Frontiers in Pediatrics
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Updated Friday, 03 December 2021

2021 ◽  
Vol 9 ◽  
Author(s):  
Zhili Wang ◽  
Yu He ◽  
Xiaolong Zhang ◽  
Zhengxiu Luo

Background: Multiple non-invasive ventilation (NIV) modalities have been identified that may improve the prognosis of pediatric patients with acute lower respiratory infection (ALRI). However, the effect of NIV in children with ALRI remains inconclusive. Hence, this study aimed to evaluate the efficacy of various NIV strategies including continuous positive airway pressure (CPAP), high flow nasal cannula (HFNC), bilevel positive airway pressure (BIPAP), and standard oxygen therapy in children with ALRI and the need for supplemental oxygen.Methods: Embase, PubMed, Cochrane Library, and Web of Science databases were searched from inception to July 2021. Randomized controlled trials (RCTs) that compared different NIV modalities for children with ALRI and the need for supplemental oxygen were included. Data were independently extracted by two reviewers. Primary outcomes were intubation and treatment failure rates. Secondary outcome was in-hospital mortality. Pairwise and Bayesian network meta-analyses within the random-effects model were used to synthesize data. The certainty of evidence was assessed using the Grading of Recommendations Assessment, Development and Evaluation framework.Results: A total of 21 RCTs involving 5,342 children were included. Compared with standard oxygen therapy, CPAP (OR: 0.40, 95% CrI: 0.16–0.90, moderate quality) was associated with a lower risk of intubation. Furthermore, both CPAP (OR: 0.42, 95% CrI: 0.19–0.81, low quality) and HFNC (OR: 0.51, 95% CrI: 0.29–0.81, low quality) reduced treatment failure compared with standard oxygen therapy. There were no significant differences among all interventions for in-hospital mortality. Network meta-regression showed that there were no statistically significant subgroup effects.Conclusion: Among children with ALRI and the need for supplemental oxygen, CPAP reduced the risk of intubation when compared to standard oxygen therapy. Both CPAP and HFNC were associated with a lower risk of treatment failure than standard oxygen therapy. However, evidence is still lacking to show benefits concerning mortality between different interventions. Further large-scale, multicenter studies are needed to confirm our results.Systematic Review Registration: https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=172156, identifier: CRD42020172156.


2021 ◽  
Vol 9 ◽  
Author(s):  
Benno Kohlmaier ◽  
Heidemarie Holzmann ◽  
Karin Stiasny ◽  
Manuel Leitner ◽  
Christoph Zurl ◽  
...  

Background: Administration of measles virus (MV)-specific IgG as post-exposure prophylaxis (PEP) is known to effectively prevent measles. Since the introduction of active immunization against measles, the levels of MV-specific IgG antibodies in the population have dropped. Therefore, the concentration of MV-specific antibodies in immunoglobulin products derived from human plasma donors has declined as the proportion of vaccinated donors has increased. Literature on the effectiveness of PEP with current available immunoglobulins is limited. Here we examine the effectiveness of 400 mg/kg intravenous immunoglobulin (IVIG) (IgVena®, Kendrion) as PEP in infants during a measles outbreak in Austria, 2019.Methods: After exposure to a highly contagious measles patient, identified infants were evaluated for eligibility for IVIG PEP. Infants were tested for measles maternal antibodies, if the result was expected to be available within 72 h after exposure. IVIG was administered to eligible infants with negative maternal IgG antibody levels (n = 11), infants with protective levels but result beyond 72 h (n = 2) and infants not tested for maternal IgG antibodies (n = 52). Telephone enquiries were made asking for measles infection. Effectiveness was calculated using exact logistic regression. Samples of four out of seven used IVIG batches were tested for MV-neutralizing antibody capacity.Results: In 63 (96.9%) of 65 infants PEP with IVIG was administered. The parents of two infants declined IVIG PEP. None of the infants with IVIG PEP got measles or symptoms suggestive for measles, but both infants who did not receive PEP were infected. Effectiveness of IVIG PEP was calculated to be 99.3% (CI 95%: 88.7–100%). No serious adverse event of IVIG treatment was observed. The investigation on MV-neutralizing antibody capacity showed a geometric mean titer ranging from 10.0 to 12.7 IU/ml, resulting in a 1.57–2.26-fold higher concentration than postulated as minimum level for immunity.Conclusions: Our findings suggest that the used IVIG preparation provided an at least non-inferior protection rate compared to IVIG preparations derived from donors before the global introduction of standard active immunization against measles.


