Objective:To explore the clinical manifestations of IgG4-related diseases（IgG4-RD） complicated with moderately differentiated adenosquamous carcinoma of the parotid gland, the diagnostic criteria for IgG4-related diseases and parotid malignant tumors, treatment regimens, and the application of fine-needle aspiration in disease diagnosis, so as to reduce clinical misdiagnosis and missed diagnosis. Methods:A retrospective analysis was conducted on the case data of a patient with IgG4-related diseases（IgG4-RD） complicated with moderately differentiated adenosquamous carcinoma of the parotid gland admitted to our department in March 2024. The clinical characteristics, imaging findings, preoperative puncture results, and postoperative pathological features were analyzed, and relevant literatures on both diseases were reviewed and summarized. Results:The elderly male patient was admitted due to "a mass in the parotid area in front of the right ear for more than 3 months". Through clinical examination, imaging examination, laboratory examination, and preoperative needle biopsy, the diagnosis of "right parotid moderately differentiated adenosquamous carcinoma complicated with IgG4-related disease" was considered. It was also considered that IgG4-related disease did not involve other organs before surgery, so no systemic hormone therapy was given before or after surgery. After surgery combined with postoperative radiotherapy, follow-up showed that neither the parotid tumor nor IgG4-related disease recurred. Conclusion:"IgG4-related disease complicated with moderately differentiated adenosquamous carcinoma"is a rare clinical disease. Both lack typical clinical manifestations and specific imaging features, and the diagnosis is mostly unclear before surgery. Pathological examination is of great significance in the diagnosis of the disease, while fine-needle aspiration has limited value in the diagnosis, which should attract the attention of clinicians. In addition, for patients with both diseases, individualized treatment plans should be formulated.
Humans ; Parotid Neoplasms/pathology* ; Male ; Carcinoma, Adenosquamous/pathology* ; Immunoglobulin G4-Related Disease/complications* ; Parotid Gland/pathology* ; Retrospective Studies ; Aged ; Biopsy, Fine-Needle ; Immunoglobulin G

Acylcarnitines are metabolic intermediates of fatty acids and branched-chain amino acids having vital biofunctions and pathophysiological significances.Here,we developed a high-throughput method for quantifying hundreds of acylcarnitines in one run using ultrahigh performance liquid chromatography and tandem mass spectrometry(UPLC-MS/MS).This enabled simultaneous quantification of 1136 acyl-carnitines(C0-C26)within 10-min with good sensitivity(limit of detection＜0.7 fmol),linearity(cor-relation coefficient＞0.992),accuracy(relative error＜20％),precision(coefficient of variation(CV),CV＜15％),stability(CV＜15％),and inter-technician consistency(CV＜20％,n=6).We also established a quantitative structure-retention relationship(goodness of fit＞0.998)for predicting retention time(tR)of acylcarnitines with no standards and built a database of their multiple reaction monitoring parameters(tR,ion-pairs,and collision energy).Furthermore,we quantified 514 acylcarnitines in human plasma and urine,mouse kidney,liver,heart,lung,and muscle.This provides a rapid method for quantifying acyl-carnitines in multiple biological matrices.

Objective:To analyze the clinical characteristics and risk factors for mortality of severe pneumocystis carinii pneumonia(PCP)in pediatric liver transplant(LT)recipients.Methods:The data of severe PCP in LT recipients diagnosed at Shanghai Children′s Medical Center from November 2019 to February 2021 were collected.The clinical characteristics and risk factors for 28-day mortality were analyzed.Results:Fifteen patients were enrolled in the study.Thirteen cases survived and 2 cases were non-survived.There was no routine anti-pneumocystis prophylaxis after LT.The median age of onset of PCP was 12(7, 26)months.The median time after LT was 3.00(0.33, 4.00)months.The onset clustered in November-December and June-August.All patients were mechanically ventilated, and some patients were given prone ventilation(11 cases), neuromuscular blocking agents(13 cases)and high concentration oxygen(more than 60%, nine cases). Fourteen cases were complicated with other infections.Two cases were complicated with pneumothorax and subcutaneous/mediastinal emphysema.There were 2 cases with septic shock-like manifestation, 1 case of right heart insufficiency, 1 case of right heart failure(death), and 1 case of multiple organ failure(death). Compared with the survived group, the non-survived group had higher pediatric risk of mortality Ⅲ score[3.5(0.0, 6.0)vs.8.5(5.0, 12.0), Z=1.993, P=0.046] and lactate dehydrogenase level[1 731.5(1 012.0, 3 270.0)U/L vs.4 387.5(3 606.0, 5 169.0)U/L, Z=2.148, P=0.032]. Conclusion:PCP in pediatric LT is critical and complicated.Pediatric risk of mortality Ⅲ scores and lactate dehydrogenase increase in 28-day hospitalized deaths.

