The purpose of this paper was to define the histologic distrito the endemic (African) and sporadic forms of Burkitt lymbution, clinical features, and treatment response of childhood phoma, which differ in their clinical presentation, regional non-Hodgkin lymphoma (NHL) in northeastern Brazil. We incidence, association with Epstein-Barr virus, and biologic Patients and methodsThere was a striking predominance of the small noncleaved cell (Burkitt) subtype, which occurred in 92 of the 98 children and adolescents diagnosed with NHL. Subsequent analyses Between January 1980 and October 1987, a total of 98 chilfocused on these patients. The majority (n = 84) had advanced dren were diagnosed with non-Hodgkin lymphoma at the parental refusal/abandonment of therapy (10%). Epstein-BarrDiagnostic imaging studies were performed as indicated. virus (EBV) was detected in tumor cells from eight of the 11The pathologic diagnosis was established by histologic Of the 98 patients evaluated during the study period, 92 had small noncleaved cell histology. There were only five cases of lymphoblastic lymphoma and one of large cell histology. Having confirmed the predominance of Burkitt lymIntroduction phoma among the pediatric patients with NHL, we then focused on these 92 cases. Records were reviewed to evaluate The non-Hodgkin lymphomas of childhood are predominantly clinical and biological features, type of treatment, and outhigh-grade extranodal tumors that include the lymphoblastic, come. When sufficient paraffin-embedded tumor tissue was large cell, and small noncleaved cell subtypes. 1 The relative available, in situ RNA/RNA cytohybridization using plasmids frequency of these subtypes varies with geographic location.containing EBV-encoded small nuclear RNA was performed In the United States, the distribution is almost equal, 2 whereas at St Jude Children's Research Hospital to detect the presence small noncleaved cell tumors (Burkitt lymphoma) account for of Epstein-Barr virus DNA. 6 In these cases, the histologic diagthe majority of childhood NHLs in equatorial Africa. 3,4 In nosis was also reviewed and confirmed by a pediatric hematonortheastern Brazil -an area in which we have established a pathologist at St Jude (CWB). cooperative clinical program -anecdotal evidence suggests an increased overall incidence of lymphoma. However, to our knowledge, there have been no reports focusing on pediatric Hodgkin or non-Hodgkin lymphomas in this region. TreatmentWe noted an apparent predominance of the small noncleaved cell histology among children treated for NHL at a There were two distinct treatment periods. From 1980 to major pediatric cancer center in Recife, Brazil. In the present 1983, all consenting patients were treated with induction study, we evaluated the accuracy of this clinical impression chemotherapy similar to that used in the LSA 2 L 2 regimen and assessed long-term treatment outcome. We also sought to described by Wollner et al, 7 followed by maintenance therapy determine the relationship of the small noncleaved...
Congenital aneurysm of the right atrium is described in a 1-year-old girl who presented with cardiomegaly and symptoms of paroxysmal supraventricular tachycardia. Echocardiography and cineangiography both established the definitive diagnosis and surgical resection was successful. The rarity of this condition is pointed out and its main features outlined.
This is a report of a nine-year-old boy with both mitral stenosis and regurgitation and extensive endomyocardial fibrosis of the left ventricle. Focus is given to the singularity of the fibrotic process, with an emphasis on the etiopatho-genic aspects.
We report the case of a 33-year-old male with primary seminoma of the anterior mediastinum with initial clinical manifestations suggestive of heart disease.
RBCCV 44205-175 LIMA, R.; ESCOBAR, M.; ALECRIM, R. ; ALVES, 1. ; LlNS, T.; ARRAES, N.; VICTOR, E.; WANDERLEY NETO, J.; TORRES, L.D.F; ELIAS, D.O.; WISZOMIRSKY, A .D.B. ; MENDONÇA, J. T.; CARVALHO, M.R.; COSTA , R.K.; LIRA, V .; LAGRECA, R. -Programa Nordeste para transplante cardíaco " NE-Tx" : experiência atual.Rev. Bras. Cir. Cardiovasc., 7(3) : [165][166][167][168][169][170][171][172][173] 1992.RESUMO: Os autores relatam a presente experiência clínica do programa NE-Tx, que é integrado por quatro diferentes Centros, bem como suas peculiaridades regionais. Foi idealizado um programa com baixo custo e integração regional. Na investigação pré-operatória, foi valorizado o nível social e intelectual do receptor e sua relação com os familiares . A busca de órgão à distância foi suprimida, com o deslocamento do receptor até o local da disponibilidade do órgão. No pós -operatório não se utilizou dosagem sérica de ciclosporina, tendo os pacientes sido controlados com monitorização seriada da função renal. Com essa estratégia, foram operados 7 pacientes, no período compreendido entre 19.7.86 e 1.11 .91 ., em três diferentes Centros Integrados. Todos eram do sexo masculino e a idade variou de 17 a 50 anos. Três (42,8%) pacientes eram portadores de miocardiopatia chagásica, 2 (28,5%) de miocardiopatia idiopática, 1 (14,3%) de miocardiopatia hipertrófica e 1 (14,3%) de miocardiopatia reumática. Três (42 ,8%) pacientes vieram de Centros diferentes: 1 de Natal para Recife, 1 de Maceió para Aracajú e·um terceiro de Aracajú para Maceió. Dois (28 ,6%) pacientes faleceram : um no período de pós-operatório imediato , de maneira ignorada e outro no pós-operatório tardio, devido a crise de rejeição. A mortalidade imediata foi de 14,3% e a tardia de 14,3%. Os restantes 5 (71,4%) pacientes encontram-se evoluindo bem, tendo o follow-up variado de 2 a 48 meses. A incidência de infecção , rejeição e demais problemas relacionados com o pós-operatório tardio é superponível com outros Centros. O programa mostrou-se eficiente, prático, compatível com nossa realidade e reproduz resultados de outras experiências.DESCRITORES : transplante cardíaco, humano; transplante cardíaco , ortotópico . LIMA, A.; ESCOBAR, M.; ALECRIM, A.; ALVES, 1.; LlNS, T.; ARRAES, N.; VICTOR, E ; WANDERLEY NETO, J.; TORRES , L.D.F; ELIAS, D.O.; WISZOMIRSKY, A .D.B.; MENDONÇA, J. T.; CARVALHO, M.A.; COSTA, A.K.; LIRA, V.; LAGRECA, A. -Programa Nordeste para transplante cardíaco "NE-Tx": experiência atual. Rev. Bras. Cir. Cardiovase., 7(3): 165-173, 1992. RBCCV 44205-175 LIMA, R. ; ESCOBAR, M.; ALECRIM, R.; ALVES, 1.; LlNS, T.; ARRAES, N.; VICTOR, E; WANDERLEY NETO, J.; TORRES, L.D.F; ELIAS , D.; WISZOMIRSKY, A .D.B.; MENDONÇA, J. T.; CARVALHO, M.R.; COSTA, A.K.; LIRA, V.; LAGRECA, R. -NE-Tx North East Brazil heart transplant program: current-experience . Rev. Bras. Cir. Cardiovase., 7(3):165-173, 1992.ABSTRACT: The authors report their current clinical experience with the "NE-Tx" program, which covers four different centres, as well as its regional peculiarities. The p...
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