Objective To explore the policy foundations of early family education and rehabilitation support systems for children with developmental disabilities aged zero to three years,based on the International Classification of Functioning,Disability and Health(ICF),and to construct a comprehensive,whole-person and whole-lifecycle support system integrating early childhood education and rehabilitation services for families;and to propose corresponding sys-tem architecture,construction pathways and governance models.Methods Policy documents were compared,which were related to children's rehabilitation from the World Health Orga-nization,early education guidelines for children with disabilities from the United Nations Educational,Scientific and Cultural Organization,the U.S.Individuals with Disabilities Education Act,European Union children's reha-bilitation policies,and the"14th Five-Year Plan"from China Disabled Persons'Federation.The related policy frameworks and key content of early family education and rehabilitation were reviewed,and the composition,implementation pathways and governance models of the support system were systymatically analyzed.Results The support system consisted of early screening and assessment,family education and parent training,counsel-ing and personalized education and rehabilitation support,community support and resource integration,and re-mote digital support platforms.The study proposed the implementation pathways for five systems,including early screening and assessment,family education and parent training,counseling and personalized rehabilitation support,community support and resource integration,and remote digital support platforms.It emphasized gover-nance mechanisms such as multidisciplinary collaboration,interdepartmental coordination and support from re-mote digital platforms to build a continuous service chain from standardized assessments to interdepartmental collaboration.Conclusion The person-centered approach and whole life span development concept based on the ICF model,as well as the guiding principles of the health services continuum,provide systematic theoretical and policy support for early childhood education and rehabilitation for children with developmental disabilities aged zero to three years.Through multidisciplinary collaboration,interdepartmental coordination and the application of digital platforms,a scientific,continuous and child-centered support system can be built.This not only facilitates early detection and precise intervention but also promotes multi-party collaboration among families,communities and profes-sional institutions.It will further integrate disability prevention,rehabilitation and healthcare services,thereby im-proving children's functional abilities and family quality of life.

Objective To construct a theoretical framework for early home-based education and rehabilitation for infants and tod-dlers with developmental disabilities,using the bio-psycho-social model of the International Classification of Functioning,Disability and Health(ICF).Methods On the base of integrating ecosystem theory,neuroplasticity theory,family systems theory and rehabilitation concept of World Health Organization,a home-based education and rehabilitation system encompassing biologi-cal,psychological,social and policy dimensions was developed.Results Within the ICF framework,it was set the operational intervention goals for infants and toddlers in dimensions of motor,language,cognition,emotion and social interaction,while personalized goals were formulated in consider-ation of different types of developmental disabilities.Furthermore,a home-based education and rehabilitation sys-tem was designed,including early screening and assessment,development of personalized intervention plans,home-based education and rehabilitation training,continuous monitoring and adjustment,digital empowerment and remote guidance,as well as disability prevention,and early education and rehabilitation.It involved five stag-es:needs assessment,plan development,plan implementation,dynamic assessment and feedback,and summary and referral.Conclusion This study has constructed a five-stage process,providing an effective framework for the comprehensive re-habilitation of infants and toddlers.ICF framework provides both theoretical and practical guidance for early in-tervention in infants and toddlers with developmental disabilities.

Recent studies have confirmed that the meningeal lymphatic system (MLS), a functional lymphatic network, exists in the dura mater of the central nervous system (CNS). It can connect to the peripheral lymphatic system and remove metabolic wastes from the CNS. MLS discovery has overturned the traditional view that the brain is an "immune-exempt" organ and provides a new perspective for Alzheimer's disease (AD) research. This article reviews the discovery, anatomical structure, and physiological functions of MLS, and deeply explores the mutual regulation mechanism between MLS and immune system and its key role in AD occurrence and development, aiming to provide new ideas for AD treatments targeting MLS.

