ABSTRACT
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.
Subject(s)
Humans , Female , Child , Adolescent , Blood Coagulation Disorders, Inherited/complications , Blood Coagulation Disorders, Inherited/diagnosis , Fibrinolysis , Menorrhagia/etiology , Blood Coagulation Disorders/complications , Blood Coagulation Disorders/diagnosis , Blood Coagulation Tests , Prevalence , Cross-Sectional Studies , Blood Coagulation Disorders, Inherited/physiopathology , Blood Coagulation Disorders, Inherited/epidemiologyABSTRACT
Propósito: describir la situación de salud oral de los niños portadores de coagulopatías atendidos en el Servicio de Hemoterapia del Centro Hospitalario Pereira Rossell de Montevideo,Uruguay, entre febrero del 2008 y diciembre del 2009, y compararla con un grupo sin coagulopatías. Método: se realizó un estudio retrospectivo de casos y controles. El grupo deestudio estuvo conformado por 39 pacientes (edad: 8,62 ±4,20 años) y el grupo de control, por 78 (edad: 6,5 ± 2,88 años). El análisis de los hallazgos fue descriptivo. Resultados: enel grupo de niños con coagulopatías se encontró un índice ceo-d 2,85 ± 2,41 y un CPO-d 1,96 ±2,59, ambos ligeramente superiores al grupo control. Según la clasificación de lacoagulopatía se registró: hemofilia A en 17 pacientes, hemofilia B en 7, deficiencia de factor XII en un paciente y enfermedad de von Willebrand en 14. La adherencia al tratamientofue calificada como buena en 15 pacientes, mientras que fue mala en los 24 pacientes restantes. Conclusión: este es un primer reporte de salud oral en niños con coagulopatías en Uruguay. Es necesario hacer un seguimiento y aumentar la cobertura de este programa para mantener la salud oral de estos pacientes...
Aim: Describe the oral health status of children with inherited bleeding disorders who attended the Pereira Rossell Hospital in Montevideo Uruguay for dental care, between February 2008 and December 2009, and compare it with children without bleeding disorders. Methods:A retrospective case-control study was carried out. The study group consisted of 39 patients (age: 8.62 ± 4.20 years), while the control group had 78 patients (age 6.5 ± 2.88). Descriptive analysis was done to the data. Results: The study group had a dmf-s index 2.85± 2.41 and a DMF-s 1.96 ± 2.59, being slightly higher than in the control group. The bleeding disorder classification was: Hemophilia A, 17 patients; Hemophilia B, 7 patients; factor XII deficit, 1 patient; and Von Willebrand disease, 14 patients. Commitment to the treatmentwas determined as good in 15 patients and bad in 24 patients. Conclusion: This is the first oral health report on children with bleeding disorders from this program in Uruguay. It isnecessary to follow up the patients and increase the program coverage in order to maintain the oral health of this kind of patients...
Subject(s)
Child , Dental Care for Children/nursing , Dental Care for Children/methods , Disseminated Intravascular Coagulation/congenital , Disseminated Intravascular Coagulation/diagnosis , Disseminated Intravascular Coagulation/blood , Disseminated Intravascular Coagulation/therapy , Pediatric Dentistry/classification , UruguayABSTRACT
Context: Von Willebrand disease is the most common inherited bleeding disorder with a prevalence of ≈ 1% in the general population. Studies available from India are limited, showing a prevalence of ≈ 10% of vWD amongst inherited bleeding disorders. Aims: This study aims to know the prevalence and subtypes of vWD in patients presenting with various bleeding manifestations to university hospital. Settings and Design: We investigated 840 patients presenting with bleeding manifestations in the period from August 2004 to August 2008 for bleeding disorders. Materials and Methods: Tests performed for the diagnosis of vWD included platelet count, bleeding time (BT), prothrombin time (PT), activated partial thromboplastin time (APTT), Factor VIII:C assay, von Willebrand Factor Antigen assay and Ristocetin-induced platelet aggregation. Results: Amongst 840 patients, 230 (27.38%) had inherited bleeding disorder. Out of these, 40 (17.39%) patients were identified as vWD. Type 1 in 17 (42.5%), Type 2 in 11 (27.5%) and Type 3 vWD was present in 12 (30.0%) patients. Patients' age ranged from five months to 45 years, with 17 males and 23 females. Positive family history was seen in 12 patients (30%). Muco-cutaneous bleeding was the most common presentation. Menorrhagia was present in 100% women of reproductive age group, and hemarthrosis was seen in two male patients. Conclusions: We felt that Type I vWD with infrequent and mild bleeding episodes remain undiagnosed either because of unawareness of the disease in society or due to paucity of diagnostic facilities available in our country. Therefore, an awareness program along with enhancement of diagnostic facilities for vWD is required in our country to identify these patients for proper management thus avoiding hemorrhagic complications.
ABSTRACT
Background: Hemophilic children must be thought of as special patients. Although there have been number of studies regarding with oral surgical, periodontal management of hemophilia there is paucity for the dental caries and its severity in hemophiliac children. The objective of this study is to assess prevalence of dental caries and treatment needs among hemophilic children attending Rajasthan hemophilia society, Kota City, Rajasthan. A descriptive cross-sectional study was conducted on a group of children suffering from haemophilia with age ranging from 0 to 15 years. Dentition Status and Treatment Needs Index (WHO 1997) was used to record dental caries. A total of 164 subjects of both the sexes were examined. Over all prevalence of dental caries was 87.19%. The present study showed dental caries prevalence and treatment needs was high among hemophilic patients indicating need for dental services at the earliest as a preventive measure for these patients.
ABSTRACT
Objective: We did a clinical audit to determine the status of coagulation disorders in a hemophilia care center in Pakistan. Setting: Fatimid foundation blood bank and hematological diseases center, Lahore. Study Design: This is a retrospective descriptive study. Materials and Methods: All patients registered at Lahore center were included. Data was collected using a questionnaire including age, gender, diagnosis, hepatitis and human immune deficiency virus (HIV) status, number of episodes of bleeding, most common site of bleeding, severity of disorder and number of transfusions required to treat the episode. Results: During the study period, a total of 923 registered patients were reviewed at Lahore center and of these, 408 patients (44.2%) were on regular follow-up. Inherited bleeding disorders identified in these patients included hemophilia A, hemophilia B, vWD, factor VII deficiency, factor V deficiency, factor X deficiency, dysfibrinogenemia, afibrinogenemia, factor XIII deficiency; and platelet function defects. Median age was 17 years with a range of three to 57 years. Median age at diagnosis was one year. There were 329 (80.6%) males and 79 (19.3%) females. The products used in these patients included factor VIII concentrate, fresh frozen plasma, cryoprecipitate, cryosupernatant and platelets. Testing for transmission of viral infections was also done in these patients and one patient (0.2%) was found hepatitis B positive, six patients (1.4%) were hepatitis C positive and two patients (0.49%) were HIV positive. Conclusion: Hemophilia A, hemophilia B and vWD are the commonly encountered inherited bleeding disorders in our patients followed by other recessively transmitted disorders with a median age of 17 years and male to female ratio of 4: 1. Most of the patients utilized services available at Fatimid foundation with good clinical results. In Pakistan, non-governmental organizations (NGOs) are trying their best for providing optimal treatment to patients with inherited bleeding disorders. There is a need for government participation to improve the availability of current hemophilia care services.