BACKGROUND:The biomechanical analysis of scoliosis cases is limited,with only independent analysis focusing on the spine or lower limbs,thus lacking a comprehensive evaluation of the multidimensional body.As a result,it becomes challenging to reflect the movement relationship between the trunk and lower limbs during daily activities,which hinders comprehensive clinical treatment guidance. OBJECTIVE:To explore the relationship between different segments of the spine and the kinematics/kinetics of the lower limbs during gait activities by measuring spinal kinematics in scoliosis patients,to provide a comprehensive and multi-level assessment of the biomechanical differences between scoliosis patients and the normal population,consequently offering evidence-based guidance for the prevention and treatment of scoliosis. METHODS:A cross-sectional study was conducted from July 2020 to June 2021 at the Rehabilitation Hospital Affiliated to Fujian University of Traditional Chinese Medicine in Fuzhou University City.A total of 28 scoliosis patients and 28 normal individuals in the same age group were included.Three-dimensional motion capture system was used to capture gait kinematic data at a sampling frequency of 100 Hz.Two force plates(AMTI 400600,sampling frequency 1 500 Hz)were embedded in a 10-meter-long 2.4-meter-wide level ground walkway(with an effective data collection length of 4 m)to collect kinetic data.The differences in spatial-temporal parameters,kinematics,and kinetics of gait between the two groups were compared.Immediately after inclusion,all subjects underwent full spinal X-ray measurements to compare the differences between the scoliosis and normal groups. RESULTS AND CONCLUSION:(1)Patients with scoliosis exhibited reduced relative rotational range of motion between the shoulder and trunk,as well as between the thorax and pelvis,compared to the normal group(P<0.05).However,the rotational range of motion in the pelvis was larger in patients with scoliosis compared to the normal group(P<0.05).(2)Patients with scoliosis showed decreased range of motion in the hip and knee joints,as well as reduced peak torque in hip joint flexion and extension,and lower peak values of ground reaction forces in the concave and convex directions,in comparison to the normal group(P<0.05).(3)Patients with scoliosis demonstrated greater asymmetry indices in knee joint range of motion,relative rotational range of motion between the shoulder and trunk,and between the thorax and pelvis,when compared to the normal group(P<0.05).(4)These findings illustrate a rigid movement pattern among the shoulder,thorax,and pelvis in patients with scoliosis during level walking.There is a reduction in range of motion in the hip and knee joints,as well as decreased peak torque values in hip joint flexion and extension,and ground reaction forces in the concave and convex directions.These characteristics can serve as foundational elements for assessing rehabilitation and developing treatment plans.

Objective:To revise the quality standards of purified siliceous earth in the Chinese Pharmacopoeia 2020 Vol Ⅳ.Methods:Referring to USP2024 and GB 14936-2012 food additive purified siliceous earth and related ref-erences,the current quality standards for pharmaceutical excipient purified siliceous earth were revised,including description,dissolved substances in water,dissolved substances in acid,and content determination.The soluble a-luminum content was also investigated.Results:Revised the description of characteristic items,methods for deter-mining dissolved substances in water and acid,and limits for content determination;Through the investigation of soluble aluminum before and after filtration of human serum albumin(HSA)samples using purified siliceous earth as a filter aid,it is shown that purified siliceous earth has a certain degree of adsorption effect on aluminum ele-ment.At present,the use of low-temperature ethanol ultrafiltration process does not increase the residual aluminum content in biological products,so an aluminum element determination item is not added to the standard.Conclusion:The comprehensive quality standards for purified siliceous earth will be included in the Chinese Phar-macopoeia 2025 edition,providing strong technical support for the quality and safety supervision of this product.

Lipoatrophic diabetes(LD) is a rare and distinct form of diabetes characterized by notable clinical heterogeneity. It is often considered one of the manifestations of lipodystrophy syndrome(LDS). In clinical practice, LD is frequently misdiagnosed as type 2 diabetes; however, its management protocols and prognostic outcomes differ significantly from those of other diabetes subtypes. Therefore, timely and accurate diagnosis is of great clinical importance. This paper presents two detailed case reports of female patients with LD. Through an in-depth analysis of their clinical features, it also provides an comprehensive review of the key clinical manifestations of LDS, potential pathogenic mechanisms, and current approaches to genetic diagnosis. The aim is to enhance clinicans′ awareness of LDS and improve corresponding diagnostic and therapeutic strategies.

