Advances in resuscitation and intensive care have led to high rates of survival among neonates with life -threatening conditions such as asphyxia,prematurity and congenital malformations.The sequelae of neurologic con-ditions arises in the neonatal period include lifelong disabilities such as cerebral palsy,epilepsy,intellectual and beha-vioral disabilities.There is an increasing demand for resource -intense strategies for acute neurological care within neo-natal intensive care unit.Neonatal neurocritical care is a multidisciplinary subspecialty that combines expertise in neo-natology,pediatric neurology,radiology,rehabilitation,surgery,and has led to improved outcomes in newborn that have critical illnesses.Neonatal neurocritical care focus the needs of the developing newborn brain,including attention to physiology to help prevent secondary brain injury,a protocol -driven approach for common conditions such as hypoxic -ischemic encephalopathy and seizures,education of specialized teams that use brain monitoring and imaging to evaluate the effect of critical illness on brain function.

Objective To study the safety of hypothermia in neonates with hypoxic-ischemic brain damage after asphyxia. Methods Twenty four full term newborns with Apgar score 0.05). Conclusions The results suggest that the selective head cooling in full term newborn has no significant adverse effect on cardiac function, kidney function and coagulation function.

Refresher training is the most important ways of continuing medical education. This paper introduces the training situation of refresher doctors of Children's Hospital of Fudan University by distribution of professional title, education departments and experience in training. We have sum-marized the management mode of refresher doctors from such aspects as the entrance management, pre-job training, authorization to work, strengthening clinical practice to expand knowledge, clinical research training, self-learning ability improvement, strengthening emotional communication, humani-zation management, emotional bonds establishment and creation of a follow-up development environment.

Objective To evaluate the value of amplitude-integrated EEG(aEEG)as a quantitative predictor of neurodevelopment outcome of full-term infants with hypoxic-ischemic encephalopathy(HIE).Method EMBASE,Ovid,PubMed,Cochrane Library,Springer Database,CNKI,CSJD and Wanfang Database were searched from when the database was founded to June 30,2010 for studies about aEEG as a predictor of neurodevelopment outcome in full-term infants with HIE.Characteristic information of each articles was extracted and QUADAS items were used to evaluate the qualities of included studies.Meta-disc software 1.4 was used to perform the metaanalysis.All included studies were combined with weighted quantity; sensitivity,specificity,positive likelihood ratio,negative likelihood ratio and their 95 ％ CI were calculated.Results Eleven studies were eligible for the primary meta-analysis and totally 717 subjects were included.There was an overall sensitivity of 86％(95％ CI:81％-89％),specificity of 90％(95％ CI:86％-93％),positive likelihood ratio of 7.70(95％ CI:5.69-10.43),negative likelihood ratio of 0.17(95％ CI:0.13-0.22)and the area under the curve(AUC)of summary receiver operating characteristic curve was 0.95 for aEEG tracings to accurately predict poor outcome of HIE in full term neonates.Conclusion aEEG is a valuable bedside tool for predicting long-term neurodevelopment outcome in term infants with HIE.

Hongkong pediatric specialist training had successful experiences in the last twenty years.Hongkong hospital authority and Hongkong college of pediatricians managed pediatric specialist training together and made a series of regulations,which have strict training rotation requirements.Training hospitals all need to obtain the authentication including basic training,higher training and overseas training agencies.After 6 years strict training,the trainees have strong pediatric basic theories,procedure abilities,evidence-based practice and team work spirit.In short,the experiences of Hongkong pediatric specialist training is deserved to be learned by the standard training of pediatric resident in mainland China.

