Bacterial antigen detection in cerebrospinal fluid by the latex agglutination test

Eighty purulent cerebrospinal fluid (CSF) samples from patients with clinical evidence of meningitis were studied using the Directigen latex agglutination (LA) kit to determine the presence of bacterial antigen in CSF. The results showed a better diagnostic performance of the LA test than bacterioscopy by Gram stain, culture and counterimmunoelectrophoresis (CIE), as far as Neisseria meningitidis groups B and C, and Haemophilus influenzae type b are concerned, and a better performance than bacterioscopy and culture considering Streptococcus pneumoniae. Comparison of the results with those of culture showed that the LA test had the highest sensitivity for the Neisseria meningitidis group C. Comparing the results with those of CIE, the highest levels of sensitivity were detected for N. meningitidis groups B and C. Regarding specificity, fair values were obtained for all organisms tested. The degree of K agreement when the LA test was compared with CIE exhibited better K indices of agreement for N. meningitidis groups B and C.

Autoantibodies before, during and after administration of recombinant interferon-alpha for chronic viral hepatitis

This study was undertaken to investigate the presence of autoantibodies in patients with chronic viral hepatitis B and C, before, during and after interferon-alpha (IFN-alpha) therapy and to study their relation to dose and type of IFN-alpha and response to treatment. Fifty patients with chronic hepatitis were divided in two groups, a control-group of 21 patients (10 type B and 11 type C) who were followed for 6 months without treatment and an IFN-group consisting of 29 patients (8 type B and 21 type C) who received IFN therapy for 6 months. Serum samples were tested for a range of antibodies at the start of the study, during therapy and at the end of the 6 month period. Antibodies tested for included: antinuclear, smooth muscle, antimitochondrial, parietal cell and thyroid microsomal. Four (8%) of the total patient group had autoantibodies at the beginning of the study (two in each group). During the follow-up period no patient in the control group developed antibodies compared with 3 (11%) patients in the treatment group. Autoantibodies developed in patients treated with higher doses of IFN and were found in those patients who tended to show a poor response to IFN-therapy. Further studies are needed to establish the relationship between poor response to IFN-alpha and development of autoantibodies.

Auditorias energéticas – definição de um plano de a��ão para edif��cios de servi��os

O setor dos edifícios representa perto de 40% do consumo de energia final na Europa e cerca de 30% no caso de Portugal [1]. Para fazer face a esta situaç��o foi elaborada e aprovada uma Diretiva Europeia Relativa ao Desempenho Energético dos Edifícios, que foi transposta a nível nacional atrav��s de um pacote legislativo assente em tr��s pilares, nomeadamente o Sistema Nacional de Certifica��ão Energética e da Qualidade do Ar Interior (SCE), o Regulamento dos Sistemas Energ��ticos de Climatização em Edif��cios (RSECE) e o Regulamento das Características de Comportamento Térmico dos Edifícios (RCCTE). Atuando ao nível da efici��ncia energ��tica o consumo de energia nos edif��cios pode diminuir para metade, para tal é necessário proceder-se à execu��ão de auditorias energéticas para poder determinar as soluções mais adequadas de forma a reduzir os desperdícios e custos associados ao consumo de energia. Nesta disserta��ão desenvolveu-se uma metodologia para a realiza��ão de auditorias energéticas em edifícios que assenta essencialmente em cinco etapas, nomeadamente: o planeamento, a análise do estado atual, o planeamento estrat��gico, a elaboraç��o de relat��rio e a implementação de medidas com acompanhamento de resultados. A aplicaç��o desta metodologia constitui uma grande ajuda na realização de auditorias energ��ticas conferindo uma maior qualidade �� sua execu��ão. De forma a validar a metodologia efetuada foi realizado o estudo de três casos práticos relativos a tr��s ag��ncias banc��rias (denominadas de A, B e C), em que duas delas pertencem a um projeto de eficiência energética que engloba 50 agências e uma outra que pertence a um outro projeto de apenas 3 agências. A metodologia segue a mesma lógica para as tr��s ag��ncias, no entanto, em termos de validaç��o, a última instalaç��o baseia-se nos consumos dos dados monitorizados em cont��nuo.

