Factors related to the readiness of Brazilian chronic pediatric patients to transition to care in adult clinics

Abstract Objective Advances in medicine have increased the life expectancy of pediatric patients with chronic illnesses, and challenges with the guided transition of adolescents and young adults from pediatric clinics to adult clinics have grown. The aim of this study was to better understand readiness and factors related to this transition process in Brazil. Method In this cross-sectional study of 308 patients aged from 16 to 21 years under follow-up in pediatric specialties, the degree of readiness for transition was assessed using the Transition Readiness Assessment Questionnaire (TRAQ) and its domains. Associations with demographic data, clinical data, socio-economic level, medication adherence, family functionality, and parental satisfaction with health care were evaluated. Results The median TRAQ score was 3.7 (3.2 - 4.2). Better readiness was associated with female patients, socio-economic class A-B, current active employment, higher level of education, not failing any school year, attending medical appointments alone, functional family, and a good knowledge of disease and medications. A low correlation was observed between TRAQ and age. TRAQ presented good internal consistency (alpha-Cronbach 0.86). In the multiple linear regression, TRAQ score showed a significant association with female gender, advanced age, socio-economic class A-B, better knowledge of disease and medications, and independence to attend appointments alone. Conclusion TRAQ instrument can guide healthcare professionals to identify specific areas of approach, in order to support adolescents with chronic disease to set goals for their own personal development and improve their readiness to enter into the adult healthcare system. In this study, some factors were related to better TRAQ scores.

Follow-up of children with sickle cell anemia screened with transcranial Doppler and enrolled in a primary prevention program of ischemic stroke

ABSTRACT Background: Stroke is a serious complication of sickle cell anemia (SCA). The transcranial Doppler (TCD) is the risk-screening tool for ischemic strokes. The objective of the study was to describe the clinical progression of children with SCA who presented with high risk for stroke by TCD or relevant changes by magnetic resonance angiography (MRA) and underwent the regular transfusion program (RTP) and/or hydroxyurea (HU) treatment between 2007 and 2018. Method: This was a neonatal retrospective/prospective cohort study with children born between 1999 and 2014 with the homozygotic form (HbSS) or Sβ0-thalassemia who underwent TCD at least once. Results: Of the 718 children screened during this period, 675 had HbSS and 43 Sβ0-thalassemia. In 54 children (7.5%), all with HbSS, a high-risk TCD (n = 45) or, when the TCD was inconclusive, an MRA with cerebral vasculopathy (n = 9) was used for detection. Of these, 51 started the RTP and the families of three refused treatment. Of the 43 children with a highrisk TCD who initiated the RTP, 29 (67.4%) reverted to low risk. In 18 of them (62%), HU was started at the maximum tolerated dose (MTD) before transfusion discontinuation. None of these 29 patients had a stroke. Eight children (18.6%) maintained a high-risk TCD, even using the RTP/HU and two had a stroke. Conclusions: The TCD was confirmed as a viable tool for tracking patients with a risk for stroke. The RTP was effective in preventing the primary event. New strategies are necessary to prevent stroke using HU and new drugs, in addition to bone marrow transplantation.

Genetic, laboratory and clinical risk factors in the development of overt ischemic stroke in children with sickle cell disease

ABSTRACT Cerebrovascular disease, particularly stroke, is one of the most severe clinical complications associated with sickle cell disease and is a significant cause of morbidity in both children and adults. Over the past two decades, considerable advances have been made in the understanding of its natural history and enabled early identification and treatment of children at the highest risk. Transcranial Doppler screening and regular blood transfusions have markedly reduced the risk of stroke in children. However, transcranial Doppler has a limited positive predictive value and the pathophysiology of cerebrovascular disease is not completely understood. In this review, we will focus on the current state of knowledge about risk factors associated with ischemic stroke in patients with sickle cell disease. A search of PubMed was performed to identify studies. Full texts of the included articles were reviewed and data were summarized in a table. The coinheritance of alpha-thalassemia plays a protective role against ischemic stroke. The influence of other genetic risk factors is controversial, still preliminary, and requires confirmatory studies. Recent advances have established the reticulocyte count as the most important laboratory risk factor. Clinical features associated with acute hypoxemia as well as silent infarcts seem to influence the development of strokes in children. However, transcranial Doppler remains the only available clinical prognostic tool to have been validated. If our understanding of the many risk factors associated with stroke advances further, it may be possible to develop useful tools to detect patients at the highest risk early, improving the selection of children requiring intensification therapy.

