Objective:To investigate the clinical characteristics and independent risk factors of severe disease in patients with anti-nuclear matrix protein (NXP) 2 antibody-positive juvenile dermatomyositis (JDM).Methods:A retrospective cohort study was conducted, including 219 anti-NXP2 antibody-positive JDM patients admitted to 23 children′s hospitals across China from July 2011 to July 2023. Patients were classified into severe and non-severe groups based on classification criteria for severe dermatomyositis. Demographic characteristics, clinical manifestations, and laboratory parameters were compared between the 2 groups using independent sample t-test, Mann-Whitney U test, or χ2 test. Univariate and multivariate Logistic regression analyses were performed to identify risk factors for severe disease. The receiver operating characteristic curve was employed to calculate optimal cut-off values. Results:Among the 219 patients, 108 were male and 111 were female, with an age at onset of 6.3 (3.5, 9.4) years. The severe group comprised 69 patients, and the non-severe group 150 patients. The severe group had significantly higher rates of fever, heliotrope rash, subcutaneous edema, periorbital edema, anti-Ro52 antibody positivity, as well as elevated levels of ferritin-to-albumin ratio (FAR), creatine kinase (CK), aspartate aminotransferase (AST), and lactate dehydrogenase (LDH) (all P<0.05). Multivariate analysis identified anti-Ro52 antibody positivity ( OR=13.26, 95% CI 1.37-128.29) and elevated FAR ( OR=1.90, 95% CI 1.09-2.31) as independent risk factors for severe anti-NXP2 antibody-positive JDM (both P<0.05). Receiver operating characteristic curve analysis revealed that a FAR cutoff value of 6.82 predicted severe disease with an area under the curve of 0.87 (95% CI 0.81-0.94, P<0.001), sensitivity of 0.85, and specificity of 0.70. All patients received glucocorticoid therapy, and the severe group received higher proportions of steroid pulse therapy, cyclophosphamide, mycophenolate mofetil, intravenous immunoglobulin, biologics, and adjuvant treatments compared to the non-severe group (all P<0.05). In terms of outcomes, 2 patients (2.9%) in the severe group died (due to neurological involvement and intestinal perforation, respectively), while the remaining patients achieved complete clinical response or remission. All patients in the non-severe group achieved remission. Conclusions:The primary clinical features of anti-NXP2 antibody-positive JDM included fever, heliotrope rash, subcutaneous edema, periorbital edema, anti-Ro52 antibody positivity, and elevated levels of CK, AST, LDH, and FAR. Furthermore, anti-Ro52 antibody positivity and a FAR>6.82 were identified as independent risk factors.

AIM:To investigate the effects of high-dose methotrexate(MTX)on alkaline phosphatase(ALP)and the effects of ALP changes on bone me-tabolism,bone marrow granulogram function,liver function and excretion.METHODS:Aspartate ami-notransferase(AST),alanine aminotransferase(ALT)and albumin(ALB)were used as liver function indi-cators,serum calcium(Ca)and phosphorus(P)were used as bone metabolism indicators,neutro-phil(ANC)and white blood cell count(WBC)were used as bone marrow granuloline function indica-tors,and methotrexate C48h concentration ≥1 μmol/L was used as the excretion delay.One-way ANOVA analysis was performed on the ALP levels before and after the first chemotherapy and the second chemotherapy,and the children were divided into normal group and low group according to the ALP level,and the seven indexes before and after che-motherapy were quantitatively and qualitatively an-alyzed,and univariate and multivariate Logistic re-gression analysis was performed on the concentra-tion of methotrexate C48h and the above indexes in the children treated with the second chemothera-py.RESULTS:After the first chemotherapy and the second chemotherapy,ALP was significantly de-creased[(204.0±83.6)U/L vs.(172.8±67.3)U/L,(179.4±59.3)U/L vs.(169.6±57.1)U/L,all P＜0.05],and the serum Ca,P,ANC,WBC,and ALB were sig-nificantly decreased(P＜0.05),and AST and ALT were increased(P＜0.05),and ALT was an indepen-dent risk factor for delayed excretion(OR=1.049,95％CI 1.023-1.077,P＜0.001),ALB was an indepen-dent protective factor for delayed excretion(OR=0.551,95％CI 0.460-0.660,P＜0.001),and ALP was not a significant contributor to MTX excretion de-lay.CONCLUSION:ALP is not a good predictor of liv-er function and bone marrow granulopathy func-tion due to a significant decrease in ALP caused by high-dose MTX,and ALP together with serum calci-um and phosphorus levels can constitute an early warning indicator of bone metabolism disorders.

