Could nanotechnology improve exercise performance? Evidence from animal studies

Abstract This review provides the current state of knowledge regarding the use of nutritional nanocompounds on exercise performance. The reviewed studies used the following nanocompounds: resveratrol-loaded lipid nanoparticles, folic acid into layered hydroxide nanoparticle, redox-active nanoparticles with nitroxide radicals, and iron into liposomes. Most of these nutritional nanocompounds seem to improve performance in endurance exercise compared to the active compound in the non-nanoencapsulated form and/or placebo. Nutritional nanocompounds also induced the following physiological and metabolic alterations: 1) improved antioxidant activity and reduced oxidative stress; 2) reduction in inflammation status; 3) maintenance of muscle integrity; 4) improvement in mitochondrial function and quality; 5) enhanced glucose levels during exercise; 6) higher muscle and hepatic glycogen levels; and 7) increased serum and liver iron content. However, all the reviewed studies were conducted in animals (mice and rats). In conclusion, nutritional nanocompounds are a promising approach to improving exercise performance. As the studies using nutritional nanocompounds were all conducted in animals, further studies in humans are necessary to better understand the application of nutritional nanocompounds in sport and exercise science.

Folha de amendoeira como aditivo em dietas para Betta splendens / Use of almond leaf as additive in diets for Betta splendens

O objetivo do presente estudo foi avaliar o desempenho zootécnico, os parâmetrosmorfométricos, o comportamento social, a viabilidade econômica e a análise microbiológica da água de cultivo e de tecidos corpóreos de Betta splendens alimentados com folha da amendoeira Terminalia catappa (FFDA) como aditivo. Foram utilizados 28 machos de Bettas, acondicionados individualmente em aquários de 1,5L, por 50 dias. O experimento foi realizado em delineamento experimental inteiramente ao acaso, com quatro tratamentos: 0,00%; 0,25%; 0,50% e 0,75% de inclusão da FFAD e sete repetições. Ao término do experimento, foram avaliados os parâmetros: desempenho zootécnico (ganho de peso diário, consumo de ração, conversão alimentar aparente, taxa de eficiência proteica, taxa de crescimento específico e fator de condição), morfométrico (comprimento total, padrão e da cabeça, altura, índice de perfil e índice de cabeça), comportamento social, viabilidade econômica da ração, análise microbiana do conteúdo intestinal, filé e escama e análise microbiológica da água. Pela ANOVA, pelo teste de Tukey e pela regressão (P>0,05), os parâmetros: peso final, ganho de peso, comprimento padrão, comprimento total e taxa de crescimento específico foram influenciados pelos tratamentos (P<0,05), apresentando um efeito quadrático. Assim, recomenda-se o nível de 0,50% de Terminalia catappa como aditivo em dietas de Betta splendens.(AU)

The objective of this study was to evaluate the performance, morphometric parameters, social behavior, economic viability, the presence of enterobacteria in the intestinal contents and a microbiological analysis of the water culture of Betta splendens fed with almond-tree-leaf flour (ATLF) as an additive. Twenty-eight male B. splendens were individually put in 1.5 L aquariums. The experiment was done in a completely randomized experimental design with four treatments: 0.00%; 0.25%; 0.50% and 0.75%, of the ATLF, dehydrated Terminalia catappa with seven repetitions each. At the end of the experimental period, the parameters were evaluated: performance (daily weight gain, feed intake, apparent feed conversion, protein efficiency rate, specific growth rate and condition factor), morphometric (total length, standard head, height, profile index and head index), social behavior, economic viability of the feed, microbial analysis of intestinal contents, fillet and scales, and microbiological analysis of the water. Though ANAVA, Tukey test and regression analysis (P> 0.05), the parameters: final weight, weight gain, standard length, total length and specific growth rate were influenced by the treatments (P< 0.05), presenting a quadratic effect. Therefore, the 0.50% level of Terminalia catappa is recommended as additive in Betta splendens diets.(AU)

2-Hydroxy-4-methylthio butanoic acid and DL-methionine for Japanese quails in production.