Objective To explore the policy foundations of early family education and rehabilitation support systems for children with developmental disabilities aged zero to three years,based on the International Classification of Functioning,Disability and Health(ICF),and to construct a comprehensive,whole-person and whole-lifecycle support system integrating early childhood education and rehabilitation services for families;and to propose corresponding sys-tem architecture,construction pathways and governance models.Methods Policy documents were compared,which were related to children's rehabilitation from the World Health Orga-nization,early education guidelines for children with disabilities from the United Nations Educational,Scientific and Cultural Organization,the U.S.Individuals with Disabilities Education Act,European Union children's reha-bilitation policies,and the"14th Five-Year Plan"from China Disabled Persons'Federation.The related policy frameworks and key content of early family education and rehabilitation were reviewed,and the composition,implementation pathways and governance models of the support system were systymatically analyzed.Results The support system consisted of early screening and assessment,family education and parent training,counsel-ing and personalized education and rehabilitation support,community support and resource integration,and re-mote digital support platforms.The study proposed the implementation pathways for five systems,including early screening and assessment,family education and parent training,counseling and personalized rehabilitation support,community support and resource integration,and remote digital support platforms.It emphasized gover-nance mechanisms such as multidisciplinary collaboration,interdepartmental coordination and support from re-mote digital platforms to build a continuous service chain from standardized assessments to interdepartmental collaboration.Conclusion The person-centered approach and whole life span development concept based on the ICF model,as well as the guiding principles of the health services continuum,provide systematic theoretical and policy support for early childhood education and rehabilitation for children with developmental disabilities aged zero to three years.Through multidisciplinary collaboration,interdepartmental coordination and the application of digital platforms,a scientific,continuous and child-centered support system can be built.This not only facilitates early detection and precise intervention but also promotes multi-party collaboration among families,communities and profes-sional institutions.It will further integrate disability prevention,rehabilitation and healthcare services,thereby im-proving children's functional abilities and family quality of life.

Objective To construct a theoretical framework for early home-based education and rehabilitation for infants and tod-dlers with developmental disabilities,using the bio-psycho-social model of the International Classification of Functioning,Disability and Health(ICF).Methods On the base of integrating ecosystem theory,neuroplasticity theory,family systems theory and rehabilitation concept of World Health Organization,a home-based education and rehabilitation system encompassing biologi-cal,psychological,social and policy dimensions was developed.Results Within the ICF framework,it was set the operational intervention goals for infants and toddlers in dimensions of motor,language,cognition,emotion and social interaction,while personalized goals were formulated in consider-ation of different types of developmental disabilities.Furthermore,a home-based education and rehabilitation sys-tem was designed,including early screening and assessment,development of personalized intervention plans,home-based education and rehabilitation training,continuous monitoring and adjustment,digital empowerment and remote guidance,as well as disability prevention,and early education and rehabilitation.It involved five stag-es:needs assessment,plan development,plan implementation,dynamic assessment and feedback,and summary and referral.Conclusion This study has constructed a five-stage process,providing an effective framework for the comprehensive re-habilitation of infants and toddlers.ICF framework provides both theoretical and practical guidance for early in-tervention in infants and toddlers with developmental disabilities.

Recent studies have confirmed that the meningeal lymphatic system (MLS), a functional lymphatic network, exists in the dura mater of the central nervous system (CNS). It can connect to the peripheral lymphatic system and remove metabolic wastes from the CNS. MLS discovery has overturned the traditional view that the brain is an "immune-exempt" organ and provides a new perspective for Alzheimer's disease (AD) research. This article reviews the discovery, anatomical structure, and physiological functions of MLS, and deeply explores the mutual regulation mechanism between MLS and immune system and its key role in AD occurrence and development, aiming to provide new ideas for AD treatments targeting MLS.

