Dynamic changes of blood free carnitine and acylcarnitine levels in premature infants receiving nutritional support / 中国新生儿科杂志

Objective To study the dynamic changes of blood carnitine and acylcarnitine levels in preterm infants during parenteral and enteral nutritional support,and the relationship between carnitine status and nutritional patterns,gestational age (GA) and weight gain.Method From January 2017 to December 2017,preterm infants admitted to the neonatal intensive care unit (NICU) within 24 hours after birth and received parenteral nutrition support were enrolled.They were assigned into 4 groups according to their GA:ultra-premature infants (＜ 28 weeks),very premature infants (28 ～ 31 weeks),mid premature infants (32 ～ 33 weeks) and late premature infants (34 ～ 36 weeks).They were assigned into 2 groups according to their average daily weight gain:＜ 15 g/(kg · d) group and ≥15 g/(kg · d) group.Blood samples were collected and examined as dried-blood spot specimens on filter paper for four times:after born,given total parenteral nutrition,given enteral combined parenteral nutrition,and given total parenteral nutrition.The concentrations of free carnitine and acylcarnitine were detected using liquid chromatographytandem mass spectrometry (LC-MS/MS).SPSS 21.0 statistical software was used for statistical analysis.Result A total of 124 preterm infants and 410 samples were collected.As the infants experienced gradual transition from parenteral nutrition to enteral nutrition,the free carnitine and most acylcarnitines levels were decreasing (C3,C4,C10DC,C12,C12∶1,C12DC,C14,C16,C16∶ 1,C16-OH and C18,P＜0.05).Preterm infants with small GA showed higher levels of C4-OH (P =0.001) and C5 (P =0.001).Preterm infants with lower velocity of weight gain showed lower concentration of C5-OH (P =0.006) in the early postnatal period.Conclusion Free carnitine and acylcarnitine in preterm infants during the early postnatal period are decreasing with the transition from parenteral nutrition to enteral nutrition,indicating that the exogenous nutrition is relatively insufficient.C4-OH and C5 levels are negatively correlated with GA.In addition,lower level of C5-OH may indicate slow weight gain during the early postnatal period.

Application value of acoustic radiation force impulse elastography in diagnosis of biliary atresia / 中华实用儿科临床杂志

Objective:To assess the value of acoustic radiation force impulse (ARFI) elastography in the diagnosis of children with biliary atresia.Methods:A prospective survey of infants with hepatitis syndrome and hyperbi-lirubinemia in Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine from January 2017 to December 2018 was performed.The children with hepatitis syndrome were divided into the biliary atresia group( n=45) and non- biliary atresia group( n=30). Thirty children with hyperbilirubinemia were selected as the control group.Shear wave speed (SWS) of all infants was collected by ARFI ultrasound and compared among 3 groups.Receiver ope-rating characteristic curve(ROC curve) was used to analyze the optimal threshold value for SWS in the diagnosis of biliary atresia. Results:The mean SWS values in the biliary atresia group, non-biliary atresia group and the control group were (1.79±0.29) m/s, (1.26±0.12) m/s and (1.08±0.06) m/s, respectively.Compared with the control group, the mean SWS values in the biliary atresia group and non-biliary atresia group were significantly higher ( t=165.43, 15.75, all P<0.05). The mean SWS value in the non-biliary atresia group was significantly lower than that in the biliary atresia group ( t=90.27, P<0.05). With the non-biliary atresia group as reference, the area under the ROC curve of SWS for diagnosis of biliary atresia was 0.98(95% CI: 0.95-1.00), the optimal threshold was 1.45 m/s, and the sensitivity and specificity were 88.9% and 96.7%, respectively. Conclusions:Rapid non-invasive ARFI elastography is effective in the diagnosis of biliary atresia, and thus has important value for early diagnosis and treatment in clinical practice.

Risk factors of postoperative acute pulmonary reperfusion injury in neonatal severe pulmonary stenosis or pulmonary atresia / 中国新生儿科杂志

Objective To study the clinical features and risk factors of acute pulmonary reperfusion injury after operation in neonates with severe pulmonary stenosis or pulmonary atresia.Method From February 2014 to February 2018,a retrospective analysis was performed in patients with critical pulmonary stenosis or pulmonary atresia who received percutaneous balloon pulmonary valvuloplasty (PBPV) in the neonatal intensive care unit of our hospital.Clinical characteristics,perioperative cardiac structure,hemodynamic data and biochemical results were collected.The neonates were assigned into injury group if they had acute lung reperfusion injury,and non-injury group if not.The risk factors of acute lung reperfusion injury were analyzed using multi-variate Logistic regression model.Result A total of 32 patients (24 prenatal diagnosis and 8 postnatal diagnosis) with severe pulmonary stenosis or pulmonary atresia with intact ventricular septum were enrolled.The main manifestations were dyspnea and cyanosis.Intravenous prostaglandin E was administered to keep the ductus arteriosus open.The age of operation ranged from 1 to 52 days and the median age was 7.5 days.Postoperative acute lung reperfusion injury occurred in 7 cases (21.9％).Preoperative and intraoperative pulmonary valve annulus diameter,balloon diameter,preoperative hemoglobin,hematocrit and blood albumin were significantly lower in the injury group.The operation duration,total length of hospital stay and postoperative duration were longer than in the non-injury group,the differences were statistically significant (P ＜ 0.05).Multi-variate Logistic regression analysis showed that the diameter of pulmonary valve annulus (OR =5.814,95％CI 1.106 ～30.568),preoperative blood albumin (OR =1.361,95％ CI 1.063 ～ 1.742),and hematocrit (OR =1.173,95％ CI 1.010 ～ 1.363) were risk factors of acute lung reperfusion injury,with statistically significant differences (P ＜ 0.05).Conclusion Acute lung reperfusion injury is one of the common complications after the operation of severe pulmonary stenosis or pulmonary atresia.The severity of pulmonary valve annulus stenosis,preoperative hematocrit and blood albumin level may be the risk factors of postoperative acute lung reperfusion injury.

