Reabilitação odontológica multidisciplinar em paciente pediátrico com hemofilia: relato de caso / Multidisciplinary rehabilitation of pediatric dentistry in patients with hemophilia: case report

A hemofilia é uma coagulopatia congênita autossômica recessiva ligada ao cromossomo X, isso é devido à deficiência do fator VIII (hemofilia A) ou do fator IX (hemofilia B) afetando apenas os homens.Os transtornos hemorrágicos constituem um dos problemas de maior interesse a serem considerados pelo cirurgião-dentista na prática odontológica. O tratamento odontológico de pacientes com hemofilia deve ser realizado sob cuidados especiais com uma equipe multidisciplinar, nos quais os profissionais da saúde devem ter experiência médica e apoio hematológico adequados. O Objetivo deste trabalho foi relatar a reabilitação odontológica de maneira multidisciplinar de um paciente pediátrico com diagnóstico de hemofilia B grave, a qual é tratada e controlada por médicos hematologos do Hospital Geral de Zona (HGZ). O diagnóstico odontológico foi de acúmulo de placa bacteriana nas superfícies dentais por má higiene bucal, lesões de cárie e hipoplasia de esmalte. Para reabilitação bucal foram realizadas extrações de alguns elementos dentais, profilaxia, flúor terapia, orientação emotivação de higiene bucal, remoção de tecido cariado com auxílio de ultrassom, restaurações com resina composta, ionômero de vidro e restaurações indiretas cerômeros. Concluiu-se que para um adequado manejo odontológico é imprescindível o conhecimento de diversas patologias sistêmicas como a hemofilia, o atendimento multidisciplinar, além do conhecimento de técnicas de mínima intervenção em Odontopediatria para assim poder oferecer ao paciente melhores alternativas de tratamento com a mínima invasão e resultados favoráveis.

Hemophilia is an autosomal recessive congenital blood coagulation desorder to X chromosome, this is due to deficiency of factor VIII (hemophilia A) or factor IX (hemophilia B) affects only men. Bleeding disorders are one of the issues of greatest interest to be considered by the dentist in the dental practice. Dental treatment of patients with hemophilia should be done under special care bya multidisciplinary team in which health professionals must have medical experience and adequate hematologic support. The objective of this study was to report the dental rehabilitation in a multidisciplinary team to a pediatric patient with severe haemophilia B, which is treated and controlled by medical hematologists in Zone General Hospital (HGZ). The dental diagnosis was accumulation ofplaque on dental surfaces by poor oral hygiene, dental caries and enamel hypoplasia. For oral rehabilitation extractions were performed in some dental elements, prophylaxis, fluoride therapy, guidance and motivation of oral hygiene, caries removal with ultrasound assistance restorations with compositeres in, glass ionomer and indirect restorations with cerômics. It was concluded that for properdental management is essential knowledge of various systemic diseases such as hemophilia, multidisciplinary care, in addition to knowledge of minimal intervention techniques in pediatric dentistry so as to offer the best treatment alternatives patient with minimal invasion and favorable results.

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.

Prevalence of hepatitis C virus infection and human immunodeficiency virus in a cohort of Egyptian hemophiliac children

The risk of blood-borne infections, especially hepatitis C virus [HCV] and human immunodeficiency virus [HIV] infection still remains in developing countries among children receiving blood products as hemophiliacs, but the risk is not known in Egypt. The objective of this study was to detect the prevalence of HCV and HIV infection among hemophiliac children to know the magnitude of the problem and determine potential risk factors. This was a cross-sectional study conducted on 100 hemophiliac children that assessed the liver clinically and by laboratory tests. All children were screened for HCV and HIV antibodies by enzyme-linked immunosorbent assay. Those with positive HCV antibody titre were tested by polymerase chain reaction [HCV-PCR]. Forty were positive for HCV antibodies with 19 children [47.5%] HCV-PCR positive as well. The mean age, average frequency of bleeds/year, dose of replacement therapy/year and alanine aminotransferase [ALT] levels were significantly high in HCV-antibody and PCR positive patients as compared to HCV antibody and PCR negative ones. None of our patients had clinical evidence of hepatic involvement or was co-infected with HIV HIV infection does not appear to be a current health problem in Egyptian hemophiliac children though the prevalence of HCV infection is still high

Qualidade de vida em pacientes hemofílicos com lesão na cavidade oral / Quality of life in hemophilic patients with lesions in the oral cavity

