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1.
Chinese Journal of Neonatology ; (6): 209-212, 2024.
Article in Chinese | WPRIM | ID: wpr-1022560

ABSTRACT

Objective:To investigate the clinical characteristics and prognosis of neonatal chylothorax.Methods:The clinical data of newborns diagnosed with chylothorax from June 2016 to June 2023 in Neonatal Center of Beijing Children's Hospital were retrospectively analyzed, and divided into congenital group and acquired group according to the pathogenesis of chylothorax. The clinical characteristics, treatment methods and prognosis of the two groups were compared.Results:A total of 23 cases were included, including 17 cases (73.9%) in the congenital group and 6 cases (26.1%) in the acquired group. There was no significant difference in gender, gestational age and birth weight between the two groups ( P>0.05). Compared with the acquired group, the proportion of lymphocytes (97.0% vs. 85.0%), the use of erythromycin (7/17 vs. 1/6) and octreotide (9/17 vs. 1/6) and special formula milk feeding (13/17 vs. 2/6) were higher in the congenital group; the proportion of right hydrothorax (1/17 vs. 3/6), invasive mechanical ventilation (6/17 vs. 6/6) and breastfeeding (0/17 vs. 3/6) were lower in the congenital group ( P<0.05). There were no significant differences in terms of the white blood cell count in pleural fluid and plasma protein content, incidence of bilateral and left pleural fluid, proportion of closed thoracic drainage, maximum daily drainage volume, drainage duration, total drainage volume, albumin utilization rate, length of stay and survival rate between the two groups ( P>0.05). 18 cases of pleural effusion absorption without recurrence after conservative treatment; 5 cases died, of which 4 cases died after their parents abandoned treatment, and 1 case died of neonatal necrotizing enterocolitis after thoracic duct ligation surgery. Conclusions:Congenital chylothorax and acquired chylothorax were similar in severity, course of disease and overall prognosis. The utilization rate of erythromycin and octreotide in congenital chylothorax was higher than that in acquired chylothorax. The neonatal chylothorax is usually with an overall good prognosis.

2.
Article in Chinese | WPRIM | ID: wpr-1031128

ABSTRACT

Early life stress in humans can affect the normal development of individual neural networks, ultimately leading to diseases such as anxiety, depression, and autism in such children. The maternal separation model is commonly used to study the effects of early adverse experiences in human infants. Studies have shown that maternal separation in mice can lead to anxiety, depression, and impairments in spatial learning and memory in young mice during adulthood. However, enriched environmental interventions have been found to ameliorate the negative outcomes of early life stress by exerting a range of beneficial effects. This article provides an overview of the positive effects of enriched environmental interventions on mice in the maternal separation model.

3.
Article in Chinese | WPRIM | ID: wpr-1039489

ABSTRACT

Children and adults differ significantly in physiology, biochemistry and immune function, which leads to significant differences in blood transfusion strategies between children and adults. To guide the clinical transfusion practice of pediatric patients and improve the prognosis of children, the National Health Commission organized the formulation and release of the health industry standard Guideline for Pediatric Transfusion (WS/T 795-2022).This paper will briefly introduce some concepts that help understand of the Standard and the preparation process of the Standard, and explain and interpret the preparation of the " scope", " general provisions" and " factors to consider" of the Standard, hoping to contribute to the understanding and implementation of the Standard.

4.
Article in Chinese | WPRIM | ID: wpr-990013

ABSTRACT

Off-lable use of antimicrobial medications in neonatal intensive care unit (NICU) draws wide attention of medical professionals both abroad and inside China.The objective of this study is to give a commentary of off-label use of antimicrobial medications in NICU.There are 4 sections in this study: the causes, current status, management flow chart, and meditation of off-lable use of antimicrobial medications in NICU.

