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Glycogen storage disease type Ⅱ (GSDⅡ) is a rare autosomal recessive disorder.Infant onset of GSDⅡ usually accompanies progressive cardiac hypertrophy and muscle weakness, and eventually dies of cardiopulmonary failure.GSDⅡ is mainly screened and diagnosed by enzymatic and genetic tests.Enzyme replacement therapy (ERT) is the only currently approved treatment of GSDⅡ, which can effectively improve the function of the affected organs and the survival.Gene therapy and substrate reduction therapy for GSDⅡ are also undergoing basic or clinical research.This review summarizes the current research status of the diagnosis and treatment of GSDⅡ at home and abroad, focusing on the influencing factors for the efficacy of specific treatment (especially ERT), dosing regimen, and ways to improve the efficacy.
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Objective:To investigate the dynamic trend of platelet（PLT）count and mean platelet volume（MPV）in children with severe community-acquired pneumonia（SCAP）in PICU and their correlation with prognosis.Methods:A retrospective study was conducted in 215 SCAP children who were admitted to the PICU of Beijing Children's Hospital Affiliated to Capital Medical University from January 2016 to December 2019.According to the disease outcome，the patients were divided into improvement group ( n=184) and unrecovered group ( n=31).The changes of PLT count and MPV at admission，on the 2nd，3rd，and 7th days of hospitalization and before discharge were observed，and the relationship between changes in PLT parameters and poor prognosis was analyzed. Meanwhile，the correlation between thrombocytopenia on admission and on the 7th day of hospitalization and prognosis was further explored. Results:The PLT count of improvement group at admission，on the 2nd，3rd，and 7th days of hospitalization and at discharge[(328±159, 329±137, 362±159, 439±168, 510±171)×10 9/L] were significantly higher than those of unrecovered group [(210±142, 207±152, 267±143, 260±162, 343±159)×10 9/L]（ P＜0.05）.Although the MPV of improvement group [(10.9±1.9)fL] on admission was significantly lower than that of the unrecovered group[(12.7±2.5) fL]（ P＜0.05），there was no significant difference in MPV between two groups on the 2nd，3rd，7th days of hospitalization and discharge（ P＞0.05）.In addition，compared with the admission，children in improvement group had significantly higher PLT count on the 7th day of hospitalization and before discharge（ P＜0.05），but there was no significant change in unrecovered group（ P＞0.05）.Compared with SCAP patients with thrombocytopenia at admission (PLT＜100×10 9/L)( n=22),those with thrombocytopenia on 7th day of hospitalization had a significant higher rate of non recovery( P＜0.05). Conclusion:The occurrence of thrombocytopenia on admission and after 7 days of hospitalization in children with SCAP is associated with poor prognosis.No significant increase or decrease in PLT count after 7 days of hospitalization is often indicative of poor prognosis.Dynamic monitoring of PLT parameter changes may help to better judge the prognosis of severe pneumonia.
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Objective:To illustrate the onset of epileptic seizures in children with brain injury admitted in pediatric intensive care unit (PICU), and to explore the risk factors and the correlation between epileptic seizures and the prognosis.Methods:Clinical data of pediatric patients with brain injury who were admitted to PICU of Peking University First Hospital from January 2013 to December 2019, and monitored by video electroencephalography (VEEG) were retrospectively collected, including general demographic information, etiological data, clinical seizures prior to VEEG performing, in-hospital mortality, brain function score, VEEG manifestations, etc.Seizures detected by VEEG were the primary outcome, and prognosis when discharged from PICU was the secondary outcome. Logistic regression was used to analyze the factors associated with seizures and poor outcome. Results:A total of 284 children were included, involving 54.9%(156/284) males.The median age of included children was 1.7(0.5, 5.0) years.Stratified by the cause of disease, 45.1%(128/284) had epilepsy, 26.1%(74/128) had genetic metabolic disease, and 14.4%(41/284) had central nervous system infection.A total of 82.0%(233/284) children had abnormal VEEG background activities, and 59.5%(169/284) had interictal epileptic discharges.Seizures were detected in 106 cases, including 39.6%(42/106) of non-convulsive seizures and 24.5%(26/106) of non-convulsive epileptic states.There were 12.0% (34/284) had poor prognosis at discharge, including 24 patients died in-hospital, and Pediatric Cerebral Performance Category scores were increased in 10 survivors.Multivariate Logistic regression analysis showed that seizures existed before VEEG monitoring and interictal epileptiform discharge were the independent risk factors for seizure.Besides, mental retardation, sepsis related encephalopathy, consciousness abnormality during VEEG, abnormal VEEG background activity, and epileptic status were significantly correlated with the poor prognosis of children with brain injury in PICU. Conclusions:The incidence of electrographic seizure is higher in children with brain injury in PICU, and VEEG monitoring is beneficial to children with brain injury that achieves an early identification of seizures and prediction of prognosis.
