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This perspective reviews the definition and current understanding of necrotizing enterocolitis and evaluates a future prevention approach to this multifactorial disease. An overview of the prevention approach in general is presented, where key aspects and emerging criticisms are identified. In addition, key elements of early diagnosis and treatment are presented, together with some of their challenges and ambiguities. Moreover, it concludes with emerging questions from the global community to reach a consensus on the definition, diagnosis, and management of necrotizing enterocolitis disease., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Repeated polygonal patterns are pervasive in natural forms and structures. These patterns provide inherent structural stability while optimizing strength-per-weight and minimizing construction costs. In echinoids (sea urchins), a visible regularity can be found in the endoskeleton, consisting of a lightweight and resistant micro-trabecular meshwork (stereom). This foam-like structure follows an intrinsic geometrical pattern that has never been investigated. This study aims to analyse and describe it by focusing on the boss of tubercles-spine attachment sites subject to strong mechanical stresses-in the common sea urchin Paracentrotus lividus . The boss microstructure was identified as a Voronoi construction characterized by 82% concordance to the computed Voronoi models, a prevalence of hexagonal polygons, and a regularly organized seed distribution. This pattern is interpreted as an evolutionary solution for the construction of the echinoid skeleton using a lightweight microstructural design that optimizes the trabecular arrangement, maximizes the structural strength and minimizes the metabolic costs of secreting calcitic stereom. Hence, this identification is particularly valuable to improve the understanding of the mechanical function of the stereom as well as to effectively model and reconstruct similar structures in view of future applications in biomimetic technologies and designs.

In biomimetic design, functional systems, principles, and processes observed in nature are used for the development of innovative technical systems. The research on functional features is often carried out without giving importance to the generative mechanism behind them: evolution. To deeply understand and evaluate the meaning of functional morphologies, integrative structures, and processes, it is imperative to not only describe, analyse, and test their behaviour, but also to understand the evolutionary history, constraints, and interactions that led to these features. The discipline of palaeontology and its approach can considerably improve the efficiency of biomimetic transfer by analogy of function; additionally, this discipline, as well as biology, can contribute to the development of new shapes, textures, structures, and functional models for productive and generative processes useful in the improvement of designs. Based on the available literature, the present review aims to exhibit the potential contribution that palaeontology can offer to biomimetic processes, integrating specific methodologies and knowledge in a typical biomimetic design approach, as well as laying the foundation for a biomimetic design inspired by extinct species and evolutionary processes: Paleomimetics. A state of the art, definition, method, and tools are provided, and fossil entities are presented as potential role models for technical transfer solutions.

Background: Transposable elements (TEs) widely contribute to the evolution of genomes allowing genomic innovations, generating germinal and somatic heterogeneity, and giving birth to long non-coding RNAs (lncRNAs). These features have been associated to the evolution, functioning, and complexity of the nervous system at such a level that somatic retrotransposition of long interspersed element (LINE) L1 has been proposed to be associated to human cognition. Among invertebrates, octopuses are fascinating animals whose nervous system reaches a high level of complexity achieving sophisticated cognitive abilities. The sequencing of the genome of the Octopus bimaculoides revealed a striking expansion of TEs which were proposed to have contributed to the evolution of its complex nervous system. We recently found a similar expansion also in the genome of Octopus vulgaris. However, a specific search for the existence and the transcription of full-length transpositionally competent TEs has not been performed in this genus., Results: Here, we report the identification of LINE elements competent for retrotransposition in Octopus vulgaris and Octopus bimaculoides and show evidence suggesting that they might be transcribed and determine germline and somatic polymorphisms especially in the brain. Transcription and translation measured for one of these elements resulted in specific signals in neurons belonging to areas associated with behavioral plasticity. We also report the transcription of thousands of lncRNAs and the pervasive inclusion of TE fragments in the transcriptomes of both Octopus species, further testifying the crucial activity of TEs in the evolution of the octopus genomes., Conclusions: The neural transcriptome of the octopus shows the transcription of thousands of putative lncRNAs and of a full-length LINE element belonging to the RTE class. We speculate that a convergent evolutionary process involving retrotransposons activity in the brain has been important for the evolution of sophisticated cognitive abilities in this genus., (© 2022. The Author(s).)