5.
Article in Chinese | WPRIM | ID: wpr-990501

ABSTRACT

Objective:To study the outcomes and post-discharge follow-up of neonatal tracheotomy in neonatal intensive care unit(NICU).Methods:This study included patients who were admitted to NICU in Beijing Children′s Hospital from January, 2016 to August, 2021, and less than 28 days or 44 weeks(corrected age)on admission, and required tracheotomy.The patients were divided into tracheotomy group and the non-tracheotomy group (the parents signed to refuse the tracheotomy) according to whether perform tracheotomy.Demographic data, general hospitalization information, diagnosis, indications for tracheotomy, follow-up outcomes at 3/6/12 months of age after discharge of patients were collected and analyzed.Results:Totally 26 patients were included in this study, 14 cases in tracheotomy group and 12 cases in non-tracheotomy group.The average gestational age was(37.7±3.80)weeks and(38.99±1.83)weeks, and birth weight was(2 823.57±948.89)g and (3 320.83±378.76)g, respectively.There were no significant differences in sex, gestational age, birth weight, age on admission, weight on admission, age at diagnosis, ratio of endotracheal intubation for respiratory support on admission between two groups( P>0.05). The commonest indications of tracheotomy group were bilateral vocal cord paralysis(50.0%) and congenital anomaly/defect of throat/larynx(21.4%), and the commonest indications of non-tracheotomy group were bilateral vocal cord paralysis(50.0%) and vocal cord/subglottic mass(25.0%), and there was no significant difference between two groups( P>0.05). The rate of discharge-against-medical order of tracheotomy and non-tracheotomy group was 7.14% and 66.67%( P=0.003), respectively.The total follow-up rate of tracheotomy and non-tracheotomy group was 88.9% and 38.9%, while the follow-up rates at 3 months, 6 months, and 12 months were 100.0% vs. 50.0%, 83.3% vs. 41.7%, and 81.8% vs. 25.0%, respectively, whose differences were statistically significant(all P<0.05). In the 14 cases of tracheotomy group, 3 cases died, 4 cases successfully removed the tracheal cannula, 5 cases did not remove the tracheal cannula, and 2 cases were lost. Conclusion:Bilateral vocal cord paralysis is the commonest indication of neonatal tracheotomy.Parents′ compliance in the tracheotomy group is significantly higher than that in non-tracheotomy group.To give caring skill training for parents of neonates with tracheotomy before discharge is beneficial for improving the overall prognosis of children.

6.
Chinese Journal of Neonatology ; (6): 489-494, 2023.
Article in Chinese | WPRIM | ID: wpr-990778

ABSTRACT

Objective:To systematically evaluate the safety of family integrated care (FICare) model in neonatal intensive care unit (NICU).Methods:Multiple medical databases were searched for clinical studies on FICare in NICU published from January 1, 2010 to May 28, 2022. The quality of the literature was evaluated using Risk?of?Bias?2 tool?and cohort evaluation criteria from the Cochrane Systematic Evaluation Manual depending on the types of studies included. Meta-analysis was performed using Review Manager 5.3 software.Results:Six randomized controlled trials and four cohort studies were included for meta-analysis. The results of meta-analysis showed that compared with the traditional care model, FICare model did not increase the risk of nosocomial infection ( RR=0.75, 95% CI 0.46-1.24, P=0.27) and unstable medical conditions ( RR=0.86, 95% CI 0.61-1.22, P=0.40). No significant difference existed in the all-cause mortality between FICare and traditional care ( RR=2.74, 95% CI 0.88-8.57, P=0.08). Conclusions:FICare does not increase the risk of nosocomial infection, unstable medical conditions and adverse events compared with traditional care. It is safe and feasible to implement FICare in NICU.

7.
Article in Chinese | WPRIM | ID: wpr-1022332

ABSTRACT

The Chinese Neonatal Network(CHNN) was established in 2018 with the mission of establishing a national collaboration platform, conducting high-quality and collaborative research, and ultimately improving the quality of neonatal-perinatal care and health in China.At present, 112 hospitals across the country have joined CHNN.CHNN has established a national standardized cohort of very premature infants/very low birth weight infants with >10 000 enrollments each year, has been leading data-driven collaborative quality improvement initiatives, conducting multicenter clinical studies, and performing multi-level training programs.Guided by the principles of collaboration and sharing, data-driven, continuous improvement, and international integration, CHNN has become an important platform for clinical and research collaboration in neonatal medicine in China.