Interleukin-10 haplotypes are not associated with acute cerebral ischemia or high-risk transcranial Doppler in a newborn cohort of 395 children with sickle cell anemia

ABSTRACT Background: The etiology of stroke, a severe complication of sickle cell anemia, involves inflammatory processes. However, the pathogenetic mechanisms are unknown. The aim of this study was to evaluate the influence of interleukin-10 polymorphisms and haplotypes on the risk of acute cerebral ischemia and high-risk transcranial Doppler in 395 children with sickle cell anemia from the state of Minas Gerais, Brazil. Methods: Interleukin-10 haplotypes were determined by polymerase chain reaction-restriction fragment length polymorphism and sequencing. The outcomes studied were acute cerebral ischemia and high-risk transcranial Doppler. Clinical data were retrieved from the children's records. Results: There was no statistically significant difference in the frequencies of polymorphisms and haplotypes between children with and without acute cerebral ischemia or children with or without high-risk transcranial Doppler. These data are consistent with a previous report that showed an absence of association between interleukin-10 plasma levels and high-risk transcranial Doppler velocity in children with sickle cell anemia. Conclusion: Interleukin-10 haplotypes were not associated with the risk of acute cerebral ischemia or high-risk transcranial Doppler velocity in children with sickle cell anemia from the state of Minas Gerais, Brazil.

Ecocardiograma: da Solicitação do Exame pelo Pediatra à Realização pelo Cardiologista Pediátrico / Echocardiography: The Examination Request by the Pediatriciant Achieving the Pediatric Cardiologist

Fundamento: O ecocardiograma é uma ferramenta diagnóstica utilizada para avaliar anomalias cardíacas e esclarecer dúvidas quanto ao caráter benigno de certos achados do exame clínico cardiovascular. Objetivos: Identificar os principais motivos da solicitação do ecocardiograma pelo pediatra e avaliar o grau de ansiedade dos familiares gerado por essa conduta. Métodos: Foram incluídos pacientes com idade inferior a 18 anos, sem diagnóstico prévio de cardiopatia e encaminhados para o primeiro exame de ecocardiograma. Os familiares responsáveis pelos pacientes responderam a um questionário para avaliar o grau de ansiedade deles desde o pedido até a realização do ecocardiograma, com pontuação entre 0 e 19. Os dados foram analisados através de porcentagens, desvio padrão e teste T de Student. Resultados: Estudados 30 pacientes com idade média de 4,45 anos. O motivo de encaminhamento mais prevalente foi sopro cardíaco (23 casos), dos quais 70% não tiveram a hipótese de cardiopatia congênita confirmada após o ecocardiograma. A pontuação média no questionário de ansiedade foi 11 ± 6, sendo relacionado significativamente à presença de cardiopatia, com média de 13,0 ± 5,3 vs. 9,3 ± 5,2 no grupo sem a hipótese da mesma (p = 0,007). Conclusão: O achado de sopro foi o principal motivo para solicitação de ecocardiograma pelo pediatra. A confirmação de cardiopatia foi maior nos menores de 1 ano e com achado de sopro. Apesar do grau de ansiedade ter sido maior no grupo dos pacientes que tiveram o diagnóstico de cardiopatia congênita, este não foi desprezível nos familiares no grupo dos pacientes sem cardiopatia

Background: Echocardiography is a diagnostic tool used to evaluate cardiac anomalies and clarify doubts about the benign nature of certain findings of cardiovascular clinical examination. Objectives: To identify the main reasons for the request of echocardiography by pediatrician and evaluate the degree of anxiety of the family generated by this conduct. Methods: We included patients younger than 18 years without previous diagnosis of heart disease and referred for the first echocardiogram. Family members responsible for the patients answered a questionnaire to assess the degree of their anxiety from order to the performance of the echocardiogram, with scores between 0 and 19. Data were analyzed using percentages, standard deviation and Student's t test. Results: Studied 30 patients with a mean age of 4.45 years. The most prevalent reason for referral was heart murmur (23 cases), of which 70% patients had no congenital heart disease hypothesis confirmed, after echocardiography. The average score on the anxiety questionnaire was 11 ± 6 significantly associated with the presence of heart disease, with a mean of 13.0 ± 5.3 vs. 9.3 ± 5.2 in the group without the possibility of it (p = 0.007). Conclusion: The murmurs findings was the main reason for echocardiography request by the pediatrician. Confirmation of heart disease was higher in children under 1 year and with murmurs findings. Despite the anxiety level was higher in the group of patients who were diagnosed with congenital heart disease, this was not negligible in the family in the group of patients without heart disease

