Objective:To investigate the clinical manifestations, pathological features, and genetic variant characteristics of children with glycogen storage disease type Ⅸa (GSD Ⅸa).Methods:A retrospective case series analysis was conducted to collected and analyzed the medical history, biochemical markers, liver ultrasound results, liver histopathological findings, genotypes, treatment regimens, and follow-up data of 4 pediatric patients diagnosed with GSD IXa in the Department of Infectious Diseases at Xiamen Children′s Hospital from January 2018 to May 2024. All patients were confirmed by genetic testing.Results:All 4 pediatric patients diagnosed with GSD Ⅸa were male. The ages of onset were 8 months, 2 years, 3 years and 3 months, 1 year and 5 months, respectively, with initial presentations including chronic diarrhea (Case 1), incidentally detected transaminase elevation during routine examinations (Cases 2 and 3), and delayed motor development (Case 4). Diagnosis was confirmed at ages 10 months, 3 years, 3 years 4 months and 1 year 6 months, respectively.At diagnosis, anthropometric parameters and biochemical profiles revealed:Height: 68 cm (< P3), 96 cm ( P25-50), 94 cm ( P3-10), and 94 cm ( P3-10).Weight: 7 kg (< P3), 17 kg ( P90-97), 14.4 kg ( P25-50), and 10.5 kg ( P25-50).Alanine aminotransferase: 299, 500, 271, and 313 U/L (reference range 0-40 U/L).Aspartate aminotransferase: 285, 543, 337 and 357 U/L (reference range 0-40 U/L).Fasting glucose: 2.80, 3.67, 2.98, and 3.66 mmol/L (reference range 3.90-6.10 mmol/L).Lactate: 4.3, 2.1, 1.3, and 2.6 mmol/L (reference range 0.5-2.2 mmol/L).Triglycerides: 5.22, 1.38, 1.32, and 1.88 mmol/L (reference range 0.56-1.70 mmol/L).Case 1 exhibited poor adherence to uncooked cornstarch therapy during initial treatment, with no significant improvement in biochemical parameters. Follow-up imaging at age 4 revealed hepatic adenoma. Subsequent improvement in therapeutic compliance led to biochemical normalization, reduced hepatic adenoma size, and growth parameters of 113 cm ( P10-25) and 26 kg ( P90-97) at 6 years 2 months. Cases 2-4 demonstrated biochemical improvement with regular uncooked cornstarch therapy and no evidence of hepatic adenoma.Liver histopathology in Cases 1-3 confirmed glycogen accumulation consistent with GSD, without cirrhotic changes. Genetic analysis identified PHKA2 variations in all cases: 2 missense variants, 1 frameshift variant and 1 nonsense variant. The c.2839dup and c.3267G>A variants represent novel pathogenic mutations. Conclusions:GSD Ⅸa in pediatric patients is predominantly characterized by hepatomegaly, hepatic dysfunction, and hypoglycemia. While uncooked cornstarch therapy typically yields favorable prognoses, a subset of patients may develop hepatic adenomas. Notably, children with hepatic adenoma exhibited younger age of onset, significant growth retardation, and more severe metabolic disturbances, suggesting that hepatic adenoma development may be closely linked to the severity of metabolic dysregulation.

