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1.
J Agric Food Chem ; 2024 May 14.
Artículo en Inglés | MEDLINE | ID: mdl-38743450

RESUMEN

Allergenicity of soybean 7S protein (7S) troubles many people around the world. However, many processing methods for lowering allergenicity is invalid. Interaction of 7S with phenolic acids, such as chlorogenic acid (CHA), to structurally modify 7S may lower the allergenicity. Hence, the effects of covalent (C-I, periodate oxidation method) and noncovalent interactions (NC-I) of 7S with CHA in different concentrations (0.3, 0.5, and 1.0 mM) on lowering 7S allergenicity were investigated in this study. The results demonstrated that C-I led to higher binding efficiency (C-0.3:28.51 ± 2.13%) than NC-I (N-0.3:22.66 ± 1.75%). The C-I decreased the α-helix content (C-1:21.06%), while the NC-I increased the random coil content (N-1:24.39%). The covalent 7S-CHA complexes of different concentrations had lower IgE binding capacity (C-0.3:37.38 ± 0.61; C-0.5:34.89 ± 0.80; C-1:35.69 ± 0.61%) compared with that of natural 7S (100%), while the noncovalent 7S-CHA complexes showed concentration-dependent inhibition of IgE binding capacity (N-0.3:57.89 ± 1.23; N-0.5:46.91 ± 1.57; N-1:40.79 ± 0.22%). Both interactions produced binding to known linear epitopes. This study provides the theoretical basis for the CHA application in soybean products to lower soybean allergenicity.

2.
iScience ; 27(5): 109616, 2024 May 17.
Artículo en Inglés | MEDLINE | ID: mdl-38706845

RESUMEN

Among various electrocatalysts, high-entropy alloys (HEAs) have gained significant attention for their unique properties and excellent catalytic activity in the hydrogen evolution reaction (HER). However, the precise synthesis of HEA catalysts in small sizes remains challenging, which limits further improvement in their catalytic performance. In this study, boron- and nitrogen-doped HEA porous carbon nanofibers (HE-BN/PCNF) with an in situ-grown dendritic structure were successfully prepared, inspired by the germination and growth of tree branches. Furthermore, the dendritic fibers constrained the growth of HEA particles, leading to the synthesis of quantum dot-sized (1.67 nm) HEA particles, which also provide a pathway for designing HEA quantum dots in the future. This work provides design ideas and guiding suggestions for the preparation of borated HEA fibers with different elemental combinations and for the application of dendritic nanofibers in various fields.

3.
Arch Pathol Lab Med ; 2024 May 16.
Artículo en Inglés | MEDLINE | ID: mdl-38749502

RESUMEN

CONTEXT.­: Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm that predominantly affects young children. OBJECTIVE.­: To investigate genetic alterations and their correlation with clinical characteristics and prognosis in pediatric LCH. DESIGN.­: We performed targeted sequencing to detect mutations in LCH lesions from pediatric patients. RESULTS.­: A total of 30 genomic alterations in 5 genes of the MAPK pathway were identified in 187 of 223 patients (83.9%). BRAF V600E (B-Raf proto-oncogene, serine/threonine kinase) was the most common mutation (51.6%), followed by MAP2K1 (mitogen-activated protein kinase kinase 1) alterations (17.0%) and other BRAF mutations (13.0%). ARAF (A-Raf proto-oncogene, serine/threonine kinase) and KRAS (KRAS proto-oncogene, GTPase) mutations were relatively rare (2.2% and 0.9%, respectively). Additionally, FNBP1 (formin-binding protein 1)::BRAF fusion and MAP3K10 (mitogen-activated protein kinase kinase 10) mutations A17T and R823C were identified in 1 case each, with possible constitutive activation of ERK1/2 phosphorylation. BRAF V600E was more frequent in patients with risk organ involvement, while MAP2K1 mutation was more prevalent in patients with single-system LCH (P = .001). BRAF V600E was associated with craniofacial bone, skin, liver, spleen, and ear involvement (all P < .05). Patients with other BRAF mutations had a higher proportion of spinal column involvement (P = .006). Univariate analysis showed a significant difference in progression-free survival among the 4 molecular subgroups for patients treated with first-line therapy (P = .02). According to multivariate analysis, risk organ involvement was the strongest independent adverse prognostic factor (hazard ratio, 8.854; P < .001); BRAF or MAP2K1 mutation was not an independent prognostic factor. CONCLUSIONS.­: Most pediatric patients with LCH carry somatic mutations involving the MAPK pathway, correlating with clinical characteristics and outcomes for first-line chemotherapy.