Clinical, hematological and genetic data of a cohort of children with hemoglobin SD

Introduction The hemoglobin FSD is very uncommon in newborn screening programs for sickle cell disease. In the program of Minas Gerais, Brazil, the clinical course of children with hemoglobin SD was observed to be heterogeneous. The objective of this study was to estimate the incidence (1999­2012) and to describe the natural history of a cohort of newborns with hemoglobin SD. Methods Isoelectric focusing was the primary method used in newborn screening. Polymerase chain reaction-restriction fragment length polymorphism and gene sequencing were used to identify mutant alleles and for haplotyping. Gap-polymerase chain reaction was used to detect alpha-thalassemia. Results Eleven cases of hemoglobin S/D-Punjab and eight of Hb S-Korle Bu were detected. Other variants with hemoglobin D mobility were not identified. All hemoglobin D-Punjab and hemoglobin Korle Bu alleles were associated with haplotype I. Among the children with hemoglobin S/D-Punjab, there were four with the ßS CAR haplotype, six with the Benin haplotype, and one atypical. Results of laboratory tests for hemoglobin S/D-Punjab and hemoglobin S-Korle Bu were: hemoglobin 8.0 and 12.3 g/dL (p-value <0.001), leukocyte count 13.9 × 109/L and 10.5 × 109/L (p-value = 0.003), reticulocytes 7.5% and 1.0% (p-value <0.001), hemoglobin F concentration 16.1% and 6.9% (p-value = 0.001) and oxygen saturation 91.9% and 97% (p-value = 0.002), respectively. Only hemoglobin S/D-Punjab children had acute pain crises and needed blood transfusions or hydroxyurea. Those with the Benin ßS haplotype had higher total hemoglobin and hemoglobin F concentrations compared to the CAR haplotype. Transcranial Doppler was normal in all children. Conclusion The clinical course and blood cell counts of children with hemoglobin S/D-Punjab were very similar to those of hemoglobin SS children. In contrast, children with hemoglobin S-Korle Bu had clinical course and blood cell counts like children with the sickle cell trait.

Indicações atuais para anticoagulacão em crianças com Cardiopatias Congênitas / Current indications for anticoagulation in children with Congenital Heart Disease

Os avanços nas técnicas cirúrgicas e manejo clínico aumentaram a sobrevida das crianças com cardiopatia congênita. Eventos tromboembólicos representam importante causa de morbimortalidade nestes pacientes, principalmente no pós-operatório da cirurgia de Fontan e implante de próteses, acrescido do risco adicional do seu tratamento. Particularidades próprias da idade, fatores hemodinâmicos e limitações na investigação de trombose contribuem para a dificuldadede definir diretrizes para anticoagulação em crianças. Os medicamentos mais usados na fase aguda são: heparina não fracionada e de baixo peso molecular, enquanto em longo prazo os antagonistas da vitamina K, principalmente a varfarina. O manejo seguro de anticoagulação nesta faixa etária é dificultado pela estreita faixa terapêutica, interação alimentar, uso simultâneo de vários medicamentos, além da dificuldade de punção venosa frequente e, com frequência os níveis desejáveis não são atingidos. Ainda faltam dados que deem suporte para recomendação de anticoagulação em crianças. Os novos anticoagulantes como os inibidores diretos da trombina e os inibidores seletivos do fator Xa, são empregados em adultos, principalmente com fibrilação atrial, o uso pediátrico ainda não está definido. Esta revisão sumariza as indicações atuais do uso de anticoagulantes e antiagregantes plaquetários em crianças com cardiopatia congênita.

