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Background: Individuals with bleeding disorders have been reported to have a number of oral health issues due to varying conditions. A comprehensive evaluation of the different oral health conditions has not been carried out in the past. This systematic review and meta-analysis was carried out to collate and critically analyse existing research, and provide a comprehensive overview of the current state of knowledge on oral health., Methods: A comprehensive search was conducted in electronic databases, including PubMed, Scopus and Embase, in October 2023. No restriction on time frame or language was applied. The risk of bias for cross-sectional studies was assessed using the Agency for Healthcare Research and Quality (AHRQ) tool, and case control studies were assessed using the New Castle Ottawa Scale (NOS)., Results: Twenty-two articles were included in the final analysis with a total sample size of 2422 subjects. Of the 22 articles assessed, nine quantitative assessments were included in the Meta analysis. Pooled data analysis was carried out. A total of 13 studies reported medium risk whereas the remaining nine studies showed low risk of bias. The weighted mean DMFT scores in individuals with bleeding disorders were found to be 2.43 [0.62. 4.24], mean dmft was 2.79 [1.05, 4.53] and mean OHI-S was reported to be 1.79 [1.00, 2.57], respectively., Conclusion: The findings emphasize that these individuals have fair oral hygiene and lower dmft/DMFT scores. Oral bleeding emerged as an important oral health component to be cautiously dealt with particularly during the stages of exfoliation/shedding., (© 2024 John Wiley & Sons Ltd.)

Introduction: There are a significant number of patients with mucocutaneous bleeding, specifically heavy menstrual bleeding (HMB), who do not have a diagnosed bleeding disorder. These patients receive nontargeted interventions and may have suboptimal treatments. Functional assays, particularly for fibrinolytic and rare platelet function defects, are not robust and not readily available., Aim: We aimed to prospectively evaluate the prevalence of genetic defects associated with rare bleeding disorders and describe alterations of coagulation and fibrinolysis in a cohort of adolescents with HMB., Methods: We performed a prospective observational cohort study of patients with HMB and unexplained bleeding. The study utilized a next generation sequencing panel and investigational global assays of coagulation and fibrinolysis. Additionally, specific functional assays were performed to help characterize novel variants that were identified., Results: In 10 of the 17 patients (∼59%), genetic variants were identified on molecular testing. Thrombin generation by calibrated thromboelastography was not significantly altered in this patient population. The clot formation and lysis assay showed a trend towards increased fibrinolysis with rapid phase of decline in 23% of the patients. Further corresponding functional assays and study population are described., Conclusion: Our study describes a unique correlative model in a homogenous cohort of patients with HMB and unexplained bleeding which may inform future diagnostic algorithms, genotype-phenotype correlations as well as aid in specific targeted treatment approaches. Larger future studies may inform risk stratification of patients and improve health related outcomes in patients with HMB., (© 2024 The Authors. Haemophilia published by John Wiley & Sons Ltd.)

Background: Heavy menstrual bleeding (HMB) is associated with a reduced quality of life and limitations in social and physical functioning. Data on HMB in women with rare bleeding disorders (RBDs), including coagulation factor deficiencies and fibrinolytic disorders, are scarce., Objectives: To analyze the prevalence, severity, and treatment of HMB in Dutch women with an RBD., Methods: The Rare Bleeding Disorders in the Netherlands (RBiN) study included 263 patients with an RBD from all 6 hemophilia treatment centers (October 2017-November 2019). In this analysis, data of 111 women aged ≥16 years were studied. According to the International Society on Thrombosis and Haemostasis bleeding assessment tool, HMB symptoms were scored from 0 (no/trivial) to 4 (severe symptoms requiring medical intervention). HMB was defined as a score ≥1. Age at RBD diagnosis was extracted from patient files., Results: HMB was reported by 80% of women (89/111) and was more prevalent in women with a fibrinolytic disorder (33/35; 94%) than in women with a coagulation factor deficiency (56/76; 74%) (P = .011). Of the 89 women with HMB, 82% (n = 73) ever required treatment. Multiple treatment modalities were frequently used, both in severe and mild deficiencies. Hormonal treatment was mostly used (n = 64; 88%), while antifibrinolytics were prescribed less frequently (n = 18; 25%). In women with HMB since menarche (n = 61; 69%), median age at RBD diagnosis was 28 years (IQR, 14-41)., Conclusion: HMB is common in women with RBDs. Women with mild deficiencies also frequently reported HMB. Only a minority of women were treated with hemostatic agents. A significant diagnostic delay was observed after the onset of HMB symptoms., Competing Interests: Declaration of competing interests K. Meijer reports speaker fees from Bayer and Alexion, participation in trial steering committee for Bayer, consulting fees from Uniqure, participation in data monitoring and endpoint adjudication committee for Octapharma. M.H. Cnossen's institution has received investigator-initiated research and travel grants as well as speaker fees over the years from the Netherlands Organisation for Scientific Research (NWO) and Netherlands National research Agenda (NWA), the Netherlands Organization for Health Research and Development (ZonMw), the Dutch Innovatiefonds Zorgverzekeraars, Stichting Haemophilia, Baxter/Baxalta/Shire/ Takeda, Pfizer, Bayer Schering Pharma, CSL Behring, Sobi Biogen, Novo Nordisk, Novartis, Roche, and Nordic Pharma, and for serving as a steering board member for Roche, Bayer and Novartis. All grants and fees go to the Erasmus MC as an institution. She is coordinator of Erasmus MC as a Health Care Provider within the European Reference Network (ERN) for rare hematological diseases EuroBloodNet and (co)leader of the local Erasmus MC Expert Centers for Rare Bleeding Disorders and Sickle Cell and Thalassemia Comprehensive Care Center. R.E.G. Schutgens reports grants from Bayer, Baxalta, Pfizer, and Novo Nordisk outside the submitted work. M. Peters reports a grant from Pfizer outside the submitted work. W.L. van Heerde reports financial support from Takeda, Bayer, Sobi and CSL Behring, and funding from Takeda and Bayer for Enzyre. The remaining authors declare no competing financial interests., (Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: In comparison with the general population, women with bleeding disorders are more prone to develop obstetrical and gynecological problems. However, no comprehensive evaluation has investigated the prevalence of hemorrhagic ovarian cysts (HOCs) in rare bleeding disorders (RBDs). In this study, we sought to determine the prevalence of HOCs in a large cohort of Iranian patients with RBDs., Methods: A total of 210 symptomatic patients suspected of HOCs with RBD were included. The median age of the study population was 24 years. Patients were diagnosed with fibrinogen disorders (n = 7, 3%), factor (F) II (n = 4, 2%), FV (n = 28, 13%), FVII (n = 4, 2%), FX (n = 6, 3%), FXIII (n = 122, 58%), combined FV and FVIII (n = 8, 4%), Glanzmann's thrombasthenia (n = 10, 5%), and von Willebrand disease (VWD) type 3 (n = 21, 10%)., Results: Following further clinical and ultrasound examinations of these 210 patients, 68 (32.4%) were confirmed with a diagnosis of HOCs. Of which, FXIII deficiency with 46 cases (67.6%), followed by VWD type 3 (6 cases, 8.8%) showed the highest number. Other coagulation defects associated with HOCs were including fibrinogen deficiency (n = 2, 3%), FII (n = 2, 3%), FV (n = 4, 6%), FVII (n = 2, 3%), FX (n = 1, 1.5%), combined FV and FVIII (n = 2, 3%), and Glanzmann's thrombasthenia (n = 3, 4.5%)., Conclusion: This study found a high prevalence of HOCs in patients with RBDs, indicating the importance of early diagnosis and optimal management of obstetric and gynecological complications in these patients., Competing Interests: Conflict-of-interest disclosure The authors declare no conflicts of interest relevant to this manuscript., (Copyright © 2023. Published by Elsevier Ltd.)