An experiment was performed using 1,000 laying Japanese quails to assess the availability of two alternative dietary methionine sources. Treatment 01 = Basal Feed that is deficient in digestible methionine + cystine (Met + Cys). The other treatments were constituted by Met + Cys levels of 0.8, 1.60 and 2.40 g/kg, supplemented with DL-Methionine-99%, HMTBA-88% and HMTBA-84%, being 10 treatments in total. The following characteristics were studied: feed intake (g/bird/day), egg production (egg/day × 100), egg weight (g/egg), egg mass (g/egg), feed conversion per egg dozen (kg feed/dozen eggs), feed conversion per egg mass (kg feed/kg eggs), relative yolk weight (g/100 g of egg), relative albumen weight (g/100 g of egg), relative shell weight (g/100 g of egg), shell thickness (mm) and specific gravity (g/cm3 ). In general result comment, supplemental methionine sources must be included in the poultry diet. The different methionine sources affect the performance of quails, and the increase in the levels within each source improves the performance variables. Significant effect was observable on performance variables and egg quality variables, being that DLM-99% is superior to the other sources. The HMTBA-88% source is superior to the HMTBA-84% source for the same aforementioned variables. In conclusion, the bioefficacy values of the HMTBA-88% and HMTBA-84% sources compared to the DLM-99% source on an equimolar basis were 81 and 79%, respectively, for the performance variables, and 83 and 74 while the methionine sources were equivalent for the variables related to egg quality.

Exigência de proteína bruta para juvenis de curimatã-pacu / Crude protein requeriment to curimatã-pacu juveniles

O objetivo deste estudo foi determinar a exigência de proteína bruta (PB) de juvenis de curimatã-pacu (Prochilodus argenteus). O experimento foi conduzido por um período de 60 dias, utilizando um delineamento inteiramente ao acaso, com quatro tratamentos (dieta contendo 24,0; 28,0; 32,0 e 36,0% de PB) e cinco repetições cada, em que 100 juvenis (peso inicial de 0,33 ± 0,01g) foram distribuídos em 20 aquários experimentais com volume de 130L, sendo considerada como unidade experimental uma caixa com cinco peixes, sob recirculação de água. Avaliaram-se o desempenho produtivo, o crescimento heterogêneo, os parâmetros morfométricos, fisiológicos e a viabilidade econômica das rações em função do nível de PB. Pela análise de regressão, foi verificado um aumento linear nos parâmetros de desempenho, morfométricos e fisiológicos, com o aumento do nível proteico nas rações. Quando comparados pelo teste de Tukey, o melhor resultado (P>0,05) foi obtido com 36% de PB. Verificou-se menor heterogeneidade de crescimento dos peixes à medida que se aumentavam os níveis de PB na ração. Assim, considerando também a viabilidade econômica, recomenda-se a utilização de rações com 36% de proteína bruta para juvenis de curimatã-pacu.(AU)

The aim of this study was to determinate crude protein (CP) requirement to curimatã-pacu juveniles (Prochilodus argenteus). The experiment was conducted during 60 days in a completely randomized design, with four treatments (24.0, 28.0, 32.0 and 36.0% CP of diet) and five replications, where a hundred juvenile fishes (initial weight de 0.33 ± 0.01g), were distributed in twenty tanks (130L each). Each tank with five juveniles was considered an experimental unit, with water recirculation. Performance, heterogeneous growth, morphometric and physiological parameters, and economic viability of the rations according to the CP levels were evaluated. For the regression analysis, a linear increase in performance, heterogeneous growth and morphometric and physiological parameters with increased of the protein in rations were observed. When compared by the Tukey test (P>0.05), the best results were obtained with 36% CP. There was lower fish growth heterogeneity as the levels of CP in the diet increased. Thus, taking into consideration the economic viability it is recommended to use feed containing 36% of crude protein to curimatã-pacu juveniles.(AU)

