Validation of the children international IgA nephropathy prediction tool based on data in Southwest China.

Background: Immunoglobulin A nephropathy (IgAN) is one of the most common kidney diseases leading to renal injury. Of pediatric cases, 25%-30% progress into end-stage kidney disease (ESKD) in 20-25 years. Therefore, predicting and intervening in IgAN at an early stage is crucial. The purpose of this study was to validate the availability of an international predictive tool for childhood IgAN in a cohort of children with IgAN treated at a regional medical centre. Methods: An external validation cohort of children with IgAN from medical centers in Southwest China was formed to validate the predictive performance of the two full models with and without race differences by comparing four measures: area under the curve (AUC), the regression coefficient of linear prediction (PI), survival analysis curves for different risk groups, and R2D. Results: A total of 210 Chinese children, including 129 males, with an overall mean age of 9.43 ± 2.71 years, were incorporated from this regional medical center. In total, 11.43% (24/210) of patients achieved an outcome with a GFR decrease of more than 30% or reached ESKD. The AUC of the full model with race was 0.685 (95% CI: 0.570-0.800) and the AUC of the full model without race was 0.640 (95% CI: 0.517-0.764). The PI of the full model with race and without race was 0.816 (SE = 0.006, P < 0.001) and 0.751 (SE = 0.005, P < 0.001), respectively. The results of the survival curve analysis suggested the two models could not well distinguish between the low-risk and high-risk groups (P = 0.359 and P = 0.452), respectively, no matter the race difference. The evaluation of model fit for the full model with race was 66.5% and without race was 56.2%. Conclusions: The international IgAN prediction tool has risk factors chosen based on adult data, and the validation cohort did not fully align with the derivation cohort in terms of demographic characteristics, clinical baseline levels, and pathological presentation, so the tool may not be highly applicable to children. We need to build IgAN prediction models that are more applicable to Chinese children based on their particular data.

Oxidative stability and sensory evaluation of sodium caseinate-based yak butter powder.

Yak butter's high unsaturated fatty acid level predisposes it to oxidation, hence must be converted into more stable forms like powder. This study aimed to spray dry yak butter using 10% yak butter and four sodium caseinate (NaCas) formulations: sample A: 100% NaCas; sample B: 50% NaCas, 50% lactose; sample C: 75% NaCas, 25% lactose; and sample D: 25% NaCas, 75% maltodextrin. The powders were vacuum and hermetically sealed, and evaluated for oxidative stability, physical and sensory properties during storage at 65 â„ƒ for 30 days. The results showed that samples B and D had similar and most favorable physical properties (such as, moisture, water activity, particle size, bulk density re-dispersion time, and encapsulation efficiency); though sample B, together with sample C, browned the most during storage. The majority of the sensory panelists preferred samples B and D; observed high caking in samples C and B; and the least whiteness loss and caking in samples D and A but high off-flavors in samples A and C. After storage, peroxide and thiobarbituric acid values of powder samples ranged from 34.98 to 69.54 meqO2/kg and 1.85-9.43 mg MD/kg, respectively, in the decreasing order of A, C, B, and D. Sample D, followed by B, showed the highest radical scavenging activity. Therefore, for optimum yak butter powder physical properties and oxidative stability, 50%:50%, NaCas: lactose, and 25%:75%, NaCas: maltodextrin formulations should be used. This study provides essential knowledge for butter powder processors.

Detection of De Novo PAX2 Variants and Phenotypes in Chinese Population: A Single-Center Study.

Background: PAX2 is a nuclear transcription factor gene that is highly conserved among species. Variants within PAX2 could result in optic nerve colobomas and kidney hypoplasia. However, little clinical and genetic information is currently available about PAX2 variants in Chinese children. Objective: This study aims to further understand the clinical manifestations and genetic characteristics of PAX2 variants in Chinese population. Methods: In this single-center retrospective study, we analyzed the clinical data of 10 children identified as carriers of PAX2 variants by gene sequencing. All the variants found in this study were analyzed using in silico prediction and American College of Medical Genetics and Genomics (ACMG) standards and guidelines. Results: The mean age for developing the first symptom in 10 unrelated children was 7.2 years old. Proteinuria and bilateral kidney dysplasia were found in every patient. Two children underwent kidney histological examination; one child showed high-intensity C1q deposition in the kidney, and the other child showed focal segmental glomerular sclerosis (FSGS). Three children had PAX2-related ocular abnormalities, including nystagmus, retinal exudation, amblyopia, microphthalmia, microcornea, and total blindness. In addition, one patient had the comorbidity of oculocutaneous albinism (OCA). Eight different PAX2 variants were found in ten patients, three of which were reported for the first time. Conclusion: We reported some patients with unique manifestations and comorbidities, and we reported three variants that have not been previously identified. The PAX2 gene is prone to spontaneous variants, and the outcome of patients is unfavorable. Because of the lack of specific therapy, genetic testing should be recommended for individuals with obvious evidence of kidney dysplasia and eye abnormalities, and kidney protective treatment should be initiated early.

A Miniature Soft Sensor with Origami-Inspired Self-Folding Parallel Mechanism.

Miniature soft sensors are crucial for the perception of soft robots. Although centimeter-scale sensors have been well developed, very few works addressed millimeter-scale, three-dimensional-shaped soft sensors capable of measuring multi-axis forces. In this work, we developed a millimeter-scale (overall size of 6 mm × 11 mm × 11 mm) soft sensor based on liquid metal printing technology and self-folding origami parallel mechanism. The origami design of the sensor enables the soft sensor to be manufactured within the plane and then fold into a three-dimensional shape. Furthermore, the parallel mechanism allows the sensor to rotate along two orthogonal axes. We showed that the soft sensor can be self-folded (took 17 s) using a shape-memory polymer and magnets. The results also showed that the sensor prototype can reach a deformation of up to 20 mm at the tip. The sensor can realize a measurement of external loads in six directions. We also showed that the soft sensor enables underwater sensing with a minimum sensitivity of 20 mm/s water flow. This work may provide a new manufacturing method and insight into future millimeter-scale soft sensors for bio-inspired robots.