Objective:To study the diagnosis, treatment and outcome of congenital complete atrioventricular block (CCAVB) in neonates before, during and after delivery and to monitor their growth and development.Methods:From December 2017 to June 2021, clinical data of 5 neonates with CCAVB admitted to the neonatal department of our hospital were analyzed.Results:A total of 3 males and 2 females were included. The diagnosis was established at (24.6±3.6) weeks during the fetus period. The average birth weight was (2 493±551) g and the gestational age was (36.7±1.5) weeks. 4 mothers had autoimmune diseases, with positive antinuclear antibodies, SSA and Ro52. The cause of the remaining 1 neonate was undetermined. 4 neonates received isoproterenol treatment after birth, but the therapeutic effects were unsatisfying. The longest continuous isoproterenol infusion in 1 neonate lasted for 18 d. Permanent pacemakers were implanted during hospitalization in 3 neonates and 2 neonates were implanted within 1 year after birth. 1 neonate died of septic shock following pacemaker pocket infection at 26 d after birth. The other 4 neonates were followed up to 1-year-old after pacemaker implantation and their growth and development were otherwise normal.Conclusions:Most of the mothers of CCAVB neonates have autoimmune diseases. Fetal arrhythmias are often found in the second trimester during pregnancy, but most of the neonates can be delivered near full-term. The effects of drug therapy are poor and pacemakers are necessary.

Abstract. Background:. Delivery room resuscitation assists preterm infants, especially extremely preterm infants (EPI) and extremely low birth weight infants (ELBWI), in breathing support, while it potentially exerts a negative impact on the lungs and outcomes of preterm infants. This study aimed to assess delivery room resuscitation and discharge outcomes of EPI and ELBWI in China. Methods:. The clinical data of EPI (gestational age [GA] <28 weeks) and ELBWI (birth weight [BW] <1000 g), admitted within 72 h of birth in 33 neonatal intensive care units from five provinces and cities in North China between 2017 and 2018, were analyzed. The primary outcomes were delivery room resuscitation and risk factors for delivery room intubation (DRI). The secondary outcomes were survival rates, incidence of bronchopulmonary dysplasia (BPD), and risk factors for BPD. Results:. A cohort of 952 preterm infants were enrolled. The incidence of DRI, chest compressions, and administration of epinephrine was 55.9% (532/952), 12.5% (119/952), and 7.0% (67/952), respectively. Multivariate analysis revealed that the risk factors for DRI were GA <28 weeks (odds ratio [OR], 3.147; 95% confidence interval [CI], 2.082–4.755), BW <1000 g (OR, 2.240; 95% CI, 1.606–3.125), and antepartum infection (OR, 1.429; 95% CI, 1.044–1.956). The survival rate was 65.9% (627/952) and was dependent on GA. The rate of BPD was 29.3% (181/627). Multivariate analysis showed that the risk factors for BPD were male (OR, 1.603; 95% CI, 1.061–2.424), DRI (OR, 2.094; 95% CI, 1.328–3.303), respiratory distress syndrome exposed to ≥2 doses of pulmonary surfactants (PS; OR, 2.700; 95% CI, 1.679–4.343), and mechanical ventilation ≥7 days (OR, 4.358; 95% CI, 2.777–6.837). However, a larger BW (OR, 0.998; 95% CI, 0.996–0.999), antenatal steroid (OR, 0.577; 95% CI, 0.379–0.880), and PS use in the delivery room (OR, 0.273; 95% CI, 0.160–0.467) were preventive factors for BPD (all P < 0.05). Conclusion:. Improving delivery room resuscitation and management of respiratory complications are imperative during early management of the health of EPI and ELBWI.

