Objective: To understand the trends in malnutrition among primary and middle school students of Han nationality in Hainan Province from 2005 to 2019, so as to provide a basis for improving nutrition intervention measures for children and adolescents. Methods: A sample of 32 949 Han nationality primary and middle school students aged 7-18 years old in Hainan Province were investigated in 2005, 2010, 2014 and 2019 based on national survey on student physical fitness and health. The Malnutrition Screening Standard of Schoolage Children and Adolescents was used to screen malnutrition. Statistical analysis was performed using the χ2 test and the χ2trend test. Results: In the four surveys conducted during 2005 to 2019, the prevalence of malnutrition among primary and middle school students were 22.12%, 18.80%, 15.89% and 9.56%, respectively, with an increase of -12.56% and an average annual increase of -5.82%. The decreasing trend of malnutrition by year was statistically significant (χ2trend=600.72, P<0.01), and the proportion of emaciation type was the highest (8.87%-20.15%). The detection rates of malnutrition among all students aged 7 to 18 showed a decreasing trend from 2005 to 2019 (χ2trend=56.44, 60.04, 61.48, 42.49, 51.81, 50.81, 72.86, 101.34, 86.38, 24.81, 17.72, 10.38, P<0.01). From 2005 to 2019, the detection rates of malnutrition in boys were higher than that of girls (in 4 surveys), and that in rural students from 2005 to 2014 of 3 surveys were higher than that in towns (χ2=92.07, 35.16, 25.29, 29.98; 64.35, 4.26, 6.32, P<0.05). Conclusions The malnutrition of Han nationality primary and middle school students aged 7-18 years in Hainan Province show a trend of improvement year by year from 2005 to 2019, despite the overall high detection rate. Wasting is the most common type of malnutrition. The epidemic of malnutrition varies by age, sex and areas. Further targeted measures should be taken to strengthen intervention in the diet of primary and middle school students, to improve the nutritional status of children and adolescents.

Frailty is a clinical syndrome characterized by the gradual decline of multiple organ func-tion and the emergence of vulnerable phenotype.Patients with frailty exhibit diminished resistance to stress,which often leads to unfavorable outcomes following surgery,including cognitive decline,prolonged hospital-ization,and increased rates of complications and mortality.Given that frailty is a dynamic and reversible process,it is recommended to implement appropriate optimization measures during the perioperative period in order to improve patient outcomes.Recent studies have revealed that abnormal energy metabolism plays a crucial role in the development and occurrence of frailty.This article will primarily focus on abnormalities in glucose metabolism,lipid metabolism,and amino acid metabolism among frail patients,and emphasize the significance of correcting these metabolic irregularities during the perioperative period in order to reduce complications and improving surgical safety.

OBJECTIVE: To explore the clinical and genetic characteristics of five children with Catecholaminergic polymorphic ventricular tachycardia (CPVT). METHODS: Five children with clinical manifestations consistent with CPVT admitted to the Department of Cardiology of Children's Hospital Affiliated to Zhengzhou University from November 2019 to November 2021 were selected as the study subjects. Their clinical data were collected. Potential variants were detected by whole exome sequencing, and Sanger sequencing was used to verify the candidate variants. All patients were treated with β-blocker propranolol and followed up. RESULTS: All patients had developed the disease during exercise and presented with syncope as the initial clinical manifestation. Electrocardiogram showed sinus bradycardia. The first onset age of the 5 patients were (10.4 ± 2.19) years, and the time of delayed diagnosis was (1.6 ± 2.19) years. All of the children were found to harbor de novo heterozygous missense variants of the RYR2 gene, including c.6916G>A (p.V2306I), c.527G>C (p.R176P), c.12271G>A (p.A4091T), c.506G>T (p.R169L) and c.6817G>A (p.G2273R). Among these, c.527G>C (p.R176P) and c.6817G>A (p.G2273R) were unreported previously. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the c.527G>C (p.R176P) was classified as a pathogenic variant (PS2+PM1+PM2_Supporting+PM5+PP3+PP4), and the c.6817G>A (p.G2273R) was classified as a likely pathogenic variant (PS2+PM2_Supporting+PP3+PP4). The symptoms of all children were significantly improved with the propranolol treatment, and none has developed syncope during the follow up. CONCLUSION Discovery of the c.527G>C (p.R176P) and c.6817G>A (p.G2273R) variants has expanded the mutational spectrum of the RYR2 gene. Genetic testing of CPVT patients can clarify the cause of the disease and provide a reference for their genetic counseling.
Child ; Humans ; Mutation ; Propranolol ; Ryanodine Receptor Calcium Release Channel/genetics* ; Syncope ; Tachycardia, Ventricular/diagnosis* ; United States

