A kwashiorkor case due to the use of an exclusive rice milk diet to treat atopic dermatitis.

Although several cases of severe hypoalbuminemia resulting from rice milk have been described in the past, today the use of rice milk without nutritional counseling to treat eczema is still a continuing, poor practice. We describe a kwashiorkor case in an infant with severe eczema exclusively fed with rice milk. It is well documented that rice milk is not a sufficient protein source. Moreover, only a small portion of eczema is triggered by food allergy. In conclusion this case raises the importance of managing dietary changes facing food allergies with responsibility for specialized consensus among pediatricians, nutritionists, endocrinologists and allergists all of them specialist professionals

Impact of IL-28B polymorphisms on pegylated interferon plus ribavirin treatment response in children and adolescents infected with HCV genotypes 1 and 4

IL-28B polymorphisms are predictors of response to therapy in adults infected with hepatitis C. We do not know whether they are markers of response to therapy in children and adolescents. The aim of this study was to determine whether single-nucleotide polymorphisms (SNPs) in the IL-28B gene could influence the probability of response to therapy compared with other known baseline prognostic factors and correlate with clinical findings in pediatric patients infected with hepatitis C virus (HCV) genotypes 1 or 4. We determined three SNPs of IL-28B (rs12979860, rs12980275, and rs8099917) in 82 patients with chronic HCV infection treated with pegylated interferon alpha and ribavirin (peg-IFNα/RBV). Treatment response and clinical data were analyzed. Overall, sustained virological response (SVR) was achieved by 45 % of patients infected with difficult-to-treat HCV genotypes 1 and 4. Except for IL-28B polymorphisms, there was no association of SVR with any other clinical data. IL-28B rs12979860 CC [odds ratio (OR), 6.81; p = 0.001] and rs8099917 TT (OR, 3.14; p = 0.013) genotypes were associated with higher SVR rates. IL-28B rs12980275 was not significantly associated with SVR ( p = 0.058). Only the distribution between CC and CT-TT genotypes of rs12979860 significantly differentiated patients achieving early virological response (EVR) (OR, 10.0; p = 0.011). Children with the rs12979860 CC genotype had significantly higher baseline viral load compared with CT-TT patients ( p = 0.010). In children and adolescents chronically infected with HCV genotypes 1 and 4, IL-28B rs12979860 and rs8099917 polymorphisms were the only predictors of response to peg-IFN/RBV

NBAS variants are associated with quantitative and qualitative NK and B cell deficiency

Purpose Biallelic pathogenic NBAS variants manifest as a multisystem disorder with heterogeneous clinical phenotypes such as recurrent acute liver failure, growth retardation, and susceptibility to infections. This study explores how NBAS-associated disease affects cells of the innate and adaptive immune system. Methods Clinical and laboratory parameters were combined with functional multi-parametric immunophenotyping methods in fifteen NBAS-deficient patients to discover possible alterations in their immune system. Results Our study revealed reduced absolute numbers of mature CD56dim natural killer (NK) cells. Notably, the residual NK cell population in NBAS-deficient patients exerted a lower potential for activation and degranulation in response to K562 target cells, suggesting an NK cell–intrinsic role for NBAS in the release of cytotoxic granules. NBAS-deficient NK cell activation and degranulation was normalized upon pre-activation by IL-2 in vitro, suggesting that functional impairment was reversible. In addition, we observed a reduced number of naïve B cells in the peripheral blood associated with hypogammaglobulinemia. Conclusion In summary, we demonstrate that pathogenic biallelic variants in NBAS are associated with dysfunctional NK cells as well as impaired adaptive humoral immunity

The development of a hyperspectral imaging method for the detection of Fusarium-damaged, yellow berry and vitreous Italian durum wheat kernels

The possibility of using hyperspectral imaging (HSI) techniques to classify different types of wheat kernels, vitreous, yellow berry and Fusarium-damaged, was investigated. Conventional optical techniques adopted by industry for wheat grain sorting usually have too high misclassification errors. Reflectance spectra of selected wheat kernels of the three types were acquired by a laboratory device equipped with an HSI system working in near infrared field (1000-1700 nm). The hypercubes were analysed applying different chemometric techniques, such as principal component analysis (PCA) for explorative purposes, partial least squares discriminant analysis (PLS-DA) for classification of the three wheat types and interval PLS-DA (iPLS-DA) for the selection of a reduced set of effective wavelength intervals. The study demonstrated that good classification results were obtained not only considering the entire investigated wavelength range, but also selecting only three narrow intervals of four wavelengths (1209-1230 nm, 1489-1510 nm and 1601-1622 nm) out of 121. The procedures developed could be utilised at industrial level for quality control purposes or for the definition of innovative sorting logics for wheat kernels after an extensive classification campaign, both at laboratory and industrial level, applied to a large wheat sample sets

Efficient chemometric strategies for PET–PLA discrimination in recycling plants using hyperspectral imaging

The effectiveness of Hyperspectral imaging (HSI) in the near infrared (NIR) range (1000–1700 nm) was evaluated to discriminate PET (polyethylene terephthalate) from PLA (poly(lactic acid)), two polymers commonly utilized as packaging for foodstuff, in order to improve their further recycling process. An internal calibration based on five reference materials was initially used to eliminate the variability existing among images, then Partial Least Squares-Discriminant Analysis (PLS-DA) was used to distinguish and classify the three classes, i.e., background, PET and PLA. Considering the high amount of data conveyed by the training image, the PLS-DA models were also calculated using as training set a reduced version of the original matrix, with the twofold aim to reduce the computational time and to deal with an equal number of spectra for each class, independently from the initial selected areas. A variable selection procedure by means of iPLS-DA was also applied on both the whole and the reduced matrix. The results obtained on the reduced matrix using only six variables provided a prediction efficiency higher than 98%. Moreover, the possibility to recognize PET and PLA polymers by HSI in the NIR range was further confirmed by using Multivariate Curve Resolution (MCR) as an alternative approach, which also allowed to evaluate the effect of thickness of the transparent plastic samples

Aortic arch interruption and persistent fifth aortic arch in phace syndrome: Prenatal diagnosis and postnatal course

PHACE is a rare congenital neurocutaneous syndrome where posterior fossa malformations, hemangiomas, cerebrovascular anomalies, aortic arch anomalies, cardiac defects, and eye abnormalities are variably associated. We describe the prenatal detection and the postnatal course of a child with PHACE syndrome with a unique type of aortic arch anomaly consisting of proximal interruption of the aortic arch and persistence of the fifth aortic arch. The fifth aortic arch represented in this case a vital systemic-to-systemic connection between the ascending aorta and the transverse portion of the aortic arch allowing adequate forward flow through the aortic arch without surgical treatment. Mini-Abstract PHACE syndrome is a rare congenital neurocutaneous disease where posterior fossa malformations, hemangiomas, cerebrovascular anomalies, aortic arch anomalies, cardiac defects, and eye abnormalities are variably associated. We describe the prenatal detection and the postnatal course of a child with PHACE syndrome with a unique type of aortic arch anomaly consisting of proximal interruption of the aortic arch and persistence of the fifth aortic arch. The fifth aortic arch represented in this case a vital systemic-to-systemic connection between the ascending aorta and the transverse portion of the aortic arch allowing adequate forward flow through the aortic arch without surgical treatment