Objective:To explore the clinical characteristics and risk factors for the progression of severe scrub typhus in children.Methods:Clinical data, laboratory and auxiliary examination results, treatment and prognosis data of children with scrub typhus admitted to Xiamen Children's Hospital from July 2014 to March 2024 were collected. The clinical characteristics of scrub typhus in children were analyzed, and the study subjects were divided into severe group and mild group according to their condition. The relevant risk factors for progression to severe cases were analyzed.Results:A total of 42 cases were included, with 34 cases (80.95%) in the mild group and 8 cases (19.05%) in the severe group. All patients(100%) had fever, with the highest incidence of eschar or ulceration (95.24%), followed by lymphadenopathy and splenomegaly. All 42 patients improved or recovered after treatment with azithromycin or doxycycline, with no deaths reported. Compared with the mild group，the severe group had a higher incidence of cough, lymphadenopathy，and hepatomegaly, with statistically significant differences( P<0.05). Compared with the mild group, the severe group showed a decrease in hemoglobin(Hb), platelet, and fibrinogen(Fib) levels, while serum C-reactive protein(CRP), procalcitonin, aspartate aminotransferase, creatine kinase MB, and lactate dehydrogenase (LDH) levels increased, with statistically significant differences( P<0.05). Multivariate Tobit model analysis showed that a decrease in Hb ( OR -0.006，95% CI -0.012-0.000， P<0.05), an increase in CRP ( OR 2.72，95% CI 0.001-0.008， P=0.010), an increase in LDH ( OR 1.76，95% CI 0.000-0.000， P=0.027), and a decrease in Fib ( OR -3.78，95% CI -0.309-0.093， P=0.001) were risk factors for scrub typhus progressing to severe cases. Conclusion:During the epidemic season of scrub typhus, fever accompanied by eschar or ulceration, along with splenomegaly and lymphadenopath aids in the diagnosis of scrub typhus. Decreased Hb and Fib, as well as elevated CRP and LDH, are risk factors for the development of severe scrub typhus in children.

Objective:To investigate the detection and clinical features of human rhinovirus（HRV）infection in children from the Xiamen area.Methods:A retrospective analysis was conducted on children treated at Xiamen Children's Hospital from November 2021 to October 2022.Thirteen types of multiplex respiratory pathogen detection kits were used to screen for 13 common respiratory pathogens. Clinical data of HRV-positive hospitalized children were collected.Results:（1）Among 8 420 children with acute respiratory infections，HRV had the highest detection rate at 20.40%（1 718/8 420），followed by HMPV（10.12%），H3N2（7.46%），HRSV（6.94%），and HPIV（6.59%）.HRV was detected throughout the year，with the highest proportion in May（18.42%）.（2）Out of 1 718 children with HRV infection，863 cases were hospitalized for pneumonia（50.23%，863/1 718）.The median age of hospitalized children was 2.58（1.07，4.20）years old，with 53.77% under 3 years old.（3）The main clinical manifestations of HRV pneumonia were cough（97.68%，843/863），fever（58.05%，501/863），runny nose（57.01%，492/863），nasal congestion（36.96%，319/863），and wheezing（24.33%，210/843）. The HRV pneumonia co-infection group showed statistically significant differences in fever and hospitalization days compared to the single HRV pneumonia infection group（all P<0.05）.（4）Compared to the common pneumonia group，the severe HRV pneumonia group showed statistically significant differences in fever，runny nose，wheezing and hospitalization days the CRP, PCT, LDH levels, and the number of antibiotic applications after hospitalization（all P<0.05）. Conclusion:HRV infection is detected year-round in Xiamen，pneumonia is common，with children under 3 years old being particularly susceptible.It is important to be alert to mixed infections or severe pneumonia.Clinical treatment should avoid unnecessary antibiotic use，actively and provide appropriate treatment.

Objective:To explore the clinical characteristics and risk factors for the progression of severe scrub typhus in children.Methods:Clinical data, laboratory and auxiliary examination results, treatment and prognosis data of children with scrub typhus admitted to Xiamen Children's Hospital from July 2014 to March 2024 were collected. The clinical characteristics of scrub typhus in children were analyzed, and the study subjects were divided into severe group and mild group according to their condition. The relevant risk factors for progression to severe cases were analyzed.Results:A total of 42 cases were included, with 34 cases (80.95%) in the mild group and 8 cases (19.05%) in the severe group. All patients(100%) had fever, with the highest incidence of eschar or ulceration (95.24%), followed by lymphadenopathy and splenomegaly. All 42 patients improved or recovered after treatment with azithromycin or doxycycline, with no deaths reported. Compared with the mild group，the severe group had a higher incidence of cough, lymphadenopathy，and hepatomegaly, with statistically significant differences( P<0.05). Compared with the mild group, the severe group showed a decrease in hemoglobin(Hb), platelet, and fibrinogen(Fib) levels, while serum C-reactive protein(CRP), procalcitonin, aspartate aminotransferase, creatine kinase MB, and lactate dehydrogenase (LDH) levels increased, with statistically significant differences( P<0.05). Multivariate Tobit model analysis showed that a decrease in Hb ( OR -0.006，95% CI -0.012-0.000， P<0.05), an increase in CRP ( OR 2.72，95% CI 0.001-0.008， P=0.010), an increase in LDH ( OR 1.76，95% CI 0.000-0.000， P=0.027), and a decrease in Fib ( OR -3.78，95% CI -0.309-0.093， P=0.001) were risk factors for scrub typhus progressing to severe cases. Conclusion:During the epidemic season of scrub typhus, fever accompanied by eschar or ulceration, along with splenomegaly and lymphadenopath aids in the diagnosis of scrub typhus. Decreased Hb and Fib, as well as elevated CRP and LDH, are risk factors for the development of severe scrub typhus in children.

