Objective: To characterize the socioeconomic and demographic aspects of sickle cell disease patients from the state of Rio Grande do Norte (RN), Northeast Brazil, and their adherence to the recommended treatment. Methods: This cross-sectional descriptive study was performed at referral centers for the treatment of hematological diseases. One hundred and fifty-five unrelated individuals with sickle cell disease who went to these centers for outpatient visits were analyzed. All the patients, or their caregivers, were informed about the research procedures and objectives, and answered a standardized questionnaire. Results: The patients were predominantly younger than 12 years old, self-declared as mulatto, lived in small towns fairly distant from the referral center, and had low education and socioeconomic levels. Individuals who were ten or younger were diagnosed at an earlier age. Almost 50% of the patients were taking hydroxyurea, 91.4% reported having received pneumococcal/meningococcal vaccinations and 76.1% received penicillin as antibiotic prophylaxis. However, the majority of them reported having difficulties following the recommendations of the physicians, mainly in respect to attaining the prescribed medications and transportation to the referral centers. Conclusion: These individuals have a vulnerable socioeconomic situation that can lead to an aggravation of their general health and thus deserve special attention from the medical and psychosocial perspectives. Thus, it is necessary to improve public policies that provide Brazilian sickle cell disease patients with better access to medical treatment, living conditions, and integration into society.

OBJETIVO: Este estudo objetivou avaliar a prevalência de alterações orais e sua associação com a doença enxerto versus hospedeiro (DEVH) em pacientes submetidos a transplante de medula óssea (TMO). METODOLOGIA: A amostra consistiu de 51 pacientes. Por meio de questionário, foram coletados dados sobre idade, sexo, doença, tipo e tempo de transplante, origem das células e ocorrência da DEVH. Foram realizados exames clínicos extra e intraoral por profissionais especializados. RESULTADOS: DEVH sistêmica foi observada em 32,5% dos pacientes transplantados alogênicos, todos apresentando manifestações orais. Também houve uma associação estatisticamente significante entre DEVH sistêmica e manifestações orais (P<0,001). CONCLUSÃO: Diante da prevalência de alterações orais relativamente alta associada à DEVH em pacientes submetidos ao TMO, o presente estudo confirma a necessidade de se considerar a odontologia no exame, diagnóstico, tratamento e prognóstico de possíveis complicações após o transplante de medula óssea.

PURPOSE: This study aimed to evaluate the prevalence of oral changes and their association with graft versus host disease (GVHD) in patients undergoing bone marrow transplantation (BMT). METHODS: The sample consisted of 51 BMT patients. A questionnaire was used to collect data on age, gender, disease, type and time of transplant, cell origin, and GVHD occurrence. The extraoral and intraoral clinical examinations were performed by specialized professionals. RESULTS: Systemic GVHD was observed in 32.5% of the allogeneic transplant patients, and all of the patients with GVHD had oral manifestations. There was a statistically significant association between systemic GVHD and oral manifestations (P<0.001). CONCLUSION: Given the relatively high prevalence of oral changes associated with GVHD in patients undergoing BMT, this study confirms the need to consider dental aspects in the examination, diagnosis, treatment and prognosis of possible complications after BMT.

Há 350 milhões de indivíduos cronicamente infectados pelo vírus da hepatite B (VHB). O desenvolvimento e a gravidade dessa infecção dependem de vários fatores, tais como a idade da primoinfecção e resposta imune, sendo o risco dessa cronificação menor que 5% em adultos e maior que 90% em neonatos. Indivíduo cronicamente infectado pelo VHB durante a infância tem 25% de chance de morrer por cirrose ou hepatocarcinoma. Tais evidências conduziram os autores a desenvolver este estudo cujo objetivo foi estimar a incidência pós-transfusional do antígeno de superfície do vírus da hepatite B (HBsAg) em crianças com neoplasias, que foram transfundidas no curso do tratamento ou seguimento da doença. Foi realizado um estudo retrospectivo, com revisão de 333 prontuários do serviço de oncologia do HIVS, de janeiro de 1993 a janeiro de 2005. Adotaram-se como critérios de inclusão: idade menor que 16 anos, diagnóstico de doença neoplásica e realização da pesquisa do HBsAg. Assim, 199 prontuários foram excluídos por não preencherem tais critérios, restando 134 que foram analisados quanto à realização de hemotransfusão. Das 134 crianças, 116 foram transfundidas e 18 não. Apresentaram pesquisa reativa para o HBsAg 32,8% das transfundidas e apenas 5,6% das não-transfundidas. O teste Exato de Fisher mostrou que houve significância estatística (p = 0,023) e observou-se que as proporções de pacientes com pesquisa reativa diferem entre os transfundidos e os não-transfundidos. O odds ratio de um paciente transfundido apresentar pesquisa reativa para o HBsAg foi calculado em 8,28 vezes maior do que um não-transfundido.