4.
J Colloid Interface Sci ; 666: 585-593, 2024 Jul 15.
Artículo en Inglés | MEDLINE | ID: mdl-38613980

RESUMEN

Capacitive deionization (CDI) is flourishing as an energy-efficient and cost-effective water desalination method. However, challenges such as electrode degradation and fouling have hindered the practical deployment of CDI technology. To address these challenges, the key point of our strategy is applying a hydrophilic coating composed of polyethylene glycol (PEG)-functionalized nano-TiO2/polyvinylidene fluoride (PVDF) to the electrode interface (labeled as APPT electrode). The PEG/PVDF/TiO2 layer not only mitigates the co-ion depletion, but also imparts the activated carbon (AC) electrode hydrophilicity. As anticipated, the APPT electrode possessed an enhanced desalination capacity of 83.54 µmol g-1 and a low energy consumption of 17.99 Wh m-3 in 10 mM sodium chloride solution compared with the bare AC electrode. Notably, the APPT maintained about 93.19 % of its desalination capacity after 50 consecutive adsorption-desorption cycles in the presence of bovine serum albumin (BSA). During the trial, moreover, no obvious overall performance decline was noted in concentration reduction (Δc), water recovery (WR) and productivity (P) over 50 cycles. This strategy realizes energy-efficient, antifouling and stable brackish water desalination and has great promise for practical applications.

5.
J Colloid Interface Sci ; 667: 192-198, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-38636221

RESUMEN

Designing and developing cost-effective, high-performance catalysts for hydrogen evolution reaction (HER) is crucial for advancing hydrogen production technology. Tungsten-based sulfides (WSx) exhibit great potential as efficient HER catalysts, however, the activity is limited by the larger energy required for water dissociation under alkaline conditions. Herein, we adopt a top-down strategy to construct heterostructure Co-WS2 nanofiber catalysts. The experimental results and theoretical simulations unveil that the work functions-induced built-in electric field at the interface of Co-WS2 catalysts facilitates the electron transfer from Co to WS2, significantly reducing water dissociation energy and optimizing the Gibbs free energy of the entire reaction step for HER. Besides, the self-supported catalysts of Co-WS2 nanoparticles confining 1D nanofibers exhibit an increased number of active sites. As expected, the heterostructure Co-WS2 catalysts exhibit remarkable HER activity with an overpotential of 113 mV to reach 10 mA cm-2 and stability with 30 h catalyzing at 23 mA cm-2. This work can provide an avenue for designing highly efficient catalysts applicable to the field of energy storage and conversion.

6.
Nanomaterials (Basel) ; 14(4)2024 Feb 15.
Artículo en Inglés | MEDLINE | ID: mdl-38392734

RESUMEN

Herein, the preparation process, morphology, structure, and magnetic properties of La1.85Sr0.15CuO4 (LSCO) cobweb-like nanofibers are reported. LSCO nanofibers with a regular grain size distribution are successfully prepared via electrospinning, followed by calcination. We conducted morphology analysis and elemental distribution using electron microscopy and energy-dispersive X-ray spectroscopy (EDS), respectively. Additionally, magnetic property testing was performed using a vibrating sample magnetometer (VSM) to confirm the superconducting properties of the samples. Interestingly, our samples exhibited a superconducting transition temperature, Tc, of 25.21 K, which showed some disparity compared to similar works. Furthermore, we observed a ferromagnetic response at low temperatures in the superconducting nanofibers. We attribute these phenomena to the effects generated by surface states of nanoscale superconducting materials.

