Although there are unified criteria for the clinical diagnosis and grading of neonatal hypoxic-ischemic encephalopathy (HIE), clinical features and neuropathological patterns vary considerably among the neonates with HIE due to birth asphyxia in the same classification. The patterns and progression of brain injury in HIE, which is closely associated with long-term neurodevelopment outcomes, can be well shown on magnetic resonance imaging (MRI), but different sequences may lead to different MRI findings at the same time. It is suggested that diffusion-weighted imaging sequence be selected at 2-4 days after birth, and the conventional MRI sequence at 4-8 days. The major patterns of brain injury in HIE on MRI are as follows:injury of the thalamus and basal ganglia and posterior limbs of the internal capsules; watershed injury involving the cortical and subcortical white matter; focal or multifocal minimal white matter injury; extensive whole brain injury. Severe acute birth asphyxia often leads to deep grey matter injury (thalamus and basal ganglia), and the brain stem may also be involved; the pyramidal tract is the most susceptible white matter fiber tract; repetitive or intermittent hypoxic-ischemic insults, with inflammation or hypoglycemia, usually cause injuries in the watershed area and deep white matter. It is worth noting that sometimes the pattern of brain injury among those described above cannot be determined exactly, but rather a predominant one is identified; not all cases of HIE have characteristic MRI findings.

Objective To examine the clinical features of children with acute lymphoblastic leukemia (ALL) complicated by pulmonary infection after chemotherapy. Methods The clinical data of 108 ALL children (115 case-times) with post-chemotherapy pulmonary infection were retrospectively reviewed. The risk factors for pulmonary infection and the relationship between pathogens and chest CT findings were evaluated. Results The highest incidence (77.4%) of pulmonary infection occurred during remission induction, peaking at 31-60 days after chemotherapy. Patients with neutropenia had the highest incidence rate of pulmonary infection (67.0%). Bacteria (36%) and fungi (41%) were the two most common pathogens in the 41 patients who were etiologically suspected of or diagnosed with pulmonary infection. There was no significant difference in chest CT findings between patients with bacterial and fungal infections. Conclusions The children with ALL are most susceptible to pulmonary infection during remission induction, especially when they are neutropenic. Bacteria and fungi are the main pathogens of pulmonary infections in these patients. However, the changes in chest CT images are poor indicators of the nature of pulmonary infection.

Objective To investigate the cardiac autonomic nerve function in girls with idiopathic central precocious puberty (ICPP). Methods A total of 66 girls with ICPP were enrolled, among whom 36 were obese and 30 were not obese. A total of 68 age-matched healthy girls (normal controls) and 51 girls with simple obesity were enrolled as controls. All the subjects underwent 24-hour ambulatory electrocardiography, and deceleration capacity of heart rate (DC), acceleration capacity of heart rate (AC), and heart rate variability (HRV), and body mass index (BMI) were compared between groups. Results Compared with the normal control group, the ICPP group had significantly lower DC, standard deviation of normal-to-normal R-R intervals (SDNN), standard deviation of the average normal-to-normal intervals (SDANN), root mean square of successive differences (RMSSD), and high-frequency power (HF) and significantly higher AC and BMI. The ICPP group had significantly lower RMSSD and BMI than the simple obesity group (P < 0.05). Compared with the ICPP girls without obesity, those with obesity had significantly lower DC, RMSSD, and HF and significantly higher AC and BMI (P < 0.05). Conclusions Cardiac autonomic dysfunction is seen in girls with ICPP, especially those with obesity, mainly presenting with reduced vagal tone.

Objective To explore the value of urine gas chromatography-mass spectrometry (GC-MS) in the screening of children at risk of inherited metabolic diseases (IMD), and to identify the disease spectrum of IMD and the clinical characteristics of children with IMD. Methods The clinical data of 15 851 children at risk of IMD who underwent urine GC-MS in the Tianjin Children's Hospital between February 2012 and December 2016 were retrospectively analyzed. Results In the 15 851 children, 5 793 (36.55%) were detected to have metabolic disorders. A total of 117 (0.74%) children were confirmed to have IMD, including 77 cases of methylmalonic acidemia (65.8%). The clinical manifestations of confirmed cases in the neonatal period mainly included jaundice, metabolic acidosis, abnormal muscular tension, feeding difficulty, poor response, and lethargy or coma. The clinical manifestations of confirmed cases in the non-neonatal period mainly included delayed mental and motor development, metabolic acidosis, convulsion, recurrent vomiting, and anemia. Conclusions GC-MS is an effective method for the screening for IMD in children at risk. Methylmalonic acidemia is the most common IMD. The clinical manifestations of IMD are different between the confirmed cases in the neonatal and non-neonatal periods.