Objective:To investigate the clinical characteristics, treatment process and prognosis of children with severe side effects after chimeric antigen receptor T cell immunotherapy(CAR-T), so as to provide evidence for timely intervention after CAR-T treatment.Methods:From June 1, 2015 to May 31, 2020, children with cytokine release syndrome(CRS)or immune cell related neurotoxicity syndrome(ICANS)who were treated with CAR-T therapy in our hospital and revealed severe effects transferred to PICU were included in the study, and their clinical course and multiple laboratory examination data were systematically analyzed.Results:Seventeen children showed CRS reaction and entered PICU after CAR-T therapy.The most common clinical symptoms were respiratory distress(13 cases) and circulatory disorder(10 cases), of which 7 cases were complicated with severe ICANS.Serum interferon -γ(IFN-γ)and interleukin-6(IL-6)levels significantly increased after CAR-T cell infusion, reaching the peak at (5.1±1.6)days.The serum levels of IFN-γ and IL-6 in children with severe CRS were significantly higher than those in children with mild CRS(all P<0.05). The level of serum IL-6 in children with high tumor load was significantly higher than that in children with low tumor load( P<0.05). The mortality rate of children with elevated level of serum TNF-α was higher(5/5 vs.3/11, P<0.05). Children with severe CRS were more likely to develop grade 4 ICANS(4/4 vs.0/3, P<0.05). The mortality rate of children with oxygenation index(P/F value)<200 mmHg(1 mmHg=0.133 kPa) was higher(5/5 vs.2/12, P<0.05). The vasoactive inotropic score[ M( Min, Max)] in the death group was significantly higher than that in survival group[29.5(14.0, 50.0) vs.1.5(0, 25.0), Z=8.000, P=0.027]. Conclusion:Serum IL-6 and IFN-γ are crucial causes of CRS.High tumor load is one of the factors causing high level of serum inflammatory factors.Respiration and circulation systems are the most frequently involved systems.Therefore, the evaluation indexes of these two systems can help us judge the prognosis of children.

Objective:To explore the implementation of individualized lung protection ventilation strategy in pediatric acute respiratory distress syndrome(pARDS)guided by transthoracic electrical impedance tomography(EIT)and critical care ultrasound(CCU).Methods:We retrospectively analyzed the therapeutic process of protective ventilation strategy in one case of severe pARDS.EIT and CCU were used to guide the implementation of lung protective ventilation strategy.Results:EIT was used to guide lung recruitment and optimal positive end-expiratory pressure titration.CCU was used to assess hemodynamics and lung status of ARDS patient, and guide the implementation of right ventricular protective ventilation and circulatory protective ventilation.Finally, the patient eventually survived.Conclusion:The idea of ARDS protective ventilation has changed from traditional lung protective ventilation to right heart protective ventilation and circulatory protective ventilation, and finally achieved the protection of pulmonary vascular endothelium.EIT and CCU enrich the understanding of the pathophysiology and protective ventilation strategy in pARDS.