7.
Small ; 20(13): e2307291, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-37964162

RESUMEN

Normally, only noncentrosymmetric structure of the materials can potentially be piezoelectric. Thus, it is limited in the field of piezoelectricity for the centrosymmetric structure of the material. In this work, the performance of piezoelectricity is successfully achieved from centrosymmetric SrFeO3- x by modulating oxygen vacancies, which have a surface piezoelectric potential up to 93 mV by using Kelvin-probe force microscopy (KPFM). Moreover, the piezoelectric effects of SrFeO3- x are also evaluated by piezoelectric catalytic effect and density functional theory calculations (DFT). The results show that the piezo-catalytic degradation of tetracycline reaches 96% after 75 min by ultrasonic mechanical vibration and the production of H2O2 by SrFeO3- x piezoelectric synthesis could reach 1821 µmol L-1. In addition, the DFT results indicate that the intrinsic effect of oxygen vacancies effectively promotes the adsorption and activation of O2 and H2O as well as intermediates and improves the piezoelectric catalytic activity. This work provides an effective basis for realizing the piezoelectricity of centrosymmetric materials and regulating the development of piezoelectric catalytic properties.

8.
Chinese Journal of Pediatrics ; (12): 60-65, 2024.
Artículo en Chino | WPRIM (Pacífico Occidental) | ID: wpr-1013250

RESUMEN

Objective: To analyze the clinical features,treatment and prognosis of drug induced hypersensitivity syndrome related hemophagocytic lymphohistiocytosis (DIHS-HLH). Methods: This was a retrospective case study. Clinical characteristics, laboratory results, treatment and prognosis of 9 patients diagnosed with DIHS-HLH in Beijing Children's hospital between January 2020 and December 2022 were summarized. Kaplan-Meier survival analysis was used to calculate the overall survival rate. Results: Among all 9 cases, there were 6 males and 3 females, with the age ranged from 0.8 to 3.1 years. All patients had fever, rash, hepatomegaly and multiple lymph node enlargement. Other manifestations included splenomegaly (4 cases), pulmonary imaging abnormalities (6 cases), central nervous system symptoms (3 cases), and watery diarrhea (3 cases). Most patients showed high levels of soluble-CD25 (8 cases), hepatic dysfunction (7 cases) and hyperferritinemia (7 cases). Other laboratory abnormalities included hemophagocytosis in bone marrow (5 cases), hypofibrinogenemia (3 cases) and hypertriglyceridemia (2 cases). Ascending levels of interleukin (IL) 5, IL-8 and interferon-γ (IFN-γ) were detected in more than 6 patients. All patients received high dose intravenous immunoglobulin, corticosteroid and ruxolitinib, among which 4 patients were also treated with high dose methylprednisolone, 2 patients with etoposide and 2 patients with cyclosporin A. After following up for 0.2-38.6 months, 7 patients survived, and the 1-year overall survival rate was (78±14)%. Two patients who had no response to high dose immunoglobulin, methylprednisolone 2 mg/(kg·d) and ruxolitinib died. Watery diarrhea, increased levels of IL-5 and IL-8 and decreased IgM were more frequently in patients who did not survive. Conclusions: For children with fever, rash and a suspicious medication history, when complicated with hepatomegaly, impaired liver function and high levels of IL-5 and IL-8, DIHS-HLH should be considered. Once diagnosed with DIHS-HLH, suspicious drugs should be stopped immediately, and high dose intravenous immunoglobulin, corticosteroid and ruxolitinib could be used to control disease.