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Objective:To analyze the difference of clinical characteristics and outcomes of infants with moderate and severe pediatric acute respiratory distress syndrome(PARDS)diagnosed according to baseline oxygenation index(OI) in pediatric intensive care unit(PICU).Methods:Second analysis of the data collected from the "Efficacy of pulmonary surfactant (PS) in the treatment of children with moderate and severe ARDS" program.Retrospectively compare of the differences in clinical data such as general condition, underlying diseases, OI, mechanical ventilation, PS administration and outcomes among infants with moderate and severe PARDS divided by baseline OI who admitted to PICUs at 14 participating tertiary hospitals from 2016 to December 2021.Results:Among the 101 cases, 55 cases (54.5%) were moderate and 46 cases (45.5%) were severe PARDS.The proportion of male in the severe group (50.0% vs.72.7%, P=0.019) and the pediatric critical illness score(PCIS)[72 (68, 78) vs.76 (70, 80), P=0.019] were significantly lower than those in the moderate group, while there was no significant difference regarding age, body weight, etiology of PARDS and underlying diseases.The utilization rate of high-frequency ventilator in the severe group was significantly higher than that in the moderate group (34.8% vs.10.9%, P=0.004), but there was no significant difference in PS use, fluid load and pulmonary complications.The 24 h OI improvement (0.26±0.33 vs.0.04±0.34, P=0.001) and the 72 h OI improvement[0.34 (-0.04, 0.62) vs.0.15 (-0.14, 0.42), P=0.029)]in the severe group were significantly better than those in the moderate group, but there was no significant difference regarding mortality, length of hospital stay and intubation duration after diagnosis of PARDS between the two groups. Conclusion:In moderate and severe(divided by baseline OI) PARDS infants with invasive mechanical ventilation, children in severe group have better oxygenation improvement in the early stage after PARDS identified and are more likely to receive high frequency ventilation compared to those in moderate group.Baseline OI can not sensitively distinguish the outcomes and is not an ideal index for PARDS grading of this kind of patient.
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Objective:To analyze the clinical characteristics of children aged two years old and above with respiratory syncytial virus (RSV) infection in pediatric intensive care unit (PICU).Methods:Children who had RSV infection admitted to PICU at Children′s Hospital of Zhengzhou University from March 2019 to December 2021 were divided into older age group(≥two years old) and younger age group(<two years old) according to age, and the differences in general information and common clinical manifestations between two groups were compared.The results of other positive pathogens, major treatments and outcomes were collected simultaneously for children in older age group.Results:(1) A total of 226 cases were included, including 193(85.4%) cases in younger age group and 33(14.6%) cases in older age group, 2-6 years old 25(75.8%) cases; the incidence of combined underlying diseases did not differ between two groups ( P>0.05); In younger age group, there were 132(64.8%) cases of fever and 125(64.8%) cases of wheezing; In older age group, there were 31(93.9%) cases of fever and 13(39.4%) cases of wheezing; the incidence of fever was higher in older age group than that in younger age group( χ2=9.147, P<0.05), and the incidence of wheezing was lower than that in younger age group( χ2=7.631, P<0.05), and the differences were statistically significant.(2) There were 15 (45.5%) cases of simple infection and 18 (54.5%) cases of mixed infection in older age group, and the P/F was significantly different between children with simple infection and those with mixed infection ( P<0.05); there were 14 (42.4%) cases of combined underlying diseases and 19 (57.6%) cases without underlying diseases, and the length of stay in PICU was different between children with combined underlying diseases and those without underlying diseases ( P<0.05), and there was no significant difference in mechanical ventilation or total length of stay ( P>0.05). Conclusion:RSV infection in children aged two years old and above in PICU occurres mostly between two and six years of age, with a relatively low incidence of wheezing and common fever; RSV infection alone may have a greater impact on oxygenation, and combined underlying diseases can affect the length of stay in PICU.
