Objective:To study the clinical features and genotypes of neonatal Glanzmann thrombasthenia(NGT).Methods:A male neonate with NGT admitted to the Department of Neonatology of our hospital was retrospectively reviewed. CNKI, Wangfang database, VIP, the Chinese Medical Journal Full Text database, PubMed and Embase database were searched using key words '(neonate OR newborn) AND (Glanzmann thrombasthenia)' both in English and Chinese. The clinical features and genotypes of NGT were summarized and analyzed.Results:A male full-term neonate was admitted to our hospital for mass on the forehead and ecchymosis and petechiae on the body within half an hour after birth. He gradually developed subgaleal hemorrhage and severe anemia. Platelet count, mean platelet volume and coagulation functions were normal. The platelet aggregation test indicated decreased platelet aggregation rate induced by arachidonic acid and adenosine diphosphate. Genetic testing revealed two heterozygous mutations in the patient's ITGA2B gene: NM_000419.4: c.886G>A(p.Gly296Arg) and NM_000419.4: c.2855dup(p.Phe953Valfs*83). A total of 42 literature involving 44 patients (our case included) with NGT were retrieved. 33 cases (75.0%) of NGT showed ecchymosis or petechiae on the first day after birth. For 13 cases with detailed information, 5 cases with severe anemia were given erythrocyte and plasma transfusion and platelet transfusion was given in 1 case. 4 cases had homozygous variants and 4 cases showed compound heterozygous variants. 10 cases had follow-up records, including 2 cases without any bleeding and 8 cases with varying degrees of bleeding during follow-up. No deaths were reported.Conclusions:Neonates with ecchymosis and petechiae in the early postnatal period should be suspected of NGT. Blood transfusion is preferred when the indication for transfusion is met.

Objective:To summarize the clinical characteristics of neonatal late-onset sepsis (LOS) caused by Leclercia adecarboxylata, and provide evidence for its diagnosis and treatment. Methods:We report a case of Leclercia adecarboxylata induced LOS in a male preterm neonate diagnosed by matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS) at the Affiliated Suzhou Hospital of Nanjing Medical University. Relavant literature was retrieved from Wanfang, VIP, CNKI, and PubMed databases up to April 2021, using terms including "neonate" "sepsis" and " Leclercia adecarboxylata". Results:The patient presented with dyspnea immediately after birth with gestational age of 34 +3 weeks and birth weight of 2 050 g. After admission at 14 min after birth, he was incubated at temperature of 33-35 ℃ and humidity of 50%-60% and received active treatment, consisting of nasal continuous positive airway pressure, tracheal intubation, intratracheal injection of pulmonary surfactant, invasive mechanical ventilation, and anti-infective treatment with piperacillin, cefoperazone/sulbactam, and meropenem. However, the patient developed LOS on day 11 of life and eventually died of disseminated intravascular coagulation and multiple organs failure despite volume expansion, anti-infective therapy, and respiratory support. The blood culture was positive for Gram-negative rod and confirmed as a multi-drug resistant strain of Leclercia adecarboxylata. Two cases of LOS caused by sensitive strain of Leclercia adecarboxylata in premature female infants were retrieved in the literature with atypical symptoms, of whom one was successfully treated and one died after active treatment. Conclusions:Leclercia adecarboxylata infection alone can lead to LOS in preterm infants without typical manifestations. MALDI-TOF MS is helpful for the diagnosis and rational application of antibiotics.