Asunto(s)
Niño , Masculino , Femenino , Humanos , Lactante , Preescolar , Linfohistiocitosis Hemofagocítica/complicaciones , Estudios Retrospectivos , Interleucina-5 , Hepatomegalia/complicaciones , Inmunoglobulinas Intravenosas/efectos adversos , Interleucina-8 , Metilprednisolona , Corticoesteroides , Diarrea/complicaciones , Exantema/complicaciones
9.
Int J Biol Macromol ; 257(Pt 2): 128698, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-38103664

RESUMEN

In order to fabricate a novel antioxidant nanofiber facial mask, a metal cone modified in-situ electrospinning with precise deposition was employed by utilizing Enteromorpha prolifera polysaccharides (EPPs). The metal cone could control the deposition area to achieve precise fabrication of facial mask on skin. The EPPs exhibited remarkable antioxidant ability, as evidenced by the half-maximal inhibitory concentrations (IC50) of 1.44 mg/mL and 0.74 mg/mL against DPPH and HO• free radicals, respectively. The antioxidant ability of the facial mask was improved by elevating the electrospinning voltage from 15 kV to 19 kV, due to the improved release capacity of EPPs by 7.09 %. Moreover, the facial mask demonstrated robust skin adhesion and moisture-retaining properties compared with commercial facial mask, which was benefited by the in-situ electrospinning technology. Furthermore, cytotoxicity assay, animal skin irritation test, and ocular irritation test collectively affirmed the safety of the facial mask. Thus, this research introduces a novel in situ electrospinning with precise deposition method and a natural antioxidant additive for preparing facial mask.


Asunto(s)
Algas Comestibles , Nanofibras , Ulva , Animales , Antioxidantes/farmacología , Ulva/química , Polisacáridos/farmacología , Polisacáridos/química
10.
Small ; : e2307810, 2023 Dec 05.
Artículo en Inglés | MEDLINE | ID: mdl-38050940

RESUMEN

The technical synergy between flexible sensing paper and triboelectric nanogenerator (TENG) in the next stage of artificial intelligence Internet of Things engineering makes the development of intelligent sensing paper with triboelectric function very attractive. Therefore, it is extremely urgent to explore functional papers that are more suitable for triboelectric sensing. Here, a cellulose nanocrystals (CNCs) reinforced PVDF hybrid paper (CPHP) is developed by electrospinning technology. Benefitting from the unique effects of CNCs, CPHP forms a solid cross-linked network among fibers and obtains a high-strength (25 MPa) paper-like state and high surface roughness. Meanwhile, CNCs also improve the triboelectrification effect of CPHP by assisting the PVDF matrix to form more electroactive phases (96% share) and a higher relative permittivity (17.9). The CPHP-based TENG with single electrode configuration demonstrates good output performance (open-circuit voltage of 116 V, short-circuit current of 2.2 µA and power density of 91 mW m-2 ) and ultrahigh pressure-sensitivity response (3.95 mV Pa-1 ), which endows CPHP with reliable power supply and sensing capability. More importantly, the CPHP-based flexible self-powered tactile sensor with TENG array exhibits multifunctional applications in imitation Morse code compilation, tactile track recognition, and game character control, showing great prospects in the intelligent inductive device and human-machine interaction.