ASSESSMENT OF IVERMECTIN THERAPEUTIC EFFICACY ON THIRD-STAGE LARVAE OF Lagochilascaris minor IN MICE EXPERIMENTALLY INFECTED

In this study we evaluated the potential action of ivermectin on third-stage larvae, both at migratory and encysted phases, in mouse tissues after experimental infection with Lagochilascaris minor. Study groups I and II consisted of 120 mice that were orally administered 1,000 parasite eggs. In order to assess ivermectin action upon migratory larvae, group I (60 mice) was equally split in three subgroups, namely I-A, I-B, and I-C. On the 7th day after inoculation (DAI), each animal from the subgroup I-A was treated with 200 µg/Kg ivermectin while subgroup I-B was given 1,000 ��g/Kg, both groups received a single subcutaneous dose. To assess the drug action on encysted larvae, group II was equally split in three subgroups, namely II-A, II-B, II-C. On the 45th DAI each animal was treated with ivermectin at 200 ��g/Kg (subgroup II-A) and 1,000 µg/Kg (group II-B) with a single subcutaneous dose. Untreated animals of subgroups I-C and II-C were used as controls. On the 60th DAI all animals were submitted to larva search. At a dose of 1,000 µg/Kg the drug had 99.5% effectiveness on third-stage migratory larvae (subgroup I-B). Ivermectin efficacy was lower than 5% on third-stage encysted larvae for both doses as well as for migratory larvae treated with 200µg/Kg.

Avaliaç��o do papel da glutationa e do vacúolo como mecanismos de defesa contra a toxicidade induzida pelo chumbo na levedura Saccharomyces cerevisiae

A presen��a de metais pesados no meio ambiente deve-se, principalmente, a actividades antropogénicas. Ao contrário do Cu e do Zn, que em baixas concentra��ões são essenciais para o normal funcionamento celular, não se conhece para o chumbo nenhuma função biol��gica. O chumbo apresenta efeitos t��xicos, e considerado poss��vel agente carcinog��neo, sendo classificado como poluente prioritário pela Agencia de Protec��ão Ambiental dos EUA (US-EPA). O presente trabalho teve como objetivo avaliar o papel da glutationa e do vac��olo, como mecanismos de defesa, contra os efeitos tóxicos induzidos pelo chumbo, usando como modelo a levedura Saccharomyces cerevisiae. A levedura S. cerevisiae quando exposta a varias concentrações de chumbo, durante 3h, perde a viabilidade e acumula espécies reativas de oxig��nio (ROS). O estudo comparativo da perda de viabilidade e acumula��ão de ROS em células de uma estirpe selvagem (WT) e de estirpes mutantes, incapazes de produzir glutationa devido a uma deficiência no gene GSH1 (gsh1) ou GSH2 (gsh2) mostrou que as estirpes gsh1 ou(���gsh2 n��o apresentavam um aumento da sensibilidade ao efeito toxico do chumbo. No entanto, o tratamento de c��lulas da estirpe WT com iodoacetamida (um agente alquilante que induz a depleç��o de glutationa) aumentou a sensibilidade das células a presença de chumbo. Pelo contrário, o enriquecimento em GSH, atrav��s da incubaç��o de células WT com glucose e uma mistura de aminoácidos que constituem a GSH (acido L-glutâmico, L-cisteína e glicina), reduziu o stress oxidativo e a perda de viabilidade induzida por chumbo. A import��ncia do vacúolo, como mecanismo de defesa, foi avaliada através da utiliza��ão de um mutante sem qualquer estrutura vacuolar (��vps16) ou de mutantes deficientes na subunidade catalítica A (��vma1) ou B (��vma2) ou no proteol��tico - subunidade C (��vma3) da V-ATPase. As células da estirpe ƒ´vps16 apresentaram uma elevada suscetibilidade a presença de chumbo. As células das estirpes deficientes na subunidade A, B ou c da V-ATPase, apresentaram uma maior perda de viabilidade, quando expostas a chumbo, do que as células da estirpe WT, mas menor do que a da estirpe vps16 Em conclusão, os resultados obtidos, no seu conjunto, sugerem que a glutationa esta envolvida na defesa contra a toxicidade provocada por chumbo; todavia, a glutationa, por si só, parece não ser suficiente para suster o stress oxidativo e a perda de viabilidade induzida por chumbo. O vacúolo parece constituir um importante mecanismo de defesa contra a toxicidade provocada por chumbo. A V-ATPase parece estar envolvida na compartimentação de chumbo no vac��olo.

