Pictorial Bleeding Assessment Chart Research Articles

Rare bleeding disorders in Egyptian females presented with heavy menstrual bleeding: single-center study

BackgroundHeavy menstrual bleeding is an important health problem in women of reproductive age and is also one of the most common symptoms in women with bleeding disorders. Data about the frequency of rare bleeding disorders are limited, and population-based studies are lacking, so we aimed to determine the frequency of rare bleeding disorders among women presented with heavy menstrual periods that cannot be attributed to obvious problems.MethodsComplete blood count and bleeding profiles include activated partial thromboplastin time, prothrombin time, factor VIII activity assay, ristocetin cofactor activity, von Willebrand antigen assay, platelet aggregation tests and other factor assays in 100 out of 300 females presented with unexplained heavy menstrual period, pictorial bleeding assessment chart (PBAC) > 100 as a screening tool for heavy menstrual periods, and or International Society of Thrombosis and Hemostasis-Bleeding Assessment Tool (ISTH-BAT) > 6.ResultsA total of 300 women with heavy menstrual periods without an obvious explained cause were included in our study. Among them, we found 100 (30%) females with a mean PBAC of 234 ± 147 and mean ISTH-BAT of 9 ± 5 denoting HMB may be due to underlying bleeding disorders. Among them, the most common diagnosis was VWD in 30 (30%). Other disorders were as follows: 28 (28%) cases with clotting factor deficiencies, 24 (24%) cases were found to have platelet dysfunction, and in 18% of our studied cases, we did not find a clear cause of their bleeding disorders (unknown).ConclusionRare bleeding disorders are not uncommon and require comprehensive hemostatic evaluation as well as simple tools like PBAC and ISTH-BAT questionnaires for the identification of females presented with unexplained HMB.

Metrics of uterine myomatosis symptomatology in clinical practice.

A review of diagnostic options and standardized methods for specifying clinical symptoms associated with uterine myomatosis. A literature search of databases aimed at finding validated metrics for assessing myomas symptomatology. Summary of obligatory and optional examination methods in the diagnosis of uterine fibroids and methods of examination of reproductive parameters of the couple in relation to myomatosis. Defining the metrics of bleeding manifestations of myomatosis with emphasis on the "Pictorial Bleeding Assessment Chart". Reviewing metrics of algic manifestations of myomatosis and quality of life with emphasis on the "Quality of Life Questionnaire - Myomatosis - Severity Symptom Scale". The purpose of the applicable metrics, which summarize objective findings and subjective complaints of patients, is to describe and evaluate possible correlations and dynamics of changes in the health status, depending on the chosen therapy.

Are Medical Learners Adept at Recognizing Heavy Vaginal Bleeding?

Background - More than half of reproductive-age females experience heavy vaginal blood loss (VBL), whether menstrual, lochial, or otherwise, and yet less than 10% seek medical attention for evaluation. Moreover, there is evidence that even when females present to medical attention that heavy VBL is serially under-recognized and untreated. Complications from heavy VBL adversely impact health-related quality of life. This makes characterizing and quantifying VBL critical. It is well documented that there are widespread knowledge and care gaps surrounding VBL due to structural multidimensional stigma. Importantly, it is unclear the extent to which medical learners confidently recognize symptoms and signs of heavy VBL. Objectives - To explore the understanding, attitudes, and perceptions of medical learners in characterizing VBL using an online survey. Methods - An online survey was launched to assess trainees' ability to identify heavy VBL. Survey questions were informed by the literature and tested for validity. The survey was distributed online to trainees working at all local University affiliated hospitals. Results were analyzed using descriptive statistics. Institutional ethics approval was obtained. Results - 73 learners (medical students, residents, and fellows) completed the survey. 88% of learners were between the ages of 25-34, and 56% self-identified as women. 27% were 3rd /4th level medical students, 30% were 2nd year residents, 43% 4th year residents or higher. 27% of residents were from Emergency Medicine, 14% Hematology, 12% Family Medicine, 10% Internal Medicine, and 10% Obstetrics and Gynecology. 94% of trainees reported asking about heavy VBL in the last 6 months. Most described feeling comfortable asking about and diagnosing VBL regardless of cultural, religious or ethnic background, sexual orientation, or gender. 56% were aware of bleeding assessment tools (BATs), 51% of menstrual cups, 21% of the pictorial bleeding assessment chart (PBAC) however, less than 60% had used BATs, less than 75% had used menstrual cups and less than 80% had used the PBAC in clinical practice (Figure 1). Overall, trainees acknowledged stigma surrounding iron deficiency without anemia, and that iron deficiency was associated with decreased health-related quality of life. They recognized the need to screen and not rely on patients being forthcoming about excessive VBL. Discussion - Heavy VBL is exceedingly common, has important clinical and psychosocial ramifications, yet it continues to be stigmatized, underdiagnosed, and thus poorly treated. We found that while a broad range of medical learners described themselves as largely feeling comfortable with their skills in assessing VBL and being aware of tools to facilitate the diagnosis of heavy VBL, surprisingly few had used these tools in clinical practice. Our findings highlight important knowledge gaps surrounding VBL and interestingly, show excessive confidence in its diagnosis amongst medical learners. Targeted knowledge translation rooted in theory- and evidence-based implementation science is urgently required in this space. Next steps involve assessing trainee skills in practice and exploring patient lived experiences with VBL through qualitative interviews.