Objective:To revise the quality standards of purified siliceous earth in the Chinese Pharmacopoeia 2020 Vol Ⅳ.Methods:Referring to USP2024 and GB 14936-2012 food additive purified siliceous earth and related ref-erences,the current quality standards for pharmaceutical excipient purified siliceous earth were revised,including description,dissolved substances in water,dissolved substances in acid,and content determination.The soluble a-luminum content was also investigated.Results:Revised the description of characteristic items,methods for deter-mining dissolved substances in water and acid,and limits for content determination;Through the investigation of soluble aluminum before and after filtration of human serum albumin(HSA)samples using purified siliceous earth as a filter aid,it is shown that purified siliceous earth has a certain degree of adsorption effect on aluminum ele-ment.At present,the use of low-temperature ethanol ultrafiltration process does not increase the residual aluminum content in biological products,so an aluminum element determination item is not added to the standard.Conclusion:The comprehensive quality standards for purified siliceous earth will be included in the Chinese Phar-macopoeia 2025 edition,providing strong technical support for the quality and safety supervision of this product.

Lipoatrophic diabetes(LD) is a rare and distinct form of diabetes characterized by notable clinical heterogeneity. It is often considered one of the manifestations of lipodystrophy syndrome(LDS). In clinical practice, LD is frequently misdiagnosed as type 2 diabetes; however, its management protocols and prognostic outcomes differ significantly from those of other diabetes subtypes. Therefore, timely and accurate diagnosis is of great clinical importance. This paper presents two detailed case reports of female patients with LD. Through an in-depth analysis of their clinical features, it also provides an comprehensive review of the key clinical manifestations of LDS, potential pathogenic mechanisms, and current approaches to genetic diagnosis. The aim is to enhance clinicans′ awareness of LDS and improve corresponding diagnostic and therapeutic strategies.

AIM: To evaluate the accuracy of the formulas, including Haigis, SRK/T, Holladay 1, and Holladay 2, in predicting the diopter of the intraocular lens implanted in high myopia cataract patients.METHODS: Prospective study. A total of 168 cases(168 eyes)of age-related cataract with an axial length(AL)≥26 mm who were treated in our hospital from August 2017 to November 2021 were selected. According to the preoperative AL measured by IOL Master 700, the patients were divided into five groups, including 37 cases(37 eyes)in group A with 26 mm≤AL<27 mm, 34 cases(34 eyes)in group B with 27 mm≤AL<28 mm, 42 cases(42 eyes)in group C with 28 mm≤AL<29 mm, 28 cases(28 eyes)in group D with 29 mm≤AL<30 mm, and 27 patients(27 eyes)in group E with AL ≥ 30 mm. Subjective refraction was performed at 3 mo postoperatively, and the mean numerical error(MNE)and mean absolute error(MAE)of each formula for predicting diopters were calculated.RESULTS: The MNE and MAE of the Haigis and Holladay 2 formulas were relatively less in each group, and MNE and MAE did not significantly increase with the growth of the axial length. However, the MAE and MNE of the SRK/T and Holladay 1 formulas significantly increased with the growth of the axial length, with the MNE and MAE of the Holladay 1 formula increasing more significantly in groups C, D, and E.CONCLUSION: For patients with age-related cataract, with an axial length of ≥26 mm, the accuracy of predicting the diopter of the intraocular lens using the Haigis and Holladay 2 formulas were higher.