Esta pesquisa tem como objetivo avaliar a qualidade de vida nos pacientes hemifílicos com lesões orais atendidos no Conjunto Hospitalar de Sorocaba. Trata-se de um estudo analítico prospectivo, comparativo e randomizado. Fizeram parte deste estudo 20 pacientes hemofílicos com lesão em cavidade oral, atendidos no Hemonúcleo do Conjunto Hospitalar de Sorocaba (CHS), foi utilizado para a coleta de dados instrumentos de avaliação sócio-demográficos, avaliação da qualidade de vida pelo questionário Short Form-36 Healty Survey. Os pacientes hemofílicos com lesões orais apresentaram alteração na qualidade de vida.

Head trauma and hemophilia B: case report about a challenging hematologic emergency

Bleeding emergencies are common chief complaints in visits to the Emergency Department (ED) but hemophilia, a rare congenital bleeding disorder requires immediate treatment. Hemophilia B is characterized by a deficiency of coagulation Factor IX. As emergency medicine physicians we must be prepared to deal with such emergencies. One of the most feared complications in this population is the spontaneous or post-traumatic development of intracranial hemorrhage (ICH). We present a case of a 69 year-old man with a past medical history of hemophilia B who presented to the emergency department after suffering a head trauma with development of acute and delayed ICH.

Deformidades e incapacidades dos hemofílicos do Centro de Hemoterapia e Hematologia do Espírito Santo, Brasil / Deformities and disabilities of hemophilics undergoing treatment at the Hemoterapy and Hematology Center of Espírito Santo, Brazil

A hemofilia é um grave distúrbio hereditário da coagulação sanguínea que pode resultar em diversas deformidades e incapacidades, permanentes ou transitórias, além das consequências psicossociais nos indivíduos afetados...

Hemophilia is a severe hereditary disorder that affects blood clotting: it can cause several permanent or transitory deformities and disabilities as well as psychosocial disorders on those affected. The proposition of this is study is not only to identify the main complications found on...

Importância da podobarometria computadorizada na precricao de órteses para redução das hemartroses de repetição dos tonrnozelos, em pacientes hemofílicos / Significance of the computadorised pedobarography to prescription of orthosis for repetitive anckle haemarthrosis reduction, on hemophilic patients

Nos países em desenvolvimento, por falta de recursos para se fazer profilaxia para todos os hemofílicos, ainda vemos muitos pacientes incapacitados pelas hemartroses de repetição. Sabe-se que a instabilidade articular contribuiria para aumentar a frequência dessas complicações e prescrevem-se órteses que limitam muito os pacientes. Diante desse quadro buscamos um instrumento para qualificar os desvios nos tornozelos instáveis durante a marcha, dinamicamente e em tempo real. Essa avaliação permitiria a prescrição de palmilhas de fácil confecção, de calçados com contrafortes posteriores e, nos casos / Developing countries, due to the lack of resources available to perform prophylaxis to all haemophilic, there can still be seen many patients disabled by the repetitive haemarthrosis. It is known that the joint instability can contribute to increase the frequency of those complications and are prescribed orthosis which severely limit the patients...

[Cerebral bleeding: a revelation mode of haemophilia]

Haemophilia is a familial hemorrhagic disease with a recessive X chromosome transmission. Revelation mode depends on the factors VIII and IX deficit degree. We report a 10 month-old boy case diagnosed as hemophilia after the occurence of convulsions with fever, and treated at first time as meningitis. The purpose of this article is to remind a diagnosis which is very uncommon in this context, and to precise haemophilia revelation modes

Leucemia mieloide crónica en hemofilia B / Chronic myeloid leukemia in hemophilia B

A known hemophiliac B male (blood coagulation factor IX level of 15 per cent), HIV negative, in whom a Philadelphia chromosome positive chronic myeloid leukemia developed at the age of 29 years, is presented. The leukemia was diagnosed after an upper digestive tract bleeding, due to a duodenal ulcer. Two years elapsed between the diagnosis of leukemia and the start of busulfan therapy. During that period and after the start of treatment, the hemorrhagic tendency did not increase. The follow up of the patient was almost 8 years, with favorable clinical response to busulfan therapy. The patient survived more than twice the average survival of chronic myeloid leukemia patients treated with busulfan. A reciprocal influence between chronic myeloid leukemia and hemophilia appears illustrated by this case report.

Management of hemophilia in developing countries.

