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.

Objective:To investigate the relationship between intra-abdominal pressure (IAP) and respiratory function in mechanically ventilated children with severe pneumonia and its prognostic value.Methods:A case-series study.Ninety-two children with severe pneumonia who were treated with mechanical ventilation in the Intensive Care Unit of Children′s Hospital Affiliated to Zhengzhou University from May 2019 to May 2021 were taken as the study subjects.The IAP and respiratory function-related indexes of the children were monitored.Those with IAP ≥12 mmHg(1 mmHg=0.133 kPa) were divided into the study group, and those with IAP <12 mmHg were divided into the control group.The difference in respiratory function related indexes between the 2 groups was compared, and the relationship between IAP and respiratory function indexes was analyzed by Pearson correlation.The children were divided into a good prognosis group and a poor prognosis group according to their prognosis.The relevant clinical data of the children in the 2 groups were collected for univariate analysis, and multivariate Logistic regression was used to analyze the risk factors affecting the prognosis of children with mechanical ventilation for severe pneumonia.A receiver operating characteristic (ROC) curve was drawn to analyze the predictive value of IAP on the prognosis of mechanically ventilated children with severe pneumonia.Results:The arterial partial pressure of oxygen (PaO 2) [(80.15±8.23) mmHg], arterial blood oxygen saturation (SaO 2) [(91.32±2.13)%], and oxygenation index (PaO 2/FiO 2) [(198.29±20.25) mmHg] in the study group were lower than those in the control group [(85.74±8.42) mmHg, (97.05±2.47)%, and (234.84±25.24) mmHg], while the respiratory rate (RR) [(56.23±2.16) breaths/min vs. (50.41±2.24) breaths/min], partial pressure of carbon dioxide (PaCO 2) [(36.48±3.72) mmHg vs.(33.29±3.46) mmHg], fraction of inspired oxygen (FiO 2) [(40.42±4.25)% vs.(36.51±3.72)%], mean arterial pressure (MAP) [(55.98±5.69) mmHg vs.(52.79±5.43) mmHg], and positive end expiratory pressure (PEEP) [(5.03±0.52) cmH 2O vs.(4.52±0.47) cmH 2O, 1 cmH 2O=0.098 kPa] were higher than those in the control group, and the differences were all statistically significant (all P<0.05).The IAP of the children in the study group was (14.25±1.83) mmHg, and that in the control group was (10.38±1.14) mmHg.Pearson correlation analysis showed that IAP was negatively correlated with PaO 2, SaO 2, and PaO 2/FiO 2 ( r=-0.615, -0.587, and -0.647, all P<0.05), and was positively correlated with RR, PaCO 2, FiO 2, MAP, and PEEP ( r=0.618, 0.634, 0.579, 0.578, 0.593, all P<0.05).IAP [(14.76±1.58) mmHg], PaCO 2 [(39.95±4.21) mmHg], FiO 2 [(50.29±5.12)%], alanine aminotransferase[(42.08±4.15) U/L], and total bilirubin [(17.92±1.87) μmol/L] in the poor prognosis group were greater than those [(10.75±1.19) mmHg, (35.37±3.64) mmHg, (45.38±4.47)%, (39.87±4.06) U/L, and (17.09±1.75) μmol/L] in the good prognosis group, while PaO 2 [(65.42±7.86) mmHg vs.(76.42±7.51) mmHg], SaO 2 [(90.65±9.26)% vs.(96.21±2.19)%], and PaO 2/FiO 2 [(130.09±15.15) mmHg vs.(168.40±20.17) mmHg] were smaller than those in the good prognosis group, and the differences were statistically significant (all P<0.05).Logistic regression analysis showed that IAP, PaO 2, PaCO 2, and SaO 2 were risk factors affecting the prognosis of mechanically ventilated children with severe pneumonia (all P<0.05).The optimal cut-off point of IAP for predicting a poor prognosis in mechanically ventilated children with severe pneumonia was 14.55 mmHg, and the area under the ROC curve was 0.873, with a sensitivity of 88.10% and a specificity of 76.00%. Conclusions:IAP has a certain relationship with the respiratory function of mechanically ventilated children with severe pneumonia, and has a good predictive value for the prognosis of the children.

