Kasabach-Merritt syndrome(KMS) is a massive haemangioma with thrombocytopenia and consumptive coagulopathy. The histopathology of KMS is thrombocytopenia and disseminated intravascular coagulation associated with massive haemangioma.A standard treatment regimen for KMS has not been established. Therapy includes surgery, embolism and medicine(steroids,α-interferon , immunosuppressant , etc).

Objective To study the effects of high concentration oxygen exposure on the Sox17 expression of vascular endothelial cells of neonatal mice lungs,and to explore the pathogenesis of blocked lung vascular development.Methods Thirty two C57B1/6J newborn mice within six hours after birth were randomly divided to hyperoxia group (n=16) and room air group (n=16).Mice of hyperoxia group were exposed to 85％ oxygen.Eight mice of either group were sacrificed at 7 and 14 days after birth respectively to observe the lung morphology and calculate radial alveolar counts (RAC),which is the number of alveoli on the straight line from the center of respiratory bronchioles to the nearest fibrous septa or the pleura.Sox 17 expression in the pulmonary vessels was detected by immunohistochemical staining.Sox17 mRNA was measured by reverse transcription polymerase chain reaction.Sox17 protein level was measured by Western blot.Two independent samples t-test was used for statistical analysis.Results Compared with day 7,the lung structures matured with more uniformed alveoli and the septas became thinner on day t4 in room air group.However,the lungs developed slowly with simplified and non-uniformed alveoli on day 14 in hyperoxia group.The Sox17 protein was positive on endothelial cells of pulmonary arteries,veins and alveolar capillarys,as well as the alveolar epithelial cells.The RAC on day 7 and day 14 in hyperoxia group were both lower than that in room air group (3.7±0.7 vs 5.0±0.8,5.3±0.6 vs 8.3±0.9,respectively,t=3.057 and 8.148,both P ＜ 0.01).Sox17 mRNA on day 7 and day 14 in hyperoxia group were both lower than that in room air group (0.62±0.10 vs 0.88±0.11,0.44±0.06vs 0.90±0.15,t=3.607 and 6.926,both P ＜ 0.01).Sox17 protein level on day 7 and day 14 in hyperoxia group were both lowered than that in room air group (0.32±0.04 vs 0.76±0.04,0.36±0.07 vs 0.96±0.06,t=3.102 and 8.421,both P ＜ 0.01).Conclusions Exposure of high concentration of oxygen may cause impairment of lung vascular development by inhibiting Sox17 expression in lungs of neonatal mice.

Objective To observe the effects of nebulized nitric oxide donors on pulmonary and systemic hemodynamics during pulmonary hypertension induced by hypoxia (FiO_2=0.1,for 1 hour)in newborn piglets. Methods Twenty-four anesthetized and mechanically ventilated piglets were randomly divided into four groups:(1)group S (n=6),control group;(2)group C (n=6),receiving saline after hypoxia;(3)group NTG,receiving nebulized nitroglycerin after hypoxia;(4)group SNP,receiving nebulized sodium nitroprusside after hypoxia. Mean arterial pressure (MAP)and mean pulmonary arterial pressure (MPAP)were monitored continuously. Arterial blood gas analysis was determined at baseline,1 hour after hypoxia,termination of nebulization and 0.5 h after nebulization. After nebulization,blood methemoglobin (MetHb)and plasma NO level were measured.Results Acute hypoxia resulted in a significant increase in MPAP. At 1 hour after hypoxia,MPAP in group C,group NTG and group SNP were significantly higher than that in group S(P0.05).After nebulization,there were no significant differences in plasma NO level and blood MetHb level between groups. No rebound pulmonary hypertension was observed after withdrawal of nebulization. Conclusion Both NTG and SNP nebulization could be used as selective pulmonary vasodilators for acute hypoxic pulmonary hypertension.

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.

Objective To study the occurrence of nosocomial infection ( NI ) situation in the neonatal intensive care unit ( NICU ) of Xinhua Hospital affiliated to Shanghai Jiaotong University School of Medicine from January 2012 to October 2013,and analyze risk factors of NI. Methods A retrospective study was per-formed in the NICU,of which all neonates hospitalized over 48 hours were included and the occurrence situation and risk factors of NI were analyzed. Results This study included 1 357 neonates,and 175 neonates developed 202 times NI. The overall incidence of NI was 14. 89% (202/1 357),and the incidence density was 10. 44 per 1 000 NICU patient-days (202/19 355). The lower the birth weight is,or the smaller gestational age is,the higher the NI rate is. The NI rate was 75. 00% in neonates whose birth weight <1 000 g,and the NI rate was 52. 63%in neonates whose gestational age <30 weeks. The most common infection type was pneumonia[43. 07% (87/202)]and sepsis[26. 73% (54/202)]. The hospital stay of the NI neonates was longer and the cost of hospitali-zation is higher than non-NI neonates ( Median stay:20 days and 10 days. Median cost:21 045. 32 yuan and 8 108. 23 yuan)(both P<0. 01). Univariate analysis showed there were significant differences between NI neo-nates and non-NI neonates on gestational age,birth weight,5 min Apgar score,asphyxia rescue history,previons antibiotics use,mechanical ventilation,peripherelly inserted central catheter( PICC) ,closed thoracic drainage tube and chest or abdominal surgery before NI. The logistic regression analysis indicated that previous antibiotics use, mechanical ventilation and PICC were important risk factors for NI. Conclusion The NI rate in NICU is still high. Previous antibiotics use,mechanical ventilation and central venous catheter are risk factors of NI.