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Objective:To evaluate the relationship between the Panton-Valentine leukocidin (pvl) strain and clinical characteristics, and to describe the molecular biological characteristics of invasive Staphylococcus aureus ( S. aureus) infected clinical isolates. Methods:The isolates of S. aureus caused by invasive infection were collected in Beijing Children's Hospital Affiliated to Capital Medical University from January 2016 to December 2019, and the clinical data of the corresponding children were collected retrospectively using electronic medical records. Multilocus sequence typing, spa typing and pvl gene were analyzed using the PCR. In addition, the minimum inhibitory concentrations (MIC) of antibiotics of all isolates were detected by the micro-broth dilution method, and the isolates were divided into the pvl+ and pvl- groups according to whether or not the S. aureus isolates carried pvl. The t test and the Mann-Whitney U test were used to compare the clinical symptoms between the pvl+ and pvl- groups. Chi-square test was used to compare the drug susceptibility between the two isolates. Results:A total of 127 cases of invasive S. aureus infection were collected during the study period. The white blood cell count, neutrophil count, and C-reaction protein level in the pvl+ group were significantly higher than those in the pvl- group ( P=0.001, P=0.001, P=0.005). The rate of pvl carrier was 44.9%. Among 57 pvl+ pathogenic strains, 64.9% (37/57) were MRSA. The multidrug resistance rate of pvl- isolates was higher than that of pvl+ isolates (70% vs. 49.12%, P=0.02). Conclusions:In invasive S. aureus infection, pvl+ strain is associated with elevated inflammatory markers in children. the positive rate of pvl is higher in clinical isolates, and the multidrug resistance rate of pvl- S. aureus is higher.
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Objective:To analyze the prognosis and influencing factors of acute necrotizing encephalopathy (ANE) in children.Methods:Clinical data of ANE patients admitted to Pediatric Intensive Care Unit, Beijing Children′s Hospital, Capital Medical University from March 2012 to February 2019 were retrospectively analyzed.Survivors were followed up by telephone or outpatient department, and the quality of life was evaluated by pediatric overall performance category scale.The t-test or rank sum test was used for comparison between groups, and the COX risk regression was used to analyze the influencing factors of prognosis. Results:A total of 38 patients were enrolled in this study with the male-to-female ratio of 1.24∶1.00, and median age of 29.5(10.0-130.0) months.They were followed up for the median of 27(15-96) months.The overall survival rate at 7 days, 14 days and 2 months after disease onset were 57.9%, 42.1%, and 34.2%, respectively.The mortality rate at discharge was 34.2%(13/38 cases), and the cumulative mortality rate at the 1 st, 3 rd and 12 th months after discharge was all 68.4%(26/38 cases). The complete reco-very rate was 10.5%(4/38 cases) after one-year follow-up.The univariate analysis indicated that cardiopulmonary resuscitation before admission, Glasgow coma score < 5 at admission, complication with shock/cerebral hernia/multiple organ dysfunction syndrome, creatine kinase isoenzyme> 100 U/L, lactate dehydrogenase>1 000 U/L, hypoalbuminemia, hyperglycemia, hyperurea, prolonged prothrombin time and elevated international standardized ratio were risk factors for the prognosis of ANE in children ( β=3.519, 6.967, 6.803, 3.000, 6.389, 3.471, 2.252, 1.616, 2.377, 3.092, 2.713, and 4.510, respectively, all P<0.05). Meanwhile, high-dose Methylprednisolone[20-30 mg/(kg·d)] and immunoglobulin (2 g/kg, divided into 2-5 days intravenous drip) treatment were protective factors ( β=0.625, 0.405, respectively, all P<0.05). The COX multivariate analysis showed that high-dose Methylprednisolone treatment [20-30 mg/(kg·d)] was an independent protective factor for the prognosis of children with ANE [95% CI: 0.449(0.213-0.944), P=0.035]. Conclusions:Early application of high-dose Methylprednisolone and immunoglobulin may contribute to the good clinical outcome.Children with neurological sequelae should be actively treated with rehabilitation, and the quality of life may be gradually improved.