目的 探讨新生儿窒息后胃肠道功能损伤的临床表现及相关危险因素.方法 回顾性分析159例新生儿窒息患儿,依据Apgar评分分为轻度窒息组和重度窒息组,比较两组胃肠损伤及喂养不耐受、消化道出血、明显腹胀、肠鸣音减弱的发生情况;并依据胃肠损伤程度分为轻症和重症,采用χ2检验比较其发生率;采用logistic回归分析胃肠道功能损伤的相关危险因素.结果 轻度窒息组136例,重度窒息组23例,两组发生胃肠道功能损伤共60例,其中轻度窒息组44例(32.4％),重度窒息组16例(69.6％),两组差异有统计学意义(χ2=11.59,P<0.01);轻度窒息组发生胃肠损害重症为12例、轻症32例,重度窒息组重症12例、轻症4例,(χ2=12.27,P<0.01);轻度窒息组在24 h和48 h开奶发生喂养不耐受、胃潴留、胃肠出血、腹胀情况均明显低于重度窒息组(χ2分别为3.227、39.380,53.550、30.390,5.118、8.280,14.300、13.440,P值均<0.05)、两组肠鸣音减弱发生率无明显差异(P值均>0.05);轻度窒息组72 h开奶时发生喂养不耐受、胃肠出血发生情况均低于重度窒息组(χ2分别为28.690,5.400,P值均<0.05),两组胃潴留、明显腹胀、肠鸣音减弱发生率均无明显差异(P值均>0.05).窒息程度、Apgar 1 min评分,24、48 h开奶,多脏器功能损伤是发生胃肠道功能损伤的独立危险因素(OR分别为26.267、1.557、4.946、8.132、2.880;P值均<0.05).结论 新生儿窒息后胃肠道功能损伤发生率较高,窒息程度与胃肠道功能损伤呈正相关.

目的 通过新生儿窒息肾脏损害的临床表现、肾功能、尿酶测定、肾血流超声检测进一步探讨新生儿窒息肾脏损害的诊断. 方法 选择2016-01-01 ～2017-06-30在清华大学第一附属医院新生儿重症监护病房(NICU)住院的新生儿窒息患儿,胎龄＞37周、出生体质量＞2500 g的患儿共111例,每例患儿出生后均进行脐动脉血气分析.采用Apgar评分诊断新生儿窒息.根据脐动脉血气将重度窒息组再分别分为严重酸中毒组(pH≤7和或碱剩余≤-12 mmol/L)、非严重酸中毒组(pH ＞7.0和或碱剩余＞-12 mmol).根据缺氧病史、临床表现、肾功能及尿酶诊断新生儿窒息肾脏损害.比较轻度窒息组(n=74)、重度窒息组(n=37)中肾脏损害发生率、肾脏损害相关的临床表现及肾功能、血、尿β2微球蛋白(β2M)及尿-N乙酰-β-D-氨基葡萄糖苷酶(NAG)和尿β-D-半乳糖苷酶(GAL). 结果 ①新生儿窒息肾脏损害的发生率为35.13％(39/111).重度窒息组肾脏损害的比例高于轻度窒息组(51.39％ vs 27.03％),差异有统计学意义(x2=6.404,P=0.011).②重度窒息组中严重酸中毒的肌酐、尿素氮明显高于非严重酸中毒组和轻度窒息组(均P＜0.05).出生后7d,三组肌酐与尿素氮均明显改善,但重度窒息合并严重酸中毒组中Cr和BUN水平仍高于其他两组,差异有统计学意义.③出生后48 h,重度窒息组中严重酸中毒的血、尿β2微球蛋白明显高于非严重酸中毒组和轻度窒息组(均P＜0.05).④出生后48 h,轻度窒息组、重度窒息中严重酸中毒组与非严重酸中毒组相比,尿NAG和尿GAL均有升高趋势,但差异无统计学意义(均P＞0.05).⑤肾血流超声检查显示,重度窒息组中严重酸中毒组的左肾收缩期峰值血流高于非严重酸中毒组、轻度窒息组及对照组(P =0.003),但其余指标组间比较差异无统计学意义. 结论 新生儿窒息可发生肾脏损害.结合脐血pH值可判断窒息严重程度,合并严重酸中毒的患儿肾脏损害更严重.