In the field of structural engineering, lightweight and resistant shell structures can be designed by efficiently integrating and optimizing form, structure and function to achieve the capability to sustain a variety of loading conditions with a reduced use of resources. Interestingly, a limitless variety of high-performance shell structures can be found in nature. Their study can lead to the acquisition of new functional solutions that can be employed to design innovative bioinspired constructions. In this framework, the present study aimed to illustrate the main results obtained in the mechanical analysis of the echinoid test in the common sea urchin Paracentrotus lividus (Lamarck, 1816) and to employ its principles to design lightweight shell structures. For this purpose, visual survey, photogrammetry, three-dimensional modelling, three-point bending tests and finite-element modelling were used to interpret the mechanical behaviour of the tessellated structure that characterize the echinoid test. The results achieved demonstrated that this structural topology, consisting of rigid plates joined by flexible sutures, allows for a significant reduction of bending moments. This strategy was generalized and applied to design both free-form and form-found shell structures for architecture exhibiting improved structural efficiency., Competing Interests: We declare we have no competing interests., (© 2022 The Authors.)

Organisms and their features represent a complex system of solutions that can efficiently inspire the development of original and cutting-edge design applications: the related discipline is known as biomimetics. From the smallest to the largest, every species has developed and adapted different working principles based on their relative dimensional realm. In nature, size changes determine remarkable effects in organismal structures, functions, and evolutionary innovations. Similarly, size and scaling rules need to be considered in the biomimetic transfer of solutions to different dimensions, from nature to artefacts. The observation of principles that occur at very small scales, such as for nano- and microstructures, can often be seen and transferred to a macroscopic scale. However, this transfer is not always possible; numerous biological structures lose their functionality when applied to different scale dimensions. Hence, the evaluation of the effects and changes in scaling biological working principles to the final design dimension is crucial for the success of any biomimetic transfer process. This review intends to provide biologists and designers with an overview regarding scale-related principles in organismal design and their application to technical projects regarding mechanics, optics, electricity, and acoustics.

The endoskeleton of echinoderms ( Deuterostomia: Echinodermata ) is of mesodermal origin and consists of cells, organic components, as well as an inorganic mineral matrix. The echinoderm skeleton forms a complex lattice-system, which represents a model structure for naturally inspired engineering in terms of construction, mechanical behaviour and functional design. The sea urchin ( Echinodermata: Echinoidea ) endoskeleton consists of three main structural components: test, dental apparatus and accessory appendages. Although, all parts of the echinoid skeleton consist of the same basic material, their microstructure displays a great potential in meeting several mechanical needs according to a direct and clear structure-function relationship. This versatility has allowed the echinoid skeleton to adapt to different activities such as structural support, defence, feeding, burrowing and cleaning. Although, constrained by energy and resource efficiency, many of the structures found in the echinoid skeleton are optimized in terms of functional performances. Therefore, these structures can be used as role models for bio-inspired solutions in various industrial sectors such as building constructions, robotics, biomedical and material engineering. The present review provides an overview of previous mechanical and biomimetic research on the echinoid endoskeleton, describing the current state of knowledge and providing a reference for future studies., (Creative Commons Attribution license.)

Iron deficiency represents a widespread problem for a large part of the population, especially for women, and has received increasing attention in food/supplement research. The contraindications of the iron supplements commercially available (e.g., imbalances in the levels of other essential nutrients, low bioavailability, etc.) led us to search for a possible alternative. In the present work, a rapid and easy method to synthetize a solid iron (II) citrate complex from iron filings and citric acid was developed to serve, eventually, as a food supplement or additive. In order to state its atomic composition and purity, an assortment of analytical techniques was employed (e.g., combustion analysis, thermogravimetry, X-ray diffractometry, UV/Vis spectrophotometry, etc.). Results demonstrate that the synthesized crystalline solid corresponds to the formula FeC₆H₆O₇∙H₂O and, by consequence, contains exclusively iron (II), which is an advantage with respect to existing commercial products, because iron (II) is better absorbed than iron (III) (high bioavailability of iron).