11.
Nat Commun ; 14(1): 7549, 2023 Nov 20.
Artículo en Inglés | MEDLINE | ID: mdl-37985662

RESUMEN

Carbon-defect engineering in metal single-atom catalysts by simple and robust strategy, boosting their catalytic activity, and revealing the carbon defect-catalytic activity relationship are meaningful but challenging. Herein, we report a facile self-carbon-thermal-reduction strategy for carbon-defect engineering of single Fe-N4 sites in ZnO-Carbon nano-reactor, as efficient catalyst in Fenton-like reaction for degradation of phenol. The carbon vacancies are easily constructed adjacent to single Fe-N4 sites during synthesis, facilitating the formation of C-O bonding and lowering the energy barrier of rate-determining-step during degradation of phenol. Consequently, the catalyst Fe-NCv-900 with carbon vacancies exhibits a much improved activity than the Fe-NC-900 without abundant carbon vacancies, with 13.5 times improvement in the first-order rate constant of phenol degradation. The Fe-NCv-900 shows high activity (97% removal ratio of phenol in only 5 min), good recyclability and the wide-ranging pH universality (pH range 3-9). This work not only provides a rational strategy for improving the Fenton-like activity of metal single-atom catalysts, but also deepens the fundamental understanding on how periphery carbon environment affects the property and performance of metal-N4 sites.

12.
Int J Ophthalmol ; 16(9): 1441-1449, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37724268

RESUMEN

AIM: To investigate the impact of 17ß-estradiol on the collagen gels contraction (CGC) and inflammation induced by transforming growth factor (TGF)-ß in human Tenon fibroblasts (HTFs). METHODS: HTFs were three-dimensionally cultivated in type I collagen-generated gels with or without TGF-ß (5 ng/mL), 17ß-estradiol (12.5 to 100 µmol/L), or progesterone (12.5 to 100 µmol/L). Then, the collagen gel diameter was determined to assess the contraction, and the development of stress fibers was analyzed using immunofluorescence staining. Immunoblot and gelatin zymography assays were used to analyze matrix metalloproteinases (MMPs) and tissue inhibitors of metalloproteinases (TIMPs) being released into culture supernatants. Enzyme-linked immunosorbent assay (ELISA) and reverse transcription-quantitative polymerase chain reaction (RT-PCR) were used to detect interleukin (IL)-6, monocyte chemoattractant proteins (MCP)-1, and vascular endothelial growth factor (VEGF) in HTFs at the translational and transcriptional levels. The phosphorylation levels of Sma- and Mad-related proteins (Smads), mitogen-activated protein kinases (MAPKs), and protein kinase B (AKT) were measured by immunoblotting. Statistical analysis was performed using either the Tukey-Kramer test or Student's unpaired t-test to compare the various treatments. RESULTS: The CGC caused by TGF-ß in HTFs was significantly inhibited by 17ß-estradiol (25 to 100 µmol/L), and a statistically significant difference was observed when comparing the normal control group with 17ß-estradiol concentrations exceeding 25 µmol/L (P<0.05). The suppressive impact of 17ß-estradiol became evident 24h after administration and peaked at 72h (P<0.05), whereas progesterone had no impact. Moreover, 17ß-estradiol attenuated the formation of stress fibers, and the production of MMP-3 and MMP-1 in HTFs stimulated by TGF-ß. The expression of MCP-1, IL-6, and VEGF mRNA and protein in HTFs were suppressed by 100 µmol/L 17ß-estradiol (P<0.01). Additionally, the phosphorylation of Smad2 Smad3, p38, and extracellular signal-regulated kinase (ERK) were downregulated (P <0.01). CONCLUSION: 17ß-estradiol significantly inhibits the CGC and inflammation caused by TGF-ß in HTFs. This inhibition is likely related to the suppression of stress fibers, inhibition of MMPs, and attenuation of Smads and MAPK (ERK and p38) signaling. 17ß-estradiol may have potential clinical benefits in preventing scar development and inflammation in the conjunctiva.