Coagulation disorder are common in India. In the absence of any epidemiological studies it is expected that there are at least 50,000 severe hemophilics in our country. The factor concentrates are not easily available in developing countries which poses a major problem while managing severe bleeding episodes in these patients. Various strategies which could be useful while managing these cases in developing countries have been reviewed.

Hemofilia y osteosarcoma. Informe de un caso / hemophilia and osteosarcoma. Case report

La hemofilia es una enfermedad relativamente rara, puede haber pacientes portadores, que si nunca sufren un traumatismo grave pueden no ser descubiertos. Tanto la hemofilia ®A¼ como la ®B¼ o enfermedad de Christmas se heredan de modo recesivo ligado al cromosoma ®X¼, con un incidencia de 10 hemofílicos, por cada 100 varones nacidos. El pseudotumor es un hecho relativamente raro, su incidencia se ha calculado en 1 por ciento al 2 por ciento en hemofílicos graves, en el pseudotumor que se origina a partir de una hemorragia subperióstica puede ocurrir una significativa destrucción ósea, se reporta el caso de un paciente masculino de 17 años de edad con el diagnóstico de hemofilia tipo ®B¼, tratada desde los 6 años, a esta edad presentó un pseudotumor en fémur izquierdo, a la edad de 17 años presenta probable pseudotumora en húmero izquierdo, posterior a la biopsia se reporta sarcoma osteogénico, iniciándose quimioterapia (2 ciclos a base de adriamicina y cisplatino), practicándosele desarticulación de dicho miembro, falleció 8 meses después de la cirugía con metástasis múltiples

Sangrado en ataxia telangiectasia. Un aspecto de la enfermedad previamente no estudiado / Bleeding in ataxia telangiectasia. An aspect of this illness wich has not been previosly studied

En un paciente con ataxia-telangiectasia se demostró la presencia de una condición hemorrágica debida a alteraciones vasculares, defectos plaquetarios funcionales múltiples y deficiencia leve de factor IX. En la revisión de 128 pacientes con ataxia-telangiectasia se encontró que 7 habían sufrido hemorragias en ausencia de alguna enfermedad subyacente capaz de provocarlas. La presencia de trastornos semejantes a los descritos en el paciente, en síndrome de Ehlers-Danlos, en osteogénesis imperfecta y en telangiectasia hemorrágica hereditaria (condiciones que tiene en común con ataxia-telangiectasia, la existencia de alteraciones vasculares), permiten plantear la existencia de alguna forma de interrelación entre el desarrollo de vasos y la síntesis de algunos factores de la coagulación y la formación de plaquetas

HIV infection in Asian Indian patients with haemophilia & those who had multiple transfusions.

A total of 124 Indian patients with haemophilia and 185 multiple transfused patients with thalassaemia, haemoglobinopathies, patients on chronic haemodialysis and others were screened for HIV-1 infection by a commercially available competitive ELISA test and supplementary Western Blot (WB) test. The results showed that HIV-1 infection was mostly confined to the haemophiliacs where 15 (12.1%) were confirmed to be positive for HIV infection. All except one had received both foreign and Indian cryoprecipitate. However, one haemophilic seroconverted within 4 months of receiving a cryoprecipitate manufactured in India.

HBV & HIV seropositivity in multi-transfused haemophilics & thalassaemics in eastern India.

Seropositivity to HBV (HBsAg) in multi-transfused patients of haemophilia A, haemophilia B, B thalassaemia and EB thalassaemia from Eastern India, was found to be 9, 0, 22.1 and 13 per cent respectively. HIV seropositivity was detected in patients of haemophilia A (4.4%) and B thalassaemia (0.8%) who received plasma components and packed cells periodically. Seropositivity to both HBsAg and HIV was found in one patient of haemophilia A. Serum alanine amino transferase (ALT), raised in multi-transfused thalassaemics suggests concurrent hepatitis which might have enhanced the transmission of viruses due to disturbed immune status. The universal voluntary blood donation programme, screening of blood for HBV and HIV by sensitive tests, early immunisation and periodic monitoring of HBV and HIV status are prerequisites for the management of transfusion dependent thalassaemia and haemophilia.