Multidisciplinary perinatal management of a woman and her baby with ornithine transcarbamylase deficiency / 中华围产医学杂志

Objective To investigate the clinical features and gene mutation of a newborn with neonatal-onset ornithine transcarbamylase deficiency (OTCD) and report the multidisciplinary perinatal management of the mother with late-onset OTCD.Methods The clinical features,biochemical data and the treatment of a newborn boy with OTCD and his mother admitted by Xin Hua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine in April,2013,were collected.The ornithine transcarbamylase (OTC) gene in the family was analyzed.Results Serum ammonium in the male newborn gradually increased to 1 020 μ mol/L at 48 h after birth.His blood amino acids level and urine organic acid level showed a pattern indicative of OTCD [blood arginine (97.43 μ mol/L,reference 1.00-25.00 μ mol/L),citrulline (27.43 μ mol/L,reference 4.00-30.00 μ mol/L),ornithine (161.66 μ mol/L,reference 10.00-120.00 μ mol/L) and methionine (70.45 μ mol/L,reference 10.00-50.00 μ mol/L); urine uracil (67.11 μ mol/mol Crea,reference 0.00-7.00 μ mol/mol Crea) and orotic acid (1 372.66 μ mol/mol Crea,reference 0.00-1.50 μ mol/mol Crea)].DNA studies revealed a c.583G ＞ A (G195R) homozygous mutation of the OTC gene.His mother was heterozygous for OTCD and developed acute hyperammonemia during pregnancy.Her blood showed a normal-leveled arginine (8.44 μ mol/L,reference 1.50-25.00 μ mol/L),a normal-leveled citrulline(8.41 μ mol/L,reference 7.00-35.00 μ mol/L),an elevated glutamate(279.15 μ mol/L,reference 45.00-200.00 μ mol/L).Her urine uracil (51.55 μ mol/mol Crea,reference 0.00-7.00 μ mol/mol Crea) and orotic acid (38.75 μ mol/mol Crea,reference 0.00-1.50 μ mol/mol) were elevated.Successful management of her prenatal and postpartum blood ammonia level was achieved after administration of pharmacologic nitrogen scavengers and protein limitation.DNA studies revealed a c.583G ＞ A (G195R) heterozygous mutation in the newborn's mother and grandmother.Conclusions General management on pregnant OTCD women is effective.Male newborn patients often have a poor prognosis.

Research progress of FLIP in cancer / 国际肿瘤学杂志

FLIP is a protein containing death domain.Human beings have three subtypes of c-FLIPL,c-FLIPs and c-FLIPR,which can inhibit the apoptosis of a variety of tumor cells.c-FLIPL plays a dual role in the apoptotic signal.The expression level of FLIP not only decides the opening and closing of the apoptosis pathway but also achieve the conversion of cells in the apeptotic signaling and proliferative signaling pathway.The regulation of FLIP's expression is a multi-layered,involving multiple signaling pathways.FLIP will probably become an attractive death receptor signaling target of therapy.

Meta analysis of continuous versus intermittent feeding in very low birth weight infants / 临床儿科杂志

Objective To compare the effects of continuous versus intermittent feedings on the growth of very low birth weight infants. Methods Databases of Pubmed,Embase,Ovid,the Cochrane library and CBM were searched through internet,and citations of relevant original studies were also searched manually by using keywords of 'ontinuous,intermittent,nasogastric,premature,very low birth weight' Meta analysis was done on the results of these studies. Results Seven eligible studies with 434 infants were identified,among them,217 were fed using continuous nasogastric gavage,and other 217 were fed by intermittent nasogastric gavage. The result of the Meta analysis revealed that there was no statistical difference in the growth of the infants between two feeding methods. Conclusions Feeding methods are associated with similar outcomes when calorie intake is guaranteed.

Clinical analysis of apnea after operation for retinopathy of prematurity / 上海交通大学学报（医学版）

Objective To retrospectively explore the incidence and causes of apnea after operation for retinopathy of prematurity(ROP). Methods The clinical data of 17 premature infants with operation for ROP(ROP group)and the other 23 premature infants without ROP(control group)were collected.The occurrence of apnea(time of onset and remission and original diseases) was recorded after operation in ROP group and at adjusted 37 weeks of gestational age in control group.Follow-up was conducted for 4 weeks,and the prevalences of apnea were compared between these two groups.Results There was significant difference in prevalenees of apnea between ROP group and control group ( 52.9%vs 21.7%,P<0.05).In ROP group,time of occurrence of apnea was (38±40)h after operation,and time of remission was (126±145)h after onset.Among the infants with apnea,there were 3 cases of pneumonia(33.3%),1 case of hypoglycemia(11.1%)and 5 cases with unexplained causes(55.5%). Conclusion Infants after operation for ROP are more prone to apnea,and pediatricians and ophthalmologists are required to collaborate in the perioperative care.