Objective:To analyze the risk factors of bronchopulmonary dysplasia(BPD)in very preterm infants(VPI), and to provide scientific basis for the prevention and treatment of BPD in VPI.Methods:A prospective multicenter study was designed to collect the clinical data of VPI in department of neonatology of 28 hospitals in 7 regions from September 2019 to December 2020.According to the continuous oxygen dependence at 28 days after birth, VPI were divided into non BPD group and BPD group, and the risk factors of BPD in VPI were analyzed.Results:A total of 2 514 cases of VPI including 1 364 cases without BPD and 1 150 cases with BPD were enrolled.The incidence of BPD was 45.7%.The smaller the gestational age and weight, the higher the incidence of BPD( P<0.001). Compared with non BPD group, the average birth age, weight and cesarean section rate in BPD group were lower, and the incidence of male infants, small for gestational age and 5-minute apgar score≤7 were higher( P<0.01). In BPD group, the incidences of neonatal respiratory distress syndrome(NRDS), hemodynamically significant patent ductus arteriosus, retinopathy of prematurity, feeding intolerance, extrauterine growth restriction, grade Ⅲ~Ⅳ intracranial hemorrhage, anemia, early-onset and late-onset sepsis, nosocomial infection, parenteral nutrition-associated cholestasis were higher( P<0.05), the use of pulmonary surfactant(PS), postnatal hormone exposure, anemia and blood transfusion were also higher, and the time of invasive and non-invasive mechanical ventilation, oxygen use and total hospital stay were longer( P<0.001). The time of starting enteral nutrition, cumulative fasting days, days of reaching total enteral nutrition, days of continuous parenteral nutrition, days of reaching 110 kcal/(kg·d) total calorie, days of reaching 110 kcal/(kg·d) oral calorie were longer and the breastfeeding rate was lower in BPD group than those in non BPD group( P<0.001). The cumulative doses of amino acid and fat emulsion during the first week of hospitalization were higher in BPD group( P<0.001). Multivariate Logistic regression analysis showed that NRDS, invasive mechanical ventilation, age of reaching total enteral nutrition, anemia and blood transfusion were the independent risk factors for BPD in VPI, and older gestational age was the protective factor for BPD. Conclusion:Strengthening perinatal management, avoiding premature delivery and severe NRDS, shortening the time of invasive mechanical ventilation, paying attention to enteral nutrition management, reaching whole intestinal feeding as soon as possible, and strictly mastering the indications of blood transfusion are very important to reduce the incidence of BPD in VPI.

Objective:To study the changes of plasma receptor interacting protein 3 (RIP3) levels in neonatal late-onset sepsis (LOS) and to determine its clinical value.Methods:From October 2019 to April 2021, plasma samples and clinical data of LOS infants admitted to our hospital were prospectively studied. Infants with similar gestational ages admitted for non-infectious diseases were assigned into the control group. Enzyme-linked immunoassay was used to determine plasma RIP3 levels. The clinical value of plasma RIP3 in the diagnosis and treatment of neonatal LOS were analyzed.Results:A total of 152 cases (76 in the LOS group and 76 in the control group) were included in the study. No significant differences existed in the baseline data between the two groups. A total of 226 plasma samples were collected (76 samples from the LOS group before treatment, 74 samples after treatment and 76 samples from the control group). The plasma RIP3 level of LOS group before treatment (19.9±6.3 ng/ml) was significantly higher than the control group (11.4±3.5 ng/ml) and the after treatment group (11.9±3.5 ng/ml) ( P<0.05). The plasma RIP3 level had good diagnostic value for neonatal LOS (AUC=0.884). With cut-off value of 15.5 ng/ml, the plasma RIP3 showed the best diagnostic efficacy (Youden index 0.658, sensitivity 72.4%, specificity 93.4%, positive likelihood ratio 11.0, negative likelihood ratio 0.3). Conclusions:Plasma RIP3 level is closely related with neonatal LOS and may be used for the early diagnosis and therapeutic evaluation of neonatal LOS.