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The number of children receiving prolonged mechanical ventilation has increased significantly in recent years.Nutritional support is very important to improve the prognosis of children on prolonged mechanical ventilation.But there is a lack of research, expert consensus or guidelines on nutritional support for children on prolonged mechanical ventilation now.This review summarized the process and strategy of nutrition support for children with prolonged mechanical ventilation based on the current research results.Multidisciplinary collaboration helps improve nutritional support outcomes.A large number of high-quality studies are needed to provide evidences to develop more rational and standardized nutritional support strategies.
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Pharmacokinetics/pharmacodynamics study fully considers the relationship among pathogens, hosts and drugs, which reflects the relationship between bactericidal effects and adverse drug reactions and the change of drug concentrations, which is of much value to the rational use of antimicrobial agents and delaying antimicrobial resistance.This review discussed design and optimization of dosing regimens for anti-infective therapy base on theory of pharmacokinetics/pharmacodynamics.
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Objective:To compare the status of pediatric intensive care unit(PICU) in general hospitals, children′s hospitals and hospitals for maternal and child health care in China, and provide the basis for standardizing and improving the construction of the PICUs.Methods:The survey was a multicenter, cross-sectional study about the PICU among 126 hospitals in Chinese mainland conducted by online questionnaire from January 1, 2014 to December 31, 2014.Results:In PICUs at general hospitals, children′s hospitals, and hospitals for maternal and child health care, the median number of beds was 14.5, 27.0 and 15.0, respectively, and the median doctor/bed ratio was 0.47, 0.43 and 0.44, respectively.PICUs in three types of hospitals were equipped with monitors and ventilators, and the expansion rate of invasive ventilators was 100%.The proportion of PICU with national continuing education classes, the development rate of two-stage resident training programs and regular examinations for critical care professionals in children′s hospitals was 67% and 75%, respectively, which were much higher than those at general hospitals(20%, 50%) and hospitals for maternal and child health care(30%, 45%), the differences were statistically significant( P<0.05). The year-round median bed utilization rate was more than 85%, especially for children′s hospital, which reached to 100%. Conclusion:In PICUs, the number of beds cannot meet the clinical needs, and the number of medical staff is insufficient.Compared with PICUs of general hospitals and hospitals for maternal and child health care, PICUs in children′s hospitals are more comprehensive in equipment configuration, personnel training and assessment system.
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Pneumonia is the most common cause of pediatric acute respiratory distress syndrome.Pneumonia combined with pediatric acute respiratory distress syndrome due to different pathogens has certain characteristics, and clinicians should pay attention to individualized anti-infective, anti-inflammatory and respiratory support therapy in the diagnosis and treatment.