Objective: Gastroesophageal reflux disease is very common in industrialized countries and rapidly and significantly increasing even in developing countries. The approach in this study is one not commonly found to date in the scientific literature. To assess the ability of reduced-carbohydrate diets and foods that are enriched with acid potential of hydrogen (pH; lemon and tomato) to quickly and exponentially reduce symptoms that are related to conditions such as gastritis and gastroesophageal reflux and unrelated to Helicobacter pylori., Methods: After the administration of an anamnestic test, 130 patients were selected including 73 women and 57 men, 21 to 67 y, and with a gastritis diagnosis for 92 patients (56 women, 36 men) and reflux gastritis for 38 patients (17 women, 21 men). Study participants followed three dietary treatments in succession. Each treatment lasted 2 wk and treatments were separated by 2 wk of washout. The patients followed a diet that consisted primarily of proteins and fats and included the exponential reduction of glycides (simple and complex). In addition, the treatment provided for the daily intake of the juice of two lemons and approximately 100 g of fresh orange tomato without seeds eaten either raw or cooked and peeled., Results: During treatment and at the end of 2 wk of treatment, the patients reported significant improvements including an almost total disappearance of symptoms that were related to the disease in question., Conclusions: This study shows that a carbohydrate-free diet and/or highly hypoglycidal diet that is enriched with acid pH foods appears to lead to a decrease in the pH of the gastric contents, thus inhibiting the further production of hydrochloric acid with a reduction or disappearance of heartburn symptoms that are typical of gastroesophageal diseases., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Pasta with eggs added (generally termed "special pasta" for Italian legislation) is made by adding no less than 4 eggs without shells (or no less than 200 g of liquid or lyophilized egg product) per kilogram of semolina, as provided by law. In this work, to determine the final content of eggs added to dough, an analytical procedure was developed for the rapid analysis of the cholesterol content in the finished pastas. The proposed procedure was simpler, faster, and more accurate than that of official methods of analysis based on the gravimetric determination of sterols. Moreover, the determination of the quality of fat content in the special pasta (egg pasta in this case) allowed the evaluation of its origin, avoiding possible fraud resulting from the addition of foreign fat as an alternative to fat derived from eggs. In this new gas chromatographic procedure, the internal standard squalene for the quantification of cholesterol was used because a more polar GC capillary column was used (RTX 65 TG-HT) for the separation of sterols, rather than 5% phenyl methylsilicone. The ratio between cholesterol and squalene allowed for the determination of the number of eggs added, while from analysis of the same gas chromatogram, it was also possible to evaluate the composition of triglycerides in the fat contained in the pasta, allowing discrimination of foreign fats with respect to fats contained in eggs and therefore avoiding adulteration of pasta. The same analytical procedure was applied to the determination of cholesterol content in lyophilized yolk.

Neuroblastoma (NBL) accounts for >7% of malignancies in patients younger than 15 years. Low- and intermediate-risk patients exhibit excellent or good prognosis after treatment, whereas for high-risk (HR) patients, the estimated 5-year survival rates is still <40%. The ability to stratify HR patients that will not respond to standard treatment strategies is critical for informed treatment decisions. In this study, we have generated a specific kinome gene signature, named Kinome-27, which is able to identify a subset of HR-NBL tumors, named ultra-HR NBL, with highly aggressive clinical behavior that not adequately respond to standard treatments. We have demonstrated that NBL cell lines expressing the same kinome signature of ultra-HR tumors (ultra-HR-like cell lines) may be selectively targeted by the use of two drugs [suberoylanilide hydroxamic acid (SAHA) and Radicicol], and that the synergic combination of these drugs is able to block the ultra-HR-like cells in G2/M phase of cell cycle. The use of our signature in clinical practice will allow identifying patients with negative outcome, which would benefit from new and more personalized treatments. Preclinical in vivo studies are needed to consolidate the SAHA and Radicicol treatment in ultra-HR NBL patients., (© The Author 2017. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