Prevalence of putative periodontopathogens from periodontal patients and healthy subjects in São Paulo, SP, Brazil

A. actinomycetemcomitans, B. forsythus, P. gingivalis, C. rectus, E. corrodens, P. intermedia, F. nucleatum, and T. denticola were identified from subgingival plaque from 50 periodontal patients and 50 healthy subjects. PCR products from each species showed a specific band and could be used to identify periodontal organisms from clinical specimens. Identical negative or positive results between PCR and culture occurred in 66% (A. actinomycetemcomitans) to 93% (F. nucleatum) of the samples. PCR detection odds ratio values for A. actinomycetemcomitans, B. forsythus, C. rectus, E. corrodens, P. intermedia, and T. denticola were significantly associated with disease having a higher OR values for B. forsythus (2.97, 95% CI 1.88 - 4.70). Cultures showed that A. actinomycetemcomitans, B. forsythus and P. intermedia were associated with periodontitis, however, P. gingivalis, C. rectus, E. corrodens and F. nucleatum were not significantly associated with the disease.

Molecular aspects of hepatic carcinogenesis

Exogenous agents correlated with hepatocellular carcinoma (HCC) have been identified and well characterized. These agents, including the different viruses that cause chronic hepatitis and cirrhosis, can lead to regenerative nodules and dysplastic nodules/adenomatous hyperplasia. These conditions associated with several molecular alterations of hepatocyte ultimately culminate in hepatocellular carcinoma. Recently, there has been a great progress in the identification of somatic and germinative mutations that may be correlated with the development of HCC, justifying a review on the subject. Hence, the factors involved in the process of hepatic carcinogenesis, such as infection by the hepatitis B and C viruses, with a special focus in the molecular alterations described in recent years are discussed herein, pointing out areas potentially relevant for clinical development.

p53 immunostaining pattern in Brazilian patients with hepatocellular carcinoma

Hepatocellular carcinoma (HCC) is an important type of cancer etiologically related to some viruses, chemical carcinogens and other host or environmental factors associated to chronic liver injury in humans. The tumor suppressor gene p53 is mutated in highly variable levels (0-52%) of HCC in different countries. OBJECTIVE: The objective of the present study was to compare the frequency of aberrant immunohistochemical expression of p53 in HCC occurring in cirrhotic or in non-cirrhotic patients as well as in liver cell dysplasia and in adenomatous hyperplasia. We studied 84 patients with HCC or cirrhosis. RESULTS: We detected p53 altered immuno-expression in 58.3% of patients in Grade III-IV contrasting to 22.2% of patients in Grade I-II (p = 0.02). Nontumorous areas either in the vicinity of HCC or in the 30 purely cirrhotic cases showed no nuclear p53 altered expression, even in foci of dysplasia or adenomatous hyperplasia. No significant difference was found among cases related to HBV, HCV or alcohol. CONCLUSION: The high frequency of p53 immunoexpression in this population is closer to those reported in China and Africa, demanding further studies to explain the differences with European and North American reports.

The influence of the human genome on chronic viral hepatitis outcome

The mechanisms that determine viral clearance or viral persistence in chronic viral hepatitis have yet to be identified. Recent advances in molecular genetics have permitted the detection of variations in immune response, often associated with polymorphism in the human genome. Differences in host susceptibility to infectious disease and disease severity cannot be attributed solely to the virulence of microbial agents. Several recent advances concerning the influence of human genes in chronic viral hepatitis B and C are discussed in this article: a) the associations between human leukocyte antigen polymorphism and viral hepatic disease susceptibility or resistance; b) protective alleles influencing hepatitis B virus (HBV) and hepatitis C virus (HCV) evolution; c) prejudicial alleles influencing HBV and HCV; d) candidate genes associated with HBV and HCV evolution; d) other genetic factors that may contribute to chronic hepatitis C evolution (genes influencing hepatic stellate cells, TGF-beta1 and TNF-alpha production, hepatic iron deposits and angiotensin II production, among others). Recent discoveries regarding genetic associations with chronic viral hepatitis may provide clues to understanding the development of end-stage complications such as cirrhosis or hepatocellular carcinoma. In the near future, analysis of the human genome will allow the elucidation of both the natural course of viral hepatitis and its response to therapy.