Objective:To investigate the immunity markers related to nosocomial infection in children with sepsis.Methods:A retrospective study including 155 cases diagnosed as sepsis from September 2015 to June 2020 in children′s intensive care unit (PICU) of Shanghai Children′s Medical Center was conducted. According to the presence of nosocomial infection occurred in PICU, septic children were divided into two groups: no nosocomial infection and nosocomial infection group. The differences about helper T-cells 1 and 2 cytokines, T cells subgroup absolute count, the proportion of CD14 + human leukocyte antigen DR (CD14 +HLA-DR), the proportion of regulatory T cells, pediatric risk of mortality Ⅲ (PRISM-Ⅲ), the treatment and outcome between the two groups were compared. Through propensity score matching (PSM), the disease severity and treatment of the two groups were matched to analyze the differences between the above indicators. Chi-square test or U test was used for comparison between groups. Receiver operating characteristic (ROC) curve was used to predict the occurrence of nosocomial infection. Results:There were 104 cases in no nosocomial infection group and 51 cases in nosocomial infection group. The first PICU-acquired infections occurred at (12±7) days after PICU admission. The most common PICU-acquired infections were pneumonia (26 cases, 51.0%) and bloodstream infections (15 cases, 29.4%). PRISM-Ⅲ of nosocomial infection group was significantly higher than that in no nosocomial infection group (8 (0-31) vs. 4 (0-17), Z=3 913.00, P<0.01).The proportion of using vasoactive drugs and invasive mechanical ventilation of nosocomial infection group was significantly higher (35.3% (18/51) vs. 10.6% (11/104), χ2=13.77, P<0.01; 86.3% (44/51) vs. 38.5% (40/104), χ2=31.51, P<0.01).The PICU length of stay of nosocomial infection group was significantly longer (20 (3-94) vs.7 (2-41) days, Z=4 585.50, P<0.01). The mortality of the nosocomial infection group was significantly higher than that of the group without nosocomial infection (29.4% (15/51) vs. 6.7% (7/104), χ2=14.45, P<0.01). Interleukin-6 and interleukin-10 of the nosocomial infection group were significantly higher than that in no nosocomial infection group (37.83 (2.23-7 209.99) vs. 13.45 (0.80~50 580.64) ng/L, Z=3 390.50, P=0.01; 10.42 (1.11-6 052.21) vs.4.10 (0.16-409.28) ng/L, Z=3 212.00, P=0.03). CD4 +/CD8 + and the percentage of CD14 +HLA-DR were significantly lower in the nosocomial infection group compared with the no nosocomial infection group (1.16 (0.44-4.96) vs. 1.61 (0.15-6.37), Z=1 955.00, P=0.01; 0.48 (0.08-0.99) vs. 0.67 (0.09-0.98), Z=1 915.50, P<0.01). After PSM, the percentage of CD14 +HLA-DR of nosocomial infection group was significantly lower than that in no nosocomial infection group (0.44 (0.08-0.99) vs. 0.64 (0.09-0.98), Z=758.00, P=0.02). The ROC curve analysis of the percentage of CD14 +HLA-DR in predicting nosocomial infection showed that the area under the curve was 0.642, the cut-off value was 0.39, and the 95% CI was 0.528-0.755. Conclusion:The level of the percentage of CD14 +HLA-DR maybe is related to the occurrence of nosocomial infection in children with sepsis.