8.
Chinese Journal of Neonatology ; (6): 582-586, 2023.
Article in Chinese | WPRIM | ID: wpr-1022512

ABSTRACT

Objective:To study the duration of invasive mechanical ventilation (MV) and its influencing factors after neonatal abdominal surgery under general anesthesia in neonatal intensive care unit (NICU).Methods:From January 2018 to December 2020, neonates received abdominal surgery under general anesthesia and needed endotracheal intubation and MV after surgery in NICU of our hospital were retrospectively studied. According to MV duration, the neonates were assigned into <72 h group and ≥72 h group. Multivariate logistic regression was used to analyze the risk factors of postoperative MV duration.Results:A total of 113 neonates were enrolled, including 57 male (50.4%) and 56 female (49.6%). The gestational age was (35.7±3.6) weeks, the birth weight was (2 497±933) g, the average operation age was 9.9(3.6, 22.2) d and the average hospital stay was 22.0(12.0,37.0) d. Congenital intestinal obstruction (37/113, 32.7%) was the most common diagnoses on discharge, followed by neonatal necrotizing enterocolitis(28/113,24.8%) and gastrointestinal perforation (18/113,15.0%). The duration of operation was 80.0 (55.8,117.3) min. All neonates needed MV with endotracheal intubation. The duration of postoperative respiratory support was 30.0(7.0,84.5) h. 48 neonates (42.5%) had endotracheal intubation removed within 24 h after surgery. Multivariate logistic regression analysis showed that preoperative respiratory support ( P=0.004), congenital heart disease( P=0.013) and intravenous midazolam ( P=0.032) were independent risk factors for prolonged postoperative MV. Conclusions:The need of preoperative respiratory support, congential heart disease and intravenous midazolam were independent risk factors for the duration of postoperative MV after neonatal abdominal surgery under general anesthesia.

9.
Chinese Journal of Neonatology ; (6): 621-625, 2023.
Article in Chinese | WPRIM | ID: wpr-1022519

ABSTRACT

Objective:To study the clinical characteristics, diagnostic methods and genotypes of neonatal chronic granulomatous disease(CGD).Methods:From April 2013 to August 2020, neonates admitted to our hospital, diagnosed with neonatal CGD and confirmed using neutrophil respiratory burst test and genetic testing were retrospectively studied. Their clinical characteristics and diagnostic methods were summarized.Results:A total of eleven neonates with CGD were enrolled, including ten males and one female. The age of onset was 3-23 d and the age of admission was 11-28 d. 3 cases had family history. The initial symptoms were fever (10 cases) and coughing (1 case). The accompanying symptoms included coughing (6 cases), dyspnea (4 cases), skin pustules (3 cases), fever (1 case) and diarrhea (1 case). Lung CT showed patchy shadows (6 cases), pulmonary nodules (3 cases), round and irregular high-density shadow (1 case), pulmonary consolidation (1 case), pleural effusion (1 case) and pleural thickening (2 cases). CGD was pathologically diagnosed in one patient. Sputum culture showed Candida albicans (3 cases), Aspergillus fumigatum (2 cases), Aspergillus fumigatum combined with Klebsiella pneumoniae and Escherichia coli (1 case), Aspergillus fumigatum combined with Staphylococcus aureus (1 case). GM tests were positive in two cases and negative in other two cases. Blood and cerebrospinal fluid cultures were negative. White blood cell counts were (10.0-44.0)×10 9/L and C-reactive proteins were (14-165) mg/L. 11 cases had positive neutrophil respiratory burst test. Genetic tests showed CYBB gene mutations (9 cases) , NCF1 gene mutation(1 case) and NCF2 gene mutation (1 case). All cases received antibacterial and antifungal treatments for 2-5 weeks and were discharged with improvement. 8 patients survived, 5 received hematopoietic stem cell transplantation, 4 succeeded and one failed and continued to survive on oral medication. 3 cases took sulfamethoxazole, voriconazole or itraconazole orally for long periods. 3 cases died of severe infections after withdrawal of medication. Conclusions:Neonatal CGD is characterized by lung infections. Pulmonary CT shows mostly nodular or patchy shadows with occasional pulmonary consolidation or pleural effusion. It can be diagnosed by neutrophil respiratory burst test and genetic testing. X-linked CYBB gene mutations are common and autosomal recessive NCF1/NCF2 gene mutations less common.