OBJECTIVE: To explore the clinical and genetic characteristics of eight children with Primary hypertrophic cardiomyopathy (HCM). METHODS: Eight children with HCM admitted to the Department of Cardiology of Henan Children's Hospital from January 2018 to December 2021 were selected as the study subjects. Clinical data of the children were collected. Whole exome sequencing was carried out on two children, and trio whole exome sequencing was carried out on the remainder 6 children. Sanger sequencing was used to verify the candidate variants in the children and their parents, and the pathogenicity of the variants was evaluated based on the guidelines from the American College of Medical Genetics and Genomics (ACMG). RESULTS: The patients had included 5 males and 3 females, with their ages ranging from 5 to 13 years old. The average age of diagnosis was (7.87 ± 4.8) years old, and the cardiac phenotype showed non-obstructive HCM in all of the patients. WES has identified variants of the MYH7 gene in 4 children, including c.2155C>T (p.Arg719Trp), c.1208G>A (p.Arg403Gln), c.1358G>A (p.Arg453His), and c.1498G>A (p.Glu500Lys). Based on the guidelines from the ACMG, the first 3 variants were classified as pathogenic, while c.1498G>A (p.Glu500Lys) was classified as likely pathogenic (PM1+PM2_Supporting+PM6+PP3), which was also unreported previously. The remaining four children had all harbored maternal variants, including MYL2: c.173G>A (p.Arg58Gln; classified as pathogenic), TPM1: c.574G>A (p.Glu192Lys) and ACTC1: c.301G>A (p.Glu101Lys)(both were classified as likely pathogenic), and MYBPC3: c.146T>G (p.Ile49Ser; classified as variant of uncertain significance). Seven children were treated with 0.5 ~ 3 mg/(kg·d) propranolol, and their symptoms had improved significantly. They were followed up until September 30, 2022 without further cardiac event. CONCLUSION Genetic testing can clarify the molecular basis for unexplained cardiomyopathy and provide a basis for clinical diagnosis and genetic counseling. Discovery of the c.1498G>A (p.Glu500Lys) variant has also expanded the spectrum of MYH7 gene mutations underlying HCM.
Female ; Male ; Humans ; Child ; Child, Preschool ; Adolescent ; Cytoskeletal Proteins ; Family ; Genetic Counseling ; Genetic Testing ; Cardiomyopathy, Hypertrophic/genetics*

【Objective】 To study the metabolism and morphology changes of platelets in vitro using traditional Chinese medicine named Qingkailing injection as the additive solution, and to explore the viability of Qingkailing in the extension of platelet storage. 【Methods】 Apheresis platelets, adding 1% final concentration of Qingkailing injection, were taken as experiment groups, and sampled on 1, 3, 5, 8, 10 and 14 days(6 time points)of storage. Apheresis platelets without adding Qingkailing injection were taken as the control, and sampled on 1, 3 and 5 days(3 time points)of storage. The platelet count, morphology scores, biochemical parameters, pH and response rate of hypotonic shock during agitated storage(22 ℃) were tested. 【Results】 1) No significant change in platelet count was noticed in both experimental group(within 14 days) and the control(within 5 days)(P>0.05). The MPV and PDW of both groups increased at any sampling time within 5 days(P<0.05). 2) The morphology score of experimental groups and the control all decreased within the storage period(P<0.05), but its decrease of the control was greater than that of the experimental groups, especially on day 8(P<0.05). 3)Glucose, lactate dehydrogenase, lactate, Na⁺, and K⁺ values increased or decreased in varying degrees(P<0.05), while Cl^- value stayed almost the same during 14 days(P>0.05). Glucose, lactate dehydrogenase, lactate and Na⁺ value changed significantly in the control within 5 days(P<0.05), while K⁺ and Cl^- value did not(P>0.05). Within 5-day storage, the glucose consumption, lactate dehydrogenase and lactate generation in the control were significantly greater than those in experimental groups(P<0.05), but the added value of Na⁺, K⁺ and Cl^- showed no significant difference(P>0.05). 4) pH value, relative to the baseline of day 1, decreased in both groups within 5 days, and its decreasing trend was significant in the control (P<0.05), but not in the experimental group(P>0.05). No significant difference was noticed in the response rate of hypotonic shock in experimental groups within 8 days, while significant decrease was noticed in the control within 5days(P<0.05). The response rate of hypotonic shock in experimental groups were significantly higher than that in the control on day 3 and day 5(P<0.05). 【Conclusion】 The comparison of apheresis platelets, stored in vitro, in terms of platelet count, morphology scores, biochemical parameters, pH and response rate of hypotonic shock showed that platelets, adding 1% final concentration of Qingkailing injection, could prolong the platelet storage to at least 10 days in vitro.

