Objective To develop a risk prediction model for postoperative malnutrition in children with congenital heart disease(CHD)and to verify it both internally and externally.Methods By a convenience sampling method,300 CHD children treated at a tertiary hospital in Shandong Province from January 2018 to December 2021 were selected as a modeling group,and 129 children from January 2022 to June 2023 were selected as a validation group.Data on patient demographics,disease-specific variables,therapeutic interventions,and nursing care parameters were collected.Single factor and logistic regression were employed to construct a risk prediction model for postoperative malnutrition in CHD children,and the nomogram was drawn and its prediction effect was evaluated.Results The incidence of postoperative malnutrition among CHD children was 33.10％.Logistic regression analysis revealed that risk factors for malnutrition in children included birth weight＜2.5 kg,preoperative malnutrition,negative fluid balance 1 week after surgery,and long duration of cardiopulmonary bypass(P＜0.05).The area under the receiver operating characteristic curve of the modeling group was 0.933;the sensitivity was 83.30％;the specificity was 90.90％.The Hosmer-Lemeshow test showed that x2=7.765(P=0.457).The AUC of the validation group was 0.918;the sensitivity was 87.20％;the specificity was 90.00％.The Hosmer-Lemeshow test showed that x2=4.947(P=0.763).Calibration curves for both groups indicated good calibration of the model,and the clinical decision curves demonstrated its practical clinical utility.Conclusion The risk prediction model developed in this study exhibits good predictive ability,which can provide a reference for medical staff to early identify high-risk infants for postoperative malnutrition following CHD surgery and to formulate targeted intervention measures.

Objective To establish the reference intervals for test indicators of thyroid function,namely thyroid stimulating hormone(TSH),free thyroxine(FT4),and free triiodothyronine(FT3),in the children aged 0 to 15 years old in Nanning,China.Methods A total of 1 289 healthy children aged 0 to 15 years old who attended the Guangxi International Zhuang Medicine Hospital Affiliated with Guangxi University of Chinese Medicine from October 2018 to August 2023 were selected.The concentrations in serum TSH,FT4,and FT3 were measured by chemiluminescent microparticle immunoassay(CMIA).According to the Clinical and Laboratory Standards Institute(CLSI)EP28-A3c guideline,the nonparametric percentile method was used to establish the reference intervals for TSH,FT4,and FT3 in the children aged 0 to 15 years old in Nanning area.Results The established reference intervals were as follows:TSH(male):0 to＜1 month:0.88-7.81 μIU/mL,1 month to 15 years:0.59-5.06 μIU/mL;TSH(female):0 to＜1 month:0.93-8.42μIU/mL,1 month to 15 years:0.60-4.30 μIU/mL.FT4(male):0 to＜1 month:0.99-1.92 pg/mL,1 month to 15 years:0.86-1.33 pg/mL;FT4(female):0 to＜1 month:1.05-2.06 pg/mL,1 month to 15 years:0.85-1.37 pg/mL;FT3:0 to＜1 month:2.16-4.24 pmol/L,1 month to＜11 years:2.75-4.49 pmol/L,11 to 15 years:2.45-4.34 pmol/L.Significant differences were observed among different gender and age groups for TSH,FT4,and FT3 levels(P＜0.05).Conclusion This study successfully established the refer-ence intervals of TSH,FT4,and FT3 in the children aged 0 to 15 years old in Nanning area,which were significantly different among various gender and age groups.

