A new type of catalyst was synthesized by immobilizing heteropolyacid on ionic liquid-modified mesostructured cellular silica foam (denoted as MCF) and applied to the oxidative desulfurization of ...fuel. The surface morphology and structure of the catalyst were characterized by XRD, TEM, N
adsorption-desorption, FT-IR, EDS and XPS analysis. The catalyst exhibited good stability and desulfurization for various sulfur-containing compounds in oxidative desulfurization. Heteropolyacid ionic liquid-based MCF solved the shortage of the amount of ionic liquid and difficult separation in the process of oxidative desulfurization. Meanwhile, MCF had a special three-dimensional structure that was not only highly conducive to mass transfer but also greatly increased catalytic active sites and significantly improved catalytic efficiency. Accordingly, the prepared catalyst of 1-butyl-3-methyl imidazolium phosphomolybdic acid-based MCF (denoted as BMIM
PMo
O
-based MCF) exhibited high desulfurization activity in an oxidative desulfurization system. The removal of dibenzothiophene could achieve levels of 100% in 90 min. Additionally, four sulfur-containing compounds could be removed completely under mild conditions. Due to the stability of the structure, sulfur removal efficiency still reached 99.8% after the catalyst was recycled six times.
Interstitial lung disease (ILD) as an initial manifestation of lupus is rare, especially in young children. Here, we report a case of a 3-year-old boy who presented with fever, shortness of breath, ...and facial erythema. Clinical examination suggested a diagnosis of active systemic lupus erythematosus (SLE) with butterfly rash, anemia, positive antinuclear antibody, positive anti-double-stranded DNA, and hypocomplementemia. On retrospective review of the patient's records, multiple chest computed tomography (CT) images showed non-specific interstitial pneumonia + organizing pneumonia pattern, with no further autoimmune work-up during the visit to a respiratory department. In our opinion, persistent interstitial pneumonia may be a clue to connective tissue disease. The patient received steroid treatment for 1 year, and the radiological and immunological resolution was noted. However, he still suffered from cough and dyspnea. After a 1-year follow-up, he was hospitalized again for SLE relapse. While continuing corticosteroid therapy, the patient was given combination therapy consisting of cyclosporine A (CsA) and monthly-pulse cyclophosphamide for 6 months, and decreased proteinuria was noted. However, the patient's respiratory symptoms and pulmonary radiologic findings did not improve significantly. With continued steroid therapy, the patient was started on a daily regimen of CsA and pirfenidone. Both drugs were sufficiently effective to allow gradual reduction of steroid dosage. After 2 years of treatment, marked improvements in symptoms, pulmonary function and chest CT images were observed. Our experience with this case emphasizes that prompt work-up for connective tissue disease (CTD) should be considered in young children with ILD, and pirfenidone might be a useful add-on therapy with immunosuppressive agents for refractory CTD-ILD in pediatric patients. Nevertheless, further clinical trials including larger numbers of patients need to assess the efficiency and safety of this combination therapy for refractory CTD-ILD.
Oculocerebrorenal syndrome of Lowe is a rare X-linked disorder characterized by congenital cataracts, mental retardation, and proximal tubulopathy. This condition is caused by a mutation of OCRL gene ...(located at chromosome Xq26.1), which encodes an inositol polyphosphate 5-phosphatase.
We identified two novel OCRL mutations in two unrelated Chinese boys, each with a severe phenotype of Lowe syndrome. A novel de novo deletion (hemizygous c.659_662delAGGG, p.E220Vfs*29) was present in patient 1 and a novel splicing mutation (hemizygous c.2257-2A > T) that was maternally inherited was present in patient 2. A renal biopsy in patient 2 indicated mild mesangial proliferative glomerulonephritis, mild focal mononuclear cells infiltration, and interstitial focal fibrosis. Moreover, renal expression of OCRL-1 protein in patient 2 was significantly reduced compared to a control patient with thin basement membrane disease.
This study reports two novel OCRL variants associated with severe ocular and neurologic deficiency, despite only mild renal dysfunction. Based on our two patients and a literature review, the genotype-phenotype correlation of OCRL mutations with this severe phenotype of Lowe syndrome suggest a possible clustering of missense, deletion, and nonsense mutations in the 5-phosphatase domain and Rho-GAP domain in the Chinese population.
