Clinical features and prognosis of newborns with congenital fissure of larynx presented with choking / 中国小儿急救医学

Objective:To summarize the clinical features and prognosis of neonates with congenital fissure of larynx due to choking, and improve the diagnosis and treatment of congenital fissure of larynx by neonatal medical professionals.Methods:This study was a single-center retrospective case series report.With "choking" as the key word, combined with the information from the first page of the medical record, we searched in the electronic medical record system, and summarized the clinical characteristics and prognosis of neonates diagnosed as congenital fissure of larynx.The study period was from January 2017 to February 2021.Results:A total of seven cases were diagnosed as congenital fissure of larynx, accounting for 2.2% of the total number of hospitalized children due to choking during the same period.Among them, six cases were male, gestational age was 38 (36, 39) weeks, birth weight was 2 820 (2 255, 3 420) g, admission age was 18(5, 20) days and hospitalization duration was 26 (6, 45) days.The common clinical symptoms were choking (7/7) and cyanosis (5/7). Six of the seven children with fissure of larynx were clearly classified as type Ⅰ(two cases), type Ⅲ(three cases), and type Ⅳ(one case). All the seven cases were accompanied by other site/tracheal malformations or congenital abnormalities, among which four (4/7) cases had VACTERL syndrome.A total of three children (two cases of type Ⅲ and one case of type Ⅳ) underwent laryngeal cleft repair operation, and all died shortly after surgery.Four cases didn′t receive laryngeal cleft repair operation (two cases of type Ⅰ, one case of type Ⅲ, one case of parting was unknown), one case with type Ⅰ fissure of larynx accepted the tracheoesophageal fistula repair operation only, who was followed up until the age of two years and six months, and he could have normal diet, one case of typeⅠfissure of larynx with gastric tube discharge, who was followed up to one year and eight months old, could be normal diet too, the two cases of children with no difference between the growth with their peers; one case with type Ⅲ fissure of larynx was lost to follow-up; One case, whose classification was unknown, was followed up until he was 40 days old, and still could be fed orally.The physical development of the child was significantly behind that of the same age.Conclusion:Congenital fissure of larynx is rare, with choking as its main symptom and other malformations.Diagnosis and classification require fiberlaryngoscope and bolting laryngoscope under general anesthesia combined with bronchoscopy, which is difficult to treat.Type Ⅰ has a good prognosis, while type Ⅲ and Ⅳ have poor prognosis.

Clinical study on the treatment of acute attack of chronic obstructive pulmonary disease with self-made Wenfei-Quyu-Huatan Decoction combined with conventional western medicine / 国际中医中药杂志

Objective:To evaluate the clinical effect of self-made Wenfei-Quyu-Huatan Decoction combined with conventional western medicine in the treatment of acute attack of chronic obstructive pulmonary disease (COPD). Methods:A total of 103 patients with acute attack of COPD who were admitted to our hospital from January 2018 to April 2019 were divided into control group (51 cases) and the intervention group (52 cases) according to the random number table method. The control group was treated with conventional western medicine, and the intervention group was treated with self-made Wenfei-Quyu-Huatan Decoction based on control group. Both groups were treated for 14 days. TCM syndrome scores were recorded before and after the treatment, the FVC, FEV1 and FEV1/FVC were detected by pulmonary function meter, the content of TNF-α, IL-8 and CRP in serum were detected by ELISA, and adverse reactions during the treatment were recorded and clinical efficacy was evaluated. Results:The total effective rate was 88.5% (46/52) in the intervention group and 70.6% (36/51) in the control group, and the difference between the two groups was statistically significant ( χ2=5.067, P=0.024). After the treatment, the TCM syndrome score of the intervention group was significantly lower than the control group (7.3 ± 0.9 vs. 12.9 ± 1.2; t=27.979, P<0.01); FVC (2.69 ± 0.35 L vs. 2.12 ± 0.24 L, t=9.621), FEV1 (1.58 ± 0.22 L vs. 1.13 ± 0.18 L, t=11.349) and FEV1/FVC (0.59 ± 0.07 vs. 0.53 ± 0.08, t=4.053) in the intervention group were significantly higher than the control group ( P<0.01); the serum IL-8 (51.48 ± 6.27 ng/L vs. 69.27 ± 7.32 ng/L, t=13.255), CRP (2.12 ± 0.46 mg/L vs. 3.51 ± 0.53 mg/L, t=14.223) and TNF-α (41.38 ± 6.22 ng/L vs. 52.24 ± 7.29 ng/L, t=8.139) level were significantly lower than the control group ( P<0.01). During the treatment, the incidence of adverse reactions was 11.8% (6/51) in the control group and 15.4% (8/52) in the intervention group, and there was no significant difference between the two groups ( χ2=0.321, P=0.571). Conclusions:The self-made Wenfei-Quyu-Huatan Decoction combined with the conventional western medicine can improve the clinical symptoms, lung function and reduce the level of inflammatory cytokines in patients with COPD acute attack.