Objective To investigate the characteristics of tidal breathing pulmonary function in children with tracheobronchomalacia (TBM). Methods In this study, 30 children who were diagnosed with TBM using electronic bronchoscopy were enrolled in the observation group; 30 healthy children were recruited in the normal control group. For individuals in each group, the assessment of tidal breath pulmonary function was performed at diagnosis and 3, 6, 9, and 12 months after diagnosis. Results There were no significant differences in tidal volume, inspiratory time, expiratory time, and inspiratory to expiratory ratio between the two groups (P > 0.05). Compared with the control group, the observation group had a significantly higher respiratory rate and significantly lower ratio of time to peak tidal expiratory flow to total expiratory time (TPTEF/TE) and ratio of volume to peak tidal expiratory flow to total expiratory volume (VPTEF/VE). There was a time-dependent increase in TPTEF/TE and VPTEF/VE for TBM children from the time of initial diagnosis to 12 months after diagnosis. Conclusions Tidal breathing pulmonary function has characteristic changes in children with TBM. Tidal breathing pulmonary function tends to be recovered with increased age in children with TBM.

Objective To investigate the differences between the Test of Infant Motor Performance (TIMP) data from the infants at 38-58 weeks of postconceptual age in three hospitals in Chongqing, China and the America norms, and to provide a reference for the introduction and application of TIMP in China. Methods TIMP was used to assess 642 infants with 38-58 weeks of postconceptual age who visited the departments of preterm infants or child healthcare in the Second Affiliated Hospital of Army Medical University, Shapingba Maternal and Child Health Hospital in Chongqing, and Chongqing Maternal and Child Health Hospital between January and December, 2016. The assessment scores were analyzed and compared with the America norms. Results The TIMP scores increased with the increasing postconceptual age, with 37±5 points in the 38-39⁺⁶ week group and 83±12 points in the 56-57⁺⁶ week group. All age groups had a significantly lower mean score than the America norms (P < 0.001). Conclusions TIMP scores can reflect the motor performance in infants with various postconceptual ages. The TIMP scores from the infants with a postconceptual age of 38-58 weeks in three hospitals in Chongqing are significantly different from the America norms, suggesting that it is very necessary in China to establish the Chinese norms for assessing motor performance in infants using TIMP.

Objective To study the reference ranges of six sex hormones, i.e., luteinizing hormone, follicle-stimulating hormone, progesterone, prolactin, estradiol, and testosterone, for healthy children aged 0-18 years in Shenzhen, China. Methods Stratified cluster sampling was performed to select 2 178 healthy children aged 0-18 years in the districts of Futian, Luohu, Nanshan, Bao'an, and Longgang in Shenzhen between September 2015 and September 2016. There were 1 219 boys and 959 girls, including 81 neonates, 335 infants, 346 young children, 469 preschool children, 419 school-aged children, and 528 adolescents. The American Beckman DXI800 chemiluminescence meter was used to measure the levels of luteinizing hormone, follicle-stimulating hormone, progesterone, prolactin, estradiol, and testosterone. Results There were significant differences in the levels of luteinizing hormone, follicle-stimulating hormone, progesterone, prolactin, estradiol, and testosterone between different age groups (P < 0.05). There were also significant differences in the levels of these sex hormones between boys and girls in the same age group (P < 0.05). The reference ranges of six sex hormones were established for healthy children aged 0-18 years in Shenzhen based on the levels of these hormones in different age groups. Conclusions There are significant differences in sex hormones between different age groups or sex groups. The reference ranges of six sex hormones established for different sexes or ages have great significance in the diagnosis and treatment of endocrine diseases in children.