Although rare bleeding disorders (RBDs) are not common diseases, they are important for life-threatening bleedings and prophylaxis approaches, especially in severe forms. In this retrospective study, the authors have analyzed data from children with severe RBDs who were examined at the center over a period of 10 years to describe the distribution, clinical features, treatment patterns, and outcome of severe RBDs in patients. Data from all children (age under 18 y) with RBDs who were examined in the center between 2005 and 2015 were retrospectively reviewed. In total, 12 patients were included in the study. Four of the cases had factor (F) VII (33.3%), 6 had FX (50%), 1 had FXIII (8.3%), and 1 had fibrinogen deficiency (8.3%). Of the 12 children with severe RBDs, 8 (67%) experienced at least 1 major bleeding. Prophylaxis was applied to 10 patients. In conclusion, RBDs are more common in our country because of the high parental consanguinity rates. So, it is necessary to raise public awareness about the risks of consanguineous marriages and increase access to genetic counseling and testing facilities. Delayed diagnosis and lack of adequate prophylactic replacement therapy are the most important risk factors that increase life-threatening bleeding., Competing Interests: The authors declare no conflict of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Hemorrhagic disease (HD) is considered one of the most significant infectious diseases of white-tailed deer in North America. Investigations into environmental conditions associated with outbreaks suggest drought conditions are strongly correlated with outbreaks in some regions of the United States. However, during 2017, an HD outbreak occurred in the Eastern United States which appeared to be associated with a specific physiographic region, the Appalachian Plateau, and not drought conditions. The objective of this study was to determine if reported HD in white-tailed deer in 2017 was correlated with physiographic region. There were 456 reports of HD from 1605 counties across 26 states and 12 physiographic regions. Of the 93 HD reports confirmed by virus isolation, 76.3% (71/93) were identified as EHDV-2 and 66.2% (47/71) were from the Appalachian Plateau. A report of HD was 4.4 times more likely to occur in the Appalachian Plateau than not in 2017. Autologistic regression models suggested a statistically significant spatial dependence. The underlying factors explaining this correlation are unknown, but may be related to a variety of host, vector, or environmental factors. This unique outbreak and its implications for HD epidemiology highlight the importance for increased surveillance and reporting efforts in the future.

Introduction: With the advent of direct-to-consumer genetic testing, mild factor XI deficiency is increasingly recognized. There are limited data regarding the risk of postpartum haemorrhage (PPH) among women with mild FXI deficiency following obstetrical delivery., Aim: To assess the risk of PPH among women with mild FXI deficiency undergoing vaginal or caesarean delivery., Methods: We conducted a retrospective, case-control study, in women with FXI levels between 20% and 70% of normal. For a control population, delivery outcomes were analysed in 200 women (between 2016 and 2018) without known bleeding disorders., Results: There was no PPH among 45 vaginal deliveries in women with mild FXI deficiency compared with one PPH among 125 vaginal deliveries in the control cohort. The rate of PPH was significantly higher among the 26 caesarean deliveries in women with mild FXI deficiency relative to 75 control caesarean deliveries (odds ratio 2.73, 95% CI 1.02-7.26, P = .04). Prior history of haemorrhage was a strong predictor of PPH following caesarean delivery. All women who developed PPH following caesarean delivery had either a history of haemorrhage or independent risk factor for PPH., Conclusion: Due to the low rates of postpartum haemorrhage following vaginal delivery, routine prophylaxis to prevent postpartum haemorrhage in the setting of mild FXI deficiency does not appear warranted, especially in the absence of a bleeding history. Mild FXI deficiency is associated with an increased risk of PPH following caesarean delivery., (© 2020 John Wiley & Sons Ltd.)

Background: Long-term outcomes in patients at high bleeding risk (HBR) undergoing percutaneous coronary intervention with a drug-eluting stent are unclear. Therefore, we aimed to evaluate long-term adverse events in HBR patients undergoing percutaneous coronary intervention with cobalt-chromium everolimus-eluting stent implantation., Methods: We analyzed stratified data from 4 all-comers postapproval registries. Patients with at least 1 of the following criteria were categorized as HBR: age ≥75 years, history of major bleeding (MB), history of stroke, chronic oral anticoagulant use, chronic kidney disease, anemia, or thrombocytopenia. Additionally, in a separate analysis, patients were categorized according to the recently published Academic Research Consortium HBR criteria. The Kaplan-Meier method was used for time-to-event analyses. Coronary thrombotic events (CTE) included myocardial infarction or definite/probable stent thrombosis. MB was defined according to the TIMI (Thrombolysis in Myocardial Infarction) or GUSTO (Global Use of Strategies to Open Occluded Coronary Arteries) scales. Impact of CTE and MB on subsequent risk of mortality was assessed using multivariable Cox regression with MB and CTE included as time-updated covariates., Results: Of the total 10 502 patients included, 3507 (33%) were identified as HBR. Compared with non-HBR patients, those at HBR had more comorbidities, higher lesion complexity, and a higher risk of 4-year mortality (Hazard Ratio [HR] 4.38 [95% CI, 3.76-5.11]). Results were qualitatively similar when using Academic Research Consortium criteria to define HBR. Risk of mortality was increased after CTE (HR 5.02 [95% CI, 3.93-6.41]), as well as after MB (HR 4.92 [95% CI, 3.82-6.35]). Of note, this effect was consistent across the spectrum of bleeding risk ( P -interaction test 0.97 and 0.06, respectively)., Conclusions: Compared with the non-HBR population, HBR patients experienced worse 4-year outcomes after percutaneous coronary intervention with cobalt-chromium everolimus-eluting stent. Both CTE and MB had a significant impact on subsequent risk of mortality irrespective of bleeding risk.

