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The hyperimmunoglobulin E syndrome is a rare complex primary immunodeficiency characterized by high serum IgE levels, eczema, and recurrent infections. We present a case of 2-years-old girl with eczema and repeated bacterial skin and lung infections since the period of infancy. The patient also had eosinophilia, high serum levels of IgE, and cows-milk hypersensitivity. We describe the case, in order to illustrate the difficulty of establishing the diagnosis hyper-immunoglobulin E syndrome in a toddler. [ABSTRACT FROM AUTHOR]

In order to preserve the quality of apple fruit, it is crucial to choose the most suitable storage technology. Nevertheless, it is also important to maintain the quality of apples during the shelf life period. In this study, changes in various biochemical quality parameters of 'Golden Delicious' and 'Idared' apples during a prolonged shelf life period, after conventional normal atmosphere (NA) and ultra-low oxygen (ULO) storage, were investigated. After the harvest, the fruit of examined cultivars were stored in two atmospheres for six months. Weight loss, changes in content of sugars, organic acids, total phenols, total flavonoids, pectic fractions, and antioxidant capacity were monitored. Samples were evaluated immediately after removal from the cold chambers and after 10 and 20 days of shelf life at 20 °C. 'Golden Delicious' apples stored in ULO exhibited better qualitative characteristics during 20 days of shelf life compared with apples of the same cultivar stored in NA. However, evaluated traits in 'Idared' apples from NA chambers were of higher quality in comparison with 'Idared' apples stored in the ULO atmosphere. [ABSTRACT FROM AUTHOR]

The aim of this work was to investigate nutritional profile in relation to proteins, amino acids, fatty acids and mineral composition, as well as phenolic profile of small edible fungal species Coprinellus disseminatus originated from Serbia. Total protein content in the analyzed fungal species was 9.72%. Fifteen protein fractions obtained by electrophoresis were identified in a range from 1.6 to 63.6 kDa. Chip-based separations showed the presence of protein fraction with molecular weight of 27.5 kDa that could possess antifungal activity. The total essential and non-essential amino acid contents were 29.57 and 96.69 mg/g DW, respectively. Among the essential amino acids, leucine was the most abundant. Fatty acid composition of C. disseminatus showed that polyunsaturated fatty acids (PUFA, 59.1% of total FA) predominated over saturated fatty acids (SFA, 23.1% of total FA) and monounsaturated fatty acids (MUFA, 17.9% of total FA). The dominant fatty acids were linoleic acid (56.6%), palmitic acid (13.9%), and oleic acid (12.0%). The most abundant macroelement in C. disseminatus was potassium, followed by calcium and magnesium, while iron dominated in microelements. Eight phenolic compounds were quantified in methanolic extract of C. disseminatus by LC-MS/MS with the highest amount of p-hydroxybenzoic acid and p-coumaric acid reaching 9.46 ± 0.2 μg/g DW and 7.8 ± 0.1 μg/g DW, respectively. [ABSTRACT FROM AUTHOR]

Cheese cannot be produced solely from equine or donkeys' milk, because of the unique physico-chemical properties of these milks. The purpose of this study was to characterize a novel dairy product, cheese produced from donkeys' and caprine milk mixture (60:40% v/ v), regarding its chemical, microbiological, textural and sensory properties. Fully ripened cheese was classified as a high-fat, extra-hard cheese, with high sodium (29.97 g.kg), magnesium (3.07 g.kg) and potassium (4.70 g.kg) content. The characterization by lab-on-a-chip electrophoresis revealed lysozyme, α-lactalbumin, immunoglobulins and casein fractions. Palmitic (C) and oleic fatty acids (C) with 25.11 and 24.70%, respectively, were found at the highest concentrations. The medium-chain fatty acids account 18.21% of the total fatty acid content in analysed cheese samples. Escherichia coli, Salmonella spp., Enterobacteriaceae, coagulase-positive staphylococci, Listeria monocytogenes, Bacillus cereus, Clostridium perfringens as well as moulds were under the limit of detection in all analysed samples. After 6 months of cheese ripening (0.94 a, pH 4.71), total bacterial count, the counts of lactic acid bacteria and yeasts were 6.34 ± 0.03, 4.80 ± 0.10 and 5.81 ± 0.11 log CFU.g, respectively. The texture of mature cheese was moderately hard and crumbly. The cheese was described as very salty with strong pronounced creamy, fatty and acidic taste. The characterized donkey/caprine cheese could position this type of cheese as a high-quality functional product, thus having a potential impact on the market. [ABSTRACT FROM AUTHOR]