10.
Chinese Journal of Neonatology ; (6): 734-739, 2023.
Article in Chinese | WPRIM | ID: wpr-1022534

ABSTRACT

Objective:To evaluate the efficacy and safety of umbilical venous catheterization (UVC) by using different methods to estimate the insertion depth.Methods:PubMed, Embase and The Cochrane Library, CBM (SinoMed), CNKI, VIP Database, Wanfang Database and Chinese Medical Journal Full-text Database were electronically searched for literature on randomized controlled trials (RCTs) comparing different methods for estimating the insertion depth of UVC. The search period was from the establishment date of the above databases to April 15th, 2022. If 2 or more studies were available for each pairwise comparison, a meta-analysis was conducted using the RevMan 5.3 software. However, if only 1 study was included for each pairwise comparison, SPSS 26.0 statistical software is used for statistical analysis using the chi-square test.Results:A total of 9 RCTs were included in the final meta-analysis, with a total of 1 281 infants, using 6 methods for estimating the depth of UVC. Compared with the Shukla formula, the Dunn method showed a statistically significant improvement in the correct place rate at the first UVC attempt ( RR=1.27, 95% CI 1.09-1.47, P=0.002). When comparing the Dunn method with the revised Shukla formula, the difference in the correct place rate at the first UVC attempt was statistically significant (21.4% vs. 33.5%, χ2=7.24, P=0.007). There was no statistically significant difference in the correct place rate at the first UVC attempt between the Dunn method and the formula based on BW, the JSS formula and the revised Shukla formula, and the method based on anatomical marks and the Shukla formula. There was no statistically significant difference in the incidence of UVC complications between the Dunn method and the Shukla formula, and between the method based on anatomical marks and the Shukla formula. Conclusions:Comparing to Shukla formula, Dunn method has a higher correct place rate at the first attempt. The incidence of complications is similar between the different pairwise comparison methods.

11.
Article in Chinese | WPRIM | ID: wpr-930809

ABSTRACT

Spontaneous intestinal perforation(SIP) is one of the causes of digestive tract perforation in premature infants and requires surgical intervention.Except for preterm delivery, the etiology and pathogenesis of SIP remain unclear.The studies have shown that it may be related to the application of magnesium sulfate, nonsteroidal anti-inflammatory drugs, hormones, vasoactive drugs and low perfusion status, infection, congenital intestinal dysplasia, non-invasive respiratory support, delayed nutrition initiation and other factors.In the management of premature infants, it is particularly important to improve the understanding of SIP, early diagnosis and treatment.

12.
Article in Chinese | WPRIM | ID: wpr-955107

ABSTRACT

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.

