Methionine synthase deficiency is very rare and characterized by megaloblastic anemia and neurological symptoms. We report the second case of MSD associated to TMA previously diagnosed as aHUS in which the patient had a poor response to eculizumab.

This study investigated the effects of acute and chronic hyperprolinemia on glutamate uptake, as well as some mechanisms underlying the proline effects on glutamatergic system in rat cerebral cortex. The protective role of guanosine on effects mediated by proline was also evaluated. Results showed that acute and chronic hyperprolinemia reduced glutamate uptake, Na(+), K(+)-ATPase activity, ATP levels and increased lipoperoxidation. GLAST and GLT-1 immunocontent were increased in acute, but not in chronic hyperprolinemic rats. Our data suggest that the effects of proline on glutamate uptake may be mediated by lipid peroxidation and disruption of Na(+), K(+)-ATPase activity, but not by decreasing in glutamate transporters. This probably induces excitotoxicity and subsequent energy deficit. Guanosine was effective to prevent most of the effects promoted by proline, reinforcing its modulator role in counteracting the glutamate toxicity. However, further studies are needed to assess the modulatory effects of guanosine on experimental hyperprolinemia.

RESUMO:O presente estudo objetivou a modelagem farmacodinâmica da ação antihiperglicemiante da farinha da casca de maracujá (Passiflora edulis Sims, Passifloraceae) em ratos diabéticos induzidos por aloxano. Os animais foram divididos em três grupos (n = 9): grupo diabético tratado com 20 mg/kg, 40 mg/kg ou 160 mg/kg da farinha. As amostras sanguíneas foram coletadas da veia lateral da cauda e a glicemia foi determinada com auxílio de um glucômetro em 0, 1, 2, 4 e 6 h após o tratamento via oral. Observou-se que o efeito antihiperglicemiante da farinha da casca de maracujá foi dependente da dose, obtendo um efeito expressivo observado em quatro horas para todas as doses, sendo mais pronunciado na dose de 160 mg/kg. O efeito, expresso como redução da glicemia basal em relação ao tempo zero, pode ser modelado através de um modelo dose-efeito clássico, o qual permitiu a determinação do efeito máximo (E max ) e da dose necessária para alcançar 50% deste efeito (DE 50 ). Após a modelagem dos dados, obteve-se um valor de E max = 58,41% e de DE 50 = 23,61 mg/kg. Esta abordagem pode permitir a comparação do efeito antihiperglicemiante de Passiflora edulis com outras espécies que apresentem atividade sobre a redução da glicemia, mediada pela presença de fibras solúveis.Unitermos: Passiflora edulis, fibras hidrossolúveis, atividade antihiperglicemiante, modelagem farmacodinâmica, aloxan.ABSTRACT: "Investigation of antihyperglycemic activity of Passiflora edulis Sims, Passifloraceae, in alloxan-induced diabetic rats". This study goals the pharmacodynamic modeling of anti hyperglycemic action of flour of Passion fruit bark (Passiflora edulis Sims, Passifloraceae) in alloxan-induced diabetic rats. The animals were divided into three groups (n = 9) that received different doses: 20, 40 or 160 mg/kg of flour. The blood samples were collected from the tail vein and the blood glucose was determined using a glucometer Prestige IQ in 0, 1, 2, 4 and 6 h after the oral treatment. It was observed that the effect of the flour of Passion fruit bark was dose dependent, getting an expressive effect observed in four hours from all doses tested, being more pronounced at the 160 mg/kg dose. This effect was expressed as reduction of basal blood glucose in respect to zero time and can be modeled through a classic dose effect model. It allowed the determination of maximum effect (E max ) and the necessary dose to reach 50% of this effect (ED 50 ). After the data modeling, it was gotten a value of E max = 58,41% and ED 50 = 23,61 mg/kg. This approach allows the comparison of antyhyperglicemic effect of Passiflora edulis with other species that present activity lowering the glycemia, through the presence of soluble fibers.Keywords: Passiflora edulis, hidrossoluble fibers, antihyperglycemic, pharmacodynamic model, alloxan.não-farmacológicas, principalmente o controle da dieta e medidas farmacológicas, as quais incluem primeiramente o uso de hipoglicemiantes orais e nos casos mais graves, reposição com insulina exógena.Assim, é indicado um consumo...

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