The aim of the present paper was to evaluate the influence of different climatic conditions on the activity of alpha-amylase in wheat samples and bread quality parameters. Three wheat varieties grown in three different localities in three years were chosen for this study. Commonly used methods for estimation of alpha-amylase activity in wheat grain were employed. The obtained results indicated that harvest year 2013, which was characterized with the excessive amount of rainfall, exhibited the highest level of alpha-amylase activity and the lowest values of the peak viscosity. The lowest alpha-amylase level and the highest peak viscosity and FN value were observed for samples harvested in 2012 which was characterized with the greatest number of days with an average daily temperature above 30 and 35 °C. In addition, a decrease in Mixolab parameter torque C3 and specific bread loaf volume, as well as increase in the breakdown torque (C3-C4) of samples harvested in 2013 were observed, which could be attributed to rainy weather influencing increase in alpha-amylase activity. It is found that specific bread loaf volume of wheat samples is highest in 2012. Moreover, a negative correlation between alpha-amylase activity and specific bread volume for all the samples grown in three years was determined. [ABSTRACT FROM AUTHOR]

The aim of this study was to compare the protein and fatty acid fractions of Balkan donkey and human milk in the early lactation stage (40 and 90 day). This study revealed that donkey milk contains αs1-casein (1.38-1.89 g/L) and higher concentration of β-casein (0.1-0.55 g/L) in comparison to human milk. The concentration of α-lactalbumin increased during the lactation phases from 40 to 90 days in both types of milk. Donkey milk contained β-lactoglobulin in low concentrations which decreased to 90th day of lactation. Donkey milk was particularly rich in two whey proteins, lactoferrin and lysozyme, which were found to have molecular weight of approximately 76 kDa and 14.9-15.4 kDa, respectively. The content of lysozyme in donkey milk ranged from 2.39 to 2.97 g/L, while human milk contained 30-50 times lower concentrations of lysozyme in comparison to donkey milk. Thus, donkey milk contained also a higher concentration of lactoferrin (0.012-0.25 g/L) than it was found in the human milk. Lysozyme and lactoferrin content in donkey milk increased during the period from 40th to 90th day of lactation. The percentage of total SFA, MUFA and PUFA was similar in donkey and human milk. The content of essential fatty acids increased during 40-90 days of lactation and was approximately 2.5 times higher in comparison to human milk. [ABSTRACT FROM AUTHOR]

This study aimed to determine the impact of ancient wheat varieties (emmer, spelt and khorasan) and spontaneous sourdough fermentation on the bioaccessibility of total phenolic content (TPC) and the DPPH antioxidant capacity evolution during breadmaking and in vitro digestion. Sourdough and yeast-fermented modern wheat breads were used as controls. After 6 h of fermentation, the total titrable acidity of the sourdough increased from 139 to 167%. The wheat variety, type of fermentation and processing affected TPC, antioxidant activity and bioaccessibility. Antioxidant activity and TPC were reduced by dough mixing, increased after sourdough fermentation and slightly decreased or remained the same after baking. Although wheat flour had the highest TPC, the modeling of TPC kinetic revealed that emmer and spelt sourdough exhibited a higher bound phenolics release rate due to the higher acidity, which contributed to increased phenolics solubility. Although wheat bread, both before and after digestion, had the lowest TPC, especially the one prepared with yeast, high TPC bioaccessibilities and antioxidant activities after the digestion suggested that, except phenolics, digestion process improved the release of additional compounds with different bioaccessibility and biological activity. The results of this study proved that the application of sourdough fermentation can increase the potential of ancient wheats in the developing of functional bakery products.