This paper deals with the environmental characterization of a large and densely populated area, with a poor reputation for contamination, considering the contribution of environmental features (air, soil, soil hydraulic and groundwater) and the potential effects on human health. The use of Geographic Information System (GIS) has made possible a georeferenced inventory and, by overlaying environmental information, an operational synthesis of comprehensive environmental conditions. The cumulative effects on environmental features were evaluated, taking into account superposition effects, by means of the Spatial MultiCriteria Decision Analysis (S-MCDA). The application of the S-MCDA for converging the combination of heterogeneous factors, related to soil, land and water, deeply studied by heterogeneous groups of experts, constitutes the novelty of the paper. The results confirmed an overall higher potential of exposure to contaminants in the environment and higher mortality rates in the study area for some tumours, but hospital admissions for tumours were generally similar to the regional trend. Besides, mortality data may be strictly dependent on the poor socioeconomic conditions, quality of therapy and a lack of welfare in the area relative to the rest of Italy. Finally, as regards the possible relationship between presence of contaminants in the environment and health conditions of the population no definite conclusions can be drawn, although the present study encourages the use of the new proposed methods, that increase the possibilities for studying the combined effect of more environmental factors., Competing Interests: The authors declare no conflict of interest.

We have previously studied the function of microRNAs (miRNAs) in thyroid cells using the differentiated rat thyroid PC Cl 3 cells that need thyrotropin (TSH) for their growth. The miRNA expression profile examination allowed the detection of a set of miRNAs downregulated and upregulated by TSH. Here, we first demonstrated that upregulation of miR-130b-3p occurs through a protein kinase A-cAMP-responsive element binding protein (CREB)-dependent mechanism. Then, we analyzed its expression in human thyroid follicular adenomas, where a constitutive CREB activation is frequently present. miR-130b-3p results in upregulation with a high fold-change in most thyroid follicular adenomas. Then, we identified CCDC6, coding for a protein that interacts with CREB1 leading to the transcriptional repression of CREB1 target genes, as a target of this miRNA. The targeting of CCDC6 by miR-130b-3p likely accounts for the mechanism by which its upregulation contributes to the development of thyroid adenomas increasing CREB1 activity.

CCDC6 was originally identified upon rearrangement with RET in human thyroid papillary carcinomas generating the RET/PTC1 oncogene. We have previously reported that CCDC6 interacts with CREB1 and represses its transcriptional activity. Since the function of at least one allele of CCDC6 is lost following RET/PTC1 rearrangements, we aimed at the generation of mice, carrying a CCDC6 mutant gene. Previous studies suggested that the coiled-coil domain of CCDC6, mainly encoded by human exon 2, is required for the protein function. Therefore, we engineered a murine Ccdc6 construct, carrying a deletion of the exon 2, that was able to exert only a mild repression on CREB1 transcriptional activity, with respect to the wild type Ccdc6. Subsequently, we generated Ccdc6-ex2 knock-in mice. These mice developed thyroid hyperplasia associated with an enhanced CREB1 activity and an increased expression of the CREB-1 regulated genes. These results strongly support a CCDC6 promoting role, ascribed to its functional impairment, in the development of thyroid papillary carcinomas harboring the RET/PTC1 oncogene.

Congenital erythropoietic porphyria (CEP) is a rare genetic disease that is characterized by a severe cutaneous photosensitivity causing unrecoverable deformities, chronic hemolytic anemia requiring blood transfusion program, and by fatal systemic complications. A correct and early diagnosis is required to develop a management plan that is appropriate to the patient's needs. Recently only one case of X-linked CEP had been reported, describing the trans-acting GATA1-R216W mutation. Here, we have characterized two novel X-linked CEP patients, both with misleading hematological phenotypes that include dyserythropoietic anemia, thrombocytopenia, and hereditary persistence of fetal hemoglobin. We compare the previously reported case to ours and propose a diagnostic paradigm for this variant of CEP. Finally, a correlation between phenotype variability and the presence of modifier mutations in loci related to disease-causing gene is described., (© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