Objective: To investigate the clinical efficacy of sequential heated humidified high flow nasal ca-nnula(HHHFNC)after extubation in premature infants with pulmonary hemorrhage. Methods: A total of 52 premature infants with pulmonary hemorrhage were selected, who were randomly (by means of random number table) given nasal intermittent positive pressure ventilation(NIPPV)(27 cases) and HHHFNC (25 cases) as a sequential respiratory su-pport from January 2017 to January 2018 at Suzhou Hospital of Nanjing Medical University were selected.The incidence of the basic conditions of the 2 groups of premature infants [gestational age, birth weight, mechanical ventilation days after pulmonary hemorrhage, high-frequency ventilation, usage of nitric oxide(NO)], blood gas analysis indicators at 1 h before extubation and ventilator parameters including the arterial oxygen partial pressure [p_a(O₂)], arterial partial pressure of carbon dioxide [p_a(CO₂)], pH value, positive end-expiratory pressure(PEEP), respiratory rates (RR), peak inspiratory pressure (PIP), fraction of inspiration oxygen (FiO₂) were compared.The blood gas analysis after extubation [p_a(O₂), p_a(CO₂), pH value] at 1 h, outcome events/complications at the end of treatment (reintubation, uration of oxygen therapy after pulmonary hemorrhage, hospitalization days, bronchial pulmonary hypoplasia, ventilator associated pneumonia, feeding intolerance, neonatal necrotizing enterocolitis, pneumothorax) were also compared. Results: There were no significant differences between the NIPPV group and the HHHFNC group in the following items: gestational age, birth weight, mechanical ventilation days after pulmonary hemorrhage, high-frequency ventilation, usage of NO[(30.5±2.9) weeks vs.(31.6±2.7) weeks, 1 325(818) g vs.1 400(800) g, 5 days vs.4 days, 25.9%(7/27 cases) vs.24.0%(6/25 cases), 7.4%(2/27 cases)vs.0(0/25 cases), all P>0.05]. There were no significant differences between the two groups in blood gas analysis indicators before extubation at 1 h and ventilator parameters [p_a(O₂), p_a(CO₂), pH value, PEEP, RR, PIP, FiO₂], blood gas analysis [p_a(O₂), p_a(CO₂), pH value] after extubation at 1 h (all P>0.05); There were no significant differences between the two groups in reintubation, hospitalization days, bronchial pulmonary hypoplasia, ventilator associated pneumonia, feeding intolerance, neonatal necrotizing enterocolitis, pneumothorax [7.4%(2/27 cases) vs.4.0%(1/25 cases), 43(29) days vs.41(22) days, 40.7%(11/27 cases ) vs.16.0%(4/25 cases), 11.1%(3/27 cases) vs.12.0%(3/25 cases), 37.0%(10/27 cases) vs.32.0%(8/25 cases), 7.4%(2/27 cases ) vs.12.0%(3/25 cases), 7.4%(2/27 cases) vs.12.0%(3/25 cases)] (all P>0.05). The duration of oxygen therapy after pulmonary hemorrhage in the HHHFNC group was shorter than that in the NIPPV group [25(30) days vs.9(22) days, P<0.05]. Conclusions As a sequential respiratory support for preterm infants with pulmonary hemorrhage, HHHFNC has shorter duration of oxygen therapy after pulmonary hemorrhage than that of NIPPV, suggesting that HHHFNC is an ideal non-invasive ventilation treatment.

Objective: To investigate the incidence and pathogen distribution of ventilator-associated pneumonia (VAP) among preterm infants admitted to level Ⅲ neonatal intensive care units (NICU) in China. Method: A prospective study was conducted in 25 level Ⅲ NICU, enrolling all preterm infants <34 weeks gestational age admitted to the participating NICU within the first 7 days of life from May 2015 to April 2016. Chi-square test, t test and Mann-Whitney U test were used for statistical analysis. Result: A total of 7 918 patients were enrolled, within whom 4 623(58.4%) were males. The birth weight was (1 639±415) g and the gestational age was (31.4±2.0) weeks; 4 654(58.8%) infants required non-invasive mechanical ventilation and 2 154(27.2%) required intubation. Of all the mechanically ventilated patients, VAP occurred in 95 patients. The overall VAP rate was 7.0 episodes per 1 000 ventilator days, varying from 0 to 34.4 episodes per 1 000 ventilator days in different centers. The incidence of VAP was 9.6 and 6.0 per 1 000 ventilator days in children′s hospitals and maternity-infant hospitals respectively, without significant differences (t=1.002, P=0.327). Gram-negative bacilli (76 strains, 91.6%) were the primary VAP microorganisms, mainly Acinetobacter baumannii (24 strains, 28.9%), Klebsiella pneumonia (23 strains, 27.7%), and Pseudomonas aeruginosa (10 strains, 12.0%). Conclusion The incidence of VAP in China is similar to that in developed counties, with substantial variability in different NICU settings. More efforts are needed to monitor and evaluate the preventable factors associated with VAP and conduct interventions that could effectively reduce the occurrence of VAP.