Cultivo in vitro de Brosimum gaudichaudii Tréc. (Moraceae) / In vitro cultivation of Brosimum gaudichaudii Tréc. (Moraceae)

O objetivo desta pesquisa foi avaliar o potencial organogenético de entrenós, discos foliares, e raízes de Brosimum gaudichaudii utilizando 12 diferentes combinações dos fitorreguladores 6-benzilaminopurina e ácido naftaleno acético, em meio MS (diluído à metade), sólido (6,5 g de ágar), e com 20 g.L-1 de sacarose. Independentemente das combinações hormonais testadas verificou-se a formação de calos friáveis (2 a 20 mm de diâmetro) em 90% dos entrenós usados como explantes. No entanto, os tratamentos testados não foram capazes induzir calos ou gemas em raízes e em discos foliares. O estudo anatômico revelou a formação de meristemóides nas regiões mais externa e mais interna dos calos. Os resultados obtidos poderão servir de base para novos testes de indução de calos na espécie.

The objective of this research was to evaluate the organogenic potential of internodes, leaf discs and roots of Brosimum gaudichaudii using 12 different combinations of the plant growth regulators 6-benzylaminopurine and naphthalene acetic acid in MS medium (half strength), solid medium (6.5 g agar) and sucrose medium (20 g.L-1). Regardless the hormonal combination tested, we observed the formation of friable calluses (2 - 20 mm wide) in 90% of the internode explants. However, the treatments were not able to induce callus or buds on roots and leaf discs. The anatomical analysis revealed meristemoid formation in the outer and inner regions of the calluses. The results may serve as the basis for further testing of callus induction in this species.

Medicinal plants used in Rondônia, Western Amazon, Brazil / Plantas medicinais usadas em Rondônia, Amazônia Ocidental, Brasil

This study refers to the use of medicinal plants by populations in the Western Amazon and provides information that can be used in phytochemical studies. It draws upon the traditional knowledge regarding the use of medicinal plants in five regions of the state of Rondônia, in the Brazilian Amazon, focusing on native species. The field research was carried out in five municipalities of the state of Rondônia: Ariquemes, Buritis, Candeias do Jamari, Cujubim and Itapoa do Oeste, characterized by primary economic sectors: agriculture, cattle farming, plant extraction and mineral exploration. Structured interviews were applied to 227 persons chosen because of their prestige in the communities in relation to the knowledge and use of medicinal plants, identifying the therapeutic purpose, parts of the plant used and methods of preparation. The species were taxonomically identified. The ethnobotanic knowledge (inferred by the number of uses of medicinal plants per person) was correlated with the Brazilian region of origin, age, and gender of the interviewees. According to the collected data, 34 botanical families and 53 native species were identified. Of the 53 species, only 7 occur exclusively in the Amazon Forest: Theobroma grandiflorum (Willd. ex Spreng.) K. Schum., Psidium densicomum Mart. ex DC, Piper cavalcantei Yunck., Pilocarpus microphyllus Stapf ex Wardlew., Euterpe oleracea Mart., Croton cajucara Benth., Baccharis altimontana G. Heiden. The most common disorders treated with the plants were kidney problems, influenza, generalized infections and inflammations, malaria and high blood pressure. Leaves were the most used parts in preparations. Barks, fruits, roots, flowers, stems, seeds, oils, buds, tubercles, and rhizomes were also mentioned. Thirteen forms of preparations were recorded, and infusion and decoction were the most used. Syrups, juices, flour, sap, oil and parts of the plant blended with milk, honey and coffee or flamed, macerated and in the form of poultice, were also found. Persons from the Southeast region had more information compared with persons from the other regions; persons in the age groups between 50-59, 60-69 and 70-79 had more information in comparison with the other age groups; and the female interviewees mentioned significantly more uses of medicinal plants than the male ones. The relatively small number of native Amazon species identified can be the result of the loss of knowledge about medicinal plants in the Amazon because of internal migration, extinction of local indigenous groups, increasing urbanization and consequent globalization of the lifestyles.