AIM:To investigate the effects of high-dose methotrexate(MTX)on alkaline phosphatase(ALP)and the effects of ALP changes on bone me-tabolism,bone marrow granulogram function,liver function and excretion.METHODS:Aspartate ami-notransferase(AST),alanine aminotransferase(ALT)and albumin(ALB)were used as liver function indi-cators,serum calcium(Ca)and phosphorus(P)were used as bone metabolism indicators,neutro-phil(ANC)and white blood cell count(WBC)were used as bone marrow granuloline function indica-tors,and methotrexate C48h concentration ≥1 μmol/L was used as the excretion delay.One-way ANOVA analysis was performed on the ALP levels before and after the first chemotherapy and the second chemotherapy,and the children were divided into normal group and low group according to the ALP level,and the seven indexes before and after che-motherapy were quantitatively and qualitatively an-alyzed,and univariate and multivariate Logistic re-gression analysis was performed on the concentra-tion of methotrexate C48h and the above indexes in the children treated with the second chemothera-py.RESULTS:After the first chemotherapy and the second chemotherapy,ALP was significantly de-creased[(204.0±83.6)U/L vs.(172.8±67.3)U/L,(179.4±59.3)U/L vs.(169.6±57.1)U/L,all P＜0.05],and the serum Ca,P,ANC,WBC,and ALB were sig-nificantly decreased(P＜0.05),and AST and ALT were increased(P＜0.05),and ALT was an indepen-dent risk factor for delayed excretion(OR=1.049,95％CI 1.023-1.077,P＜0.001),ALB was an indepen-dent protective factor for delayed excretion(OR=0.551,95％CI 0.460-0.660,P＜0.001),and ALP was not a significant contributor to MTX excretion de-lay.CONCLUSION:ALP is not a good predictor of liv-er function and bone marrow granulopathy func-tion due to a significant decrease in ALP caused by high-dose MTX,and ALP together with serum calci-um and phosphorus levels can constitute an early warning indicator of bone metabolism disorders.

Objective:To investigate the clinical characteristics and independent risk factors of severe disease in patients with anti-nuclear matrix protein (NXP) 2 antibody-positive juvenile dermatomyositis (JDM).Methods:A retrospective cohort study was conducted, including 219 anti-NXP2 antibody-positive JDM patients admitted to 23 children′s hospitals across China from July 2011 to July 2023. Patients were classified into severe and non-severe groups based on classification criteria for severe dermatomyositis. Demographic characteristics, clinical manifestations, and laboratory parameters were compared between the 2 groups using independent sample t-test, Mann-Whitney U test, or χ2 test. Univariate and multivariate Logistic regression analyses were performed to identify risk factors for severe disease. The receiver operating characteristic curve was employed to calculate optimal cut-off values. Results:Among the 219 patients, 108 were male and 111 were female, with an age at onset of 6.3 (3.5, 9.4) years. The severe group comprised 69 patients, and the non-severe group 150 patients. The severe group had significantly higher rates of fever, heliotrope rash, subcutaneous edema, periorbital edema, anti-Ro52 antibody positivity, as well as elevated levels of ferritin-to-albumin ratio (FAR), creatine kinase (CK), aspartate aminotransferase (AST), and lactate dehydrogenase (LDH) (all P<0.05). Multivariate analysis identified anti-Ro52 antibody positivity ( OR=13.26, 95% CI 1.37-128.29) and elevated FAR ( OR=1.90, 95% CI 1.09-2.31) as independent risk factors for severe anti-NXP2 antibody-positive JDM (both P<0.05). Receiver operating characteristic curve analysis revealed that a FAR cutoff value of 6.82 predicted severe disease with an area under the curve of 0.87 (95% CI 0.81-0.94, P<0.001), sensitivity of 0.85, and specificity of 0.70. All patients received glucocorticoid therapy, and the severe group received higher proportions of steroid pulse therapy, cyclophosphamide, mycophenolate mofetil, intravenous immunoglobulin, biologics, and adjuvant treatments compared to the non-severe group (all P<0.05). In terms of outcomes, 2 patients (2.9%) in the severe group died (due to neurological involvement and intestinal perforation, respectively), while the remaining patients achieved complete clinical response or remission. All patients in the non-severe group achieved remission. Conclusions:The primary clinical features of anti-NXP2 antibody-positive JDM included fever, heliotrope rash, subcutaneous edema, periorbital edema, anti-Ro52 antibody positivity, and elevated levels of CK, AST, LDH, and FAR. Furthermore, anti-Ro52 antibody positivity and a FAR>6.82 were identified as independent risk factors.