Li-Fraumeni syndrome (LFS) is a hereditary cancer predisposition syndrome, with the characteristics of early onset of cancer and high cancer incidence. TP53 is widely accepted as a pathogenic gene of LFS. A 2 years and 6 months old boy is reported in this article, who was diagnosed with embryonal rhabdomyosarcoma (RMS) in the left submandibular region. His brother died of RMS, and his grandmother was diagnosed with breast cancer. TP53 gene mutation detection was performed in this patient and some family members, indicating a missense mutation in exon 8 of the patient:c.844C > T (p.Arg282Trp, heterozygous). TP53 mutation was also found in his mother and sister. The boy met the diagnostic criteria for LFS. Among pediatric patients, the most common LFS diseases include osteosarcoma, adrenocortical cancer, central nervous system tumor, and soft tissue tumor. Additionally, leukemia and lymphoma are also involved. LFS patients have a high risk to suffer secondary or even multiple cancers. Therefore, it is necessary to perform genetic detection for pediatric cancer patients, especially those with hereditary predisposition cancers. TP53 mutation often indicates poor prognosis, so it is important to take active treatment and systematic monitoring for LFS family.

No abstract available

Objective To investigate the neuroprotective effect of leptin by observing its effect on spatial memory of rats with white matter damage in developing brain. Methods A total of 80 neonatal rats were randomly divided into 3 groups:sham-operation (n=27), model (n=27) and leptin intervention (n=27). The rats in the model and leptin intervention groups were used to prepare a model of white matter damage in developing brain, and the rats in the leptin intervention group were given leptin (100 μg/kg) diluted with normal saline immediately after modelling for 4 consecutive days. The survival rate of the rats was observed and the change in body weight was monitored. When the rats reached the age of 21 days, the Morris water maze test was used to evaluate spatial memory. Results There was no significant difference in the survival rate of rats between the three groups (P > 0.05). Within 10 days after birth, the leptin intervention group had similar body weight as the sham-operation group and significantly lower body weight than the model group (P < 0.05); more than 10 days after birth, the leptin intervention group had rapid growth with higher body weight than the model and sham-operation groups (P > 0.05). The results of place navigation showed that from the second day of experiment, there was a significant difference in the latency period between the three groups (P < 0.05); from the fourth day of experiment, the leptin intervention group had a similar latency period as the sham-operation and a significantly shorter latency period than the model group (P < 0.05). The results of space search experiment showed that compared with the sham-operation group, the model group had a significant reduction in the number of platform crossings and a significantly longer latency period (P < 0.05); compared with the model group, the leptin intervention group had a significantly increased number of platform crossings and a significantly shortened latency period (P < 0.05), while there was no significant difference between the leptin intervention and sham-operation groups. Conclusions Leptin can alleviate spatial memory impairment of rats with white matter damage in developing brain. It thus exerts a neuroprotective effect, and is worthy of further research.

Objective To investigate the changes in the expression of EphA5 and its ligand ephrinA5 in the hippocampus of rats with epilepsy and their role in the pathogenesis of temporal lobe epilepsy (TLE). Methods A total of 240 Sprague-Dawley rats were randomly divided into control group and TLE group, with 120 rats in each group. A rat model of lithium-pilocarpine TLE was established, and then the rats were divided into subgroups at 12 and 24 hours and 7, 15, 30, and 60 days after epilepsy was induced. In-situ hybridization was used to measure the mRNA expression of ephrinA5 in the CA3 region and the dentate gyrus of the hippocampus in 9 rats; immunohistochemistry was used to measure the protein expression of EphA5 in the CA3 region and the dentate gyrus of the hippocampus in 9 rats; Neo-Timm silver staining was used to observe mossy fiber sprouting in the CA3 region of the hippocampus in 2 rats. Results In-situ hybridization showed mRNA expression of ephrinA5 in the CA3 region of the hippocampus, but this was not found in the dentate gyrus. Compared with the control group at the same time point, the TLE group had a significant reduction in the mRNA expression of ephrinA5 in the CA3 region of the hippocampus at 7 and 15 days after epilepsy was induced (P < 0.05); at 30 and 60 days after epilepsy was induced, the TLE group had a gradual increase in the mRNA expression of ephrinA5 in the CA3 region of the hippocampus, and there was no significant difference between the TLE and control groups (P > 0.05). Immunohistochemistry showed that EphA5 protein was expressed in the CA3 region and the dentate gyrus of the hippocampus and had a similar trend of change as ephrinA5 mRNA. Neo-Timm silver staining showed that the TLE group developed marked mossy fiber sprouting in the CA3 region of the hippocampus at 7 and 15 days after epilepsy was induced. Conclusions Downregulation of ephrinA5 and EphA5 in the CA3 region of the hippocampus may participate in the mechanism of mossy fiber sprouting and is closely associated with the development and progression of epilepsy.