>B and T lymphocytes are responsible for the acquisition of adaptive immune response, among which, B cells dominate humoral immunity. In vivo, B cells utilize surface expressed B cell receptors (BCRs) to sense antigens presented by antigen-presenting cells (APCs), and eventually mediate antibody response and immune memory. It is well recognized

目的 总结13例侵袭性真菌感染(IFI)患儿的临床特点、感染菌种及有效治疗方法.方法 对13例IFI新生儿的临床资料进行回顾性分析,总结其临床特点、感染菌种及有效治疗方法.结果 13例患儿均为早产儿,以极低出生体重儿(VLBW)为主10例,存在广谱抗生素应用史13例,侵袭性检查治疗操作气管插管5例、中心静脉置管7例,发生真菌败血症时间(16.6±6.1)天,临床表现为刺激反应弱12例、呼吸暂停9例、心率增快6例、肤色发花7例,8例患儿发病时间为夏季.真菌培养结果13例中感染热带假丝酵母菌6例、白假丝酵母菌5例、近平滑假丝酵母菌1例、光滑假丝酵母菌1例.氟康唑治疗后13例患儿症状均减轻,后均好转出院.结论 VLBW容易发生IFI,存在抗生素应用、侵袭性检查治疗操作的新生儿好发IFI,IFI好发于夏季,缺乏典型临床表现.真菌培养是确诊IFI的主要方法,IFI主要为热带假丝酵母菌、白假丝酵母菌感染.氟康唑治疗新生儿IFI效果较好.

Background: Hypophosphatasia (HPP) is a rare hereditary disorder characterized by defective bone and tooth mineralization and deficiency of tissue non-specific alkaline phosphatase (TNAP) activity. The clinical presentation of HPP is highly variable, and the prognosis for the infantile form is poor. Case presentation: This study reports a male infant diagnosed with lethal perinatal HPP. His gene analysis showed two heterozygous missense variants c.406C > T (p.R136C) and c.461C > T (p.A154V). The two mutations originated separately from his parents, consistent with autosomal recessive perinatal HPP, and the c.461C > T (p.A154V) was the novel mutation. Three-level structure model provide an explanation of the two mutated alleles correlating with the lethal phenotype of our patient. Results of SIFT, PolyPhen_2, and REVEL showed two mutations were pathogenic. Conclusions: We demonstrated a case of perinatal lethal HPP caused by two heterozygous mutations, and one of which was novel. This finding will prove relevant for genetic counseling and perinatal gene testing for affected families.

目的 了解新生儿窒息并发医院感染的临床特点,为该病的预防及治疗提供依据.方法 回顾性分析128例新生儿窒息并发医院感染患儿临床资料,对医院感染部位、病原菌及耐药性、相关影响因素进行分析.结果 128例新生儿窒息并发医院感染患儿中呼吸系统感染43例(33.6％)、全身感染38例(29.7％).共培养出病原菌143株主要有肺炎克雷伯菌41株(28.7％),大肠埃希菌39株(27.3％).肺炎克雷伯菌和大肠埃希菌对β-内酰胺类抗菌药物有较高的耐药率,而对碳青霉烯类、加酶抑制剂β-内酰胺类抗菌药物比较敏感.胎龄＜37周、宫内窘迫、出生体质量＜2 500 g、住院时间≥7天、机械通气、肠外营养是医院感染发生的影响因素,除宫内窘迫外其他均为独立影响因素.结论 新生儿窒息并发医院感染主要以呼吸系统及全身感染为主,肺炎克雷伯菌、大肠埃希菌是主要致病菌且多数为多药耐药菌,对β-内酰胺类抗菌药物耐药率高,而对碳青霉烯类及加酶抑制剂的β-内酰胺类抗菌药物敏感.胎龄低(＜37周)、宫内窘迫、出生低体质量(＜2 500g)、住院时间长(≥7天)、机械通气、肠外营养为新生儿窒息并发医院感染影响因素.

FcγRIIB, the only inhibitory IgG Fc receptor, functions to suppress the hyper-activation of immune cells. Numerous studies have illustrated its inhibitory function through the ITIM motif in the cytoplasmic tail of FcγRIIB. However, later studies revealed that in addition to the ITIM, the transmembrane (TM) domain of FcγRIIB is also indispensable for its inhibitory function. Indeed, recent epidemiological studies revealed that a non-synonymous single nucleotide polymorphism (rs1050501) within the TM domain of FcγRIIB, responsible for the I232T substitution, is associated with the susceptibility to systemic lupus erythematosus (SLE). In this review, we will summarize these epidemiological and functional studies of FcγRIIB-I232T in the past few years, and will further discuss the mechanisms accounting for the functional loss of FcγRIIB-I232T. Our review will help the reader gain a deeper understanding of the importance of the TM domain in mediating the inhibitory function of FcγRIIB and may provide insights to a new therapeutic target for the associated diseases.

