The value of the ratio of heparin binding protein to albumin in evaluating the state of shock syndrome in Kawasaki disease / 中华急诊医学杂志

Objective:To investigate the value of the ratio of heparin binding protein (HBP) to albumin (ALB) in diagnosis and predict the severity of Kawasaki disease shock syndrome (KDSS).Methods:This study was a retrospective study. Pediatric patients with Kawasaki disease (KD) admitted to the Children's Intensive Care Unit and department of Pediatric Emergency Center of Hunan Children's Hospital from January 2019 to May 2022 were enrolled. The HBP/ALB ratio was calculated according to HBP and serum ALB. The children were divided into three groups (low, medium, and high ratio groups) according to the median and upper and lower quartiles of the HBP/ALB ratio. The differences of each index among the three groups were compared. The receiver operating characteristic curves were drawn to evaluate the clinical value of the HBP/ALB ratio in diagnosis of KDSS and the severity of the disease.Results:A total of 111 cases were included in this study, including 28 cases in the low ratio group, 56 cases in the medium ratio group, and 27 cases in the high ratio group. There were 24 cases with coronary artery damage, 87 cases without coronary artery damage, 27 cases with abnormal ECG findings, and 17 children with KDSS (including 5 cases in the medium ratio group, and 12 cases in the high ratio group). The incidence of KDSS, coronary involvement, and abnormal electrocardiogram proportions in the high ratio group were significantly higher than those in the other two groups. Compared with low and medium ratio groups, the levels of cardiac troponin I, N-terminal pro-brain natriuretic peptide, lactate, stroke output variation, trends in thoracic fluid content, white blood cell count, C-reactive protein, procalcitonin, and D-dimer levels were higher in the high ratio group, while ALB and blood sodium levels were lower in the high ratio group (all P<0.05). There was no significant difference in above indicators between the low and medium ratio groups (all P>0.05). The HBP/ALB ratio had a higher area under the curve, sensitivity, and specificity (0.942, 0.882, and 0.883, respectively) in predicting KDSS compared to HBP alone (0.776, 0.842, and 0.670, respectively). Conclusion:The HBP/ALB ratio could reflect the severity of children with KD and has certain clinical value for prognostic evaluation.

Research progress on the role and changes of natural killer cells in sepsis / 中国小儿急救医学

Sepsis is the organ dysfunction caused by infection.It is one of the most common critical diseases in clinic.Its morbidity and mortality are increasing year by year，which has seriously threatened human health.Innate immunity is the first line of defense against pathogens.Nature killer（NK）cells are important cells involved in the regulation of innate immunity in sepsis，and can play an important role on the progression of sepsis by secreting cytokines，inducing apoptosis and mediating cytotoxic effects.It has been found that the changes of NK cells in the early stage of sepsis are related to the prognosis of the disease.Therefore，further study on the role of NK cells in sepsis can provide a new idea for the clinical diagnosis and treatment of sepsis，and contribute to the early identification of sepsis and the improvement of prognosis.This review summarized the role and changes of NK cell in sepsis.

Clinical characteristics and drug resistance of children with septic shock during six years in a single center / 中国小儿急救医学

Objective:To explore the clinical characteristics of children with septic shock and analyze the drug resistance of blood culture positive bacteria.Methods:The clinical data,positive blood culture strains and drug sensitivity results of 127 children with septic shock admitted to the Department of Intensive Care Medicine of Hunan Children's Hospital from September 2015 to August 2021 were retrospectively analyzed.Results:A total of 134 strains of bacteria or fungi were isolated from the blood culture samples of 127 children with septic shock,and gram-negative strains were the main ones,accounting for 67.16% (90/134).Haemophilus influenzae and Escherichia coli were the main gram-negative bacteria,accounting for 38.81% (52/134) and 20.15% (27/134)，respectively,while Streptococcus pneumoniae was the main gram-positive bacteria,accounting for 8.21% (11/134),and Candida albicans was the main fungus,accounting for 10.45% (14/134).The number of white blood cells,the levels of serum C-reactive protein,procalcitonin,venous blood sugar and arterial blood lactic acid in patients were all significantly higher than normal values,and the white blood cells count and neutrophil percentage in gram-positive bacterial infections were significantly higher than those with gram-negative bacterial infections and fungal infections( P<0.05).Procalcitonin increased most obviously when infected by gram-negative bacteria,and the difference was statistically significant ( P<0.05).Gram-positive strains were sensitive to vancomycin,teicoplanin,and linezolid,but only 50% of Streptococcus pneumoniae were sensitive to penicillin.Gram-negative strains had relatively high drug resistance,among which Klebsiella pneumoniae were only highly resistant to imipenem,cilastatin and levofloxacin,reaching 50%.Haemophilus influenzae was resistant to cephalosporins and β-amides enzyme antibiotic,and the drug sensitivity rate of lactamase antibiotics was high,with a resistance rate of 50% only to ampicillin,cefuroxime,amikacin,and compound sulfamethoxazole.There were not many fungal strains,and most antifungal drugs were effective against blood culture-positive fungi. Conclusion:The main pathogens of infection in children with septic shock are gram-negative bacteria,and have high resistance to general antibiotics.We should pay attention to their drug resistance when using antibiotics empirically.