Objective:To explore the risk factors of catheter-related thrombosis（CRT）in femoral vein indwelling catheter for patients undergoing artificial liver therapy.Methods:The clinical data 1 060 patients who received non biological artificial liver therapy with femoral vein catheterization at the Artificial Liver Center of the First Affiliated Hospital，Zhejiang University School of Medicine from February 26，2019 to March 30，2022 were retrospectively analyzed. During the treatment，CRT occurred in femoral vein indwelling catheter in 574 cases（54.2%，CRT group）and did not occurred in remaining 486 cases（non-CRT group）. The influencing factors of CRT were analyzed with multiple Logistic regression.Results:Univariate analysis showed that gender，D-dimer before and after catheterization，international normalized ratio（INR），total bilirubin，catheterization location，and catheter retention time were significantly associated with the occurrence of CRT（all P<0.05）. Multiple Logistic regression analysis showed that male gender（ OR=1.627，95% CI 1.233-2.148， P<0.001），right femoral vein catheterization（ OR=2.101，95% CI 1.130-3.908， P=0.019），catheter retention time（ OR=1.039，95% CI 1.003-1.077， P=0.035），D-dimer >500 μg/L FEU after catheterization（ OR=1.697，95% CI 1.191-2.416， P=0.003）and INR≥1.5（ OR=1.343，95% CI 1.001-1.801， P=0.049）were independent risk factors for CRT in femoral vein indwelling catheter for artificial liver therapy. Conclusion:The incidence of CRT is high in patients undergoing artificial liver therapy with femoral vein catheterization. In clinical practice，medical staff should early identify high-risk populations for CRT and take timely preventive measures.

OBJECTIVE: To explore the clinical and genetic characteristics of two children with developmental delay. METHODS: Two children who had presented at the Children's Hospital Affiliated to Shandong University on August 18, 2021 were enrolled as the study subjects. Clinical and laboratory examination, chromosomal karyotyping and high-throughput sequencing were carried out for both children. RESULTS: Both children had a 46,XX karyotype. High-throughput sequencing showed that they have respectively carried a c.489delG (p.Q165Rfs*14) and a c.1157_1158delAT (p.Y386Cfs*22) frameshifting variant of the CTCF gene, both had a de novo origin and were unreported previously. CONCLUSION The CTCF gene variants probably underlay the development delay in the two children. Above discovery has enriched the mutational spectrum of the CTCF gene and has important implications for revealing the genotype-phenotype correlation for similar patients.
Child ; Humans ; Developmental Disabilities/genetics* ; High-Throughput Nucleotide Sequencing ; Intellectual Disability/genetics* ; Karyotyping ; Mutation

OBJECTIVE: To evaluate the effectiveness of robot-guided percutaneous fixation and decompression via small incision in treatment of advanced thoracolumbar metastases. METHODS: A clinical data of 57 patients with advanced thoracolumbar metastases admitted between June 2017 and January 2021 and met the selection criteria was retrospectively analyzed. Among them, 26 cases were treated with robot-guided percutaneous fixation and decompression via small incision (robot-guided group) and 31 cases with traditional open surgery (traditional group). There was no significant difference in gender, age, body mass index, lesion segment, primary tumor site, and preoperative Tokuhashi score, Tomita score, Spinal Instability Neoplastic Score (SINS), visual analogue scale (VAS) score, Oswestry disability index (ODI), Karnofsky score, and Frankel grading between groups ( P>0.05). The operation time, hospital stays, hospital expenses, intraoperative blood loss, postoperative drainage volume, duration of intensive care unit (ICU) stay, blood transfusion, complications, and survival time were compared. The pedicle screw placement accuracy was evaluated according to the Gertzbein-Robbins grading by CT within 4 days after operation. The pain, function, and quality of life were evaluated by VAS score, ODI, Karnofsky score, and Frankel grading. RESULTS: During operation, 257 and 316 screws were implanted in the robot-guided group and the traditional group, respectively; and there was no significant difference in pedicle screw placement accuracy between groups ( P>0.05). Compared with the traditional group, the operation time, hospital stays, duration of ICU stay were significantly shorter, and intraoperative blood loss and postoperative drainage volume were significantly lesser in the robot-guided group ( P<0.05). There was no significant difference in hospital expenses, blood transfusion rate, and complications between groups ( P>0.05). All patients were followed up 8-32 months (mean, 14 months). There was no significant difference in VAS scores between groups at 7 days after operation ( P>0.05), but the robot-guided group was superior to the traditional group at 1 and 3 months after operation ( P<0.05). The postoperative ODI change was significantly better in the robot-guided group than in the traditional group ( P<0.05), and there was no significant difference in the postoperative Karnofsky score change and Frankel grading change when compared to the traditional group ( P>0.05). Median overall survival time was 13 months [95% CI (10.858, 15.142) months] in the robot-guided group and 15 months [95% CI (13.349, 16.651) months] in the traditional group, with no significant difference between groups ( χ ²=0.561, P=0.454) . CONCLUSION Compared with traditional open surgery, the robot-guided percutaneous fixation and decompression via small incision can reduce operation time, hospital stays, intraoperative blood loss, blood transfusion, and complications in treatment of advanced thoracolumbar metastases.
Humans ; Blood Loss, Surgical ; Quality of Life ; Retrospective Studies ; Robotics ; Surgical Wound ; Decompression