Objective To explore the ideal choice of feeding artery which is used for regional arterial infusion (RAI) in severe acute pancreatitis. Methods Forty-five patients with SAP were treated with RAI. The ideal feeding artery was that can supply entire pancreas according to arteriography and can maximize concentration of drug at pancreatic tissue. The pancreatic arteriography was considered as the final objective evidence for choice. Results (1)Gastroduodenal artery was chosen as feeding artery in forty-four cases, and superior mesenterlc artery was chosen in only one case because of vascular abnormity. (2)According to splenic arteriography, blood of splenic artery was supplied to spleen chiefly, and only partial tail of pancreas was applied by splenic artery. (3)According to celiac trunk arteriography, blood of celiac trunk could be supplied to entire pancreas, but a considerable proportion of the total blood was supplied to spleen through splenic artery and liver through hepatic artery proper.Therefore, the drug utilization index was lower. (4)According to gastroduodenal arteriography, blood of gastroduodenal artery could be supplied to entire pancrea, and almost all of the blood that contains drug flowed into pancreas. Therefore, the drug utilization index was higher. Conclusions Gastroduodenal artery is the ideal choice of artery which is used for regional intra-arterial infusion in sever acute pancreatitis. Pancreatic arteriography should be applied routinely when yever acute pancreatitis was treated with RAI.

Objective To screen for genomic copy number variants(CNVs)in six neonates with Pierre Robin sequence(PRS)by Affymetrix 2.7 M chip to identify possible loci related to PRS.Methods Six neonates with PRS admitted into the Department of Neonatology,Children's Hospital of Fudan University from June 2009 to May 2010 were enrolled in this study.CNVs were detected by Cytogenetic Whole Genome 2.7 M array.Rare CNVs with potential clinical significance that deletion segments' size ＞50 kb and duplication segments' size ＞200 kb were selected based on the analysis of Chromosome Analysis Suite(ChAS)software,false positive CNVs and segments of normal population were excluded.The identified CNVs were compared with those in relative published literatures.Results(1)Among 6 PRS patients,two patients had facial deformation,two had congenital heart defects,one had congenital dysplasia of the laryngeal cartilage and one had choroidal space occupying lesion.(2)Seven rare CNVs whose size from 51-11 956 kb were identified in four neonates,including a 739 kb duplication on lp26.23-p36.22,a 6273 kb deletion on lq43-44,a 51 kb and a 55 kb deletions on 14q32.31,a 1022 kb duplication on 14q11.1-11.2,a 11 956 kb duplication on 20p13 and a 105 kb deletion on 4q23.3.(3)Published literatures showed that deletions of 1q43-44 and 14q32.31 might relate to micro/retrognathia and abnormal palate.Region of chromosome 1q43-q44 contained AKT3 and heterogeneous nuclear ribonucleoprotein U(hnRNPU)genes,and the haploinsufficiency of AKT3 and hnRNPU genes might cause developmental human microcephaly and agenesis of the corpus callosum,speech delay and seizures respectively.Region of chromosome 14q32.31 contained some C/D small nucleolar RNA,and the human imprinted 14q32 domain shared common genomic features with the imprinted 15q11-q13 loci.Conclusions This study established a method to discover whole genome CNVs in identifying novel submicroscopic deletions and duplications.Reviewing of published literatures suggested that deletions of chromosome 1q43-q44 and 14q32.31 might cause Pierre Robin sequence.

Objective To investigate the clinical and laboratory diagnosis in a rare case with dwarifsm and multisystem abnormalities. Methods Whole-exome sequencing was performed and data was processed using high-throughput data analysis pipeline. Genetic test result is veriifed by Sanger sequencing. Results This is a 14-year-old boy with short stature (the height is 132 cm) and autoimmune hemolytic anemia. He was treated with long-term oral prednisone. Head CT from other hospital found multiple calciifcations on both sides of the basal ganglia, two sides of the frontal lobe, and the left side of parietal lobe. Lateral spinal X-ray photography showed lfat in thoracolumbar vertebral body. Valgus was surgically corrected. He also has facial pigmentation spot and onychomycosis. Whole-exome sequencing combined with Sanger sequencing identiifed a known homozygous pathogenic mutation in ACP 5 genes (c. 643 G>A, p.G 215 R). Identiifcation of such a mutation results in the diagnosis of spondylo enchondrody splasia with immune dysregulation (SPENCDI). Conclusions Whole-exome sequencing is one of the effective methods for detection of rare disease, the SPENCDI case reported here is a good example of it.