Objective:To explore the pathogenicity and genotype-phenotype correlation of a c. 158G>A variant of phenylalanine hydroxylase ( PAH) gene among patients with PAH deficiency. Methods:Thirty seven children diagnosed with PAH deficiency at the Obstetrics and Gynecology Hospital Affiliated to Nanjing Medical University between July 2016 and June 2021 were selected as the study subjects. Clinical data and results of genetic testing were retrospectively analyzed.Results:Among the 37 patients, mild hyperphenylalaninemia (HPA) was observed in 34 cases, two PAH variants (including c. 158G>A), which formed a compound heterozygous mutation genotype, were detected in 33 patients, and the remainder one was found to harbor three PAH variants, including homozygous c. 158G>A variants and a heterozygous c. 842+ 2T>A variant. Classical phenylketonuria (PKU) was observed in 3 patients, and three PAH variants were detected in each of them, including two with c. [158G>A, 842+ 2T>A]/c.728G>A and c. [158G>A, 842+ 2T>A]/c.611A>G, respectively, and one with c. [158G>A, c. 722G>A]/c.728G>A. The c. 158G>A variant has a minimal influence on the PAH activity and is associated with a mild HPA phenotype. The variant should thereby be classified as likely benign. Conclusion:When the c. 158G>A variant and other pathogenic variants are arranged in cis position, the ultimate phenotype will be determined by the pathogenicity of other variants.

Objective:To preliminarily investigate the effect of blue light on the biological activity of human skin keratinocytes, fibroblasts and melanocytes.Methods:Discarded foreskin tissues were collected from 10 healthy children aged from 3 to 12 years after circumcision surgery in the First Affiliated Hospital of Kunming Medical University from June 2021 to December 2021. After epidermis-dermis separation, selective culture was performed to isolate keratinocytes, fibroblasts, and melanocytes. According to the pre-experiment results, the above three types of cells were irradiated with 440 - 450 nm blue light at doses of 0, 5, 10, 20, 30, and 40 J/cm 2, and then continued to be cultured for 0, 6, 24, and 48 hours. Cell counting kit 8 (CCK8) assay was performed to evaluate cellular proliferative activity at each time point, enzyme-linked immunosorbent assay (ELISA) to detect levels of interleukin (IL) -18, IL-33, nerve growth factor (NGF), and granulocyte-macrophage colony-stimulating factor (GM-CSF) secreted by keratinocytes, as well as levels of IL-33 and keratinocyte growth factor (KGF) secreted by fibroblasts, NaOH lysis method to determine melanin synthesis rates in melanocytes, and Western blot analysis to determine the relative expression of tyrosinase (TYR), tyrosine-related protease 1 (TRP-1) and dopachrome isomerase (DCT) in melanocytes. Two-way analysis of variance was used to analyze group effects, time effects and interaction effects. Results:After irradiation with blue light, the cellular proliferative activity significantly differed among different doses of blue light irradiation groups and different time points in keratinocytes ( Ftime = 516.20, Fdose = 421.20, Finteraction = 25.05, all P < 0.003), fibroblasts ( Ftime = 129.30, Fdose = 477.80, Finteraction = 10.91, all P < 0.003), and melanocytes ( Ftime = 77.61, Fdose = 138.70, Finteraction = 3.50, all P < 0.003) ; immediately after irradiation, the proliferative activity of keratinocytes and fibroblasts was significantly lower in the 20 - 40 J/cm 2 blue light group than in the 0 J/cm 2 blue light group (all P < 0.003), and the proliferative activity of melanocytes was significantly higher in the 5 J/cm 2 blue light group than in the 0 J/cm 2 blue light group ( P < 0.003) ; the proliferative activity of the 3 types of cells showed decreasing trends with the increase of blue light irradiation doses and culture time. ELISA showed that the concentrations of IL-18, IL-33, NGF, and GM-CSF secreted by keratinocytes, as well as the concentrations of IL-33 and KGF secreted by fibroblasts, tended to increase with the increase of blue light irradiation doses and culture time. The melanin synthesis rates in melanocytes significantly differed among different doses of blue light irradiation groups and different time points ( Ftime = 833.50, Fdose = 249.40, Finteraction = 81.38, all P < 0.003) ; during 0 - 24 hours after blue light irradiation, the melanin synthesis rates tended to increase with the increase of blue light irradiation doses and time; during 24 - 48 hours, the melanin synthesis rates showed decreasing trends with the increase of blue light irradiation doses and culture time compared with that at 24 hours after irradiation; 24 hours after irradiation, the melanin synthesis rates were significantly higher in the 5, 10, 20, 30 and 40 J/cm 2 blue light groups (159.50% ± 10.88%, 218.76% ± 8.49%, 333.72% ± 7.72%, 393.29% ± 6.00%, 427.21% ± 8.39%, respectively) than in the 0 J/cm 2 blue light group (102.29% ± 6.57%, all P < 0.003). The relative expression of TYR ( Ftime = 67.94, Fdose = 28.99, Finteraction = 3.71, all P < 0.003), TRP-1 ( Ftime = 21.73, Fdose = 8.38, both P < 0.003) and DCT ( Ftime = 34.51, Fdose = 11.79, both P < 0.003) in melanocytes significantly differed among different doses of blue light irradiation groups and different time points, and tended to increase with the increase of blue light irradiation doses and culture time. Conclusion:Blue light irradiation at doses of 5 - 40 J/cm 2 could inhibit the proliferative activity of human skin keratinocytes, fibroblasts, and melanocytes, and the inhibitory effect tended to increase with the increase of blue light irradiation doses, except an enhancing effect on the proliferative activity of melanocytes observed immediately after irradiation with blue light at 5 J/cm 2; additionally, blue light irradiation at 5 - 40 J/cm 2 could enhance the expression of melanin synthesis-related enzymes in melanocytes, and increase the melanin synthesis rate in melanocytes over a short period of time.

