Objective:To investigate the clinical features and prognosis of neuro-Beh?et′s syndrome (NBS) in children.Method:The clinical, brain magnetic resonance imaging and laboratory data of 5 children with NBS diagnosed in the Department of Pediatrics, General Hospital of Ningxia Medical University and Department of Rheumatology and Immunology, Children′s Hospital Affiliated to Capital Institute of Pediatrics from April 2014 to April 2024 were analyzed retrospectively. The follow-up method was retrospective outpatient or inpatient visit to evaluate the treatment effect of NBS.Result:Among the 5 NBS cases, 2 were male and 3 were female. The age of admission ranged from 8 to 17 years, the time from onset to diagnosis was 2 days to 4 years. Two patients had dizziness, headache and convulsions during the treatment of NBS, 1 patient had disturbance of consciousness, 1 patient gradually developed aphasia, limb movement disorder, dysphagia and muscle weakness after 4 years of Behcet's syndrome, and 1 patient had no clinical symptoms. C-reactive protein and erythrocyte sedimentation rate were increased in 4 cases, and cerebrospinal fluid white blood cells and immunoglobulin G were increased in 1 case. Brain magnetic resonance imaging of 4 children showed multiple lesions, including bilateral frontal lobe, occipital lobe, parietal lobe, periventricular and corpus callosum lesions. Brain magnetic resonance imaging showed multiple demyelinating diseases in 1 case, and cervical and thoracic magnetic resonance imaging showed slender cervical and thoracic spinal cord. All patients were treated with corticosteroids combined with immunosuppressants or biological agents. The children were followed up for 6 months to 4 years, and 4 cases had good treatment results, and 1 case finally gave up treatment.Conclusions:The clinical manifestations of NBS are not specific, and brain magnetic resonance imaging shows that the lesion location and morphology are not specific. NBS children treated with corticosteroids combined with immunosuppressive agents or biological agents have a good prognosis.

OBJECTIVE To provide references for further improving the management level of Chinese herbal pieces covered by the healthcare security drug catalog （hereinafter referred to as “healthcare security covered Chinese herbal pieces”）. METHODS Policy documents related to healthcare security covered Chinese herbal pieces were retrieved from official websites of National Healthcare Security Administration and medical insurance bureaus of various provinces （autonomous regions， municipalities directly under the central government）. Using text analysis， the current management status of healthcare security covered Chinese herbal pieces in various provinces was analyzed from two dimensions： management regulations and catalog formats. RESULTS In terms of the management regulations for healthcare security covered Chinese herbal pieces， some provinces implemented both national and provincial standards for Chinese herbal pieces， while a small number of provinces adopted national standards alongside their own provincial standards. Regarding the payment categories of healthcare security covered Chinese herbal pieces， most were classified as category A， though some provinces managed them as category B， and others included both categories A and B or category C. In terms of applicants， there were three kinds： only medical institutions， only herbal piece enterprises， or both medical institutions and herbal piece enterprises being allowed to apply. During the application process， some provinces required preliminary evaluations by local healthcare security departments with recommended lists submitted， while some provinces omitted preliminary evaluations and relied solely on unified evaluation by provincial healthcare security departments. Regarding the methods for catalog formulation and adjustment， there was little variation among the provinces. In terms of the catalog format for healthcare security covered Chinese herbal pieces， some provinces had supplemented the catalog fields based on the national medical insurance catalog. In contrast， some provinces had relatively simple catalogs. Regarding the sorting of the herbal pieces catalog， some catalogs were arranged by the number of Chinese character strokes in the names of the herbal pieces， but most catalogs lacked clear sorting rules. CONCLUSIONS Significant resultshave been achieved in formulating management measures and the catalog of healthcare security covered Chinese herbal pieces. However， there is still a great necessity to develop targeted and adaptable management clauses based on local conditions and the characteristics of Chinese herbal pieces. The content and format of healthcare security drug catalog of Chinese herbal pieces should be improved； in addition， continuous tracking of the latest management policies is also essential to improve the accuracy and operability of formulated catalog.