Este estudo se refere ao uso de plantas medicinais por populações da Amazônia Ocidental, fornece informações passíveis de utilização em estudos fitoquímicos e resgata o conhecimento tradicional a respeito do uso de plantas medicinais em cinco regiões do estado de Rondônia na Amazônia Brasileira com foco nas espécies nativas. A pesquisa de campo foi realizada nos cinco municípios de Rondônia: Ariquemes, Buritis, Candeias do Jamari, Cujubim e Itapoã do Oeste, caracterizados por atividades econômicas primárias: agricultura, pecuária e extrativismo vegetal e mineral. Entrevistas estruturadas foram aplicadas a 227 pessoas escolhidas por seu prestígio nas comunidades em relação ao conhecimento e uso de plantas medicinais, identificando a finalidade terapêutica, as partes das plantas utilizadas, e os métodos de preparação. As espécies foram taxonomicamente identificadas. O conhecimento etnobotânico (inferido a partir do número de citações por entrevistado) foi correlacionado com a região de origem, idade e gênero dos entrevistados. De acordo com os dados coletados, 34 famílias botânicas e 53 espécies nativas foram identificadas. Dentre as 53 espécies, apenas sete ocorrem exclusivamente na Amazônia: Theobroma grandiflorum (Willd. ex Spreng.) K.Schum., Psidium densicomum Mart. ex DC, Piper cavalcantei Yunck., Pilocarpus microphyllus Stapf ex Wardlew., Euterpe oleracea Mart., Croton cajucara Benth., Baccharis altimontana G. Heiden. As afecções mais comuns tratadas com plantas foram: problemas de fígado, gripe, infecções e inflamações generalizadas, malária, e hipertensão arterial. As folhas são as partes mais utilizadas nas preparações. Também foram mencionadas cascas, frutos, raízes, flores, caules, sementes, óleos, brotos, tubérculos e rizomas. Treze formas de preparação foram registradas sendo mais comuns as infusões e decoctos. Outras formas de preparação observadas foram xarope, suco, farinha, seiva, óleo, partes vegetais misturadas com leite, mel ou café, flambada, macerada e em forma de cataplasma. Pessoas da região Sudeste apresentaram maior número de informações do que as outras regiões; faixas etárias entre 50-59, 60-69 e 70-79 apresentaram maior número de citações que os outros grupos etários; e as mulheres mencionaram significativamente mais usos medicinais em comparação com os homens. O número relativamente pequeno de espécies nativas amazônicas identificadas pode ser um resultado da perda de conhecimento sobre plantas medicinais na Amazônia, devido à migração interna, extinção dos grupos indígenas locais, crescente urbanização e consequente globalização dos estilos de vida.

Use of enzymes in diets with different percentages of added fat for broilers / Uso de enzimas em dietas com diferentes porcentagens de adição de gordura para frangos de corte

We assessed the extent to which the removal of fat source, and consequently its compounds, such as linoleic acid, can affect the performance of broilers. We used 600 male Cobb 500 day old chicks. The birds were distributed in a completely randomized experimental design, with five treatments and six replicates of 20 birds each. The treatments were: (T1) diet - positive control (PC), which met the nutritional needs; (T2) diet - negative control (CN), a reduction of 100kcal/kg and low linoleic acid content; (T3): diet - negative control reformulated for low linoleic acid content and a set of Quantum phytase XT and Econase XT 25 (BAL + QFit-Eco), (T4): diet - negative control reformulated, with the percentage of linoleic acid adjusted to an intermediate value between the value of the diet and diet CP and CN to use a set of Quantum phytase XT and XT Econase 25 (IAL + QFit-Eco) and (T5): diet - negative control reformulated, with the percentage of linoleic acid adjusted to a value similar to that of the positive control diet and joint use of Quantum phytase XT and XT Econase 25 (AAL + QFit-Eco). The joint use of Quantum Phytase and Econase promoted improvement in the performance of broilers from 1 to 21 days. The greatest weight gain was obtained with diets containing percentages of total fat and linoleic acids. Dietary supplementation with enzymes resulted in higher levels of calcium in the tibia, whatever the percentage of linoleic studied.