Objective:To investigate the detection and clinical features of human rhinovirus（HRV）infection in children from the Xiamen area.Methods:A retrospective analysis was conducted on children treated at Xiamen Children's Hospital from November 2021 to October 2022.Thirteen types of multiplex respiratory pathogen detection kits were used to screen for 13 common respiratory pathogens. Clinical data of HRV-positive hospitalized children were collected.Results:（1）Among 8 420 children with acute respiratory infections，HRV had the highest detection rate at 20.40%（1 718/8 420），followed by HMPV（10.12%），H3N2（7.46%），HRSV（6.94%），and HPIV（6.59%）.HRV was detected throughout the year，with the highest proportion in May（18.42%）.（2）Out of 1 718 children with HRV infection，863 cases were hospitalized for pneumonia（50.23%，863/1 718）.The median age of hospitalized children was 2.58（1.07，4.20）years old，with 53.77% under 3 years old.（3）The main clinical manifestations of HRV pneumonia were cough（97.68%，843/863），fever（58.05%，501/863），runny nose（57.01%，492/863），nasal congestion（36.96%，319/863），and wheezing（24.33%，210/843）. The HRV pneumonia co-infection group showed statistically significant differences in fever and hospitalization days compared to the single HRV pneumonia infection group（all P<0.05）.（4）Compared to the common pneumonia group，the severe HRV pneumonia group showed statistically significant differences in fever，runny nose，wheezing and hospitalization days the CRP, PCT, LDH levels, and the number of antibiotic applications after hospitalization（all P<0.05）. Conclusion:HRV infection is detected year-round in Xiamen，pneumonia is common，with children under 3 years old being particularly susceptible.It is important to be alert to mixed infections or severe pneumonia.Clinical treatment should avoid unnecessary antibiotic use，actively and provide appropriate treatment.