【Objective】 To study the feasibility of whole blood thromboelastogram (WB-TEG) and its correlative kit for plasma thromboelastogram (P-TEG) detection and the characteristics of P-TEG in healthy subjects. 【Methods】 17 healthy volunteers were detected by WB-TEG instrument and its correlative kit, and the results were compared with those by P-TEG. The P-TEG characteristics of 17 healthy volunteers were analyzed. Three groups (7 cases/group)of plasma samples with different platelet (Plt) count and the other three groups of plasma(7, 6 and 4 cases, respectively) with different fibrinogen(Fib) concentration were tested for P-TEG. The effects of Plt and Fib on P-TEG detection were observed. 【Results】 There was no significant difference in R and MA value (P>0.05)as WB-TEG was compared with P-TEG in healthy subjects, while in K(min) (1.71±0.47 vs 1.07±0.45), A(°) (66.1±5.41 vs 75.59±5.77), and CI value (0.9±1.8 vs 2.52±2.58)(all P <0.05). Various parameters of healthy individuals were basically within the range of 95% CI of WB-TEG, but there were significant diffferences in K, A and CI value(P<0.05). When Plt count (×10¹¹/L) was≥2.5 in plasma, the MA value of P-TEG was significantly extended than that of normal individuals(P<0.05); when Plt count (×10¹¹/L) was 6.0 ~12.0, the MA and CI value of P-TEG significantly decreased(P<0.05). When Fib(g/L) was 6.4~6.91 in plasma, the R and K value of P-TEG were prolonged, but A, MA and CI value all decreased(P <0.05); when Fib(g/L) <1, the A and MA value significantly decreased(P<0.05), and K and CI value could not be detected. 【Conclusion】 The WB-TEG and its correlative kit can be used in P-TEG detection, and corresponding reference values of TEG parameters should be established in combination with the conditions of laboratories.

Objective:To retrospectively analyze the clinical effects and complications of alcohol inactivation, irradiation inactivation, and liquid nitrogen inactivation in the treatment of femur osteosarcoma in children, in an attempt to provide a theoretical basis for clinical selection of in vitro inactivation methods of tumor bone segment. Methods:The clinical data of 93 children with femur osteosarcoma admitted to the Department of Bone and Soft Tissue, the Affi-liate Cancer Hospital of Zhengzhou University from January 2008 to December 2017 were retrospectively analyzed, and 40 children, including 21 males and 19 females, aged 8-18 (13.65±2.87) years, who were treated with in vitro inactivation and replantation of autogenous tumor bone segment, were screened.Among these children, there was alcohol inactivation in 15 cases, irradiation inactivation in 12 cases, and liquid nitrogen inactivation in 13 cases.A comparison was drawn on these 3 inactivation methods with respect to bone healing time, bone healing rate, tumor recurrence rate, infection rate, fracture or fixation failure rate, and revision rate. Results:All those 40 children were subject to valid medical followed-up, with the alcohol inactivation group for (102.60±16.55) months, the irradiation inactivation group for (59.33±6.39) months, and the liquid nitrogen inactivation for (36.85±6.49) months.The difference in follow-up time of 3 groups was statistically significant ( P<0.05). Compared with other 2 groups, the index of bone healing time, bone healing rate, infection rate and revision rate in the alcohol inactivation group were unfavorable, which showed a significant difference (all P<0.05); However, there was no significant difference in the recurrence rate, fracture rate or fixation failure rate compared with other 2 groups (all P>0.05); There was no significant difference in all above indexes between the irradiation group and the liquid nitrogen group (all P>0.05). Conclusions:Three in vitro inactivation methods for the treatment of tumor bone segment are safe and reliable.The alcohol inactivated bone has a long healing time and more complications.Both irradiation inactivation and liquid nitrogen inactivation are clinical options, but irradiation inactivation requires professional equipment, which may limit the clinical application.