Avaliou-se até que ponto a retirada de fonte de gordura e, consequentemente, de seus compostos, como o ácido linoleico, pode afetar o desempenho dos frangos de corte. Foram utilizados 600 pintos de um dia, machos da linhagem Cobb 500. As aves foram distribuídas num delineamento experimental inteiramente ao acaso, com cinco tratamentos e seis repetições de 20 aves cada. Os tratamentos foram: (T1) dieta-controle positivo (CP), que atendeu às necessidades nutricionais; (T2) dieta-controle negativo (CN), com redução de 100kcal de EM/kg e baixo teor de ácido linoleico; (T3): dieta- controle negativo reformulada, para baixo teor de ácido linoleico e com uso conjunto de Quantum fitase XT e Econase XT 25 (BAL+ Qfit-Eco); (T4): dieta-controle negativo reformulada, com porcentagem de ácido linoleico ajustada para um valor intermediário entre o valor da dieta CN e da dieta CP e com uso conjunto de Quantum fitase XT e Econase XT 25 (IAL + Qfit-Eco) e (T5): dieta-controle negativo reformulada, com porcentagem de ácido linoleico ajustada para um valor semelhante ao da dieta-controle positivo e com uso conjunto de Quantum fitase XT e Econase XT 25 (AAL+ Qfit-Eco ). O uso conjunto de Quantum Fitase e Econase promoveu melhora no desempenho dos frangos de corte de um a 21 dias. O maior ganho de peso foi obtido com dietas que continham porcentagens de gorduras totais e de ácido linoleico. A suplementação com as enzimas resultou em maior teor de cálcio nas tíbias, independentemente da porcentagem de ácido linoleico estudada.

Substituição de minerais inorgânicos por orgânicos na alimentação de poedeiras semipesadas / Substitution of inorganic minerals for organic minerals in the diet of semi heavy layers

Avaliou-se o efeito da suplementação dietética de fontes orgânicas de microminerais para galinhas poedeiras sobre o desempenho e a qualidade do ovo. Foram utilizadas 216 aves de linhagem comercial, distribuídas num delineamento inteiramente ao acaso, com quatro tratamentos e nove repetições, com seis aves por parcela. O experimento teve duração de 140 dias, divididos em cinco ciclos de 28 dias. Os tratamentos consistiram de uma dieta basal formulada para atender às exigências nutricionais das aves, suplementada com minerais na forma inorgânica ou porcentagens de minerais orgânicos: T1 - Basal + 100% minerais inorgânicos; T2 - Basal + 100% minerais orgânicos; T3 - Basal + 66% minerais orgânicos; T4 - Basal + 33% minerais orgânicos. A produção de ovos, peso do ovo, massa de ovo, conversão alimentar por massa e dúzia de ovo, porcentagem de albúmen, gravidade específica, espessura de casca e margem bruta relativa apresentaram melhores resultados quando os minerais inorgânicos são substituídos em 66% pelos orgânicos.

This study evaluated the effect of the supplementation with organic microminerals on performance and egg quality of semi-heavy layers. Dekalk Brown layers (n=216) were distributed according to a completely randomized design into four treatments, nine repetitions and six birds per repetition. The experiment lasted 140 days and was comprised of five 28 day cycles. The basal diet was formulated to fulfill the nutritional requirements and was supplemented with organic or inorganic minerals, resulting in four treatments: T1 - basal + 100% inorganic minerals; T2 - basal + 100% organic minerals; T3 - basal + 66% organic minerals; T4 - basal + 33% organic minerals. Egg production, egg weight, egg mass, feed conversion for dozen and mass, albumen percentage, specific gravity, shell thickness and relative gross margin have better results when the inorganic minerals are replaced with 66% organic.