OBJECTIVES: To evaluate the performance of different multi-modality fusion models for predicting radiation-induced oral mucositis (RIOM) following radiotherapy in patients with nasopharyngeal carcinoma (NPC). METHODS: We retrospectively collected the data from 198 patients with locally advanced NPC who experienced RIOM following radiotherapy at the Affiliated Tumor Hospital of Guangzhou Medical University from September, 2022 to February, 2023. Based on oral radiation dose-volume parameters and clinical features of NPC, basic classification models were developed using different combinations of feature selection algorithms and classifiers and integrated using a multi-criterion decision-making (MCDM)-based classifier fusion (MCF) strategy and its variant, the H-MCF model. The basic classification models, MCF model, the H-MCF model with a single modality or multiple modalities and other ensemble classifiers were compared for performances for predicting RIOM by assessing the area under the ROC curve (AUC), accuracy, sensitivity, and specificity. RESULTS: The H-MCF model, which integrated multi-modality features, achieved the highest accuracy for predicting severe RIOM with an AUC of 0.883, accuracy of 0.850, sensitivity of 0.933, and specificity of 0.800. CONCLUSIONS Compared with each of the individual classifiers, the multimodal multi-classifier fusion algorithm combining clinical and dosimetric modalities demonstrates superior performance in predicting the incidence of severe RIOM in NPC patients following radiotherapy.
Humans ; Nasopharyngeal Carcinoma/radiotherapy* ; Nasopharyngeal Neoplasms/radiotherapy* ; Retrospective Studies ; Stomatitis/diagnosis* ; Algorithms ; Radiation Injuries/diagnosis* ; Female ; Male ; ROC Curve

Objective To compare the clinical efficacy between air-free trans-axillary laparoscopic thyroidectomy and open thyroidectomy. Methods A retrospective analysis was performed on the data of both 96 patients who underwent air-free trans-axillary laparoscopic thyroidectomy (laparoscopic group) and 232 patients who underwent open thyroidectomy (open group) from August 2015 to July 2020. Comparative analysis were performed on operative data, the postoperative data of recovery and complications between two groups. Results The operative time in laparoscopic group was longer significantly than that in open group [(168±23) min vs. (95±18) min, P=0.022]. There was no significantly difference in the rate of recognition of recurrent laryngeal nerve between two groups (100.0% vs. 99.1%, P=0.138). The rate of recognition of both superior (98.0% vs. 97.0%, P=0.003) and inferior parathyroid glands (95.8% vs. 99.1%, P=0.028) in laparoscopic group was higher significantly than those in open group. The amount of drain of the first day after operation in laparoscopic group was more significantly than that in open group[(42±22) mL vs. (25±10) mL, P=0.006]. The postoperative length of hospital stay in laparoscopic group was longer significantly [(2.8±0.6) d vs. (1.8±1.2) d, P=0.016]. The cost of hospital stay was (13 186±1 015) yuan vs. (12 632±1 456) yuan （P=0.365） and the rate of postoperative complication was 10.4% vs. 9.1%（P=0.126）, which all showed no significant difference between two groups. Conclusions Air-free trans-axillary laparoscopic thyroidectomy could be feasible and safe, and have better appearance. However, both operative time and hospital stay were longer.

