This consensus will introduce the characteristics of fillers used in the surgical cavities of domestic nasal surgery patients based on relevant literature and expert opinions. It will also provide recommendations for the selection of cavity fillers for different nasal diseases, with chronic sinusitis as a representative example.
Humans ; Nasal Cavity/surgery* ; Nasal Surgical Procedures ; China ; Consensus ; Sinusitis/surgery* ; Dermal Fillers

Objective:To explore the clinical and genetic characteristics of dyskeratosis congenita (DC).Methods:A retrospective analysis was conducted on the clinical, laboratory, imaging, pathological, genetic, and treatment data of 6 DC patients diagnosed at the Children′s Hospital of Zhejiang University School of Medicine from January 2010 to June 2025.Results:Among the 6 DC patients, 1 case was of Hoyeraal-Hreidarsson syndrome, 4 were male, and 2 were female. The diagnosis age 0.9-6.1 years. All 6 cases presented with bone marrow failure; 5 cases had a classic triad of skin and mucous membrane (mucosal leukoplakia, abnormal skin pigmentation, nail dystrophy); 5 cases had growth retardation, among which 2 cases had intrauterine growth retardation. Two cases had diarrhea and 1 case had abnormal liver function; 1 case had stiff and deformed limbs, accompanied by limited mobility, and dry and obstructive balanitis; 1 case had recurrent eyelid inflammation, middle ear inflammation, and nasal inflammation. All 6 cases had decreased B cell numbers, and 4 cases also had decreased natural killer cell numbers. There were 3 cases of children with cytomegalovirus (CMV) infection, of which 1 case of CMV infection led to retinal frosted branch angiitis and subsequent intracranial CMV infection resulting in death, and 1 case had CMV enteritis and died of hemophagocytic syndrome. Among 4 cases of boys, 3 cases had DKC1 gene variations and 1 case had an unknown variation gene; 2 cases of girls had TINF2 gene variations. The TINF2 c.860T>A (p.L287Q) variation site was a new mutation. Among 6 patients with DC, 2 cases died, 3 cases survived and 1 case was lost to follow-up.Conclusions:The DKC1 and TINF2 genes are common pathogenic genes in patients with DC. Bone marrow failure is a clue for the early identification of DC. The triad of skin and mucous membrane is its typical clinical manifestation. Children with DC generally have reduced B cells and natural killer killer cells, and have a high risk of fatal CMV infection. The overall prognosis is poor.

Objective:To investigate the clinical characteristics and independent risk factors of severe disease in patients with anti-nuclear matrix protein (NXP) 2 antibody-positive juvenile dermatomyositis (JDM).Methods:A retrospective cohort study was conducted, including 219 anti-NXP2 antibody-positive JDM patients admitted to 23 children′s hospitals across China from July 2011 to July 2023. Patients were classified into severe and non-severe groups based on classification criteria for severe dermatomyositis. Demographic characteristics, clinical manifestations, and laboratory parameters were compared between the 2 groups using independent sample t-test, Mann-Whitney U test, or χ2 test. Univariate and multivariate Logistic regression analyses were performed to identify risk factors for severe disease. The receiver operating characteristic curve was employed to calculate optimal cut-off values. Results:Among the 219 patients, 108 were male and 111 were female, with an age at onset of 6.3 (3.5, 9.4) years. The severe group comprised 69 patients, and the non-severe group 150 patients. The severe group had significantly higher rates of fever, heliotrope rash, subcutaneous edema, periorbital edema, anti-Ro52 antibody positivity, as well as elevated levels of ferritin-to-albumin ratio (FAR), creatine kinase (CK), aspartate aminotransferase (AST), and lactate dehydrogenase (LDH) (all P<0.05). Multivariate analysis identified anti-Ro52 antibody positivity ( OR=13.26, 95% CI 1.37-128.29) and elevated FAR ( OR=1.90, 95% CI 1.09-2.31) as independent risk factors for severe anti-NXP2 antibody-positive JDM (both P<0.05). Receiver operating characteristic curve analysis revealed that a FAR cutoff value of 6.82 predicted severe disease with an area under the curve of 0.87 (95% CI 0.81-0.94, P<0.001), sensitivity of 0.85, and specificity of 0.70. All patients received glucocorticoid therapy, and the severe group received higher proportions of steroid pulse therapy, cyclophosphamide, mycophenolate mofetil, intravenous immunoglobulin, biologics, and adjuvant treatments compared to the non-severe group (all P<0.05). In terms of outcomes, 2 patients (2.9%) in the severe group died (due to neurological involvement and intestinal perforation, respectively), while the remaining patients achieved complete clinical response or remission. All patients in the non-severe group achieved remission. Conclusions:The primary clinical features of anti-NXP2 antibody-positive JDM included fever, heliotrope rash, subcutaneous edema, periorbital edema, anti-Ro52 antibody positivity, and elevated levels of CK, AST, LDH, and FAR. Furthermore, anti-Ro52 antibody positivity and a FAR>6.82 were identified as independent risk factors.