Objective:Four cases with NLRP3-related autoinflammatory diseases were reported to summarize the clinical characteristics, genotype, and treatment responses of the disease, and to improve clinical pediatricians' understanding of the disease.Methods:A retrospective analysis was performed on 4 cases with NLRP3-related autoinflammatory diseases diagnosed in Children's Hospital of Anhui Province in 2016—2021, and the clinical features and treatment progress of NLRP3-related autoinflammatory diseases were retrospectively analyzed based on the clinical features, gene reports, and literature review.Results:① All 4 cases were male. Cases 1, 2, and 3 had the disease onset after birth, and case 4 had the disease onset 6 months after birth. All showed periodic fever, repeated urticaria-like rash, protruding forehead, and saddle nose. White blood cells count, erythrocyte sedimentation rate, and C-reactive protein were increased during the attack period, and those in the interval period were normal, and antibiotic treatment was ineffective. ② The genetic test of all these 4 children showed NLRP3 mutation. Children 1, 2, and 3 were heterozygous mutations, and their parents were wild-type. The mutation was located at chromosome Chr1: 247587658, exon c913 (exon3). G>A, the 305th aspartic acid (Asp) of the protein was changed to asparagine (Asn) in child 1. The mutation was located at the chromosomal Chr1: 247588072, the nucleic acid was changed to c1327(exon3)T>C, and the amino acid was changed to p.Y443H in cases 2 and 3. Somatic heterozygous mutation was found in case 4, and the child's parents were wild-type. In this case, the mutation was located at chromosomal Chr1: 247587658, exon3 G>A, and the 305th Asp of the protein was changed to Asn. ③Children in cases 1, 2, and 3 were treated with glucocorticoids and non-steroidal anti-inflammatory drugs at the initial stage, but the effects were limited. After receiving IL-1 antagonist treatment fever, skin rash, joint swelling and pain disappeared, and the inflammatory indexes were returned to normal. The child 4 received non-steroidal anti-inflammatory drugs and methotrexate, but he failed to respond to the treatment. Treatment with tocilizumab was not effective, however, fever, skin rash, or joint pain disappeared after treated with Khanna.Conclusion:①NLRP3-related autoinflammatory diseases can cause periodic fever, urticaria, joint involvement, and severe involvement of the central nervous system and organ amyloidosis. Which are early misdiagnosis is prone to systemic juvenile idiopathic arthritis. ②The disease was an inflammatory disease mediated by interleukin-1. At present, non-steroidal anti-inflammatory drug, glucocorticoid and chronic anti-rheumatic drugs have limited effects. IL-1 antagonists are effective and safe in the treatment of the disease.