Objective:To investigate the effect of the nucleotide-binding domain, leucine-rich-containing family, pyrin domain-containing 3 (NLRP3) inflammasome on the proliferation and apoptosis of human retinal pigment epithelial cell line ARPE-19 exposed to high glucose and its mechanism.Methods:ARPE-19 cells cultured in vitro were divided into normal control group and high-glucose group, and were cultured in conventional medium and medium containing 30 mmol/L glucose for 48 hours, respectively.The content of reactive oxygen species (ROS) were detected by fluorescent probe, and the activity of superoxide dismutase (SOD) and the concentration of malondialdehyde (MDA) were tested by biochemical assay.The cells of the two groups were cultured with 0, 2, 5, 10, 15 and 20 μmol/L NLRP3 inhibitor CY-09 for 48 hours, respectively.The proliferation rate of ARPE-19 cells under various concentrations of CY-09 treatment was detected by cell counting kit-8, and the appropriate concentration of CY-09 was determined.ARPE-19 cells were divided into normal control group, normal+ CY-09 group, high-glucose group and high glucose+ CY-09 group.The culture medium in the normal+ CY-09 group and high glucose+ CY-09 group was supplemented with 15 μmol/L CY-09.Flow cytometry was used to detect the apoptosis rate of each group, and Western blot was used to detect the relative expression levels of NLRP3, apoptosis-associated point protein (ASC), Caspase-1 precursor (pro-Caspase-1) and active fragments (cleaved-Caspase-1), B lymphocytoma-2 protein (Bcl-2), Bcl-2-associated X protein (Bax), Caspase-3 precursor (pro-Caspase-3) and active fragments (cleaved-Caspase-3). Results:The intensity of ROS fluorescence and MDA concentration were 120 020±3 245, (4.92±0.09) nmol/mg in the high-glucose group, which were both significantly higher than 35 426±811 and (1.78±0.03) nmol/mg in the normal control group, and the SOD activity was (35.65±1.22) μmol/(min·mg) in the high-glucose group, which was significantly lower than (74.96±1.41) μmol/(min·mg) in the normal control group, showing statistically significant differences between the two groups ( t=35.760, 46.960, 29.830; all at P<0.05). The proliferation rate of RPE cells in high-glucose group was significantly lower than that in normal control group, and the difference was statistically significant ( t=18.820, P<0.05). With the increase of CY-09 concentration, the proliferation rate of cells in the high-glucose group was gradually increased.The proliferation rates of cells treated with 10, 15 and 20 μmol/L CY-09 were all significantly higher than those treated with 0 μmol/L CY-09, showing statistically significant differences between them (all at P<0.05). The proliferation rates of cells treated with 15 μmol/L and 0 μmol/L CY-09 were not significantly different in the normal control group ( P>0.05). The apoptosis rate of cells in the high-glucose group was (21.68±0.41)%, which was significantly higher than (6.67±1.05)% in the normal control group and (13.96±0.07)% in the high-glucose+ CY-09 group, and the differences were statistically significant (both at P<0.05). The relative expression levels of NLRP3, ASC, cleaved-Caspase-1, cleaved-Caspase-3 and Bax proteins were significantly higher and the relative expression levels of Bcl-2 protein was significantly lower in the high-glucose group compared with the normal control group, and the differences were statistically significant (all at P<0.05). The relative expression levels of NLRP3, ASC, the active fragment of cleaved-Caspase-1, Bax and cleaved-Caspase-3 proteins were decreased and the relative expression levels of Bcl-2 protein were increased in the normal+ CY-09 group and high glucose+ CY-09 group compared with the normal control group and high glucose group, and the differences were statistically significant (all at P<0.05). Conclusions:NLRP3 inflammasome mediates the high glucose induced RPE cells apoptosis through ROS/NLRP3/Caspase-1 signaling pathway.

Objective:To investigate the clinical, skeletal muscle pathological, and genetic characteristics of fatal infantile hypertonic myofibrillar myopathy (FIHMM).Methods:The clinical manifestations, laboratory assessments data and gene sequencing results of 10 patients diagnosed with FIHMM in Shenzhen Children′s Hospital from February 2017 to April 2021 were retrospectively analyzed.Magnetic resonance imaging (MRI) of both musculoskeletal system and the brain, and electromyogram (EMG) were performed in 3 cases, while muscle biopsy was performed in 2 cases.Results:Among these 10 cases, 1 case was from Northeast China and 1 case from East China, while the rest 8 cases were from South China.Eight of the 10 patients were male, and the other 2 cases were female.They were all born normal and not related to each other.The age of onset varied from 2 to 12 months.The main clinical manifestations for all the patients were progressive rigidity of the rectus abdominis (8 cases), neck muscles (7 cases), rectus abdominis (2 cases) and intercostal muscles (1 case), resulting in respiratory failure.Mildly to moderately elevated serum creatine kinase level was detected (436-5 804 IU/L) (reference range: 24-229 IU/L). Complex repetitive discharges can be seen in the EMG, without any myotonic potential.Muscle fiber degeneration, necrosis, and vacuolar degeneration were noted in the histopathological examination of the vastus lateralis and rectus abdominis.An abnormal red granular deposit was observed in a portion of the field of the modified Gomory Trichrome staining.Immunohistochemistry showed substantial deposition of desmin.Under the electron microscopy, the sarcomere structure of the muscle fibers was seriously disordered, with the destruction of Z-bands and the presence of granular deposits.The whole-exome sequencing identified the same homozygous variation c. 3G>A, p.Met1? of CRYAB gene in all the patients, but heterozygous variation in their parents. Conclusions:Axial muscles involvement, such as rectus abdominis rigidity, is the main clinical characteristic of FIHMM.c.3G>A, p.Met1? mutation in the CRYAB gene is a hotspot mutation in Chinese children.