Acidentes de Serviço em Profissionais de Sa��de: Identificaç��o, Representa��ões e Comportamentos Face à Exposição Microbiológica Acidental

Este estudo de investigaç��o teve subjacente a preocupa��ão com a exposi��ão microbiol��gica acidental nos profissionais de saúde, sabendo que os acidentes de trabalho por exposi��ão microbiol��gica acidental constituem um dos principais riscos de transmissão ocupacional de infecções por contacto com sangue e outros fluidos corporais. Os agentes infecciosos mais frequentemente envolvidos neste tipo de acidente são os v��rus da hepatite B, C e o vírus da imunodefici��ncia humana (VIH). Teve como objectivo identificar e caracterizar os acidentes de trabalho por exposiç��o microbiológica acidental ocorridos nos profissionais de sa��de de um Hospital Central da região de Lisboa durante os anos de 2002 a 2006 e conhecer as Representações e os Comportamentos dos profissionais de saúde aquando da ocorrência de uma exposi��ão microbiol��gica acidental. Tratou-se de um estudo descritivo e exploratório, inserido numa abordagem quantitativa e qualitativa. Os participantes deste estudo foram os profissionais de saúde (m��dicos, enfermeiros e t��cnicos de diagnóstico e terapêutica) que tenham sofrido um acidente de trabalho com exposiç��o microbiológica acidental do ano de 2002 até Julho do ano de 2007, tendo-se constituído uma amostra não probabilística de 20 participantes utilizando a técnica de amostragem por conveniência e que se disponibilizaram a participar no estudo após o Consentimento Informado. Para a realiza��ão do estudo optou-se por recorrer a um conjunto de métodos de colheita de dados, privilegiando a análise documental através do questionário epidemiológico de caracterizaç��o e análise dos acidentes de trabalho e do processo de sa��de individual e da realizaç��o de uma entrevista semi-directiva no âmbito da qual se aplicou um questionário de caracterizaç��o sócio-demográfica, apoio social e actividades de tempos livres, aspectos relacionados com a percepção sobre os riscos a que se encontram expostos no ambiente hospitalar e com o circuito de notifica��ão do acidente de serviço/trabalho. Elaborou ��� se um guião de entrevista semi-directiva de acordo com os objectivos da investigaç��o. Optou-se por uma metodologia quantitativa no que diz respeito �� análise dos acidentes de trabalho que envolveram exposição microbiológica durante o per��odo em estudo e dos dados sócio-demográficos do question��rio aplicado no ��mbito da entrevista, através do programa informático pakage estat��stico ��Statistica/Program for Social Sciences-SPSS��� para Windows (versão 12). Posteriormente optou-se por uma metodologia qualitativa no que concerne ao tratamento dos dados relativos às entrevistas efectuadas, que consistiu na análise de conteúdo tem��tico das entrevistas. A análise dos resultados obtidos revelaram que os acidentes de trabalho ocasionados por material perfuro-cortante entre os profissionais de saúde são frequentes devido ao n��mero elevado de manipulação destes instrumentos, principalmente de agulhas e apresentam prejuízos aos profissionais e �� Instituição. Verifica-se que dos 305 acidentes notificados, a categoria profissional que tem o maior n��mero de notifica��ões é a dos enfermeiros com 142 acidentes no total dos anos representando 46,6%. A picada acidental é o tipo de acidente mais frequente com 205 acidentes o que representa 67% do total. O instrumento que esteve associado directamente com a ocorr��ncia destes acidentes foram as agulhas (41,6%), nomeadamente as agulhas subcutâneas (18,8%), as agulhas intravenosas (9,4%), as agulhas intramusculares (5%), outras agulhas (8,4%). As lâminas de bisturi representam 13,1% dos acidentes ocorridos nos anos de 2002 a 2006. Estes acidentes podem oferecer riscos �� saúde física e mental dos profissionais de saúde, com repercuss��es psicossociais, levando a mudanças nas rela��ões sociais, familiares e de trabalho. Da forma como o indiv��duo viv��ncia e ultrapassa a situa��ão e dos meios encontrados para lidar com o stress provocado pelo acidente, depende o seu bem-estar futuro na profiss��o e na Organização evitando o turnover. Todos os entrevistados manifestam a percepç��o que o maior risco na sua actividade é a relacionada com a exposi��ão a agentes biológicos com ênfase na preocupação em adquirir uma doença infecto-contagiosa. A percep��ão do risco de contamina��ão através do sangue e fluidos corporais est�� relacionada com a ideia do VIH conduzir a uma doença fatal e incurável apesar de existir uma perspectiva de esperan��a e qualidade de vida devido aos avan��os das terapêuticas actualmente. Os sentimentos e as emo��ões ocasionadas ap��s a exposi��ão microbiol��gica e manifestadas pelos participantes são a ansiedade, o medo, a angústia e a preocupação, raramente referem a frustra��ão e a revolta por se sentirem traídos, daí a import��ncia do apoio social que tiveram por parte da saúde ocupacional, da família e amigos, bem como dos colegas de trabalho, fundamental para ultrapassar um episódio cr��tico da sua vida. No entanto a representa��ão que têm das doenças como o VIH/SIDA e das hepatites B e C, como podem ser prevenidas e o nível de controlo que sentem exercer sobre os factores de transmiss��o, reflectem a percepção de que as medidas preventivas podem reduzir o risco de contamina��ão. Uma das conclusões deste estudo prende-se com a necessidade de reformulação do circuito de notificaç��o dos acidentes de trabalho, com o objectivo de diminuir o tempo e os vários constrangimentos durante o percurso. A análise dos dados referentes aos acidentes de serviço por exposi��ão microbiol��gica, os relatórios de análise das causas, a selec��ão e avaliação dos instrumentos aplicados promovem a criação de uma cultura de seguran��a. Como medidas organizacionais, a preven��ão pressupõe o treino e a formação dos profissionais de saúde nos procedimentos de preven��ão da transmiss��o da infec��ão, o incentivo à notifica��ão das exposiç��es e o desenvolvimento de um sistema adequado de monitorizaç��o e gestão das exposi��ões ocupacionais. Sendo que a preven��ão destes acidentes passa essencialmente pela introdução de dispositivos médicos com sistemas de segurança, por práticas de trabalho seguras, e pela forma��ão dos profissionais de saúde.