A 17-year-old male patient received rituximab due to nephrotic syndrome. No obvious discomfort occurred during the first treatment (500 mg by intravenous infusion). After half a year, when the second treatment (300 mg by intravenous infusion) was given, the patient developed symptoms such as fatigue of limbs and inability to lift both lower limbs. Laboratory test showed blood potassium 2.37 mmol/L. Before treatment, the serum potassium was 4.16 mmol/l, and other laboratory test indicators were basically normal. Acute hypokalemia caused by rituximab was considered. Oral potassium chloride was given. On the 2nd day, the symptoms in the patient were relieved, the feeling of powerlessness disappeared, and the blood potassium was 3.06 mmol/L. On the 3rd day, the blood potassium was 4.34 mmol/L. the potassium supplement was stopped after 3 days of medication. The next day, the blood potassium was 4.70 mmol/l, and the patient′s limb weakness and discomfort symptoms disappeared. After that, the patient did not use rituximab again, and no hypokalemia recurred.

A 17-year-old male patient received rituximab due to nephrotic syndrome. No obvious discomfort occurred during the first treatment (500 mg by intravenous infusion). After half a year, when the second treatment (300 mg by intravenous infusion) was given, the patient developed symptoms such as fatigue of limbs and inability to lift both lower limbs. Laboratory test showed blood potassium 2.37 mmol/L. Before treatment, the serum potassium was 4.16 mmol/l, and other laboratory test indicators were basically normal. Acute hypokalemia caused by rituximab was considered. Oral potassium chloride was given. On the 2nd day, the symptoms in the patient were relieved, the feeling of powerlessness disappeared, and the blood potassium was 3.06 mmol/L. On the 3rd day, the blood potassium was 4.34 mmol/L. the potassium supplement was stopped after 3 days of medication. The next day, the blood potassium was 4.70 mmol/l, and the patient′s limb weakness and discomfort symptoms disappeared. After that, the patient did not use rituximab again, and no hypokalemia recurred.

Objective? To investigate the effects of Ramsay sedation score on the nursing intervention for children undergoing continuous renal replacement therapy(CRRT). Methods? By retrospective non-random sampling, a total of 30 children treated with CRRT in Anhui Children＇s Hospital from January 2016 to May 2017 were assigned into the control group and nursed according to the standard operating procedures of CRRT for children. Another 30 children treated with CRRT from June 2017 to September 2018 were put into the observation group and the Ramsay sedation score was applied to adjust the sedation administration plan according to the results and doctors＇ prescriptions, and nursing intervention was given at the early stage of agitation. Machine alarm, complications and adverse events were compared between the two groups. Results? After nursing intervention with Ramsay sedation score, the incidence of complications including machine alarm (1 time), failed bloodletting (1 time), bleeding at catheter puncture point (4 cases) and unplanned accidental termination of treatment (1 time) in the observation group were lower than those in the control group with statistical significance (P＜0.05). Conclusions? Ramsay sedation score is simple and practical to apply and it is convenient for pediatric blood purification specialized nurses to evaluate its function. It can effectively reduce incidences of CRRT machine alarm, complications and adverse events.

Objective To compare the myocardial protective effects of post-treatment with sevoflurane and isoflurane on myocardial ischemia-reperfusion injury(MIRI) in adult rats.Methods Twenty-four adult male SD rats were divided into four groups (n =6) by using the random number table,control group (C),isehemia-reperfusion group (R),sevoflurane post-treatment (S) and isoflurane post-treatment group(I).The Langendorff isolated heart perfusion model was established.The heart rate(HR),left ventricular end-diastolic pressure(LLVEDP),left ventricular developed pressure(LVDP),maximum rate of rise of left ventricular pressure(LV+-dp/dtmax),and maximum rate of decrease of left ventricular pressure(LV-dp/dtmax) were recorded at the end of equilibrium perfusion,and at 30,90 min of reperfusion,respectively.At the end of infusion,1 mm3.of apical myocardial tissue was removed for observing mitochondrial structure under electron microscopy and scoring.The myocardial infarct size(MIS) in the remaining heart tissue was measured by TTC staining.Results Compared with the R group,the S and I groups showed improved cardiac function indicators,decreased MIS,and reduced mitochondrial damage after reperfusion(P＜0.05).Compared with the S group,the I group showed worse heart function,increased MIS,and more severe mitochondrial damage after reperfusion(P＜0.05).Conclusion Post-treatment with sevoflurane and isoflurane has a protective effect on MIRI in adult rats.Post-treatment with sevoflurane has a better cardioprotective effect than that with isoflurane.