Real-World Use of Recombinant Von Willebrand Factor in People with Clinically Severe Congenital Von Willebrand Disease: Interim Analysis of Athn 9: A Natural History Study for People with Severe VWD

Background: People with von Willebrand disease (VWD) present with a spectrum of bleeding patterns and clinical symptoms, independent of subtype (Rugeri. Haematologica. 2007; Kalot. Haemophilia. 2021). In certain situations, prophylaxis may be appropriate, particularly when there is recurrent and severe bleeding (Connell, Blood Advances, 2021). There are limited real-world data on the use of recombinant von Willebrand factor (rVWF) in the United States. Aim: To characterize the safety and real-world effectiveness of routine prophylaxis with rVWF in people with clinically severe congenital VWD. Study Design and Methods:This is an interim analysis of a phase 4, longitudinal, prospective, observational cohort study (NCT03853486). Participants with severe VWD types 1, 2, and 3 (≤30% of normal) or “clinically severe VWD” (≤40% of normal with severe bleeding and requiring prophylaxis with factor concentrates) were identified from the ATHNdataset. The American Thrombosis & Hemostasis Network (ATHN) is the steward of the ATHNdataset, a Health Insurance Portability and Accountability Act-compliant, de-identified patient health dataset containing data from individuals with bleeding disorders receiving care through ATHN affiliates who opt in to contribute. Treatment regimens were at the discretion of each participant's respective bleeding disorder provider. Participants are followed for 2 years from the time of enrollment. Safety was measured using definitions from the European Haemophilia Safety Surveillance program. Bleeding events (BEs) were measured by individual bleeding components and calculated per the International Society on Thrombosis and Haemostasis Bleed Assessment Tool and/or the Pictorial Bleeding Assessment Chart. Health-related quality of life measures, including an ATHN9 rVWF substudy form, were used to describe real-world effectiveness of rVWF treatment. Descriptive statistics were used to analyze study outcomes. This study was not designed to detect differences in safety or efficacy between individual treatment products. Interim Results:As of June 16, 2023, 23 participants enrolled in the ATHN9 study were receiving rVWF; 14 participants have been prescribed rVWF for at least 1 full year, 13 of whom received continuous prophylaxis. This abstract reports on those 13 participants in the first year of the study. Mean (SD) age was 42.9 (29.2) years; 69.2% (n=9) of participants were female. Most participants had no comorbidities. As expected, most participants had a family history of VWD (84.6%; n=11) and no documented VWF inhibitors (84.6%; n=11); inhibitor status for the remaining 2 participants was unknown. Baseline VWF and factor VIII measurements were reported for most participants ( Table 1). The majority had type 2 VWD (84.6%; n=11), 92.3% (n=12) had identifiable genetic sequence defects in VWF, and 30.8% (n=4) had an abnormal VWF multimer profile. As dictated by the local investigator, rVWF prophylactic regimens ranged from 36.6 IU/kg once weekly to 57.6 IU/kg every 3 days; 7 (53.8%) participants received 40-60 IU/kg at a frequency of 2-3 times per week. Twenty-one BEs occurred in 5 (38.5%) of the participants receiving rVWF prophylaxis during the first year ( Table 2). Most BEs were spontaneous (61.9%; 13/21), occurring in the nose (n=7), gastrointestinal tract (n=5), or ankle (n=1). The remaining BEs were associated with activity with no known trauma (n=4), trauma (n=2), or unknown cause (n=2). One participant reported 3 rVWF treatment-unrelated adverse events (AEs) of humerus shaft closed fracture, metabolic encephalopathy, and acute exacerbation of chronic obstructive airways disease. When surveyed about their experience with rVWF, participants reported very or extremely satisfied as follows: 76.9% (n=10) with infusion time, 84.6% (n=11) with breakthrough bleed control time, 46.2% (n=6) with number of infusions required for breakthrough bleed control, and 69.2% (n=9) with frequency of achieving bleed control without concomitant hemostatic medication. Discussion:In participants with clinically severe congenital VWD, continuous prophylaxis with rVWF was associated with minimal AEs. Most participants receiving rVWF prophylaxis experienced no BEs during the first year of the study. Most participants expressed overall satisfaction with the use of rVWF.

Relugolix combination therapy and symptoms of uterine myomatosis – selected case reports of indication spectrum and treatment outcomes

Summary: Objective: An illustrative review of the indications for relugolix combination therapy (RCT) in the management of symptoms associated with uterine myomatosis. Methods: A set of annotated case reports from outpatient and clinical practice. Results: The fi le includes a non-invasive methodology for defining excessive menstrual bleeding using the pictorial bleeding assessment chart (PBAC). It also presents the use of RCT as a fertility-sparing procedure prior to elective myomectomy and the management of isthmic fibroids as an uterine factor of infertility. Cases of RCT of adenomyosis in primary sterility and in extragenital forms of endometriosis are commented. Emergent events associated with complications of myomatosis in pregnancy are represented by a case report of necrotizing diff use myomatosis in puerperium. The differential-diagnostic confusion of adnexal pathology and myomatosis, RCT as a final solution to failed pharmacotherapy, and the alternative of hysterectomy in premenopause illustrate the diverse spectrum of indications for pharmacological treatment, including the possibility of dual therapy with RCT and aGnRH. Conclusion: Relugolix combination therapy as an effective and safe causal treatment expands the therapeutic spectrum and options for reproductive medicine specialists and registering gynaecologists. The availability of conservative treatment in combination with surgical treatment leads to optimalization and greater effectiveness of therapeutic procedures and increased quality of life for women with myomatosis. Key words: relugolix combination therapy – uterine fibroid symptoms