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Objective:To explore the efficacy of andrographolide sulfonate(ADS) nebulized inhalation on pediatric moderate to severe acute respiratory distress syndrome(ARDS) requiring invasive mechanical ventilation.Methods:We conducted a prospective randomized controlled single-blind study.Children with moderate-to-severe ARDS admitted to the PICU at Beijing Children′s Hospital of Capital Medical University from November 1, 2018 to December 31, 2019, aging from 29 days to 18 years, and requiring invasive mechanical ventilation therapy were collected.The experimental group received ADS, while the control group received normal saline.Bronchoalveolar lavage fluid was collected to detect cytokines before and after the experiment.The differences of demography, cytokines and management between two groups were analyzed.Results:Twenty children with a median age of 2.15(1.48, 8.01)years were included and 15(75.00%)cases were boys.Median score of pediatric index of mortality-2 was 12.25(4.53, 16.30). There was no significant differences in demography, basic clinical data and prognosis between two groups( P>0.05). Monocyte chemoattractant protein-1 decreased in the experimental group while increased in the control group with statistic difference[967.50(119.25, 5 206.00)pg/mL vs.-945.00(-3 935.50, 495.09)pg/mL, P=0.041]. Interleukin(IL)-8 decreased in the experimental group but increased in the control group[303.22(-452.00, 1 172.38)pg/mL vs.-490.14(-780.25, 240.52)pg/mL, P=0.151]; and the IL-6 increase of the experimental group was lower than that of the control group[-24.53(-501.76, 135.27)pg/mL vs.-325.85(-633.22, 133.75)pg/mL, P=0.364]; all with no statistic differences( P>0.05). The oxygenation index[11.35(6.00, 15.83) vs.20.65(6.23, 38.35), P=0.374] and the improvement rate of ARDS(80%vs.60%, P=0.628) of the experimental group was better than that of the control group, but with no statistic difference( P>0.05). There was no statistic difference of mortality and mechanical ventilation time between two groups( P>0.05). Conclusion:Inhalation of ADS might reduce the increase of IL-6 and the concentration of monocyte chemoattractant protein-1 and IL-8 in bronchoalveolar lavage fluid of children with ARDS, and might improve pulmonary oxygenation function.Further research is needed to verify the above conclusion.
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Objective:To describe the treatment strategies in children with septic shock in China.Methods:A questionnaire was prepared and 368 pediatric intensivists from the Pediatric Critical Care Physician Branch of Chinese Medical Doctor Association were surveyed about the treatment of pediatric septic shock from April to June 2017.Results:Surveys were received from 87.2%(68/78) institutions and 368 questionnaires (response-rate 45.1%) were included.59.2% and 77.7% of the respondents chose debridement surgery and fluid drainage as source control intervention.Antibiotics were used within 1 hour of shock in 90.8% of respondents.98.4% of respondents chose normal saline, 72.3% of respondents chosen albumin, and 53.8% of respondents chosen plasma for fluid resuscitation.When no venous access was available during shock resuscitation, 57.1% of respondents preferred intraosseous access.79.3% and 83.2% of the respondents used the adjuvant therapy such as glucocorticoids and intravenous immunoglobulin.96.7%, 85.3% and 22.0% of respondents were likely to provide oxygen and mechanical ventilation, continuous renal replacement, and extracorporeal membrane oxygenation as organ support, respectively.Additionally, 322 (88.7%), 188 (51.1%), and 85 (23.1%) respondents chose the "best advice" options to simulated clinical cases of fluid resuscitation, inotropic agents, and vasoactive agents, respectively.In the simulated cases of vasoactive drugs and inotropic drugs, 69.3% and 24.2% of the respondents chose fluid resuscitation strategy, respectively.In cases of fluid resuscitation, 49.7% (183/368) of respondents reported performing fluid responsiveness and volume status assessment, and instruments used in the assessment included bedside echocardiography[39.4% (145/368)], bioreactance[10.3% (38/368)], transpulmonary thermodilution devices[6.3% (23/368)]. Pediatricians who received advanced life support courses for children ( P=0.006) and intensive care specialist training center training ( P=0.002) were more likely to choose the " best recommendation" option than those who did not attend the training. Conclusion:The current status of pediatric septic shock treatment strategies in China are active source control intervention, antibiotic use and organs support, and increased awareness of non-invasive hemodynamic monitoring.However, there may be excessive fluid infusion and inappropriate use of plasma, glucocorticoids and intravenous immunoglobulin.Different training and continuing education may improve rational treatment strategies.