B cells exert traction forces on antigen-presenting surfaces during activation.

目的 探讨振幅整合脑电图在早产儿脑损伤早期诊断中的临床应用价值.方法 选取40例脑损伤早产儿(27～36周)作为研究组,另选同期住院的50例无脑损伤早产儿(27～36周)作为对照组.两组早产儿出生6 h内均进行振幅整合脑电图描记,并采用aEEG评分系统分析连续性电压评估、睡眠觉醒周期及aEEG背景活动异常率.结果 研究组早产儿aEEG图形校正总分为(0.9±1.1)分,对照组早产儿aEEG图形校正总分为(0.2±1.3)分,研究组aEEG图形校正总分明显高于对照组,差异具有统计学意义(P<0.05).结论 振幅整合脑电图监测可准确直观地观察到早产儿脑功能的变化情况,这一检测方法对早产儿脑损伤的早期诊断具有较高的临床应用价值,值得临床广泛应用.

患儿男，生后1 h，主因“发现先天性完全性房室传导阻滞”于2016年4月入住清华大学第一附属医院心脏小儿科。母亲孕25周时发现胎儿心率慢，超声检查提示胎儿房室传导阻滞，孕35周超声提示胎儿完全性房室传导阻滞（心房率135次／min，心室率44次／min），全心扩大，心胸比例0．7，左室舒张末内径20 mm，射血分数59％，心脏结构未见异常。胎儿期生长发育尚正常，胎龄36周剖宫产出生，出生体重为2．5 kg，1、5、10 min Apgar评分分别为9、9、10分。为行“起搏治疗”收入我科。患儿为第2胎第2产，其姐姐（12岁）体健，出生史及既往史无特殊，母亲（43岁）和父亲（44岁）既往体健，否认晕厥、猝死家族史，母亲自身抗体谱［抗核抗体、干燥综合征（ SS ）－A、SS－B、双链DNA （ dsDNA ）抗体、抗心磷脂抗体等］阴性。

The initial infection and transmission of HIV-1 requires C-C chemokine receptor type 5 (CCR5). Here, we report that the membrane proximal region (MPR, aa 22-38) of CCR5 participates in the infection of HIV-1. First, MPR-specific antibodies elicited in mice dose-dependently inhibited the infection of CCR5-tropic HIV-1. Second, substituting MPR with the same region from other co-receptors significantly impaired HIV-1 infection, while the key residues identified by alanine scanning mutagenesis formed an exposed leucine zipper-like structure. Moreover, a peptide derived from MPR could block the infection of a number of HIV-1 strains only before the formation of gp41 six-helix bundle, coincide with the early interaction between CCR5 and the gp120 protein during HIV-1 infection. These promising results ensured the potential of this previously uncharacterized domain as a starting point for the development of antiviral drugs, blocking antibodies and HIV vaccines.

Enhancement of endocytic uptake of HIV-1 virions into CD4-negative epithelial cells by HIV-1 gp41 via its interaction with POB1

目的：比较 T - piece 复苏器与自动充气式气囊在需气管插管的危重新生儿转运中的应用效果。方法回顾性分析2012—2014年清华大学第一附属医院转运中需气管插管危重患儿79例，依据气管插管后连接设备的不同分成 T - piece 组37例和自动充气式气囊组42例。T - piece 组于气管插管后连接 T - piece 复苏器和氧源；自动充气式气囊组于气管插管后连接自动充气式气囊和氧源。观察所有患儿疾病分布及转归情况，比较两组患儿血气分析指标〔pH 值、二氧化碳分压（PCO2）和氧分压（PO2）〕、上机时间、多器官功能损害发生率及转归情况。结果两组患儿 pH 值、PCO2、PO2、上机时间比较，差异无统计学意义（P >0.05）。T - piece 组患儿多器官功能损害发生率低于自动充气式气囊组，差异有统计学意义（P <0.05）。结论 T - piece 复苏器与自动充气式气囊在改善通气方面效果相当，但 T - piece 复苏器多器官功能损害发生率低，在危重新生儿转运中具有优势。