Bleeding disorder of unknown cause: Results from Iranian study

BackgroundDefinite diagnosis of patients with mild to moderate bleeding is challenging. Some studies reported that even more than 50% of their patients remained undiagnosed which is classified as a Bleeding disorder of unknown cause (BDUC). This study aims to document the clinical characteristics and proportion of patients with BDUC in the Iranian Comprehensive Hemophilia Care Center (ICHCC) one of the referral centers for diagnosis of congenital bleeding disorder in Iran. MethodsThis study was conducted on 397 patients who were referred with a bleeding manifestation to ICHCC from 2019 to 2022. Demographic and laboratory data were documented for all patients. Bleeding questionnaires including ISTH-Bleeding Assessment tool (ISTH-BAT) and the Molecular and Clinical Markers for the Diagnosis and Management of Type 1 (MCMDM-1 (ISTH-BAT, MCMDM-1, and the Pictorial Bleeding Assessment Chart (PBLAC) were filled out for all patients. The data were analyzed by the statistical package for social science (SPSS version 22, SPSS, Chicago, Illinois, USA). ResultsBDUC was diagnosed in 200 patients and 197 patients reached the final diagnosis. Hemophilia, von Willebrand disease (VWD), factor (F) VII deficiency, and platelet functional disorders (PFDs) were confirmed in 54, 49, 34, and 15 of the patients, respectively. No significant difference was found in bleeding scores between patients with BDUC and those with confirmed disease. In contrast, after setting cut-off (ISTH-BAT for males ≥ 4 and females ≥ 6 and MCMDM-1 for males ≥ 3 and females ≥ 5) clinically significant difference was found. There was no association between having a positive consanguineous marriage and setting a diagnosis; however, significant associations were seen for having a positive family history of bleeding. Age (OR =0.977, 95% CI.965–0.989), gender (BDUC female, 151/200; final diagnosis female, 95/197) (OR=3.3, 95% CI 2.16–5.06), family history (OR = 3.19, 95% CI 1.99–5.11), and consanguineous marriage (OR=1.59, 95% CI 1.03–2.45) were considered as a risk factor for categorizing the patients with BDUC or final diagnosis. ConclusionThe findings are mainly in line with previous studies about BDUC patients. The large number of patients with BDUC underlines the incompleteness of available routine laboratory tests and shows the necessity of progress in the development of reliable diagnostic tools to identify underlying bleeding disorders.

Comparison of Bleeding Pattern and Quality of Life Before and After the Insertion of a Levonorgestrel Intrauterine System for Heavy Menstrual Bleeding: A Seven-Year Review.

Background This study aimed to examine the role of a levonorgestrel intrauterine system (LNG-IUS) in the treatment of heavy menstrual bleeding (HMB) regarding improvements in bleeding patterns and quality of life (QOL) and determine the reason for its failure or withdrawal from treatment in some patients. Methodology This retrospective study was conducted in a tertiary care center in eastern India. A seven-year assessment, with both qualitative and quantitative assessments, of the effect of LNG-IUS in women with HMB was performed using the Menorrhagia Multiattribute Scale (MMAS) and Medical Outcomes Study 36-Item Short-Form Health Survey (MOS SF-36) score as a tool to assess the QOL, and the pictorial bleeding assessment chart (PBAC) to assess bleeding patterns. The study population was divided into the following four groups based on duration: three months to one year, one to two years, two to three years, and more than years.The continuation, expulsion, and hysterectomy rates were evaluated. Results The mean MMAS and MOS SF-36 scores increased significantly (p < 0.05) from 36.73 ± 20.40 to 93.72 ± 14.62 and 35.33 ± 6.73 to 90.54 ± 15.89, respectively. The mean PBAC score decreased from 176.36 ± 79.85 to 32.19 ± 63.87. In total, 348 women (94.25%) continued the LNG-IUS, and 3.44% had uncontrolled menorrhagia. Furthermore, at the end of seven years, the expulsion rate was 2.28% due to adenomyosis and pelvic inflammatory disease, and the hysterectomy rate was 5.75%. In addition, 45.97% and 48.27% of the participants had amenorrhea and hypomenorrhea, respectively. Conclusions LNG-IUS improves bleeding and QOL in women with HMB. In addition, it requires less skill and is a non-invasive and nonsurgical option, which should be considered first.

Reduced Activity and Quality of Life in Women Soldiers with Heavy Menstrual Bleeding and Dysmenorrhea.

The aim of the study was to examine the effect of heavy menstrual bleeding (HMB) and dysmenorrhea on daily activity and quality of life (QoL) in young women engaged in demanding activities. Cross-sectional study. A total of 422 Israeli women soldiers in combat and non-combat roles. Participants were asked to provide consent and to complete study questionnaires. A pictorial bleeding assessment chart (PBAC), visual analog scale (VAS), verbal multidimensional scoring system for assessment of dysmenorrhea, and approved Hebrew-translated age-appropriate Pediatric Quality of Life Inventory (PedsQL). HMB (PBAC >100) and severe HMB (PBAC >185) were demonstrated in 181 (50%) and 96 (26%) participants, respectively. A high PBAC score was recorded in 20% who answered "no" or "unknown" in the questionnaire on having HMB. Mild, moderate, and severe dysmenorrhea were demonstrated in 80 (21.5%), 115 (31%), and 142 (38%) participants, respectively. The prevalence of HMB and dysmenorrhea was similar in soldiers in combat and non-combat roles. Diagnosis of HMB was related to the lower fitness-for-service score, history of bleeding, and dysmenorrhea. Daily activity and QoL were both affected by the severity of HMB and dysmenorrhea. Underdiagnosis of HMB and dysmenorrhea results from a combination of unawareness from the women's side and inattention from the system. In an era of female empowerment, each woman should be at the optimal physiological and psychological level to start her career; thus, addressing the menstrual burden and providing effective treatment is needed in the military scenario and other settings with demanding activities.