OBJECTIVE: To analyze the clinical and genetic characteristics of three Chinese pedigrees affected with Citrullinemia type I (CTLN1). METHODS: Three children diagnosed at the Children's Hospital Affiliated to Shandong University from 2017 to 2020 were selected as the study subjects. Genomic DNA was extracted from peripheral blood samples of the probands and their parents. Next generation sequencing (NGS) was carried out to detect pathological variants of the probands. Sanger sequencing was used for validating the candidate variant among the pedigrees. RESULTS: The probands have respectively carried compound heterozygous variants of c.207_209delGGA and c.1168G>A, c.349G>A and c.364-1G>A, c.470G>A and c.970G>A of the ASS1 gene, which were respectively inherited from their parents. CONCLUSION The newly discovered c.207_209delGGA and c.364-1G>A variants have enriched the mutational spectrum of the ASS1 gene. And the mutation spectrum of Chinese CTLN1 patients is heterogeneous.
Child ; Humans ; Argininosuccinate Synthase/genetics* ; Citrullinemia/genetics* ; East Asian People ; Mutation ; Pedigree

Objective:To understand the epidemiology, clinical characteristics and associated risk factors of severe adenovirus(ADV)pneumonia in children, providing the basis for targeted prevention and treatment.Methods:Clinical features of children with ADV pneumonia at Children′s Hospital of Soochow University from January 2011 to December 2020 were retrospectively analyzed.According to the severity of the disease, cases were divided into severe ADV pneumonia group and common ADV pneumonia group.The epidemiological and clinical characteristics of two groups were compared, and risk factors for the occurrence of severe ADV pneumonia were analyzed.Results:A total of 1 158 patients with ADV pneumonia were enrolled, including severe ADV pneumonia 104 cases(8.98%) and ordinary ADV pneumonia 1 054 cases(91.02%).The median age of severe ADV pneumonia group was 1.17 (0.83, 2.73) years, which was significantly younger than that of common ADV pneumonia group 3.16 (1.50, 4.50) years( P<0.05), and 77.89% (81/104) of them were younger than 3 years old.The occurrence of severe ADV pneumonia was predominant in winter and spring, accounting for 71.15% (74/104).Cough was present in 89.42% (93/104) and fever in 99.01% (103/104) of the severe ADV pneumonia group.Compared with the common ADV pneumonia group, the severe ADV pneumonia group had a significantly longer febrile time[10(6, 14)d vs. 5(4, 7)d, P<0.05], significantly higher incidence of shortness of breath, wheezing, convulsions/coma[100% vs. 2.09%, 45.19% vs. 13.57%, 10.57% vs. 1.99%, P<0.05], and significantly higher incidences of emphysema, pleural effusion, bronchial signs, pulmonary solids, and atelectasis [21.15% vs. 2.09%, 5.77% vs. 0.19%, 4.81% vs. 0, 3.85% vs. 0.09%, P<0.05].Multivariable Logistic regression showed that age younger than 1.71 years old, wheezing, and the presence of underlying diseases (moderate to severe anaemia, congenital heart disease, neurological disease) were risk factors for the development of severe ADV pneumonia ( P<0.05).Receiver operating characteristic curve analysis showed that the sensitivity and specificity of age<1.71 years old(20 months old) for predicting the occurrence of severe ADV pneumonia were 65.4% and 71.5%, respectively. Conclusion:The age of most severe ADV pneumonia is less 3 years in Suzhou.It usually occurres in winter and spring, with fever, cough, shortness of breath, and wheezing as the main symptoms.Pulmonary manifestations such as pleural effusion, emphysema, pulmonary consolidation, and atelectasis may occur.The underlying disease, wheezing, and age of onset less than 1.71 years (20 months) old are independent risk factors for severe ADV pneumonia.