13.
Small ; 19(49): e2304086, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-37612815

RESUMEN

Space charge transfer of heterostructures driven by the work-function-induced built-in field can regulate the electronic structure of catalysts and boost the catalytic activity. Herein, an epitaxial heterojunction catalyst of CoO/Mo2 C with interfacial electron redistribution induced by work functions (WFs) is constructed for overall water splitting via a novel top-down strategy. Theoretical simulations and experimental results unveil that the WFs-induced built-in field facilitates the electron transfer from CoO to Mo2 C through the formed "Co─C─Mo" bond at the interface of CoO/Mo2 C, achieving interfacial electron redistribution, further optimizing the Gibbs free energy of primitive reaction step and then accelerating kinetics of hydrogen evolution reaction (HER). As expected, the CoO/Mo2 C with interfacial effects exhibits excellent HER catalytic activity with only needing the overpotential of 107 mV to achieve 10 mA cm-2 and stability for a 60-h continuous catalyzing. Besides, the assembled CoO/Mo2 C behaves the outstanding performance toward overall water splitting (1.58 V for 10 mA cm-2 ). This work provides a novel possibility of designing materials based on interfacial effects arising from the built-in field for application in other fields.

14.
World Neurosurg ; 178: e777-e790, 2023 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-37562682

RESUMEN

OBJECTIVE: The anterior clinoid process (ACP) is surrounded by nerves and vessels that, together, constitute an intricate anatomical structure with variations that challenges the performance of individualized anterior clinoidectomy in treating lesions with different extents of invasion. In the present study, we established a 6-surface system for the ACP based on anatomical landmarks and analyzed its value in guiding ACP drilling and resection of paraclinoid meningiomas. METHODS: Using the anatomical characteristics of 10 dry skull specimens, we set 9 anatomical landmarks to delineate the ACP into 6 surfaces. Guided by our 6-surface system and eggshell technique, 5 colored silicone-injected anatomical specimens were dissected via a frontotemporal craniotomy to perform anterior clinoidectomy. Next, 3 typical cases of paraclinoid meningioma were selected to determine the value of using our 6-surface system in tumor resection. RESULTS: Nine points (A-H and T) were proposed to delineate the ACP surface into frontal, temporal, optic nerve, internal carotid artery, cranial nerve III, and optic strut surfaces according to the adjacent tissues. Either intradurally or extradurally, the frontal and temporal surfaces could be identified and drilled into depth, followed by skeletonization of the optic nerve, cranial nerve III, internal carotid artery, and optic strut surfaces. After the residual bone was removed, the ACP was drilled off. In surgery of paraclinoid meningiomas, our 6-surface system provided great benefit in locating the dura, nerves, and vessels, thus, increasing the safety of opening the optic canal and relaxing the oculomotor or optic nerves and allowing for individualized ACP drilling for meningioma removal. CONCLUSIONS: Our 6-surface system adds much anatomical information to the classic Dolenc triangle and can help neurosurgeons, especially junior ones, to increase their understanding of the paraclinoid spatial structure and accomplish individualized surgical procedures with high safety and minimal invasiveness.


Asunto(s)
Aneurisma Intracraneal , Neoplasias Meníngeas , Meningioma , Humanos , Meningioma/diagnóstico por imagen , Meningioma/cirugía , Aneurisma Intracraneal/cirugía , Base del Cráneo/cirugía , Hueso Esfenoides/cirugía , Hueso Esfenoides/anatomía & histología , Neoplasias Meníngeas/diagnóstico por imagen , Neoplasias Meníngeas/cirugía
15.
J Colloid Interface Sci ; 648: 963-971, 2023 Oct 15.
Artículo en Inglés | MEDLINE | ID: mdl-37331077

RESUMEN

The photo-stimulus response has the advantage of non-invasiveness, which could be used to control the "on" and "off" of drug release achieving on-demand release. Herein, we design a heating electrospray during electrospinning to prepare photo-stimulus response composite nanofibers consisting of MXene@Hydrogel. This heating electrospray enables to spray MXene@Hydrogel during the electrospinning process, and the hydrogel is uniformly distributed which cannot be achieved by the traditional soaking method. In addition, this heating electrospray can also overcome the difficulty that hydrogels are hard to be uniformly distributed in the inner fiber membrane.The "on" and "off" state of drug release could be controlled by light. Not only near infrared (NIR) light but also sunlight could trigger the drug release, which could benefit outdoor use when cannot find NIR light. Evidence by hydrogen bond has been formed between MXene and Hydrogel, the mechanical property of MXene@Hydrogel composite nanofibers is significantly enhanced, which is conducive to the application of human joints and other parts that need to move. These nanofibers also possess fluorescence property, which is further used to real-time monitor the in-vivo drug release. No matter the fast or slow release, this nanofiber can achieve sensitive detection, which is superior to the current absorbance spectrum method.