Comprehensive clinical and statistical analysis of hemophilia in Korea

A Total of 498 cases of hemophilia which were reported by sixteen medical centers in Korea were reviewed and analyzed. Hemophilia A comprised 425 cases (85.3%) and the remaining 73 cases (14.7%) were hemophilia B. One case was female and all other cases were male. There were known hemophilia patients in the family in 43.0% of cases and the involved members were brothers, maternal cousins, maternal uncles, and maternal grandfathers in descending order of frequency. The major symptoms of the patients were hemorrhagic, such as easy bruising and hemarthrosis followed by prolonged bleeding after trauma and soft tissue hematoma. The incidence of hemarthrosis increased significantly with age. The pediatric age group below the age of 15 consisted of 67.1% of the cases. According to the age at diagnosis, half (54.2%) of the severe cases were diagnosed before the age of 1 year. APTT was prolonged over 40 seconds in all cases and 291 cases showed severe prolongation over 80 seconds. Of 498 cases 273 cases (54.8%) belonged to the severe form (factor VII or IX level, less then 1%), whereas 182 cases (36.5%) and 43 cases (8.7%) belonged to the moderate (factor VIII or IX, 2-5%) and mild form (factor VIII or IX, 6-25%), respectively, Chronic arthropathy was present in 236 cases (49.6%), and the incidence increased significantly with age. The management of chronic arthropathy most commonly employed was rehabilitation in 25.4% of cases, but in 50.8% no management was given at all. The involved joints in descending order of frequency were knees, elbows and ankles. The complications were intracranial hemorrhage, Gl bleeding and nerve palsy in 48, 24, and 13 cases, respectively.

Comprehensive clinical and statistical analysis of hemophilia in Korea

A Total of 498 cases of hemophilia which were reported by sixteen medical centers in Korea were reviewed and analyzed. Hemophilia A comprised 425 cases (85.3%) and the remaining 73 cases (14.7%) were hemophilia B. One case was female and all other cases were male. There were known hemophilia patients in the family in 43.0% of cases and the involved members were brothers, maternal cousins, maternal uncles, and maternal grandfathers in descending order of frequency. The major symptoms of the patients were hemorrhagic, such as easy bruising and hemarthrosis followed by prolonged bleeding after trauma and soft tissue hematoma. The incidence of hemarthrosis increased significantly with age. The pediatric age group below the age of 15 consisted of 67.1% of the cases. According to the age at diagnosis, half (54.2%) of the severe cases were diagnosed before the age of 1 year. APTT was prolonged over 40 seconds in all cases and 291 cases showed severe prolongation over 80 seconds. Of 498 cases 273 cases (54.8%) belonged to the severe form (factor VII or IX level, less then 1%), whereas 182 cases (36.5%) and 43 cases (8.7%) belonged to the moderate (factor VIII or IX, 2-5%) and mild form (factor VIII or IX, 6-25%), respectively, Chronic arthropathy was present in 236 cases (49.6%), and the incidence increased significantly with age. The management of chronic arthropathy most commonly employed was rehabilitation in 25.4% of cases, but in 50.8% no management was given at all. The involved joints in descending order of frequency were knees, elbows and ankles. The complications were intracranial hemorrhage, Gl bleeding and nerve palsy in 48, 24, and 13 cases, respectively.

Hemofilia ¿ enfermedad invalidante? / Is hemophilia a disabling disease?

Se revisaron retrospectivamente los registros clínicos de 35 pacientes con hemofilia controlados en un hospital de niños de Santiago entre los años 1968 y 1983, treinta y dos con hemofilia A y 3 con hemofilia B. El tiempo de sangría de Ivy estaba prolongado en 7/29 pacientes (24%). En 24/35 (68%) de los pacientes los síntomas comenzaron antes de los 2 años de edad, en relación con la deambulación. En la mitad de los casos del diagnóstico definitivo se confirmó en menos de un año desde las primeras manifestaciones, siendo los hematomas las causas más comunes de consulta. Sólo 11/35 (31%) de los pacientes tenían se cuelas articulares en el momento de la última evaluación, las que no guardaron relación con el tiempo de seguimiento, pero 7/11 tenían hemofilia severa (Factor VIII < 1%). En 19 pacientes se realizaron intervencicones quirúrgicas incluyendo 2 tonsilectomías, una apendicectomía y una reparación completa de tratalogía de Fallot con circulación extracorpórea: ninguno sufrió complicaciones debidas a su hemofilia. La vida de los pacientes con hemofilia puede ser prácticamente normal, con escasas complicaciones y secuelas desde la introducción de los crioprecipitados para suplir el factor deficiente