Multiple myeloma (MM) is a tumor of plasma cells representing approximately 1% of all canine tumors. Clinical evident bleeding is often referred to as the main finding. The aim of the study was to evaluate the occurrence of clinical bleedings in dogs with MM and its prognostic implications compared to a population of dogs not affected by MM. Two groups of dogs (# 78 each) individually matched for breed, age and gender were considered. Group-1 (exposed) was affected by MM and group-2 (unexposed) was affected by other diseases. They were compared for bleeding and mortality at 90 days after diagnosis (relative risk, RR; attributable risk, AR). Among group-1, bleeding patients (B) were compared with non-bleeding patients (NB) in terms of mortality at 90 days (RR, AR). Incident cases of MM were 78/57,694 (0.13%). Signs of bleeding up to 30 days before the referral presentation were found in 33 (42.3%) group-1 dogs in comparison to 6 (7.7%) group-2 dogs (RR, 5.50, CI 95% 2.55-12.3, p = 0.0001; AR, 0.34, CI 95% 0.22-0.47, p = 0.0001). Epistaxis was the most frequent sign of bleeding recorded. Nineteen dogs from group-1 (24.3%) and eight from group-2 (10.2%) were non-survivors (RR = 2.37, CI 95% 1.14-5.06, p = 0.01; AR = 0.14, CI 95% 0.02-0.26, p = 0.01). Among the group-1, the B dogs, 4/33 (12.1%) were non-survivors, while 15/45 NB dogs (33.3%) were non-survivors (RR = 2.75, CI 95% 1.08-7.44, p = 0.03; AR = 0.21, CI 95% 0.20-0.38, p = 0.03). Epistaxis at diagnosis was frequent in MM dogs, and signs of bleeding were associated with a more favorable 90-day prognosis., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Objective: To develop and validate a risk-scoring model for predicting recurrent hypertensive cerebral hemorrhage (RHCH) occurring within 1 year after initial hypertensive cerebral hemorrhage and to facilitate preemptive clinical intervention for the prevention of secondary hemorrhage., Methods: Patient gender, age, blood pressure, Glasgow Coma Scale (GCS) score, location of cerebral hemorrhage, surgery, past medical history, blood biochemical parameters, and Glasgow Outcome Scale score were analyzed using logistic regression analysis to determine independent predictors of RHCH. A risk-scoring model was constructed by assigning coefficients to each predictor and validating it in another independent cohort. The accuracy of the model was then assessed by the area under the receiver operating characteristic curve (AUC), and the calibration ability of the model was assessed by the Hosmer-Lemeshow test., Results: Of 520 patients in the derivation cohort, 38 developed RHCH within 1 year after discharge. Independent risk factors of RHCH were age >60 years; stage 3 hypertension at admission; GCS score 9-12 (admission); GCS score 3-8 (discharge); history of cerebral ischemic stroke, smoking, alcoholism; and plasma homocysteine (Hcy) level ≥10 μmol/L. The recurrence rates for the low-risk (0-13 points), intermediate-risk (14-26 points), and high-risk (27-39 points) groups were 1.73%, 6.11%, and 57.14%, respectively (P < 0.001). The corresponding rates in the validation cohort, of whom 10/107 (9.35%) developed RHCH, were 3.45%, 7.14%, and 71.43%, respectively (P < 0.001). The risk-scoring model showed good discrimination in both the derivation and validation cohorts, with an AUC of 0.802 versus 0.863. The model also showed good calibration ability (the Hosmer-Lemeshow P values of the two cohorts were 0.532 vs. 0.724)., Conclusions: This model will help identify high-risk groups for RHCH in order to facilitate and improve preemptive clinical intervention., (Copyright © 2019. Published by Elsevier Inc.)

Introduction: Historically, issues faced by women with bleeding disorders (WBD) have been underestimated. While advances in genetic testing have resulted in improvements, significant challenges remain in the initial recognition of abnormal bleeding and referral of WBD., Methods: The European Haemophilia Consortium (EHC) developed a questionnaire for WBD to provide insights into the barriers and challenges faced by WBD in Europe., Results: In total, 709 WBD responded to the survey from 32 countries, predominantly from western European countries (94%). A delay in ascertaining the diagnosis of a congenital bleeding disorders (CBD) remains, with a median age at diagnosis of 16 years. The presence of family history is strongly associated with a lower median age at diagnosis of 6 years. WBD reported significant disease impact on their day-to-day life, most evident for the rarer CBD. The bleeding symptom of biggest impact on daily life is heavy menstrual bleeding (HMB), reported by 55% of women. Importantly, 25% of WBD reports that their condition severely impacted their decision to have or has prevented them from having children. Respondents registered with Haemophilia Treatment Centres (HTC) are 2.2 times more likely to receive treatment compared to WBD in other hospital services., Conclusion: Improved education for both patients and healthcare providers is essential to improve time to diagnosis, access to treatment and psychosocial supports for WBD in Europe., (© 2019 John Wiley & Sons Ltd.)

Study Objective: Heavy menstrual bleeding (HMB) is a common gynecological complaint among young women with up to 40% having experienced HMB. Bleeding disorders are increasingly being recognized in adolescents and young adults with HMB. The aim of this study was to determine the prevalence of bleeding disorders in adolescents with HMB, among patients who presented to the Queensland Statewide Paediatric and Adolescent Gynaecology Service between July 2007 and July 2017. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: The study was a retrospective review of 124 female adolescents aged 8 to 18 years with HMB who presented to the Queensland Paediatric and Adolescent Gynaecology Service, Brisbane, Australia. The primary outcome measure was diagnosis of a bleeding disorder, with secondary outcomes including iron deficiency and/or anemia and treatment modalities., Results: Screening for bleeding disorders was performed in 77/124 (62.1%) of patients with HMB. Twenty-seven adolescents were diagnosed with a bleeding disorder, giving a prevalence of 27/124 (21.7%) in those with HMB, and 27/77 (35%) with HMB who were screened. Of these 35%, von Willebrand disease was the most common bleeding disorder, found in 14/27 (51.6%), followed by inherited platelet function disorders diagnosed in 9/27 (33.3%), thrombocytopenia (inherited or acquired) in 3/27 (11.1%), and Factor IX deficiency in 1/27 (3.7%). Iron deficiency and/or anemia was diagnosed in 53/107 (49.5%) of patients with HMB who were screened for this, and 19/27 (70.3%) of those diagnosed with a bleeding disorder., Conclusion: Adolescents with HMB who present to a tertiary pediatric and adolescent gynecology service should be screened for bleeding disorders, because of the considerably high prevalence in this at-risk population., (Crown Copyright © 2018. Published by Elsevier Inc. All rights reserved.)

Objective: To describe the implantation of a care quality indicator associated to the nursing diagnosis of patients at high risk of bleeding, based on the alarming results of prothrombin time (PT), partially activated thromboplastin time (aPTT) and platelets., Methods: Retrospective experience report of multidisciplinary actions developed in a university hospital. The stages of the study involved team meetings, search for effective communication strategies and creation of a new indicator of quality of care., Results: The indicator was called "Compliance of Nursing Diagnosis Risk for bleeding", monitored monthly since June 2016. The technical file includes the characteristics and attributes of the indicator. Based on the analyzes of the indicator, action plans are established for its qualification., Conclusion: The implementation of the quality of care indicator associated to the nursing diagnosis improved the communication process, the monitoring and the nursing care to patients at risk of bleeding.