In this study, the hot water extraction process of crude polysaccharides from Morindae officinalis radix (cMORP) was conducted and optimized through a single-factor test and orthogonal experimental ...design. With the optimal extraction process (extraction temperature of 80 °C, extraction time of 2 h, liquid/solid ratio of 15 mL/g, and number of extraction of 1), the cMORP was obtained by the ethanol precipitation method. The chemical properties and preliminary characterization of the cMORP were analyzed by chemical or instrumental methods. Furthermore, to indicate a preliminary study on safety, a single oral dose of 5000 mg/kg body weight (BW) was administered orally to Kunming (KM) mice for acute toxicity, and the cMORP was administered orally to KM mice once a day at doses of 25, 50, and 100 mg/kg BW for 30 days. General behaviors, body weight variations, histopathology, relative organ weights, and hematological and serum biochemical parameters were observed and recorded. The results suggested there were no toxicologically significant changes. Based on the safety study, cMORP can be initially considered non-toxic with no acute oral toxicity up to 5000 mg/kg BW and safe at up to 100 mg/kg BW in KM mice for 30 days.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is relatively rare in children. This article aimed to analyze clinical and renal histology findings and different responses to ...induction treatment associated with the long-term renal outcomes in children with AAV in a single center.
All pediatric patients with AAV admitted to Tongji Hospital from January 2002 to January 2021 were included in the study. The demographic, clinical, pathological, laboratory, and treatment data and outcomes were collected and analyzed to identify predictors associated with response to induction treatment and progression to end-stage renal disease (ESRD).
In total, 48 children with AAV were included in this cohort; 81.25% of them were women, and 91.7% were microscopic polyangiitis (MPA). Kidney involvement was found in 45 patients (93.75%). The most common histopathological subtype was crescentic form in this cohort according to Berden's classification. In total, 34 patients (70.8%) showed eGFR <60 ml/min/1.73 m
at the time of diagnosis. Complete and partial remission was achieved in 8 patients (16.7%) and 19 patients (39.6%), respectively, following 6-month induction treatment. Half of the patients eventually progressed to ESRD at a mean time of (13.04 ± 15.83) months after diagnosis. The independent predictors of nonremission following induction treatment and progression to ESRD were baseline eGFR <60 ml/min/1.73 m
and hypertension at diagnosis. Renal survival significantly decreased over time in patients with renal sclerotic subtypes or those with nonremission following induction treatment by Kaplan-Meier curve estimation.
Our study demonstrates that women, MPA, and crescentic subtypes are predominant in pediatric AAV in China. Initial renal failure (eGFR <60 ml/min/1.73 m
), hypertension, sclerotic pathological subtype, and nonremission following induction treatment are predictive of long-term renal outcomes.
Grasslands, a vital ecosystem and component of the global carbon cycle, play a significant role in evaluating ecosystem health and monitoring the global carbon balance. In this study, based on the ...Carnegie–Ames–Stanford Approach (CASA) model, we estimated the Net Primary Productivity (NPP) of grasslands in northern Shaanxi from 2000 to 2020. Employing trend analysis, stability analysis, multiple regression analysis, and residual analysis, the research examined the dynamic changes of grassland NPP and its response to climatic and human factors. Key findings include: (1) Grassland NPP showed a significant increasing trend during 2000–2020, with high-coverage grasslands showing a higher rate of increase than medium and low-coverage grasslands. (2) Most grasslands (>90%) exhibited unstable growth and high NPP fluctuation. (3) While temperature, precipitation, and radiation undulate, the trends were not significant. Rainfall and radiation emerged as dominant factors affecting NPP, with temperature suppressing NPP increase to some extent. (4) Policies like returning farmland to grassland had a positive impact on grassland recovery, vegetation productivity, and regional ecosystem health.
Objectives
To explore whether multiparametric approach including blood oxygenation level-dependent MRI (BOLD-MRI) and intravoxel incoherent motion diffusion-weighted imaging (IVIM-DWI) can be applied ...in the assessment of renal function in children with chronic kidney disease (CKD).
Materials and methods
This prospective study included 74 children (CKD stage 1–3, 51; CKD stage 4–5, 12; healthy volunteers, 11) for renal MRI examinations including coronal T2WI, axial T1WI and T2WI, BOLD-MRI, and DWI sequences. We measured the renal cortex and medulla T2*, ADC,
D
t
,
D
p
, and
f
p
values on BOLD and DWI images. Appropriate statistical methods were applied for comparing MRI-derived parameters among the three groups and calculating the correlation coefficients between MRI-derived parameters and clinical data. Receiver operating characteristic (ROC) curves were used to assess the diagnostic performance of MRI-derived parameters.
