With the advances in surgical technique,increasingly complicated and knotty surgeries were performed on neonatal and pediatric patients,and the success of many of these surgeries are ensured by an adequate and safe blood supply.Autologous and/or allogeneic blood may be used,depending upon the anticipated blood loss,types of components required,and urgency of surgery.However,in all cases,every attempt should be made to minimize the number of donor exposures to reduce the risk of transfusion,for dangers of transfusion having been payed more attention.The strategy and practice of blood support for pediatric surgery show very important.

Objective To investigate the therapeutic efficacy of somatostatin on chylothorax after congenital heart disease surgery in children.Methods Retrospective analysis was performed in 13 postoperative chylothorax cases from Jan.2003 to Dec.2012,who were divided into control group (n =6) and treatment group (n =7),and there was no significant difference in age,weight,and time of occurrence between the 2 groups.The diagnosis standard for chylothorax was the same.The changes of chylous volume during the treatment were analyzed between 2 groups,and healing time and other data were analyzed too.Results Control group:clinical cure in 3 cases,conservative treatment failed in 3 cases(2 cases recovered after operation and 1 case died).The chyle volume of control group reduced obviously compared with post treatment from beginning [(256.6 ± 124.2) mL/d] to 1 week [(155.5 ± 85.7) mL/d] and 2 weeks [(142.3 ± 110.3) mL/d] later(t =4.623,2.099 ; P =0.002,0.044).But it did not reduce obviously in 3 weeks later[(139.4 ± 113.4) mL/d] (t =1.745,P =0.07).Treatment group:6 cases in treatment group were successful in recovery after conservative treatment and 1 case underwent operation.The chyle volume of treatment group reduced obviously compared with post treatment from beginning to 1 week [(51.2 ± 18.7) mL/d] and 2 weeks [(19.3 ± 7.05) mL/d] later (t =5.549,6.638 ;P =0.001,0.001).Compared with the control group,the difference between 2 groups in post treatment (1 week later,2 weeks hater) was of statistical significance (t =2.900,2.412 ; P =0.014,0.034).The healing time of conservative treatment in 2 groups (only for recovery) had obvious difference [(32.8 ± 1.8) d,(25.2 ± 1.7) d] (t =2.512,P =0.028).Conclusions The use of somatostatin can significantly promote the recovery of chylothorax in children,and it should be actively used in early conservative treatment.

Glial cell line-derived neurotrophic factor(GDNF) is a kind of protein factor which can regulate the enteric nervous system in survival,differentiation,colonization and injury repair.It has been confirmed in the disorders of the enteric nervous system,such as hirschsprung and anorectal malformations,but the specific mechanism in regulation of this factor is still unknown.Studies have found that GDNF/GFRa1/RET and GDNF/GFRa1 / NCAM pathway may be involved in the growth and maturation of the enteric nervous system,the disorders of those pathways above may lead to diseases by affecting the differentiation,proliferation and migration of intestinal neural stem cells,causing dysfunctions in the anatomical structure and function of the intestinal neurons.

Newborn brain damage is the main cause of new-borns' death and disabilities.Current research difficulties lie in analyzing characteristics of cerebral injuries,making objective prognosis and early intervention,as well as analysis of therapeutic effects after recovery.Since subjects are not requested to complete complex tasks while doing resting state functional MRI (rs-fMRI) tests,rs-fMRI is reckoned to be suitable for neonatal brain function research.So far,most rs-fMRI reports regarding cerebral injury are for adults,with only a few have been done on neonates.Foreign research are mainly focused on new borns' brain development.If relevant rs-fMRI research can be done on newborn brain damage,it would be helpful to accurately evaluate structure and function of patients' brain tissue damage.Further research can provide more valuable information in clinics.