There are 350 million individuals infected by the hepatitis B virus (HBV). The development and the severity of the infection depend on several factors, such as: age at the first infection and the immunity response of the subject. The risk of chronic infection is less than 5% for adults and greater than 90% for newborn babies. An individual who is chronically infected by HBV during childhood has a 25% risk of dying due to cirrhosis or liver cancer. This data led the authors to design a study with the objective of estimating the post-transfusion incidence of the hepatitis B virus surface antigen (HBsAg) in children with neoplasias who were transfused during treatment or during the follow up. A retrospective study was performed that revisited 333 medical records from the oncology service in the HIVS from January 1993 to January 2005. The inclusion criteria were: age less than 16 years old, diagnosis of cancer and the results of the HBsAg test. Thus, 199 patients were excluded because they did not fulfill the criteria. The remaining 134 patients’ records were analyzed in regards to blood transfusion. Of the 134 children who satisfied the criteria, 116 were transfused and 18 were not. Results of the HBsAg test were positive in 32.8% of the transfused patients and in only 5.6% of non-transfused individuals. The Fisher Exact Test demonstrated a statistically significant difference (p = 0.023). The ODDS ratio of a transfused patient presenting with reactive results for HBsAG was calculated at 8.28 times greater than non-transfused individuals.

OBJETIVOS: CD5 é um marcador normalmente expresso nas células T e de forma aberrante nas células B da leucemia linfocítica crônica (LLC) e no linfoma de células do manto (LCM). Outras doenças linfoproliferativas crônicas como a hairy cell leukemia (HCL) e leukemia prolinfocítica de células B (LPL-B), são geralmente CD5 negativas ou expressam fracamente este antígeno. Neste trabalho investigou-se o padrão de expressão do CD5 em 42 pacientes com doenças linfoproliferativas crônicas de células B (DLC-B). METODOS: Investigamos a expressão de CD5 em células leucêmicas de 42 pacientes com DLC-B através da citometria de fluxo. Dados demográficos, tais como idade e sexo, bem como dados clínicos e laboratoriais também foram analisados. RESULTADOS: A imunofenotipagem mostrou que 35 casos foram LLC, 3 LPL-B, 3 HCL e um caso de LMC. O CD5 mostrou-se fortemente expresso em todos os casos de LLC e LMC. Baixa expressão desse antígeno foi observada em um caso de LPL-B, mostrando-se negativamente expresso em todos os casos de HCL. CONCLUSÃO: Nossos resultados demonstram que o padrão de expressão do CD5 pode auxiliar na distinção entre LLC da HCL e LPL-B, sendo no entanto similares na HCL e LCM.

PURPOSE: CD5 is a T cell marker, aberrantly express in B cell chronic lymphocytic leukemia (B-CLL) and mantle cell lymphoma (MCL). Other chronic B cell malignancies including hairy cell leukemia (HCL) and B cell prolymphocytic leukemia (B-PLL) are CD5 negative or express this antigen in a weak way. In this study, CD5 expression was investigated in leukemic cells from 42 patients with chronic B cell lymphoproliferative disease. METHODS: We studied the CD5 expression in leukemic cells from 42 patients with chronic B-cell malignancies by flow cytometry. Demographic features such as age, sex and clinical date were also analyzed. RESULTS: There were 22 males and 20 females. The immunophenotyping showed that 35 cases were B-CLL, 3 B-PLL and HCL and one patient was MCL. CD5 expression was present in all B-CLL and MCL. Low expression of CD5 was observed in one patient with B-PLL and negative in all cases of HCL. CONCLUSION: Our date demonstrated that CD5 expression can help distinguish among B-CLL from HCL and B-PLL, but is similar expressed in MCL.

