Inflammatory bowel disease (IBD) is a chronic nonspecific intestinal inflammatory disease of unknown etiology. This disease includes three main types:Crohn's disease (CD), ulcerative colitis (UC), and IBD-unclassified (IBD-U). IBD is frequently presented in adults, but in recent years, there is a rising incidence in pediatric populations. Very early onset IBD (VEO-IBD) is a fraction of pediatric IBD, but they have exclusive phenotypic and genetic characteristics such that they are accompanied by severe disease course and resistance to conventional therapy. The purpose of this review is to provide a contemporary overview of the clinical features, pathogenesis, and management of VEO-IBD.

Objective To evaluate the effect of early application of recombinant human erythropoietin (rhEPO) on white matter development in preterm infants using fractional anisotropy (FA) of magnetic resonance diffusion tensor imaging (DTI). Methods A total of 81 preterm infants with gestational age ≤ 32 weeks, birth weight < 1 500 g, and hospitalization within 24 hours after birth were randomly divided into rhEPO group (42 infants) and control group (39 infants). The infants in the rhEPO group were administered rhEPO, while those in the control group were given the same volume of normal saline. The preterm infants of both groups took examinations of head magnetic resonance imaging, diffusion-weighted imaging, and DTI at the corrected gestational age of 35-37 weeks. FA was calculated for the regions of interest in both groups. Results There was no significant difference in the incidence of intracranial hemorrhage, periventricular leukomalacia, focal cerebral white matter damage (CWMD), and extensive CWMD between rhEPO and control groups (P > 0.05). Compared with the control group, the rhEPO group showed higher FA values at the posterior limb of the internal capsule, the splenium of the corpus callosum, frontal white matter, and occipital white matter (P < 0.05). There was no significant difference in FA values at the parietal white matter, thalamus, lenticular nucleus, and caudate nucleus between the two groups (P > 0.05). Conclusions Early application of rhEPO has a neuroprotective effect on white matter development in preterm infants.

Objective To study the combined effect of gestational age and birth weight on metabolites related to inherited metabolic diseases (IMD). Methods A total of 3 381 samples ruled out of IMD by follow-up were randomly selected from 38931 newborns who participated in the neonatal IMD screening during 2014-2016. The 3 381 neonates were categorized into seven groups according to their gestational age and birth weight:extremely preterm appropriatefor-gestational age (AGA) group (n=12), preterm small-for-gestational age (SGA) group (n=18), preterm AGA group (n=219), preterm large-for-gestational age (LGA) group (n=18), full-term SGA group (n=206), full-term AGA group (n=2677), and full-term LGA group (n=231). Heel blood samples were collected from each group on postnatal days 3-7 after adequate breastfeeding. Levels of 17 key IMD-related metabolic indices in dried blood spots were measured using tandem mass spectrometry. Spearman's correlation analysis was used to investigate the relationships between 17 IMDrelated metabolic indices and their influencing factors, while covariance analysis was used to compare the metabolic indices between these groups. Results After adjusting the influencing factors such as physiological and pathological status, compared with the full-term AGA group, the extremely preterm AGA, preterm SGA, and preterm AGA groups had significantly reduced levels of leucine\isoleucine\hydroxyproline and valine (P < 0.05); the preterm AGA group had a significantly decreased ornithine level (P < 0.05); the extremely preterm AGA and preterm AGA groups had a significantly reduced proline level (P < 0.05). Besides, the phenylalanine level in the extremely preterm AGA and preterm AGA groups, the methionine level in the preterm SGA group, and the tyrosine level in the preterm AGA group all significantly increased (P < 0.05). The increased levels of free carnitine, acetylcarnitine, and propionylcarnitine were found in the preterm SGA and preterm AGA groups. The oleylcarnitine level also significantly increased in the preterm SGA group (P < 0.05). Most carnitine indices showed significant differences between the SGA group and the AGA/LGA group in both preterm and full-term infants (P < 0.05). Conclusions Low gestational age and low birth weight may result in abnormal results in IMD screening. Therefore, gestational age and birth weight should be considered to comprehensively judge the abnormal results in IMD screening.