Gene mutation and treatment of neonatal congenital hyperinsulinism / 中国新生儿科杂志

Objective To summarize the gene mutation,treatment and prognosis of neonatal congenital hyperinsulinism (CHI) cases.Method The gene mutation and treatment of newborn infants who were admitted to the neonatal center of Beijing Children's Hospital between February 2015 and February 2018 with confirmed CHI diagnosis and whole exome sequencing (WES) results were retrospectively summarized.Result Totally 8 infants (5 boys and 3girls) were included.The onset age was (13.2±6.1) h (2 h ～ 2 d).The main clinical presentations were inactivity,seizures and poor feeding.Among the 8 patients,3 were positively responded to diazoxide,5 were negative and then been treated by octreotide.Among these 5 cases,3 were positively responded to octreotide (with stable blood glucose level after discharged home and till 6 months of age) and 2 were negative to both diazoxide and octreotide (1 had partial pancreatectomy afterwards but still had 1～2 episodes pf hypoglycemia till 6 months post-operational periods,another 1 died with unknown detail information soon after the parents quit further medical intervention).All 8 cases had heterozygous gene mutations.7 out of the 8 cases were ABCC8 gene (encoding islet beta-cell potassium channel protein sulfonylurea receptor protein SUR1) mutation on different sites including c.4414G＞A,c.221G＞A,c.1773C＞G,c.4352T＞C,c.2924-9C＞T,c.4198+ 1G＞T,c.2509C＞T,c.2331G＞A,c.4316A＞G and c.47G＞C.Among these 7 cases,3 were complicated heterozygous mutations with completely no response to diazoxide (2 out of the 3 were positively responded to octreotide,1 out of the 3 was negative to octreotide)and the other 4 were simple heterozygous mutations (2 out of the 4 were positively responded to diazoxide,1 out of the 4 was negative to diazoxide but positive to octreotide,and 1 out of the 4 was negative to both diazoxide and octreotide).1 out of the 8 cases was heterozygous GLUD1 gene mutation at c.1388A＞T site and had positive therapeutic responses to diazoxide.All 5 out of the 8 cases with negative therapeutic response to diazoxide had ABCC8 gene mutation.Conclusion ABCC8 gene encoding islet beta-cellpotassium channel protein sulfonylurea receptor protein SUR1 is the most common gene to have mutation io CHI.Mutations at multiple sites of this gene may cause CHI.Various therapeutic responses to medications might be related to different types of gene mutation.