Advances in surgical techniques and clinical management have led to increased survival ofchildren with congenital heart disease. Thromboembolic events represent a major cause of morbidity and mortality in these patients, particularly following Fontan surgery and the implantation of prosthesis, added to the risk of their treatment. Characteristics such as age, hemodynamic factors and limitations in the investigation of thrombosis have contributed to the difficulty of defining guidelines for anticoagulation in children. The most commonly used medications in the acute phase are and low molecular weight heparin, and in the long term, vitamin K antagonists, particularly warfarin. The safe management of anticoagulation in this age range is hindered by the narrow therapeutic range, food interactions, simultaneous use of other medications, and the difficulty of frequent venous puncture. As a result, the desired levels are often not reached. There is also a lack of data to support the recommendation of anticoagulation in children. The pediatric use of the new anticoagulants, such as direct thrombin inhibitors and selective factor Xa inhibitors used in adults, particularly with atrial fibrillation, is not yet defined. This review summarizes the current indications of the use of anticoagulants and platelet antiaggregants in children with congenital heart disease.

Abnormal transcranial Döppler ultrasonography in children with sickle cell disease

BACKGROUND: Stroke is a potentially fatal complication of sickle cell disease in children between 2-16 years and transcranial Döppler has been recommended as a screening method in these cases. OBJECTIVE: The main goal of this study was to correlate transcranial Döppler results to complications related to stroke in sickle cell disease and baseline characteristics of the population. METHODS: This was an observational study of children and adolescents with ages between 2-16 years with sickle cell disease who were followed in three centers. RESULTS: From January 2008 to July 2009, 902 patients were enrolled in this study. The median age was 6.5 years (range: 1.8-15.8), 52.3% were male, 74.4% had hemoglobin SS; 221 (28.6%) had at least one complication associated with sickle cell disease. A total of 773 patients performed transcranial Döppler; in 91.2% this was a method of screening. Conditional or abnormal transcranial Döppler results were more common in patients with sickle cell disease complications versus those without complications (ODDS ratio = 3.18; 95% Confidence interval = 1.92-5.27). There was a significant difference in the frequency of conditional or abnormal transcranial Döppler results in patients with abnormal laboratory results compared to those without abnormalities (OR=4.03); 95% confidence interval = 2.30-7.06. CONCLUSIONS: Conditional or abnormal transcranial Döppler results were significantly more frequent in patients with complications of sickle cell disease confirming the increased risk of stroke in this subgroup of patients. This observation reinforces the recommendation of transcranial Döppler as a screening test for all patients with sickle cell disease with ages between 2 and 16 years.

Hipertensão pulmonar nas cardiopatias congênitas: implicações terapêuticas e prognósticas / Pulmonary hypertension in congenital cardiopathy therapeutic and prognostic implications

A Hipertensão arterial pulmonar (HAP) é uma importante complicação na evolução das cardiopatias congênitas de hiperfluxo pulmonar. A síndrome de Eisenmenger (SE) é o espectro máximo desta condição e se caracteriza por inversão do shunt com cianose e irreversibilidade até o presente momento. Nos últimos anos, novas opções terapêuticas se expandiram e a terapia avançada (TA), com o uso de vasodilatadores pulmonares, tornou-se uma opção atraente para estes pacientes. Estudos recentes têm demonstrado melhora da qualidade de vida e capacidade física desta população em uso de TA. Entretanto, a melhor forma de tratamento ainda é a prevenção pela correção cirúrgica da cardiopatia na época adequada. A presente revisão tem como objetivo abordar o prognóstico e tratamento na HAP associada a cardiopatias congênitas na atualidade e discutir sobre futuras abordagens terapêuticas, como a possibilidade de posterior correção cirúrgica de pacientes em TA, antes considerados inoperáveis.

