Objective:To analyze the clinical characteristics,diagnosis and treatment of pediatric myocardial infarction (MI) patients with coronary artery lesions (CAL) after Kawasaki disease (KD).Methods:Clinical data including baseline characteristics, KD and CAL information, clinical symptoms at MI onset, electrocardiogram (ECG) and imaging findings, MI treatment, and clinical outcomes of 41 MI patients with CAL after KD admitted to the Children′s Hospital of Fudan University from January 2017 to August 2024 were analyzed retrospectively.Results:(1) Demographic characteristics: a total of 41 patients were included (36 males and 5 females). The age at MI was 4.6 (2.3, 5.7) years, and time from KD onset to MI was 397 (50, 1 095) d. (2) Treatment of acute KD: only 15 patients (37%) received standard initial treatment within 10 days of KD onset with intravenous immunoglobulin 2 g/kg. The other 26 cases (63%) received non-standard treatment or no treatment. (3) Treatment of CAL before MI: the time from KD onset to CAL was 14 (10, 116) d, with CAL not identified before MI onset in 15 patients. Among the 26 cases diagnosed with CAL prior to MI, 9 cases received only single or dual antiplatelet drug, of which 7 cases received oral dipyridamole. The remaining 16 cases received antiplatelet drug combined with warfarin, but only 1 case achieved the target international standardized ratio of 1.5-2.5. Out of all 41 cases, only 1 case (2%) received standard antithrombotic treatment before MI onset. (4) Clinical symptoms of MI: at MI onset, 32 patients presented with different clinical symptoms, with typical MI symptoms such as chest tightness, chest pain, precordial discomfort in 18 cases, and cardiopulmonary arrest accompanied by syncope or convulsions in 10 cases. Other non-specific symptoms included abdominal pain, nausea, vomiting and pallor. Nine patients were asymptomatic and were found to have silent MI on follow-up. (5) ECG and imaging findings: ECG showed ST-T changes in 33 cases, and abnormal Q waves, and arrhythmias in the remaining patients; echocardiography indicated coronary artery aneurysm with thrombosis in 27 cases, reduced left ventricular ejection fraction in 18 cases, abnormal wall motion in 15 cases, and ventricular aneurysm in 3 cases. Thirty-seven patients underwent coronary angiography and (or) multi-slice spiral CT angiography, with 39 occluded vessels and 3 severe stenosis (≥75%), all of which were caused by giant aneurism with thrombus formation. (6) Treatment of MI: of the 32 patients with acute MI, 9 patients received successful cardiopulmonary resuscitation, 7 patients received intravenous thrombolysis, and 1 patient underwent percutaneous coronary balloon angioplasty. All of these patients received dual antiplatelet drugs and low-molecular-weight heparin at therapeutic doses following MI treatment. Sixteen patients received coronary artery bypass graft (CABG) treatment, all of which were successful. (7) Outcomes: the follow-up time was 994 (215, 1 832) d. Thirty-one patients showed improvement, 5 patients experienced disease progression or no change, 1 patient died, and 4 patients were lost to follow-up.Conclusions:MI in children with CAL after KD often occurs within 1 year after the onset of KD. MI can present with atypical clinical symptoms in children. CABG is the main treatment option in children severe CAL after KD who developed MI.

Objective:To summarize the clinical and genetic characteristics of pseudoachondroplasia and multiple epiphyseal dysplasia caused by COMP gene variants in pediatric patients.Methods:This retrospective study concluded 15 pediatric patients with COMP-related pseudoachondroplasia and multiple epiphyseal dysplasia at Shanghai Children′s Medical Center, Shanghai Jiao Tong University School of Medicine from July 2013 to August 2024. This paper analyzed clinical manifestations, laboratory findings and genetic testing.Results:This cohort comprised 15 pediatric patients (8 males and 7 females) with a diagnostic age of 5.3 (1.8,9.3) years. The major clinical presentations included abnormal gait (15/15), brachydactyly (11/15), genu varum (12/15), irregular metaphyseal changes (14/14) and epiphyseal dysplasia (14/14). Genetic analysis revealed 13 cases of pseudoachondroplasia and 2 multiple epiphyseal dysplasias cases associated with COMP gene variants. Fifteen variants were identified (8 pathogenic and 7 likely pathogenic), including 2 novel variants (c.1223A>G, c.1378G>C). Thirteen of these patients had variations clustered in exons 8-14 encoding the calmodulin-like domains, with c.1414_1419dupGACGAC emerging as a hotspot variant.Conclusions:COMP-related pseudoachondroplasia and multiple epiphyseal dysplasia predominantly manifest with gait abnormalities and skeletal deformities. COMP gene pathogenic variations were mainly located in calmodulin-like domains.