Newborn arrhythmia with cardiac enlargement: etiology and clinical features / 中国新生儿科杂志

Objective To study the etiology and clinical features of arrhythmia in neonates with cardiac cardiomegaly.Method A retrospective study was conducted on newborns with arrhythmia hospitalized in the neonatal intensive care unit of our Hospiatal from January 2016 to May 2018.The eases were assigned into arrhythmia with cardiac enlargement group and non-benign arrhythmia without cardiac enlargement group cardiomegaly.The etiology,clinical features and prognosis of newborns with arrhythmia and cardiac enlargement were analyzed.Result There were 15 newborns with arrhythmia accompanied with cardiac enlargement,all of them were non-benign arrhythmias,which accounted for 26.8％ (15/56) of all non-benign arrhythmias in the same period.The causes included infections viral myocarditis,pneumonia and digestive tract infection,electrolytes and acid-base imbalance hypocalcemia,hyperkalemia and metabolic acidosis,structural cardiac abnormalities,metabolic cardiomyopathy,nodular sclerosis and indwelling central venous catheter.The median age of onset was 9 (0,19) days,and the median medical history was 2 (1,7) days,the significance of which were not different from those of non-benign arrhythmias without cardiac enlargement group (41 cases).The rate of cesarean section and ventricular rate＞160 bpm at admission were higher than those in the non-benign arrhythmias without cardiac enlargement group.12 of 15 cases began with weak responses,poor appetite and dyspnea.The common arrhythmia types were paroxysmal supraventricular tachycardia (6 cases),atrial tachycardia (4 cases) and atrial flutter (4 cases).The proportion of atrial flutter was higher than that of the non-benign arrhythmias without cardiac enlargement group.8 cases received respiratory support.The average length of stay was (18.4±8.6) days.The mortality rate during hospitalization was 6.7％.The echocardiographic results showed that left atrioventricular enlargement and ejection fraction of left ventricle less than 60％ during arrhythmic episode.The cardiac size and systolic functions of left ventricle reverting to normal after improvement.During the follow-up period,1 case died and 2 cases lost to follow-up,all of them were discharged against medical advice.The median follow-up time of other cases was 1.6 (1.3,2.2) years.All of them recovered well without abnormalities in cardiac size or cardiac function by echocardiography.Conclusion Most newborns with arrhythmia accompanied by cardiac enlargement had insidious onset.Tachyarrhythmia was common and infection was the main cause.Attention should be paid to screening of rare causes in clinical practice.Non-benign arrhythmias with or without cardiac enlargement have no significant difference in the overall prognosis by active treatment.

Establishment of a new congenic inbred mouse strain named Foxn1nu.B6-CAG-EGFP/SU / 中国比较医学杂志

Objective To establish a new congenic inbred mouse strain carrying and expressing EGFP and Foxn1nugene for cancer research including human glioma as well .Methods According to criterion of GB14923-2010, the male Foxn1nu nude mice backcross the female C57BL/6-Tg (CAG-EGFP) transgenic mice for 10 times, Then identify the phenotype using the methods and equipment as below: fluorescent flashlight and matching glasses; multifunction vivo imager; fluorescence microscopy.Results The congenic inbred mouse strain named Foxn1nu.B6-CAG-EGFP/SU ( Soochow University ) .All the 14 biochemical loci are homozygous and same with Balb/c mouse in addition to the Pep3 loci (“b” type instead of “a” type).Peripheral blood lymphocyte count shows the lymphocytes occupy 15%of nucleated cells;T lymphocytes occupy 0.3%, meet the requirement of inbred strain of EGFP nude mice .Conclusions Established a new congenic inbred strain -Foxn1nu.B6-CAG-EGFP/SU which both express EGFP stably, and own immunodeficiency with lack of T lymphocytes .The phenotype “b” of biochemical loci “Pep3” is the unique characteristic that distinguish SU to Foxn1nu.

Mechanism of bone marrow-derived mesenchymal stem cell-promoted apoptosis of hepatic stellate cells / 中华肝脏病杂志