Significance of serum insulin combined with cardiac markers in evaluating sepsis associated encephalopathy / 中华急诊医学杂志

Objective:To explore the clinical value of serum insulin combined with cardiac-related markers in evaluating the severity of sepsis associated encephalopathy (SAE).Methods:The clinical data of 130 children with sepsis who admitted to the Pediatric Intensive Care Unit of Hunan Children's Hospital from January 2018 to December 2021 were analyzed retrospectively, and the differences of serum insulin and cardiac-related markers in children with sepsis and SAE were compared.Results:The levels of serum insulin, creatine kinase isoenzyme, hypersensitive troponin T, and N-terminal cerebral urine peptide in the SAE group were significantly higher than those in the non-SAE group ( P<0.05), but there was no significant difference in heart rate and lactic acid ( P>0.05). The levels of serum insulin, creatine kinase isoenzyme, hypersensitive troponin T, N-terminal cerebral urine peptide and lactic acid in the death group were significantly higher than those in the survival group ( P<0.05), while the heart rate was not significantly different ( P>0.05). The area under ROC curve of serum insulin, creatine kinase isoenzyme, hypersensitive troponin T, and N-terminal cerebral urine peptide in predicting SAE were 0.841, 0.599, 0.700, and 0.667, respectively; in terms of judging the prognosis of sepsis, the area under ROC curve were 0.647, 0.669, 0.645, and 0.683, respectively; and in terms of judging the prognosis of children with SAE, the areas under the ROC curve were 0.509, 0.682, 0.666 and 0.555, respectively. Binary logistic regression equation was established with serum insulin, creatine kinase isoenzyme, hypersensitive troponin T, and N-terminal cerebral urine peptide: Y=8.153×NT-proBNP+1.704×CTnT-hs+27.121×insulin+0.946×CK-MB+1.573. The area under the ROC curve of the new variable Y in predicting sepsis SAE, evaluating the prognosis of sepsis, and predicting the prognosis of children with sepsis and SAE was 0.890, 0.756, and 0.729, respectively. Conclusions:Serum insulin, creatine kinase isoenzyme, hypersensitive troponin T, and N-terminal cerebral urine peptide can be used alone to determine the severity of sepsis and sepsis in children with SAE. The combined value of the four indicators is obviously better than that of the single indicator. The combined application of the four indicators may better evaluate the severity of sepsis and SAE.

Significance of sputum heparin binding protein in prognostic evaluation of children with sepsis complicated with acute respiratory distress syndrome / 中国小儿急救医学

Objective:To investigate the predictive value of sputum heparin binding protein(HBP) in sepsis related acute respiratory distress syndrome(ARDS).Methods:This study was a prospective case-control study.A total of 134 children with sepsis who were admitted in PICU at Hunan Children′s Hospital from January 2020 to November 2021 were included, including 63 children who had completed fiberoptic bronchoscopy.The 63 children were divided into sepsis without ARDS group, sepsis with mild ARDS group, and sepsis with moderate to severe ARDS group according to the presence and severity of ARDS.Sputum was collected and HBP was detected in all children with sepsis when they were admitted to the hospital.The alveolar lavage fluid within 72 hours of admission was reserved for HBP.The levels of interleukin (IL)-6 and tumor necrosis factor (TNF)- α were detected, and the blood biochemistry, pulmonary imaging, pediatric critical case score and other data within 72 hours were collected.Results:(1) Among 63 children with fiberoptic bronchoscopy, 29 were in sepsis without ARDS group, 18 were in the sepsis with mild ARDS group, and 16 were in the sepsis with moderate to severe ARDS group.There was no significant difference in the pediatric critical case score and the location of primary infection focus among the three groups at admission.The primary infection focus was respiratory system in 36 cases, whose sputum HBP level was (42.1±9.8) ng/mL, and 27 children with other systems infection, whose sputum HBP level was (37.8±10.8) ng/mL, there was no significant difference between two groups ( t=1.65, P=0.104). (2) There were significant differences in sputum HBP, alveolar lavage fluid HBP, IL-6 and TNF-α levels among sepsis with mild ARDS group, sepsis with moderate and severe ARDS group and sepsis without ARDS group ( P<0.05). The sputum HBP of 34 children with sepsis combined with ARDS was positively correlated with alveolar lavage fluid HBP, IL-6, TNF-α levels and lung injury score, and negatively correlated with SpO 2/FiO 2 ( P<0.05). (3)Among the 34 children with sepsis combined with ARDS, the sputum HBP concentration of children with invasive ventilation was significantly higher than that of children with non-invasive ventilation ( P<0.05). The sputum HBP concentration in children with three or more organ damage was significantly higher than that of children with two or less organ damage ( P<0.05). The sputum HBP concentration of dead children was higher than that of surviving children ( P<0.05). (4) The area under curve of sputum HBP for predicting ARDS was 0.772 (95% CI: 0.655~0.889). When the cut-off point value of sputum HBP was 27.9 mU/L, whose sensitivity and specificity were 70.6% and 79.3%, respectively.The area under curve of sputum HBP for predicting moderate and severe ARDS was 0.793 (95% CI: 0.661~0.926). When the cut-off point value of sputum HBP was 51.55 mU/L, whose sensitivity and specificity were 81.3% and 76.6%, respectively. Conclusion:Sputum HBP is elevated in children with sepsis and ARDS, which is related with the severity of the disease.Sputum HBP has a good predictive value for the diagnosis and severity of children with sepsis and ARDS, and can be used as a clinically effective and convenient evaluation index for children with sepsis related ARDS.