Objective To investigate the molecular mechanism of action of BET bromodomain inhibitor JQ1 in treating airway remodeling in asthmatic mice. Methods A total of 24 mice were randomly divided into control group, ovalbumin (OVA)-induced asthma group (OVA group), and JQ1 intervention group (JQ1+OVA group), with 8 mice in each group. OVA sensitization/challenge was performed to establish a mouse model of asthma. At 1 hour before challenge, the mice in the JQ1+OVA group were given intraperitoneal injection of JQ1 solution (50 μg/g). Bronchoalveolar lavage fluid (BALF) and lung tissue samples were collected at 24 hours after the last challenge, and the total number of cells and percentage of eosinophils in BALF were calculated. Pathological staining was performed to observe histopathological changes in lung tissue. RT-PCR and Western blot were used to measure the mRNA and protein expression of E-cadherin and vimentin during epithelial-mesenchymal transition (EMT). Results Compared with the control group, the OVA group had marked infiltration of inflammatory cells in the airway, thickening of the airway wall, increased secretion of mucus, and increases in the total number of cells and percentage of eosinophils in BALF (P < 0.01). Compared with the OVA group, the JQ1+OVA group had significantly alleviated airway inflammatory response and significant reductions in the total number of cells and percentage of eosinophils in BALF (P < 0.01). Compared with the control group, the OVA group had significant reductions in the mRNA and protein expression of E-cadherin and significant increases in the mRNA and protein expression of vimentin (P < 0.01); compared with the OVA group, the JQ1+OVA group had significant increases in the mRNA and protein expression of E-cadherin and significant reductions in the mRNA and protein expression of vimentin (P < 0.01); there were no significant differences in these indices between the JQ1+OVA group and the control group (P > 0.05). Conclusions Mice with OVA-induced asthma have airway remodeling during EMT. BET bromodomain inhibitor JQ1 can reduce airway inflammation, inhibit EMT, and alleviate airway remodeling, which provides a new direction for the treatment of asthma.

Mesenchymal stem cell (MSC) transplantation is considered one of the most promising therapeutic strategies for the repair of brain injuries and plays an important role in various links of nerve repair. Recent studies have shown that MSC-derived exosomes may dominate the repair of brain injuries and help to promote angiogenesis, regulate immunity, inhibit apoptosis, and repair the nerves, and therefore, they have a great potential in the treatment of brain injuries in neonates. With reference to these studies, this article reviews the mechanism of action of exosomes in the repair of brain injuries and related prospects and challenges, in order to provide new directions for the treatment of brain injuries in neonates with stem cells.

Infant hemangioma, the most common benign tumor in children, is characterized by rapid proliferation, followed by slower spontaneous involution. However, some patients with facial segmental hemangioma are associated with PHACE syndrome. PHACE syndrome is characterized by vascular nerve and vascular cutaneous lesions of multiple systemic systems, often resulting in structural and functional impairments. Recent studies have demonstrated that the possible pathogeneses of PHACE syndrome mainly include hypoxia, abnormality of mesodermal vascular endothelial cells, genetic abnormality, and abnormality of interstitial mesenchymal stem cells. The current medications for hemangioma with PHACE syndrome include beta blockers, glucocorticoids, and mTOR inhibitors. This review article mainly describes the pathogenesis, diagnoses and treatments of PHACE syndrome, in order to provide directions for diagnosis and treatment of this disorder.

Congenital heart disease (CHD) is the most common birth defect at present and has a complex etiology which involves the combined effect of genetic and environmental factors. Pregestational diabetes mellitus is significantly associated with the development of CHD, but the detailed mechanism remains unknown. This article reviews the research advances in the molecular mechanism of CHD caused by pregestational diabetes mellitus.

Preterm birth is a major factor which induces neurological and motor impairments, particularly cerebral palsy, in high-risk infants. Early identification of potential neurodevelopmental impairments provides the opportunity to improve neurodevelopmental outcomes in preterm infants through early rehabilitation interventions. Clinically, the general movement assessment is a pivotal tool to predict neurodevelopmental outcomes, especially motor developmental outcomes, in high-risk infants. Movement recognition can continuously capture relevant limb movements and perform objective and quantitative assessment using computerized approaches. Various methods of recording and analyzing spontaneous general movements for infants at a risk of cerebral palsy have been extensively explored. This article summarizes the general movement assessment method and reviews the translational research on using movement recognition technology for the assessment of spontaneous general movements of preterm infants.