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.

Relation of factor VIII and IX inhibitors with ABO blood groups in 150 patients with haemophilia A and B

Many investigations have proved relations between ABO blood groups with some diseases and factor VIII and von willebrand level in plasma. In this study we investigated a relation between ABO blood groups and factor VIII and IX inhibitors in 102 patients with haemophilia A and 48 patients with haemophilia B. The assay of inhibitor was done by Bethesda method. There were no relation between ABO blood groups and factor VIII and IX inhibitors

Prevalence of hepatitis C virus infection and HCV genotypes among hemophiliacs in the State of Bahia, Northeastern Brazil: analysis of serological and virological parameters

O objetivo deste estudo foi analisar parâmetros sorológicos e virológicos em hemofílicos no Estado da Bahia. O anti-VHC foi investigado por ELISA em uma coorte de 268 hemofílicos A/B sob acompanhamento em uma unidade de referência do Estado da Bahia. A viremia do VHC e genótipos foram determinados em um subgrupo de 66 hemofílicos soropositivos para o anti-VHC. A soroprevalência do anti-VHC entre os hemofílicos foi de 42,2% (IC 95% 36,5-48,1) e foi associada significativamente (p<0,05) a idade >10 anos, presenca de anticorpos antifator VIII/IX e outros marcadores sorológicos de infeccão. Nenhum dos hemofílicos com idade inferior a 5 anos foram anti-VHC positivos. A viremia foi detectada em 77,3% (51/66), sendo o genótipo 1 do VHC (74%) o mais prevalente, seguido pelos genótipos 3 (22%) e 2 (4%). Nossos resultados indicam que a prevalência do VHC é ainda alta entre os hemofílicos, muito embora a transmissão não tenha sido observada entre os menores de 5 anos.

The limitation of factor IX coagulant activity determination in the diagnosis of hemophilia B carriers.

A preliminary study of factor IX coagulant activity (FIX:C) for determining hemophilia B carriers was conducted at the Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital in Bangkok. Twenty-eight females (8 obligate, 20 potential carriers) from 17 hemophilia B families were enrolled in the study. Additionally, 25 normal females were included. They were not pregnant and not using oral contraceptives. Then, three cut-off levels of FIX:C including 50 per cent which was the commonly used level; 57 per cent which was the mean-2 SD of normal females and 75 per cent which was the level reported by Knobe and Ljung in 1999 were used for the diagnosis of hemophilia B carriers. The sensitivities of these three cut-off levels were 12.5 per cent (1/8) for 50 per cent, 37.5 per cent (3/8) for 57 per cent and 50 per cent (4/8) for 75 per cent. Also, the specificities were 100 per cent (25/25) for both 50 and 57 per cent, and 96 per cent (24/25) for 75 per cent. Although the low cut-off levels of 50 per cent and 57 per cent had low sensitivities, they yielded a high specificity (100%) compared to the higher level of 75 per cent. In the present study, the sensitivity of the cut-off level at 75 per cent was much lower than that of the study by Knobe and Ljung (93%) since the presented sample size of obligate carriers was rather small. So, enrollment of more subjects should be further carried out. In conclusion, FIX:C determination alone showed a limitation in the diagnosis of hemophilia B carriers. The addition of genetic analysis of linkage analysis or mutation detection is required for a definite diagnosis.

Aclinico- haematological study of factor IX deficiency. Haemophilia B

A total of 100 cases of hereditary coagulation disorders were investigated in the city of Lahore - Punjab. Out of them 38 were found to have factor IX deficiency, Haemophilia B. All were males. 25 patients were children below the age of 12 years where as 13 were adults. Common presenting symptoms in these patients were easy bruising, prolonged bleeding from cuts, post circumcision bleeding, haemarthrosis, and haemotoma formation. Clotting time and Activated partial thromboplastin time were prolonged in all the cases. Mixing experiments were performed on 38 cases and all [100%] showed partial or complete correction of APTT with serum. A mild to moderate reduction of factor IX level was found in most of the cases. Only 3 patients had factor IX level of less than 2 U/dl i.e. severe deficiency of factor IX. Message to be conveyed is that, although factor IX deficiency is clinically indistinguishable from Haemophilia A, correct diagnosis of deficient factor is very important as further therapy and management of the patient in factor IX and factor VIII deficiency is different

Hemophilia with factor VIII and factor IX inhibitors, incidence, bleeding problems and management.

Among 117 cases of hemophilia, there were 7 hemophilia A and 2 hemophilia B with factor VIII and factor IX inhibitors diagnosed at the Department of Pediatrics, Siriraj Hospital, Bangkok, Thailand. The overall incidence of hemophilia with inhibitors was 7.7%. Eight cases (6 hemophilia A. 2 hemophilia B) were severe hemophilia and 1 moderate hemophilia A. The average age of the inhibitor detection was about 5 years. Of the 9 cases, 7 had high inhibitor titers and 2 had low inhibitor titers. The frequency of bleeding problems before and after inhibitor detection were not different. The bleedings included hemarthrosis, mucosal bleed, hematoma, oozing from wound, hematuria and intracranial hemorrhage. The treatment of hemarthrosis in hemophilia A with low inhibitor titers was the combination of short course of prednisolone and single large dose factor VIII. In high inhibitor titer patients with acute hemarthrosis (both hemophilia A and hemophilia B), the treatment consisted of prednisolone short course and single high dose of PCC. For bleeding control in both high and low inhibitor titer with mucosal bleeds, oozing from wounds, central nervous system bleeding and hematuria, the combination was used of high dose factor VIII or factor IX for 2 days, and tranexamic acid, prednisolone, cyclophosphamide were required. In life-threatening hemorrhage and surgical operation, plasmapheresis and large dosage factor VIII or factor IX were the treatment of choice. All supportive measures were also important in every case of mucosal bleeds, wounds and surgical operations. The result of treatment revealed one death from massive intracranial hemorrhage and 8 survivals, with joint contracture in 2 cases. All still have inhibitor detected, but in low titer.

Coagulopathy in nephrotic syndrome at the time of renal biopsy.

Two cases of nephrotic syndrome with coagulopathy were presented. The first case was due to acquired factor IX deficiency. The second case was due to unexplanable prolonged thrombin time.