Objective To investigate the cerebral haemodynamics change and morbidity of periventricular-intraventricular hemorrhages(PIVH) in premature infants with or without patent ductus ateriosus (PDA).Methods Bedside Echocardiography and transcranial sonography (TCS) were performed on 85 cases of preterm infants in 48 h,48 to 96 h,96 to 120 h,120 to 168 h after birth (gestational age≤34 weeks and birth weight≤2 000 g).PDA,peak systolic velocity (Vs),end-diastolic velocity (Vd),pulsatility index (PI) and resistant index (RI) of anterior and middle cerebral artery(ACA and MCA) and PIVH were monitored simutaneously.Results According to the Echocardiography and clinical symptoms,all the cases were divided into 3 groups:haemodynamic significant PDA group (hsPDA group,n =23),non-hsPDA group(nhsPDA group,n =38) and non-PDA group(nPDA group,n =24).The mean birth weight and gestational age did not differ significantly among the 3 groups.Single and multiple Logistic analysis indicated that invasive mechanical ventilation less than 48 h after birth was related to hsPDA (x2 =11.182,P =0.019; OR =10.06,P =0.039).Repetitive measurement deviation analysis found that:Vd of ACA and MCA were lower in the hsPDA group than those in the nhsPDA group (P =0.000,P =0.001) and the nPDA group (P =0.003,P =0.013),while PI and RI were higher than in the other 2 groups.Compared with non-closed group,at 7 days after birth in hsPDA group,RI and PI of closed group were significantly lower,while Vd was significantly higher.Nevertheless,there was no significant difference in hemodynamic parameters when comparison with nPDA group at 7 days after birth.There was no statistical difference in the grading and severity of PIVH with or without PDA.However,the rate of severe PIVH was found higher in hsPDA group than the other 2 groups(17.39％ vs 8.33％ vs 5.26％,x2 =2.405,P =0.280).Conclusions The invasive mechanical ventilation less than 48 h after birth was probably associated with preterm hsPDA.HsPDA could result in major cerebral haemodynamic changes and increase the morbidity of severe PIVH.

Biliary atresia (BA) is characterized of progressive inflammation and obstruction with fibrosis in biliary system and presents symptoms with obstructive jaundice in neonatal period,if its treatment is not ineffectively may lead to progressive liver fibrosis,end-stage liver failure and die within 2 years.Kasai procedure is the major method in BA treatment,but not all cases have postoperative jaundice clearance and survive with native liver,it is need to optimize the choice of surgical time and methods,postoperative protocol with medicine,prevention and treatment of complications for improving the postoperative therapeutic effect.In this paper,the current option of clinical research and statue in Kasai procedure were summarized.

The pathogenesis of obesity in children is unclear.Genetic play an important role,including gene mutations,polymorphisms,epigenetics.And the other hand,environment factors such as intrauterine environment,nutrition,physical exercise,and gut microflora also affect the obesity.The genetic and environment factors have interaction,leading to the occurrence of childhood obesity and development.With the advances in molecular biology techniques and large-scale,large sample size of population screening,new obesity-related genes,single nucleotide polymorphisms,the apparent genetic markers will continue to be found,looking forward to the future predict obesity,to choose to guide effective treatment,or even contribute to the development of genetic targeting drugs.

Objective Multiple carboxylase deficiency(MCD) is an autosomal recessively inherited defect of organic acid metabolism.The underlying mechanism is biotinidase(BT) or holocarboxylase synthetase(HLCS) deficiency.The authors reported 15 cases of MCD(clinical characteristics,diagnosis and treatment) and outcomes of long-term follow-up.Methods The clinical data of 15 patients with MCD admitted to Guangzhou Women and Children's Medical Center between Aug.2001 and Feb.2013 were analyzed.The diagnosis was confirmed by urinary organic acid analysis with gas chromatography/mass spectrometry (GC/MS),blood enzymatic determination and gene analysis.The patients consisted of 12 male and 3 female.The onset age ranged from newborn infants to 16 months.Results 1.Remarkable elevations of urinary lactate,3-oxy-butyric acid,3-OH-isovalerate,methylcitrate,3-methylcrontonylglycine,3-OH-propionate were detected in all of 15 cases.Fourteen cases with HLCS deficiency and 1 case with BT deficiency were confirmed by gene analysis.2.Most of patients with HLCS deficiency presented in the neonatal period or early infancy.The main clinical manifestations were skin rash (14 cases),tachypnea (9 cases),developmental retardation (8 cases),vomiting(5 cases),poor feeding (3 cases),developmental regradation (1 case),convulsion (1 case).Laboratory evaluation showed persistent metabolic acidosis and varied degree of ketosis,lactic acidosis,hyperuricacidemia,ammoniemia and hypoglycemia.Biotin was given to 13 patients in 10 mg/d and their metabolic disorders were corrected within 48 h,except one who gave up treatment and died.Treatment with Biotin in 5 mg/d provided clinical stability and normal neurodevelopmental outcome on follow-up for 3-11 (6.47 ± 2.70) years.3.One patient with BT deficiency presented with severe neurological symptoms(impaired consciousness,limbs trembling,tachypnea with irregular respiratory rhythm) at 16 months old.Demyelination of corpus callosum was evident on magnetic resonance imaging.Biotin treatment was given to him on the second of onset,and 1-year follow-up indicated significant improvement of his clinical symptoms,but the right limb weakness did not completely recover.Conclusions The main clinical features of HLCS deficiency include frequent skin rash,tachypnea,and metabolic disorders.BT deficiency may produce variable neurologic manifestations including demyelination of corpus callosum.Urinary organic acid analysis with GC/MS is critical to the early diagnosis of MCD.Prompt biotin treatment is recommended to correct metabolic derangements and continued therapy is essential to the improvement of long-term prognosis.Delayed commencement of therapy in BT deficiency can result in irreversible neurological damage.