The aim of this study was to test the suitability of three different ancient wheat varieties (emmer, spelt and khorasan) to produce spontaneously fermented sourdough bread and to evaluate the impact on the dough rheological properties, ultrastructure and baking quality. Modern wheat sourdough bread and bakery yeast fermented bread were used as controls. Sourdoughs produced from modern and ancient wheats exerted different effects on dough viscoelastic properties, bread specific volume, texture, firming rate, colour and sensory properties, while there was no influence on bread water activity. Both khorasan sourdough, being characterised with the highest dough strength and dense gluten protein matrix, and emmer sourdough, with loose and thin gluten strands of low strength, yielded breads characterised by low specific volume and hard crumb texture. Spelt and modern wheat sourdough were characterised by foam-like dough structures with entrapped gas cells leading to breads of similar specific volume and texture. Although the yeast-fermented wheat flour exerted a higher specific volume and the lowest firmness, the sourdough wheat flour bread had a lower firming rate. A comparison of sourdough bread prepared with modern and ancient wheats revealed that breads based on ancient varieties possess a less noticeable sour taste, odour and flavour, thus contributing to more sensory-appealing sourdough bread., Competing Interests: The authors declare no conflict of interest. The funders had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript, or in the decision to publish the results.

Gluten-free cookies based on rice and chickpea flour with reduced-fat and increased protein content compared with conventional commercial gluten-free cookies were developed and used as a base for further vegetable fat replacement with chia seed hydrogel. Rheological properties of chia seed hydrogel revealed that 8% gels exhibited the optimal properties as a fat substitute. Designed cookie samples were characterized for their chemical composition, fatty acid profile, mineral content, physical, textural and color parameters, and sensory properties. All gluten-free cookies developed in this study could be labeled as "a source of iron and potassium", while those with chia seed hydrogel and cocoa powder could bear the additional claim "high in zinc and magnesium". Fat replacement with chia seed hydrogel resulted in a more favorable fatty acid composition with a PUFA/SFA ratio over 0.40 and nonsignificant changes in the cookies' hardness, weight, eccentricity, and specific volume, indicating that the chia seed hydrogel addition did not disturb the cookie structure and texture. The results of the sensory analysis confirmed that it is possible to apply chia seed hydrogel to produce reduced-fat cookies with sensory properties comparable to their full-fat counterpart and available commercial samples, and they are more appealing than commercial reduced-fat gluten-free cookies.

Whey protein and sourdough ferment were used in different combinations to prepare functional sponge cakes, and their mutual influence on batter rheological behaviour as well as product physico-chemical, textural, colour and sensory properties were evaluated. All samples containing whey protein concentrate could bear the nutrition claim 'a source of protein'. The substitution of wheat flour with whey protein significantly influenced batter viscoelastic behaviour, lowered cake-specific volume, increased product hardness, chewiness, gumminess, and browning index and modified its sensory characteristics. The incorporation of sourdough in protein-enriched sponge cakes improved product-specific volume and appearance compared to a protein-containing sample without sourdough. Although sourdough addition has less of a deteriorating effect on sponge cake rheological and textural properties, when combined with whey protein, it led to a significant reduction in batter elasticity and an increase in product hardness. It was also shown that spontaneously fermented sourdough cannot act as the only leavening agent in sponge cake production. In general, the results of this study have shown that sourdough addition can contribute to improvement in protein-enriched sponge cake quality and that further investigations are necessary in terms of different sourdough and flour type incorporation to minimize the negative effects of protein addition.

Vegetable by-products, obtained from cauliflower (CA), broccoli (BRL), cabbage (CB) and beetroot (BR) can be a potentially good source of proteins. The proteins were obtained from leaves (LPs) of vegetables with alkaline extraction at pH 10, and their isoelectric precipitation at pH 4. Protein contents were in the range of 39.76 - 53.33%, and the molecular weights of fractions were mostly about 45, 25 and 14 kDa. Their solubility is higher in the alkaline environment, where they reach the highest solubility at pH 10 (9.7 mg/mL for CALP, 8.45 for BRLP, 5.35 mg/mL for CBLP, 5.5 mg/mL for BELP). Moreover, they showed favorable emulsifying abilities, water absorption capacities (0.62 to 1.61 g/g) and foaming capacity (86.3 to 92%) as well as stability (48.57 to 79.30%). Digestibility was studied using gastrointestinal proteases (pepsin and pancreatin), and all four LPs can easily be digested. The biologically active potential of the digests was evaluated measuring antioxidant capacity by two complementary methods - DPPH + and ABTS + radical cation scavenging activity. The values for DPPH+ and ABTS+ were in the range from 59 to 65.1% at 0.1 and 0.3 mg/ml to 0.22 mg/ml IC 50 values, respectively. Therefore, it can be indicated from these results, that obtained LPs, owing to their good functional properties, may be considered as potential ingredients of health-promoting food and cosmetic products.