Crystallization and crystallographic analysis of the apo form of the orange protein (ORP) from desulfovibrio gigas

Acta Crystallographica Section F Structural Biology and Crystallization Communications Volume 65, Part 8

Characterization of Cryptococcus neoformans isolated from urban environmental sources in Goiânia, Goi��s State, Brazil

Cryptococcus neoformans is an opportunistic fungal pathogen that causes meningoencephalitis as the most frequent clinical presentation in immunocompromised patients, mainly in people infected by HIV. This fungus is an environmental encapsulated yeast, commonly found in soil enriched with avian droppings and plant material. A total of 290 samples of pigeon and the other avian droppings, soil, ornamental trees and vegetable material associated with Eucalyptus trees were collected to study environmental sources of Cryptococcus species in Goiânia, Goiás State. The determination of varieties, serotypes and the susceptibility in vitro to fluconazole, itraconazole and amphotericin B of C. neoformans isolates were performed. C. neoformans var. grubii (serotype A) was found in 20.3% (36/177) of pigeon dropping samples and in 14.3% (5/35) of samples of Eucalyptus. None of the environmental isolates of C. neoformans showed in vitro resistance to three antifungal agents. The knowledge of major route for human cryptococcal infection (inhalation of infectious particles from saprophytic sources) and a total of 60 C. neoformans isolates obtained from AIDS patients with cryptococcal meningitis between October 2001 and April 2002 justify the study of the habitats of these yeasts as probable sources of cryptococcosis in this city.

Tratamento Cir��rgico dos Defeitos do Septo Auriculo-Ventricular. Experiência Uni-Institucional