ObjectiveTo review the interoceptive assessment tools in terms of structure, content and psychometric properties, based on the framework of the International Classification of Functioning, Disability and Health (ICF). MethodsThe literature on interoceptive evaluation tools was retrieved from databases of CNKI, PubMed, Medline and EBSCO. The principal structures and contents of the assessment tools were analyzed based on the ICF framework, and the quality of the psychometric properties were appraised using COSMIN. ResultsA total of 13 interoceptive assessment tools were ultimately included, involving 16 literature references. There were five interoceptive sensitivity tools, four accuracy tools and four awareness tools. In terms of content, interoceptive sensitivity tools involved 33 categories of body functions, six categories of activities and participation, and one of environmental factors; while interoceptive accuracy tools only involved seven categories of body function, and two of activities and participation items; interoceptive awareness tools involved 30 categories of body function, four categories of activities and participation, and three of environmental factors. In terms of psychometric properties, Body Perception Questionnaire-Short Form (BPQ-SF) was the sensitivity tool with the best reliability and validity (qualified rate of 7/8), followed by Interoceptive Sensitivity Questionnaire (ISQ) (qualified rate of 6/8). Most of the accuracy tools adopted standardized measurement methods, but lacked sufficient reliability and validity verification. The awareness tools were good in reliability and validity (qualified rate above 5/8), especially Multidimensional Assessment of Interoceptive Awareness (MAIA-1) and Body Awareness Questionnaire (BAQ) (qualified rate of 8/8). ConclusionBPQ-SF and ISQ are recommended for interoceptive sensitivity assessment, Water-loading Test and Heart-beat Tracking Task for interoceptive accuracy assessment, and MAIA-1 and BAQ for interoceptive awareness assessment.

Objective To explore the surgical treatment experience of primary giant gastrointestinal stromal tumors(GIST)(with isolated lesions with a maximum diameter＞10 cm).Methods A retrospective analysis was conducted on the clinical and pathological data of 67 patients with primary giant GIST admitted from January 2018 to December 2024.Among them,35 cases underwent surgical operations after preoperative neoadjuvant therapy(25 effective cases and 10 ineffective cases)(neoadjuvant therapy group).Due to the initial diagnosis assessment expecting radical(R0)resection(13 cases),or preoperative complications(12 cases),or difficulty in obtaining a pathological diagnosis through puncture biopsy(7 cases),32 cases underwent direct surgery without neoadjuvant therapy(direct surgery group).Compare the general information,tumor condition,surgical condition,postoperative recovery,postoperative pathology,postoperative adjuvant therapy,and recurrence between two groups.Results Comparative analysis revealed that there was no statistically significant difference(P＞0.05)between the neoadjuvant therapy group and the direct surgery group in terms of gender,age,primary tumor location,initial maximum diameter,growth type,localized or locally advanced stage,and postoperative follow-up time.The maximum diameters of the tumors before surgery in the neoadjuvant therapy group and the direct surgery group were(12.4±7.1)cm and(18.2±5.0)cm respectively,and the operation times were(125.4±30.6)minutes and(153.0±31.7)minutes respectively.The intraoperative blood loss was(228.3±76.4)ml and(300.3±67.2)ml,respectively.The postoperative hospital stay was(9.1±2.6)days and(11.1±3.2)days,respectively.There was a statistically significant difference between the two groups(P＜0.05).The proportion of laparoscopic surgery in the neoadjuvant therapy group was 17.1％,which was higher than that in the direct surgery group(0),and the difference was statistically significant(P＜0.05).There was no statistically significant difference between the two groups in terms of the proportion of tumor rupture,combined organ resection,postoperative complications and postoperative recurrence(P＞0.05).Conclusion Primary giant gastrointestinal stromal tumors can mostly be reduced in size and progression through neoadjuvant therapy,improving the chances of minimally invasive surgery.However,there is also a risk of tumor progression during neoadjuvant therapy leading to increased surgical difficulty or even loss of curative surgical opportunities.