Brazilian Guidelines for transcranial doppler in children and adolescents with sickle cell disease

BACKGROUND: Sickle cell disease is the most common monogenic hereditary disease in Brazil. Although strokes are one of the main causes of morbidity and mortality in these patients, the use of transcranial Doppler to identify children at risk is not universally used. OBJECTIVE: To develop Brazilian guidelines for the use of transcranial Doppler in sickle cell disease children and adolescents, so that related health policies can be expanded, and thus contribute to reduce morbidity and mortality. METHODS: The guidelines were formulated in a consensus meeting of experts in transcranial Doppler and sickle cell disease. The issues discussed were previously formulated and scientific articles in databases (MEDLINE, SciELO and Cochrane) were carefully analyzed. The consensus for each question was obtained by a vote of experts on the specific theme. RESULTS: Recommendations were made, including indications for the use of transcranial Doppler according to the sickle cell disease genotype and patients age; the necessary conditions to perform the exam and its periodicity depending on exam results; the criteria for the indication of blood transfusions and iron chelation therapy; the indication of hydroxyurea; and the therapeutic approach in cases of conditional transcranial Doppler. CONCLUSION: The Brazilian guidelines on the use of transcranial doppler in sickle cell disease patients may reduce the risk of strokes, and thus reduce the morbidity and mortality and improve the quality of life of sickle cell disease patients.

Isolation and characterization of carbendazim degrading trichoderma harzianum rifai mutants

Mutants of Trichoderma harzianum Rifai, obtained after ultraviolet (UV) light exposure, showed high resistant to the fungicide benomyl. A mutant (2B6)was capable of degrading carbendazim, other fungicide of the benzimidazole fungicide. This mutant degraded 41.5% of the molecule within five days. This and others mutants (2B1 and 2B2) presented variation in size and frequency of uni-nucleated and/ or bi-nucleated spores compared to the wild type. Four primers generated RAPDs patterns that allowed the mutant to be differentiated from the wild-type. It is concluded that using UV mutagenization, it is feasible to obtain strains of T. harzianum with improved pesticide degradation ability.

Uso da angiografia rotacional 3D (3D-RA) em portadores de cardiopatias congênitas: experiência de 53 casos / Use of 3D rotational angiography (3D-RA) in congenital heart disease: experience with 53 patients

Introdução: Os avanços no diagnóstico e tratamento das cardiopatias congênitas estão relacionados com os avanços na obtenção de imagens. As representações anatômicas obtidas com tomografia computadorizada, ressonância magnética e ecocardiografia têm ajudado, mas esses métodos não conseguem obter de forma precisa os dados hemodinâmicos. A angiografia rotacional 3D (3D-RA), um novo método de reconstrução tridimensional realizado no laboratório de cateterismo, tem sido amplamente utilizada em procedimentos neurológicos e urológicos. Nosso objetivo foi avaliar o desempenho da 3D-RA na avaliação das cardiopatias congênitas. Método: Revisão dos exames dos pacientes com cardiopatia congênita encaminhados para estudo hemodinâmico e que foram submetidos a aquisição de imagens pela 3D-RA. Foi utilizado equipamento Philips Allure FD10 e contraste de baixa osmolaridade para as angiografias. Resultados: No total, foram avaliados 53 pacientes, sendo utilizados, em média, 2,2 ± 1,1 ml/kg de contraste por paciente. Foi possível observar detalhes anatômicos não revelados em angiografias planares em 23% dos pacientes. Adicionalmente, 49% dos pacientes tiveram benefícios das imagens 3D para decisões terapêuticas. A exposição à radiação não foi estatisticamente diferente das angiografias planares. Nenhum paciente apresentou complicações relacionadas ao método. Conclusão: A 3D-RA forneceu informações que algumas das angiografias convencionais não fornecem e que ajudaram no tratamento de pacientes selecionados portadores de cardiopatia congênita. O emprego desse método poderá reduzir o número de aquisições de imagem por procedimento e, como consequência, limitar a exposição do paciente à radiação e ao contraste.

Background: Advances in the diagnosis and treatment of congenital heart disease are associated to advances in imaging techniques. Anatomic images obtained by computed tomography scan, magnetic resonance imaging (MRI) and echocardiography have been useful but cannot provide accurate hemodynamic data. 3D rotational angiography (3D-RA) is a new 3D reconstruction method carried out inthe cath lab that has been widely used in neurological and urological procedures. This study was aimed at evaluating the use of 3D-RA in the diagnosis and treatment of congenitalheart disease. Method: Review of catheterization results of patients with congenital heart disease referred for diagnostic assessment in which the 3D reconstruction method was employed. Philips Allure FD 10 equipment and low osmolarity contrast medium were used for angiographies. Results: Overall,53 patients were reviewed and 2.2 ± 1.1 mL/kg of contrast medium were used per patient. Anatomic details notpreviously shown by 2D angiographies were observed in 23% of the patients. Furthermore, 3D-RA imaging was usedto make treatment decisions in 49% of the patients. Exposure to radiation was not statistically different from 2D angiography. None of the patients had complications related to the method. Conclusion: 3D-RA provided information not usually seen by conventional angiography which was useful in thetreatment of selected patients with congenital heart disease. The use of 3D-RA may reduce the number of imaging tests per procedure and as a consequence, limit patient exposureto radiation and contrast media.

