Spontaneously fermented traditional beverages as a source of bioactive compounds: an overview.

Fermented food has been present throughout history, since fermentation not only helps preserving food, but also provides specific organoleptic characteristics typically associated to these foods. Most of the traditional fermented foods and artisanal beverages are produced by spontaneous generation, meaning no control of the microbiota, or the substrate used. Nevertheless, even not being standardized, they are an important source of bioactive compounds, such as antioxidant compounds, bioactive beeps, short chain fatty acids, amino acids, vitamins, and minerals. This review compiles a list of relevant traditional fermented beverages around the world, aiming to detail the fermentation process itself-including source of microorganisms, substrates, produced metabolites and the operational conditions involved. As well as to list the bioactive compounds present in each fermented food, together with their impact in the human health. Traditional fermented beverages from Mexico will be highlighted. These compounds are of high interest for the food, pharmaceutical and cosmetics industry. To scale-up the home fermentation processes, it is necessary to fully understand the microbiology and biochemistry behind these traditional products. The use of good quality raw materials with standardized methodologies and defined microorganisms, may improve and increase the production of the desirable bioactive compounds and open a market for novel functional products.

Galactosemia: genotipo y fenotipo de siete pacientes / Galactosemia: the genotype and phenotype of seven patients

Introducción. A pesar de los tratamientos precoces basados en la nutrición, muchos pacientes con galactosemia presentan una enfermedad neurodegenerativa que se manifiesta fundamentalmente a través de alteraciones del lenguaje y discinesias. En las imágenes obtenidas mediante la resonancia magnética (RM) cerebral se pueden visualizar dos tipos distintos de alteraciones: una primera, que se manifiesta con hiperseñal difusa y poco intensa de la sustancia blanca de los centros semiovales, y una segunda, con focos de hiperseñal más intensos rodeando las puntas ventriculares de predominio posterior. Pacientes y métodos. Presentamos los datos clínicos y de imagen de siete niños de edades comprendidas entre los 3 y los 12 años con galactosemia clásica. El inicio había ocurrido típicamente durante la lactancia. Dos niños se desarrollaron de forma normal (10 y 12 años), cuatro presentaban un retraso del desarrollo (10, 7, 4 y 3 años) y uno presentaba una parálisis cerebral distónica (kernícterus). Resultados. La RM cerebral reveló la afectación típica de la sustancia blanca en cinco de los niños: uno con alteración difusa y cuatro con ambos tipos de alteraciones. El paciente con kernícterus mostró lesiones en los ganglios basales. Tres pacientes son homocigóticos para la mutación Q188R, y dos son heterocigóticos compuestos. Conclusión. Se ha hallado una correlación positiva entre el retraso en el desarrollo, la afectación de la sustancia blanca y la mutación clásica Q188R (AU)

Introduction. Despite early dietary therapy, many patients with galactosemia show a neurodegenerative disease specially evident in speech impairment and movement disorders. Magnetic resonance imaging of the brain, show cerebral white matter changes with hypomielinization bilateral and symetrical periventricular hypersignal in T2. Patients and methods. We presented clinical and neuroradiological data of seven children (3 to 12 years of age) with classical galactosemia. All had a typical presentation in neonatal period. Two children had normal development (10 and 12 years-old), four presented developmental delay (10, 7, 4 and 3 years-old), and one showed a dystonic cerebral palsy (kernicterus). Results. The brain MRI showed the typical involvement of white matter, in five children, and basal ganglia abnormalities in the kernicterus patient. Three patients are homozygous for Q188R mutation and two are compound heterozygous. Conclusion. We found a positive correlation among developmental delay, white matter involvement and Q188R mutation (AU)