Risk factors and prognosis of pulmonary hypertension associated with bronchopulmonary dysplasia in extremely preterm infants / 中国新生儿科杂志

Objective:To study the risk factors and prognosis of pulmonary hypertension(PH) associated with bronchopulmonary dysplasia (BPD) in extremely preterm infants(EPIs).Methods:From January 2020 to December 2021, EPIs [gestational age (GA) <32 w] with BPD admitted to NICU of our hospital were retrospectively assigned into two groups: BPD with late-onset PH(PH group) and BPD without late-onset PH(non-PH group). Their general condition, treatment and prognosis were compared and the risk factors of late-onset PH were analyzed.Results:A total of 229 EPIs with BPD were enrolled, including 24(10.5%) in the PH group and 205(89.5%) in the non-PH group. The PH group had significantly smaller GA [(27.9±2.3) w vs. (28.7±1.8) w], longer mechanical ventilation [42.0(16.0, 84.0) d vs. 9.0(2.0, 23.0) d], longer hospital stay [100.5(86.3, 142.0) d vs. 77.0(56.5, 96.5)d],higher incidence of early-onset PH(54.2% vs. 9.3%) and higher mortality rate(33.3% vs. 9.8%) than the non-PH group ( P<0.05). Multivariate logistic regression analysis showed prolonged mechanical ventilation ( OR=1.046, 95% CI 1.011~1.064), early-onset PH ( OR=5.414, 95% CI 1.796~16.323) were independent risk factors for BPD with late-onset PH. 8(33.3%) patients in the PH group died, including 2 with grade Ⅱ BPD and 6 grade Ⅲ BPD. Conclusions:Prolonged mechanical ventilation and early-onset PH are independent risk factors for late-onset PH in BPD infants. BPD infants with late-onset PH have longer hospital stay, higher mortality and worse prognosis.

Single-center clinical analysis of 9 049 children hospitalized for accidental injury / 中国实用护理杂志

Objective:To analyze the characteristics of child cases admitted to accidental injury and provide reliable basis for the prevention of accidental injury.Methods:The clinical data of children admitted to Hunan Children′s Hospital aged 0-18 due to accidental injuries from January 1, 2018 to December 31, 2021 were retrospectively analyzed. The type of accidental injury and characteristic factors such as prognosis, gender, age, time, and location of the child were analyzed.Results:A total of 9 049 children with accidental injury were admitted, accounting for 3.72%(9 049/33 697) of the total number of hospitalized children. The top three types of accidental injuries were falls/drop (3 695 cases), foreign bodies/suffocation (2 639 cases) and traffic accidents (1 165 cases), accounting for 82.87%(7 499/9 049). There were 8 760 cases (96.81%) of improvement and recovery, 178 cases (1.97%) of disability, and 111 cases (1.23%) of unhealed/dead. Among the accidental injuries, 5 833 cases (64.46%) were boys and 3 216 cases (35.54%) were girls, and the incidence ratio was 1.81∶1. There was significant difference between boys and girls in the composition ratio of the type of accidental injury such as falls/falls, foreign bodies/suffocation, poisoning, sharp object injury, drowning ( χ2 values were 3.90-20.56, all P<0.05). Among the accidental injuries, the children aged 1 to<3 years had higher accidental injuries than the other age groups (3 263 cases, accounting for 36.06%), and the composition ratio of accidental injuries in different age groups was different ( χ2 values were 12.98-573.97, all P<0.05). Among the accidental injuries, the accidental injuries occurred in the second quarter and the third quarter were higher than those in the other two quarters (4 892 cases, accounting for 54.06%), and the composition ratio of accidental injuries such as falls/falls, foreign bodies/suffocation, burn and scald, drowning occurred in different quarters was different ( χ2 values were 10.79-18.88, all P<0.05). In the case of accidental injuries, the family was the most likely place of accidental injury, with different types of accidental injuries occurring in different places ( χ2 values were 10.08-2 186.54, all P<0.05). Conclusions:Children′s unintentional injuries are most likely to occur in boys aged 1-<3 years, and fall/fall is the main injury type. Traffic accidents are the most important unintentional injury type leading to children′s unhealed/dead. Different injury types were related to child gender, age, quarter, and place of occurrence.Due to the differences in the occurrence mechanism and injury mode of accidental injuries in different countries and regions, and the majority of accidental injuries can be prevented, targeted preventive measures should be taken according to the characteristics of children′s accidental injuries in different regions, and a comprehensive prevention system for children′s accidental injuries should be constructed to ensure children′s safety.