Platelet count represents a useful tool in clinical practice to discriminate individuals at higher risk of bleeding. Less obvious is the role of platelet count variability within the normal range of distribution in shaping the individual's disease risk profile. Epidemiological studies have shown that platelet count in the adult general population is associated with a number of health outcomes related to hemostasis and thrombosis. However, recent studies are suggesting a possible role of this platelet index also as an independent risk factor. In this review of adult population studies, we will first focus on known genetic and non-genetic determinants of platelet number variability. Next, we will evaluate platelet count as a marker and/or a predictor of disease risk and its interaction with other risk factors. We will then discuss the role of platelet count variability within the normal distribution range as a contribution to disease and mortality risk. The possibility of considering platelet count as a simple, inexpensive indicator of increased risk of disease and death in general populations could open new opportunities to investigate novel platelet pathophysiological roles as well as therapeutic opportunities. Future studies should also consider platelet count, not only platelet function, as a modulator of disease and mortality risk., (© 2018 International Society on Thrombosis and Haemostasis.)

The impact of inherited bleeding disorders on the socioeconomic status (SES) of affected individuals is not clear. The SES of adult patients with congenital bleeding disorders (PWBD) from a centre in Germany (age 42.3 ± 15.0 years) was compared to that of a gender- and age-matched control group of patients with thrombophilia or a thrombotic event (PWT). Patients completed a questionnaire including aspects of SES, impact of the disease on their lives, and health-related quality of life (HRQoL). Forty-five patients were enrolled in each group; 71 % of PBWD had a severe form of the bleeding disorder (FVIII/IX activity < 1 % or VWD type 3), and 60 % of all PWBD were treated on-demand. PWBD had a lower monthly income ( p  = 0.029) and a worse occupational status ( p  = 0.047) than PWT, but there was no difference regarding the project-specific SES index. PWBD also reported a worse HRQoL in the physical summary component score of the SF-36 ( p  < 0.001). More PWBD (69.8 %) reported a high impact of the disease on their lives than PWT (33.3 %, p  < 0.001). In summary, PWBD had a worse occupational status, monthly income, health behaviour, HRQoL, and impact of the disease on their lives compared to PWT, but not a significantly different SES in general., Competing Interests: Disclosure The authors report no conflicts of interest in this work., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: The treatment of bleeding disorders improved in the last decades. However, the effect of growing up with bleeding disorders on developmental, emotional, and social aspects is understudied. Therefore, this study assesses HRQOL, developmental milestones, and self-esteem in Dutch young adults (YA) with bleeding disorders compared to peers., Methods: Ninety-five YA (18-30 years) with bleeding disorders (78 men; mean 24.7 years, SD 3.5) and 17 women (mean 25.1 years, SD 3.8) participated and completed the Pediatric Quality of Life Inventory Young Adult version, the Course of Life Questionnaire, and the Rosenberg Self-Esteem Scale. Differences between patients with bleeding disorders and their peers, and between hemophilia severity groups, were tested using Mann-Whitney U tests., Results: YA men with bleeding disorders report a slightly lower HRQOL on the total scale, physical functioning, and school/work functioning in comparison to healthy peers (small effect sizes). YA men with severe hemophilia report more problems on the physical functioning scale than non-severe hemophilia. YA men with bleeding disorders achieved more psychosexual developmental milestones than peers, but show a delay in 'paid jobs, during middle and/or high school.' A somewhat lower self-esteem was found in YA men with bleeding disorders in comparison to peers (small effect size). For YA women with bleeding disorders, no differences were found on any of the outcomes in comparison to peers., Conclusion: This study demonstrates some impairments in HRQOL and self-esteem in YA men with bleeding disorders. By monitoring HRQOL, problems can be identified early, especially with regard to their physical and professional/school functioning.

Haemorrhagic diathesis (HD) in cattle is a relatively rare syndrome that can have many different causes. With the occurrence of bovine neonatal pancytopenia (BNP) in 2007, the number of cases of HD in cattle has increased. This led to an enhanced interest in diseases presenting with bleeding disorders. The possible causes of HD in cattle, the clinical findings, and the course of various diseases are described and evaluated. Furthermore, we determined whether cases of BNP occurred before the introduction of the vaccine Pregsure BVD since its widespread use was associated with the syndrome. Records of 215 cases of HD in cattle that had been referred to the Clinic for Ruminants with Ambulatory and Herd Health Services at the Centre for Clinical Veterinary Medicine, Ludwig Maximilian University, Munich, between 1982 and 2014 were evaluated. The two most commonly diagnosed diseases were BNP (n = 95) and septicaemia (n = 35), with fatality rates of 82% and 66%, respectively. In 27 (13%) cases, no clear cause for the HD could be designated. Statistically significant differences were found with regard to the course of the various disorders and the clinical findings. A receiver operating characteristic analysis of thrombocyte counts of affected animals at the time of arrival at the clinic did not provide any predictive information on disease outcome. Two cases of HD occurred before the introduction of Pregsure BVD (1989, 1991). In both cases, clinical, haematological, and pathological findings were identical to BNP. The cause of HD in these two cases could not be determined retrospectively., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

More than 100 young captive and wild Asian elephants are known to have died from a rapid-onset, acute hemorrhagic disease caused primarily by multiple distinct strains of two closely related chimeric variants of a novel herpesvirus species designated elephant endotheliotropic herpesvirus (EEHV1A and EEHV1B). These and two other species of Probosciviruses (EEHV4 and EEHV5) are evidently ancient and likely nearly ubiquitous asymptomatic infections of adult Asian elephants worldwide that are occasionally shed in trunk wash secretions. Although only a handful of similar cases have been observed in African elephants, they also have proved to harbor their own multiple and distinct species of Probosciviruses-EEHV2, EEHV3, EEHV6, and EEHV7-found in lung and skin nodules or saliva. For reasons that are not yet understood, approximately 20% of Asian elephant calves appear to be susceptible to the disease when primary infections are not controlled by normal innate cellular and humoral immune responses. Sensitive specific polymerase chain reaction (PCR) DNA blood tests have been developed, routine monitoring has been established, the complete large DNA genomes of each of the four Asian EEHV species have now been sequenced, and PCR gene subtyping has provided unambiguous evidence that this is a sporadic rather than epidemic disease that it is not being spread among zoos or other elephant housing facilities. Nevertheless, researchers have not yet been able to propagate EEHV in cell culture, determine whether or not human antiherpesvirus drugs are effective inhibitors, or develop serology assays that can distinguish between antibodies against the multiple different EEHV species., (© The Author 2016. Published by Oxford University Press on behalf of the Institute for Laboratory Animal Research. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Background: Some studies have found that egg consumption is associated with a higher risk of ischemic heart disease in patients with diabetes. Epidemiologic studies of egg consumption in relation to risk of heart failure (HF) and stroke types are scarce., Objective: The aim of this study was to examine whether egg consumption is associated with incidence of HF, myocardial infarction (MI), or stroke types., Design: In prospective cohorts of 37,766 men (Cohort of Swedish Men) and 32,805 women (Swedish Mammography Cohort) who were free of cardiovascular disease (CVD), egg consumption was assessed at baseline with a food-frequency questionnaire. Incident CVD cases were identified through linkage with the Swedish National Patient and Cause of Death Registers. The data were analyzed with the use of a Cox proportional hazards regression model., Results: During 13 y of follow-up, we ascertained 1628 HFs, 3262 MIs, 2039 ischemic strokes, and 405 hemorrhagic strokes in men and 1207 HFs, 1504 MIs, 1561 ischemic strokes, and 294 hemorrhagic strokes in women. There was no statistically significant association between egg consumption and risk of MI or any stroke type in either men or women or HF in women. In men, consumption of ≤6 eggs/wk was not associated with HF risk; however, daily egg consumption (≥1/d) was associated with a 30% higher risk of HF (RR: 1.30; 95% CI: 1.01, 1.67). Egg consumption was not associated with any CVD outcome in individuals with diabetes., Conclusions: Daily egg consumption was not associated with risk of MI or any stroke type in either men or women or with HF in women. Consumption of eggs ≥1 time/d, but not less frequent consumption, was associated with an elevated risk of HF in men., (© 2015 American Society for Nutrition.)