Objective To study the Kaup index (KI), an index used to evaluate body burliness and nutritional status, of neonates with a gestational age (GA) of 27-42 weeks at birth, and to establish the percentile curves of KI. Methods Cross-sectional cluster sampling was performed from April 2013 to September 2015 to select 16 887 singleton neonates with a GA of 27-42 weeks in two hospitals in Shenzhen, China. Body weight and body length were measured to calculate KI. The percentile curves of KI were plotted in these neonates. Results Mean KIs and corresponding standard deviations were obtained for singleton neonates with a gestational age of 27-42 weeks (in male, female, and mixed groups), and the 3rd-97th percentile curves of KI were plotted. The singleton neonates with a GA of 27 weeks had the lowest 50th percentile value of KI, and KI gradually increased with GA. Boys had a higher 50th percentile value of KI than girls in each GA group. In all groups except the 33-week GA group, boys had a higher mean KI than girls, and there was a significant difference in the mean KI between boys and girls in the GA groups of 34 and 36-40 weeks (P < 0.05). Conclusions KI of neonates at birth increases with GA, suggesting that body density and body burliness increase with GA. Boys have better body burliness than girls at birth. The percentile curves of KI plotted for singleton neonates with a GA of 27-42 weeks (in male, female, and mixed groups) can provide a reference for evaluating the body burliness and nutritional status of neonates at birth in Shenzhen.

Objective To study the efficiency of electrocardiogram (ECG) monitor for positioning the catheter tip in the placement of peripherally inserted central catheter (PICC) in neonates. Methods A total of 160 neonates who were admitted to the neonatal intensive care unit (NICU) from January 2015 to December 2017 and underwent the PICC placement via the veins of upper extremity were enrolled. They were randomly divided into an observation group and a control group, with 80 neonates in each group. The neonates in the control group were given body surface measurement and postoperative X-ray localization, while those in the observation group were given body surface measurement, ECG localization, and postoperative X-ray localization. The two groups were compared in terms of general information, onetime success rate of PICC placement, and time spent on PICC placement. Results There were no significant differences between the two groups in sex composition, gestational age, age in days at the time of PICC placement, disease type, and site of puncture (P > 0.05). Compared with the control group, the observation group had a significantly higher one-time success rate of PICC placement (95% vs 79%; P < 0.05) and a significantly shorter time spent on PICC placement (P < 0.05). Localization under an ECG monitor during PICC placement had a sensitivity of 97% and a specificity of 100%. Conclusions During the PICC placement in neonates, the use of ECG monitor to determine the position of catheter tip can improve the one-time success rate of placement and reduce the time spent on placement.

Objective To investigate the incidence and mortality rates of preterm infants and the main causes of death. Methods The basic information of preterm infants was collected from their medical records and admission/discharge records to analyze the incidence and mortality rates of preterm infants and the causes of their death. Results There were 76 812 neonates born in the Xuzhou Maternal and Child Health Hospital from January 2006 to December 2016, among whom 5585 (7.27%) were preterm infants. The incidence rate of preterm infants tended to increase over these years (P < 0.001). The overall mortality rate was 5.01% (280/5585), and the mortality rate tended to decrease over these years (P < 0.001). The mortality rate increased with the reductions in birth weight and gestational age (P < 0.001). The top four causes of death in preterm infants were respiratory distress syndrome (44.3%), severe asphyxia (12.9%), neonatal malformation (4.3%), and pulmonary hemorrhage (2.9%) respectively. With the increase in birth weight, there were significant reductions in the constituent ratios of death due to respiratory distress syndrome and severe asphyxia (P < 0.001). Conclusions The incidence rate of preterm infants tended to increase and their mortality rate tended to decrease from 2006 to 2016. The mortality rate of preterm infants is associated with gestational age and birth weight. Respiratory distress syndrome and severe asphyxia are the main causes of death in preterm infants.

Objective To study the clinical and laboratory characteristics of juvenile myelomonocytic leukemia (JMML). Methods The clinical characteristics and laboratory results were retrospectively analyzed in 10 children with newly diagnosed JMML. They were compared with those of 28 children with myelodysplastic syndrome (MDS) and 44 children with chronic myeloid leukemia (CML). Results Compared with the children with CML or MDS, the children with JMML had significantly higher rates of skin rashes, ecchymosis, and lymphadenectasis, a significantly lower serum cholinesterase (ChE) level, and a significantly higher fetal hemoglobin level (P < 0.05). The white blood cell count of children with JMML was significantly higher than that of children with MDS, but significantly lower than that of children with CML (P < 0.05). In addition, the myeloid/erythroid ratio and rate of dyshaematopoiesis were significantly lower in children with JMML than those in children with CML or MDS. The children with JMML had a significantly higher expression of mature monocyte marker CD14 than those with CML or MDS (P < 0.05). The levels of myeloid markers CD33, CD11b, CD13, and CD15 in children with JMML were significantly higher than those in children with MDS, but significantly lower than those in children with CML (P < 0.05). The levels of CD2 and CD7 in children with JMML were higher than those in children with CML, but lower than those in children with MDS (P < 0.05). Conclusions Skin rashes, ecchymosis, lymphadenectasis, and ChE reduction are more common in children with JMML than in those with CML or MDS, while dyshaematopoiesis is less common. In addition, CD14 level increases significantly in children with JMML.