Objective To explore the surgical treatment experience of primary giant gastrointestinal stromal tumors(GIST)(with isolated lesions with a maximum diameter＞10 cm).Methods A retrospective analysis was conducted on the clinical and pathological data of 67 patients with primary giant GIST admitted from January 2018 to December 2024.Among them,35 cases underwent surgical operations after preoperative neoadjuvant therapy(25 effective cases and 10 ineffective cases)(neoadjuvant therapy group).Due to the initial diagnosis assessment expecting radical(R0)resection(13 cases),or preoperative complications(12 cases),or difficulty in obtaining a pathological diagnosis through puncture biopsy(7 cases),32 cases underwent direct surgery without neoadjuvant therapy(direct surgery group).Compare the general information,tumor condition,surgical condition,postoperative recovery,postoperative pathology,postoperative adjuvant therapy,and recurrence between two groups.Results Comparative analysis revealed that there was no statistically significant difference(P＞0.05)between the neoadjuvant therapy group and the direct surgery group in terms of gender,age,primary tumor location,initial maximum diameter,growth type,localized or locally advanced stage,and postoperative follow-up time.The maximum diameters of the tumors before surgery in the neoadjuvant therapy group and the direct surgery group were(12.4±7.1)cm and(18.2±5.0)cm respectively,and the operation times were(125.4±30.6)minutes and(153.0±31.7)minutes respectively.The intraoperative blood loss was(228.3±76.4)ml and(300.3±67.2)ml,respectively.The postoperative hospital stay was(9.1±2.6)days and(11.1±3.2)days,respectively.There was a statistically significant difference between the two groups(P＜0.05).The proportion of laparoscopic surgery in the neoadjuvant therapy group was 17.1％,which was higher than that in the direct surgery group(0),and the difference was statistically significant(P＜0.05).There was no statistically significant difference between the two groups in terms of the proportion of tumor rupture,combined organ resection,postoperative complications and postoperative recurrence(P＞0.05).Conclusion Primary giant gastrointestinal stromal tumors can mostly be reduced in size and progression through neoadjuvant therapy,improving the chances of minimally invasive surgery.However,there is also a risk of tumor progression during neoadjuvant therapy leading to increased surgical difficulty or even loss of curative surgical opportunities.

Objective:To explore the clinical and genetic characteristics of dyskeratosis congenita (DC).Methods:A retrospective analysis was conducted on the clinical, laboratory, imaging, pathological, genetic, and treatment data of 6 DC patients diagnosed at the Children′s Hospital of Zhejiang University School of Medicine from January 2010 to June 2025.Results:Among the 6 DC patients, 1 case was of Hoyeraal-Hreidarsson syndrome, 4 were male, and 2 were female. The diagnosis age 0.9-6.1 years. All 6 cases presented with bone marrow failure; 5 cases had a classic triad of skin and mucous membrane (mucosal leukoplakia, abnormal skin pigmentation, nail dystrophy); 5 cases had growth retardation, among which 2 cases had intrauterine growth retardation. Two cases had diarrhea and 1 case had abnormal liver function; 1 case had stiff and deformed limbs, accompanied by limited mobility, and dry and obstructive balanitis; 1 case had recurrent eyelid inflammation, middle ear inflammation, and nasal inflammation. All 6 cases had decreased B cell numbers, and 4 cases also had decreased natural killer cell numbers. There were 3 cases of children with cytomegalovirus (CMV) infection, of which 1 case of CMV infection led to retinal frosted branch angiitis and subsequent intracranial CMV infection resulting in death, and 1 case had CMV enteritis and died of hemophagocytic syndrome. Among 4 cases of boys, 3 cases had DKC1 gene variations and 1 case had an unknown variation gene; 2 cases of girls had TINF2 gene variations. The TINF2 c.860T>A (p.L287Q) variation site was a new mutation. Among 6 patients with DC, 2 cases died, 3 cases survived and 1 case was lost to follow-up.Conclusions:The DKC1 and TINF2 genes are common pathogenic genes in patients with DC. Bone marrow failure is a clue for the early identification of DC. The triad of skin and mucous membrane is its typical clinical manifestation. Children with DC generally have reduced B cells and natural killer killer cells, and have a high risk of fatal CMV infection. The overall prognosis is poor.