Until 1990, congenital hemorrhagic disorders were treated by plasma-derived concentrates. The first recombinant drug, recombinant factor VIII was available after this date and few years later recombinant factor IX could also be proposed to patients. The evolution of market share in France was different between these two drugs: while recombinant factor VIII took a large place in hemophilia A treatment (85%), plasma-derived factor IX represent 50% of the French market. In the next years, the arrival of long-acting antihemophilic factors may lead to the dramatically reduce the amount of plasma-derived antihemophilic factors used to treat hemophilia. For rare bleeding coagulation disorders, plasma-derived concentrates are still widely used, while they are the only concentrates available in most diseases. This situation is unlikely to evolve significantly in the next years., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Objective: To evaluate the perinatal results from epileptic women using antiepileptic drugs during prenatal care., Methods: This was a retrospective longitudinal study assessing the perinatal results of women exposed to antiepileptic drugs during pregnancy, and we compared these results with those of pregnant women who were not exposed. The development of pregnancy, gestational age at delivery, Apgar scores, biometric data, morbidity, stillbirths and neonatal mortality were analyzed. The chi-square test and Fisher's exact test were used for the categorical variables, while Student's t-test was used for independent numerical variables., Results: Over a 10-year period, 12 790 singleton gestations were analyzed, among which 104 (0.8%) consisted of epileptic pregnant women. From this total, 82 evolved to childbirth and their neonatal data were compared with those of 316 newborns from non-epileptic women. The most-used antiepileptic drug was phenobarbital in 70% of the cases. There was greater neonatal mortality (p = 0.006), occurrence of neonatal hemorrhagic disorders (p = 0.005), and occurrence of minor congenital anomalies (p = 0.03) among the children of women exposed to antiepileptic drugs., Conclusion: The antenatal exposure to antiepileptic drugs is associated mainly with occurrences of hemorrhagic complications during the neonatal period; furthermore, great prevalence of newborns presenting minor congenital anomalies and elevated risk of neonatal mortality.

Background: Dietary cholesterol has been suggested to increase the risk of cardiovascular disease (CVD), which has led to US recommendations to reduce cholesterol intake., Objective: The authors examine the effects of dietary cholesterol on CVD risk in healthy adults by using systematic review and meta-analysis., Design: MEDLINE, Cochrane Central, and Commonwealth Agricultural Bureau Abstracts databases were searched through December 2013 for prospective studies that quantified dietary cholesterol. Investigators independently screened citations and verified extracted data on study and participant characteristics, outcomes, and quality. Random-effect models meta-analysis was used when at least 3 studies reported the same CVD outcome., Results: Forty studies (17 cohorts in 19 publications with 361,923 subjects and 19 trials in 21 publications with 632 subjects) published between 1979 and 2013 were eligible for review. Dietary cholesterol was not statistically significantly associated with any coronary artery disease (4 cohorts; no summary RR), ischemic stroke (4 cohorts; summary RR: 1.13; 95% CI: 0.99, 1.28), or hemorrhagic stroke (3 cohorts; summary RR: 1.09; 95% CI: 0.79, 1.50). Dietary cholesterol statistically significantly increased both serum total cholesterol (17 trials; net change: 11.2 mg/dL; 95% CI: 6.4, 15.9) and low-density lipoprotein (LDL) cholesterol (14 trials; net change: 6.7 mg/dL; 95% CI: 1.7, 11.7 mg/dL). Increases in LDL cholesterol were no longer statistically significant when intervention doses exceeded 900 mg/d. Dietary cholesterol also statistically significantly increased serum high-density lipoprotein cholesterol (13 trials; net change: 3.2 mg/dL; 95% CI: 0.9, 9.7 mg/dL) and the LDL to high-density lipoprotein ratio (5 trials; net change: 0.2; 95% CI: 0.0, 0.3). Dietary cholesterol did not statistically significantly change serum triglycerides or very-low-density lipoprotein concentrations., Conclusion: Reviewed studies were heterogeneous and lacked the methodologic rigor to draw any conclusions regarding the effects of dietary cholesterol on CVD risk. Carefully adjusted and well-conducted cohort studies would be useful to identify the relative effects of dietary cholesterol on CVD risk., (© 2015 American Society for Nutrition.)

Purpose: High glycemic load (GL) has been associated with excess stroke risk. Data suggest a different role of diet in the etiology of ischemic and hemorrhagic stroke., Methods: We analyzed data from 19,824 participants of the Greek cohort of the population-based European Prospective Investigation into Cancer and nutrition (EPIC), who were free of cardiovascular diseases, cancer, and diabetes at baseline and had not developed diabetes. Diet was assessed at enrollment through a validated, interviewer-administered semi-quantitative food frequency questionnaire. The average daily GL was derived using standard tables. We also conducted a meta-analysis on GL and stroke (overall, ischemic and hemorrhagic), using random-effects models., Results: In the Greek EPIC cohort, 304 incident stroke cases were identified (67 ischemic, 49 hemorrhagic). Using Cox proportional hazards regression models adjusted for potential confounders, the hazard ratios for the highest versus the lowest GL tertiles were 1.07 [95 % confidence interval (CI) 0.74-1.54] for overall stroke, 1.55 (95 % CI 0.72-3.36) for ischemic and 0.48 (95 % CI 0.18-1.25) for hemorrhagic stroke (p-heterogeneity <0.01). The meta-analysis, including a total of 3,088 incident cases and 247 deaths from stroke (1,469 cases and 126 deaths ischemic; 576 cases and 94 deaths hemorrhagic), estimated pooled relative risks for the highest versus the lowest GL levels of 1.23 (95 % CI 1.07-1.41) for overall, 1.35 (95 % CI 1.06-1.72) for ischemic, and 1.09 (95 % CI 0.81-1.47) for hemorrhagic stroke (p-heterogeneity = 0.275)., Conclusions: This study indicates that GL is an important determinant of the more common ischemic-though not of the hemorrhagic-stroke.