Objective To evaluate the appilcation value of intraoperative transesophageal echocardiography (IOTEE) in aortic valve replacement with bovine pericardium.Methods Totally 106 patients were ready to underwent the surgery of aortic valve replacement.The parameters of the aortic valves (diameter of aortic annular,aortic sinus and aortic sinus tube,effective height [eH]) were measured before cardiopulmonary by using IOTEE and during intraoperation.Results Eight cases were removed from the study due to supplemental preoperative diagnosis and modification of operative method,the rest of the 98 cases were enrolled.The coincidence rate of initial diagnosis hy using IOTEE and intraoperative diagnosis was 100 ％;5 cases (5/98,5.10 ％) of secondary diagnosis were inconsistent with intraoperative diagnosis.The coincidence rate of the number of aortic valves with preoperative IOTEE diagnosis and intraoperative diagnosis was 100 ％,the aortic valves perforation fistulas was 80.00％,the aortic valves vegetation was 85.71％,the senile calcified valvular disease was 100％,and the rheumatic disease was 100％.Diameter of aortic annular,aortic sinus and aortic sinus tube,eH measured by preoperative IOTEE had good correlation with intraoperative measurement (all r＞0.8,all P＜0.05).Conclusion IOTEE has feasibility and guiding value in aortic valve replacement with bovine pericardium,and is also of great significance to evaluate the prognosis of surgery.

Objective To study early diagnostic methods,treatment and prognosis of neonatal upper airway abnormalities.Method From Jan.2013 to Dec.2016,clinical data of neonates with upper airway abnormalities in neonatal department of our hospital was reviewed,including diagnostic methods,clinical manifestations,examination results,treat-ment and prognosis.Result Fifty-five cases of neonatal upper airway abnormalities were collected,including 34 males and 21 females,51 term infants and 4 premature infants.The most common diagnosis was congenital laryngomalacia (31 cases,14 of them had other upper airway abnormalities),followed by congenital cyst,vocal cord paralysis,laryngeal granuloma,Pierre Robin syndrome,etc.Most cases had presenting symptoms immediately or within days after birth,including dyspnea,cyanosis,laryngeal stridor,feeding difficulties,hoarseness and weak cry.Pneumonia occurred in 49 cases and respiratory failure in 16 cases.Thirty one cases received CT or MRI,abnormalities were found in 17 cases (54.8％).Thirty four cases received fiberoptic bronchoscopy,abnormalities were found in 33 cases (97.0％).The duration of hospital stay were 14 (7 ～20) days.Forty six cases were cured (20 cases received surgical treatment,26 cases conservative treatment),7 cases died (1 case died of chaotic atrial tachycardia and heart failure at 69-day after birth,and 6 cases died after discontinue of treatment),2 cases were lost on follow-up after discharge.Conclusion Newborn infants with upper airway abnormalities develop symptoms early,and some severe cases have respiratory failure.Imaging and fiberoptic bronchoscopy are helpful for diagnosis.Early surgical treatment can relieve airway obstruction and improve prognosis for congenital cyst,laryngeal granuloma,nasopharyngeal tumor and other space occupying lesions,and also severe upper airway obstruction caused by severe laryngomalacia,vocal cord paralysis,choanal atresia.

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.

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.

Objective To study the clinical and genetic characteristics of neonatal asymmetric crying facies (ACF).Method From January 2007 to December 2016,clinical data were retrospectivelyreviewed in patients with ACF admitted to Neonatal Unit of Xinhua Hospital.The data included maternalpregnancy and delivery history,clinical manifestation,results of chromosome florescent in situ hybridization(FISH) and gene chip test.Result Among 32 patients with classic ACF,8 were female and 24 weremale.5 cases were one of the twins or muhiplets.16 patients were born from mothers with history ofspontaneous abortion or induced abortion.16 patients presented with ACF on the left side and 16 patients onthe right side.7 patients had single ACF malformation,7 with malformation of another organ,and18 patients with malformation of more than two other organs.Combined malformation included earmalformation in 11 cases,facial malformation in 6 cases,cardiovascular malformation in 19 cases,urinarytract malformation in 3 cases,digestive system malformation in 3 cases,abnormal nervous system image in7 cases,and immune/endocrine system abnormality in 3 cases.10 patients received genetic analysis withFISH and/or gene chip tests and 4 patients had positive results.Among the patients who completed geneticanalysis,8 patients received FISH test and 2 patients had 22q11.2 distal deletion.1 patient hadheterozygous deletion in 22q21 region using gene chip test after he got a negative result with FISH.Amongthe 32 cases,6 patients died until now,among them,5 patients had multi-organ malformation.ConclusionACF is a tiny facial deformity,however it is often associated with other congenital malformations.Earlygenetic detection and systematic multiple malformations screening are particularly important for diagnosis andprediction of prognosis.