We have previously reported that the expression of the CL2/CCDC80 gene is downregulated in human papillary thyroid carcinomas, particularly in follicular variants. We have also reported that the restoration of CL2/CCDC80 expression reverted the malignant phenotype of thyroid carcinoma cell lines and that CL2/CCDC80 positively regulated E-cadherin expression, an ability that likely accounts for the role of the CL2/CCDC80 gene in thyroid cancer progression. In order to validate the tumour suppressor role of the CL2/CCDC80 gene in thyroid carcinogenesis we generated cl2/ccdc80 knock-out mice. We found that embryonic fibroblasts from cl2/ccdc80(-/-) mice showed higher proliferation rate and lower susceptibility to apoptosis. Furthermore, cl2/ccdc80(-/-) mice developed thyroid adenomas and ovarian carcinomas. Finally, ret/PTC1 transgenic mice crossed with the cl2/ccdc80 knock-out mice developed more aggressive thyroid carcinomas compared with those observed in the single ret/PTC1 transgenic mice. Together, these results indicate CL2/CCDC80 as a putative tumour suppressor gene in human thyroid carcinogenesis., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Objectives: Currently, there are numerous studies on risk factors for cardiovascular disease and the potential for functional foods to bring benefits or improve people's health. However, most of these studies are conducted with middle-aged individuals. The aim of this study was to evaluate the effects of supplementing a typical diet with some functional components, which are substances that when consumed in small quantities can improve people's welfare., Methods: The participants in this study were young; slightly overweight; had normal glucose tolerance; and had lipid values indicating dyslipidemia or close dyslipidemia. Following a 4-wk run-in phase, participants followed either a diet containing foods enriched with ω-3 fatty acids, β-glucans, phytosterols, and vitamin E or an isoenergetic diet without the active components. Sixteen individuals (age range 20 to 37 y) were randomly assigned to one of two groups. At the end of treatment, while fasting, plasma concentrations of triglycerides, high-density lipoprotein cholesterol, low-density lipoprotein cholesterol, and total cholesterol were measured. Furthermore, blood glucose was evaluated after fasting and after a meal enriched with β-glucans., Results: There was a statistically significant reduction (α < 0.05) across the lipid profile. A meal rich in β-glucans produced a glycemic response significantly lower than the nonenriched meal., Conclusion: The dietary supplements used in this work, based on the integration of functional components into the usual diet of the population, have proved effective in reducing peak levels of postprandial glucose and the risk for dyslipidemia. Therefore, these functional components proved a valuable aid in the prevention of cardiovascular diseases and metabolic disorders., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Evolution shows that photonic structures are a constituent part of many animals and flora. These elements produce structural color and are useful in predator-prey interactions between animals and in the exploitation of light for photosynthetic organisms. In particular, diatoms have evolved patterned hydrated silica external valves able to confine light with extraordinary efficiency. Their evolution was probably guided by the necessity to survive in harsh conditions of sunlight deprivation. Here, we exploit such diatom valves, in conjunction with structured illumination, to realize a biological super-resolving lens to achieve sub-diffractive focusing in the far field. More precisely, we consider a single diatom valve of Arachnoidiscus genus which shows symmetries and fine features. By characterizing and using the transmission properties of this valve using the optical eigenmode technique, we are able to confine light to a tiny spot with unprecedented precision in terms of resolution limit ratio, corresponding in this case to 0.21λ/NA.

Congenital Dyserythropoietic Anemia II (CDA II) is a rare hyporegenerative anemia of variable degree, whose causative gene is SEC23B. More than 60 causative mutations in 142 independent pedigrees have been described so far. However, the prevalence of the CDA II is probably underestimated, since its clinical spectrum was not yet well-defined and thus it is often misdiagnosed with more frequent clinically-related anemias. This study represents the first meta-analysis on clinical and molecular spectrum of CDA II from the largest cohort of cases ever described. We characterized 41 new cases and 18 mutations not yet associated to CDA II, thus expanding the global series to 205 cases (172 unrelated) and the total number of causative variants to 84. The 68.3% of patients are included in our International Registry of CDA II (Napoli, Italy). A genotype-phenotype correlation in three genotypic groups of patients was assessed. To quantify the degree of severity in each patient, a method based on ranking score was performed. We introduced a clinical index to easily discriminate patients with a well-compensated hemolytic anemia from those with ineffective erythropoiesis. Finally, the worldwide geographical distribution of SEC23B alleles highlighted the presence of multiple founder effects in different areas of the world., (© 2014 Wiley Periodicals, Inc.)