Results
There were significant differences in cortex T2*, ADC,
D
t
,
f
p
and medulla T2*, ADC,
D
t
among the three groups. Cortex T2*, ADC,
D
t
,
f
p
and medulla T2*, ADC,
D
t
had a trend: CKD stage 4–5 < CKD stage 1–3 < healthy volunteers. Cortex and medulla T2*, ADC,
D
t
were significantly correlated with eGFR, serum creatinine (Scr), cystatin C. In addition, cortex T2* and eGFR showed the highest correlation coefficient (
r
= 0.824,
p
< 0.001). Cortex
D
t
and medulla T2* were optimal parameters for differentiating healthy volunteers and CKD stage 1–3 or CKD stage 4–5 and CKD stage 1–3, respectively.
Conclusions
BOLD-MRI and IVIM-DWI might be used as a feasible method for noninvasive assessment of renal function in children with CKD.
Background
Acquired von Willebrand syndrome (AVWS) is a less common bleeding disorder, primarily manifested as mild to moderate mucocutaneous bleeding and laboratory tests are similar to hereditary ...von Willebrand disease (VWD). AVWS is secondary to other diseases, and systemic lupus erythematosus (SLE) is a relatively rare cause.
Case presentation
We report a case of AVWS as onset clinical presentation of SLE manifested as epistaxis and pulmonary hemorrhage. A 13-year-old male child presented to the hospital with a six-month history of recurrent epistaxis and a one-month history of anemia. Routine blood tests demonstrated severe normocytic anemia and normal platelet count. Von Willebrand test revealed a significantly lower level. High-resolution chest computed tomography (CT) showed patchy ground glass opacities consistent with hemorrhagic changes. After ruling out the family history, the patient was diagnosed with AVWS. Additional tests confirmed positive antinuclear and anti-Sm antibodies. The underlying SLE was diagnosed and treated with methylprednisolone with disease recovery.
Conclusion
We recommend screening for bleeding disorders in patients with recurrent epistaxis. AVWS should be considered when laboratory findings suggest hereditary von Willebrand disease without a personal or familial history of bleeding. In addition, the underlying disease should be explored.
Minimally invasive peritoneal dialysis (PD) catheterization is increasingly common, and percutaneous PD catheters may be placed using a trocar or the Seldinger technique. There are few reports of ...pediatric percutaneous PD catheter insertion. We retrospectively compared the outcomes from percutaneous placement of Tenckhoff catheters using a modified Seldinger technique with catheter placement by open surgery. This single-center retrospective study compared 14 pediatric patients who received percutaneous PD catheter insertion using an ultrasound-guided modified Seldinger technique (August 2018–February 2021) with 10 patients who received open-surgical PD catheter insertion (2015–2018). Complications and catheter survival were evaluated. The overall technical success rate was 100%, but the Seldinger technique required less time (30 vs. 45 min) and smaller incisions (1.1 vs. 4.4 cm). The early complications in the Seldinger and control groups were bleeding (1 vs. 0), catheter dysfunction (1 vs. 1), abdominal pain (3 vs. 7), and exit leakage (0 vs. 1). In the Seldinger group, the median time from insertion to first use was 3 days, and the minimum follow-up was 6 months. Catheter survival at 6 months was 93% (Seldinger group) and 90% (open surgery group). The adoption of this technique at our institution led to a significant increase in the percentage of new pediatric dialysis patients commencing PD rather than hemodialysis. Collectively, the modified Seldinger technique described here was safe and feasible in pediatric patients. This approach is simpler and more rapid than open surgery, and reduces early complications and increases PD uptake.
For many practical high-dimensional problems, interactions have been increasingly found to play important roles beyond main effects. A representative example is gene-gene interaction. Joint analysis, ...which analyzes all interactions and main effects in a single model, can be seriously challenged by high dimensionality. For high-dimensional data analysis in general, marginal screening has been established as effective for reducing computational cost, increasing stability, and improving estimation/selection performance. Most of the existing marginal screening methods are designed for the analysis of main effects only. The existing screening methods for interaction analysis are often limited by making stringent model assumptions, lacking robustness, and/or requiring predictors to be continuous (and hence lacking flexibility). A unified marginal screening approach tailored to interaction analysis is developed, which can be applied to regression, classification, and survival analysis. Predictors are allowed to be continuous and discrete. The proposed approach is built on Coefficient of Variation (CV) filters based on information entropy. Statistical properties are rigorously established. It is shown that the CV filters are almost insensitive to the distribution tails of predictors, correlation structure among predictors, and sparsity level of signals. An efficient two-stage algorithm is developed to make the proposed approach scalable to ultrahigh-dimensional data. Simulations and the analysis of TCGA LUAD data further establish the practical superiority of the proposed approach.
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