Objective To study the clinical effect and mechanism of hemoperfusion (HP) in the treatment of children with severe abdominal Henoch-Schönlein purpura (HSP). Methods A total of 24 children with severe abdominal HSP were divided into two groups:conventional treatment and HP (n=12 each). Ten healthy children who underwent physical examination were enrolled as the control group. Before and after treatment, chemiluminescence was used to measure the serum levels of interleukin-6 (IL-6) and tumor necrosis factor-α (TNF-α); thiobarbituric acid colorimetry was used to measure the plasma level of malondialdehyde (MDA); the hydroxylamine method was used to measure the plasma level of superoxide dismutase (SOD); chemical colorimetry was used to measure the plasma level of total anti-oxidant capability (T-AOC). Results Compared with the control group, the conventional treatment and HP groups had significantly higher IL-6, TNF-α, and MDA levels and significantly lower SOD and T-AOC levels before treatment (P < 0.05), but there were no significant differences between the conventional treatment and HP groups (P > 0.05). After treatment, the conventional treatment and HP groups had significant reductions in IL-6, TNF-α, and MDA levels and significant increases in SOD and T-AOC levels (P < 0.05). The HP group had significantly greater changes than the conventional treatment group; however, there were still significant differences in these indices between the HP and control groups (P < 0.05). Compared with the HP group, the conventional treatment group had a significantly lower percentage of children with disappearance of digestive tract symptoms at 4 days after treatment and significantly longer time to disappearance of rash and digestive tract symptoms (P < 0.05). Compared with the conventional treatment group, the HP group had a significantly lower amount of glucocorticoid used during treatment and a significantly lower percentage of children who experienced hematuria and/or proteinuria within 6 months of the disease course (P < 0.05). There were no significant differences between the two groups in length of hospital stay and recurrence rates of rash and abdominal pain within 6 months of the disease course. Conclusions HP can reduce the amount of glucocorticoid used during treatment and the incidence rate of kidney injury in children with severe abdominal HSP, possibly by eliminating IL-6, TNF-α, and MDA.

Objective To study the association between overweight/obesity in parents before maternal pregnancy and the development of autism spectrum disorders (ASD) in offspring. Methods A total of 36 children who were diagnosed with ASD (ASD group) and 72 normal children matched for sex and age (control group) were enrolled. A questionnaire survey was performed to collect the general information, including body height and body weight of parents before maternal pregnancy and maternal weight gain during pregnancy. Univariate and multivariate logistic regression analyses were used to investigate the association between overweight/obesity in parents before maternal pregnancy and ASD in offspring. Results The ASD group had a significantly higher detection rate of overweight/obesity in the father than the control group (56% vs 32%; P=0.018) before maternal pregnancy. The univariate and multivariate logistic regression analyses showed that overweight/obesity of the father before maternal pregnancy was a risk factor for ASD in offspring (OR=2.66 and 2.58 respectively; P < 0.05). Conclusions Overweight/obesity of the father before maternal pregnancy is an independent risk factor for ASD in offspring, and therefore, it is important for the father to control his body mass index within the normal range before maternal pregnancy.

Both children (one boy and one girl) experienced disease onset in infancy and visited the hospital due to growth retardation. They had unusual facies including thick hair, arched and confluent eyebrows, long and curly eyelashes, short nose, and micrognathia. Patient 1 had congenital heart disease (atrial septal defect and pulmonary stenosis) and special dermatoglyph (a single palmar crease). Patient 2 had cleft palate and moderate-to-severe deafness. Clinical features suggested Cornelia de Lange syndrome in both children. High-throughput sequencing was used to detect the seven known pathogenic genes of Cornelia de Lange syndrome, i.e., the NIPBL, SMC1A, SMC3, HDAC8, RAD21, EP300, and ANKRD11 genes. Sanger sequencing was used to analyze and verify gene mutations. Both patients were found to have novel mutations in the NIPBL gene. One patient had a frameshift mutation in exon 45, c.7834dupA, which caused early termination of translation and produced truncated protein p.R2612fsX20. The other patient had a nonsense mutation, c.505C > T, which caused a premature stop codon and produced truncated protein Q169X. Such mutations were not found in their parents or 50 unrelated healthy individuals.