Objective To investigate the difference of two models of respiratory failure in preterm infants with nasal continuous positive airway pressure(nCPAP)and nasal biphasic positive airway pressure (nBiPAP).Methods Comparative analysis of 81cases of premature infants with respiratory failure requi-ring respiratory support was needed.They were applied nCPAP and nBiPAP ventilation mode treatment.The use of breathing machine and total oxygen inhalation time,the number of treatment failures,the PEEP param-eters of the ventilator,the total number of recent complications and secondary infections were compared.Re-sults In the nBiPAP treatment group,the average time of noninvasive ventilation was(96.34±31.5)h,the total time of oxygen inhalation was(10.11 ±9.39)d,3cases of treatment failure,the incidence of severe complications was 5.56%(2/36),PEEP (4.08±0.73)cmH2O (1cmH2O =0.098kPa ).In the nCPAP group,the average time of noninvasive ventilation was(114.14±41.69)h,the total time of oxygen inhalation was(17.53±15.96)d,15cases were failed,the incidence of severe complications was 15.56%(7/45), PEEP(5.11±1.01)cmH2O.There were significant differences between the two groups(P﹤0.05).But there were no significant differences in infection control and patent ductus arteriosus (P ﹥0.05).Conclusion nBiPAP as one of the first choice treatment for premature infants with respiratory failure significantly better than nCPAP,as soon as the proper use can avoid invasive ventilation.

Objective To evaluate the clinical efficacy and safety of vancomycin and linezolid for the treatment of gram-positive neonatal bacterial sepsis.Method The data of neonates diagnosed as grampositive bacterial sepsis in neonatology department of Suzhou Municipal Hospital from June 2009 to December 2015 were retrospectively collected.These neonates were divided into vancomycin group and linezolid group.Propensity score matching (PSM) on baseline variables was used to balance the two groups by identifying a comparable group of neonates who received vancomycin and linezolid therapy.Clinical and microbiologic success rates were compared by chi-square test,and changes of laboratory parameters before and after treatment at the end of treatment were then directly compared by rank-sum test between the matched groups.In vancomycin group,correlation between trough concentration of vancomycin and clinical efficacy were evaluated.Result Totally 108 and 209 cases were respectively selected in vancomycin and linezolid groups;108 cases with well-matched baseline characteristics were included in matched linezolid group.The clinical success rates of vancomycin vs.linezolid therapy were 86.1％ (93/108) and 88.9％ (96/108) (P =0.681),and the microbiologic success rates were 91.7％ (99/108) and 93.5％ (101/108) (P =0.795).The average trough concentration of vancomycin was (12 ± 8) mg/L.The rate of reaching the high trough concentration standard was only 33.3％ (36/108).In 50 (46.3％) cases the dose was adjusted according to the initial concentration data.Compared to less than 10 mg/L,the clinical efficacy of trough concentration in 10-20 mg/L was much higher(93.9％ (46/49) vs.78.6％ (33/42),P =0.031).Total bilirubin and platelet count had significant difference between the two-matched groups (34.1 (14.9,91.0)μmol/L vs.53.0(27.0,121.6) μmol/L,P =0.034;301.0 (198.8,416.0) × 109/L vs.195.5 (94.0,283.2) × 109/L,P =0.000).The incidence of linezolid related thrombocytopenia was 13.4％ (28 cases).Conclusion The clinical effect of vancomycin and linezolid on gram-positive bacterial sepsis in neonates is comparable.The rate of trough concentration of vancomycin reaching the high trough concentration standard is low,and the clinical efficacy is related to trough concentration.Linezolid have an effect on bilirubin and platelet count,the risk of thrombocytopenia should be monitored closely during linezolid treatment.

ObjectiveTo explore the pathogenesis, clinical manifestations, diagnosis, and treatment of autoanti-body-associated congenital heart block.MethodsThe clinical data of one child with autoantibody-associated congenital heart block was retrospectively analyzed.ResultsIn 24 week gestation, fetal bradycardia had been found by routine fetal echocar-diography. After birth, the anti-SSA/Ro antibodies and anti-SSB/La antibodies were positive in both infant and her mother. The diagnosis of autoantibody-associated congenital heart block was conifrmed. Intravenous immunoglobulin at 1 g/kg was adminis-trated. At 6 months follow-up, the electrocardiogram suggested type I second degree atrioventricular block.ConclusionIn the fetus or neonates found to have bradycardia and excluded the cardiac structural abnormalities, the autoimmune antibody should been tested and early intervention should been promoted.

