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

Objective:To analyze and summarize the clinical features and short-medium term follow-up results of children with multisystem inflammatory syndrome（MIS-C）following coronavirus infection.Methods:The data of six children with MIS-C admitted to the Intensive Care Unit of Shanghai Children's Medical Center from January to March 2023 were retrospectively analyzed.Results:All six cases were in shock，requiring vasoactive drugs，and one case required invasive mechanical ventilation.All the six patients had multiple organ function injury and increased inflammation indicators.After admission，they received organ support，glucocorticoids and gamma globulin treatment.Two patients were treated with biological agents.Both organ function and inflammation indicators showed significantly improvement after therapy.Six patients had mild coronary artery widening.All patients had good prognosis following short-medium term follow-up.Conclusion:Children with severe MIS-C may suffer life-threatening hemodynamic instability.Timely assessment，active anti-inflammatory and organ support therapy can obtain favorable prognosis.

Objective:To analyze and summarize the clinical features and short-medium term follow-up results of children with multisystem inflammatory syndrome（MIS-C）following coronavirus infection.Methods:The data of six children with MIS-C admitted to the Intensive Care Unit of Shanghai Children's Medical Center from January to March 2023 were retrospectively analyzed.Results:All six cases were in shock，requiring vasoactive drugs，and one case required invasive mechanical ventilation.All the six patients had multiple organ function injury and increased inflammation indicators.After admission，they received organ support，glucocorticoids and gamma globulin treatment.Two patients were treated with biological agents.Both organ function and inflammation indicators showed significantly improvement after therapy.Six patients had mild coronary artery widening.All patients had good prognosis following short-medium term follow-up.Conclusion:Children with severe MIS-C may suffer life-threatening hemodynamic instability.Timely assessment，active anti-inflammatory and organ support therapy can obtain favorable prognosis.

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 effect of applying the multi-disciplinary team（MDT）model led by specialized nursing care in the treatment carried out at early stage in intensive care unit of hyperbaric oxygen（ICU-HBO）for patients with brain complications after Stanford type A aortic dissection（AD）surgery.Method:From January 2018 to December 2020，a total of 18 patients with brain complications after AD surgery were included and treated by ICU-HBO with MDT model. The patients’ ventilator tolerance and weaning time，outcome，occurrence of adverse events，and satisfaction rate of nursing care were observed.Results:（1）Fifteen mechanically ventilated patients were successfully weaned off the machine after 1-5 times of ICU-HBO treatment.（2）Sixteen patients regained their consciousness to the normal level after 2-13 times of ICU-HBO treatments，with the total effective rate of 88.89%；two patients in persistent vegetative state（deep coma）discontinued the treatment after two times of ICU-HBO treatments as their family gave up. During the follow-up period of six months to two years，11 cases were found clinically cured，one case was considered effective，four cases lost contact，and the two cases that had gave up treatment died of complications.（3）No adverse medical event（related to safety）occurred during the ICU-HBO treatment.（4）The satisfaction rate of nursing care was 100%.Conclusion:In the early ICU-HBO treatment of patients with brain complications after Stanford type A AD surgery，the application of MDT mode led by specialized nursing care is beneficial to the treatment risk control，ensure the treatment effectiveness，and improve the long-term prognosis，and quality of life of AD patients.

Objective:To explore the effect of applying the multi-disciplinary team（MDT）model led by specialized nursing care in the treatment carried out at early stage in intensive care unit of hyperbaric oxygen（ICU-HBO）for patients with brain complications after Stanford type A aortic dissection（AD）surgery.Method:From January 2018 to December 2020，a total of 18 patients with brain complications after AD surgery were included and treated by ICU-HBO with MDT model. The patients’ ventilator tolerance and weaning time，outcome，occurrence of adverse events，and satisfaction rate of nursing care were observed.Results:（1）Fifteen mechanically ventilated patients were successfully weaned off the machine after 1-5 times of ICU-HBO treatment.（2）Sixteen patients regained their consciousness to the normal level after 2-13 times of ICU-HBO treatments，with the total effective rate of 88.89%；two patients in persistent vegetative state（deep coma）discontinued the treatment after two times of ICU-HBO treatments as their family gave up. During the follow-up period of six months to two years，11 cases were found clinically cured，one case was considered effective，four cases lost contact，and the two cases that had gave up treatment died of complications.（3）No adverse medical event（related to safety）occurred during the ICU-HBO treatment.（4）The satisfaction rate of nursing care was 100%.Conclusion:In the early ICU-HBO treatment of patients with brain complications after Stanford type A AD surgery，the application of MDT mode led by specialized nursing care is beneficial to the treatment risk control，ensure the treatment effectiveness，and improve the long-term prognosis，and quality of life of AD patients.

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.