Clinical characteristics and prognostic analysis of pancreatic enzyme abnormalities in children / 中华实用儿科临床杂志

Objective:To investigate the etiology, prognosis and clinical characteristics of abnormal serum amylase and lipase in children.Methods:This study was a retrospective study.A total of 7 813 children older than 28 days who had their serum amylase and lipase detected in Hunan Children′s Hospital from August 2017 to August 2020 were included as the study subjects.Children with acute and chronic pancreatitis were excluded.The age, gender, impatient department, imaging exams, discharge outcomes, main diagnosis, diagnostic ICD10 code, and the highest values of serum amylase and lipase during hospitalization were collected through the medical record system.According to the levels of serum amylase and lipase, the children were divided into 3 groups.Patients in group A had normal serum amylase and serum lipase levels.The serum amylase or lipase levels of patients in group B was 1 to 3 times higher than that of group A. The serum amylase or lipase levels in group C was 3 times higher than that of group A. Group B and group C had abnormal pancreatic enzyme levels.According to the prognosis, patients were divided into the survival group and the death group.The relationship of the occurrence of abnormal serum amylase and lipase levels with the age, sex, disease type and prognosis of children was analyzed.Results:The ratio of abnormal trypsin in male and female was 11.5% and 12.9%, respectively.The number of children with abnormal pancreatic enzyme levels in the 28 day -1 year old group, >1-3 years old group, >3-6 years old group, >6 -12 years old group and > 12 year old group were 37 cases (4.6%), 185 cases (15.4%), 199 cases (10.5%), 431 cases (13.9%), and 94 cases (11.7%), respectively.The mortality rate was 1.6% (112/6 867 cases) in group A, 5.2% (32/617 cases) in group B, and 7.6% (25/329 cases) in group C. The mortality risk of group B and C was both higher than that of group A. Compared with group A, the OR (95% CI) of group B and group C was 3.30 (2.21-4.93) and 4.96 (3.17-7.77), respectively.In group C, the top five diseases were parotitis (26.4%), cholangiectasis (11.6%), choledochal cysts (8.5%), gastroenteritis (4.5%) and sepsis (3.3%). Conclusions:Pancreatic enzyme abnormalities in children are associated with adverse prognosis.Pancreatic enzyme abnormalities are more prone to occur in children aged >1-3 with mumps, digestive diseases and congenital digestive system structural deformities.In addition, children with sepsis are also easy to present pancreatic enzyme abnormalities.Clinical attention should be paid to the possibility of secondary pancreatic damage in children with sepsis.

Clinical characteristics of pneumocystis carinii pneumonia in children without human immunodeficiency virus infection / 中国小儿急救医学

Objective:To investigate the clinical characteristics and prognosis factors in children with pneumocystis carinii pneumonia (PCP) without human immunodeficiency virus (HIV) infected.Methods:From January 2017 to December 2020, 35 non-HIV infected patients with PCP were admitted to Hunan Children′s Hospital.According to the prognosis at discharge, they were divided into survival group and death group.The clinical characteristics of two groups were compared, and the prognostic factors were analyzed.Results:The age of 35 patients ranged from 1 month to 15 years, including 24 males and 11 females.Seven patients(20.0%) had primary immunodeficiency, 5 patients(14.2%) had autoimmune disease, and 4 patients(11.4%) had renal disease.Eighteen patients were treated with long-term hormone and 13 patients were treated with immunosuppressive agents before the onset of the disease.Clinical symptoms included shortness of breath or dyspnea, cough, fever and so on, while with few pulmonary signs.Peripheral blood lymphocyte count was less than 1.5×10 9/L in 18 cases.The median LDH was(654.94±57.66)U/L; Fungal D-glucan increased in 13 cases.The median P/F was(121.29±23.25)mmHg, and P/F was less than 200 mmHg in 16 cases.CD4 cells were less than 500/μL in 15 cases and less than 200/μL in 8 cases.The imaging findings were mainly consolidation or patellar shadow, diffuse ground glass shadow, 3 cases with pleural effusion, and 1 case with pneumothorax.Twenty-two cases survived and 13 died, with a mortality rate of 37.1%.There were statistically significant differences in hospitalization days, CD4 cell count, Fungal D-glucan, P/F, ICU admission and invasive mechanical ventilation between two groups( P<0.05). Logistic multivariate analysis showed that decreased P/F value was an independent risk factor affecting the prognosis of non-HIV infected children with PCP ( OR=0.006, 95% CI 0.975-1.000). Conclusion:The clinical manifestations, laboratory examinations and imaging examinations of non-HIV infected patients with PCP lack specificity.When a diagnosis is suspected, high-resolution CT should be performed based on the results of peripheral blood lymphocyte count, CD4 cell count, fungal D, LDH, and blood gas analysis results as soon as possible, compound sulfamethoxazole should be used timely.Decreased P/F value is an independent factor affecting the prognosis of non-HIV children with PCP.

Clinical characteristics of pneumocystis carinii pneumonia in children without human immunodeficiency virus infection / 中国小儿急救医学

