A case of mitochondrial and peroxisome fission deficiency-related encephalopathy caused by DNM1L gene mutation / 中华神经科杂志

Mitochondrial and peroxisome fission deficiency-related encephalopathy caused by DNM1L gene mutation is a rare and fatal epileptic encephalopathy, with clinical phenotype and genetic heterogeneity. The acute stage is drug-resistant epilepsy with poor prognosis and serious neurological sequelae. A case of genetically confirmed encephalopathy related to mitochondrial and peroxisome fission defects is reported, the clinical data, treatment process are summarized, and the previous literature is reviewed to improve the understanding of the rare disease.

Clinical characteristics of critically ill children aged two years old and above with respiratory syncytial virus infection in PICU / 中国小儿急救医学

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.

Transfusion-related acute lung injury / 中国小儿急救医学

Transfusion-related acute lung injury (TRALI) is an acute and fatal complication of blood product transfusion.TRALI is a syndrome, which diagnosed by the basis of clinical signs.The pathogenesis of TRALI is unclear and the hypothesis of two-hit model is generally accepted.At present, there is no specific treatment for TRALI.Treatment of the patient with TRALI is mainly supportive.The rational transfusions can avoid the occurrence of TRALI.The incidence of TRALI has been decreased by making the mainly measure of avoiding transfusions of plasma from multiparous female donors.

Effect of thrombelastography on the assessment of sepsis severity and septic shock with disseminated intravascular coagulation condition in children / 中华实用儿科临床杂志

Objective To explore the effect of thrombelastography in sepsis and septic shock with disseminated intravascular coagulation (DIC) condition in children.Methods Ninety-one cases of children admitted to the Pediatric Intensive Care Unit,Zhengzhou Children's Hospital between January 2013 and December 2016 were enrolled in this study.Fifty-eight cases of sepsis,17 cases of severe sepsis and 16 cases of septic shock (including 7 cases DIC and 9 cases non-DIC) were included in 91 cases of children.After admission,they were given conventional treatment according to their condition of illness,such as expansion of rehydration,applying vascular active drags,anti-infection,mechanical ventilation,maintaining internal environment,nutrition support,etc.Thrombelastography of all the patients were detected for 6 hours after admission.The test indexes included blood coagulation reaction time (R),blood clot formation time (K) and blood clot formation rate (alpha),maximum width (MA),coagulation index (CI),etc.And pediatric critical illness scores(PCIS) were also evaluated for 6 hours after admission.Results With the progression of sepsis severity,R value,K value increased dramatically (F =3.629,4.237,all P ＜ 0.05),alpha angle,MA value,CI value decreased (F =32.631,19.938,10.849,all P ＜ 0.05);R value,K value and PCIS scores showed a significant positive correlation (r =0.591,0.827,all P ＜ 0.05),alpha angle,MA value,CI and PCIS scores showed a significant negative correlation (r =-0.793,-0.827,-0.839,all P ＜ 0.05).R and K values in DIC group were significantly greater than the values of non-DIC group (t =4.381,2.613,all P ＜ 0.05),alpha angle was less than that of DIC group obviously (t =5.627,P ＜ 0.05).In DIC group MA and CI levels were significantly less than those of non-DIC group (t =5.416,2.951,all P ＜ 0.05).R value,K value,alpha Angle,MA,CI levels between the dead and surviving patients in the septic shock group had no significant difference (all P ＞ 0.05).Conclusions TEG has a great significance in evaluating severity of children with sepsis.It can also guide clinical assessment in children with septic shock DIC so as to give accurate effective intervention and improve the rescue success rate and the prognosis.

Effect of thrombelastography on the assessment of sepsis severity and septic shock with disseminated intravascular coagulation condition in children / 中华实用儿科临床杂志