Bleeding in Patients with Clinically Severe Von Willebrand Disease: Interim Analysis of Athn 9: A Natural History Study for People with Severe Von Willebrand Disease (VWD)

Background:Von Willebrand Disease (VWD) is the most common inherited bleeding disorder, occurring in ~0.1% of the population. VWD results from either a quantitative (Type 1 or 3) or qualitative (Type 2) defect in von Willebrand Factor (VWF), a multimeric plasma protein involved in platelet adhesion and aggregation at the vascular injury site. Accurate diagnosis of VWD is complex due to pre-analytical variables, a wide coefficient of variation in testing, and incomplete penetrance. Though individuals with mild VWD and bleeding symptoms are common, less is known regarding persons with VWD and a clinically severe bleeding phenotype.Aims:To characterize the bleeding phenotype and safety of treatment regimens in participants with clinically severe VWD in the United States (US).Study Design and Methods:ATHN 9 is sponsored by the American Thrombosis and Hemostasis Network (ATHN) and is being conducted at 25 ATHN-affiliated sites across the US. Participants are identified by the site investigators with a projected goal to enroll 130 individuals. Patients with severe VWD defined as type 3 VWD, or a VWF:RCo, VWF:GPIbM or VWF:Ag ≤ 30%, or persons with “clinically severe VWD” defined by VWF:RCo, VWF:GPIbM or VWF:Ag ≤ 40% and requiring recurrent use of factor concentrates and prior enrollment in the ATHNdataset national surveillance data collection project are included. Participants with platelet-type or acquired VWD are excluded. Laboratory assessments including a standardized diagnostic battery, VWF genetic analysis (Next-gen sequencing), and inhibitor testing, performed by a central laboratory. Bleeding tendency is assessed by International Society for Thrombosis and Haemostasis (ISTH) Bleeding Assessment Tool (BAT) (abnormal BS is ≥4 in adult males, ≥6 in adult females and ≥3 in children) and the Pictorial Bleeding Assessment Chart (PBAC; heavy menstrual blood loss ≥100) if applicable.Results:Initial data on 81 participants was analyzed. Given that the enrollment occurred during the pandemic, the completion of data varied as follows: Baseline Demographics Forms 81 completed, Baseline History Forms 73 completed, and VWD Diagnostic Testing Results Forms 38 completed. Most were adult (53%), female (58%), Caucasian (82%) and non-Hispanic (82%). Approximately half (38/81, 47%) have undergone central lab diagnostic testing (Table 1) while the remaining had previous diagnostic studies. About half of the patients had Type 1, a quarter have Type 3, and the remaining had Type 2 VWD or unknown. The majority of patients (91%) had VWF:GPIbM activity <30IU/dL and (61%) had a VWF:Ag <30IU/dL. One participant had an inhibitor (1/22, 4%) to VWF. Of the participants with a baseline history form submission (73 in total), more than half (42/73, 57%) had undergone surgery, the most common being nasal cautery (11/42, 26.2%) and dental extraction (11/42, 26.2%); complicated by bleeding most commonly during or following dental extraction (2/11, 18.2%) and endoscopy/colonoscopy (2/4, 50%). Few participants (6/73, 8%) reported the presence of a target joint at enrollment, ankle being most common. The bleeding phenotype was variable with a mean ISTH BAT score of 10 (range 0-39) with the first bleeding event commonly occurring prior to age 10 years (51%), with 20% occurring prior to age 12 months (20%). The PBAC was performed on 6 of the 47 female participants in reference to their last period and was abnormal(mean 247; range 0-754). The majority (70 participants, 96%) utilized factor concentrates for prophylaxis or on-demand treatment; 27 participants (39%) were on continuous prophylaxis, while 8 (11%) were on event-based while less (10%) were on heavy menstrual bleeding (HMB) prophylaxis, and the remainder (40%) received episodic treatment. Participants used plasma derived VWF concentrate most commonly (64.3%) with the remainder using recombinant VWF or DDAVP/antifibrinolytics.Discussion: Initial evaluation of 81 participants with clinically severe VWD were diagnostically determined to be type 1 VWD with a majority having a bleeding phenotype (mean (ISTH BAT 10) and HMB (mean PBAC 247). In contrast to patients with mild disease where antifibrinolytics and desmopressin are frequently used, factor replacement was the most common treatment modality. Future analysis will focus on response to factor replacement therapy, genotype-phenotype correlation and quality of life. DisclosuresWeyand: Novo Nordisk: Research Funding; Genentech: Consultancy; Sanofi: Consultancy, Research Funding; Takeda: Consultancy. Friedman: Siemens: Consultancy; Bayer: Consultancy; Alexion: Speakers Bureau; Genentech: Consultancy; Instrumentation Laboratories: Consultancy; Sanofi: Consultancy. Haley: American Thrombosis and Hemostasis Network: Research Funding. He: ATHN: Ended employment in the past 24 months. Roberts: Takeda; Speakers Bureau: Novo Nordisk, Octapharma, Sanofi, Takeda.: Research Funding; Genentech, Novo Nordisk, Octapharma, Pfizer, Sanofi, Takeda, uniQure: Consultancy. Wynn: Sanofi: Research Funding; Takeda: Research Funding; Genentech: Research Funding. Sidonio: Sanofi, Takeda, Octapharma, Bayer, Biomain, Grifols, Kedrion, Genentech. Catalyst, Guardian Therapeutics, Novo Nordisk, Hema Biologics, Uniqure.: Consultancy, Honoraria. OffLabel Disclosure:Vonvendi (recombinant VWF) does not have a product indication for prophylaxis

Quality of life in adolescents with heavy menstrual bleeding: Validation of the Adolescent Menstrual Bleeding Questionnaire (aMBQ)