Objective:To establish the cut-off value of tetradecenoyl carnitine (C14∶1)/dodecenoyl carnitine(C12∶1) based on non-derivatized tandem mass spectrometry (MS/MS), and to explore the application value of C14∶1/C12∶1 to screen newborns for very long chain acyl-CoA dehydrogenase deficiency (VLCADD), determining the best combination of indicators for screening VLCADD.Methods:This retrospective study included data from 17 newborns with VLCADD detected by MS/MS and confirmed by acyl-CoA dehydrogenase very long chain ( ACADVL) gene detection, and 423 507 newborns with normal MS/MS results. The data from these newborns were collected from January 2014 to December 2021 as the newborns received neonatal screening in Nanjing Neonatal Disease Screening Center and Suzhou Neonatal Disease Screening Center. All newborns were divided into 3 groups: all newborns group, full-term newborns group and normal-birth-weight newborns group, and the cut-off values of C14∶1/C12∶1 for VLCADD in these 3 groups were determined by their receiver operating characteristic (ROC) curves individually. With these results, a total of 5 interpretation schemes were composed using different indicators alone or jointly: scheme 1 being C14∶1/C12∶1, scheme 2 being C14∶1, scheme 3 being C14∶1+C14∶1/C2+C14∶1/C16, scheme 4 being C14∶1/C12∶1+C14∶1, and scheme 5 being C14∶1/C12∶1+C14∶1+C14∶1/C2+C14∶1/C16. The detection rate, false-positive rate and positive predictive value of each scheme were calculated, and their screening efficiencies were statistically compared by Chi-square tests. Results:The cut-off values of C14∶1/C12∶1 for VLCADD in the 3 newborn groups were all 2.80. The detection rates of VLCADD with all 5 interpretation schemes were 17/17. Scheme 1 had the highest false positive rate [26.15‰ (11 075/423 524)] and the lowest positive predictive value [0.15% (17/11 092)]. Scheme 4 (Scheme 5) had the lowest false positive rate [0.02‰ (10/423 524)] and the highest positive predictive value [62.96% (17/27)]. Comparing scheme 4 (Scheme 5) with scheme 1, scheme 2 and scheme 3, the differences of false positive rate (χ2=302.30,11 191.50,32.06) and positive predictive value (χ2=102.51,3 485.61,13.83) were statistically significant (all P<0.001). Conclusion:C14∶1/C12∶1 was an effective auxiliary interpretive indicator for VLCADD in newborn screening, and the combination of C14∶1/C12∶1+C14∶1 was tested to be the best indicator for VLCADD screening based on non-derivatized tandem mass spectrometry.

The performance appraisal of tertiary public hospitals is key to their high-quality development. Since 2019, Children′s Hospital of Zhejiang University School of Medicine has taken the following measures to leverage performance appraisal. Namely promoting medical technology innovation to enhance the diagnosis and treatment capabilities of difficult and critical diseases; Reasonably setting a target system, improving the performance appraisal mechanism of the hospital; Improving operational efficiency, enhancing the sense of gain by children patients; Building a high-quality talent pool, promoting sustainable development, and effectively promoting high-quality development of hospitals. These measures can provide reference for promoting the high-quality development of hospitals.