Objective To investigate the current status of exclusive breastfeeding for the second child in the context of the universal two-child policy and the factors influencing exclusive breastfeeding. Methods A self-designed questionnaire for the current status of breastfeeding and related factors influencing breastfeeding for the second child were used to survey 836 mothers with a second child, who were selected by cluster sampling, in Quzhou, Zhejiang, China. Results A total of 680 usable questionnaires were obtained. The rate of exclusive breastfeeding for the second child was significantly lower than for the first child (34.9% vs 42.2%; P < 0.05). The univariate analysis revealed that there were significant differences between the exclusive and non-exclusive breastfeeding groups in the mother's age, education background, occupation and time of maternity leave, mode of delivery of the first child, sex of the first child, feeding pattern of the first child, mode of delivery of the second child, whether the second child was admitted to the intensive care unit, whether the father supported breastfeeding, and whether the grandmother/maternal grandmother supported breastfeeding (P < 0.05). The multivariate logistic regression analysis showed that artificial feeding+partial breastfeeding for the first child (OR=12.286, P < 0.05), cesarean section for the second child (OR=1.724, P < 0.05), and having no breastfeeding support from the maternal grandmother (OR=1.651, P < 0.05) were main factors for influencing exclusive breastfeeding. Conclusions The current status of exclusive breastfeeding for the second child is not optimistic in the context of the universal two-child policy. Education about breastfeeding should be taken seriously at the birth of the first child, the rate of cesarean section should be reduced, and the family members should support exclusive breastfeeding, in order to improve the status of exclusive breastfeeding.

Objective To study the effect of astrocyte exosomes on hypoxic-ischemic neurons. Methods Rat astrocytes were cultured in vitro, and differential centrifugation was used to obtain the exosomes from the cell supernatant. Transmission electron microscopy, Nanosight, and Western blot were used for the identification of exosomes. BCA method was used to measure the concentration of exosomes. Rat neurons were cultured in vitro and then divided into control group, exosome group, oxygen glucose deprivation (OGD) group, and OGD+exosome group (n=3 each). The OGD and OGD+exosome groups were cultured in glucose-free medium under the hypoxic condition. The exosome and OGD+exosome groups were treated with exosomes at a final concentration of 22 μg/mL. The control and OGD groups were given an equal volume of phosphate-buffered saline. ELISA was used to measure the level of lactate dehydrogenase (LDH) in neurons. The terminal deoxynucleotidyl transferase-mediated dUTP nick-end labeling was used to measure the apoptotic index of neurons. Results The identification of exosomes showed that the exosomes extracted by differential centrifugation had the features of exosomes. Compared with the control and exosome groups, the OGD group had significant increases in LDH level and apoptotic index (P < 0.05). Compared with the OGD group, the OGD+exosome group had significant reductions in LDH level and apoptotic index (P < 0.05). Conclusions The exosomes from astrocytes have a protective effect on neurons with hypoxic-ischemic injury.

Objective To study the expression of SUMO-modified CCAAT enhancer binding protein α (C/EBPα) in preterm rat model of bronchopulmonary dysplasisa (BPD) induced by hyperoxia exposure and its role. Methods Eighteen preterm rats were randomly divided into an air group and a hyperoxia group (n=9 each). The model of BPD was prepared in preterm rats exposed to hyperoxia. The rats from the two groups were sacrificed on postnatal days 4, 7 and 14 respectively (3 rats at each time) and lung tissues were harvested. Periodic acid-Schiff (PAS) staining was used to observe the differentiation of rat lung tissues. Ki67 expression was detected by immunohistochemistry. Western blot was used to measure the protein expression of small ubiquitin-related modifier-1(SUMO1) and C/EBPα. A co-immunoprecipitation assay was performed to measure the protein expression of SUMO-modified C/EBPα. Results Compared with the air group, the hyperoxia group showed a decreased glycogen content in the lung tissue on postnatal day 4, and an increased content on postnatal days 7 and 14. Over the time of hyperoxia exposure, the hyperoxia group showed an increased expression of Ki67 in the lung tissue compared with the air group at all time points. Compared with the air group, the protein expression of C/EBPα increased on postnatal day 4 and decreased on postnatal days 7 and 14 in the hyperoxia group (P < 0.05). The hyperoxia group had significantly upregulated expression of SUMO1 and SUMO-modified C/EBPα compared with the air group at all time points (P < 0.05). In the hyperoxia group, the protein expression of SUMO-modified C/EBPα was positively correlated with the glycogen content (r=0.529, P < 0.05) and the expression of Ki67 (r=0.671, P < 0.05). Conclusions Hyperoxia may induce over-proliferation and differentiation disorders of alveolar epithelial cells in preterm rat model of BPD, possibly through an increased expression of SUMO-modified C/EBPα.

