Objective To investigate the effect of heparin-binding epidermal growth factor-like growth factor (HB-EGF) on mitochondrial pathway of apoptosis in rats with neonatal necrotizing enterocolitis (NEC).Methods Sprague-Dawley neonatal rats were randomly divided into three groups with ten in each.NEC group rats were formula fed,and hypoxia exposed by 100％ N2 for 90 s and cold stress at 4 ℃ for 10 min twice a day for three days.Additionally,rats in HB-EGF group received HB-EGF 800μg/kg by gavage four times a day for three days.Rats in control group were given breast milk feeding for three days without any interventions.Seventy-two hours after born,all neonatal rats were sacrificed after fasting for 12 h,from which the terminal ileum was removed.HE-staining was done for histologic evaluation.Mitochondrial ultrastructure was observed under electron microscopy.Cytochrome C was detected by immunohistochemical analysis and apoptosis inducing factor (AIF) and apoptotic protease activating factor-1 (APAF-1) were measured by Western blot.Analysis of variance and q-test were used to compare the difference among groups.Results (1) The incidence of NEC in HB-EGF group was lower than that in NEC group (2/10 vs 9/10,x2 =7.27,P＜0.01).(2) In NEC group,mitochondria in epithelial cells and muscle cells of intestine were significantly swelling,appearing many electron-lucent zones in matrix.Ultrastructure of mitochondria were severely damaged.In HB-EGF group,mitochondria were less swelling and showed milder damage than those in NEC group.(3) The expression of cytochrome C in ileal tissue in NEC group was higher than that in control group (0.030±0.018 vs 0.002±0.001,q=6.15,P＜0.01).The expression of cytoehrome C in ileal tissue in HB-EGF group was lower than that in NEC group (0.014±0.018 vs 0.030±0.018,q=3.53,P＜0.05).The expression of APAF-1 and AIF in NEC group was higher than those in control group (1.364±0.299 vs 0.215±0.033,q=15.31,P＜0.05;0.181±0.050 vs0.127±0.045,q=3.71,P＜0.05).Compared to NEC group,the expression of APAF-1 was lower (0.455±0.123 vs 1.364±0.299,q=4.04,P＜0.05) and the expression of AIF was higher (0.289±0.045 vs 0.181±0.050,q=7.32,P＜0.05) in HB-EGF group.Conclusions HB-EGF could reduce the incidence of NEC in neonatal rats by inhibiting the mitochondrial pathway related apoptosis through down regulation of APAF-1.

Objective:To summarize the clinical characteristics of neonates with Pierre-Robin sequence (PRS) who received mandibular distraction osteogenesis (MDO), in order to provide a basis for perioperative evaluation and management.Methods:Data of neonates diagnosed with PRS and receiving MDO in the Department of Neonatology, Guangzhou Women and Children′s Medical Center between December 2016 and December 2019 were collected for a retrospective study.According to the types of respiratory support needed before surgery, they can be categorized into 3 groups: the invasive ventilation group, the noninvasive ventilation group, the oxygen therapy/positioning therapy group.According to the usage of sedative and analgesic drugs after surgery, they were categorized into 2 groups: the sedative-analgesic group(Fentanyl Citrate and Midazolam) and the single sedative group(Midazolam alone).Results:A total of 31 children were admitted, 22 cases of whom received MDO at an age of (22.09±8.42) d, including 11 cases in the invasive ventilation group, 5 cases in the non-invasive ventilation group, and 6 cases in the therapy/positioning therapy group before surgery; there was statistical significance in severe respiratory distress (81.8% vs.60.0% vs.0) and feeding difficulties (100.0% vs.80.0% vs.50.0%) among the 3 groups (all P<0.05), while there was no difference in laryngomalacia rate(63.6% vs.20.0% vs.33.3%), non-gain in weight before surgery(81.8% vs.80.0% vs.66.7%), postoperative ventilation duration[(7.5±3.4) d vs.(7.2±5.3) d vs.(6.9±4.1) d]and hospital stay[(36.3±9.3) d vs.(34.1±0.3) d vs.(33.8±7.5) d] (all P>0.05). Among these 22 patients, there were 9 cases in the sedative-analgesic group, and 13 cases in the single sedative group.Compared with the single sedative group, cases in the sedative-analgesic group had significantly lower extubation rate (0 vs.15.4%) and shorter hospital stay[(32.3±5.2) d vs.(39.8±7.3) d], and the difference were statistically significant (all P<0.05). Besides, there were no differences in postoperative ventilation duration[(7.4 ± 3.9) d vs.(7.6 ± 4.2) d]between both groups ( P>0.05). The postoperative remission rate of airway obstruction was 100%. Conclusions:MDO in children in their neonatal period could achieve favorable efficacy.The duration of postoperative ventilator support and hospital stay in children with severe airway obstruction could not be prolonged compared with children with mild obstruction, and postoperative sedation and analgesic management can reduce the extubation rate.

This article reported a case of primary coenzyme Q10 deficiency caused by a variation in the COQ4 gene. On the first day after birth, the neonate exhibited unexplained feeding difficulties, intermittent cyanosis, and respiratory and circulatory failure. Similar symptoms were observed in his sister, who passed away on the 9th day after birth but no pathogenic variant was detected in whole exome sequencing. After a pathogenic homozygous variant of COQ4 gene c.370G>A was detected in this patient using whole exome sequencing, his sister's result of whole exome sequencing was got and the same variant was found (identified as uncertain significance at that time), and both parents carried a heterozygous variant of c.370G>A. Supplement with clinical manifestations, the infant was diagnosed with coenzyme Q10 deficiency. The infant received respiratory and circulatory support, and after oral supplement of coenzyme Q10, the symptoms were improved. Subsequent follow-up examinations showed that the child had developed epilepsy and psychomotor retardation at about the age of one.