Objective:To assess the current status of geriatrics department development in public general hospitals at or above the second grade in Shandong province.Methods:A cross-sectional survey was conducted from October 27 to November 3, 2023 using a web-based electronic questionnaire to investigate the current status of geriatrics department development in all public general hospitals at or above the second grade across 16 prefecture-level cities in Shandong province.The survey included participation from medical department staff and managers of geriatric medicine departments.Results:Shandong province has 355 public general hospitals at or above the second grade, of which 337 completed the questionnaire.Among these 337 hospitals, 92.28%(311/337)have established geriatric departments, 83.09%(280/337)have set up geriatric clinics, 69.14%(233/337)have independent geriatric wards, and 71.51%(241/337)have implemented comprehensive geriatric assessments(CGA).Regarding the configuration of geriatric departments, 60.24%(203/337)of hospitals met the requirement of having at least 20 ward beds, 34.42%(116/337)met the doctor-to-bed ratio requirement of at least 0.3, and 22.26%(75/337)met the nurse-to-bed ratio requirement of at least 0.6.Only 13 hospitals met all the requirements for geriatric wards, beds, doctors, and nurses, accounting for 3.86%(13/337)of the participating hospitals.Conclusions:The establishment of geriatrics departments in second-grade or higher public general hospitals in Shandong province has surpassed the national target in China ahead of schedule.Most hospitals have established geriatric clinics and wards and have implemented CGA.However, significant challenges remain, including a shortage of ward beds and a lack of medical staff.

Objective:To explore the effect of CO2 fractional laser combined with glucocorticoid(GC)on T follicular helper cell(Tfh)and B cell subsets in patients with advanced vitiligo(VL).Methods:Patients with advanced VL(128 cases)who were treated in Affiliated Hospital of Hebei University of Engineering from August 2019 to March 2022 were selected as research objects,and divided into group A(64 cases,CO2 fractional laser combined with GC)and group B(64 cases,CO2 fractional laser)according to different treatment methods,clinical efficacy and safety of the two groups were compared.Another healthy subjects(64 cases)were selected as control group.Collected clinical data of the three groups of subjects for comparative analysis.Randomized walking model was used to evaluate the effect of CO2 fractional laser combined with GC on immune function and inflammatory response in patients with advanced VL.Results:Compared with group B,group A had a higher clinical total effective rate(P<0.05)and a lower probability of adverse reactions(P<0.05).After treatment,compared with control group,proportion of Tfh2 and Tfh17 in group A had no significant difference(P>0.05),while proportion of Tfh2 and Tfh17 in group B still had significant difference(P<0.05);compared with group B,proportion of Tfh2 in group A after treatment was higher(P<0.05),while proportion of Tfh17 type was lower(P<0.05).After treat-ment,compared with control group,proportion of memory transformed B cell in group B was still significantly different(P<0.05).After treatment,compared with control group,levels of IgA,IgE and IgM in group B were still significantly different(P<0.05);compared with group B,level of IgM in group A was higher after treatment(P<0.05).After treatment,compared with control group,levels of IL-21 and IL-1β in group B were still significantly different(P<0.05);compared with group B,level of IL-10 in group A after treatment was higher(P<0.05),while levels of IL-21 and IL-1β were lower(P<0.05).Evaluation results of random walking model showed that the improvement of immune function and inflammatory reaction in group A was better than that in group B.Conclusion:CO2 fractional laser combined with GC can improve the immune function and inflammatory reaction of patients with advanced VL better after treatment,and the probability of adverse reactions is lower.