Objective To investigate the effect of bronchoscopic balloon dilation treatment of different intervals on the tracheal intubation-induced subglottic stenosis in children. Methods Children with tracheal intubation-induced subglottic cicatricial stenosis were selected as the research objects. Among the 36 children with pure balloon dilation treatment, 20 cases were enrolled in the observation group and 16 cases were enrolled in the control group. In the observation group, electronic bronchoscopy was performed at 3 days after the first-time balloon dilation treatment, and those with grade 2 or higher stenosis received the second-time balloon dilation treatment. The follow-up treatment principle was as follows. If there was obvious retraction compared with the previous time and the stenosis was grade 2 or higher, the balloon dilation treatment would be performed again after an interval of 3 days; if there was no obvious retraction, the evaluation would be performed again after an interval of 2 weeks until there was no obvious retraction in the stenosis site under endoscopy for 3 times in 6 consecutive weeks, and the degree of stenosis was less than 25%, and then the electronic bronchoscopy evaluation would be stopped. In the control group, electronic bronchoscopy was performed 7 days after the first-time balloon dilation treatment, and those with grade 2 or higher stenosis received the second-time balloon dilation treatment. The follow-up treatment principle was as follows. If there was obvious retraction compared with the previous time and the stenosis was grade 2 or higher, the balloon dilation treatment would be performed again after an interval of 7 days; the evaluation method for those without obvious retraction was the same as the observation group. The follow-up duration ranged from 6 to 9 months. Results There was no significant difference in the total number of treatments and outcomes between the two groups (P>0.05). The treatment duration of the observation group was significantly shorter than that of the control group (P < 0.05). There were no significant differences in intraoperative complications such as bleeding, oxygen saturation decrease, heart rate decrease, and blood pressure decrease during anesthesia between the two groups (P>0.05). There were no significant differences in postoperative fever, aggravated laryngeal stridor, and dyspnea between the two groups (P>0.05). There were no significant differences in dyspnea, activity tolerance, and the need for long-term oxygen therapy between the two groups (P>0.05). Conclusion Most of the tracheal intubation-induced subglottic cicatricial stenosis in children has a good medium- and long-term effect through pure balloon dilation treatment. Shortening the interval of pure balloon dilation to 3 days can shorten the total treatment duration, alleviate the psychological burden of parents, but does not increase their economic burden. There are no significant differences in intraoperative and postoperative complications, outcomes, and long-term follow-up compared with the original interval of 5 to 7 days.

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(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 (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.

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.

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.

Despite the complex vascular effects of dexmedetomidine (DEX), its actions on human pulmonary resistance arteries remain unknown. The present study tested the hypothesis that DEX inhibits vascular tension in human pulmonary arteries through the endothelial nitric oxide synthase (eNOS) mediated production of nitric oxide (NO). Pulmonary artery segments were obtained from 62 patients who underwent lung resection. The direct effects of DEX on human pulmonary artery tension and changes in vascular tension were determined by isometric force measurements recorded on a myograph. Arterial contractions caused by increasing concentrations of serotonin with DEX in the presence or absence of L-NAME (endothelial nitric oxide synthase inhibitor), yohimbine (α2-adrenoceptor antagonist) and indomethacin (cyclooxygenase inhibitor) as antagonists were also measured. DEX had no effect on endothelium-intact pulmonary arteries, whereas at concentrations of 10⁻⁸~10⁻⁶ mol/L, it elicited contractions in endothelium-denuded pulmonary arteries. DEX (0.3, 1, or 3×10⁻⁹ mmol/L) inhibited serotonin-induced contraction in arteries with intact endothelium in a dose-dependent manner. L-NAME and yohimbine abolished DEX-induced inhibition, whereas indomethacin had no effect. No inhibitory effect was observed in endothelium-denuded pulmonary arteries. DEX-induced inhibition of vasoconstriction in human pulmonary arteries is mediated by NO production induced by the activation of endothelial α₂-adrenoceptor and nitric oxide synthase.
Arteries ; Dexmedetomidine* ; Endothelium ; Humans ; Indomethacin ; Lung ; NG-Nitroarginine Methyl Ester ; Nitric Oxide ; Nitric Oxide Synthase ; Nitric Oxide Synthase Type III* ; Pulmonary Artery ; Serotonin ; Vasoconstriction* ; Yohimbine

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.

Objective To determine the effect of resveratrol on constrictions of isolated human intrapulmonary arteries and its mechanisms. Methods Intrapulmonary arteries (1-1.5 mm in diameter) were dissected and cut into rings (1.8-2.0 mm in length) under microscope, and were then mounted in a Multi Myograph system. The rings were stimulated with 100 nmol/L U46619, 30 nmol/L endothelin-1, or 60 mmol/L KCl to produce sustained contraction of the intrapulmonary arteries, after which resveratrol was applied cumulatively. Endothelium denudation, L-NAME and indomethecin were used to investigate the effect of resveratrol on constrictions of the isolated arteries, suing DMSO as the control. Results Resveratrol induced concentration-dependent relaxations in endothelium-intact rings that contracted in response to stimulations with U46619, ET-1 and KCl, with pD2 of 3.82 ± 0.20, 3.84 ± 0.57, and 3.68 ± 0.27, Emax of (99.58 ± 0.83)%, 100%, and (99.65 ± 0.98)%, respectively. Treatment of the arterial rings with the eNOS inhibitor L-NAME, but not with indomethecin or endothelium denudation, obviously affected the relaxant effects of resveratrol. Conclusion Resveratrol can concentration-dependently produce relaxant effect on human intrapulmonary arteries independent of the endothelium possibly by promoting synthesis and release of NO.