OBJECTIVES: Atrio-ventricular septal (AVSD) defects include a variable spectrum of congenital malformations with different forms of clinical presentation. We report the surgical results, from a single institution, with this type of congenital cardiac malformation. Patients with hypoplasia of one of the ventricles were excluded from this analysis. POPULATION: Between November of 1998 and June of 2005, 49 patients with AVSD were operated on by the same team and in the same department. The average age was 37.3 months (medium 6 months) and 31 patients were female. In 38 patients (78%) an inter-ventricular communication was present (AVSD-complete) and of these, 26 were of the type A of Rastelli, being 13 of type B or C. The age for defect correction of the complete form was of 5.5 months, palliative surgery was not carried out on any of the patients. Associated lesions included: Down's syndrome in 22 patients (45%), patent arterial duct in 17 patients (35%), severe AV regurgitation in 4 patients (8%), tetralogy of Fallot in two (4%) and sub-aortic stenosis in one patient (2%). Pre-operatively 10 patients presented severe congestive heart failure and two were mechanically ventilated. RESULTS: Complete biventricular correction was carried out in all patients. The average time on bypass (ECC) was 74.1+/-17.5 min. and time of aortic clamping was 52.0+/-12.9 min. The complete defects were corrected by the double patch technique, and in all patients the mitral cleft was closed, except in two with single papillary muscle. There was no intra-operative mortality, but hospital mortality was 8%(4 patients), due to pulmonary hypertension crises, in the first 15 post-operative days. The mean ventilation time was of 36.5+/-93 hours (medium 7 h) and the average ICU stay was of 4.3+/-4.8 days (medium 3 days). The minimum follow-up period is 1 month and the maximum is 84 months (medium 29.5 months), during which time 4 re-operations (8%) took place: two for residual VSD's and two for mitral regurgitation. There was no mortality at re-do surgery. At follow up there was residual mitral regurgitation, mild in 17 patients and moderate in two. Four other patients presented with minor residual defects. CONCLUSIONS: The complete correction of AVSD can be carried out with acceptable results, in a varied spectrum of anatomic forms and of clinical severity. Despite the age of correction, for the complete forms, predominantly below 12 months, pulmonary hypertension was the constant cause for post operative mortality. Earlier timing of surgery and stricter peri-operative control might still improve results.

Susceptibility of two karyotypic forms of Anopheles aconitus (Diptera: Culicidae) to Plasmodium falciparum and P. vivax

Four laboratory-raised colonies of two karyotypic forms of Anopheles aconitus, i.e., Form B (Chiang Mai and Phet Buri strains) and C (Chiang Mai and Mae Hong Son strains), were experimentally infected with Plasmodium falciparum and P. vivax using an artificial membrane feeding technique and dissected eight and 12 days after feeding for oocyst and sporozoite rates, respectively. The results revealed that An. aconitus Form B and C were susceptible to P. falciparum and P. vivax, i.e., Form B (Chiang Mai and Phet Buri strains/P. falciparum and P. vivax) and Form C (Chiang Mai and Mae Hong Son strains/P. vivax). Comparative statistical analyses of the oocyst rates, average number of oocysts per infected midgut and sporozoite rates among all strains of An. aconitus Form B and C to the ingroup control vectors, An. minimus A and C, exhibited mostly no significant differences, confirming the high potential vector of the two Plasmodium species. The sporozoite-like crystals found in the median lobe of the salivary glands, which could be a misleading factor in the identification of true sporozoites in salivary glands were found in both An. aconitus Form B and C.

Results of Surgery for Ebstein Anomaly: a Multicenter Study from the European Congenital Heart Surgeons Association

OBJECTIVE: Since most centers' experience with Ebstein anomaly is limited, we sought to analyze the collective experience of participating institutions of the European Congenital Heart Surgeons Association with surgery for this rare malformation. METHODS: The records of all 150 patients (median age 6.4 years) who underwent surgery for Ebstein anomaly in the 13 participating Association centers between January 1992 and January 2005 were reviewed retrospectively. Patients with congenitally corrected transposition were excluded. RESULTS: Most patients (81%) had Ebstein disease type B or C and significant functional impairment (61% in New York Heart Association class III or IV) and 16% had prior operations. Surgical procedures (n = 179) included valve replacement (n = 60, 33.5%), valve repair (n = 49, 27.3%), 1(1/2) ventricle repair (n = 46, 25.6%), palliative shunt (n = 13, 7.26%), and other complex procedures (n = 11, 6.14%). There were 20 hospital deaths (operative mortality 13.3%) after valve replacement in 5 patients, valve repair in 3, 1(1/2) ventricle repair in 7, palliative procedures in 3, and miscellaneous procedures in 2. Younger age and palliative procedures were univariate risk factors for operative death, but only age was an independent predictor on multivariable analysis. CONCLUSIONS: Most patients coming to surgery presented in childhood and were significantly symptomatic. More than half underwent valve replacement or repair, but a considerable proportion had severe disease necessitating 1(1/2) ventricle repair or palliative procedures. Operative mortality did not differ significantly among repair, replacement, and 1(1/2) ventricle repair but was associated with palliative procedures for severe disease early in life, young age being the only independent predictor of operative death.