Objective:To investigate the clinical characteristics and prognosis factors in children with pneumocystis carinii pneumonia (PCP) without human immunodeficiency virus (HIV) infected.Methods:From January 2017 to December 2020, 35 non-HIV infected patients with PCP were admitted to Hunan Children′s Hospital.According to the prognosis at discharge, they were divided into survival group and death group.The clinical characteristics of two groups were compared, and the prognostic factors were analyzed.Results:The age of 35 patients ranged from 1 month to 15 years, including 24 males and 11 females.Seven patients(20.0%) had primary immunodeficiency, 5 patients(14.2%) had autoimmune disease, and 4 patients(11.4%) had renal disease.Eighteen patients were treated with long-term hormone and 13 patients were treated with immunosuppressive agents before the onset of the disease.Clinical symptoms included shortness of breath or dyspnea, cough, fever and so on, while with few pulmonary signs.Peripheral blood lymphocyte count was less than 1.5×10 9/L in 18 cases.The median LDH was(654.94±57.66)U/L; Fungal D-glucan increased in 13 cases.The median P/F was(121.29±23.25)mmHg, and P/F was less than 200 mmHg in 16 cases.CD4 cells were less than 500/μL in 15 cases and less than 200/μL in 8 cases.The imaging findings were mainly consolidation or patellar shadow, diffuse ground glass shadow, 3 cases with pleural effusion, and 1 case with pneumothorax.Twenty-two cases survived and 13 died, with a mortality rate of 37.1%.There were statistically significant differences in hospitalization days, CD4 cell count, Fungal D-glucan, P/F, ICU admission and invasive mechanical ventilation between two groups( P<0.05). Logistic multivariate analysis showed that decreased P/F value was an independent risk factor affecting the prognosis of non-HIV infected children with PCP ( OR=0.006, 95% CI 0.975-1.000). Conclusion:The clinical manifestations, laboratory examinations and imaging examinations of non-HIV infected patients with PCP lack specificity.When a diagnosis is suspected, high-resolution CT should be performed based on the results of peripheral blood lymphocyte count, CD4 cell count, fungal D, LDH, and blood gas analysis results as soon as possible, compound sulfamethoxazole should be used timely.Decreased P/F value is an independent factor affecting the prognosis of non-HIV children with PCP.

Clinical features of pertussis and risk factors of severe pertussis in children / 中国小儿急救医学

Objective:To study the clinical features of children with pertussis and the risk factors of severe pertussis.Methods:A retrospective analysis was performed based on clinical data and laboratory examination results of hospitalized children with pertussis who admitted to the intensive care unit, respiratory department, and emergency general department at Hunan Children′s Hospital from January 2019 to March 2020.According to the age, the patients were divided into age ≤3 months group( n=58)and age >3 months group( n=64). According to sputum culture, 63 cases were divided into negative sputum culture group and 59 cases were positive sputum culture group.The patients were also divided into vaccinated group( n=19)and unvaccinated group( n=103). Severe disease was seen in 28 cases, and the other 94 cases had the modest disease.The clinical characteristics between two groups were compared, and the risk factors of severe pertussis pneumonia were analyzed. Results:The hospitalization days in age ≤3 months group was higher than that in age >3 months group.It was also found that shortness of breath, apnea, cyanosis after coughing, heart rate decline were more common in age ≤3 months group than those in age >3 months group( P<0.05). The incidences of respiratory failure and heart failure in positive sputum culture group were higher than those in negative sputum culture group.Clinical characteristics such as hospitalization days, hospitalization expenses, peak white blood cell count, peak lymphocyte count, and incidence of bacterial infection were higher in severe pertussis group than those in non-severe pertussis group( P<0.05). Four patients were treated with exchange blood transfusion, and one patient died.Logistic regression analysis revealed that fever, wheezing, cyanosis after coughing and white blood cell count>20×10 9/L were risk factors for severe pertussis.White blood cell count of 20×10 9/L and lymphocyte count of 14×10 9/L had the highest sensitivity and specificity in predicting severe pertussis(0.71, 0.78; 0.54, 0.79). Conclusion:The younger the children are, the more likely they have shortness of breath, apnea, cyanosis, heart rate falls, and the longer the hospital stay.Bacterial infection will aggravate pertussis.Patients with fever, wheezing, cyanosis after coughing, and white blood cell count>20×10 9/L are more likely to develop severe pertussis.The white blood cell count >20×10 9/L and the lymphocyte count >14×10 9/L are associated with severe pertussis.

Research progress on the role of nuclear factor erythroid 2-related factor 2 in ferroptosis of sepsis / 中华危重病急救医学

Sepsis is a life-threatening organ dysfunction caused by the disorder of the body's response to infection, and is one of the main causes of death in critically ill patients. Ferroptosis is a kind of iron dependent cell death, characterized by intracellular reactive oxygen species (ROS) accumulation. Sepsis can cause a substantial accumulation of ROS in cells. The transcription factor nuclear factor erythroid 2-related factor 2 (Nrf2) is a key regulator of antioxidant and plays a critical protective role in sepsis induced ferroptosis by regulating the expression of proteins related to the ferroptosis pathway. Current studies have found that activation of Nrf2 has a protective effect on ferroptosis induced by sepsis. In this paper, we summarized the regulation mechanism of Nrf2 in ferroptosis, in order to provide references for the treatment of sepsis.

Analysis of a child with carnitine palmitoyl transferase 1A deficiency due to variant of CPT1A gene / 中华医学遗传学杂志

OBJECTIVE@#To report on the clinical, metabolic and genetic characteristics of a child with carnitine palmitoyl transferase 1A (CPT1A) deficiency.@*METHODS@#Clinical data and the level of acylcarnitine for a child who initially presented as epilepsy were analyzed. Genomic DNA was extracted from peripheral blood samples of the child and her parents and subjected to next-generation sequencing (NGS).@*RESULTS@#Mass spectrometry of blood acylcarnitine indicated increased carnitine 0 (C0) and significantly increased C0/ (C16+C18). DNA sequencing revealed that the child has carried compound heterozygous variants of the CPT1A gene, namely c.1846G>A and c.2201T>C, which were respectively inherited from her mother and father.@*CONCLUSION@#CPT1A presenting initially as epilepsy was unreported previously. Analysis of blood acylcarnitine C0 and C0/ (C16 + C18) ratio and NGS are necessary for the identification and diagnosis of CPT1A deficiency. The c.1846G>A and c.2201T>C variants of the CPT1A gene probably underlay the disease in this child. Above finding has also enriched the spectrum of CPT1A gene variants.