Background/ObjectivesHeavy menstrual bleeding (HMB) affects 34% to 37% of adolescent girls. The Menstrual Bleeding Questionnaire (MBQ) is a validated measure of menstrual bleeding–specific health‐related quality of life (HRQoL) for women aged ≥18 years. No similar measure existed for adolescents with HMB. Patients/MethodsHMB was defined by the Pictorial Bleeding Assessment Chart (PBAC) score ≥100. In Phase 1, a focus group of adolescents with HMB adapted the MBQ, to generate the Adolescent MBQ (aMBQ). In phase 2, participants with and without HMB were recruited from clinics and self‐referral. Each participant completed 3 questionnaires (aMBQ, Pediatric Quality of Life module [PedsQL]©, PBAC) at two time points. Validity of the aMBQ was assessed by Pearson’s correlation with the PedsQL©. Reliability was calculated using intra‐class correlation (ICC) in those without HMB. The receiver operating characteristic curve assessed the aMBQ’s ability to identify those with HMB. ResultsPhase 1 included five girls with a mean age of 17.1 (13‐18) years. The aMBQ was adapted from the MBQ by substituting four words/phrases that altered 8 of the 20 questions and by adding 1 new question. The 21‐item aMBQ has a score range of 0 to 77 (77 = worst HRQoL). Phase 2 included 52 participants: 20 with and 32 without HMB, with a mean age of 14.8 (11‐17) years. The validity of the aMBQ was confirmed by a moderate correlation with PedsQL© (r = −0.63; P< .001). Test‐retest reliability was substantial (ICC = 0.73; P= .04). An aMBQ score of >30 identified those with HMB with excellent discrimination (area under the curve = 0.82; sensitivity, 70.0%; specificity, 84.4%). ConclusionsThe aMBQ is a valid and reliable tool to assess HRQoL in adolescents with HMB.

Congenital Combined Bleeding Disorders, a Comprehensive Study of a Large Number of Iranian Patients

Congenital combined bleeding disorders (CBDs) are extremely rare disorders which mainly occur in regions with a high rate of consanguineous marriage. These disorders can present with a variety of symptoms ranging from mucocutaneous bleeding to life-threatening episodes. This study aims to evaluate the prevalence and clinical course of Iranian patients with congenital CBDs. This study is conducted on 450 patients with CBDs who were referred to the Iranian Comprehensive Hemophilia Care Center (ICHCC) between 2010 and 2020. All these patients were diagnosed through evaluation of past medical history and coagulation laboratory investigation. Out of 450 patients, 33 were entered in this study. Having excluded cases with factor (F) V and FVIII deficiency, as well as those with hereditary combined Vitamin K dependent clotting factor deficiency (VKCFD), We found the most common CBDs to be FV-FVII deficiency (n: 6, 18.1%), together with FVII and FX deficiency (n: 6, 18.1%). The most common reason for referral of these patients to ICHCC was postoperative bleeding (14.3%). The mean of The International Society on Thrombosis and Hemostasis-Bleeding Assessment Tool (ISTH-BAT) and condensed MCMDM-1VWD bleeding assessment tool were 9.6 ± 4.79 and 9.1 ± 4.87, respectively (P < 0.005). In 10 females of reproductive age, the mean of Pictorial Bleeding Assessment Chart (PBAC) score was 649.3 ± 554. Among all patients, 23 (69.7%) received on-demand replacement therapy, whereas 5 patients (15.1%) received prophylaxis. In Iran, the coinheritance of bleeding disorders is surprisingly higher than expected. Moreover, patients with congenital CBDs may experience serious bleeding manifestations.

Bleeding in Patients with Clinically Severe Von Willebrand Disease: Preliminary Results of Athn 9, a Natural History Study of the Safety, Effectiveness, and Practice of Treatment for People with Severe Von Willebrand Disease (VWD)

Bleeding in Patients with Clinically Severe Von Willebrand Disease: Preliminary Results of Athn 9, a Natural History Study of the Safety, Effectiveness, and Practice of Treatment for People with Severe Von Willebrand Disease (VWD)

Validation Study of the Heavy Menstrual Bleeding Questionnaire in Adolescents

Introduction Heavy menstrual bleeding (HMB) is a common problem among adolescent girls that affects many aspects of their lives. Estimating the effect of HMB on health-related quality of life (HRQoL) is important but challenging, as there is no instrument that measures menstrual bleeding-specific HRQoL for girls ages &amp;lt; 18 years. Aim To develop and assess the psychometric properties of a menstrual bleeding-specific HRQoL tool adapted for use by girls with HMB aged &amp;lt; 18 years. Methods HMB was defined using a pictorial bleeding assessment chart (PBAC) score &amp;gt;100 and/or hemoglobin &amp;gt;2 SD below age appropriate means. Phase 1 utilized a focus group of girls with HMB to review items of the Menstrual Bleeding Questionnaire (MBQ), validated for use in women ages ≥ 18 years, to generate new items and develop the Adolescent MBQ (aMBQ). In phase 2, participants were divided in two groups: those with and those without HMB. Each participant completed 3 questionnaires (aMBQ, Pediatric Quality of Life core module [PedsQL] and PBAC) at 2 time points. Validity of the aMBQ was measured by Spearman's correlation with the PedsQL. Reliability was calculated using an intra-class correlation (ICC) random effect model in those without HMB who repeated the 3 questionnaires within 30-60 days from baseline. Receiver Operating Characteristic (ROC) curve analysis assessed the ability of the aMBQ to distinguish between participants with and without HMB. Ethics approval and informed consent were obtained prior to participation. Results Phase 1 included 5 girls with previously diagnosed HMB. The MBQ was revised to be appropriate for adolescents by substituting 4 words/phrases that altered 8 of 20 questions (Table 1). With the addition of one new question, a 21-item aMBQ was developed with a score range of 0-77, with 77 representing the worst HRQoL. Phase 2 included 73 participants: 19 with HMB and 56 without HMB. Mean age of participants was 14.7 years (range 11-17 years). The validity of the aMBQ was confirmed by a moderate correlation with PedsQL (rho=-0.61). Test-retest reliability was substantial (ICC=0.71, p=0.03). An aMBQ score of &amp;gt;30 identified those with HMB with excellent discrimination (AUC=0.826, sensitivity 71.4%, specificity 88.0%). Conclusion The aMBQ is a valid and reliable measurement tool to assess HRQoL in adolescents with HMB that is easily implemented in the office setting. Furthermore, it may assist clinicians in identifying those with HMB and aid in the evaluation of treatment effectiveness both in clinical practice and research. Disclosures Belletrutti: Takeda Canada: Consultancy, Membership on an entity's Board of Directors or advisory committees, Research Funding; Novo Nordisk Canada: Membership on an entity's Board of Directors or advisory committees; Bayer Canada: Membership on an entity's Board of Directors or advisory committees; Roche Canada: Consultancy, Membership on an entity's Board of Directors or advisory committees; CSL Behring: Membership on an entity's Board of Directors or advisory committees. Matteson:ABOG: Honoraria, Other: Received stipend for being an oral boards examiner.; Myovant: Membership on an entity's Board of Directors or advisory committees; Bayer Ensure: Other: Co-Investigator for longitudinal research study and clinical trial. All funds go to site of research, Research Funding.

