Oral health in individuals with bleeding disorders: A systematic review and meta-analysis.

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.

Assessment of rare bleeding disorders in adolescents with heavy menstrual bleeding.

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.

Diagnóstico de enfermagem risco de sangramento como indicador de qualidade assistencial à segurança de pacientes / Diagnóstico de enfermería riesgo de sangrado como indicador de calidad asistencial de la seguridad de pacientes / Nursing diagnosis risk for bleeding as an indicator of quality of care for patient safety

Resumo OBJETIVO Descrever a implantação de um indicador de qualidade assistencial associado ao diagnóstico de enfermagem de pacientes com alto risco de sangramento, com base nos resultados alarmantes de tempo de protombina (TP), tempo de tromboplastina parcialmente ativada (TTPa) e plaquetas. MÉTODOS Relato de experiência retrospectivo de ações multidisciplinares desenvolvidas em um hospital universitário. As etapas do estudo envolveram reuniões de equipes, busca de estratégias de comunicação efetiva e criação de um novo indicador de qualidade assistencial. RESULTADOS O indicador foi denominado "Conformidade do Diagnóstico de Enfermagem Risco de Sangramento", monitorado mensalmente desde junho de 2016. A ficha técnica contempla as características e atributos do indicador. Com base nas suas análises são estabelecidos planos de ações para sua qualificação. CONCLUSÃO A implantação do indicador de qualidade assistencial associado ao diagnóstico de enfermagem aprimorou o processo de comunicação, monitoramento e cuidado de enfermagem a pacientes com risco de sangramento.

Resumen OBJETIVO Describir la implementación de un indicador de calidad asistencial asociado al diagnóstico de enfermería de pacientes con alto riesgo de sangrado, con base en los resultados alarmantes de tiempo de protombina (Tp), tiempo de tromboplastina parcialmente activada (TTPa) y plaquetas. MÉTODO Relato de experiencia retrospectiva de acciones multidisciplinares desarrolladas en un hospital universitario. Las etapas del estudio involucraron reuniones de equipos, búsqueda de estrategias de comunicación efectiva y creación de un nuevo indicador de calidad asistencial. RESULTADOS El indicador se denominó "Conformidad del Diagnóstico de Enfermería Riesgo de Sangrado", y se monitoreó mensualmente desde junio de 2016. La ficha técnica contempla las características y atributos del indicador. Con base en los análisis del indicador se establecen planes de acción para su cualificación. CONCLUSIÓN La implementación del indicador de calidad asistencial asociado al diagnóstico de enfermería mejoró el proceso de comunicación, el monitoreo y el cuidado de enfermería a pacientes con riesgo de sangrado.

Abstract 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.

Ancho de distribución eritrocitaria y predicción adicional del riesgo de hemorragia mayor en el síndrome coronario agudo sin elevación del ST / Red Cell Distribution Width and Additive Risk Prediction for Major Bleeding in Non-ST-segment Elevation Acute Coronary Syndrome

Introducción y objetivos El ancho de distribución eritrocitaria se ha relacionado con incremento del riesgo hemorrágico intrahospitalario en pacientes con síndrome coronario agudo sin elevación del ST. Sin embargo, se desconoce su utilidad para predecir complicaciones hemorrágicas tras el ingreso hospitalario. El objetivo fue evaluar el papel complementario del ancho de distribución eritrocitaria sobre la escala CRUSADE en la predicción del riesgo a largo plazo de hemorragias en estos pacientes. Métodos Se midió el ancho de distribución eritrocitaria al ingreso en 293 pacientes con síndrome coronario agudo sin elevación del ST; a todos se les dio seguimiento clínico y se registró la aparición de hemorragias mayores, definidas según los criterios del Bleeding Academic Research Consortium. Resultados Durante un seguimiento de 782 [intervalo intercuartílico, 510-1.112] días, 30 pacientes (10,2%) presentaron eventos hemorrágicos. El análisis por cuartiles reveló un incremento abrupto de hemorragias a partir del cuarto cuartil (> 14,9%; p = 0,001). Tras el análisis multivariable, el ancho de distribución eritrocitaria >14,9% se asoció con mayor riesgo de eventos (hazard ratio=2,67; intervalo de confianza del 95%, 1,17-6,10; p = 0,02). Los pacientes con valores ≤ 14,9% y CRUSADE ≤ 40 presentaron las menores tasas de hemorragias, mientras que los pacientes con valores >14,9% y CRUSADE >40 puntos (alto y muy alto riesgo) presentaron las mayores (log rank test, p < 0,001). Además, la adición del ancho de distribución eritrocitaria a la escala CRUSADE para predecir hemorragias mostró tasas de mejora integrada del 5,2% (p < 0,001) y de reclasificación del 10% (p = 0,001). CONCLUSIONES: Los valores elevados del ancho de distribución eritrocitaria se asocian a mayor riesgo hemorrágico y aportan información adicional a la escala CRUSADE