Objective The report was focused on polycystic ovary syndrome(PCOS) in female adolescents related to homosexual precocious puberty,and the new awareness of its influence on long-term health of homosexual precocious girls.Methods A girl with idiopathic central precocious puberty (ICPP) but diagnosed as PCOS in adolescence was reported and the data were reviewed.Results A girl was diagnosed as ICPP when she was near 8 years old with obesity,but without hyperinsulinemia,then she received the treatment of a 3.75 mg dose gonadotropin hormone analogues (GnRHa) every 28 days.Her gonads development was under control while her growth was arrested.Growth hormone(GH) injection started at 13 months.She stopped all medications when finished GH and GnRHa treatment for 12 months and 25 months.She went to see doctors again because of no menarche after discontinuing medication for nearly 2 years.She appeared obese and acne,hirsutism,athanens negricans and purple purple striae on the skin.Hyperinsulinemia and hypertestosterone were demonstrated.Pelvic B ultrasonography showed polycystic ovary,and she was diagnosed as PCOS.She was ordered to lose weight and to take metformin.And adrenocorticotropic hormone stimulating test was done,and B ultrasonography again ruled out atypical congenital adrenal hyperplasia and tumor of adrenal gland.She got her menarche 1 month later.Twelve months after the PCOS diagnosis and treatment,she had 4 menses,her insulin level decreased,glucose tolerance and her serum testosterone level turned normal.At the same time,the form of ovarian and follicular was significantly reduced.After taking mefformin for 20 months,her height and weight did not change,her menstrual was regular every 35-40 days,each time lasting about 7 days.She was followed up.We also reviewed literatures and learnt that precocious puberty might not be a benign situation and it might have an intrinsic relation to obesity,precocious puberty and PCOS.It might be the cofactors for causing PP and PCOS that accessed luteinizing hormone(LH) secretion and disorder of hypothalamus pituitary gonad(HPG) axis except obesity with hyperinsulinemia.Reported pubertas praecox in childhood developed to PCOS at 30％ with high prevalence of obesity.GnRHa suppressive therapy might relate to PCOS and had disputation for improving final height.There were fewer investigations on adolescent PCOS,and no consensus guideline on it in China.Conclusions Girls with ICPP may develop to PCOS some time later.The clinical features of the reported girl and the knowledge from literatures support the hypothesis that inner relationship between the CPP and the PCOS.The LH high secretion and disorder of HPG axis may be the causes of them; LH treatment on suppressing obesity should be cautious during treatment.The benefit and risk from GnRHa treatment should be evaluated thoroughly.Further clinical research should be conducted on adolescent PCOS.

Objective To investigate the association between Down's syndrome(DS) and congenital heart diseases(CHD).Methods A total of 575 cases with DS from Jan.1997 to Mar.2013 in Children's Hospital of Chongqing Medical University were recruited.Retrospective study was conducted to analyze the prevalence and types of CHD in DS children,the relationship between the karyotype of DS and the types of CHD,and pulmonary hypertension (PH) and operation treatment.Results There were 370 cases(64.35％,370/575 cases) with CHD in 575 cases with DS.Among the 370 cases of CHD,322 cases (87.03 ％) were septal defects.In which,57 cases (15.41％) were atrial septal defects,36 cases (9.72％) were ventricular septal defects,12 cases (3.24％) were atrioventricular septal defects,and 157 cases(47.30％) were complex septal defects.Forty-eight cases(12.97％,48/370 cases) were nonseptal defect types of CHD (including patent ductus arteriosus,tetralogy of Fallot,double outlet right ventricle,pulmonary atresia,and so on).There was no statistical significance between the karyotype of DS and the types of CHD.There were 246 cases(66.49％,246/370 cases) with PH.Seventy cases(18.92％,70/370 cases) had interventional or surgical operations.All of them had descending pulmonary artery pressure after operation.Forty cases had other malformations such as gastrointestinal tract malformation,polydactylism / polydactyly,visual impairment,and so on.Conclusions The most common type of CHD with DS was atrial septal defect,and the second one was ventricular septal defect.There was no relationship between the karyotype of DS and the types of CHD.The patients with CHD in DS were prone to develop PH.So the comprehensive treatment plan should be developed as early as possible.