Objective:To investigate the clinical manifestations, pathological features, and genetic variant characteristics of children with glycogen storage disease type Ⅸa (GSD Ⅸa).Methods:A retrospective case series analysis was conducted to collected and analyzed the medical history, biochemical markers, liver ultrasound results, liver histopathological findings, genotypes, treatment regimens, and follow-up data of 4 pediatric patients diagnosed with GSD IXa in the Department of Infectious Diseases at Xiamen Children′s Hospital from January 2018 to May 2024. All patients were confirmed by genetic testing.Results:All 4 pediatric patients diagnosed with GSD Ⅸa were male. The ages of onset were 8 months, 2 years, 3 years and 3 months, 1 year and 5 months, respectively, with initial presentations including chronic diarrhea (Case 1), incidentally detected transaminase elevation during routine examinations (Cases 2 and 3), and delayed motor development (Case 4). Diagnosis was confirmed at ages 10 months, 3 years, 3 years 4 months and 1 year 6 months, respectively.At diagnosis, anthropometric parameters and biochemical profiles revealed:Height: 68 cm (< P3), 96 cm ( P25-50), 94 cm ( P3-10), and 94 cm ( P3-10).Weight: 7 kg (< P3), 17 kg ( P90-97), 14.4 kg ( P25-50), and 10.5 kg ( P25-50).Alanine aminotransferase: 299, 500, 271, and 313 U/L (reference range 0-40 U/L).Aspartate aminotransferase: 285, 543, 337 and 357 U/L (reference range 0-40 U/L).Fasting glucose: 2.80, 3.67, 2.98, and 3.66 mmol/L (reference range 3.90-6.10 mmol/L).Lactate: 4.3, 2.1, 1.3, and 2.6 mmol/L (reference range 0.5-2.2 mmol/L).Triglycerides: 5.22, 1.38, 1.32, and 1.88 mmol/L (reference range 0.56-1.70 mmol/L).Case 1 exhibited poor adherence to uncooked cornstarch therapy during initial treatment, with no significant improvement in biochemical parameters. Follow-up imaging at age 4 revealed hepatic adenoma. Subsequent improvement in therapeutic compliance led to biochemical normalization, reduced hepatic adenoma size, and growth parameters of 113 cm ( P10-25) and 26 kg ( P90-97) at 6 years 2 months. Cases 2-4 demonstrated biochemical improvement with regular uncooked cornstarch therapy and no evidence of hepatic adenoma.Liver histopathology in Cases 1-3 confirmed glycogen accumulation consistent with GSD, without cirrhotic changes. Genetic analysis identified PHKA2 variations in all cases: 2 missense variants, 1 frameshift variant and 1 nonsense variant. The c.2839dup and c.3267G>A variants represent novel pathogenic mutations. Conclusions:GSD Ⅸa in pediatric patients is predominantly characterized by hepatomegaly, hepatic dysfunction, and hypoglycemia. While uncooked cornstarch therapy typically yields favorable prognoses, a subset of patients may develop hepatic adenomas. Notably, children with hepatic adenoma exhibited younger age of onset, significant growth retardation, and more severe metabolic disturbances, suggesting that hepatic adenoma development may be closely linked to the severity of metabolic dysregulation.

Objective:To construct a spatial radiomics model based on the spatial distribution characteristics of supratentorial pilocytic astrocytoma (PA) and ganglioglioma (GG) and to evaluate its differential diagnosis efficiency.Methods:The study was a cross-sectional study. A retrospective collection of 244 patients with episodic PA and GG who attended Beijing Tiantan Hospital of Capital Medical University (Center 1) from June 2016 to June 2022 and 116 patients with episodic PA and GG who attended General Hospital of Eastern Theater Command (Center 2) from March 2019 to October 2022 was performed. The patients in Center 1 were divided into a training set (171 patients) and a validation set (73 patients) in a 7∶3 ratio according to the random number table method, and the patients in Center 2 as a whole were regarded as test sets. All patients underwent MRI. Segmentation of tumor based on enhanced T 1WI and T 2WI images, alignment to standard space to generate a statistical parametric mapping of tumor locations and intergroup comparison was conducted. The Johns Hopkins University template was used to extract 189 tumor location features to construct a spatial model of tumor location; PyRadiomic 3.0.1 software was used to extract tumor radiomics features to construct a radiomics model; and the two models were fused to construct a spatial radiomics model. The efficacy of spatial radiomics model, spatial model, and radiomics model to discriminate PA from GG was analyzed using receiver operating characteristic curves and area under the curve (AUC). The generalization ability of the model was assessed by the difference in accuracy between the test sets and the validation sets (ΔACC). The clinical utility of the model was compared using clinical decision curves and calibration curves. Results:The statistical parametric mapping of lesions showed that supratentorial PA was vulnerable to medial structure areas such as suprasellar region, thalamus, basal ganglia and frontal lobe, temporal lobe, parietal lobe. GG was mainly distributed in bilateral temporal lobes, as well as frontal lobe, occipital lobe and parietal lobe. The AUCs of spatial radiomics model, radiomics model and spatial model to identify PA and GG in the test set were 0.876, 0.785, and 0.819, with accuracies of 77.59%, 72.41%, and 77.14%, respectively, and ΔACCs in the test set and validation set were 11.6%, 15.43%, and 6.94%, respectively. The clinical decision curves showed an overall greater clinical benefit of the spatial radiomics model compared with the conventional radiomics model and spatial model.Conclusion:Spatial radiomics model containing spatial information on lesion location can improve the diagnostic efficacy of supratentorial PA and GG, and enhance the generalization of the prediction model.