Characterization of the spleen B-cell compartment at the early and late blood-stage Plasmodium chabaudi malaria.

Polyclonal B-cell activation is a feature of the early spleen cell response to blood-stage Plasmodium chabaudi malaria. Immunity to blood-stage malaria is guaranteed by the generation of B cells able to produce parasite-specific antibodies mainly from the immunoglobulin (Ig)G2a isotype. In the present study, we characterized the spleen B-cell compartment during blood-stage P. chabaudi infection. The numbers of B220(+) and B220(LOW) CD138(+) (plasma) cells increased sharply between days 4 and 7 post-infection (p.i.). At this time B220(+) cells expressed surface (s)IgM, but nearly all B220(LOW) CD138(+) cells showed concomitantly intracellular (i)IgM and IgG2a. Both follicular and marginal zone B cells were activated expressing high amounts of CD69. At day 40 p.i., B220(LOW) CD138(+) cell population was still increased but, differently from acute infection, 61.1% of these cells were positive for iIgG2a while only 14.2% expressed iIgM. Moreover, at days 20 and 40 p.i., 29.2% and 13.0% of B220(+) cells expressed sIgG2a, respectively. According to cell size and expression of CD80, CD86, CD11b, CD44 and CD38, B220(+) sIgG2a(+) cells had a phenotype characteristic of activated/memory B cells. Furthermore, 14.1% of B220(+) sIgG2a(+) cells at day 30 p.i. expressed a marginal zone B-cell phenotype. Importantly, B cells from 40-day-infected mice were very efficient in presenting parasite antigens leading to proliferation of both CD4(+) and CD8(+) cells. Our results contribute for understanding the dynamics of B cells during P. chabaudi infection, underlying the mechanisms of antigen presentation and antibody production, which are essential for the acquisition of protective immunity against malaria.

Influence of the polyclonal activation induced by Plasmodium chabaudi on ongoing OVA-specific B- and T-cell responses.

Infection by Plasmodium chabaudi results in polyclonal activation, massive proliferation and differentiation of lymphocytes with parasite-unrelated specificities. To verify if polyclonal activation includes experienced B and T lymphocytes and if it modifies pre-established cytokine and Ig-isotype patterns, mice were immunized with ovalbumin (OVA) in alum, a condition that favours T helper 2/immunoglobulin G1 (Th2/IgG1) responses, and infected with P. chabaudi 7 or 80 days later. Polyclonal activation markedly increased the number of anti-OVA Ig-secreting cells in the spleen, an effect more patent in mice infected 7 days after OVA immunization, but also evident in mice infected after 80 days. The Ig-isotype profile predefined by immunization was not qualitatively modified by polyclonal activation. Thus, although P. chabaudi infection preferentially induces IgG2a, the expanded anti-OVA response is dominated by IgG1. Polyclonal expansion of the anti-OVA response did not yield an enlarged memory B-cell pool that could be recalled months later by OVA boosting. Moreover, polyclonal activation of anti-OVA IgG1-secreting cells did not increase this antibody in serum, a probable consequence of the high Ig turnover observed during infection. When OVA-specific T-cell cytokines were evaluated, we observed an increase of both interleukin-4 (IL-4) and interferon-gamma (IFN-gamma) in mice infected 7 days after immunization, whereas in those infected after 80 days, only IL-4 was augmented. These results suggest that polyclonal activation expands experienced B- and T-cell compartments, preserving their antibody and cytokine patterns.

Most parasite-specific CD8+ cells in Trypanosoma cruzi-infected chronic mice are down-regulated for T-cell receptor-alphabeta and CD8 molecules.