Objective To explore the effect of thrombelastography in sepsis and septic shock with disseminated intravascular coagulation (DIC) condition in children.Methods Ninety-one cases of children admitted to the Pediatric Intensive Care Unit,Zhengzhou Children's Hospital between January 2013 and December 2016 were enrolled in this study.Fifty-eight cases of sepsis,17 cases of severe sepsis and 16 cases of septic shock (including 7 cases DIC and 9 cases non-DIC) were included in 91 cases of children.After admission,they were given conventional treatment according to their condition of illness,such as expansion of rehydration,applying vascular active drags,anti-infection,mechanical ventilation,maintaining internal environment,nutrition support,etc.Thrombelastography of all the patients were detected for 6 hours after admission.The test indexes included blood coagulation reaction time (R),blood clot formation time (K) and blood clot formation rate (alpha),maximum width (MA),coagulation index (CI),etc.And pediatric critical illness scores(PCIS) were also evaluated for 6 hours after admission.Results With the progression of sepsis severity,R value,K value increased dramatically (F =3.629,4.237,all P ＜ 0.05),alpha angle,MA value,CI value decreased (F =32.631,19.938,10.849,all P ＜ 0.05);R value,K value and PCIS scores showed a significant positive correlation (r =0.591,0.827,all P ＜ 0.05),alpha angle,MA value,CI and PCIS scores showed a significant negative correlation (r =-0.793,-0.827,-0.839,all P ＜ 0.05).R and K values in DIC group were significantly greater than the values of non-DIC group (t =4.381,2.613,all P ＜ 0.05),alpha angle was less than that of DIC group obviously (t =5.627,P ＜ 0.05).In DIC group MA and CI levels were significantly less than those of non-DIC group (t =5.416,2.951,all P ＜ 0.05).R value,K value,alpha Angle,MA,CI levels between the dead and surviving patients in the septic shock group had no significant difference (all P ＞ 0.05).Conclusions TEG has a great significance in evaluating severity of children with sepsis.It can also guide clinical assessment in children with septic shock DIC so as to give accurate effective intervention and improve the rescue success rate and the prognosis.

Application of pulse indicator continuous cardiac output monitoring in the fluid management of children with acute respiratory distress syndrome / 中华实用儿科临床杂志

Objective To explore the application value of pulse indicator continuous cardiac output (PiCCO) monitoring in the fluid management of children with acute respiratory distress syndrome (ARDS).Methods Thirty-two children with ARDS admitted to Pediatric Intensive Care Unit(PICU) of Zhengzhou Children's Hospital,from April 2013 to April 2016,were divided into intervention group (15 cases) and control group (17 cases) by adopting random number table method.Fluid management of intervention group by PiCCO,control group by central venous pressure,the 2 groups' oxygenation index (OI),acute lung injury score,mechanical ventilation time and 28 days mortality were statistically compared.The categorical data were analyzed by using SPSS 11.0 software,and the t test was used for the measurement data.The categorical data and mortality comparison were analyzed by adopting x2 test.The difference was statistically significant at P ＜ 0.05.Results After 3 days of mechanical ventilation,the changes of OI in the intervention group were significantly higher than those in the control group [(175.0 ±-43.7) mmHg vs.(143.0 ± 42.8) mmHg (1 mmHg =0.133 kPa),t =2.090 0,P ＜ 0.05].The intervention group was significantly shorter than the control group [(10.45 ± 3.12) d vs.(12.63 ± 2.87) d,t =2.058 7,P ＜ 0.05].There was no significant difference between 2 groups in acute lung injury score,PICU length of stay and 28 days mortality (all P ＞ 0.05).Conclusions PiCCO monitoring and guidance in the fluid management of pediatric ARDS can improve oxygenation after 3 days,reduce mechanical ventilation time,but can not significantly reduce the 28-day mortality.

Application of fiber bronchoscopy with three-way laryngeal mask airway continuous ventilation in 1-6 month old infants with oxygen-dependent respiratory disease / 临床儿科杂志

ObjectiveTo discuss the values of fiber bronchoscopy with three-way laryngeal mask airway continuous ventilation in 1-6 month old infants with oxygen-dependent respiratory diseases.MethodsFrom January 2010 to May 2011, 29 cases of 1-6 month old infants with oxygen-dependent respiratory diseases who underwent conventional ifber bronchoscope and 148 cases of 1-6 month old infants with oxygen-dependent respiratory diseases who underwent ifber bronchoscopy with three-way laryngeal mask airway continuous ventilation were enrolled in control and treatment groups respectively. The success rate of ifber bronchoscope and the incidence rate of complications were compared between two groups.ResultsIn the treatment group, the success rate of ifber bronchoscope was 89.2% which was signiifcantly higher than 58.6% in the control group and the rate of laryngeal edema was 9.1% which was signiifcantly lower than 35.3% in the control group. The rates of endotracheal intu-bation ventilation and postoperative hemorrhage were lower than those in the control group, but the difference was not statistical ly signiifcant.ConclusionFiber bronchoscopy with three-way laryngeal mask airway continuous ventilation is superior to the traditional ifber bronchoscope in 1-6 month old infants with oxygen dependent respiratory system diseases.