Objective To study the effect of rhubarb on neonatal rats with bronchopulmonary dysplasia (BPD) induced by hyperoxia. Methods A total of 64 rats (postnatal day 4) were randomly divided into four groups:air control, rhubarb control, hyperoxia model, and hyperoxia+rhubarb (n=16 each). The rats in the hyperoxia model and hyperoxia+rhubarb groups were exposed to hyperoxia (60% O₂) to establish a BPD model. The rats in the rhubarb control and hyperoxia+rhubarb groups were given rhubarb extract suspension (600 mg/kg) by gavage daily. The pathological changes of lung tissue were evaluated by hematoxylin-eosin staining on postnatal days 14 and 21. The content of malondialdehyde (MDA) and the activity of superoxide dismutase (SOD) were measured by spectrophotometry. The mRNA and protein expression levels of tumor necrosis factor-alpha (TNF-α) and interleukin-6 (IL-6) were determined by RT-PCR and Western blot respectively. Results The hyperoxia model group showed reduced alveolar number, increased alveolar volume, and simplified alveolar structure, which worsened over the time of exposure to hyperoxia. These pathological changes were significantly reduced in the hyperoxia+rhubarb group. On postnatal days 14 and 21, compared with the air control and rhubarb control groups, the hyperoxia model group had significantly reduced radical alveolar count (RAC), significantly reduced activity of SOD in the lung tissue, and significantly increased content of MDA and mRNA and protein expression levels of TNF-α and IL-6 (P < 0.05). Compared with the hyperoxia model group, the hyperoxia+rhubarb group had significantly increased RAC, significantly increased activity of SOD in the lung tissue, and significantly reduced content of MDA and mRNA and protein expression levels of TNF-α and IL-6 (P < 0.05). Conclusions Rhubarb may play a protective role in rats with BPD induced by hyperoxia through inhibiting inflammatory response and oxidative stress.

In recent years, great progress has been made in the treatment outcome of childhood acute leukemia with the improvement of chemotherapy regimens and the introduction of risk-stratified therapy; however, minimal residual disease (MRD) is still a difficult problem which affects the prognosis of acute leukemia. MRD influences the selection of chemotherapy regimens and recurrence risk stratification, and meanwhile, it can be used for prognostic prediction. At present, flow cytometry and polymerase chain reaction are mainly used for MRD detection. The next-generation sequencing also plays an important role in MRD detection, especially in MRD detection after stem cell transplantation. This article reviews the methodology and significance of MRD detection in childhood acute leukemia.

Aptamers are single-stranded DNA or RNA which are isolated from synthesized random oligonucleotide library in vitro via systematic evolution of ligands by exponential enrichment (SELEX) and can bind to metal ions, small molecules, carbohydrates, lipids, proteins, and others targets with high affinity and specificity. Aptamers have the advantages of simple preparation, good thermal stability, and low immunogenicity and have great potential in the medical fields such as molecular imaging, biosensing, early diagnosis of diseases, and targeted therapy. Aptamer technology may be useful for early diagnosis and targeted therapy of pediatric cancer, and may avoid the side effects of conventional chemotherapy, such as growth and development disorders and long-term organ dysfunction. This article reviews the latest research advances in the selection and application of aptamers for pediatric cancer.

Hirschsprung's disease (HSCR) is one of the major causes of chronic incomplete intestinal obstruction in children. HSCR is considered a type of neurocristopathy caused by no colonization of ganglion cells on some parts of the bowel wall due to abnormal termination of the migration of vagal neural cells during embryonic development. This disease can be classified into different types according to the length of the affected intestinal canal. Most HSCR patients present with single deformity, but some HSCR patients are affected by other deformities, which constitutes syndromic HSCR, such as congenital central hypoventilation syndrome, Fryns syndrome, and cartilage-hair hypoplasia syndrome. Most syndromes have abnormal genetic material. An adequate knowledge of syndromic HSCR is of vital importance for accurate diagnosis and prognostic evaluation. This article reviews the clinical manifestations, genetic basis, and genetic modes of different types of syndromic HSCR.