Introduction and objectives Red cell distribution width has been linked to an increased risk for in-hospital bleeding in patients with non-ST-segment elevation acute coronary syndrome. However, its usefulness for predicting bleeding complications beyond the hospitalization period remains unknown. Our aim was to evaluate the complementary value of red cell distribution width and the CRUSADE scale to predict long-term bleeding risk in these patients. Methods Red cell distribution width was measured at admission in 293 patients with non-ST-segment elevation acute coronary syndrome. All patients were clinically followed up and major bleeding events were recorded (defined according to Bleeding Academic Research Consortium Definition criteria). Results During a follow-up of 782 days [interquartile range, 510-1112 days], events occurred in 30 (10.2%) patients. Quartile analyses showed an abrupt increase in major bleedings at the fourth red cell distribution width quartile (> 14.9%; P = .001). After multivariate adjustment, red cell distribution width >14.9% was associated with higher risk of events (hazard ratio = 2.67; 95% confidence interval, 1.17-6.10; P=.02). Patients with values≤14.9% and a CRUSADE score≤40 had the lowest events rate, while patients with values >14.9% and a CRUSADE score >40 points (high and very high risk) had the highest rate of bleeding (log rank test, P<.001). Further, the addition of red cell distribution width to the CRUSADE score for the prediction of major bleeding had a significant integrated discrimination improvement of 5.2% (P<.001) and a net reclassification improvement of 10% (P=.001). Conclusions: In non–ST-segment elevation acute coronary syndrome patients, elevated red cell distribution width is predictive of increased major bleeding risk and provides additional information to the CRUSADE scale

Hemorrhagic Cystitis Following Allogeneic Hematopoietic Cell Transplantation

We conducted a retrospective study to investigate the incidence, risk factors, and clinical features of hemorrhagic cystitis (HC) following allogeneic hematopoietic cell transplantation (allo-HCT). Adult patients who developed HC after allo-HCT were identified from the HCT database of the Asan Medical Center and their medical records were reviewed. From December 1993 to August 2001, a total of 210 adult patients underwent allo-HCT. Fifty-one patients developed HC with a cumulative incidence of 25.7%. The median onset of HC was post-transplant day 24 (range, -2 to 474), and the median duration was 31 days (range, 8 to 369). Significant risk factors for HC by univariate analysis included diagnosis of chronic myelogenous leukemia (p=0.028), unrelated HCT (p=0.029), grade III-IV acute graft-versus-host disease (GVHD) (p<0.001), extensive chronic GVHD (p=0.001), and positive cytomegalovirus antigenemia between post transplant days 31 and 60 (p=0.031). Multivariate analysis showed that grade III-IV acute GVHD was the most important risk factor for the occurrence of HC after allo-HCT (odds ratio, 3.38; 95% CI, 1.36-8.39). Late-onset HC, which occurred beyond 3 weeks after allo-HCT, was more frequently associated with GVHD than earlyonset HC (p=0.007). Our data suggest that a portion of late-onset HC might be a manifestation of GVHD.