O objetivo do presente estudo foi verificar a eficácia de um protocolo oral preventivo em crianças com leucemia linfoblástica aguda (LLA), submetidas ao tratamento antineoplásico, antes de iniciar um estudo mais abrangente. Durante um período de sete meses, foram avaliadas, consecutivamente, quatorze crianças com LLA, de dois a dez anos de idade. As crianças com LLA que fizeram bochechos com a solução de clorexidina 0,12% (sete crianças) foram comparadas com aquelas do grupo controle (sete crianças) no que diz respeito ao surgimento de lesões orais. Todas as crianças do estudo receberam diariamente orientação de higiene oral e foram igualmente examinadas pelo odontopediatra. Observou-se uma diminuição significativa na incidência de mucosite e ulceração oral nas crianças que fizeram bochecho profilático com clorexidina 0,12% (p < 0,05, teste exato de Fisher). Os achados obtidos no presente estudo são satisfatórios, e sugerem que uma aplicação sistemática de um protocolo preventivo reduza a incidência de complicações orais em crianças com LLA submetidas à quimioterapia.

This study was designed to assess the effectiveness of a preventive oral protocol in children receiving antineoplastic treatment for acute lymphoblastic leukemia (ALL) before initiating a larger intervention study. During a seven month period, fourteen children from two to ten years old with a diagnosis of ALL were evaluated. Patients with ALL who received a 0.12% chlorhexidine mouth rinse (seven children) were compared to a control group of patients who were not given the same preventive treatment (seven children) as to the occurrence of oral mucosal complications. Children in both groups received daily oral hygiene care, and were examined daily by the pediatric dentistry team until discharge. A significant decrease in the incidence of oral mucositis and ulceration was observed in the children who received a 0.12% chlorhexidine mouth rinse (p < 0.05 by Fisher's exact test). The findings obtained in the present trial are encouraging, and suggest that the systematic application of a preventive protocol reduces the incidence of oral complications in children with ALL receiving chemotherapy.

Objetivos: Detectar os principais achados fundoscópicos em crianças portadoras de hemoglobinopatias falciformes. Métodos: Foram estudados 26 pacientes com hemoglobinopatias falciformes, no Serviço de Oftalmologia do Hospital Universitário Onofre Lopes, Natal, RN, que foram submetidos a protocolo de pesquisa pré-estabelecido. Os resultados foram avaliados estatisticamente pelo teste qui-quadrado. Resultados: A idade média foi de 10,6 anos, com acuidade visual igual ou melhor que 20/25 na maioria, excetuando-se 3 olhos, que apresentavam outras doenças associadas. O tipo mais freqüente foi o SS com 57,7% (15/26) dos casos, seguido pelos SC e SA com 15,4% (4/26) cada, e pelo S-Thal com 11,5% (3/26). A freqüência da retinopatia por células falciformes foi maior após os 10 anos de idade, sendo mais freqüente, em valores relativos, no tipo S-Thal (100% dos casos) e, em valores absolutos, no tipo SS (9 casos). Os dois achados mais comuns foram tortuosidade venosa (12/26) e "black sunburst" (7/26). Conclusões: Observamos que a incidência de retinopatia por células falciformes aumentou após os 10 anos de idade e não evidenciamos achados da doença proliferativa. Portanto, enfatizamos a necessidade do exame oftalmológico precoce nos portadores de anemia falciforme, como forma de prevenir futuras complicações oculares.

Purpose: To point out the main fundoscopic findings in children with sickle cell trait hemoglobinopathies. Methods: We studied 26 patients with sickle cell hemoglobinopathies in the ophthalmological ward at the Hospital Universitário Onofre Lopes, Natal, RN. The patients were submitted to a research questionnaire and the results were statistically evaluated by the chi² test. Results: The average age was 10,6 years, with visual acuity equal or better than 20/25 in the majority, except for three eyes which presented other associated pathologies. The most frequent type was SS in 57.7% (15/26) of cases, followed by SC e SA in 15.4% (4/26) each, and by S-Thal in 11.5% (3/26). The frequency of sickle cell retinopathy was higher after 10 years of age, being more frequent, in relative values, in type S-Thal (100%) and, in absolute values, in type SS (9 cases). The two most common findings were venous tortuosity (12/26) and "black sunburst" (7/26). Conclusion: We observed that the incidence of retinopathy in sickle cell increased with age over 10 years and no evidence of proliferative disease was found. Therefore, we emphasize the need for early ophthalmological examination in carriers of sickle cell disease as a form of preventing future complications.