The application of small molecule targeted drugs for lung cancer has significantly improved the survival of lung cancer patients. However, these drugs have a wide variety of types, fast development and market launch of new drugs, complex adverse reactions, and are mostly used at home, which increases the risk of irrational drug use. At the same time, insufficient monitoring of efficacy and safety is also prone to occur, ultimately affecting treatment outcomes. This consensus focuses on 43 small molecule targeted drugs or combinations for lung cancer, providing standardized recommendations for rational drug use and monitoring of efficacy/adverse reactions in clinical practice. The recommendations are regarding drug selection, dosage adjustment, efficacy monitoring, adverse reaction monitoring, and improvement of patient compliance. This consensus aims to improve the rational use and efficacy/safety monitoring quality of small molecule targeted drugs for lung cancer, ensure the effectiveness and safety of drug treatment, prolong the survival of lung cancer patients and improve their quality of life.
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Humans ; Lung Neoplasms/drug therapy* ; Antineoplastic Agents/adverse effects* ; Consensus ; Molecular Targeted Therapy ; Drug Monitoring ; Small Molecule Libraries/therapeutic use*

This study investigates the pharmacokinetics and metabolic characteristics of three marine-derived piericidins as potential drug leads for kidney disease: piericidin A (PA) and its two glycosides (GPAs), glucopiericidin A (GPA) and 13-hydroxyglucopiericidin A (13-OH-GPA). The research aims to facilitate lead selection and optimization for developing a viable preclinical candidate. Rapid absorption of PA and GPAs in mice was observed, characterized by short half-lives and low bioavailability. Glycosides and hydroxyl groups significantly enhanced the absorption rate (13-OH-GPA > GPA > PA). PA and GPAs exhibited metabolic instability in liver microsomes due to Cytochrome P450 enzymes (CYPs) and uridine diphosphoglucuronosyl transferases (UGTs). Glucuronidation emerged as the primary metabolic pathway, with UGT1A7, UGT1A8, UGT1A9, and UGT1A10 demonstrating high elimination rates (30%-70%) for PA and GPAs. This rapid glucuronidation may contribute to the low bioavailability of GPAs. Despite its low bioavailability (2.69%), 13-OH-GPA showed higher kidney distribution (19.8%) compared to PA (10.0%) and GPA (7.3%), suggesting enhanced biological efficacy in kidney diseases. Modifying the C-13 hydroxyl group appears to be a promising approach to improve bioavailability. In conclusion, this study provides valuable metabolic insights for the development and optimization of marine-derived piericidins as potential drug leads for kidney disease.
Animals ; Male ; Mice ; Aquatic Organisms/chemistry* ; Biological Availability ; Cytochrome P-450 Enzyme System/metabolism* ; Glucuronosyltransferase/metabolism* ; Microsomes, Liver/metabolism* ; Molecular Structure ; Biological Products/pharmacokinetics* ; Pyridines/pharmacokinetics*

This paper summarized the experience of Professor FANG Dingya in staged treatment of Sjögren's syndrome from the perspective of dryness toxin. It is believed that the cause of Sjögren's syndrome is externally-contracted dryness， consumption of essence and fluid， congenital and acquired essence deficiency， depleted essence and insufficient blood， and the core mechanism is internal accumulation of dryness toxin. The treatment can be divided into three stages， that is dryness toxin transforming into fire-heat， damp-heat and phlegm-stasis， from the perspective of dryness metal qi transformation. It is emphasized to dispel pathogen mainly， to clear and moisten with yin-nourishing medicinals in supplementation， and to treat by stages based on syndrome differentiation. For dryness toxin with fire-heat， it is suggested to moisten dryness， resolve toxins and subdue fire， with self-made Runzao Jiedu Decoction （润燥解毒汤） in modification. For dryness toxin with damp-heat， the method of nourishing yin， clearing heat and draining dampness should be used， and Chunze Decoction （春泽汤） in modification is suggested. For dryness toxin with phlegm-stasis， it is recommended to unblock collaterals， disperse phlegm and dissipate stasis， with self-made Sanyu Xiaotan Decoction （散瘀消痰汤） in modification.

Objective To analyze the correlation between the expression of soluble glycoprotein A(GPA)in plasma of healthy blood donors and anti-M and anti-"Mia"antibodies.Methods Plasma from healthy donors from February 9,2022 to February 15,2023 was collected:irregular antibody-negative NN type(group Ⅰ,n=118)and MM type(group Ⅱ,n=51),anti-M antibody positive NN type(group Ⅲ,n=145)and anti-"Mia"antibody positive companion type(group Ⅳ,n= 87),the GPA content in plasma of different individuals in 4 groups was detected,and the difference in GPA expression was analyzed by t-test.Results The average plasma GPA contents in groupsⅠ,Ⅱ,Ⅲ and Ⅳ were 9.941±0.252,10.97±0.256,5.139±0.129 and 4.28±0.139ng/ml,respectively.The average GPA content of groups Ⅰ and Ⅱ was higher,and the average GPA content of groups Ⅲ and Ⅳ was lower,and the differences were statistically significant(all P＜0.01).Conclusion The GPA content in plasma of healthy donors with anti-M and anti-"Mia"antibodies was significantly lower than that of the antibody-negative group.The results of this study lay a foundation for further investigation of whether GPA in plasma has the ability to neutralize anti-M and anti-"Mia"antibodies,improve disease diagnosis and safe blood transfusion.