Under-Diagnosed and Under-Treated Menorrhagia in Young Women Produce Suboptimal Performance

Background: Menorrhagia can have a significant impact on a woman's life. It can lead to anemia, fatigue, difficulty with physical activities, absence from work, and avoidance of social activities and may be the first sign of a bleeding disorder. We presumed that many young women with menorrhagia do not seek medical advice or are under-treated. This study aimed to document the prevalence of menorrhagia, by questionnaire, visual scores and self-assessment; its association with bleeding disorders, and the effect of menorrhagia on activity and quality of life (QoL) in young adults engaged in compulsory military service. The military service in Israel is obligatory for women as for men, making this cohort similar to the general population. Method: In this cross-sectional study we gave a short oral lecture presenting the study objective and plan to female soldiers in combat and non-combat roles. Participants were asked to consent to complete a one page coded, unidentified, initial questionnaire. After providing an additional informed consent, study participants were asked to complete additional study questionnaires, including personal and family bleeding history, pictorial bleeding assessment chart (PBAC), dysmenorrhea questionnaire, and age-appropriate PedsQL™. All forms were coded with the same number as the initial questionnaire. PBAC scores above 100 were diagnostic for menorrhagia and above 200 signified severe menorrhagia. Results: Of 422 women soldiers completing the initial questionnaire, 365 (86.5%) completed all study questionnaires. Characteristics of women not completing all study questionnaires were similar to those with complete study data except there were more soldiers in a combat role in the former (P=0.02). The median (range, 25th-75th interval) of the PBAC score was 100 (4-590, 55-189). PBAC scores &amp;gt; 100 and 200 were reported by 181 (50%) and 81 (22%), respectively. Most participants correctly self-assessed menorrhagia; still, a high PBAC score was also recorded in some who answered "no" or "unknown" in the initial questionnaire (Figure 1). Diagnosis of menorrhagia was associated with a history of bleeding, specifically cutaneous and bleeding from the oral cavity (Figure 2), yet none of the participants reported a diagnosis of a known bleeding disorder. The use of NSAIDs during menstruation was associated with a higher rate of menorrhagia (Table 1). No association was found with the current army role. However, the diagnosis of menorrhagia was related to the pre-enrolment fitness ranking [a score reflecting the overall per-recruitment health status] (Table 1). Those with menorrhagia suffered from higher pain levels and moderate to severe negative effects on activity during the days of menstruation compared to those without menorrhagia (Table 1, Figure 3). A trend for lower QoL was found in those with menorrhagia (Table 1). Discussion: Menorrhagia in young adults is a significant clinical problem effecting activity and quality of life. It is under-recognized and under-treated. The association with other bleeding manifestations may suggest an under diagnosis of bleeding disorders. In a world of women empowerment, any treatable cause, leading to suboptimal performance should be addressed. Thus, assessment for menorrhagia and dysmenorrhea should be part of well-being visit also in young adults to provide appropriate management to reduce the undesired effects of menorrhagia. Disclosures Revel-Vilk: Takeda: Honoraria; sanofi-Genzyme: Honoraria; Pfizer: Honoraria.

Progestogen-releasing intrauterine systems for heavy menstrual bleeding.

Progestogen-releasing intrauterine systems for heavy menstrual bleeding.

Clinical significance of diagnostic algorithm in detection of mild hemostasis disorders in women with menorrhagia

Background/Aim. Coagulation disorders could be a cause of menorrhagia in women of reproductive age. The aim of the study was to estimate frequency of coagulation disorders and design an appropriate algorithm for detection of coagulation disorders. Methods. We investigated coagulation in 115 women (36.1 ? 9.6 years) with anamnestic data of menorrhagia, verified using semiquantitative method ? Pictorial Bleeding Assessment Chart (PBAC) with score ? 100. Results. Menorrhagia was objectively verified in sixty-four women (55.7%) and in comparison with those with normal menstruation they had higher PBAC score of menstrual cycle [median (Md) = 150.0 vs. Md = 50.0; p &lt; 0.001] but not its duration (7.2 ? 2.1 days vs. 7.3 ? 1.9 days; p &gt; 0.05). Coagulation defects was found in 12 (10.4%) women ? decreased F IX: Ac in 4 (3.5%), decreased F VII: Ac in 1 (0.9%), decreased F X: Ac in 1 (0.9%), decreased F XI: Ac in 1 woman (0.9%), while 5 (4.3%) women matched criteria for mild von Willebrand disease (VWD) type 1. Women with coagulation disorders had prolonged prothrombin time (PT) [Md = 13.1 s, range: 12.2?14.8 s vs. Md = 12.5 s, range 10.6?18.3 s; p = 0.032]. Anemia was diagnosed in 61 (53.0%) women. The strongest predictor of the hemostasis disorder was menorrhagia (Quotient of probability 0.018), then anemia presence (12.43), P? (2.35), menstrual cycle duration (1.16) and the PBAC score (0.98). Conclusion. The results of the study indicate the need to form a diagnostic algorithm for hemostasis disorders, primarily VWD. Sophisticated analysis of hemostasis is required, especially if predictive factors of statistical models are detected: objectively verified menorrhagia, anemia, prolonged menstrual cycle, PBAC score &gt; 100 and extended PT.