Microbial degradation of sulfentrazone in a Brazilian rhodic hapludox soil

Sulfentrazone is amongst the most widely used herbicides for treating the main crops in the State of São Paulo, Brazil, but few studies are available on the biotransformation of this compound in Brazilian soils. Soil samples of Rhodic Hapludox soil were supplemented with sulfentrazone (0.7 µg active ingredient (a.i.) g-1 soil) and maintained at 27ºC. The soil moisture content was corrected to 30, 70 or 100 percent water holding capacity (WHC) and maintained constant until the end of the experimental period. Herbicide-free soil samples were used as controls. Another experiment was carried out using soil samples maintained at a constant moisture content of 70 percent WHC, supplemented or otherwise with the herbicide, and submitted to different temperatures of 15, 30 and 40º C. In both experiments, aliquots were removed after various incubation periods for the quantitative analysis of sulfentrazone residues by gas chromatography. Herbicide-degrading microorganisms were isolated and identified. After 120 days a significant effect on herbicide degradation was observed for the factor of temperature, degradation being higher at 30 and 40º C. A half-life of 91.6 days was estimated at 27º C and 70 percent WHC. The soil moisture content did not significantly affect sulfentrazone degradation and the microorganisms identified as potential sulfentrazone degraders were Nocardia brasiliensis and Penicillium sp. The present study enhanced the prospects for future studies on the bio-prospecting for microbial populations related to the degradation of sulfentrazone, and may also contribute to the development of strategies for the bioremediation of sulfentrazone-polluted soils.

Stents farmacológicos: existe lugar para seu uso? / Pharmacological stents: is there room for their use?

As intervenções coronarianas percutâneas representam importante estratégia de revascularização do miocárdio. Na evolução dos procedimentos de intervenção percutânea sobre as coronárias, os stents farmacológicos ou recobertos com drogas surgiram como opção mais eficaz que os não-farmacológicos, por diminuírem, de forma significativa,os riscos de reestenose angiográfica. Mais recentemente, surgiram publicações apontando para um risco maior de trombose associada aos stents farmacológicos. Sendo a trombose um evento agudo que, do ponto de vista fisiopatológico, representa o substrato anatômico da síndrome coronariana aguda, inclusive do infarto agudo do miocárdio, os resultados destas publicações desencadearam dúvidas quanto à segurança dos stents recobertos com drogas. Os stents farmacológicos não oferecem ganho adicional, se comparados aos stents não farmacológicos, no tratamento da doença coronariana nas indicações primárias para uso dos mesmos (as chamadas indicações on label, estabelecidas pelo FDA, quando aprovou o uso destes dispositivos). Há consistência entre vários ensaios publicados apontando que o uso que extrapola estas indicações, e, que tem sido o mais comum, associa-se a riscos mais altos de morte e infarto. Os resultados com o uso de stents farmacológicos e não farmacológicos se equivalem, portanto deve ser utilizado aquele com melhor relação custo-benefício.

Forâmen oval patente: embolia paradoxal e enxaqueca / Patent foramen ovale: paradoxical embolism and migraine

O forâmen oval patente com ou sem aneurisma septal tem sido associado a vários problemas, como acidente vascular isquêmico, embolia paradoxal, doença da descompressão, platipnéia ortostática e enxaqueca, muito embora a relação causa-efeito entre forâmen oval patente e esses eventos ainda não esteja bem esclarecida. Avanços tecnológicos ofereceram mais opções terapêuticas, como a oclusão percutânea do forâmen oval patente. Todavia, evidência definitiva de sua eficácia ainda em discussão, e o fechamento parece ser recomendado apenas como profilaxia secundária para recorrência de acidente vascular cerebral isquêmico.