Objective To investigate the clinical manifestations in patients with 22q11.2 deletion syndrome (22q11.2DS) to improve the understanding of the disease.Methods Twenty patients with 22q11.2 DS were enrolled from Children's Hospital of Fudan University between August 2008 and April 2014.Cytogenetic and molecular genetic methods included fluorescence in situ hybridization (10 cases),and multiplex ligation-dependent probe amplification (10 cases).Age at the time of the diagnosis,sex and clinical manifestations were analyzed.Results The subject group consisted of 20 patients.Among them,13 cases (65％) were male and 7 cases (35％) were female.The median diagnostic age was 3.9 months.The presence of congenital heart diseases was identified in 17 patients (85％) and surgical correction was performed in 9 cases of them.The most frequent of complex congenital heart diseases were tetralogy of Fallot (20％) and pulmonary atresia (20％).Ten patients had varying degrees of T-cell immune function defects.Decrease in total lymphocytes and only CD8 counts were present in 45％ and 5％,respectively.Hypogammaglobulinemia was not detected in any patient.Six eases with T-cell immune function defects were treated with thymosin,4 of which were followed up for months,and the prognosis was good.Hypocalcemia was detected in 6 patients (30％),3 of whom presented with hypocalcemic seizures and hypoparathyroidism.Craniofacial dysmorphisms were detected in 3 patients(15％),2 of them only presented with micrognathia.Otorhinolaryngologic abnormalities were found in 4 cases (20％),3 of whom had laryngeal abnormalities,one of whom had cleft palate.Psychomotor developmental delay was found in 9 cases.Conclusions Congenital heart defects,hypocalcemia and/or impaired immune function are diagnostic features for 22q1 1.2 deletion syndrome,and they should be considered for cytogenetic analysis.

Objective To discuss the safety and effectiveness of transurethral resection of the prostate (TURP) on large-size (≥ 80 ml) benign prostatic hyperplasia (BPH).Methods Retrospective analysis of 958 BPH patients in Southwest Hospital during January 2010 to January 2013 was conducted.The patients were grouped into ≥80 ml prostate group (Group A) and ＜80 ml prostate group (Group B) according to the volume of prostate.Comparison was made between the 2 groups on the safety and effectiveness of TURP.Results There were 276 patients in Group A and 682 in Group B.No significant differences were shown in average age and preoperative American society of anesthesiology score of Group A and B.Compared with Group B,decrement in hemoglobin level and blood Na+ concentration of Group A was more significant (P＜0.01).There were more prostate tissues excised and duration of the operations was longer (P＜0.01).No significant difference was observed in peri-operative complications graded by the modified Clavien classification system,catheter durations and durations of hospital stay between the 2 groups (P＞0.05).At 6 months after the surgery,average maximum urinary flow rate (Qmax) increased from 5.9±2.9 ml/s to 17.1±8.2 ml/s for Group A and 6.1±3.0 ml/s to 17.5±6.4 ml/s for Group B,both groups showed significant increase in Qmax after surgery(P＜0.01).Six months after surgery,international prostate symptom score (IPSS) of Group A decreased from 23.7±6.1 to 5.9±4.9 while IPSS of Group B decreased from 23.1±5.5 to 6.2±4.4,both groups showed a significant decrease (P＜0.01).No significant difference was shown in IPSS,quality of life,Qmax,postvoid residual urine volume and occurrence rate of long-term complications after 6 months between the 2 groups (P＞0.05).Conclusion TURP is as safe and effective in treating large-size BPH as treating medium and small-size BPH.