Comparative study of dexmedetomidine and midazolam for noninvasive continuous positive airway pressure in children with acute respiratory failure / 国际儿科学杂志

Objective:To investigate the efficacy and safety of dexmedetomidine in noninvasive continuous positive airway pressure(NCPAP)for acute respiratory failure in children.Methods:Clinical data of children with acute respiratory failure who underwent NCPAP from January 2018 to March 2020 in PICU of Hunan Children′s Hospital were prospectively collected.They were randomly divided into dexmedetomidine group(group D)and midazolam group(group M), with a total of 100 children.We compared the sedation depth of the two groups at 7 time points after sedation at 0.5 h(t1), 1 h(t2), 2 h(t3), 6 h(t4), 12 h(t5), 24 h(t6), and 48 h(t7), time to reach proper sedation, NCPAP time, NCPAP failure rate, oxygenation index(P/F value)before sedation(T0)and 1h(T1), 24h(T2), and 48h(T3)after sedation, and the main vital signs and adverse reactions before sedation(T0)and 1h(T1), 24h(T2), 48h(T3)after sedation.Results:(1)The proportion of proper sedation at T4, T5, T6 and T7 after sedation in group D was higher than that in group M[98%(49/50)vs.84%(42/50), 94%(47/50)vs.90%(45/50), 96%(48/50)vs.88%(44/50), 90%(45/50)vs.88%(44/50), χ2=6.538, 8.043, 8.174, 7.678, all P<0.05]. Time to reach proper sedation in group D was shorter[(58.6±7.9)s vs.(66.7±9.3)s, t=4.682, P<0.01]. (2)The treatment time and failure rate of NCPAP in group D were lower than those in group M[(134.9±25.5)h vs.(147.8±24.3)h, 10%(5/50)vs.28%(14/50), all P<0.05]. P/F after NCPAP treatment in the two groups was improved as compared with that before treatment(all P<0.01), and the improvement was more significant in group D than in group M at T2 and T3 after sedation[(199.3±26.1)vs.(188.5±24.2)mmHg, (212.2±25.4)mmHg vs.(200.8±24.8)mmHg, t=2.132, 2.278, all P<0.05]. (3)There were no significant differences in heart rate(HR), mean arterial pressure(MAP), and respiratory rate(RR)before sedation between the two groups(all P>0.05). HR and RR after sedation in both groups decreased as compared with those before sedation( P<0.01). HR at T1, T2, and T3 after sedation in group D decreased more significantly than that in group M[(116.3±17.6)bpm vs.(124.8±14.1)bpm, (110.2±18.4)bpm vs.(121.9±15.2)bpm, (108.5±18.7)bpm vs.(117.6±12.8)bpm, t=0.479, -3.474, -2.840, all P<0.05]. There was no significant difference in RR after sedation between the two groups( t=1.872, 1.632, 1.675, all P>0.05). MAP at T1 in group D decreased as compared with T0( P<0.01). MAP at T1 in group D was lower than that in group M[(65.5±5.1)mmHg vs.(68.0±5.7)mmHg, t=-2.297, P=0.024]. (4)There was no significant difference in the incidence of total adverse reactions between the two groups[20%(10/50)vs.14%(7/50), P=0.595]. The incidence of bradycardia was higher in group D than in group M[16%(8/50)vs.2%(1/50), P=0.031]. Conclusion:The incidence of adverse reactions of dexmedetomidine and midazolam in the sedation of NCPAP in children with acute respiratory failure is similar, but the sedative effect of dexmedetomidine is better than that of midazolam in the improvement of pulmonary oxygenation.

Application of heparin-binding protein in severe adenovirus pneumonia / 中华实用儿科临床杂志

Objective:To explore the application value of heparin-binding protein (HBP) in the early diagnosis and assessment of severe adenovirus pneumonia.Methods:A total of 90 children diagnosed with adenovirus pneumonia admitted in the Department 1 of Emergency and Pediatric Intensive Care Unit 1 in Hunan Children′s Hospital from January 2019 to March 2020 were recruited.HBP levels in children with adenovirus pneumonia were detected.The correlation between HBP with white blood cell count (WBC), neutrophil ratio (N), C-reactive protein (CRP), interleukin-6(IL-6) and erythrocyte sedimentation rate (ESR) were examined.Receiver operating characteristic(ROC) curve analysis was conducted to explore the value of HBP in the early diagnosis and assessment of severe adenovirus pneumonia.Children with adenovirus pneumonia were divided into severe adenovirus pneumonia group (severe group) and non-severe adenovirus pneumonia group (non-severe group) according to their severity.Those in the severe group were further divided into bronchiolitis obliterans(BO) group and non-BO group according to the occurrence of BO.Results:(1) The HBP level in children with adenovirus pneumonia was (49.47±34.19) μg/L, which was significantly higher in the severe group than that of non-severe group[(82.88±44.02) μg/L vs.(35.15±13.08) μg/L, t=15.349, P<0.05]. Children in the severe group were significantly younger, and they had a significantly longer length of stay, lower Pediatric Critical Illness Scores (PCIS), and higher inflammatory markers like HBP, WBC, N, CRP, IL-6, and ESR compared with those of the non-severe group (all P<0.05). No significant difference in the procalcitonin (PCT) level was detected between groups.(2) The HBP was positively correlated with inflammatory markers like WBC ( r=0.38, P<0.05), N ( r=0.26, P<0.05), CRP ( r=0.47, P<0.05), IL-6 ( r=0.76, P<0.05), and ESR ( r=0.35, P<0.05). However, HBP did not have a significant correlation with PCT ( r=0.097, P>0.05). (3) In the severe group, the HBP level of the children with invasive mechanical ventilation, oxygenation index(P/F index)≤ 200 mmHg (1 mmHg=0.133 kPa) and BO was significantly higher than that of the non-invasive mechanical ventilation, P/F index> 200 mmHg and non-BO (all P<0.05). (4) The area under the ROC curve of HBP, WBC, N, CRP, ESR and IL-6 in predicting the severity of adenovirus pneumonia were 0.915, 0.748, 0.770, 0.740, 0.820 and 0.798, respectively.When the cut-off value of HBP was 45 μg/L, the sensitivity and specificity of HBP were 81.48% and 85.71%, respectively. Conclusions:As an inflammatory mediator, HBP is involved in the inflammatory response of the body.It may be a useful new marker for the early diagnosis of severe adenovirus infection, which also has a certain value in the evaluation of the severity and prognosis of the disease.The findings provide a basis for early clinical intervention and treatment of adenovirus infection in children.