Initial Evaluation of Adolescent Females Hospitalized with Heavy Menstrual Bleeding

Background: Heavy menstrual bleeding (HMB) in adolescents can be severe and life-threatening. Up to 30% of young women who are hospitalized with anemia due to HMB have a bleeding disorder. Guidelines from the American College of Obstetrics and Gynecology (ACOG) and the National Heart, Lung, and Blood Institute (NHLBI) recommend evaluation for bleeding disorders in such patients. ACOG recommendations include testing for von Willebrand disease (VWD) and specify that consultation with a hematologist may help in interpreting results. NHLBI recommends testing for vWD be done in conjunction with a hematologist. As von Willebrand factor is an acute phase reactant, testing when patients are severely anemic and bleeding may not provide accurate results. ACOG guidelines do not include testing for platelet function disorders (PFD), though PFD may be as prevalent as VWD in females with HMB. Early and accurate diagnosis of bleeding disorders is important for health and quality of life, yet limited data exists on the diagnostic evaluation for bleeding disorders in adolescent females hospitalized for HMB.Objectives: To evaluate the diagnostic evaluation of bleeding disorders in adolescent females hospitalized for HMB.Methods: A retrospective, single center chart review of female patients aged 9-21 years hospitalized for HMB and anemia at a tertiary care children's hospital from January 1, 2000 until December 31, 2017 was done. HMB was defined as menses ≥7 days in length, use of 8 or more pads or tampons per day during menses, pictorial bleeding assessment chart (PBAC) score greater than 100, or symptomatic anemia. Patients were identified from our Hemophilia Treatment Center (HTC) registry, review of patients seen at a comprehensive clinic staffed by pediatric hematologists and gynecologists for adolescent females with HMB and bleeding disorders, and by an Electronic Medical Record (EMR) query of admission and discharge diagnoses of HMB and anemia. Data obtained included clinical features, diagnostic evaluation, and laboratory results.Results: 118 patients hospitalized for HMB and anemia were included. Inpatient Hematology consult or outpatient referral occurred in 68 (58%) of the patients; 60/68 (88%) had a bleeding disorder evaluation completed. 34 patients had a hematologic disorder. PFD was the most common (15/34; 44%) followed by VWD (9/34; 26%). 42% (50/118) of the patients did not have a Hematology consult or outpatient referral (Table 1). While hospitalized for HMB and anemia, 29 of the 50 patients had testing for vWD performed and only 4/29 (14%) had testing repeated as an outpatient once hemoglobin normalized. No patients tested for VWD while inpatient had results consistent with the diagnosis. Platelet function testing was performed in 10/50 patients using the platelet function analyzer (PFA-100) in 8 patients and platelet aggregometry in 2 patients.Conclusions: Despite national guidelines and the presence of known risk factors such as HMB since menarche and HMB causing severe anemia, the hematology service was not involved in the diagnostic process for a significant number of adolescent females. In these patients, testing often occurred while patients were hospitalized and was not repeated. Testing for platelet function disorders occurred infrequently and mainly consisted of the PFA-100 which lacks sensitivity and specificity. When patients were evaluated by Hematology and tested for bleeding disorders, a large proportion had a bleeding disorder, of which PFD were most common. This study demonstrates the need for standardization of the evaluation of adolescent females hospitalized for HMB. Guidelines should be updated to include testing for PFD. Hematologists should be involved when females are hospitalized for HMB and anemia. [Display omitted] DisclosuresCarpenter:Genentech Incorporated: Membership on an entity's Board of Directors or advisory committees; Nationwide Children's Hospital: Speakers Bureau; Bayer: Honoraria; Kedrion Pharmaceuticals: Membership on an entity's Board of Directors or advisory committees; Novo Nordisk Pharmaceuticals, Inc: Consultancy; HEMA Biologics: Consultancy; American Academy of Pediatrics: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Novo Nordisk: Consultancy; National Hemophilia Foundation (Impact Education): Speakers Bureau; Kane County State's Attorney: Consultancy; CSL Behring: Speakers Bureau; 4th Judicial District Attorney's Office- Colorado: Consultancy; Kedrion Biopharmaceuticals: Consultancy.