Objective:To explore the effect of CO2 fractional laser combined with glucocorticoid(GC)on T follicular helper cell(Tfh)and B cell subsets in patients with advanced vitiligo(VL).Methods:Patients with advanced VL(128 cases)who were treated in Affiliated Hospital of Hebei University of Engineering from August 2019 to March 2022 were selected as research objects,and divided into group A(64 cases,CO2 fractional laser combined with GC)and group B(64 cases,CO2 fractional laser)according to different treatment methods,clinical efficacy and safety of the two groups were compared.Another healthy subjects(64 cases)were selected as control group.Collected clinical data of the three groups of subjects for comparative analysis.Randomized walking model was used to evaluate the effect of CO2 fractional laser combined with GC on immune function and inflammatory response in patients with advanced VL.Results:Compared with group B,group A had a higher clinical total effective rate(P<0.05)and a lower probability of adverse reactions(P<0.05).After treatment,compared with control group,proportion of Tfh2 and Tfh17 in group A had no significant difference(P>0.05),while proportion of Tfh2 and Tfh17 in group B still had significant difference(P<0.05);compared with group B,proportion of Tfh2 in group A after treatment was higher(P<0.05),while proportion of Tfh17 type was lower(P<0.05).After treat-ment,compared with control group,proportion of memory transformed B cell in group B was still significantly different(P<0.05).After treatment,compared with control group,levels of IgA,IgE and IgM in group B were still significantly different(P<0.05);compared with group B,level of IgM in group A was higher after treatment(P<0.05).After treatment,compared with control group,levels of IL-21 and IL-1β in group B were still significantly different(P<0.05);compared with group B,level of IL-10 in group A after treatment was higher(P<0.05),while levels of IL-21 and IL-1β were lower(P<0.05).Evaluation results of random walking model showed that the improvement of immune function and inflammatory reaction in group A was better than that in group B.Conclusion:CO2 fractional laser combined with GC can improve the immune function and inflammatory reaction of patients with advanced VL better after treatment,and the probability of adverse reactions is lower.

Objective:To assess the current status of geriatrics department development in public general hospitals at or above the second grade in Shandong province.Methods:A cross-sectional survey was conducted from October 27 to November 3, 2023 using a web-based electronic questionnaire to investigate the current status of geriatrics department development in all public general hospitals at or above the second grade across 16 prefecture-level cities in Shandong province.The survey included participation from medical department staff and managers of geriatric medicine departments.Results:Shandong province has 355 public general hospitals at or above the second grade, of which 337 completed the questionnaire.Among these 337 hospitals, 92.28%(311/337)have established geriatric departments, 83.09%(280/337)have set up geriatric clinics, 69.14%(233/337)have independent geriatric wards, and 71.51%(241/337)have implemented comprehensive geriatric assessments(CGA).Regarding the configuration of geriatric departments, 60.24%(203/337)of hospitals met the requirement of having at least 20 ward beds, 34.42%(116/337)met the doctor-to-bed ratio requirement of at least 0.3, and 22.26%(75/337)met the nurse-to-bed ratio requirement of at least 0.6.Only 13 hospitals met all the requirements for geriatric wards, beds, doctors, and nurses, accounting for 3.86%(13/337)of the participating hospitals.Conclusions:The establishment of geriatrics departments in second-grade or higher public general hospitals in Shandong province has surpassed the national target in China ahead of schedule.Most hospitals have established geriatric clinics and wards and have implemented CGA.However, significant challenges remain, including a shortage of ward beds and a lack of medical staff.

Objective:To analyze the clinical characteristics,diagnosis and treatment of pediatric myocardial infarction (MI) patients with coronary artery lesions (CAL) after Kawasaki disease (KD).Methods:Clinical data including baseline characteristics, KD and CAL information, clinical symptoms at MI onset, electrocardiogram (ECG) and imaging findings, MI treatment, and clinical outcomes of 41 MI patients with CAL after KD admitted to the Children′s Hospital of Fudan University from January 2017 to August 2024 were analyzed retrospectively.Results:(1) Demographic characteristics: a total of 41 patients were included (36 males and 5 females). The age at MI was 4.6 (2.3, 5.7) years, and time from KD onset to MI was 397 (50, 1 095) d. (2) Treatment of acute KD: only 15 patients (37%) received standard initial treatment within 10 days of KD onset with intravenous immunoglobulin 2 g/kg. The other 26 cases (63%) received non-standard treatment or no treatment. (3) Treatment of CAL before MI: the time from KD onset to CAL was 14 (10, 116) d, with CAL not identified before MI onset in 15 patients. Among the 26 cases diagnosed with CAL prior to MI, 9 cases received only single or dual antiplatelet drug, of which 7 cases received oral dipyridamole. The remaining 16 cases received antiplatelet drug combined with warfarin, but only 1 case achieved the target international standardized ratio of 1.5-2.5. Out of all 41 cases, only 1 case (2%) received standard antithrombotic treatment before MI onset. (4) Clinical symptoms of MI: at MI onset, 32 patients presented with different clinical symptoms, with typical MI symptoms such as chest tightness, chest pain, precordial discomfort in 18 cases, and cardiopulmonary arrest accompanied by syncope or convulsions in 10 cases. Other non-specific symptoms included abdominal pain, nausea, vomiting and pallor. Nine patients were asymptomatic and were found to have silent MI on follow-up. (5) ECG and imaging findings: ECG showed ST-T changes in 33 cases, and abnormal Q waves, and arrhythmias in the remaining patients; echocardiography indicated coronary artery aneurysm with thrombosis in 27 cases, reduced left ventricular ejection fraction in 18 cases, abnormal wall motion in 15 cases, and ventricular aneurysm in 3 cases. Thirty-seven patients underwent coronary angiography and (or) multi-slice spiral CT angiography, with 39 occluded vessels and 3 severe stenosis (≥75%), all of which were caused by giant aneurism with thrombus formation. (6) Treatment of MI: of the 32 patients with acute MI, 9 patients received successful cardiopulmonary resuscitation, 7 patients received intravenous thrombolysis, and 1 patient underwent percutaneous coronary balloon angioplasty. All of these patients received dual antiplatelet drugs and low-molecular-weight heparin at therapeutic doses following MI treatment. Sixteen patients received coronary artery bypass graft (CABG) treatment, all of which were successful. (7) Outcomes: the follow-up time was 994 (215, 1 832) d. Thirty-one patients showed improvement, 5 patients experienced disease progression or no change, 1 patient died, and 4 patients were lost to follow-up.Conclusions:MI in children with CAL after KD often occurs within 1 year after the onset of KD. MI can present with atypical clinical symptoms in children. CABG is the main treatment option in children severe CAL after KD who developed MI.