Clinical application of extracorporeal membrane oxygenation in 4 children with severe adenovirus pneumonia / 中华实用儿科临床杂志

Objective:To retrospectively analyze the clinical application of extracorporeal membrane oxygenation (ECMO) in severe adenovirus pneumonia, and to evaluate the application value of ECMO in children with severe adenovirus pneumonia.Methods:Children diagnosed with severe adenovirus pneumonia and intervened with ECMO in the Hunan Children′s Hospital from January 1, 2018 to December 31, 2019 were recruited in this study for analyzing.The gender, age, clinical manifestations, mechanical ventilation duration, ECMO duration, the length of hospital stay, complications and prognosis were collected and analyzed.Results:A total of 4 children were included in the study, involving 2 cases were successfully evacuated from ECMO.Finally, 3 children died, and 1 case survived.Three death cases had a longer than 18 days of duration from the onset to the start with ECMO.Their ventilator assist time before star-ting ECMO was 3-5 days, and ECMO intervention time was longer, with the maximum of 27.5 days.The survived case had an 11-day duration from the onset to the start with ECMO, and the ventilator assisted time and ECMO intervention time were 5 days, and less than 10 days, respectively.Conclusions:ECMO treatment for children with severe adenovirus pneumonia has a low success rate, but it is still the most important way to save children.Early application of ECMO can improve the prognosis of children with severe adenovirus pneumonia.

Evaluation of cytokine immunotyping on the condition and prognosis of children with sepsis / 中国小儿急救医学

Objective:To evaluate the effect of serum cytokines and immune typing on the severity and prognosis of children with sepsis.Methods:One hundred and sixty children with sepsis admitted to No.2 PICU from May 2018 to May 2019 at Hunan Children′s Hospital were enrolled.Vital signs, blood routine and blood biochemical indexes of the children were collected within 24 hours after admission.Serum cytokine concentrations of interleukin(IL)-2, IL-4, IL-6, IL-10, tumor necrosis factor(TNF)-α, and interferon (IFN)-γ were tested at the same time.According to the worst condition during the first 24 h after admission, and the consensus diagnostic criteria of experts in the diagnosis and treatment of septic shock in children in 2015, the patients were divided into sepsis group(76 cases) and severe sepsis group(84 cases). According to the prognosis of 28 days, the patients were divided into survival group(134 cases)and death group(26 cases). The differences of cytokine concentration and the composition of immune state among groups were compared.Results:The levels of IL-2[2.40(2.40, 2.55)pg/mL vs. 2.40(2.40, 3.76)pg/mL], IL-6[60.54(23.22, 250.38)pg/mL vs. 21.47(8.83, 57.94)pg/mL], IL-10[50.85(21.74, 217.12)pg/mL vs. 14.99(9.23, 28.79)pg/mL] , TNF-α[2.75(2.40, 5.15)pg/mL vs. 2.40(2.40, 3.23)pg/mL] and IFN-γ[8.01(4.34, 37.28)pg/mL vs.2.62(2.62, 28.56)pg/mL] in the severe sepsis group were significantly higher than those in the sepsis group( P<0.05). The levels of IL-10[99.57(10.51, 646.96)pg/mL vs. 23.17(11.74, 57.30)pg/mL] and TNF-α[3.08(2.40, 5.13)pg/mL vs. 2.45(2.40, 3.80)pg/mL] in the death group were significantly higher than those in the survival group( P<0.05). The risk of death in children with mixed antagonistic response syndrome (MARS) was significantly higher than those without MARS( OR 3.75, 95% CI 1.302-10.804). Conclusion:The increased concentrations of IL-2, IL-6, IL-10, TNF-α, and IFN-γ are significantly correlated with the severity of sepsis in children.Increased IL-10 concentration and the occurrence of MARS are significantly correlated with poor prognosis.The occurrence of MARS in children with sepsis should be highly vigilant for the poor prognosis.