Objective:To investigate the clinical characteristics and independent risk factors of severe disease in patients with anti-nuclear matrix protein (NXP) 2 antibody-positive juvenile dermatomyositis (JDM).Methods:A retrospective cohort study was conducted, including 219 anti-NXP2 antibody-positive JDM patients admitted to 23 children′s hospitals across China from July 2011 to July 2023. Patients were classified into severe and non-severe groups based on classification criteria for severe dermatomyositis. Demographic characteristics, clinical manifestations, and laboratory parameters were compared between the 2 groups using independent sample t-test, Mann-Whitney U test, or χ2 test. Univariate and multivariate Logistic regression analyses were performed to identify risk factors for severe disease. The receiver operating characteristic curve was employed to calculate optimal cut-off values. Results:Among the 219 patients, 108 were male and 111 were female, with an age at onset of 6.3 (3.5, 9.4) years. The severe group comprised 69 patients, and the non-severe group 150 patients. The severe group had significantly higher rates of fever, heliotrope rash, subcutaneous edema, periorbital edema, anti-Ro52 antibody positivity, as well as elevated levels of ferritin-to-albumin ratio (FAR), creatine kinase (CK), aspartate aminotransferase (AST), and lactate dehydrogenase (LDH) (all P<0.05). Multivariate analysis identified anti-Ro52 antibody positivity ( OR=13.26, 95% CI 1.37-128.29) and elevated FAR ( OR=1.90, 95% CI 1.09-2.31) as independent risk factors for severe anti-NXP2 antibody-positive JDM (both P<0.05). Receiver operating characteristic curve analysis revealed that a FAR cutoff value of 6.82 predicted severe disease with an area under the curve of 0.87 (95% CI 0.81-0.94, P<0.001), sensitivity of 0.85, and specificity of 0.70. All patients received glucocorticoid therapy, and the severe group received higher proportions of steroid pulse therapy, cyclophosphamide, mycophenolate mofetil, intravenous immunoglobulin, biologics, and adjuvant treatments compared to the non-severe group (all P<0.05). In terms of outcomes, 2 patients (2.9%) in the severe group died (due to neurological involvement and intestinal perforation, respectively), while the remaining patients achieved complete clinical response or remission. All patients in the non-severe group achieved remission. Conclusions:The primary clinical features of anti-NXP2 antibody-positive JDM included fever, heliotrope rash, subcutaneous edema, periorbital edema, anti-Ro52 antibody positivity, and elevated levels of CK, AST, LDH, and FAR. Furthermore, anti-Ro52 antibody positivity and a FAR>6.82 were identified as independent risk factors.

Objective:Pediatric chronic sinusitis （CRS） is a common disease within the field of otolaryngology-head and neck surgery. Due to the immaturity of sinus development and immune competence in children, its etiology and pathophysiology are complex, and its clinical features and outcomes differ significantly from those in adult patients. Currently, there are issues in the diagnosis and treatment of pediatric CRS, particularly in areas such as antibiotic use and surgical interventions, owing to a lack of sufficient attention. In recognition of this, the Chinese Rhinopathy Research Cooperation Group developed this expert consensus based on a systematic review of the latest literatures from both domestic and international sources, with reference to the latest evidence-based medical evidence worldwide, and in combination with their own clinical experience. The consensus covers various aspects including epidemiology, predisposing factors, pathophysiology, diagnosis and differential diagnosis, as well as treatment strategies such as medical therapy and surgical intervention. It aims to standardize the clinical diagnosis and treatment of pediatric CRS, improve clinical efficacy and patient satisfaction, reduce clinical expenditures, and decrease the occurrence of adverse reactions.
Humans ; Sinusitis/therapy* ; Chronic Disease ; Child ; Consensus ; Anti-Bacterial Agents/therapeutic use*

Objective To explore the characteristics of response to joint attention(RJA)under diverse guiding behaviors for pre-schoolers with moderate to severe autism spectrum disorder(ASD). Methods From March to May,2023,21 children with moderate to severe ASD and 16 children with developmental de-lays matched the physiological ages were selected from Jiaxing Sunlight Rehabilitation Kindergarten,and 16 typ-ical developmental children matched the physiological ages were selected from the kindergartens nearby.They accepted a behavioral experiment on RJA.The number of RJA,frequence of RJA and the coefficient of variation of guiding behaviors needed to RJA were compared among the three groups. Results About half of the ASD group responded after guiding of head-turning,and the others required higher levels of guiding.The frequence of RJA after guiding of head-turning was less in the ASD group than in the typical devel-opment group(χ2>6.170,P<0.05),and the coefficient of variation of guiding behaviors was more(d=4.039,P<0.001). Conclusion Preschoolers with moderate to severe ASD are able to respond to joint attention,and this ability is poorer than typically developing children.The guiding behavior of the evaluator should be considered in assessing and intervening RJA in preschoolers with ASD.