Objective:To investigate the sedation weaning strategies in critically ill patients with mechanical ventilation in pediatric intensive care unit (PICU) and to explore the effect of different sedative weaning patterns on withdrawal syndrome.Methods:A single-center prospective cohort study was conducted from April 1, 2016 to April 30, 2017. One hundred and twelve patients who required mechanical ventilation and benzodiazepines and (or) opioids for at least 5 consecutive days in PICU of Shanghai Children's Medical Center were enrolled. Twenty patients (17.9%) had an intermittent weaning pattern, defined as a 50% or greater increase in daily benzodiazepine and (or) opioid dose after the start of weaning, and the remaining 92 cases (82.1%) had a steady weaning pattern. The demographic and clinical features, duration and dose of sedative and analgesics, and the incidence of withdrawal syndrome were evaluated. Mann-Whitney U test was used for comparison about clinical features between different weaning pattern groups and children with withdrawal syndrome or not. Logistic regression was used to explore the risk factors of withdrawal syndrome. Results:Among the 112 patients, 46 (41.1%) had withdrawal syndrome. The patients with the intermittent weaning pattern had a high score of pediatric risk of mortality Ⅲ (PRISM-Ⅲ) (10.0 (3.5, 12.0) vs. 6.0 (2.0, 10.0), U=654.50, P=0.043) and were prone to re-intubation (35.0% (7/20) vs. 7.6% (7/92), P=0.003). The patients with withdrawal syndrome had longer duration of sedation (19.5 (16.8, 24.3) vs. 10.0 (7.0, 17.3) days, U=743.50, P<0.01), higher incidence of intermittent weaning pattern (32.6% (15/46) vs. 7.6% (5/66),χ 2=11.58, P=0.001), longer PICU hospitalization (19.0 (15.8, 25.3) vs. 12.0 (8.8, 17.0) days, U=755.00, P<0.01) and higher cost (89 (57,109) vs. 53 (32, 79) thousand yuan, U=804.00, P<0.01). Logistic regression showed that intermittent weaning pattern (odds ratio ( OR) =4.85, 95 % confidence interval ( CI) 1.39-16.91, P=0.013), perioperative period of liver transplantation ( OR=6.97, 95 %CI 1.25-39.04, P=0.027) and a cumulative dose of midazolam ≥ 34.7 mg/kg ( OR=8.12, 95 %CI 3.09-21.37, P<0.01) were risk factors of withdrawal syndrome. Conclusions:Withdrawal syndrome is more likely to occur in children who are intermittently weaned from sedation. Steady weaning strategy may help prevent iatrogenic withdrawal syndrome.

Objective To explore the characteristics and value for predicting prognosis of cytokines in septic children with or without neutropenia.Methods Totally 138 septic children were divided into the neutropenia and non-neutropenia groups according to absolute neutropenic count.Septic children were divided into the shock and non-shock groups according to circulation function and organ perfusion.The levels of C-reactive protein,procalcitonin,cytokines,PRISM-Ⅲ and clinical outcomes were analyzed between the relative groups.Results (1) Totally 138 septic children were recruited,64 with neutropenia and 74 without neutropenia.The level of PRISM-Ⅲ of the neutropenia group was significantly higher than that of the non-neutropenia group (P=0.048).Mortality showed no significant difference between the two groups,but hospital stay in the neutropenia group was longer than that in the non-neutropenia group.The levels of C-reactive protein,IL-6,and IL-10 ihe neutropenia group were significantly higher than those of the non-neutropenia group (P=0.001;P=0.001;P=0.032).The level of TNF-α in the neutropenia group was significantly lower than that of the non-neutropenia group (P=0.032).(2)Among the 64 septic children with neutropenia,23 were combined with shock.The PRISM-Ⅲ level of the shock group was significantly higher than that of the non-shock group (P=0.001).The mortality of the shock group (43.5％,10/23) was significantly higher than the non-shock group (2.4％,1/41) (P=0.001).C-reactive protein,procalcitonin,IL-6,IL-10 and TNF-α in the shock group elevated obviously than those in the non-shock group (P=0.001;P=0.001;P=0.001;P=0.005;P=0.019).The area under receiver operating characteristic curve was 0.8 for IL-6 (cut-offvalue 315.38 pg/mL),0.8 for IL-10 (cutoff value 45.18 pg/mL),and 0.85 for TNF-α (cut-off value 1.95 pg/mL).(3) Among the 74 septic children without neutropenia,19 were combined with shock The PRISM-Ⅲ level of the shock group was significantly higher than that of the non-shock group (P=0.022).There was no significant difference of mortality between the two groups (P=0.3).IL-10 level in the shock group elevated obviously than that in the non-shock group (P=0.015).(4) Among the 42 children with sepsis shock,23 were combined with neutropenia.The PRISM-Ⅲ level of the neutropenia group was significantly higher than that of the non-neutropenia group (P=0.005).There was no significant difference of mortality between the two groups (P=0.29).The levels of C-reactive protein,procalcitonin,IL-6 and IL-10 in the neutropenia group were significantly higher than those in the non-neutropenia group (P=0.001;P=0.001;P=0.001;P=0.035).There was no difference of TNF-α level between the two groups.(5) Among the 96 children without sepsis shock,41 were combined with neutropenia.No difference of PRISM-Ⅲ level was observed between the neutropenia and nonneutropenia groups.The mortality of the neutropenia group was significantly lower than that in the non-neutropenia group (2.4％ vs 20％,P=0.02).The levels of C-reactive protein and IL-6 in the neutropenia group were significantly higher than those in the non-neutropenia group (P=0.005;P=0.033).The TNF-α level was significantly lower than that in the non-neutropenia group (P=0.007).Conclusions Compared to children without neutropenia,septic children combined with neutropenia have longer hospital stay,and septic shock children combined with neutropenia have higher mortality,and levels of IL-6,IL-10 and TNF-α were also significantly increased.The levels of IL-6,IL-10 and TNF-α can help to predict prognosis of children with sepsis.