<p><b>OBJECTIVE</b>To explore the role of the Rho pathway in the hepatocyte growth factor (HGF) paracrine signal-mediated bone marrow-derived mesenchymal stem cell (BMSC) promotion of apoptosis of hepatic stellate cells (HSCs).</p><p><b>METHODS</b>A BMSC-HSC co-culture system was established using plates with transwell inserts. Dynamic changes in response to pretreatment with the c-met blocker PHA665752 and the Rho pathway inhibitor Y-27632 were observed under an inverted phase contrast microscope at 24, 48 and 72 h of culture. Optimal intervention concentrations of Y-27632 and PHA665752 were determined by MTT assay. Expression of alpha-smooth muscle actin in HSCs was evaluated by immunohistochemistry, and the apoptosis rate of HSCs was measured by Annexin-V-FITC/propidium iodide. RhoA protein and mRNA levels were measured by western blot and quantitative real-time PCR respectively. Concentrations of HGF and hepatocyte growth factor activator (HGFA) were quantified by enzyme-linked immunosorbent assay. Between-group differences were evaluated by one-way ANOVA with P less than 0.05 indicating significance.</p><p><b>RESULTS</b>The apoptosis rates of HSCs gradually and steadily increased in a time-dependent manner. The apoptosis rate of the PHA665752 pretreated group was lowest and that of the Y-27632 pretreated group was highest, with the most robust difference occurring at the 72 h time point (P less than 0.05). The mRNA and protein expression levels of RhoA decreased in a time-dependent manner in the Y-27632 pretreated group (all time points, P less than 0.05) but the expression levels increased in a time-dependent manner in the PHA665752 pretreated group (all time points, P less than 0.05). For both the PHA665752 and the Y-27632 pretreated groups, the concentration of HGF decreased in a time-dependent manner, but the concentrations in both remained significantly higher than that in the control group at all time points examined (P less than 0.05). The concentration of HGFA increased in a time-dependent manner, and the PHA665752 pretreated group showed significantly higher levels than any of the other groups at all time points examined (P less than 0.01).</p><p><b>CONCLUSION</b>BMSC promotes HSC apoptosis in a co-culture system by activating HGF and down-regulating the RhoA signaling pathway.</p>

Risk factors of retinopathy of prematurity / 中华急诊医学杂志

ObjectiveTo investigate the incidence and risk factors in retinopathy of prematurity (ROP) at matched gestational age.Methods Data collected by the medical records of neonates from November 2007 to December 2010 in our neonatal database were analyzed.Patients'information was recorded from birth to discharge from NICU.Data included age after birth (hours),gestational age,body weight at birth,treatment,and maternal demographics.The statistical study was carried out by SPSS version 13.0 software.Mann - Whitney U test was used for numerate data.Fisher's exact probability test and Pearson's chi - square test were used to compare quantitative variables between independent groups.P values were considered significant when they were less than 0.05 ( two - sided).ResultsThe incidence of ROP was 5.38％.Gestational age of ROP group and control group was matched ( P =0.387 ),but difference in body weight at birth was significant ( P =0.045 ).Age after birth was significantly different ( P =0.013 ).Apnea and anemia were significantly different between two groups (P ＜ 0.01). The differences in duration of hospitalization,NCPAP and use of antibiotics between two groups were significant ( P ＜ 0.002,0.000 and 0.000,respectively).ROP group differed from control group greatly in hospitalization expenses.Multiple stepwise logistic regression analysis showed that oxygen supplenentation ( P =0.0237 ) and infection ( P =0.0118) were risk factors of ROP. ConclusionsThe incidence of ROP in NICU in Beijing Children's Hospital was 5.38％.Inhalation of oxygen supplementation and infection were risk factors of ROP.

Significance of the disagreement between transmitral pulsed wave Doppler and mitral annulus tissue Doppler imaging in the estimation of left ventricular filling pressures / 中国医学影像技术

