Ophthalmoplegia-ataxia-areflexia in pediatrics. Three new patients and review of the literature

OBJECTIVE: The objective of this study was to investigate if pediatric patients with benign brainstem encephalitis (Bickerstaff Syndrome) or with Miller-Fisher Syndrome are the extremes of the same nosological entity which, in adults, has been named ophalmoplegia-ataxia-areflexia syndrome. PATIENTS AND METHODS: The subjects included in the study were three patients of our institution and 24 patients found in the revision of the English and Spanish pediatric literature who fulfilled the diagnostic criteria of ophtalmoplegia-ataxia-areflexia syndrome. The topographical location of the lesion in the nervous system was based on previously established criteria by using clinical and complementary studies. RESULTS: Of the 27 patients included in the study we were able to reach an accurate topographical diagnosis in 9. None had an exclusive involvement of the peripheral nervous system, (6) had exclusively central nervous system involvement and 2 showed involvement of both system. In 12, the topographical location of the lesion could be only ascertained as probable; 3 of them in the peripheral nervous system, 2 in the central nervous system and mixed involvement in 7. In the remaining 7 patients there were insufficient clinical data to allow topographical classification. CONCLUSIONS: The ophtalmoplegia-ataxia-areflexia syndrome can also be found in pediatric patients. The lesion in the majority of patients in this age group is located in the central nervous system, either alone or combined with peripheral nervous system involvement.     

Polysplenia. Description of a case

A clinical and hemodynamic study of a case of polysplenia, with typical left bilateral symmetry, anomalies of systemic venous drainage and defect of the endocardial cushions. A brief revision of the literature is given and the embriological and clinical concept of the left bilateral symmetry is discussed.     

Protoporphyrin IX sensitized photohemolysis: stoichiometry of the reaction and repair by reduced glutathione

Protoporphyrin IX acts as a sensitizer in the photohemolysis of bovine erythrocytes by binding to a limited number of membrane sites. The cholesterol-specific antibiotic lucensomycin competes with protoporphyrin in binding to the membranes. The possibility of cholesterol peroxidation as a primary event in photohemolysis is supported by the repairing effect of exogenous cholesterol and by the increased susceptibility of the photosensitized erythrocytes to lucensomycin. Glutathione, if present within the erythrocyte, postpones the onset of lysis; if added after irradiation, it may repair the membrane damage and prevent hemolysis. This effect appears to be related to a redox reaction (possibly involving glutathione peroxidase) between reduced glutathione and the cholesterol peroxide molecules.     

Nanofibrillated Cellulose as Functional Ingredient in Emulsion-Type Meat Products

The objective of this work was to introduce nanofibrillated cellulose (NFC) in the formulation of cooked emulsion-type sausages with the aim of assessing its feasibility to assume the role or compensate the lack of some conventional functional ingredients, such as polyphosphates, maize starch, and sodium caseinate. For this aim, sausages based on standard commercial formula (control) including all three ingredients and sausages containing 0.5% NFC instead of phosphates and starch (NFC-1) or instead of phosphates, starch, and sodium caseinate (NFC-2) were produced and characterized. In NFC-1 samples, 0.5% nanofibrillated cellulose succeeds in replacing 0.5% polyphosphates and 1% starch without significantly altering the composition, nor negatively affecting the fat and water retention properties, neither of the raw batter or the cooked sausages. However, less stable meat batters and sausages with significantly reduced water-holding capacity were obtained when 1.5% sodium caseinate, in addition to phosphates and starch, was also removed (NFC-2). Nevertheless, results were hopeful enough to encourage further optimization studies, using several NFC concentrations and/or cellulose with different nanofibrillation degrees, in order to clarify whether it is possible to successfully replace also non-meat proteins in cooked emulsion-type sausages.     

Study of the ultrasound effects on yeast lees lysis in winemaking

This work focused on the study of ultrasound (US) treatment effects on wine lees, in order to verify the possibility of accelerating the protective colloids (i.e., polysaccharides) release. Moreover, these effects were compared with the usual practices of aging wine over lees and with the enzyme treatment of lees by means of beta-glucanases able to demolish the glucans and facilitate the release of intracellular components. This work was performed considering the possibility of setting up a technology applicable in a cellar. Ultrasound treatment on wine lees resulted in an increase in total colloids, proteins, and polysaccharides in the medium with a reduction in the colloids particle diameter. These results were mainly correlated with treatment duration rather than its intensity. Treatment at 90 % amplitude for 3 min provided good enough results in terms of colloids release, and therefore, it was used for the comparative study with natural lysis and enzyme treatment. The parameters evaluated showed that the ultrasound treatment resulted in an increased release of colloids from yeast (polysaccharides and glycoproteins); therefore, a significant effect on the extraction of cellular components compared to the long stay on the lees was detected, indicating the interesting advantage that this new technological application can bring to the winemaking process.