Heavy Menstrual Bleeding in Adolescent Females with Platelet Function Disorders

Background: Heavy menstrual bleeding (HMB) occurs frequently in adolescent females and a significant proportion have an underlying bleeding disorder. As diagnostic techniques for platelet function disorders (PFD) improve, these disorders are now recognized to commonly cause HMB and may be as prevalent as von Willebrand disease. Limited data exists about the prevalence of PFD in adolescents with HMB. Even less is reported on management and treatment outcomes in patients with HMB and PFD. HMB can negatively impact quality of life and cause serious adverse effects. In order to appropriately treat heavy menstrual bleeding in adolescent females with PFD, more information is needed about the prevalence, clinical manifestations, and management of these patients.Objectives: To evaluate the prevalence, clinical features, management, and outcomes of HMB in adolescent females with platelet function disordersMethods: A retrospective, single center chart review was performed of female patients aged 9-21 years with HMB and a diagnosed PFD treated at a tertiary care pediatric hospital from January 1, 2000 until December 31, 2017. Heavy menstrual bleeding was defined as menses lasting longer than 7 days, use of 8 or more pads or tampons per day during menstrual cycle, pictorial bleeding assessment chart score greater than 100, or symptomatic anemia. Patients were identified from our Hemophilia Treatment Center (HTC) registry, review of patients seen at a comprehensive clinic staffed by pediatric hematologists and gynecologists for adolescent females with HMB and bleeding disorders, and by an Electronic Medical Record (EMR) query of admission and discharge diagnoses of HMB and anemia. Data obtained included demographics, clinical features, laboratory results, treatment modalities, and outcomes.Results: 41 patients with PFD who achieved menarche were identified. 36 of these (88%) met criteria for HMB. The median age at presentation of HMB was 14 years (range 10-18). 35/36 patients had documented abnormal platelet aggregometry (PA) and the majority of patients (27/35) had at least 2 sets of abnormal PA. All patients were diagnosed with non-specific PFD. 15 of the 36 patients (41%) required hospitalization and packed red blood cell transfusion for severe anemia at time of presentation. Mean and median hemoglobin at presentation of HMB respectively were 9.5 gm/dL and 11.3 gm/dL (range 3.1-14.8) and 21 patients (58%) were anemic at presentation. 26 patients had ferritin obtained at presentation and 18 (69%) were iron deficient using our lab's reference range of ferritin less than 13 ng/dL. Half of the patients (18/36, 50%) failed first-line treatment. Successful first-line treatment included hormonal therapy alone (4 patients), hemostatic therapy alone with tranexamic acid (4 patients), hormonal plus hemostatic therapy (7 patients), and intra-uterine device plus hemostatic therapy (1 patient). The mean duration of HMB until report of resolution was 8.2 months (median 5 months; range 1-24). 8% (3/33) of patients reported continued HMB at last documented clinic visit.Conclusions: HMB occurred in the majority of adolescent female patients with PFD. These patients were frequently anemic and iron deficient. Severe and life-threatening anemia requiring hospitalization and packed red blood cell transfusion was common. First line treatment of HMB was not uniform and failed in 50% of the patients. Prospective studies are needed to standardize treatment of HMB in adolescents with PFD. DisclosuresCarpenter:Kedrion Pharmaceuticals: Membership on an entity's Board of Directors or advisory committees; Bayer: Honoraria; CSL Behring: Speakers Bureau; Kane County State's Attorney: Consultancy; National Hemophilia Foundation (Impact Education): Speakers Bureau; HEMA Biologics: Consultancy; Novo Nordisk: Consultancy; Nationwide Children's Hospital: Speakers Bureau; American Academy of Pediatrics: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Genentech Incorporated: Membership on an entity's Board of Directors or advisory committees; Kedrion Biopharmaceuticals: Consultancy; 4th Judicial District Attorney's Office- Colorado: Consultancy; Novo Nordisk Pharmaceuticals, Inc: Consultancy.

Outcomes of Levonorgestrel Intrauterine System and Rollerball Ablation in Treatment of Menorrhagia

Aim: To compare the efficacy, safety and satisfaction of rollerball ablation (RBA) with that of levonorgestrel intrauterine system (LNGIUS) in control of heavy menstrual bleeding (HMB). Patients and Methods: An open label prospective randomized controlled trial was conducted between October 2014 and March 2017 in Benha University Hospital, Benha, Egypt. Eighty four Eligible women with functional HMB (Aged ≥ 35 years and ≤45 years, uterine size ≤ 10 weeks gestational size, uterocervical length ≤ 10 cm) were randomly assigned (1 : 1), 42 women in each group, to undergo RBA procedures or LNG- IUS insertions by closed envelops method and followed up to 24 months after the procedures. Themain outcomes were menstrual blood loss as assessed by pictorial bleeding assessment chart (PBAC) and hemoglobin values (g/dl) at 3, 6, 12, 24 months. Also, women satisfaction, patients quality of life were evaluated by questionnaire administered at 3, 6, 12, 24 months. Treatment modality side effects, safety and treatment failures were also assessed. Results: Both treatment modalities resulted in a significant reduction of PBAC scores and elevation of hemoglobin values. Also, both treatments were associated with higher significant satisfaction, and quality of life. Despite that the results in term of PBAC scores and in hemoglobin were initially with RBA, eventually both two treatmentmodalities shown no significant difference at 12, 24 months. Follow up both the treatment modalities were associated with comparable treatment failure rates (13%). Conclusions: Women treated with both LNG-IUS and RBA for HMB follow up to 24 months shows that both LNG - IUS and RBA were effective in control HMB as well as safe and associated with high level of satisfaction and better quality of life than to pretreatment period. Choice between this two treatment modalities should be based on surgeon skill and women preferences.

A randomized control trial to assess efficacy of Mifepristone in medical management of uterine fibroid

BackgroundFibroids are the most common benign tumours of uterus. Heavy menstrual bleeding is the commonest concern for which medical attention is sought. Hysterectomies for leiomyoma constitute a third of all hysterectomies. Thus, healthcare cost to society due to uterine leiomyomas is of considerable importance. MethodsA prospective study was conducted at tertiary care hospital of armed forces. 120 women in pre-menopausal age group with complaints of menorrhagia, Pictorial Bleeding Assessment Chart (PBAC) scoring ≥100 and at least one fibroid ≥2.5cm in size were recruited in the study. Patients in Group 1 were given Tab Tranexemic acid (500mg) and Tab Mefenemic acid (500mg) three times a day during menstrual bleeding for a period of 6 months. Patients in Group 2 were given Tab Mifepristone 50mg twice a week. They were followed up at 1, 3 and 6 months of starting the medicine. Results were statistically analysed using Microsoft Excel sheet and paired t-test. ResultsThe average age was 40 years in the Group 2 and 45 years in Group 1. A size reduction of 36.99% in intramural and 39.39% in submucosal fibroids after six months of treatment with Mifepristone resulted in marked clinical improvement. 10% patients had side effects. In 30% of patients symptoms reappeared during the follow up period. ConclusionMifepristone when given in bi-weekly doses was found to be safe, efficacious, and cost effective as compared to treatment with tranexemic acid and mefenemic acid for management of fibroid uterus.