13.
Article in Chinese | WPRIM | ID: wpr-955151

ABSTRACT

Objective:To estimate the incidence and risk factors of acute kidney injury (AKI) in preterm infants, and provide basis for better evaluation and treatment of renal function in preterm infants.Methods:All the hospitalized premature infants who were admitted to three research centers (Department of Neonatology at Beijing Children′s Hospital; Department of Neonatology at Beijing Obstetrics and Gynecology Hospital; Department of Neonatology at Shunyi Maternal and Children′s Hospital of Beijing Children′s Hospital)from January 1, 2017 to June 30, 2019 and had more than two serum creatinine values or urine output were included.The incidence of AKI in preterm infants was calculated and the difference among different gestational weeks was compared.Preterm infants were divided into AKI and non-AKI groups according to AKI diagnostic criteria, and the clinical characteristics between two groups were compared, and the risk factors of AKI in preterm infants were analyzed.Results:A total of 763 premature infants were included in the analysis.Twenty two cases were diagnosed with AKI.The incidence of AKI in premature infants was 2.9%.The incidence of AKI was 33.3% (3/9), 3.7% (5/134), and 2.3% (14/620) in the 24-27 + 6 weeks, 28-31 + 6 weeks, and 32-36 + 6 weeks gestational age, respectively, and the difference was statistically significant ( χ2=31.010, P<0.001). Preterm infants in AKI group had a higher proportion of males(77.3% vs. 53.3%), lower gestational weeks[29(27, 33) weeks vs.31(29, 33)weeks], higher proportions of infants with diabetic mothers(40.9% vs.19.4%), lower Apgar scores at 1 and 5 minutes[8(7, 10) vs.9(8, 10), 9(9, 10) vs.10(9, 10), respectively], higher proportions of invasive and noninvasive respiratory support(45.5% vs.11.3%, 63.6% vs.19.2%, respectively), longer duration of invasive respiratory support[260(136, 742)h vs.72(18, 160)h], longer hospital stays[66(19, 88)d vs.42(26, 58)d], and higher rates of sepsis (27.3% vs. 6.5%), respiratory distress syndrome(40.9% vs. 11.6%), and patent ductus arteriosus that requiring ibuprofen or surgical closure(13.6% vs. 3.0%), diuretic(27.3% vs. 3.9%), and vasoactive drug use (22.7% vs. 3.6%) than those in non-AKI group, and the differences were statistically significant(all P<0.05). Multivariate regression analysis showed that sepsis was an independent risk factor for AKI in preterm infants ( P=0.039, OR=3.498, 95% CI 1.065-11.490) after adjustment of gestational age and birth weight. Conclusion:The incidence of AKI is relatively high in preterm infants with gestational age<28 weeks.Compared with preterm infants without AKI, preterm infants with AKI have smaller gestational weeks and longer hospital stay.Sepsis is an independent risk factor for AKI in preterm infants.

14.
Article in Chinese | WPRIM | ID: wpr-911962

ABSTRACT

Objective:To analyze the diagnosis, treatment, and outcomes of neonates with deep venous thrombosis (DVT) of the extremities and torso.Methods:The clinical diagnosis, treatment and outcomes of seven neonates with DVT of the extremities and torso admitted to Beijing Children's Hospital, Capital Medical University from March 2016 to March 2020 were retrospectively analyzed. Paired t test and paired rank sum test were used to compare the difference of coagulation indexes before and after the anticoagulant therapy. Results:Among the seven neonates with DVT of the extremities and torso, six were male and five were term infants, with the gestational age of (37.9±2.5) weeks and birth weight of (2 989±619) g. The median age at admission was 2.0 d and the age at diagnosis was 3.0 d. Except for one case of left common femoral vein thrombosis with limb swelling on the affected side, the other cases were all found with DVT by routine abdominal ultrasound examination after admission. Six cases received heparin treatment with the median duration of 8.5 d (1.8-28.8 d), including four cases of thrombosis in the portal venous, one in the postcava and renal venous, and one in the left common femoral vein. Among the six cases, the thrombus disappeared in five cases, which were confirmed by vascular ultrasound examination during follow-up, and in another case, the thrombus was shrinked significantly but remained. After the treatment, the platelet count [(464.5±128.9)×10 9/L vs (142.5±104.2)×10 9/L, t=-5.019, P=0.004] and antithrombin-Ⅲ level [(67.08±28.87)% vs (46.05±12.60)%, Z=-2.201, P=0.028] were increased and the D-dimers was decreased [0.392 mg/L(0.250-0.884 mg/L) vs 2.511 mg/L(0.755-14.033 mg/L), Z=-2.201, P=0.028] with no reports of heparin-related side-effect. One case with advanced postcaval thrombosis did not receive heparin anticoagulant therapy, but the thrombosis disappeared 270 d after diagnosis during follow-up. Conclusions:DVT of the extremities and torso may have no specific symptoms during the neonatal period and the overall prognosis is good. Heparin anticoagulant therapy is recommended until thrombosis disappears for patients with large thrombosis or significantly high level of D-dimer. The course of heparin treatment varies greatly among individuals, and close monitoring is required.