Objective:To study the feasibility for transumbilical single-incision laparoscopic appendectomy (SILA) with conventional laparoscopic instruments, and compare SILA with the traditional three-port laparoscopic appendicectomy (LA).Methods:The clinical data of 113 patients with acute appendicitis from January 2018 to August 2020 in the Second Hospital of Jiaxing City were retrospectively analyzed. Among them, 61 patients received traditional three-port LA (three-port LA group), 52 patients received transumbilical SILA (SILA group). The operative time, intra-operative blood loss, surgical complications, length of hospital stay and hospitalization costs were recorded. Pain score 6 h after operation was assessed by visual analogue scale. C-reactive protein (CRP) on the first day after operation was detected. All patients were followed up for 1 month.Results:In 2 groups, all patients did not suffered from the conversion to open operation or multi-port method, massive bleeding and accessory injury during the operation. Moreover, severe pain, severe incision infection, residual abdominal abscess and incisional hernia did not occur. Patients in SILA group had more hidden abdominal scars. there were no statistical differences in operative time, intra-operative blood loss and CRP on the first day after operation between 2 groups ( P>0.05); the rate of moderate pain degree 6 h after operation, length of hospital stay and hospitalization costs in SILA group were significantly lower than those in three-port LA group: 15.38% (8/52) vs. 34.43% (21/61), (3.63 ± 1.22) d vs. (4.31 ± 1.38) d and (8 802 ± 1 466) yuan vs. (9 559 ± 1 617) yuan, and there were statistical differences ( P<0.05 or <0.01). Conclusions:The transumbilical SILA with conventional laparoscopic instruments is safe and feasible, the scar is more difficult to see, the cosmetic effect is much clearer, and the length of hospital stay is shorter.

Objective:To investigate the value of 95% spectral edge frequency (SEF) in identifying the depth of anesthesia in patients with schizophrenia subjected to modified electroconvulsive therapy center.Methods:A total of 195 patients with schizophrenia who received treatment in The Affiliated Kangning Hospital of Wenzhou Medical University in April to December 2020 were included in this study. They were randomly divided into three groups with 65 patients each. Three groups of patients received different doses of anesthesia before undergoing MECT as follows: group A: propofol 1.5 mg/kg, atropine 0.5 mg/kg, succinylcholine chloride 1.0 mg/kg; group B: propofol 2.0 mg/kg, atropine 0.5 mg/kg, succinylcholine chloride 1.0 mg/kg; group C: propofol 2.5 mg/kg, atropine 0.5 mg/kg, succinylcholine chloride 1.0 mg/kg. 95% SEF and bispectral index (BIS) were measured when patients were awake before treatment (T 1), when eyelash reflex disappeared (T 2), at the beginning of electrical stimulation (T 3), at 3 minutes after electrical stimulation (T 4), and when patients were completely awake (T 5) and compared between groups. The incidence of adverse reaction was recorded at 1 day after treatment. Results:At T 1-T 5, 95% SEF in the group A was (28.50 ± 0.87) Hz, (21.49 ± 0.91) Hz, (21.99 ± 0.92) Hz, (28.42 ± 1.29) Hz, (28.40 ± 1.15) Hz respectively, and it was (28.34 ± 0.91) Hz, (18.93 ± 0.86) Hz, (19.05 ± 0.83) Hz, (27.54 ± 0.73) Hz, (28.42 ± 1.21) Hz respectively in group B and (28.26 ± 0.90) Hz, (16.41 ± 0.75) Hz, (16.36 ± 0.75) Hz, (26.58 ± 0.64) Hz, (28.48 ± 1.19) Hz respectively in group C. 95% SEF measured at T 2 ( t = 24.49, 48.60, both P < 0.05), T 3 ( t = 28.47, 54.51, both P < 0.05), and T 4 ( t = 7.61, 15.91, both P < 0.05) in groups B and C were significantly lower than those in group A. 95% SEF measured at T 2 ( t = 24.11, P < 0.05), T 3 ( t = 26.04, P < 0.05) and T 4 ( t = 8.30, P < 0.05) in group C were significantly lower than those in group B. At T 1-T 5, BIS in group A was (94.16 ± 2.07), (55.34 ± 1.93), (56.61 ± 1.84), (76.29 ± 1.94) and (93.84 ± 2.39) respectively, and it was (94.51 ± 2.25), (52.39 ± 1.58), (52.45 ± 1.94), (73.58 ± 2.19), (93.28 ± 2.52) respectively in group B and (93.97 ± 2.16), (50.57 ± 1.96), (51.60 ± 2.03), (69.51 ± 2.12), (93.57 ± 2.66) respectively in group C. BIS values measured at T 2 ( t = 24.49, 48.60, both P < 0.05), T 3 ( t = 28.34, 54.28, both P < 0.05), and T 4 ( t = 7.61, 15.91, both P < 0.05) in groups B and C were significantly lower than those in group A. BIS measured at T 2 ( t = 24.11, P < 0.05), T 3 ( t = 25.93, P < 0.05), and T 4 ( t = 8.30, P < 0.05) in group C were significantly lower than those in group B. Correlation analysis showed that 95% SEF measured at T 2 ( r = 0.65, P < 0.05), T 3 ( r = 0.68, P < 0.05) and T 4 ( r = 0.49, P < 0.05) were positively correlated with BIS measured at corresponding time points. There were no significant differences in duration of electrical stimulation [(61.25 ± 4.32) seconds, (45.19 ± 3.68) seconds, and (27.54 ± 2.54) seconds, F = 1 434.14, P < 0.05], post-onset inhibition index [(87.68 ± 5.82)%, (81.59 ± 5.35)%, (75.27 ± 4.87)%, F = 87.09, P < 0.05], and average seizure energy index [(5 668.38 ± 1 264.01) μV2, (4 555.61 ± 1 058.96) μV2, (3 642.25 ± 792.68) μV2, F = 59.97, P < 0.05] among the three groups. Duration of electrical stimulation ( t = 36.07, 75.71, both P < 0.05), post-onset inhibition index ( t = 9.15, 18.66, both P < 0.05), and average seizure energy index ( t = 8.49, 15.46, both P < 0.05) in groups B and C were significantly lower than those in group A. Duration of electrical stimulation, post-onset inhibition index and average seizure energy index in group C were significantly lower than those in group B ( t = 39.64, 9.50, 6.97, all P < 0.05). BIS was positively correlated with duration of electrical stimulation ( r = 0.68, P < 0.05), post-onset inhibition index ( r = 0.55, P < 0.05) and average seizure energy index ( r = 0.42, P < 0.05). There were no significant differences in the incidences of headache, myalgia, nausea and vomiting among the three groups ( P > 0.05). Conclusion:95% SEF was positively correlated with BIS in patients with schizophrenia. BIS measured at T 2 was positively correlated with effect of modified electroconvulsive therapy center.