Background: Liver transplantation regularly requires transfusion of red blood cells (RBCs), plasma, and platelets. Compared to fresh frozen plasma (FFP) from single blood donors, solvent/detergent-treated plasma (SD-plasma) pooled from several hundred blood donors has advantages with respect to pathogen reduction, standardized content of plasma proteins, and significantly reduced risk of transfusion related lung injury and allergic/immunologic adverse reactions. However, SD-plasma has been suspected to increase the incidence of hyperfibrinolysis and thromboembolic events., Study Design and Methods: We investigated the transfusion practices, hyperfibrinolysis parameters, and thrombosis outcomes in 195 consecutive adult primary liver transplants in our center using SD-plasma (Octaplas) as the exclusive source of plasma., Results: Perioperatively, median (interquartile range) 4 (1 to 9) RBC-units, 10 (4 to 18) plasma-bags, and 0 (0 to 2) platelet-units were transfused. Hyperfibrinolysis defined as LY30 ≤ 7.5% was detected in 12/138 thrombelastography-monitored patients (9%). These patients received significantly more RBCs, plasma, and platelets than did patients without hyperfibrinolysis. Thrombotic graft complications were observed in three patients (2%). Pulmonary embolism was not observed in any patient., Conclusion: SD-plasma is a safe plasma product for liver transplant recipients, and the incidences of hyperfibrinolysis and thromboembolic events are not significantly different from those seen in centers using FFP., (© 2014 Marshfield Clinic.)

Purpose: Laparoscopic surgery (LS) is gaining popularity worldwide because of benefits like faster recovery, earlier hospital discharge, and better cosmetic results. In hemophiliacs, surgery in general harbors an increased risk for severe complications. Whether LS or conventional surgery (CS) should be recommended in these patients is controversial and therefore the issue of our present study., Methods: We performed a retrospective matched-pair analysis including laparoscopically operated non-hemophiliacs (LONH), laparoscopically operated hemophiliacs (LOH), and conventionally operated hemophiliacs (COH) concerning duration of surgery, drainages, hospital stay, complications, factor use (VIII, IX, and X), and blood values. Mann-Whitney U test was used (significance level P = 0.05)., Results: No significant differences were found in duration of surgery and drains in laparoscopically or conventionally operated hemophiliacs versus matched pairs. Complication rate did not differ among the different groups. Concerning the total duration of hospital stay (t-DHOS) and the postoperative duration of hospital stay (p-DHOS), there was no statistical difference between LOH versus matched LONH. However, in COH versus matched LOH, a longer time was required for preparation and recovery (t-DHOS, P = 0.04; p-DHOS, P < 0.001). Also, the median factor supply perioperatively including the day of surgery did not differ between laparoscopically versus conventionally operated hemophiliacs., Conclusions: Our study underscores the safety and benefits of laparoscopic procedures in hemophiliacs by showing a significantly shorter hospital stay for these patients resulting in reduced therapeutic costs and a faster mobilization. Still, the surgical and perioperative management of hemophiliacs continues to be a challenge requiring an experienced interdisciplinary team.

To promote management and awareness of bleeding disorders in Lebanon, a pilot programme was launched in 2009 by the Lebanese Hemophilia Association assisted by World Federation of Hemophilia. The aim of this study was to diagnose patients with bleeding disorders and to assess the potential challenges in implementing a screening programme. The pilot project was launched in 26 social health centres in the Bekaa valley. The study tools included the evaluation of the Tossetto Bleeding Score and the Pictorial Bleeding Assessment Chart (PBAC) for menstruation. Persons with a bleeding score higher than 2 and PBAC higher than 185 were eligible for further blood tests including the prothrombin time, partial thromboplastin time, complete blood count, bleeding time and von Willebrand ristocetin cofactor activity. 643 patients were enrolled, of whom 60.6% were women. Overall, 91 persons had an abnormal score. 50 eligible patients were tested: 32 had normal tests, nine new patients with severe Von Willebrand were discovered, 4 had VW:RiCo of 40, 3 prolonged APTT and 2 thrombocytopaenia. There was a clear correlation between the severity of the score and the willingness to perform the tests (P = 0.02). Women were reluctant to participate fully when investigators were men. The probability of adherence to the screening protocol is significantly increased when directed by women health care professional. For patients with milder forms, global screening programmes were neither feasible nor acceptable but those more severely affected have to be identified. Providers are crucial in preselecting patients with blood problems who are not coping well., (© 2013 John Wiley & Sons Ltd.)

This study was undertaken to determine whether there was a correlation between fine particle (PM2.5) levels and hospital admissions for hemorrhagic stroke (HS) in Taipei, Taiwan. Hospital admissions for HS and ambient air pollution data for Taipei were obtained for the period 2006-2010. The relative risk of hospital admissions was estimated using a case-crossover approach, controlling for weather variables, day of the week, seasonality, and long-term time trends. For the single-pollutant model (without adjustment for other pollutants), increased HS admissions were significantly associated with PM2.5 levels both on warm days (>23°C) and cool days (<23°C), with an interquartile range rise associated with a 12% (95% CI = 7-18%) and 4% (95% CI = 0-8%) elevation in admissions for HS, respectively. In the two-pollutant models, PM2.5 remained significantly high after inclusion of SO2 or O3 on both warm and cool days. This study provides evidence that higher levels of PM2.5 increase the risk of hospital admissions for HS.

Bleeding symptoms are common in healthy children but occasionally may indicate an underlying congenital or acquired bleeding diathesis. The rare bleeding disorders (RBDs) comprise inherited deficiencies of coagulation factors I (congenital fibrinogen deficiencies), II, V, VII, X, XI, and XIII and combined factor deficiencies, most notably of factors V and VIII and of vitamin K-dependent factors. These disorders often manifest during childhood and may present with recurrent or even serious or life-threatening bleeding episodes, particularly during the neonatal period. Accordingly, primary care and other nonhematologist pediatric providers should be familiar with the clinical presentation and initial evaluation of these rare disorders. Bleeding manifestations generally vary within the same RBD and may be indistinguishable from 1 RBD to another or from other more common bleeding disorders. Serious bleeding events such as intracranial hemorrhage may be heralded by less serious bleeding symptoms. The results of initial coagulation studies, especially prothrombin time and activated partial thromboplastin time, are often helpful in narrowing down the potential factor deficiency, with factor XIII deficiency being an exception. Consultation with a hematologist is advised to facilitate accurate diagnosis and to ensure proper management and follow-up. The approach to bleeding episodes and invasive procedures is individualized and depends on the severity, frequency, and, in the case of procedures, likelihood of bleeding. Prophylaxis may be appropriate in children with recurrent serious bleeding and specifically after life-threatening bleeding episodes. When available, specific purified plasma-derived or recombinant factor concentrates, rather than fresh frozen plasma or cryoprecipitate, are the treatment of choice.

Objective: The purpose of this study was to assess the prevalence of underlying bleeding disorders in women with heavy menstrual bleeding (HMB) with and without gynecologic abnormalities., Study Design: We performed a single-center prospective cohort study of 112 consecutive patients who were referred for heavy menstrual bleeding. Control subjects were 28 healthy volunteers who reported no HMB. Patients and control subjects had hemostatic testing in the first week after menstruation. Patients underwent gynecologic evaluation., Results: The median age was 42.5 years (range, 17-55 years) in patients and 40.0 years (range, 25-55 years) in control subjects. Forty-six percent of patients had anemia; the median pictorial bleeding assessment chart score was 271. Seven percent of the control subjects with a subjectively normal menstruation had anemia. Twenty-six percent of patients had gynecologic abnormalities, which was considered to explain HMB. Overall, we found an underlying bleeding disorder in 29% of the patients, which was comparable for unexplained and explained HMB (31% vs 27%; P = .75). We diagnosed 6 cases of Von Willebrand's disease, 4 cases of factor XI deficiency, and 1 case of factor VII deficiency. The only abnormalities that we found in control subjects were platelet aggregation defects (11% in control subjects vs 23% in patients). Patients had a significantly longer activated partial thromboplastin time compared with control subjects (26.5 vs 25.0 seconds; P = .001) that was caused by lower median levels of factor XI (100 vs 124 IU/dL; P < .001)., Conclusion: Bleeding disorders play an equally important role in the cause of both unexplained and explained heavy menstrual bleeding. A novel finding is the occurrence of low, but not deficient, levels of factor XI., (Copyright © 2013 Mosby, Inc. All rights reserved.)