Trombocitopenia aislada y gestación / Isolated thrombocytopoenia and pregnancy

Se analizan la etiopatogenia y las características clínicas e inmunológicas de 52 gestantes con trombocitopenia aislada (cifra de plaquetas < 150 x 109/l), así como la presencia de trombocitopenia o de complicaciones hemorrágicas en los neonatos, y se sugiere el tratamiento adecuado en función del tipo de trombocitopenia. En todas las gestantes se practicaron: anamnesis, recuento plaquetario (EDTA K3, citrato Na2 y examen microscópico) e investigación de anticuerpos antiplaquetarios (inmunofluorescencia). Para confirmar una macrotrombocitopenia constitucional se procedió al estudio familiar y ultraestructural de las plaquetas. Se aplicó el test de Levine de homogeneidad de variancias para comparar los recuentos plaquetarios medios de cada grupo diagnóstico, así como una regresión lineal entre los recuentos de madres e hijos. En 31 pacientes (59,61%) con trombocitopenia no se detectaron anticuerpos plaquetarios, ni tampoco historia previa de trombocitopenia o de diátesis hemorrágica. Cuatro pacientes (7,69%) fueron diagnosticadas de seudotrombocitopenia dependiente de EDTA, y 7 más (13,46%) de macrotrombocitopenia constitucional. Finalmente, en 10 mujeres (19,23%) se sospechó una etiología autoinmune, y en todas los anticuerpos antiplaquetarios resultaron positivos. No se detectaron diferencias significativas entre los recuentos plaquetarios medios de las madres con trombocitopenia de etiología inmune respecto del resto de grupos. No se observó hemorragia (..) (AU)

Hemorragias mucocutáneas: evaluación clínica, secuencia de estudio y frecuencia relativa de enfermedades hereditarias de la hemostasia en población chilena / Mucocutaneous hemorrhages: clinical evaluation, study sequency and related frequency of hemostatic hereditary disases in Chilean population

Patients and methods: Five hundred eighty nine patients whose main symptom was the presence of mucocutaneous hemorrhages were studied. Bleeding time, platelet count, coagulant activity of factor VIII (FVIII:C), FvW: Ag and FvW:CoRis and ABO blood group were measured in all patients in a first stage. According to the results of these tests, further studies were decided. Results: In patients younger than 13 years old, males predominated and, in older patients, females consulted with higher frequency. There was a higher proportion of individuals with O blood type than in the normal population. Bleeding time was abnormal in 330 patients (56 percent). One hundred ten patients (19 percent) had von Willebrand disease and, among them, one third had a normal bleeding time. Isolated reduction of factor VIII activity was found in 66 patients (11 percent, 51 males) and 32 of these had normal bleeding time. Eighty one patients (14 percent) were considered to have an hereditary platelet function defect. A precise diagnosis was not achieved in 332 patients (56 percent). Conclusions: Among patients consulting for mucocutaneous hemorrhages, 19 percent had von Willebrand disease, 11 had an isolated reduction of factor VIII activity, 14 percent had platelet function defects and in 56 percent, a precise diagnosis was not reached

Estudio de pacientes con trastornos hemorrágicos en la IX región / Study of patients with hemorrhagic disorders in the IX región

Dada la necesidad de establecer un diagnóstico adecuado que permita un eficaz tratamiento en pacientes con problemas hemorrágicos, se estudiaron 68 sujetos, niños y adultos de la novena región con antecedentes de este tipo, durante el período de un año, quienes fueron remitidos de la policlínica de Hematología. Se realizaron los seguientes exámenes de laboratorio: Tiempo de Sangría de Ivy (T.S.Ivy), Actividad Coagulante del Factor VIII (FVIII: C), Actividad Antigénica del Factor VIII VW(FVIII:AG), Capacidead de las Plaquetas pra aglutinarse de Factor VIII y Ristocetina (FVIII:coR). En 40 de la totalidad de los pacientes se confirmó la enfemedad hemorrágica, de los cuales el 85% corresponde a Enfermedad de von Willebrand y el 15% corresponde a Hemofilia. Del grupo von Willebrand, el 23% presentan 4 criterios alterados, el 24,4% 3 criterios alterados y el 38,2% sólo 2 criterios alterados, presentnado todos el T.S. prolongado. Un 8.82% presenta 3 criterios alterados y T.S. normal. De los resultados obtenidos se concluye que el T.S. Ivy está prolongado en el 91,1% de los pacientes con Enfermedad de von Willebrand. En el grupo estudiado se observa una mayor frecuencia de la enfermedad de von Willebrand en relación a la hemofilia, siendo la proporción 5:1, respectivamente, lo que es coincidente con los resultados de otros estudios nacionales