2021 ◽  
Vol 9 ◽  
Author(s):  
Nadim Cassir ◽  
Isabelle Grandvuillemin ◽  
Manon Boxberger ◽  
Priscilla Jardot ◽  
Farid Boubred ◽  
...  

Necrotizing enterocolitis is a life-threatening acquired gastrointestinal disorder among preterm neonates and is associated with a high mortality rate and long-term neurodevelopmental morbidity. No etiologic agent has been definitively established; nonetheless, the most implicated bacteria include members of the Clostridium genus. We reported here on a case of Clostridium neonatale bacteremia in a preterm neonate with necrotizing enterocolitis, providing more information regarding the potential role of this bacterium in pathogenesis of necrotizing enterocolitis. We emphasized the sporulating form of C. neonatale that confers resistance to disinfectants usually applied for the hospital environmental cleaning. Further works are needed to establish the causal relationship between the occurrence of NEC and the isolation of C. neonatale, with promising perspectives in terms of diagnostic and therapeutic management.


2021 ◽  
Vol 9 ◽  
Author(s):  
Magdalena Klaniewska ◽  
Krystian Toczewski ◽  
Anna Rozensztrauch ◽  
Michal Bloch ◽  
Agata Dzielendziak ◽  
...  

The MYCN oncogene encodes a transcription factor belonging to the MYC family. It is primarily expressed in normal developing embryos and is thought to be critical in brain and other neural development. Loss-of-function variants resulting in haploinsufficiency of MYCN, which encodes a protein with a basic helix–loop–helix domain causes Feingold syndrome (OMIM 164280, ORPHA 391641). We present an occurrence of esophageal atresia (EA) with tracheoesophageal fistula in siblings from a three-generation family affected by variable expressivity of MYCN mutation p.(Ser90GlnfsTer176) as a diagnostic effect of searching the cause of familial esophageal atresia using NGS-based whole-exome sequencing (WES). All of our affected patients showed microcephaly and toe syndactyly, which were frequently reported in the literature. Just one patient exhibited clinodactyly. None of the patients exhibited brachymesophalangy or hypoplastic thumbs. The latest report noted that patients with EA and Feingold syndrome were also those with the more complex and severe phenotype. However, following a thorough review of the present literature, the same association was not found, which is also confirmed by the case we described. The variable phenotypic expression of the patients we described and the data from the literature guide a careful differential diagnosis of Feingold syndrome even in cases of poorly expressed and non-specific symptoms.


2021 ◽  
Vol 9 ◽  
Author(s):  
Na Li ◽  
Jia Hao ◽  
Tong Fu ◽  
Yue Du

Objective: This study aims to investigate the quality of life of children with primary nephrotic syndrome (PNS), assess their parents' disease awareness, and provide a basis for the comprehensive management of children with PNS.Methods: A total of 231 children with PNS who were hospitalized in the Department of Pediatric Renal Rheumatology and Immunology in the ShengJing Hospital of the China Medical University from March 2019 to October 2020 were selected as the study subjects. The subjects and their parents were surveyed via a disease education and communication WeChat group and online questionnaire to investigate the children's quality of life, the needs of the parents, and their knowledge related to the disease.Results: In 93.51% of cases, the child's quality of life was affected, with mild to moderate effects being the most frequent (90.47%). The lowest overall quality of life scores were recorded for children who had been diagnosed 1–3 year prior to inclusion in the study, and the scores plateaued thereafter. On the physical functioning scale, the longer the illness, the greater the physical impact, with children typically experiencing pain and fatigue. The children generally scored low on the emotional functioning scale, exhibiting sleep disturbances for up to 5 years and worrying about accidents. The children's average score on the social functioning scale was high, with males achieving significantly higher scores (69.61 ± 25.42) than females (62.30 ± 27.51), and more than one-third of the children experiencing problems getting along with other teenagers and making friends. The primary problems expressed by parents were anxiety (59%), sadness (44%), fear (43%), and depression (40%), and several parents indicated that they struggled with issues of self-blame.Conclusion: PNS impacts the physical and psychological wellbeing of children suffering from the condition, significantly reduces their quality of life, and negatively impacts the psychological wellbeing of their parents. Therefore, children with PNS and their families need integrated management by doctors, nurses, dieticians, psychotherapists, educational institutions, and social stakeholders to improve their quality of life.


