Trastornos hereditarios de la coagulación en adolescentes con sangrado menstrual excesivo, ¿debemos evaluar la vía fibrinolítica? / Inherited bleeding disorders in adolescents with excessive menstrual bleeding: should we evaluate the fibrinolytic pathway?

Resumen: Introducción: El Sangrado Menstrual Excesivo (SME) es un problema frecuente en la adolescencia. La prevalencia de trastornos hereditarios de la coagulación (THC) como causa del SME no está bien establecida y la participación de defectos de la vía fibrinolítica ha sido poco explorada. Objetivo: Determinar la prevalencia de THC y defectos de la fibrinólisis en adolescentes con SME. Pacientes y Método: Se incluyeron 93 adolescentes, edad 11 a 18 años. Los antecedentes personales y familiares de sangra do se obtuvieron con un cuestionario estandarizado. Se controló exámenes: tiempo de protrom- bina (TP), tiempo de tromboplastina parcial activada (TTPa), estudio del factor Von Willebrand, recuento y función plaquetaria. Los pacientes que no fueron diagnosticados como THC, se evaluaron adicionalmente con el tiempo de lisis del coágulo. Resultados: 41 pacientes (44%) fueron diagnos ticados como THC: Enfermedad de Von Willebrand n = 28, defectos de la función plaquetaria n = 8, hemofilia leve n = 5. Se confirmó disminución del tiempo de lisis del coágulo en 31 pacientes. El 54% de pacientes diagnosticado como THC, tuvo SME como la primera manifestación hemorrágica. Conclusión: Estos resultados apoyan la necesidad de evaluación de la coagulación, incluyendo la vía fibrinolítica, en el estudio de adolescentes con SME.

Abstract: Introduction: Heavy Menstrual Bleeding (EMB) is a frequent problem in adolescence. The prevalence of inherited bleeding disorders (IBD) as a cause of EMB is not well established and the involvement of fibri nolytic pathway defects has been poorly explored. Objective: To determine the prevalence of IBD and fibrinolysis defects in adolescents with EMBs. Patients and Method: 93 adolescents (11 to 18 years old) were included. Personal and family history of bleeding were obtained through a standard ized questionnaire. The following lab tests were performed: prothrombin time (PT), activated partial thromboplastin time (aPTT), von Willebrand factor quantification, and platelet count and function. Those patients who were not diagnosed with IBD were further evaluated with clot lysis time assay. Results: 41 patients (44%) were diagnosed as IBD (Von Willebrand disease n = 28, platelet func tion defects n=8, mild hemophilia n = 5. Decreased clot lysis time was found in 31 patients. 54% of patients diagnosed with IBD had EMB as the first hemorrhagic manifestation. Conclusion: These results support the need to evaluate the coagulation process, including the fibrinolytic pathway in the study of adolescents with EMB.

Temporomandibular disorder: prevalence among hemophiliac patients / Trastorno temporomandibular: prevalencia en pacientes hemofílicos

The objective was to investigate the prevalence of temporomandibular dysfunction ­TMD - in severe and moderate hemophiliac A and B patients and healthy men as control group. Hemophilia complication is chronic arthropathy that results from repeated joint bleeding, leading to limited movement. Limitation of jaw movement is present in patients with TMD. Hemophiliac patients were recruited in the Hemophilia outpatient clinic at Escola Paulista de Medicina, Universidade Federal de São Paulo (UNIFESP). The control group was composed of voluntary subjects recruited among medical and dental students of UNIFESP. Both groups were screened for TMD symptoms according to the European Academy of Craniomandibular Disorders questionnaire. The Research Diagnostic Criteria further evaluated those considered positive for TMD. The results showed a similar prevalence of TMD in the hemophiliac group compared to the control group (n= 38, n= 79; p= 0.7). There were no significant differences in severity of sign and symptoms between the groups. In conclusion, patients with hemophilia do not have a higher prevalence of temporomandibular disorders, indicating absence of hemorrhage in temporomandibular joint.

El objetivo fue investigar la prevalencia de trastornos temporomandibulares (TTM) entre pacientes hemofílicos A y B severos y moderados, y hombres sanos como grupo de control. Una complicación de la Hemofilia es la artropatía crónica como resultado de una hemorragia articular a repetición, limitando el movimiento en el tiempo. La limitación del movimiento de la mandíbula está presente en pacientes con TTM. Los pacientes hemofílicos fueron reclutados en la clínica de atención ambulatoria de hemofilia en Escola Paulista de Medicina, Universidade Federal de São Paulo (UNIFESP). El grupo control estaba compuesto por sujetos voluntarios reclutados entre los estudiantes de Medicina y Odontología de UNIFESP. Ambos grupos fueron evaluados por síntomas de TTM según cuestionario de trastornos craneomandibulares de la Academia Europea. Los criterios de diagnósticos de investigación evaluados se consideraron positivos para TTM. Los resultados mostraron una prevalencia similar de TTM en el grupo de hemofílicos en comparación con el grupo control (n= 38, n= 79; p= 0,7). No se encontraron diferencias significativas en la gravedad de los signos y síntomas entre los grupos. En conclusión, los pacientes con hemofilia no tienen una mayor prevalencia de trastornos temporomandibulares, indicando la ausencia de hemorragia en la articulación temporomandibular.

Study of clinical characteristics, presentation, and complications among patients with congenital coagulation disorders

To study the clinical characteristic, presentation, complications in patients with congenital coagulation disorders who attended the Congenital Coagulation Disorders Center, Baghdad, Iraq. This cross-sectional study was conducted in the Center of Congenital Coagulation Disorders, Al-Mansour Pediatric Teaching Hospital, Baghdad, Iraq between March 2008 and August 2008. The sample comprised 243 patients with different congenital coagulation disorders. We collected the data through structured questionnaire form and carried out the physical examination of the patient under the supervision of the physician-in-charge at the center. Hemophilia and Von Willebrand Disease [VWD] constituted 90.1% of the sample, while rare bleeding disorders constituted 9.9%. Most patients [82.7%] have had onset of symptoms during the first year of life. Clinically, 52.7% of patients presented with hemarthroses were hemophiliacs and factors X deficiency. This distribution was also true for joint limitations. Most patients were hemophiliacs and the highest prevalent of symptom was 6-12 months old, and the diagnosis of the disease was highest within one month after the onset of symptoms. Most patients with joint limitation was hemophiliacs and approximately 50% had limitation of 3 or more joints