The present study shows that CD8+ T lymphocytes expressing low levels of T-cell receptor (TCR)alphabeta, CD8 and CD3 accumulate in the spleen, blood, peritoneum and liver, but not in the lymph nodes of mice chronically infected with Trypanosoma cruzi. Analysis of spleen lymphocytes reveals that most CD8LOW TCRLOW T cells have an experienced phenotype (CD44HIGH CD62LLOW and CD45RA,B,CLOW). These cells have small size, lack activation markers such as CD69, CD25 and CD11b (Mac-1), and do not spontaneously secrete cytokines, suggesting they are at the resting state. When stimulated in vitro with T. cruzi-infected macrophages, TCRLOW CD8LOW T cells behave as parasite-specific memory cells, readily responding with interferon-gamma (IFN-gamma) production. Indeed, among parasite-activated CD8+ lymphocytes, IFN-gamma production was mostly due to TCRLOW CD8LOW cells. Upon in vitro stimulation with anti-CD3/CD28 monoclonal antibodies, down-regulated cells produce IFN-gamma and tumour necrosis factor-alpha, but not interleukin IL-10 or IL-4. Our results indicate that despite parasite persistence, most T. cruzi-specific experienced CD8+ cells are resting. Nevertheless, when encountering infected macrophages these cells differentiate to Tc1 effectors.

Cardiovascular manifestations in Marfan syndrome.

INTRODUCTION: Cardiovascular complications are the major cause of morbidity and mortality in Marfan syndrome (MS), a common connective tissue disorder. Currently it is considered that the prognosis and morphologic characteristics in infantile Marfan syndrome may be quite different from those reported in older patients. The objective of this study was to analyze the cardiovascular manifestations and evolution of the patients with Marfan syndrome followed at our pediatric cardiac unit. METHODS: The authors reviewed the clinical files of all the patients that fulfilled the diagnostic criteria for MS according to De Paepe et al. (1996). We analyzed the following parameters: gender, age at referral, race, family history, clinical examination, diagnostic exams, therapy and evolution. The patients were divided into two groups according to the age at diagnosis: infants (group 1) and older patients (group 2). RESULTS: Group 1 included 3 infants, two boys and one girl, sporadic cases, presenting congestive heart failure. The major cardiac diagnoses were aortic dilatation (1/3) and mitral valve prolapse with severe mitral regurgitation (2/3). Congenital heart disease was associated in two cases (patent ductus arteriosus and atrial septal defect). Two needed cardiac surgery at an early age and one was recently proposed for surgery. There were no deaths. Group 2 included 20 patients, 14 boys and 6 girls, first seen at a mean age of 8 years. Ten had a positive family history and none presented cardiac symptoms. The major cardiac manifestations were mitral valve prolapse (18/20) and aortic dilatation (17/20). There was no significant progression of the cardiac lesions, except for one case, during the 12 years of follow-up. CONCLUSION: Infantile MS presented high morbidity; mitral regurgitation was severe in two cases. All patients presented heart failure, two needing early operations. In classic MS evolution was favorable, and the commonest cardiac lesions were mitral valve prolapse and aortic dilatation. We emphasize the need for beta-blockers to prevent progression of aortic dilatation. The decision for surgery rests upon the severity of valve regurgitation and the rate of progression of aortic dilatation. It is important to inform patients and family about physical exercise, prevention of endocarditis, risks associated with pregnancy and genetic counseling.

Axial mesodermal dysplasia complex: a new case with parental consanguinity.

A female is described with axial mesodermal dysplasia complex (AMDC) born to a consanguineous couple. This is thought to be the first description of a patient with AMDC born to consanguineous parents.

[Case report of tuberous sclerosis and congenital heart disease]. / Esclerose tuberosa e cardiopatia congénita caso clínico.

The cardiac manifestation usually associated with Tuberous Sclerosis is rhabdomyoma. The authors present a clinical case of Tuberous Sclerosis with the particular coexistence of congenital heart disease (mitral anomaly and pulmonary stenosis) and a single intracardiac rhabdomyoma that appeared at the age of four years.