Late-onset hemorrhagic cystitis (LOHC) is a common complication following allogeneic hematopoietic stem cell transplantation (HSCT), but its cause remains obscure. More attention to risk factors for LOHC is needed. We retrospectively analyzed patients with advanced leukemia who had been treated with ATG-containing conditioning regimens to evaluate the influence of different doses of ATG on LOHC after haploidentical HSCT. Seventy-five patients undergoing haploidentical HSCT from January 2003 to February 2011 were evaluated. A total of 39 patients receiving transplantation before June 2008 were treated with high-dose ATG (10 mg/kg), whereas 36 patients received low-dose ATG (6 mg/kg) thereafter. LOHC occurred in 16.7% of the patients with low-dose ATG, and in 38.5% of the patients with high-dose ATG (P = 0.027). Univariate analysis showed high-dose ATG, male gender and cytomegalovirus reactivation to be significant risk factors for LOHC. However, only high-dose ATG (HR 2.96, 95% CI 1.143-7.663, P = 0.025) and male gender (HR 4.033, 95% CI 1.355-12.008, P = 0.012) were independent risk factors, as shown by multivariate analysis. In the setting of haploidentical HSCT, we concluded that LOHC is more prevalent in recipients of high-dose ATG and male patients. Future studies should focus on immune reconstitution and virus infection after haploidentical HSCT with 6 mg/kg or 10 mg/kg ATG.

Background: Although the mortality from bariatric surgery is low, perioperative determinants of morbidity and mortality in the bariatric surgery population to date have not been fully defined. This study aimed to evaluate the factors capable of predicting perioperative mortality based on preoperative characteristics with a national patient sample., Methods: From the American College of Surgeons National Surgical Quality Improvement Program (ACS-NSQIP) database, all the primary bariatric procedures performed between 2007 and 2009 were identified. Using univariate analysis, factors associated with increased perioperative (30-day) mortality were identified. Logistic regression was used to select correlates of 30-day mortality, which were subsequently integrated into a simplified clinical scoring system based on the number of comorbid risk factors., Results: The study identified 44,408 patients (79 % women, 21 % men) with a mean age of 45 ± 11 years. The cumulative 30-day perioperative mortality rate was 0.14 %. The majority of the procedures performed included laparoscopic gastric bypass (54 %) followed by laparoscopic gastric banding (33 %) and open gastric bypass (7 %). Independent predictors associated with significantly increased mortality included age >45 years [adjusted odds ratio (AOR), 2.45], male gender (AOR = 1.77), a body mass index (BMI) of 50 kg/m(2) or higher (AOR, 2.48), open bariatric procedures (AOR, 2.34), diabetes (AOR, 2.88), functional status of total dependency before surgery (AOR, 27.6), prior coronary intervention (AOR, 2.66), dyspnea at preoperative evaluation (AOR, 4.64), more than 10 % unintentional weight loss in 6 months (AOR, 13.5), and bleeding disorder (AOR, 2.63). Ethnicity, hypertension, alcohol abuse, liver disease, and smoking had no significant association with mortality in this study. Risk stratification based on the number of preoperative comorbid factors showed an exponential increase in mortality as follows: 0-1 comorbidities (0.03 %), 2-3 comorbidities (0.16 %), and 4 comorbidities or more (7.4 %)., Conclusion: This model provides a straightforward, precise, and easily applicable tool for identifying bariatric patients at low, intermediate, and high risk for in-hospital mortality. Notably, baseline functional status before surgery is the single most powerful predictor of perioperative survival and should be incorporated into risk stratification models.

Background: Health care officials and legislators need accurate data on prevalence and numbers of individuals with bleeding disorders in order to plan and allot their budgets; the manufacturers of coagulation factors also need these data to estimate the amount of factors required to prevent scarcity of these products., Materials and Methods: We surveyed the prevalence of haemophilia A, haemophilia B, von Willebrand's disease and rare bleeding disorders in North-Eastern Iran. The survey was done in the period from September 2009 to March 2011. Information was collected from the medical records in three major hospitals and a haemophilia centre; the patients' updated data were obtained by telephone., Results: Overall in the current survey 552 patients with inherited coagulation disorders were identified and their medical records obtained. Of these, 429 (77.5%) had common bleeding disorders (haemophilia A, haemophilia B, von Willebrand's disease), 85 (15.6%) had rare bleeding disorders (deficiency of coagulation factors V, VII, X, XIII, I, XI, combined factor V and VIII deficiency) and 38 (6.9%) had platelet disorders.The commonest bleeding disorders were haemophilia A (n=287, 51.9%), haemophilia B (n=92, 16.6%), von Willebrand's disease (n=50, 9%), factor V deficiency (n=21, 3.8%), factor VII deficiency (n=19, 3.4%), factor X deficiency (n=2, 0.36%), combined factor V and VIII deficiency (n=28, 5.8%), factor XIII deficiency (n=11, 1.99%), factor XI deficiency (n=2, 0.4%), afibrinogenaemia (n=2, 0.36%) and platelet disorders (n=38, 6.9%)., Discussion: There is notable population of individuals with bleeding disorders in North-Eastern Iran.

Background: Hemorrhage is a common and serious symptom in the neonatal period. In developing countries, means of exploration are insufficient. Our purpose was to describe the epidemiological, diagnostic, therapeutic, and evolutionary aspects of hemorrhagic syndromes of the newborn in Dakar, Senegal, to identify the risk factors and diseases associated with them in order to propose recommendations for their management., Methods: We conducted a case-control study of 82 newborns with 41 cases of hemorrhagic syndromes and 41 controls. We first described the epidemiological, diagnostic, therapeutic, and evolutionary parameters of the group that submitted a hemorrhagic syndrome and compared their diagnostic data with those of the control group to highlight the risk factors associated with the occurrence of hemorrhagic syndrome., Results: The prevalence of hemorrhagic syndrome among hospitalized newborns was 9.2%, the sex-ratio (M/F) was 1.9. Preterm infants accounted for 26.8%. We often found early onset of bleeding (46.4% before 72h of life). Visceral bleeding was predominant, especially respiratory (34.1%), digestive (31.7%), and cerebral (17%), followed by cutaneous hemorrhages (26.8%). The risk factors identified were respiratory distress, shock, and a stained amniotic fluid. The concomitant diagnoses were dominated by neonatal infection (58.5%), hemorrhagic disease of the newborn (21.9%), and disseminated intravascular coagulation (19.5%). The mortality rate was high (34.1%), most often associated with disseminated intravascular coagulation., Conclusion: This study reveals the high mortality due to hemorrhagic syndromes caused by these three diseases for which prevention must be stressed., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Thrombotic thrombocytopenic purpura (TTP), immune thrombocytopenia (ITP) and acquired haemophilia are very rare haemorrhagic disorders (1) associated with surgery, hospitalization (2) and death being reported (3). Despite the rapid development of interventional techniques, therapeutic options for patients with these illnesses remain limited. We suppose that depression and anxiety disorders appear more frequently than in the normal population in patients with rare haemorrhagic disorders.