Função sistólica do ventrículo esquerdo pela ecocardiografia em crianças e adolescentes com osteossarcoma tratados com doxorrubicina com e sem dexrazoxane / Left ventricular systolic function assessed by echocardiography in children and adolescents with osteosarcoma treated with doxorubicin alone or in combination with dexrazoxane

OBJETIVO: Avaliar o comportamento da função sistólica do ventrículo esquerdo (VE) pela ecocardiografia em pacientes com osteossarcoma tratados com doxorrubicina com e sem dexrazoxane. MÉTODOS: Foram estudados 55 pacientes com osteossarcoma com ou sem metástase submetidos à quimioterapia (QT) com seis ciclos de doxorrubicina, divididos em dois grupos, conforme o uso de dexrazoxane. Grupo I: 37 pacientes, os quais receberam dexrazoxane (28 do sexo masculino, com média de idade de 15,4 anos). Grupo II: 18 pacientes, que não receberam dexrazoxane (15 do sexo masculino, com média de idade de 15,1 anos). Foram realizadas quatro avaliações ecocardiográficas: 1) antes do início da QT (avaliação inicial); 2) até duas semanas após o terceiro ciclo; 3) até duas semanas após o quinto ciclo e 4) até quatro semanas após o sexto ciclo da QT (avaliação final). A função sistólica do VE foi avaliada pela porcentagem de encurtamento (PE) com o ecocardiograma. Alteração da função contrátil ou toxicidade miocárdica foi definida com valores de PE iguais ou inferiores a 29 por cento e/ou diminuição da PE, em valor absoluto, igual ou superior a 10 unidades do valor inicial de cada paciente. RESULTADOS: Não houve diferença significativa entre os grupos quanto à idade, sexo e raça. A dose cumulativa de doxorrubicina foi significantemente maior no grupo II em todas as fases do tratamento: 174 x 203 mg/m²; 292 x 338 mg/m² e 345 x 405 mg/² (p < 0,0001). A ocorrência de disfunção sistólica do VE, de acordo com os critérios previamente definidos, foi de sete indivíduos no grupo I (18,92 por cento) e de dois no grupo II (11,1 por cento), diferença não significativa (p=0,248). A análise de variância com medidas repetidas não mostrou diferença significativa nas médias da PE ao longo do período de estudo (p=0,967). Entretanto foi encontrada diferença significativa (p=0,029) entre as médias da PE dos grupos I e II nas avaliações 2 (35,67 x 37,21 por cento), 3 (34,95 x 38,47 por cento) e 4...

OBJECTIVE: To evaluate left ventricular (LV) systolic function by means of echocardiography in patients with osteosarcoma treated with doxorubicin alone or in combination with dexrazoxane. METHODS: The study analyzed 55 patients with osteosarcoma, with or without metastasis, undergoing a six-cycle chemotherapy regimen of doxorubicin, who were divided into two groups according to dexrazoxane use. Group I: Thirty-seven patients who did not receive dexrazoxane (28 males, average age 15.4 years). Group II: Eighteen patients who did receive dexrazoxane (15 males, average age 15.1 years). Four echocardiographic evaluations were performed: 1) before the beginning of the chemotherapy (initial evaluation); 2) up to two weeks after the third cycle; 3) up to two weeks after the fifth cycle; and 4) up to four weeks after the sixth cycle of chemotherapy (final evaluation). The left ventricular systolic function was assessed by the fractional percentage of systolic shortening (FS percent) on echocardiography. Alterations in the contractile function or cardiac toxicity were defined as FS percent values equal to or lower than 29 percent, and/or by a drop in FS percent by an absolute value equal to or greater than 10 units of the baseline value of each patient. RESULTS: No significant difference as to age, gender, and race was observed between the groups. The cumulative dose of doxorubicin was significantly higher in group II throughout all phases of the treatment: 174 x 203 mg/m²; 292 x 338 mg/m² and 345 x 405 mg/² (p < 0.0001). According to previously established criteria, the incidence of LV systolic dysfunction was not significantly different (p=0.248) between patients in group I (18.92 percent) and patients in group II (11.1 percent). The variance analysis with repeated measurements did not show significant differences in the means of fractional percentage of systolic percentage (FS percent) throughout the study (p=0.967). However, a significant difference (p=0.029) was...