Objective:To summarize the clinical and genetic characteristics of pseudoachondroplasia and multiple epiphyseal dysplasia caused by COMP gene variants in pediatric patients.Methods:This retrospective study concluded 15 pediatric patients with COMP-related pseudoachondroplasia and multiple epiphyseal dysplasia at Shanghai Children′s Medical Center, Shanghai Jiao Tong University School of Medicine from July 2013 to August 2024. This paper analyzed clinical manifestations, laboratory findings and genetic testing.Results:This cohort comprised 15 pediatric patients (8 males and 7 females) with a diagnostic age of 5.3 (1.8,9.3) years. The major clinical presentations included abnormal gait (15/15), brachydactyly (11/15), genu varum (12/15), irregular metaphyseal changes (14/14) and epiphyseal dysplasia (14/14). Genetic analysis revealed 13 cases of pseudoachondroplasia and 2 multiple epiphyseal dysplasias cases associated with COMP gene variants. Fifteen variants were identified (8 pathogenic and 7 likely pathogenic), including 2 novel variants (c.1223A>G, c.1378G>C). Thirteen of these patients had variations clustered in exons 8-14 encoding the calmodulin-like domains, with c.1414_1419dupGACGAC emerging as a hotspot variant.Conclusions:COMP-related pseudoachondroplasia and multiple epiphyseal dysplasia predominantly manifest with gait abnormalities and skeletal deformities. COMP gene pathogenic variations were mainly located in calmodulin-like domains.

Objective:To investigate the value of systemic inflammatory response syndrome (SIRS), pediatric sequential organ failure assessment (pSOFA) and pediatric critical illness score (PCIS) in predicting mortality of pediatric sepsis in pediatric intensive care units (PICU) from Southwest China.Methods:This was a prospective multicenter observational study. A total of 447 children with sepsis admitted to 12 PICU in Southwest China from April 2022 to March 2023 were enrolled. Based on the prognosis, the patients were divided into survival group and non-survival group. The physiological parameters of SIRS, pSOFA and PCIS were recorded and scored within 24 h after PICU admission. The general clinical data and some laboratory results were recorded. The area under the curve (AUC) of the receiver operating characteristic curve was used to compare the predictive value of SIRS, pSOFA and PCIS in mortality of pediatric sepsis.Results:Amongst 447 children with sepsis, 260 patients were male and 187 patients were female, aged 2.5 (0.8, 7.0) years, 405 patients were in the survival group and 42 patients were in the non-survival group. 418 patients (93.5%) met the criteria of SIRS, and 440 patients (98.4%) met the criteria of pSOFA≥2. There was no significant difference in the number of items meeting the SIRS criteria between the survival group and the non-survival group (3(2, 4) vs. 3(3, 4) points, Z=1.30, P=0.192). The pSOFA score of the non-survival group was significantly higher than that of the survival group (9(6, 12) vs. 4(3, 7) points, Z=6.56, P<0.001), and the PCIS score was significantly lower than that of the survival group (72(68, 81) vs. 82(76, 88) points, Z=5.90, P<0.001). The predictive value of pSOFA (AUC=0.82) and PCIS (AUC=0.78) for sepsis mortality was significantly higher than that of SIRS (AUC=0.56) ( Z=6.59, 4.23, both P<0.001). There was no significant difference between pSOFA and PCIS ( Z=1.35, P=0.176). Platelet count, procalcitonin, lactic acid, albumin, creatinine, total bilirubin, activated partial thromboplastin time, prothrombin time and international normalized ratio were all able to predict mortality of sepsis to a certain degree (AUC=0.64, 0.68, 0.80, 0.64, 0.68, 0.60, 0.77, 0.75, 0.76, all P<0.05). Conclusion:Compared with SIRS, both pSOFA and PCIS had better predictive value in the mortality of pediatric sepsis in PICU.

