Early- Onset Stroke and Vasculopathy Associated with Mutations in ADA2

Adenosine deaminase 2 (ADA2) is an enzyme involved in purine metabolism and a growth factor that influences the development of endothelial cells and leukocytes. This study shows that defects in ADA2 cause recurrent fevers, vascular pathologic features, and mild immunodeficiency. Patients with autoinflammatory disease sometimes present with clinical findings that encompass multiple organ systems.(1) Three unrelated children presented to the National Institutes of Health (NIH) Clinical Center with intermittent fevers, recurrent lacunar strokes, elevated levels of acute-phase reactants, livedoid rash, hepatosplenomegaly, and hypogammaglobulinemia. Collectively, these findings do not easily fit with any of the known inherited autoinflammatory diseases. Hereditary or acquired vascular disorders can have protean manifestations yet be caused by mutations in a single gene. Diseases such as the Aicardi-Goutieres syndrome,(2),(3) polypoidal choroidal vasculopathy,(4) sickle cell anemia,(5) livedoid vasculopathy,(6) and the small-vessel vasculitides(7),(8) are examples of systemic ...</p

Rendimento, composição e teor de ácidos graxos da carcaça de frangos de corte alimentados com diferentes fontes lipídicas Carcass yield, composition and fat acid percentage of carcass for broiler fed on different lipid source diets

Avaliou-se o efeito de diferentes fontes de lipídios da dieta (óleo degomado de soja, óleo de vísceras de aves, óleo ácido de soja, mistura de 50% de óleo de soja e 50% de óleo de vísceras e mistura de 50% de óleo de soja e 50% de óleo ácido de soja) sobre rendimento, composição de peito, coxa e carcaça inteira e perfil de ácidos graxos da carcaça inteira de frangos de corte. O delineamento experimental foi o inteiramente ao acaso, com cinco tratamentos e seis repetições de uma ave cada. Não houve diferenças entre os tratamentos (P>0,05) para rendimentos de carcaça e cortes, composição de proteína bruta, umidade e extrato etéreo da carcaça inteira e da musculatura da coxa e do peito. As aves alimentadas com dietas com óleo de vísceras apresentaram maior percentual de ácidos graxos monoinsaturados na carcaça do que as alimentadas com dietas contendo as demais fontes lipídicas. A deposição de ácidos graxos poliinsaturados foi influenciada pela fonte lipídica das dietas. O perfil de ácidos graxos da carcaça inteira de frangos de corte foi influenciado pela fonte lipídica da dieta.<br>A completely randomized design, with six replicates of one bird per experimental unit was used to evaluate the effect of different fat sources of diets (soybean oil, poultry fat, acidulated soybean oil soapstock and a mix of soybean oil plus poultry fat and soybean oil plus acidulated soybean oil soapstock) on carcass yield, composition of the breast, thigh and whole chicken and fatty acid profile of the whole carcass of broilers. No effects of fat source on carcass yield and cuts, composition of crude protein, humidity and ether extract of the whole chicken, whole leg and breast were observed (P>0,05). The birds fed on poultry fat diet showed a higher percentage of monounsaturated fatty acid on the carcass than birds fed on diets with the others fat sources. The deposition of polyunsaturated fatty acids was influenced by fat source added to the diet. The fatty acid profile of the whole carcass of broilers was influenced by the fat source added to the diets

Composição em ácidos graxos da gordura na carcaça de frangos de corte sob dietas com diferentes fontes de energia Carcass fatty acid composition of broiler fed different sources of energy

Avaliaram-se os níveis de energia metabolizável normalmente utilizados nas rações de frangos de corte, obtidos pela inclusão de óleos vegetais (soja, canola e palma) e seus efeitos sobre o perfil de ácidos graxos da pele e dos músculos da coxa e do peito, levando-se em consideração a linhagem e o sexo. Foram utilizados 2400 pintos de um dia, machos e fêmeas, das linhagens comerciais Hubbard e Avian Farms. As aves receberam ração inicial e final com níveis de energia metabolizável de 3050 e 3150kcal/kg, respectivamente, obtidos pela inclusão dos óleos vegetais, fornecedores de ácidos graxos, inclusive "ômega-3". Utilizou-se um delineamento inteiramente ao acaso em um arranjo fatorial com 16 tratamentos (4 rações × 2 sexos × 2 linhagens) com duas repetições cada. Diferenças entre médias foram testadas pela dms. Não houve efeito significativo da inclusão de óleos na ração sobre o perfil de ácidos graxos na carcaça. O mesmo ocorreu quanto ao efeito de sexo e de linhagem.<br>An experiment was carried out with the objective of evaluating the effect of metabolizable energy levels, normally utilized in ration for broilers by addition of vegetable oils (soybean, rapeseed, and palm), on the profiles of fatty acids of the skin and meat of breast and thigh according sex and lineage, based on 2,400 one-day-old chicks, males and females, from Hubbard and Avian Farms. The fowls were given initial and finisher rations with metabolizable energy levels of 3,050 and 3,150kcal/kg, respectively, obtained by the inclusion of vegetable oils, which provided fatty acids, including "omega-3". The initial and finisher control rations were based on corn and soybean, without oil inclusion, with metabolizable energy levels of 2,900 and 3,000, respectively. A completely randomized design with factorial arrangement (4 rations × 2 sexes × 2 lines) and SMD test were used. No effects of sex, lineage and oil inclusion on carcass fatty acid profiles were observed