Hipertensão arterial pulmonar associada a cardiopatias congênitas / Pulmonary hypertension in congenital heart disease

A hipertensão arterial pulmonar é frequentemente encontrada nas cardiopatias congênitas associadas a hiperfluxo pulmonar secundário a grandes shunts da esquerda para a direita. A hipertensão pulmonar é um fator complicador importante no tratamento desses pacientes, podendo impedir a correção cirúrgica da cardiopatia naqueles com doença vascular pulmonar já estabelecida. O diagnóstico precoce e o conhecimento da história natural do defeito cardíaco usualmente levam à prevenção da doença vascular pulmonar, por permitir seu fechamento em tempo adequado. Nos pacientes com doença vascular pulmonar irreversível, o tratamento clínico convencional continua indicado, embora novos medicamentos demonstrem seus efeitos potenciais sobre variáveis hemodinâmicas e sobrevida. Após a falência ao tratamento clínico otimizado, existe a opção de transplante pulmonar.

Padrões de pressão e de velocidade de fluxo num modelo in vitro de anastomose sistêmico-pulmonar. Importância para análise do estado funcional / Patterns of pressure and flow velocity distribution in a in vitro model of systemic to pulmonary artery anastomosis. Importance for functional evaluation

A função das anastomoses sistêmicos-pulmonares(S-P) e a detecção de obstrução parcial são dificeis pela ecocardiografia. Objetivo: Analisar os padrões de pressão e de distribuição espacial da velocidade do fluxo num modelo in vitro de S-P com ou sem obstrução parcial. Método: Foi utilizado um modelo de fluxo contínuo: dois tubos - circulações sistêmica(S) e pulmonar(P), foram conectados por outro - anastomose sistêmico-pulmonar (S-P). O modelo foi estudado sem obstrução e com uma obstrução longa na S-P, com medida direta das pressões em S,P e na S-P antes e após a obstrução, e análise simultânea com Doppler contínuo (DC) e mapeamento de fluxo em cores (MFC). Resultados: Sem obstrução a pressão de 5 (36,6 mmHg) foi maior do que em P(9,4mmHg) e S-P(6,3 mmHg)(p menor 0,05), sem diferença significante entre S-P e P. O maior gradiente de pressão por cateter (30,4mmHg) e por DC(27,9mmHg) foi na junção de S com S-P com sinais de aceleração de fluxo proximal ao MFC. Com obstrução, as pressões em S(95,8 mmHg) e S-P(75,4 mmHg) antes da obstrução não foram diferentes entre si, mas maiores do que as pressões de P(35,9 mmHg) e S-P(33 mmHg) após a obstrução (p menor 0,05), com gradientes de pressão pelo DC semelhantes e com sinais de aceleração proximal à junção de S com S-P, e antes da abstrução em S-P ao MFC. Conclusão: Com obstrução de S-P observou-se gradiente de opressão baixo ao DC na junção de S com S-P, e altos no trajeto da S-P com sinais de aceleração de fluxo, sem obstrução os maiores gradientes de pressão e aceleração de fluxo ocorreram na junção de S com S-P.

Production of phenol-oxidases and peroxidases by fungi isolated from irrigated rice

A proposta deste trabalho foi estudar o potencial das linhagens fúngicas, consideradas potenciais degradadoras dos herbicidas quinclorac e propanil, para produção de enzimas ligninolíticas. Oito linhagens fúngicas foram cultivadas em meio de cultura líquido King's B suplementado com 0,05% de Remazol Brilliant Blue R (RBBR) e em meio de cultura líquido contendo farelo de trigo como substrato. Os sistemas enzimáticos avaliados foram: lignina peroxidase, manganês peroxidase e lacases. Os resultados demonstraram padrões diferenciados quanto à produção de enzimas ligninolíticas entre as linhagens, sendo que as maiores atividades enzimáticas estiveram relacionadas à produção de lignina peroxidase. Das oito linhagens, duas (P3SA1F e P11SA2F) apresentaram descoloração do RBBR, sugerindo a possibilidade de sua aplicabilicação em estudos de.