Objective:To analyze the distribution of clopidogrel metabolism-related gene variability in Kawasaki disease (KD) children with coronary artery lesions (CAL) across different age groups and the impact of genetic variability on the efficacy of clopidogrel antiplatelet therapy.Methods:A retrospective cohort study was conducted. Clinical data were collected from 46 KD children with CAL who were hospitalized in the Cardiovascular Center of Children′s Hospital of Fudan University between January 2021 and August 2022 and were treated with clopidogrel, including gender, age, body mass index, course of KD, CAL severity grade, and baseline platelet count. According to their age, the children were divided into ≥2-year-old group and <2-year-old group. Their platelet responsiveness was assessed by adenosine diphosphate-induced platelet inhibition rate (ADPi) calculated via thromboelastography, and children were categorized into high on-treatment platelet reactivity (HTPR) and normal on-treatment platelet reactivity (NTPR) groups. Genotypes of CYP2C19, PON1 and ABCB1 were detected. The t test, one-way analysis of variance and Chi-square test were used for intergroup comparison. Results:Among the 46 KD children with CAL, 34 were male and 12 were female; 37 were ≥2-year-old and 9 were <2-year-old; 25 cases were in the HTPR group and 21 cases were in the NTPR group, with 19 HTPR and 18 NTPR in the ≥2-year-old group, and 6 HTPR and 3 NTPR in the <2-year-old group. Genetic analysis showed that 92 alleles among the 46 children, with frequencies of CYP2C19*1, CYP2C19*2, CYP2C19*3, CYP2C19*17, PON1 192Q, PON1 192R, ABCB1 3435C, ABCB1 3435T at 59% (54/92), 32% (29/92), 9% (8/92), 1% (1/92), 36% (36/92), 64% (59/92), 63% (58/92) and 37% (34/92), respectively. Analysis of the impact of genotype on ADPi revealed that in children aged ≥2 years, those with CYP2C19*1/*3 genotype had significantly lower ADPi than those with CYP2C19*1/*1 genotype ((34±15)% vs. (61±29)%, t=2.18, P=0.036). There were also no significant difference in ADPi among children with PON1 192Q homozygous, PON1 192R heterozygote and PON1 192R homozygous genotypes ((40±22)% vs. (52±33)% vs. (65±27)%, F=2.17, P=0.130), or among those with ABCB1 3435C homozygous, ABCB1 3435T heterozygote and ABCB1 3435T homozygous genotypes ((55±34)% vs. (60±27)% vs. (49±24)%, F=0.33, P=0.719). In <2-year-old group, there were no significant differences in ADPi across CYP2C19*1/*1, CYP2C19*1/*2 and CYP2C19*2*2 genotypes ((40±20)% vs. (53±37)% vs. (34±16)%, F=0.37, P>0.05). There were no significant differences in ADPi across CYP2C19*1/*1 and CYP2C19*1/*3 genotypes ((44±27)% vs. (42±20)%, t=0.08, P>0.05). There were no significant differences in ADPi across PON1 192Q homozygous, PON1 192R heterozygote and PON1 192R homozygous genotypes (45% vs. (55±27)% vs. (24±5)%, F=1.83, P>0.05). There were no significant differences in ADPi across ABCB1 3435C homozygous, ABCB1 3435T heterozygote and ABCB1 3435T homozygous genotypes ((36±16)% vs. (50±35)% vs. 45%, F=0.29, P>0.05). The risk analysis of HTPR in different genotypes revealed that in children aged ≥2 years, carrying at least 1 or 2 loss-of-function alleles of CYP2C19 was a risk factor for HTPR ( OR=4.69, 10.00, 95% CI 1.11-19.83, 0.84-119.32, P=0.033, 0.046, respectively), and PON1 192R homozygosity and carrying at least one PON1 192R allele were protective factors against HTPR ( OR=0.08, 0.13, 95% CI 0.01-0.86, 0.01-1.19, P=0.019, 0.043, respectively). Conclusion:KD children aged ≥2 years carrying CYP2C19 loss-of-function alleles and PON1 192Q are more likely to develop HTPR.