Asunto(s)
Hidrogeles , Nanofibras , Humanos , Hidrogeles/química , Nanofibras/química , Liberación de Fármacos
16.
Langmuir ; 39(19): 6885-6894, 2023 May 16.
Artículo en Inglés | MEDLINE | ID: mdl-37129447

RESUMEN

Traditional SrTiO3 (STO) materials have high brittleness and poor deformation resistance. In this work, macroscopically flexible iron-doped SrTiO3 (SFTO) nanofibrous membranes were prepared by electrospinning and calcination, which can be easily isolated and can maintain integrity to recycle as photocatalysts. Moreover, the SFTO nanofibrous membranes showed enhanced photocatalytic performance under strong acids (pH = 2) and strong alkalis (pH = 12). The SFTO nanofibrous membranes increased the catalytic rate of Congo red (CR) dye by about 10 times in visible light. The mechanism of photocatalytic activity enhancement was discussed by the combined effects of hydroxyl radicals and superoxide radicals. The successful preparation of SFTO nanofibrous membranes has offered a simple and economical approach to photocatalysis as well as environmental remediation.

17.
Br J Haematol ; 202(1): 135-146, 2023 07.
Artículo en Inglés | MEDLINE | ID: mdl-37062931

RESUMEN

Frequent germline mutations of HAVCR2, recently identified in subcutaneous panniculitis-like T-cell lymphoma (SPTCL), are associated with an increased risk of hemophagocytic lymphohistiocytosis (HLH). However, SPTCL-HLH represents a challenge because of the difficulties in treatment with poor survival. Its malignant nature, specifically harbouring HAVCR2 mutations, has also been questioned. To better understand its pathology and treatment, we analysed the clinical data of six patients diagnosed at our centre. The median age at onset was 10.5 years (range, 0.8-12.4). Five patients presented with skin lesions of subcutaneous nodules/plaques and/or ulceration. All patients developed HLH; notably, one infant only had HLH without skin involvement. Histopathologically, only two patients were diagnosed with SPTCL and three were reported as panniculitis with no sufficient evidence of lymphoma. Genetically, germline homozygous mutation of HAVCR2 (p.Y82C) was identified in all patients, with a median diagnosis time of 4.6 months. All patients initially received corticosteroids, immunosuppressants or chemotherapy, achieving unfavourable responses. Strikingly, they responded well to ruxolitinib targeting inflammatory cytokines, allowing rapid disease resolution and/or long-term maintenance of remission. The excellent efficacy of ruxolitinib highlights this disease as an inflammatory condition instead of neoplastic nature and indicates novel agents targeting key inflammatory pathways as an encouraging approach for this disease entity.


Asunto(s)
Linfohistiocitosis Hemofagocítica , Paniculitis , Niño , Preescolar , Humanos , Lactante , Mutación de Línea Germinal , Receptor 2 Celular del Virus de la Hepatitis A/genética , Linfohistiocitosis Hemofagocítica/tratamiento farmacológico , Linfohistiocitosis Hemofagocítica/genética , Linfohistiocitosis Hemofagocítica/complicaciones , Paniculitis/tratamiento farmacológico , Paniculitis/genética
18.
RSC Adv ; 13(17): 11697-11705, 2023 Apr 11.
Artículo en Inglés | MEDLINE | ID: mdl-37063728