Objective To compare the sedation and anti-inflammatory effects of dexmedetomidine and midazolam on critical ill children with multiple trauma. Methods A prospective randomized controlled trial was conducted. Sixty-five critical ill children with multiple trauma admitted to pediatric intensive care unit (PICU) of Anhui Province Children's Hospital from January 2014 to September 2016 were enrolled, who were randomly divided into dexmedetomidine group (33 cases) and midazolam group (32 cases). Children of both groups received sufentanil for analgesia. Children in dexmedetomidine group firstly received 1.0 μg/kg intravenous bolus of dexmedetomidine for 10 minutes, then continuous infusion of 0.2-0.7 μg·kg-1·h-1, while in midazolam group children received 1-5 μg·kg-1·min-1 of midazolam in continuous infusion. The goal of sedation was to maintain a Richmond agitation-sedation scale (RASS) score of -1 to 0. The level of serum interleukin (IL-6, IL-8, IL-10, IL-1β), tumor necrosis factor-α (TNF-α) and C-reactive protein (CRP) were detected by enzyme linked immunosorbent assay (ELISA) at 24, 48, 72 hours after treatment, and the duration of mechanical ventilation, ratio of continuous renal replacement therapy (CRRT), length of stay in the PICU, ratio of sepsis and multiple organ failure (MOF) and mortality were also recorded. Results Compared with midazolam, dexmedetomidine decreased the level of pro-inflammatory cytokines and increased the level of anti-inflammatory cytokines. At 24 hours after treatment, the levels of serum IL-1β, TNF-α significantly decreased and IL-10 significantly increased [IL-1β (ng/L):6.48±2.89 vs. 8.07±3.14, TNF-α (μg/L): 11.25±5.21 vs. 15.44±5.97, IL-10 (ng/L): 12.10±5.35 vs. 9.58±4.71, all P < 0.05]. At 48 hours after treatment, the levels of serum IL-6, IL-8, IL-1β, TNF-α and CRP significantly decreased and IL-10 significantly increased [IL-6 (ng/L): 209.67±80.49 vs. 336.31±123.94, IL-8 (ng/L):229.09±80.81 vs. 298.28±90.25, IL-1β (ng/L): 7.31±3.02 vs. 8.74±3.17, TNF-α (μg/L): 12.52±4.79 vs. 16.58±5.98, CRP (g/L): 47.82±24.92 vs. 72.35±31.71, IL-10 (ng/L): 12.90±5.42 vs. 10.01±4.79, all P < 0.05]. At 72 hours after treatment, the levels of serum IL-8 and CRP significantly decreased [IL-8 (ng/L): 234.64±96.24 vs. 290.28±103.97, CRP (g/L): 53.24±29.12 vs. 86.58±38.30, both P < 0.05]. Compared with midazolam, dexmedetomidine could significantly reduce the duration of mechanical ventilation (days: 4.7±1.3 vs. 6.6±2.1), length of PICU stay (days: 9.5±2.7 vs. 12.3±3.9, both P < 0.05), and the ratio of sepsis (33.3% vs. 53.1%, P < 0.05). But there were no significant differences in ratio of CRRT (18.2% vs. 18.8%), MOF (9.1% vs. 18.8%) and mortality (6.1% vs. 12.5%) between two groups (all P > 0.05). Conclusion Compared with midazolam, dexmedetomidine had better efficacy in the treatment of severe multiple trauma in children and reduce the level of inflammation.