15.
Article in Chinese | WPRIM | ID: wpr-930784

ABSTRACT

Objective:To explore the clinical manifestations, genetic disorder, prognosis of 14 neonates with primary immunodeficiency disease(PID).Methods:A total of 14 newborns with PID admitted to Department of Neonatology at Beijing Children′s Hospital from January 2017 to December 2019 were enrolled for retrospective analysis, focusing on their clinical manifestation, peripheral blood cell examnations, gene mutation, and outcomes after hemotopoietic stem cell transplantation(HSCT).Results:The average gestational age of the newborn was (38.6±1.2) weeks, the birth weight was (3 265±325)g, and the median diagnosis time was 57.5 days.Fourteen newborns with PID were diagnosed by whole exome sequencing as chronic granuloma (6/14), DiGeogre syndrome (3/14), Wiskott-Aldrich syndrome (2/14), severe combined immunodeficiency (2/14) and selective IgA deficiency (1/14). Regarding the clinical manifestations, fever, pneumounia and colitis accounted for 7/14, the decrease of T lymphocytes in peripheral blood accounted for 6/14, and the decrease of B lymphocytes accounted for 5/14.The absolute value of eosinophils increased (>500 cells/mm 3) accounted for 12/14, of which moderately increased (1 500 to 5 000 cells/mm 3) accounted for 5/12, and the absolute value of monocytes increased (median>1.5×10 9/L) accounted for 7/14.Follow-up children received HSCT accounted for 7/14, and the median time of receiving transplantation was 330 days after birth.By the time of follow-up, the primary disease resolved after HSCT accounted for 5/7, and the survival rate was 85.7%.Among them, two children with chronic granulomatosis were diagnosed with inflammatory bowel disease before transplantation, and the primary disease improved after HSCT.Three-quarters of the deaths had inflammatory bowel disease-like manifestations and died of infectious shock. Conclusion:The clinical manifestations of children with PID during the neonatal period are not specific.The manifestations of colitis need more attention.Some of the newborns with PID will evolve into inflammatory bowel disease or have inflammatory bowel disease-like manifestations or even die of it.HSCT is a fundamental treatment for the primary disease.

16.
Article in Chinese | WPRIM | ID: wpr-908358

ABSTRACT

Objective:To explore the clinical characteristics and prognosis of children with inborn error of immunity (IEI) onset in the neonatal period.Methods:The clinical data of 21 cases of IEI neonates admitted to the Neonatal Center of Beijing Children′s Hospital were collected, and their clinical manifestations, peripheral blood test characteristics, genetic diagnosis, and primary disease during hospitalization were collected.The prognosis follow-up results were summarized and analyzed.Results:Twenty-one children with IEI were finally diagnosed by whole exome sequencing, including 15 cases of primary immunodeficiency(including 6 cases of chronic granulomatous disease, 3 cases of DiGeogre syndrome, 2 cases of Wiskott-Aldrich syndrome, 2 cases of severe combined immunodeficiency disease, 1 case of selective IgA deficiency, and 1 case of ectodermal dysplasia with immunodeficiency), 5 cases of infantile inflammatory bowel disease, and 1 case of familial haemophagocytic lymphohistiocytosis.Clinical manifestations of sepsis and colitis were the most common(accounting for 12/21), and 16/21 of the children had an increase in the absolute value of eosinophils(>0.5×10 9/L). Children received hematopoietic stem cell transplantation accounted for 7/21, and the median time of receiving transplantation was 11 months after birth.By the time of follow-up, the primary disease remission after hematopoietic stem cell transplantation accounted for 5/7.Among them, 2 cases were diagnosed with CGD associated inflammatory bowel disease before transplantation, and the primary disease resolved after hematopoietic stem cell transplantation.Of the 14 children who did not receive hematopoietic stem cell transplantation, 10 children died.Five of the 11 deaths were treated with systematic steroid before diagnosised. Conclusion:The clinical manifestations of IEI in the neonatal period are not specific.Sepsis and colitis are the most common manifestations.Most of the cases have elevated eosinophils in the peripheral blood.Systematic streoid therapy needs to be cautious, and timely evaluation for hematopoietic stem cells transplantation is an effective option to resolution.