2021 ◽  
Vol 9 ◽  
Author(s):  
Suhua Xu ◽  
Peng Zhang ◽  
Liyuan Hu ◽  
Wenhao Zhou ◽  
Guoqiang Cheng

Objective: The aim of this single-center retrospective study was to analyze the clinical characteristics, treatment options, and course of neonatal-onset congenital portosystemic shunts (CPSS).Methods: We included all patients with CPSS who presented with clinical symptoms within the neonatal period in our institution between 2015 and 2020.Results: Sixteen patients were identified, including 13 patients with intrahepatic portosystemic shunts (IPSS) and three patients with extrahepatic portosystemic shunts (EPSS). The median age of diagnosis was 16 days (range prenatal 24 weeks−12 months). Hyperammonemia (60%), neonatal cholestasis (44%), elevated liver enzyme (40%), hypoglycemia (40%), thrombocytopenia (38%), and coagulation abnormalities (23%) appeared in neonatal CPSS. Twelve patients (75%) presented with congenital anomalies, of which congenital heart disease (CHD) (44%) was the most common. Thirteen patients with IPSS initially underwent conservative treatment, but two of them were recommended for the catheter interventional therapy and liver transplantation, respectively, due to progressive deterioration of liver function. Spontaneous closure occurred in nine patients with IPSS. The shunt was closed using transcatheter embolization in one patient with EPSS type II. Another patient with EPSS type II underwent surgical treatment of CHD firstly. The remaining patient with EPSS type Ib received medical therapy and refused liver transplantation.Conclusion: Hyperammonemia, neonatal cholestasis, elevated liver enzyme, hypoglycemia, and thrombocytopenia are the main complications of neonatal CPSS. Moreover, CPSS is associated with multiple congenital abnormalities, especially CHD. Intrahepatic portosystemic shunts may close spontaneously, and conservative treatment can be taken first. Extrahepatic portosystemic shunts should be closed to prevent complications.


2021 ◽  
Vol 9 ◽  
Author(s):  
Xiaolei Shi ◽  
Yuepeng Hu ◽  
Na Pu ◽  
Guofu Zhang ◽  
Jingzhu Zhang ◽  
...  

Background: Acute pancreatitis in pregnancy is a rare but highly life-threatening gestational and perinatal disease.Objective: This study aimed to identify the risk factors for fetal death and acute pancreatitis severity.Methods: This retrospective cohort study enrolled patients with acute pancreatitis in pregnancy in our center from January 1, 2012, to August 1, 2020, and classified them according to two clinical endpoints, fetal outcome and disease severity. The groups were examined and compared according to gestational week, etiology, gravidity and parity, complications in pre- and post-delivery, and medical history. Logistic regression analysis was performed to identify the independent risk factors for fetal death and acute pancreatitis severity.Results: Of the 90 enrolled patients, 28 (31.1%) had fetal death and 43 (47.8%) had severe acute pancreatitis. Logistic regression analysis showed that pre-delivery acute respiratory distress syndrome (OR, 5.8; 95% CI, 1.5–22.4; p = 0.010) and gestational week (OR, 0.9; 95% CI, 0.8–1.0; p = 0.011) were risk factors for fetal death. Gestation week (OR, 1.2; 95% CI, 1.1–1.3; p = 0.003) and fetal intrauterine death (OR, 5.9; 95% CI, 1.8–19.4; p = 0.003) were risk factors for severe acute pancreatitis.Conclusions: Pre-delivery acute respiratory distress syndrome and gestational week were independent risk factors for fetal death. Fetal intrauterine death and gestational week were independent risk factors for severe acute pancreatitis.