The aim of this study was to provide new approach in creating gluten-containing and gluten-free breads without additives by combining thermal and hydrothermal pretreatments of flours (rye, oat, sorghum and millet). The applied methodology included determinations of chemical composition of flours and breads, water absorption index, empirical and fundamental rheological measurements, and scanning electron microscopy, differential scanning calorimetry, colour, textural and sensory evaluations of breads. Novel rye, oat, sorghum and millet breads based on the blend of heat treated and extruded corresponding flours in ratio 70:30 were produced by conventional breadmaking process. All breads were characterized by increased fibre content and had appearance similar to common wheat bread. Gluten-free breads were harder, less elastic with more granular structure due to higher degree of starch crystallinity. Mixolab curves indicated on many possible ways for further breads optimisation., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

The objective of this study was to evaluate albumins profile, proteolytic and amylolytic activity level and baking performance of wheat varieties grown in two production years with different climate conditions (2011 and 2012) in four locations. The results of ANOVA showed that variety, location, production year, and their interactions all had significant effects on all tested wheat quality parameters. The enzymatic activity and specific bread volume were mainly influenced by the variety. The samples from 2012 production year, had the lower values of albumin content, proteolytic and amylolytic activity, and bread specific volume. The correlation analysis, performed for 2011 production year, showed that albumin fraction (15-30 kDa) and proteolytic activity were negatively correlated with bread specific volume indicating the role of this fraction on lowering the crucial bread quality parameter. In 2012 production year, albumin fractions (5-15 kDa; 50-65 kDa) showed the most correlations, especially with parameters of bread quality., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

The aim of the present study was to determine the characteristics of an analytical method for determination of free sulphydryl (SH) groups of wheat gluten performed with previous gluten incubation for variable times (45, 90 and 135min) at variable temperatures (30 and 37°C), in order to determine its fitness-for-purpose. It was observed that the increase in temperature and gluten incubation time caused the increase in the amount of free SH groups, with more dynamic changes at 37°C. The method characteristics identified as relevant were: linearity, limit of detection, limit of quantification, precision (repeatability and reproducibility) and measurement uncertainty, which were checked within the validation protocol, while the method performance was monitored by X- and R-control charts. Identified method characteristics demonstrated its acceptable fitness-for-purpose, when assay included previous gluten incubation at 30°C. Although the method repeatability at 37°C was acceptable, the corresponding reproducibility did not meet the performance criterion on the basis of HORRAT value (HORRAT<2)., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Introduction: Dystrophic calcifications are the most common subtype of skin calcinosis. Tumorous soft tissue calcium deposits usually contain hydroxyapatite and amorphous calcium phosphate. Differential diagnosis of skin calcinosis encompasses Thibierge-Weissenbach syndrome, systemic sclerosis, scleroderma, CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia), dermatomyositis, systemic lupus erythematosus, ad myositis ossificans progressiva., Case Outline: We present the case of an eight-year old girl with tumorous soft tissue calcium deposits and Raynaud's phenomenon. At the age of 3.5 years, our patient was admitted to Pediatric Surgery Clinic because of bilateral acrocyanosis localized at the fingertips area of hands, with the signs of vascular trauma. Therapy with vasodilators and hyperbaric oxygen treatment were completed. This therapy resulted in improvement. At the age of eight, the patient was admitted again due to intermittent, painful cramps localized in both hands. Punctiform deposits were present at the tips of fingers and toes, which looked like calcifications and were spontaneously eliminated, with the remnants of crater-shaped defects. A hard tumorous deformity localized in soft tissue was present in the extensor area of the right elbow. Laboratory indicators of inflammation were within the reference values, and antinuclear antibodies were positive. A nodus localized at the right elbow was extirpated. Pathohistological findings: connective and fat tissue with large deposits of calcium., Conclusion: Further follow-up of our patient is necessary due to possible development of complete picture of CREST syndrome or systemic sclerosis.