17.
Article in Chinese | WPRIM | ID: wpr-908516

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Objective:To study the clinical features and prognosis of infantile hepatic hemangioendothelioma-arteriovenous fistula (IHHE-AVF) complicated with heart failure in neonates.Method:From May 2016 to June 2020, neonates with IHHE-AVF complicated with heart failure admitted were retrospectively studied. The clinical presentation, treatment and outcomes were analyzed.Result:A total of 11 cases of IHHE-AVF complicated with heart failure were enrolled (male 5, female 6). The onset age of heart failure was 12.0 (0.0, 17.0) d. 6 cases showed IHHE on fetal ultrasound. All patients had significantly enlarged heart on chest X-ray. All patients had decreased left ventricular systolic function and pulmonary hypertension on echocardiography. All patients required respiratory support and 6 of them were intubated. 3 cases received conservative treatment (all dead). 1 case received surgery (dead). 7 cases received interventional therapy at the age of (25.6±18.5) d. 1 case was dead, and the other 6 cases were improved and discharged. All the 6 cases were followed up to 3~18 months. None of them had heart failure again. The IHHE were shrunk or completely disappeared. Coagulation function and platelet count were normal.Conclusion:The fatality rate of neonatal-onset IHHE-AVF complicated with heart failure is extremely high. Interventional therapy may be more effective than conservative therapy and surgery.

18.
Article in Chinese | WPRIM | ID: wpr-885579

ABSTRACT

Umbilical venous catheterization (UVC) is associated with many complications, such as displacement of the catheter, infection, exudation, thrombosis, pericardial effusion/pleural effusion, of which central line-associated bloodstream infections has been of long-standing interest. There is currently no optimal method for estimating the depth of UVC insertion. Ultrasound examination can be used to evaluate the position of the end of the tube after catheterization to avoid organ damage and complications caused by displacement and dislocation. However, whilst it is known that there is a correlation between the duration of UVC and central line-associated bloodstream infection, a consensus is yet to be reached regarding the optimal duration of UVC retention. More, high quality evidence through multi-center, prospective randomized controlled study is needed.

19.
Article in Chinese | WPRIM | ID: wpr-864176

ABSTRACT

Objective:To summarize the clinical characteristics and treatment outcome of neonates with laryngopharyngeal congenital structural abnormalities in intensive care unit.Methods:The clinical data of neonates with congenital laryngopharyngeal structural abnormalities in the Neonatal Intensive Care Unit of the National Center of Beijing Children′s Hospital, Capital Medical University from January 2017 to December 2018 were retrospectively analyzed.The general data, birth status, disease types and clinical characteristics of abnormal laryngeal structure, complications, treatment and follow-up of some children with special diseases were summarized.These neonates were divided into the operation group and the conservative treatment group according to treatment methods, and then the outcomes of the two groups were compared.Results:A total of 133 cases of neonates with laryngopharyngeal congenital structural abnormalities were enrolled, including 73 cases(54.88%) with laryngomalacia, and 60 cases(45.12%) with special structural abnormalities.Of 60 cases with special structural abnormalities, 26 cases (19.54%) had pharynx and larynx cysts, 18 cases (13.53%) had vocal cord paralysis, 4 cases (3.00%) had laryngeal cleft, 2 cases (1.50%) had subglottic hemangioma, 3 cases (2.25%) had Pireer Robin, 1 case (0.75%) had laryngeal poof, 5 cases (3.75%) had pharynx softening, 1 case (0.75%) had subglottic stenosis.Nine patients had special structural abnormalities and laryngomalacia simultaneously.Fiber nasopharyngoscope and enhanced CT were main auxiliary examinations.Twenty-two(16.5%) cases received surgical treatment.Conclusions:Early diagnosis is needed for the neonates and abnormal laryngeal structure.The best treatment scheme should be evaluated according to the condition of the newborn.For some acute cases, early operation and multidisciplinary comprehensive treatment are warranted.