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Objective:To investigate the safety and efficacy of moxifloxacin in children with severe Mycoplasma pneumoniae pneumonia (SMPP).Methods:The patients with SMPP in the Pediatric Intensive Care Unit of Beijing Children's Hospital between January 2017 and April 2020 were retrospectively analyzed. Clinical data were collected to assess therapeutic efficacy, analyze drug safety and summarize positive rate of macrolide-resistant Mycoplasma pneumoniae genes mutation.Results:Thirty-nine children diagnosed SMPP treated with moxifloxacin were included. The positive rate of macrolide-resistant Mycoplasma pneumoniaegenes mutation was 95.2%. In the 39 patients, 6 (15.4 %) were cured, 29 (74.4 %) were effective, 2 (5.1 %) were no response, 2 (5.1 %) were discharged automatically during treatment with moxifloxacin, and the overall response rate was 89.8 %. The situations of consciousness, skin, joint, heart rhythm and gastrointestinal function were carefully observed; Blood routine test, liver and kidney function were closely monitored. There were no adverse drug reactions in the period of medication. No children were discontinued due to adverse reactions.Conclusions:Moxifloxacin can improve efficacy and prognosis for pediatric patients with SMPP. There are no drug adverse reactions during treatment with moxifloxacin, indicating that short-term medication is safe. The application of quinolones in pediatric patients is off-label drug use, and clinical pharmacists should assist clinicians in reducing medical risks.
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Disseminated intravascular coagulation (DIC) is a common and critical complication in children with acute leukemia.Acute promyelocytic leukemia (APL) is the most-common subtype of acute leukemia complica-ted with DIC.Although all-trans retinoic acid plus chemotherapy has significantly improved the overall survival of children with APL, the incidence of DIC-induced early mortality has not been improved.This review summarizes the advances on the pathogenesis and treatment of DIC in children with acute leukemia, aiming to provide evidences for clinical management.
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Objective:To identify clinical characteristics of slit ventricle syndrome (SVS) in children, thus improving pediatricians′ understanding of pediatric SVS.Methods:Clinical data of children diagnosed as SVS in the Emergency Department of Beijing Children′s Hospital, Capital Medical University from June 2015 to May 2019 were collected and retrospectively analyzed.Results:A total of 10 children with SVS were included, including 3 cases of arachnoid cyst, 3 cases of congenital hydrocephalus, 2 cases of Dandy-Walker syndrome, 1 case of idiopathic intracranial hypertension syndrome, and 1 case of hydrocephalus secondary to intracranial hemorrhage as the primary disease.The age of first shunting, and that at diagnosis of SVS were 1.3 (0.3-12.8) years, and 9.9 (3.8-13.3) years, respectively.SVS-associated symptoms appeared in 4.4 (0.5-12.0) years after shunting, including intermittent headache (10/10 cases), vomiting (10/10 cases), irritability (4/10 cases), seizures (5/10 cases), diminution of vision (2/10 cases), and intracranial hypertension (10/10 cases) at varying severities.When SVS occurred, the cerebral ventricle presented slit-like morphology.Three cases were relieved with mannitol treatment and 7 cases were treated with emergency lumbar-peritoneal shunt after mannitol failure.During 1-5 years of follow-up, 9 patients did not have SVS-associated symptoms and 1 case with medical therapy had recurrences.Conclusions:SVS is a rare complication after shunt surgery, with the clinical manifestations of intermittent headache and slow valve refilling conforming; imaging showed that the cerebral ventricle was slit-like morphology.When there are signs of intracranial hypertension after shunting and there is no cerebral ventricle dilatation in imaging, SVS should be highly vigilant.Once SVS is confirmed, surgical intervention should be introduced as early as possible after treatment failure of medication, so as to improve the prognosis.
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Acute necrotizing encephalopathy (ANE) is a rare but distinctive acute encephalopathy with global distribution.It usually begins with a virus-associated febrile illness, which deteriorates rapidly, and leading to convulsion, unconsciousness and even coma.The neuroradiologic features of ANE are multi-focal and symmetrical brain lesions, involving bilateral thalamus.ANE has no specific treatment method at present.The majority of ANE patients are accompanied with varying degrees of neurological sequelae, and even death.In this review, we summarized the epidemiology, diagnosis and prognosis of this rare but fatal disease in children.