Keeping an updated registry of bleeding disorders is crucial for planning care and documenting prevalence. We aimed to assess the prevalence of various bleeding disorders including rare inherited coagulation and platelet disorders concerning their clinico-epidemiological, diagnostic data and bleeding manifestations severity. Patients suffering from manifestations of bleeding or coagulation disorders presented to Hematology Clinic during 16 years were included and prospectively followed up. Demographics, clinical characteristics, complete blood count, bleeding, prothrombin and activated partial thromboplastin times, platelet aggregation tests and bone marrow aspiration were recorded. Overall 687 patients with bleeding disorders from total 2949 patients were identified. Inherited coagulation defects were found in 27.2%; hemophilia A (70.6%), hemophilia B (13.9%), factor I deficiency (2.3%), factor V deficiency (1.6%), factor X deficiency (4.2%), factor VII deficiency (2.6%), factor XIII deficiency (1.1%), combined factor deficiency (2.1%) and unclassified coagulation disorders in 1.6% of studied patients. Overall 72.7% had diagnosed with platelet disorders; immune thrombocytopenia was the commonest (74.8%), and inherited conditions represent (25.2%) in the following order: Glanzman's thrombasthenia (11.2%), von Willebrand disease (6.6%), Bernard-Soulier syndrome (1%) and Chediak Higashi in 0.4% and unclassified in 6%. Median age of diagnosis of coagulation and platelet disorders were 33 and 72 months. Presenting symptoms of coagulation disorders were: 25.1% post circumcision bleeding, 22.5% ecchymosis, 20.9% hemoarthrosis and 15% epistaxis. Symptoms of rare coagulation disorders were postcircumcision bleeding (20%), bleeding umbilical stump (20%), epistaxis (12%), hemoarthrosis (8%) and hematomas (4%). Presenting symptoms in rare inherited platelet disorders were purpura, ecchymosis, epistaxis and bleeding gums, respectively. Analysis of the clinico-epidemiological data of patients with bleeding disorders is a useful tool for monitoring and improving their quality of care.

Objective: To identify prevalence of previously undiagnosed bleeding disorders in children with severe epistaxis who failed medical therapy requiring intraoperative nasal cautery., Study Design: Retrospective chart review 10/15/2006-12/31/2010., Setting: Single provider outpatient otolaryngology clinic., Inclusion Criteria: children (<19 years) with epistaxis referred to otolaryngology, no known bleeding disorder, failed medical therapy and received surgical nasal cautery., Data Collected: duration/severity of epistaxis, bleeding history, family history of bleeding. A screening CBC, PT and PTT were performed on all patients., Results: Of 248 subjects referred for epistaxis, 47(19%) met inclusion criteria (mean age 9.2±0.5 years; 61.7% male). 31.9% (15/47) had abnormal coagulation studies but on repeat testing only 2 patients had persistent coagulation abnormalities. 15 patients were referred to hematology, 5 were diagnosed with a bleeding disorder (3 - type 1 von Willebrand's disease, 1 - platelet aggregation disorder, 1 - mild factor VII deficiency). Out of the entire cohort 10.6% (5/47) had a bleeding diathesis. Clinical predictive factors for having a bleeding diathesis were explored and included previous emergency room visits for epistaxis (p=0.04). A trend was found in those presenting with epistaxis at a younger age (p=0.07)., Conclusion: Children with recurrent epistaxis despite medical therapy are at higher risk of having a bleeding disorder. In this highly selected group of patients 10.6% (5/47) were found to have a bleeding disorder. Screening coagulation studies (PT, PTT) only revealed 20% (1/5) of patients with a bleeding disorder. Only a subsequent comprehensive hematology evaluation revealed the diagnosis in the majority of patients., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

The objective of the study was to determine the frequency, types, presenting symptoms and management options in patients with bleeding disorders in the local obstetric and gynaecological practice. This was a descriptive study of oneyear duration conducted at the Pakistan Atomic Energy Commission General Hospital, Islamabad. Types of bleeding disorders in decreasing order of frequency were HELLP syndrome in 27.7% (n=5), gestational thrombocytopenia in 22% (n=4), Von Willbrand disease in 16.66% (n=3), Glanzmann's thrombesthenia in 11.11% (n=2), autoimmune haemolytic anaemia in 11.11% (n=2), post-transfusion purpura in 6% (n=1) and factor V deficiency in 6% (n=1). HELLP syndrome and gestational thrombocytopenia were the commonest bleeding disorders in pregnant patients and Von Willebrand disease is the the commonest bleeding disorder in gynaecological patients with menorrhagia.

Background: The emergency department (ED) is an essential component of the medical service offered in any hospital. Yet, the published information about patients' profile and utilization of emergency services in both developing and developed countries is scarce., Aim: This study aimed to characterize the presentation and outcome of patients visiting the ED of a university-based tertiary pediatric hospital., Methods: This is a descriptive study reporting the profile of patients visiting the ED for 1 year., Results: The total number of patients visiting the hospital was 249,552, with a monthly average of 20,046 patients, including 3639 monthly ED visits. "Treat-and-release" cases represented 46.7% and infants represented the largest age group (44.4%). The main presentation was respiratory distress (24.4%) and wheezy chest (17.3%), followed by convulsions (15%) and then pallor (10.7%). Long-term illnesses were predominantly congenital heart diseases, cerebral palsy, chronic hemolytic anemia, and asthma. The overall mortality rate was 0.8%., Conclusions: Appropriate and ongoing data collection and analysis could guide more efficient utilization of pediatric emergency services to achieve better outcomes.

Objective: We aimed to identify the incidence, clinical features, management, and outcome of subdural hematomas (SHs) in infancy and childhood., Methods: Twenty-one children younger than 11 years with SH were analyzed. Clinical features and possible child abuse were considered in each case., Results: Eight children experienced minor injuries due to hitting of solid items on their head. Five of these children also had coagulation disorders. Three of the children suffered from child abuse, only one of the children had head trauma due to car accident. Nine of the patients experienced SH due to fall down. Nine patients have acute SH, 7 had subacute SH, 4 had chronic SH, and 1 had acute and subacute SH together. Clinical presentation varied greatly. Most of them presented with vomiting and seizure. The outcome patterns were different among the patients. Deep coma on admission was associated with an unfavorable outcome., Conclusions: Subdural hematoma is common in infancy and childhood and carries a poor prognosis. Most of the cases are due to head trauma, coagulation disorders, and child abuse. We believe that clinical investigation of such children should be carried out in a multidisciplinary approach with the collaboration of pediatricians, social workers, and neurosurgeons.