20.
Article in Chinese | WPRIM | ID: wpr-871104

ABSTRACT

Objective:To analyze the diagnosis and treatment of neonatal neuroblastoma (NB) by summarizing its clinical characteristics.Methods:This study retrospectively recruited 14 neonates with NB in Beijing Children's Hospital (National Center for Children's Health) from February 1, 2015, to February 1, 2020. Medical records and follow-up data as of February 29, 2020, were collected, and clinical staging based on International Neuroblastoma Staging System, risk grouping based on American Childhood Oncology Group risk grouping system, diagnosis, treatment, and prognosis were analyzed. According to whether surgical treatment was performed in the neonatal period or not, these subjects were divided into surgical and non-surgical groups. A descriptive statistical analysis was used for data analysis.Results:(1) Neonates with NB accounted for 0.063% (14/22 006) of the total number of newborns admitted to the hospital during the same period. The 14 cases were all full-term aged 15 d (8 h-23 d) at admission. Tumors were found in seven cases in prenatal examinations, while others presented with shortness of breath (three cases), abdominal distension (two cases), fever (one case), and dysuria and difficult defecation after birth (one case). (2) The primary tumor sites included the adrenal gland (eight cases), posterior mediastinum (three cases), retroperitoneum (two cases), and sacrococcygeal (one case). Three cases had extensive diffuse liver metastasis at admission. (3) Except for two cases who refused to examine, the serum neuron-specific enolase of 12 cases was 57.2 ng/ml (35.9-158.3 ng/ml) during hospitalization, and the urine vanillyl-mandelic acid creatinine of four cases was 2 304.940 (685.748-9 595.314). (4) Primary tumor sites were found in 14 cases by imaging examination. Bone scanning was performed in three cases, including one with a concentrated shadow of the right sacroiliac joint and two with no abnormalities. Ten cases underwent bone marrow aspiration and all with normal results. (5) Of the ten neonates received surgery (the surgical group), nine had the primary tumor wholly removed, without chemotherapy after the operation, and the tumor-free survival period was 19 months (1-45 months). One case (case 5) had a substantial primary tumor that could not be completely resected. The patient underwent a second surgery five months after the first operation due to disease progression and received postoperative chemotherapy. The child had stopped chemotherapy for 24 months and survived without a tumor. (6) In the non-surgical group (cases 11 to 14), the tumor in case 11 who refused chemotherapy shrank spontaneously after discharge, and the patient survived for 20 months with the tumor. The parents of the case 12 withdrew treatment during hospitalization, while the primary tumor and metastases disappeared after discharge, and the specific tumor markers gradually decreased to normal levels. The patient has been tumor-free survived for 25 months. Case 13 received mediastinal tumor resection and chemotherapy during infancy. At the end of the follow-up, chemotherapy had been stopped for 12 months, and the patient survived without a tumor. Case 14 withdrew treatment and died. (7) Among the ten cases in the surgical group, one patient's pathological result indicated a composite tumor, while the others were low differentiated neuroblastoma. There was no MYCN gene amplification, 1p36 deletion, or 11q23 deletion in the ten cases. (8) Among the ten children in the surgical group, nine were in stage 1, and one was in stage 4S (case 5). Nine cases were classified into extremely low-risk groups, while the other was in the low-risk group. The four cases in the non-surgical group could not be grouped by risk. Conclusions:Clinical manifestations of neonatal NB are often atypical. NB in stage 4S might resolve spontaneously, and expectant observation may be considered. The overall prognosis of neonatal NB is generally good, but further researches are needed.

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