OBJECTIVE Disturbance of K+ ion balance in inner ear is associated in age-related hearing loss. Our study is to investigate the role of NKCC1 and Na-K-ATPase in cochlea and auditory function regulated by with different expression of NKCC1 and Na-K-ATPase. METHODS Auditory threshold of young or old C57BL/6J mice was measured by auditory brainstem response(ABR). The expression of NKCC1 and Na-K-ATPase in mice cochlea were evaluated by reverse transcription polymerase chain reaction(RT-PCR) and western blotting. Furosemide and Ouabain were applied in vivo to inhibit NKCC1 and Na-K-ATPase in C57BL/6J mice. RESULTS C57BL/6J mice developed hearing loss at 12M by ABR threshold shifting to (75±10), (78±26) and (81±14)dB SPL at frequencies of 8, 16 and 32 kHz; PCR showed that the relative expression of NKCC1 and Na-K-ATPase mRNA in the aged group decreased, which were 0.52±0.06 and 0.35±0.04 times higher than those in the young control group, the difference was statistically significant(t =7.466 and 16.11, all P＜0.05). WB showed that relative expression of NKCC1 and Na-K-ATPase protein level in the aged group decreased by 0.79±0.02 and 0.68±0.05 times as much as that of the young control group, the difference was statistically significant(t =8.857 and 6.771, P all＜0.05). After applied with Furosemide and Ouabain to suppress the two ion transporters, the ABR threshold increased to (50±17), (53±21), (55±17)dB SPL and (56±6), (70±17), (73±6)dB SPL at frequencies of 8, 16 and 32 kHz. CONCLUSION In vivo experiment of C57BL/6J suggested that NKCC1 and Na-K-ATPase might be related to age related hearing loss.

Objective To explore the expression and clinical value of long non-coding RNA ( lncRNA) in sepsis children. Methods The peripheral blood samples were analyzed from 15 sepsis children ( sepsis group) ,7 septic shock children( septic shock group) and 21 healthy children( healthy control group) . The real time-polymerase chain reaction was used to explore the expression of 9 kinds of lncRNA (AK092960,LOC100192426,VHDJH,AC006230. 3,AC019097. 7,RP4-652L8. 2,RP11-108A15. 2,NKILA and AK023660) which are closely related to the nuclear factor-κB pathway of the sepsis. And further analysis of lncRNA expression between sepsis,septic shock and health children were carried out. The specificity and sensitivity of the lncRNAs compared with C-reactive protein, procalcitonin and WBC for identification of patients with sepsis or septic shock were also evaluated. Results The expression of VHDJH,AC019097. 7, RP4-652L8. 2,RP11-108A15. 2, NKILA and AK02366 in the sepsis group were significantly higher than those in the healthy control group(P < 0.01). Among them,the expression of VHDJH,AC019097.7, RP4-652L8. 2,NKILA and AK023660 in the septic shock group were higher than those in the sepsis group (P<0. 01). Although the specificity and sensitivity of VHDJH, AC019097. 7, RP4-652L8. 2, NKILA and AK023660 were higher than C-reactive protein,procalcitonin and WBC for sepsis and septic shock,respectively, there was no significant difference statistically(P >0.05). Conclusion VHDJH,AC019097.7,RP4-652L8.2, RP11-108A15. 2,NKILA and AK023660 could be the potential diagnostic biomarkers of sepsis and might reflect its severity.