Objective To investigate the clinical effect of irradiated-host bone ends' union after transplantation of pedicled vascularized fibular periosteum in the treatment of Children's tibia sarcoma.Methods From June,2016 to December,2016,there were 5 children of tibia sarcoma,which were 2 boys and 3 girls,aged of 9-15 years (mean,12 years).They were treated by the re-transplantation of extracorporal irradiated segmental autograft,and used ipsilateral pedicled vascularized fibular periosteum cover the ends of irradiated-host bone to shorten the bone union time of irradiated tibia autograft and prevent nonunion.Patients were 3 cases of osteosarcoma,1 of Ewing's sarcoma,and 1 of relapse of Langerhans's cell histocytosis in tibia.The length of resect bone was 14.0-20.0 cm (mean,17.2cm),constitute of 2 osteoarticular resections and 3 intercalary resections.The method of inactivation of bone segment was intraoperative extracorporal irradiation.Regular followed-up were done postoperative.The X-ray and CT were applied to observe the function of affect limb.The bone union time and complication were record.Results All patients were followed-up of 12-18 months (mean,14.2 months).Eight ends of irradiated-host bone in 5 patients healed completely in 7.8 (6-10) months postoperative.The region of ends were covered by periosteum and showed excellent osteogenic power.There was no leg length discrepancy occurred in patients who received intercalary inactivation because of the preservation of growth plate.But the other 2 osteoarticular inactivated patients suffered leg length discrepancy of 1.0 cm and 1.5 cm respectively because of the inactivation of growth plate.At the follow-up of 12 months post-operation,the mean MTSS of affect limb was 25.8(22-28),and the mean of MTSS％ was 86％(73％-93％).Conclusion Transplantation of pedicled vascularized fibular periosteum can promote effectively healing of irradiated tibia bone after replantation in Children,with simple operation and less complications.

Objective To study the effects of fibroblast growth factor 2 (FGF2) on the proliferation and gene expression profiles of rabbit articular chondrocytes in vitro after different time periods of stimulation.Methods The chondrocytes were isolated and cultured in vitro,and the 3rd generation cells were harvested.Cells were divided into three groups.In the group 1 (FGF2 short-time action group),chondrocytes were cultured in medium with FGF2 for one day,and then transferred to fresh culture medium without FGF2 and cultured for another 6 days.In the group 2 (FGF2 long-time action group),chondrocytes were cultured in medium with FGF2 for 7 days.In the Group 3 (control group),chondrocytes were cultured in culture medium without FGF2 for 7 days.After culture for 1,3,and 7 days,the proliferation of chondrocytes in the all groups was detected respectively.Following extraction of mRNA,the gene expressions of BMP2,BMP4,SOX9 and COL2A1 of the chondrocytes in the all groups were determined by quantitative real-time polymerase chain reaction (qRT-PCR).The content of type Ⅱ collagen was measured via immunofluorescence staining.Results Compared with the control group,FGF2 promoted the proliferation of chondrocytes in the short-and long-time action groups and there was no significant difference between the two FGF2-treated groups.The results of qRT-PCR indicated that different treatment induced different gene expression profile.Particularly,compared with the control group and the FGF2 long-time action group,the expression of BMP2,BMP4,SOX9 and COL2A1 in the short-time action group were significantly upregulated at the 7th day.Immunofluorescence intensity of type Ⅱ collagen in the group 1 was stronger than that in the control group and group 2.Conclusions Different administration of FGF2 for different time periods induced different responses of chondrocytes.Short-term FGF2 stimulation was more beneficial to maintain the phenotype of chondrocytes and the synthesis of extracellular matrix.

Objective To investigate the safety and efficacy of photoselective green laser vaporization of bladder tumor (PVBT). Methods A total of 522 patients with bladder tumor were enrolled in present study from January 2010 to May 2015, including 405 cases of non muscle-invasive bladder cancer (NMIBC) and 117 cases of muscle-invasive bladder cancer (MIBC). All of patients were treated with PVBT and intravesical instillation of epirubicin. Patients with MIBC received intravenous chemotherapy (kisi-hama and cisplatin). Results The hospitalization time was (7.32±1.28) days, the operation time was (27.08±5.36) min, and the indwelling urinary catheter was (2.42±0.34) days for patients in NMIBC group. During the follow-up period (12-60 months), 38 cases (9.4％) relapsed, of which 3 cases underwent radical cystectomy, and other 35 cases underwent PVBT again. All 405 patients were alive at the end of follow-ups. The hospitalization time was(26.18 ± 1.92) days, the operation time was (38.32 ± 6.54) min, and the time of indwelling urinary catheter was (2.72 ± 0.85) days for patients of MIBC group. During the follow-up period (12-60 months), 19 cases (16.2％) relapsed. Among them, 4 patients underwent radical cystectomy, and other 15 cases underwent PVBT. Six patients died from distant organ metastasis (including 2 cases of pulmonary metastasis and 4 cases of bone metastasis), and other 111 patients survived. Conclusion PVBT is safe and effective in the clinical application, especially for NMIBC and MIBC patients who are unable or unwilling to undergo radical cystectomy.