Rhizomelic chondrodysplasia punctata-like phenotype in a newborn male with normal peroxisomal function.

A newborn male with the characteristic phenotype of classic rhizomelic chondrodysplasia punctata (RCDP) and with the usual and severe radiographic skeletal abnormalities is described. The parents were young, healthy, and not consanguineous; the mother had not used licit or illicit drugs, alcohol, or tobacco during pregnancy and had not been exposed to radiation or teratogenic chemicals. The clinical phenotype led us to study peroxisomal function. Plasmalogen content in erythrocytes, membrane, and fibroblasts; dihydroxyacetone phosphate acyltransferase (DHAP-AT), alkyldehydroxyaceton phosphate synthetase (a gift from Professor Henk van der Boch, Utrech) in fibroblasts; and phytanic and pristanic acids in plasma showed normal values. Immunocytofluorescence study with antibodies against peroxisomal membrane showed normal organelles. We found no reference in the literature of a case of RCDP with normal peroxisomal functions, but non-CDP has been described with peroxisomal dysfunction. This phenotype (RCDP) may be due to other metabolic error.

A new mutant hairless mouse with lymph node hyperplasia and late onset of autoimmune pathology.

Adult BALB/c male mice were injected with a single dose of ethyl nitroso urea (ENU; 250 mg/kg, i.p.) and mated to C57BL/6, DBA/2 and A/J adult females 13 weeks later. F1 males were mated with BALB/c females and F2 females were than backcrossed to the F1 parents. One BALB/c male mouse thus treated gave origin to a mutant presenting hair and skin alterations similar to those of natural hairless mutants. The new mutation is located on chromosome 14 near the Es10 locus, and probably at the same locus for the hairless mutation. Similar to the hairless mouse, this new mutant has a normal phenotype at birth and after three weeks starts to loose hair which is never replaced. Additionally, the skin becomes thickened and wrinkled. One feature that distinguishes this mutant from other hairless mice is the peculiar enlargement of its axillary and cervical lymph nodes. The new mutant develops membranoproliferative glomerulonephritis similar to the rhino mouse, one of the hairless allele mutants already described in the literature, but with a much later onset.

A new mutant hairless mouse with limph node hyperplasia and late onset of autoimmune pathology

Adult BALB/c male mice were injected with a single dose of ethyl nitroso urea (ENU; 250 mg/Kg, ip) and mated to C57BL/6, DBA/2 AND A/J adult females 13 weeks later. F1 males were mated with BALB/c females and F2 females were then backcrossed to the F1 parents. One BALB/c male mouse thus teated gave origin to a mutant presenting hair and skin alterations similar to those of natural hairless mutants. The new mutation is located on chromosome 14 near the Es10 locus, and probably at the same locus for the hairless mutation. Similar to the hairless mouse, this new mutant has a normal phenotype at birth and after three weeks starts to loose hair which is never replaced. Additionally, the skin becomes thickened and wrinkled. One feature that distinguishes this mutant from other hairless mice is the peculiar enlargement of its axillary and cervical lymph nodes. The new mutant develops membranoproliferative glomerulonephritis similar to the rhino mouse, one of the hariless allele mutants already described in the literature, but with a much later onset

Experimental paracoccidioidomycosis: early immunosuppression occurs in susceptible mice after infection with pathogenic fungi.

The course of Paracoccidioides brasiliensis infection depends on the natural resistance of the host and on the virulence of the fungus isolate. In the present study the immune response to sheep red blood cells (SRBC) was investigated in susceptible (B10.A) and resistant (A/SN) mice after intraperitoneal (ip) infection with pathogenic (Pb18) and apathogenic (IVIC Pb267) P. brasiliensis isolates. Infection with Pb18 yeast cells quickly induces a state of immunodepression only in the susceptible mice. This immunosuppression was not observed when mice were inoculated with IVIC Pb267 yeast.