Objective To observe the effect of nasal continuous positive airway pressure (nCPAP) and Biphase positive airway pressure (BIPAP) on the oxygen exchange indexes in newborns with mild to moderate neonatal respiratory distress syndrome (NRDS).Methods After infusion of pulmonary suffactant into lung bilaterally,116 cases with mild to moderate NRDS were treated with nCPAP(nCPAP group,n =75) and BIPAP(BIPAP group,n =41) respectively.Oxygen exchange indexes including arterial partial pressure of oxygen [pa (O2)],modified oxygenation index (P/F),alveolar-arterial oxygen tension difference (A-aDO2),respiratory index (RI),artery/alveolar oxygen partial pressure ratio[pa (O2)/pA (O2)] and pulmonary shunt fraction (Qs/Qt) were measured or calculated at 0 (before treatment),2,8 and 24 h after being treated with nCPAP or BIPAP.Results Six different oxygen exchange indexes of Pa (O2),P/F,A-aDO2,RI,pa (O2)/pA(O2) and Qs/Qt in both groups didn't show statistical significance at 0 hour:pa(O2) (kPa) (6.1 ±0.6 vs 6.1 ±0.6,t =0.11,P ＞0.05),P/F(kPa) (15.59 ± 1.45 vs 15.71 ± 1.45,t =1.59,P ＞ 0.05),A-aDO2(kPa)(6.04 ±0.64 vs 6.24 ±0.69,t =1.59,P ＞0.05),RI(6.0 ± 1.0 vs 6.2 ± 1.1,t =1.35,P ＞ 0.05),p,(O2)/pA(O2) (0.24 ±0.02 vs 0.25 ±0.03,t =1.63,P ＞0.05)and Qs/Qt(％)(11.9±1.6 vs 11.6 ± 1.6,t =1.10,P ＞ 0.05).A-aDO2,RI,pa (O2)/pA (O2) and Qs/Qt of BIPAP group had more improvement than those of nCPAP at 2 and 24 h,and oxygen exchange indexes at 2 h of BIPAP group and those of nCPAP group were:A-aDO2 (kPa) (3.83 ±0.49 vs 4.24 ± 0.67,t =18.26,P ＜ 0.05),RI(2.7 ± 0.5 vs 3.3 ±0.7,t =20.59,P ＜ 0.05),p,(O2)/pA(O2) (0.35 ±0.03 vs 0.32 ±0.04,t =15.35,P ＜0.05) and Qs/Qt(％) (8.8 ± 1.6 vs 9.8 ±2.0,t =7.68,P ＜ 0.05) ; 24 h indexes in BIPAP group and the indexes of nCPAP group were:A-aDO2 (kPa) (2.29 ± 1.19 vs 3.07 ± 1.67,t=18.43,P ＜0.05),RI(1.4 ±1.4 vs 2.3 ± 1.6,t=25.02,P ＜0.05),pa(O2)/pA(O2) (0.49 ±0.10 vs 0.42 ±0.11,t =14.96,P ＜0.05) and Qs/Qt(％) (6.5 ±3.0 vs 8.5 ±4.4,t =9.59,P ＜0.05).pa(O2) and P/F of both groups didn't show statistical difference significantly [Pa (O2)(kPa) (12.6 ± 1.0 vs 12.7 ± 1.0,t =3.76,P ＞ 0.05),P/F(28.49 ± 3.17 vs 31.85 ± 2.85,t =3.76.P ＞ 0.05)].Nineteen cases in nCPAP group needed invasive mechanical ventilation,and only 3 cases in BIPAP group underwent the procedure;there was a significant difference (x2 =4.01,P ＜ 0.05).Oxygen exchange indexes in both groups were gradually improved from 2 h,8 h to 24 h,but 22 deteriorated cases showed no improvement,who underwent invasive ventilation.Positive end-expiratory pressure (PEEP) positive airway pressure (EPAP) was (0.42 ± 0.19) kPa in BIPAP group,and (0.56 ± 0.23) kPa in nCPAP group (t =3.45,P ＜ 0.01).Conclusions 1.BIPAP and nCPAP can improve the ability of oxygen exchange in newborns with mild to moderate NRDS,but BIPAP is more effective than nCPAP,which can shorten the possibility of invasive ventilation and reduce the PEEP level.2.p,(O2),P/F,pa (O2)/pA (O2) and RI may be more suitable for evaluating oxygen exchange for neonatal NRDS.