Introduction: This article summarizes the contribution of epidemiology to the understanding of childhood asthma. The first task in epidemiology is to determine prevalence and incidence of any disease., Prevalence: Epidemiological investigations are aimed at evaluating hypotheses about causes of disease by defining demographic characteristics of a certain population as well as by determining possible effects of environmental factors. In spite of some limitations, data obtained by epidemiological investigations have been valuable in confirming both the increasing incidence of asthma and the differences in prevalence in certain population groups. The observance of this phenomenon has led to much speculation and a lot of attempts to identify the reasons behind the rising prevalence., Risk Factors: Epidemiological studies have identified risk factors for the development of childhood asthma and provided insight into natural history of disease and prognosis. Factors ranging from increased numbers of immunizations to increased air pollution have been suggested, but subsequent analysis has failed to provide the supporting evidence to implicate most of these possibilities. The concept known as the hygiene hypothesis has gained some support from epidemiological studies., Conclusion: The development of asthma as well as its severity are affected by numerous factors and their interactions can he explained by the heterogeneous nature of this disease.

Introduction: The natural course of asthma is unpredictable and appears to be unaffected by any therapeutic strategy. Under such circumstances, the attention must be focused on the opportunities for prevention of a disease which is chronic, life long and incurable, even thought it can be very effectively controlled. During the past decades, a lot of a studies have been performed and started, in which relatively large numbers of children were included and followed prospectively to determine the incidence of risk factors for asthma in childhood. All these studies have contributed significant new information. The levels of prevention must be considered in all patients. There are two main separate components to the strategy., Primary Prophylaxis: Primary prophylaxis (time course of allergic sensitization, timing of exposure to allergens, influence of tobacco smoke, maternal health and allergen exposure) is introduced before there is any evidence of sensitization to factors which might have caused the disease. There is increasing evidence that allergic sensitization is a very common precursor to the development of asthma., Secondary Prophylaxis: Secondary prophylaxis (allergen avoidance, hygiene hypothesis) is important after primary sensitization to allergen has occurred, but before there is any evidence of asthma., Conclusion: In this article the authors reviewed all results of studies about primary and secondary prophylaxis of asthma and its influence on the course of disease.

Hyperimmunoglobulin-E syndrome (HIES) is a rare immunodeficiency disorder that is characterized by elevated serum concentration of IgE, eosinophilia and severe, recurrent bacterial and fungal infections. Poor regulation of immune system is evident, with decreased production of cytokines, especially interferon. Production of specific antibodies to capsular polysaccharide antigens is decreased Skeletal malformations have been reported in these patients. They can be caused by excessive production of interleukin-4, which may lead to pathologic bony tissue resorption. Due to immune system deficiency and malformations of skeletal and connective tissue, HIES is a multisystem disorder. We present a patient with recurrent bacterial infections since the early age. At the age of two years he presented with severe destructive staphylococcal pneumonia with pleural effusion, pneumatocela formation and pneumothorax. The patient also had a dysmorphic face and skeletal malformations that were most evident at the head. The diagnosis of HIES was made on the basis of elevated serum concentrations of IgE, hypereosinophily, and decreased leukocyte function in vivo and in vitro. Family history of our patient showed an autosomal-dominant inheritance pattern of HIES.

Introduction: Food allergy is a form of adverse food reaction to different nutritive agents caused by immunological mechanisms. This condition is frequent in individuals with genetic predisposition. Frequency of food allergies in childhood varies in general population from 0.3-7.5%. Characteristics of neonatal and infant gastrointestinal tracts are described, as well as the role of gastrointestinal tract's immunological immaturity in development of food sensitivity., Pathogenesis: Features of physical and immunological barriers of gastrointestinal tract are described as well as their role in physiological conditions, in digestion and absorption of foreign proteins and prevention of foreign agents penetration and potentially dangerous proteins into the systemic circulation. Gastrointestinal-associated lymphoid tissue (GALT) is the basic part of immunological barrier. Its first role is to induce oral tolerance. In normal conditions cytokins induce T cells towards Th2 response and also promote synthesis of IgA antibodies. Pathogenic mechanisms in evolution of reaginic hypersensibility in cases of chronic inflammation are also described. Pathological status of gama/delta T cells plays an important role in this process, resulting in loss of oral tolerance and development of sensibilitivity., Conclusion: Diagnostic procedures are multiple and complex. They consist of detailed history taking, clinical findings, in vitro and in vivo tests, endoscopy, elimination diets and oral provocation tests. Food allergy in childhood is often transient and resolves spontaneously.