Objective:To understand the cognition and related factors on the use of HIV non-occupational post-exposure prophylaxis (nPEP) among men who have sex with men (MSM).Methods:The snowballing method was applied to recruit research subjects who were ≥18 years old, had sex with men in the past three months, and were aware of nPEP in MSM groups in Beijing, Shenzhen, and Kunming from March 15 to April 14, 2019. Data on social demographics, behavioral characteristics, basic knowledge of nPEP, consultation, and using nPEP were collected through "i guardian Platform". The logistic regression model was used to analyze the related factors affecting the use of nPEP.Results:Among 1 809 investigated, 39.8% (720 persons) were aware of the basic knowledge of nPEP, 33.4% (605 persons) had consulted nPEP, and 15.0% (271 persons) had used nPEP. In addition, multivariate logistic regression analysis showed that factors as whether to have sex with men infected with HIV in the last three months ( OR=2.58, 95% CI: 1.64-4.07), the frequency of HIV testing in the past year ( OR=2.47, 95% CI: 1.28-5.11), nPEP knowledge awareness ( OR=0.70, 95% CI: 0.49-0.99), whether to consult nPEP ( OR=70.98, 95% CI: 40.51-136.83) were related to the use of nPEP. Conclusions:MSM still have poor cognition of nPEP. It is necessary to strengthen the publicity and education of nPEP in MSM and promote the use of nPEP after HIV exposure as soon as possible.

OBJECTIVE: To detect pathogenic variant in a child featuring Usher syndrome type II. METHODS: Peripheral blood samples of the child and his parents were collected for the analysis of variants of hearing impairment-related genes. The findings were verified in 100 individuals with normal hearing. RESULTS: The child was found to harbor compound heterozygous variants of the USH2A gene, namely c.8224-1G>C in intron 41 and c.5678C>G(p.Ser1893X) in exon 28, which were inherited respectively from his mother and father. Based on the American College of Medical Genetics and Genomics standards and guidelines, both c.8224-1G>C and c.5678C>G(p.Ser1893X) variants of USH2A gene were predicted to be pathogenic(PVS1+PM2+PM3). CONCLUSION The compound heterozygous variants c.8224-1G>C and c.5678C>G of the USH2A gene probably underlay the disease in this child. Above finding has enriched the spectrum of USH2A gene variants.
Child ; Exons ; Extracellular Matrix Proteins/genetics* ; Family ; Humans ; Introns ; United States ; Usher Syndromes/genetics*