2021 ◽  
Vol 9 ◽  
Author(s):  
Juin Yee Kong ◽  
Srabani Samanta Bharadwaj ◽  
Amutha Chinnadurai ◽  
Selina Kah Ying Ho

Background: Rapid spread of the COVID-19 pandemic raised an urgent need for preparedness in the healthcare sector, including training of healthcare workers to cope with the burden of infected cases while ensuring proper protection of themselves. Improper infection prevention and control measures were key reasons for infection in healthcare workers during the early phase of the outbreak.Objectives/Methods: This paper describes the combined approach of 3 restructured hospitals in Singapore in preparing and training neonatal healthcare workers' during the COVID-19 pandemic crisis, as well as lessons learnt during this process.Results: Information sharing was conducted in the form of e-learning, emphasizing on topics like disease knowledge and infection prevention and control procedures. Skills and competency training were carried out in the form of simulation, with sessions scaled into 4 levels progressing from individual task training to larger group simulations involving multiple disciplines and departments. Challenges encountered included information fatigue by large amount of constantly changing information and multiple amendments to workflows as more information arose. Difficulties conducting training and simulation sessions included restriction of group size to mitigate infection risk amongst participants and the limited supply of personal protective equipment prioritized for direct patient care.Conclusion: Healthcare institutions should ensure adequate dissemination of conceptual knowledge as well as skills competency training of staff in infection control measures for the protection of healthcare workers and patient safety. Ongoing training for sustainability of knowledge and skills, while adapting to the rapidly evolving situation is important in the preparation for future outbreaks.


2021 ◽  
Vol 9 ◽  
Author(s):  
Jianli Zhou ◽  
Yuzhen Zhao ◽  
Xia Qian ◽  
Yongwei Cheng ◽  
Huabo Cai ◽  
...  

Background: Congenital sucrase-isomaltase deficiency (CSID) is an autosomal recessive inherited disease that leads to the maldigestion of disaccharides and is associated with mutation of the sucrase-isomaltase (SI) gene. Cases of CSID are not very prevalent in China or worldwide but are gradually being identified and reported.Case Presentation: We report a case involving a 14-month-old male who presented with failure to thrive that had begun after food diversification and was admitted for chronic diarrhea. We used a whole-exome sequencing (WES) approach to identify mutations in this patient's genome. WES revealed two novel heterozygous mutations in the SI gene, c.2626C > T (p.Q876*) and c.2872C > T (p.R958C), which were confirmed by Sanger DNA sequencing. With a strict sucrose- and starch-restricted diet, the patient's diarrhea was resolved, and he began to gain weight.Conclusions: We report a case of novel variants in the SI gene that caused CSID. This report provides valuable information for the clinical field, especially in China.


2021 ◽  
Vol 9 ◽  
Author(s):  
Yao Cheng ◽  
Haoyue Teng ◽  
Yue Xiao ◽  
Mengxin Yao ◽  
Jieyun Yin ◽  
...  

Background: Previous studies on the pneumonia outbreak caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have focused on the general population and pregnant women, while little is known about the effects of SARS-CoV-2 on retardation during and after pregnancy. The purpose of this study was to evaluate the potential influence of SARS-CoV-2 on infant neurobehavioral development.Methods: A case-control study was conducted in Wuhan Maternal and Child Health Hospital, China. Nine pregnant women with SARS-CoV-2 infection and 9 controls matched by maternal age, parity, and status of chronic disease were included. Infantile neurobehavioral development was assessed through the Ages and Stages Questionnaires Edition 3 (ASQ-3).Results: The majority of pregnant women with SARS-CoV-2 experienced cesarean section (7 of 9), which was higher than the control group (5 of 9). The throat swabs of all newborn were negative. We found that compared with the control group, neonates of mothers with SARS-CoV-2 infection during pregnancy had lower scores in communication, gross movement, fine movement, problem solving, and personal-social domains; but only fine movement domain yielded statistical significance (P = 0.031).Conclusion: Infection with SARS-CoV-2 during pregnancy may have a certain impact on infant neurobehavioral development. Further studies with larger sample size are warranted for validation.


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