of individuals with VWD but also leads to elevated levels of VWF, potentially limiting at the accuracy of diagnostic labs during acute bleeding episodes. Delayed testing until resolution of anemia and active bleeding may provide more accurate diagnostic evaluation for VWD.

Impact of dialect use on a basic component of learning to read.

Can some black-white differences in reading achievement be traced to differences in language background? Many African American children speak a dialect that differs from the mainstream dialect emphasized in school. We examined how use of alternative dialects affects decoding, an important component of early reading and marker of reading development. Behavioral data show that use of the alternative pronunciations of words in different dialects affects reading aloud in developing readers, with larger effects for children who use more African American English (AAE). Mechanisms underlying this effect were explored with a computational model, investigating factors affecting reading acquisition. The results indicate that the achievement gap may be due in part to differences in task complexity: children whose home and school dialects differ are at greater risk for reading difficulties because tasks such as learning to decode are more complex for them.