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Objective:To investigate the resting energy expenditure of children with mechanical ventilation and analyze their association with anthropometric indicators (height, weight, and body mass index) and serum protein markers (pre-albumin, albumin, transferrin, and retinol).Methods:An observational study was held in Pediatric Intensive Care Unit(PICU) of Beijing Children′s Hospital, Capital Medical University, from August 2015 to April 2016.Critically ill children with mechanical ventilation were enrolled in this study.Inclusion criteria were as follows: (1)pediatric critical illness score<90 scores or meet the United States PICU admission criteria; (2)age>29 days, <18 years old; (3)mechanical ventilation>24 hours; (4) tidal volume >60 mL.Resting energy expenditure was determined by US Med Graphic Company CCM/D energy metabolism test system.German Famed Zywier intensive care bed with weight scale weighing, uniform measuring bed or meter measuring height, automatic biochemical analyzer and radioimmunoassay was adopted for the determination of albumin, pre-albumin, transferrin, retinol binding protein.SPSS 20.0 software was applied for carrying out Pearson line analysis correlation. Results:A total of 68 children were included in the study, including 36 males and 32 females, body weight (22.34±14.89) kg, height (122.23±14.89) cm, resting energy consumption (784.63±278.25) kcal(1 kcal=4.185 kJ), and resting energy consumption per kilogram of body weight (48.80±31.26) kcal.In anthropometric indicators, resting energy expenditure was significantly positively correlated with height, body weight ( r=0.764, 0.734, all P<0.05), a low positive correlation with body mass index( r=0.396, P<0.05). In serum proteomics, resting energy expenditure was negatively correlated with pre-albumin, transferrin ( r=-0.318, -0.282, all P<0.05), and a moderate negative correlation with retinol protein ( r=-0.505, P<0.05), while without correlation with albumin ( r=-0.075, P>0.05). Conclusions:The height, weight and serum proteomics of children with mechanical ventilation may affect resting energy expenditure.
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Mycoplasma pneumoniae (MP) is the most common pathogens of community-acquired pneumonia in children.Macrolides are the first-line antibiotics for mycoplasma pneumoniae pneumonia (MPP) treatment.However, in recent years, with macrolide resistant MP (MRMP) becoming prevalent, refractory MPP (RMPP) and fulminant MPP (FMPP) increased significantly.It has become a hot issue for clinicians.This paper reviewed the recent studies on MRMP, RMPP and FMPP, discussed the diagnosis and treatment options for MRMP, RMPP and FMPP in detail.Prioritizing the selection of sensitive antibiotics and early initiation of effective antibiotic therapy, appropriate use of immunoregulatory and anti-inflammatory therapies, and aggressive treatment of complications and co-infections are the keys to RMPP and FMPP therapy.
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Objective:To investigate the clinical features and prognosis of septic shock(SS) children with different basic diseases in pediatric intensive care unit (PICU).Methods:The medical records of SS children admitted to PICU at Beijing Children′s Hospital from January 1, 2017 to December 31, 2019 were collected retrospectively.They were grouped according to the presence or absence of basic diseases and types of basic diseases.The clinical characteristics, prognosis and pathogens of SS under different basic diseases were summarized.Results:A total of 218 children with SS were included during the study period, and the overall case fatality rate was 21.6%(47/218). There were 141 cases with basic diseases, accounting for 64.7%(141/218) and 24.1%(34/141) case fatality rate.The mortality rate was highest(37.5%, 17/45) in the malignant hematologic diseases and tumors patients with post-chemotherapy bone marrow suppression, and lowest(16.9%, 13/77) in patients with no underlying diseases.Gram-negative bacterial infection was more common in SS children with underlying diseases(63.1%, 41/65), and was highest in the malignant hematologic diseases and tumors patients with post-chemotherapy bone marrow suppression(80.0%, 20/25). Gram-positive bacteria accounted for the highest proportion in the group without underlying disease(52.1%, 25/48). The incidence of multiple organ dysfunction syndrome(MODS) was the highest(95.6%, 43/45) in the malignant hematologic diseases and tumors patients with post-chemotherapy bone marrow suppression, and the lowest(59.7%, 46/77) in the group without underlying disease.Conclusion:Gram-negative bacteria is the most common pathogen in SS children with underlying diseases, especially in malignant hematologic diseases and tumors patients with post-chemotherapy bone marrow suppression, and with high mortality and incidence of MODS.Gram-positive bacteria is the most common pathogen for those without underlying diseases, with a relatively low mortality and incidence of MODS.