A industrialização de lácteos ovinos é recente no Brasil e a produção é concentrada em regiões de serra, como a Gaúcha. O leite ovino possui alto conteúdo de sólidos comparado ao bovino, com maior rendimento na produção de queijo. A produção deste leite sofre variações sazonais e poderia ser estocado até obter uma quantidade suficiente para processamento. O objetivo deste trabalho foi avaliar parâmetros microbiológicos e físico-químicos no leite proveniente de seis produtores sob armazenamento refrigerado. O leite foi analisado durante sete dias, incluindo contagem padrão em placas (CPP), psicrotróficos, coliformes totais e termotolerantes, Staphylococcus sp. e Staphylococcus aureus, acidez titulável, pH, atividade de água e proteína.Os resultados demonstraram altas contagens microbianas e CPP acima dos limites estabelecidos pela legislação para a maioria das amostras. Contagens de psicrotróficos, coliformes totais e fecais foram elevadas durante este período. A acidez titulável aumentou ao longo dos dias e o conteúdo de proteína diminuiu. As amostras do produtor B apresentaram menores contagens microbianas dentre todas testadas. A estocagem a frio torna-se uma alternativa, a curto prazo, quando há diminuição na produção de leite ovino para beneficiamento a derivados. No entanto, o período de armazenamento refrigerado não deve ultrapassar dois dias, devido à deterioração.
Hereditary fructose intolerance (HFI) is an inborn error of fructose metabolism of autosomal recessive inheritance caused by pathogenic variants in the ALDOB gene that lead to aldolase B deficiency in the liver, kidneys, and intestine. Patients manifest symptoms, such as ketotic hypoglycemia, vomiting, nausea, in addition to hepatomegaly and other liver and kidney dysfunctions. The treatment consists of a fructose-restricted diet, which results in a good prognosis. To analyze the distribution of ALDOB variants described in patients and to estimate the prevalence of HFI based on carrier frequency in the gnomAD database, a systematic review was conducted to assess ALDOB gene variants among patients with HFI. The prevalence of HFI was estimated from the carrier frequency of variants described in patients, as well as rare variants predicted as pathogenic by in silico tools. The p.(Ala150Pro) and p.(Ala175Asp) variants are the most frequent and are distributed worldwide. However, these variants have particular distribution patterns in Europe. The analysis of the prevalence of HFI showed that the inclusion of rare alleles predicted as pathogenic is a more informative approach for populations with few patients. The data show that HFI has a wide distribution and an estimated prevalence of ~1:10,000.
Recently, patients with glycogen storage disease (GSD) have been described as having gut dysbiosis, lower fecal pH, and an imbalance in SCFAs due to an increase in acetate and propionate levels. Here, we report the fecal measurement of bacterial-related metabolites formic, acetic, lactic, propionic, and succinic acid, a key metabolite of both host and microbiota, on a previously described cohort of 24 patients (GSD Ia = 15, GSD Ib = 5, 1 GSD III = 1 and GSD IX = 3) and 16 healthy controls, with similar sex and age, using the high-performance liquid chromatography technique. The succinic acid levels were higher in the GSD patients than in the controls (patients = 38.02; controls = 27.53; p = 0.045), without differences between the groups for other metabolites. Fecal pH present inverse correlation with lactic acid (R = −0.54; p = 0.0085), while OTUs were inversely correlated with both lactic (R = −0.46; p = 0.026) and formic (R = −0.54; p = 0.026) acids. Using two distinct metrics of diversity, borderline significance was obtained for propionic acid, affecting the microbial structure on Euclidean basis in 8% (r2 = 0.081; p = 0.079), and for lactic acid, affecting 6% of microbial structure using Bray–Curtis distance (r2 = 0.065; p = 0.060). No correlation was found between SCFAs and total carbohydrate consumption among the participants or uncooked cornstarch consumption among the patients.
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