MicroRNA (miRNA) deregulation plays a critical role in tumorigenesis. miR-23b and miR-130b are induced by thyrotropin in thyroid cells in a cAMP-dependent manner. The aim of our work has been to investigate the possible role of miR-23b and miR-130b in pituitary tumorigenesis. We have analyzed their expression in a panel of pituitary adenomas (PAs) including GH and NFPA adenomas. We report that miR-23b and miR-130b are drastically reduced in GH, gonadotroph and NFPA adenomas in comparison with normal pituitary gland. Interestingly, the overexpression of miR-23b and miR-130b inhibits cell proliferation arresting the cells in the G1 and G2 phase of the cell cycle, respectively. Moreover, we demonstrate that miR-23b and miR-130b target HMGA2 and cyclin A2 (CCNA2) genes, respectively. Finally, downregulation of miR-23b and miR-130b expression is associated with increased levels of their respective targets in human PAs. These findings suggest that miR-23b and miR-130b downregulation may contribute to pituitary tumorigenesis., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

CDA are a group of inherited, rare diseases that are characterized by dyserythropoiesis and ineffective erythropoiesis associated with transfusion dependency in approximately 10% of cases. For these latter patients, the only curative treatment is HSCT. There are very limited data on HSCT experience in this rare disease. Herein, we report a five-yr six-month-old girl with compound heterozygous mutations in SEC23B gene, who was diagnosed to have CDA type II and underwent successful HSCT from her matched sibling donor., (© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Congenital dyserythropoietic anemia type II, a recessive disorder of erythroid differentiation, is due to mutations in SEC23B, a component of the core trafficking machinery COPII. In no case homozygosity or compound heterozygosity for nonsense mutation(s) was found. This study represents the first description of molecular mechanisms underlying SEC23B hypomorphic genotypes by the analysis of five novel mutations. Our findings suggest that reduction of SEC23B gene expression is not associated with CDA II severe clinical presentation; conversely, the combination of a hypomorphic allele with one functionally altered results in more severe phenotypes. We propose a mechanism of compensation SEC23A-mediated which justifies these observations., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Introduction: Venous malformations are usually easy to recognise. We describe one case in which the clinical aspect was suggestive of Maffucci's syndrome., Observation: A 44-year-old male, had numerous angiomatous nodules which could be emptied by pressure since infancy. The progressive increase in volume of these lesions on the arms and forearms led to enormous deformation and major disability. The thoracic area and the right foot were also affected to a lesser degree. Two endobuccal lesions were also found on the clinical examination The cutaneous lesions were tender and occasionally hyperhidrosis was present. The presence of phleboliths on Xray and the presence of venous and capillary cavities with numerous thrombi confirmed the diagnosis of venous malformation. Following the failure of the Ethibloc(R) embolization, eight excisions were made in a two-year-period with a good functional result. Elastic strapping was then applied., Discussion: The diagnosis of venous malformation was based on clinical, radiological and histological findings. The absence of chondroma excluded the diagnosis of Maffucci's syndrome. Spindle cell hemangioendothelioma, "blue rubber bleb nevus" and glomangiomatosis were confirmed by the histological findings. The absence of port-wine stain or bone hypertrophy and the presence of multiple nodules excluded Klippel-Trenaunay syndrome. The presence of multifocal involvement with oral lesions, the severity of the deformation and the relative success of the surgical procedure make this case unusual.

The authors evaluated technical performance of two automated haematological counters, the Coulter T-660 in comparison to Hemalog 8/90. Both systems showed a good correlation and a good precision. The Coulter T-660 revealed to be a high standard analyzer, that can adequately support more complex haematological analyzers in laboratory routine.