Objective To compare the effects of different crown materials on the stress distribution of dental implants by finite ele-ment method.Methods A three-dimensional finite element model of implant-supported crown restoration of the right mandibular first molar was established.Four kinds of crown restoration materials were used,including polymer infiltrated ceramic network Vita Enamic,resin nanoceramic Lava Ultimate,zirconia ceramic Cercon,and polyether-ether-ketone breCAM.A displacement load of 0.01 mm or 200 N was applied to the occlusal surface of the right mandibular first molar,and the analysis of reaction force,the maximum variability of shape,the maximum principal stress,the maximum equivalent stress was performed.Results The results of stress analysis under 0.01 mm displacement load showed that the maximum equivalent stress of Cercon was 937.30 MPa,and that of breCAM was 67.09 MPa.The stress concentration of crowns and implants was obvious.The maximum equivalent stress of the surrounding alveolar bone of Cercon was the highest(26.61 MPa),and that of breCAM was the lowest(3.87 MPa),which was about 1/7 of that of maximum group.The stress concentration range of resin-matrix ceramics and polyether-ether-ketone was relatively small,and the distribution was more uniform.When 200 N was applied,the maximum principal stress and the maximum equivalent stress distribution of the four models were similar,and the maximum variability of shape of breCAM was larger at the loading site.Conclusion Under the same displace-ment load,the stress concentration of polyether-ether-ketone and resin-matrix ceramics crown was lower than that of zirconia.There was no significant difference in stress concentration in the implant and surrounding alveolar bone among polyetheretherketone,resin-ceramic composite,and zirconia crown restorations under the same load.Further research on long-term durability of PEEK and resin-matrix ce-ramicsis needed through practical and clinical trials.

Sepsis is a life-threatening condition for patients. Biomarkers can be used for the diagnosis, treatment, and prognostic assessment of sepsis. In recent years, new biomarkers for sepsis have been discovered, and more than 250 biomarkers have been identified so far. The complexity of the sepsis process and the increased sensitivity of various detection techniques will lead to the emergence of new biomarkers. However, there is still a lack of specific diagnostic biomarkers and effective therapeutic approaches for sepsis in clinical practice. Therefore, the search for reliable biomarkers and the evaluation of the role of biomarkers in sepsis will undoubtedly aid in clinical decision-making. This article reviews the advances on research of sepsis biomarkers in order to improve understanding of current biomarkers of sepsis, and provide reference for the application of biomarkers in clinical diagnosis, treatment, and prognosis of sepsis.

Objective:To investigate the effectiveness, safety, and advantages of modified radical neck dissection by gasless unilateral axillary approach (GUA-MRND) in the surgical management of selected patients with papillary thyroid cancer.Methods:We retrospectively analyzed patients with papillary thyroid cancer who underwent GUA-MRND (endoscopic group, n=16) versus unilateral open modified radical neck dissection (MRND) (open group, n=32) during the period from Jan. 2019 to Jun. 2021, including the differences in surgical efficiency, complication rate, and incisional satisfaction.Results:Compared MRND with GUA-MRND, the patients were younger ( P<0.05) , operative time and postoperative drainage anterior ( P<0.01) were slightly inferior in the latter, but it had obvious advantages in cervical swallowing discomfort and incision satisfaction evaluation ( P<0.05) . There was no significant difference in the incidence of temporary recurrent laryngeal nerve injury, intraoperative and postoperative bleeding, hematoma, infection, lymphatic or chylous leakage and supraclavicular numbness after surgery ( P>0.05) . The number of dissected lymph nodes in area II in the GUA-MRND was lower ( P<0.05) , but it was significantly higher ( P<0.01) in area III. And the average regional cleaning efficiency in the GUA-MRND was level Ⅲ (35.5%) , level Ⅵ (28.59%) , level Ⅳ (23.21%) , level Ⅱ (7.18%) and level Ⅴ (7.12%) , suggested that GUA-MRND had higher efficacy for level III, level Ⅵ and Level IV. Conclusion:GUA-MRND is safe, effective, and has high cosmetic satisfaction in the treatment of selected patients with lateral cervical lymph node metastases from papillary thyroid cancer.