窒息缺氧胎盘血流阻断时为保证心脑供血会导致心输出量重新分布而引起其他脏器的损害，窒息持续加重时形成严重代谢性酸中毒（pH≤7），并导致心脑损害[1]。窒息酸中毒引起新生儿主动脉舒张压下降，促使冠状动脉收缩使心脏灌注及全身灌注不足，不仅心脏缺血缺氧加重，还进一步累及各器官的灌注压而发生损害[2-6]。但是目前，国内外尚无新生儿窒息多器官损害的多中心研究，更无公认的诊断标准、常规或指南。在清华大学自主科研基金资助下，全国新生儿窒息多器官损害临床诊断多中心研究协作组（简称协作组）成立，提出结合Apgar评分和出生时脐动脉血pH诊断新生儿窒息，及新生儿窒息多器官损害的诊断标准[7]，并根据此标准进行了新生儿窒息多器官损害的多中心前瞻性研究[8-9]。研究结果认为该新生儿窒息多器官损害的临床诊断标准[7]较全面，且更深入，具有较好的临床意义，现对该诊断标准进行解读，以指导临床实施。

A dying neonate with congenital complete atrioventricular block underwent an emergency temporary pacing via the umbilical vein 1 h after birth. Implantation of a permanent epicardial pacemaker system was performed at the age of 10 days. During the follow-up period of 3 months, the child had been growing well with the VVIR pacemaker.

A previous amino acid sequence analyses from our laboratory reported nine potential sites in gp41 glycoprotein of HIV-1 that may contribute to virus escape from antibody neutralization. Besides four sites found outside the membrane of HIV-1 virus, five located in the C-terminal tail of gp41 specifically in the lentivirus lytic peptides motifs (LLPs). To further study the bioinformatical results, the virus infectivity assay and the standard neutralization assay were conducted on conservatively mutated virus. Two sites in the LLP3 domain stood out with the ability to alter the resistance of HIV-1 virus to certain broadly neutralizing antibodies (bNAbs). While the glycoprotein incorporation on the viral membrane and the interaction of the LLP3 domain with the lipid membrane remained unaltered, the increase in neutralization resistance of the mutant virus was associated with the changes on Env conformation. Our findings demonstrate different sensibility of bNAbs to mutations in the C-terminal tail and indicate an unrecognized potential role for even minor sequence variation in the C-terminal tail in modulating the antigenicity of the ectodomain of HIV-1 envelope glycoprotein complex.

目前，国内外尚无新生儿窒息多器官损害的多中心研究，更无公认的诊断标准、常规或指南。在清华大学自主科研基金资助下，全国新生儿窒息多器官损害临床诊断多中心研究协作组成立，制定了本标准。

新生儿转运系统最早1950年在美国成立。此后围产保健区域化概念的提出，促进了高危新生儿转运工作的全面发展。我国20世纪80年代后期、90年代初期新生儿转运工作开始启动，并逐渐推广，近年来许多地方已开始创建新生儿转运系统。我国建立的新生儿转运网络是以省会城市和地级市为中心，转运的覆盖面广泛，距离长，花费时间久，难以使危重患儿获得及时高质量救治。2008年，上海市将所有19区县划分区域，组织成立6家新生儿区域急救转运中心，各中心负责转运本区域内危重新生儿，这种划区分片，对口转运，提高了转运效率，但他们采取的转运模式为120与接收医院的联合转运，使转运环节增加，转运耗时延长。我院自2004年开展转运工作以来，转运病人逐渐增多，转运技术的日趋成熟，近3年来，我院将转运模式基本固定为我院独立转运，即转出医院直接与我院NICU联系，我院派人派车接回患儿。转出医院固定为周边十余家医院，具备长期合作关系的至少4家。这样就形成了以我院NICU为中心，辐射周边50公里内的朝阳区东北部地区区域内新生儿转运网络雏形。这种相对固定的对口转运方式不仅保证了转运网络的高效畅通，而且减少了转运中间环节，集中了我院的新生儿急救医疗实力，为患儿争取了宝贵的救治时间。