Objective To explore the clinical significance of the disagreement between transmitral pulsed wave Doppler (PWD) and mitral annulus Doppler tissue imaging (DTI) in the estimation of left ventricular (LV) filling pressures. Methods One hundred and eighty-two sinus rhythm patients without arrhythmia, congenital heart disease and valvular disease underwent routine echocardiography and synchronous electrocardiogram for assessment of LV function. Early and late diastolic velocities of LV, which were composed of e and a waves, were recorded using DTI at the mitral annulus. Six sites at the mitral annuli were selected corresponding to the septal, lateral, anterior septal, posterior, inferior, and anterior walls of LV from apical 4-, 3-and 2-chamber views. Transmitral diastolic flow velocity, which was represented by E and A wave, was measured with PWD from apical 4-chamber view. Ratio of early and late diastolic transmitral valve (MV-E/A), ratio of DTI-e/a-ann and ratio of E/e-ann were calculated, respectively. The mean value of e-ann from the above 6 sites was selected to describe the early diastolic velocities of mitral annular. Results According to the results of MV-E/A ratio and DTI-e/a-ann ratios of the 6 sites, these 182 patients were divided into 4 groups: groupⅠ(n=68): MV-E/A＜1.0, DTI-e/a-ann＜1.0 at all the 6 sites at the same time, with mean MV-E/A ratio being 0.71±0.16 and mean E/e-ann ratio 15.91±6.78; groupⅡ(n=38): MV-E/A＜1.0, DTI-e/a-ann≥1.0 at 1-6 sites among the total sites, with mean MV-E/A ratio being 0.76±0.12 and mean E/e-ann ratio 10.37±2.63; group Ⅲ(n=23): MV-E/A≥1.0, DTI-e/a-ann≥1.0 at all the 6 sites at the same time, with mean MV-E/A ratio being 1.74±0.42 and mean E/e-ann ratio 9.57±2.39; group Ⅳ(n=53): MV-E/A≥1.0, DTI-e/a-ann＜1.0 at 1-6 sites among the total sites, with mean MV-E/A ratio being 1.31±0.31 and mean E/e-ann ratio 13.27±9.46. The mean ages of group Ⅰ, Ⅱand Ⅳ were older than that of group Ⅲ. Although there was no obvious difference between group Ⅰand groupⅡ in the mean age and mean MV-E/A (P＞0.05), the mean E/e-ann was much higher in group Ⅰthan that in groupⅡ (P＜0.05). The mean MV-E/A was similar in group Ⅲ and group Ⅳ (P＞0.05), but the mean age and mean E/e-ann in the latter were older and higher than those in the former (P＜0.05), respectively. The mean age in group Ⅳ was younger than that in group Ⅰand Ⅱ, while the mean E/e-ann in group Ⅳ was higher than that in group Ⅱ, but lower than group Ⅰ (P＜0.05). Conclusion ①Ratio of MV-E/A ＜1.0 and ratios of DTI-e/a-ann ＜1.0 at all the 6 sites indicates increasing LV filling pressures; ②MV-E/A≥1.0 and DTI-e/a-ann＜1.0 at 1-6 sites among the total sites predicts a tendency of high LV filling pressures; ③Wide variability may present in those with MV-E/A＜1.0, DTI-e/a-ann≥1.0 at 1-6 sites among the total 6 sites or MV-E/A≥1.0, DTI-e/a-ann≥1.0 at all the 6 sites at the same time; ④Age of the patient has great influence on the measurement of MV-E/A ratio and DTI-e/a-ann ratio.

Two-dimensional strain echocardiography in evaluation of left ventricular twist with normal ventricular wall motion / 中国医学影像技术

Objective To evaluate the left ventricular twist in patients with coronary heart disease (CHD) with two-dimensional strain echocardiography (2DSE). Methods Thirty-eight patients with CHD and 31 control subjects proved with selected coronary angiography were enrolled. Two-dimensional images from LV short-axis views at the levels of mitral annulus and apex in 3 continuous cardiac cycles were recorded. LV ejection fraction (LVEF) was calculated with bi-plane Simpson's method, torsion and torsional rate were obtained in each plane, and the corresponding curve was accessed. Results No statistical difference of conventional echocardiographic parameters such as LV diameter, left atria diameter, LVEF, FS, E/A and E-wave deceleration time were detected, while significant difference of torsional direction (P＜0.01) and global torsion rate (P＜0.05) were found between the two groups. Conclusion The global torsional rate and torsional direction have higher sensitivity than conventional echocardiography in the diagnosis of CHD.