Objective:To explore the clinical characteristics and complementary tests of a Chinese patient with central mixed phenotype acute leukemia and improve the awareness and diagnosis of MPAL among clinicians.Methods:Retrospectively analyzed a patient's clinical feature, laboratory examination, diagnosis and literature review who had headache as the first symptom and finally diagnosed with MPAL.Results:A 59-year-old female presented with recurrent headaches and can not relieved, at last , was confirmed as mixed phenotype acute leukemia by flow cytometry of cerebrospinal fluid、immunophenotyping and molecular biology of bone marrow. Remission was achieved after chemotherapy for lymphoid and myeloid leukemia.Conclusion:Patients with an unclear diagnosis of central nervous system disease, leukemia should be considered, and cerebrospinal fluid flow cytology and bone marrow biopsy should be refined.

Objective : To investigate the role of Epac1 / CaMK Ⅱ signaling pathway in myocardial ischemia reper- fusion injury (MIRI) in mice，and to investigate the protective effect of vitexin ( VT) on acute MIRI． Methods: C57 / BL mice were randomly divided into 5 groups : Sham surgery group ( Sham) ，ischemia reperfusion group ( I / R) ，and ischemia reperfusion + vitexin group ( function 3，6，12 mg / kg groups) ．Ligation of the left anterior descending coronary artery (LAD coronary artery) in mice resulted in ischemia of part of the heart tissue for 30min and reperfusion of the blood for 120min．Mouse myocardial ischemia reperfusion injury ( MIRI) model was established．In the sham operation group，only the LAD was not ligated．Serum LDH levels of mice were detected．Hema- toxylin-eosin (H＆E) staining was performed on the left ventricular myocardium of mice to observe the histopatho- logical changes．The expression level of Epac1 in myocardial tissue was observed by immunohistochemistry．The protein expressions of Epac1，Rap1，CaMK Ⅱ and ERK / p-ERK were determined by Western Blot. Results : Compared with Sham group，serum LDH level of mice in I / R group was significantly increased，protein expressions of Epac1， Rap1 and CaMK Ⅱ in myocardial tissue were significantly up-regulated，and ERK1 /2 phosphorylation level was decreased．Compared with I / R group，vitexin (3，6，12 mg / kg) pretreatment group decreased serum LDH level，inhib- ited Epac1，Rap1 and CaMK Ⅱ protein expression in mouse myocardial tissue，and promoted ERK1 /2 phosphoryla- tion(P＜0. 05 or P＜0. 01) ．The histopathological results showed that the myocardial fibers in the I / R group were disordered and broken，with increased gaps and obvious inflammatory cell infiltration．In the vitexin treatment group，the myocardial fibers were arranged more neatly and inflammatory cells were infiltrated less. Conclusion Vitexin may regulate Epac1 / CaMK Ⅱ signaling pathway，down-regulate CaMK Ⅱ protein expression，increase ERK phosphorylation，and effectively reduce MIRI.

Objective:To systematically analyze and integrate the psychological experience of heart failure patients using mobile medical applications (App) for self-management.Methods:Qualitative research on the self-management experience of heart failure patients using mobile medical App was retrieved through computer on PubMed, Web of Science, Embase, Cochrane Library, CINAHL, PsycINFO, Australian Joanna Briggs Evidence-Based Health Care Center Database, China National Knowledge Infrastructure, WanFang Data, VIP, and China Biomedical Literature Database. The search period was from the establishment of the database to December 30, 2022. The quality of literature was evaluated in accordance with the quality evaluation criteria for qualitative research of the Joanna Briggs Institute Evidence-Based Health Care Center (2016) . The results were integrated using the aggregative integration method.Results:A total of 14 articles were included, 32 research results were extracted, and 7 new categories were summarized. Finally, three integrated results were obtained (positive experience of heart failure patients using mobile medical App, risks and challenges of heart failure patients using mobile medical App, and preferences and expectations of heart failure patients towards mobile medical App) .Conclusions:The mobile medical App provides new ideas for self-management of heart failure patients, and its effectiveness is recognized by most heart failure patients, who perceive significant benefits. Future research should enrich the functionality and participation forms of the App based on the personalized needs of patients and their families, explore remote health management intervention models at home, and promote the promotion and application of mobile medical App.