RESUMEN

The corrosion of materials severely limits the application scenarios of triboelectric nanogenerators (TENGs), especially in laboratories, chemical plants and other fields where leakage of chemically corrosive solutions is common. Here, we demonstrate a chemical-resistant triboelectric nanogenerator (CR-TENG) based on polysulfonamide (PSA) and polytetrafluoroethylene (PTFE) non-woven fabrics. The CR-TENG can stably harvest biological motion energy and perform intelligent safety protection monitoring in a strong corrosive environment. After treatment with strong acid and alkali solution for 7 days, the fabric morphology, diameter, tensile properties and output of CR-TENG are not affected, showing high reliability. CR-TENG integrated into protective equipment can detect the working status of protective equipment in real time, monitor whether it is damaged, and provide protection for wearers working in high-risk situations. In addition, the nonwoven-based CR-TENG has better wearing comfort and is promising for self-powered sensing in harsh environments.

19.
J Hazard Mater ; 445: 130594, 2023 Mar 05.
Artículo en Inglés | MEDLINE | ID: mdl-37055951

RESUMEN

Rapid cleanup of highly-viscous oil spills the sea is eagerly desired while still remains a great challenge. Hydrophobic and lipophilic adsorbents are regarded as ideal candidate for oil spill remediation. However, traditional adsorbents are not suitable for viscous crude oil, which would block the porous structure and lead to poor adsorption efficiency. In this work, a non-contact responsive superhydrophobic SiO2 aerogel blankets (SAB) with excellent magnetic and solar heating effect for efficient removal of viscosity oils under harsh environments was developed, via assembled MXene and Fe3O4/polydimethylsiloxane layer-by-layer along the SAB skeleton (Fe3O4/MXene@SAB). The Fe3O4/MXene@SAB exhibited excellent compression tolerance (compression stress 70.69 kPa), superhydrophobic performance (water contact angle 166°), and corrosion resistance (weak acid/strong base). Due to high water repellency and stable porous structure, the Fe3O4/MXene@SAB could successfully separate oil-water mixture, while with remarkable separation flux (1.50-3.19 × 104 L m-2 h-1), and separation efficiency (99.91-99.98 %). Furthermore, the responsive Fe3O4/MXene@SAB also showed outstanding magnetic-heating and solar-heating conversion efficiency, which could continuously separate high viscosity crude oil from seawater by pump even under relatively low magnetic fields and mild sun. The superhydrophobic blankets hold great promise for efficient treatment of heavy oil spills.

20.
J Clin Invest ; 133(11)2023 06 01.
Artículo en Inglés | MEDLINE | ID: mdl-37053010

RESUMEN

Germline or somatic loss-of-function mutations of fumarate hydratase (FH) predispose patients to an aggressive form of renal cell carcinoma (RCC). Since other than tumor resection there is no effective therapy for metastatic FH-deficient RCC, an accurate method for early diagnosis is needed. Although MRI or CT scans are offered, they cannot differentiate FH-deficient tumors from other RCCs. Therefore, finding noninvasive plasma biomarkers suitable for rapid diagnosis, screening, and surveillance would improve clinical outcomes. Taking advantage of the robust metabolic rewiring that occurs in FH-deficient cells, we performed plasma metabolomics analysis and identified 2 tumor-derived metabolites, succinyl-adenosine and succinic-cysteine, as excellent plasma biomarkers for early diagnosis. These 2 molecules reliably reflected the FH mutation status and tumor mass. We further identified the enzymatic cooperativity by which these biomarkers are produced within the tumor microenvironment. Longitudinal monitoring of patients demonstrated that these circulating biomarkers can be used for reporting on treatment efficacy and identifying recurrent or metastatic tumors.


Asunto(s)
Carcinoma de Células Renales , Neoplasias Renales , Humanos , Carcinoma de Células Renales/patología , Neoplasias Renales/patología , Fumarato Hidratasa/genética , Fumarato Hidratasa/metabolismo , Ácido Succínico , Mutación , Microambiente Tumoral
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