Application of bronchoscopic lavage in children with severe adenovirus pneumonia / 中国小儿急救医学

Objective:To investigate the efficacy and safety of bronchoscopic lavage in children with severe adenoviral pneumonia.Methods:Patients with severe adenovirus pneumonia who were admitted to ICU department of Hunan Children′s Hospital for bronchoscopy were collected from February to June 2019 and divided into lavage group( n=36) and non-lavage group( n=15) in line with whether lavage was performed.Their results, namely, bronchoscopic diagnosis, blood gas analysis before and 2 hours, 24 hours and 48 hours after bronchoscopy, improvement time of clinical symptoms(fever and pulmonary moist rales), the positive rate of pathogen detection and mortality rate, main vital signs such as heart rate, respiratory rate, mean arterial pressure and bronchoscopy-related complications were recorded before and 1 hour, 2 hours and 24 hours after bronchoscopy. Results:A total of 51 children were collected, all of whom suffered from endobronchitis.More secretions were observed in the airways of 36 patients in the lavage group, and only a little or no secretions were observed in 15 patients in the non-lavage group.P/F value and PCO 2 at 2 hours, 24 hours and 48 hours after treatment in the lavage group were improved comparing to those before treatment and were superior to those in the non-lavage group( P<0.05). P/F values at 24 hours and 48 hours after treatment in the non-lavage group increased and PCO 2 decreased at 48 hours after treatment( P<0.05). The thermal duration, time to resolution of moist rales in the lungs in the lavage group were shorter than those in the non-lavage group( P<0.05). The mortality rate in the lavage group was lower than that in the non-lavage group[2.8%(1/36) vs.26.7%(4/15), P<0.05]. The positive rate of pathogen detection in lavage group was higher than that in non-lavage group[55.6%(20/36) vs. 20.0%(3/15), P<0.05]. There was no significant difference in heart rate, respiratory rate, and mean arterial pressure at each time point before and after bronchoscopic treatment( P>0.05). Associated complications were 11 cases of intraoperative transient hypoxemia, four cases of bronchial mucosal bleeding, and one case each of postoperative hypoxemia, intraoperative hypertension and hypotension.There was no significant difference in the incidence of complications between the two groups( P>0.05). Conclusion:Bronchoscopic lavage, in treating children with severe adenovirus pneumonia, may improve clinical symptoms, respiratory function, and rate of pathogen detection, reduce mortality, and is effective and safe.

A model study of cecal ligation and perforation for the development of sepsis-induced pancreatic injury / 中国小儿急救医学

Objective:To investigate the changes of inflammatory factors, serum amylase and lipase and pancreatic damage at different time points after establishment of cecal ligation and perforation in a rat model of sepsis, which provides a stable and reliable animal model for the study of pancreatic injury following sepsis in children.Methods:Sixty male SD rats were randomly divided into 6 h group, 12 h group, 24 h group and 48 h group in control group, sham group and cecal ligation and puncture (CLP) group.Rat model of sepsis was established by cecal ligation and perforation in CLP group, the sham group was treated with sham operation, and rats in control group were not treated.The levels of interleukin (IL)-1β, IL-10, amylase and lipase in each group were detected, and pancreatic tissue was taken for HE staining.Results:In this model, the 48 h survival rate of rats in the CLP group was 70%.With the prolongation of postoperative time in the CLP group, pancreatic tissue congestion, cell edema, and inflammatory cell infiltration gradually worsened.The pathological changes of pancreatic tissue in the Sham group were mild.The expression levels of IL-1β in CLP group and sham group increased first and then decreased, and reached the peak at 12 h. The expression levels of IL-1β in CLP group were significantly higher than those in control group and sham group at all time points( P<0.05). The expression level of IL-10 showed a gradually increasing trend, reaching a peak at 48 h. The expression level of IL-10 in CLP group was significantly higher than that in control group and sham group at all time points( P<0.05), which in sham group was significantly higher than that in control group( P<0.05). Serum amylase and lipase in CLP group showed an upward and then downward trend, reaching the peak at 24 h. The serum amylase levels at 24 h and 48 h were significantly higher than those in control group and sham group( P<0.05). Serum amylase levels in sham group were significantly different from those in control group at 12 h, 24 h and 48 h( P<0.05). Serum lipase level at 12 h, 24 h and 48 h was significantly higher than that in control group and sham group( P<0.05). There was no significant difference in serum lipase between sham group and control group( P>0.05). Conclusion:After CLP modeling, IL-1β, IL-10 elevation and pancreatic pathological changes appeared at the early stage, and the pathological changes gradually increased with time.Significant differences in serum amylase and lipase began to appear at 24 h and 12 h respectively.

Progress in diagnosis of sepsis-associated acute kidney injury in children / 中国小儿急救医学

Acute kidney injury(AKI)is a very common critical disease worldwide, especially in pediatric intensive care unit(PICU). There are many pathogenic factors for AKI, among which sepsis is essential to the development of AKI.Sepsis-associated acute kidney injury(SA-AKI) often have sudden onset and poor prognosis.Because there is still a lack of powerful diagnostic tools, resulting in clinical intervention in SA-AKI often lags behind.Therefore, awareness of SA-AKI risk and early identification of injury severity, as well as timely initiation of supportive treatment, remain the main contents of current research.There are hundreds of new biological markers and diagnostic tools for early identification and prognosis of AKI.This review introduced the progress on diagnosis of SA-AKI.The aim is to improve clinicians′ understanding of SA-AKI.