Objective To analyse the reasons for patients′long PICU stay and to discuss how to de-crease their PICU stay length and optimize medical resource utilization.The another objective is to identify these patients′long-term outcome.Methods We retrospectively analysed the basic information of patients who stayed in our PICU more than 30 days during January 1,2013 to July 31,2014,including pediatric criti-cal illness score on admission,primary diseases and outcome on discharge,in order to determine main reasons for their long PICU stay.Follow-up prognosis was made by Paediatric Overall Performance Category.We used SPSS 19.0 to do data analysis.Results Sixty-seven patients enrolled in this study included 43 boys and 24 girls,49.2%of whom came from emergency department.The median age was 24 months(IQR 8,108), while median length of PICU stay was 39 days(IQR 31,49).Mean pediatric critical illness score on admi-ssion was 78.39 ±9.57.Mean hospital fees was(144 071.74 ±76 944.74)Yuan.Thirty(44.8%)patients did not have any medical insurance.Top three primary diseases were respiratory disease(30 cases),neurolog-ical disease(8 cases)and systemic infection(6 cases).Top three underlying diseases were tracheostenosis or bronchopulmonary dysplasia(6 cases),congenital heart disease(5 cases)and immunodeficiencies(4 cases). Top three complications were respiratory failure(30 cases),multiple organ dysfunction or failure(16 cases), shock(9 cases).Top three reasons for longer PICU stay were weaning off mechanical ventilation difficulty (29 cases),department or hospital transference difficulty(20 cases),feeding difficulty(16 cases).Fifty-four patients were discharged with better health condition,10 patients were auto-discharge.The mortality at dis-charge,1 month after discharge,3 months after discharge and 6 months after discharge were 4.5%,20.9%, 25.4% and 25.4%,respectively.Total 36(53.7%)patients had a good or mildly abnormal Paediatric Over-all Performame Category score(1-2 points)at 6 months after discharge.Conclusion Sequential noninvasive ventilation,timely tracheotomy,timely patients′transference to general ward,development of family ward and rehabilitation wards,appropriate nutrition delivery,regularly difficult disease consultation,reduction of hospi-tal infection and complications may do contribution to shorten these patients′length of PICU stay.Children who survive longer than 3 months after discharge have better long-term prognosis.

Objective To summarize the experience on treatment for 15 cases of acute pulmonary embolism(PE).Methods Fifteen acute PE patients admitted from June 2009 to May 2011 were analyzed retrospectively.All patients were diagnosed as PE and deep vein thrombosis,and treated with placement of inferior vena caval filters(IVC).Five patients with main pulmonary artery embolism accepted intrapulmonary arterial interventional therapy of thrombus fragmenlation and suction and catheter-directed thrombolysis (CDT).Ten patients with embolization on pulmonary artery branch and acute iliofemoral vein thrombus accepted therapy of peripheral thrombolysis.During postoperative course improvement was observed on the clinical symptoms,occurrence of complications,Miller index,change of mean pulmonary arterial pressure (mPAP) and arterial partial pressure of oxygen(PO2),as well as the patency of pulmonary artery.Result Five main pulmonary artery embolization patients gained complete patency of pulmonsnary artery,and the clinical symptoms immediately improved.Miller index reduced from (0.51 ± 0.04) to (0.27 ± 0.38),mPAP decreased from (55.3 ± 3.1 ) mm Hg to ( 32.7 ± 2.2 ) mm Hg,and PO2 elevated from ( 40 ±3 ) mm Hg to ( 63 ± 4) mm Hg,showing a significant difference ( P ＜ 0.01 ).Ten patients with pulmonary artery branch embolization gained patency of pulmonary artery branch,iliofemoral venous thrombosis cleared,and clinical symptoms significantly improved.All patients recovered after two weeks of intravenous thrombolytic,anticoagulation and antiplatelet therapy.During three to twelve months' follow up,the therapeutic effects persisted and there was no recurrence.Conclusions Emergency intrapulmonary arterial interventional therapy of acute PE has remarkable effectiveness,safety and feasibility,improving pulmonary obstruction and clinical symptoms.

Objective To evaluate the safety and efficacy of SilverHawk directional atherectomy device in the treatment of arterial stenoses/occlusions of the femoropopliteal regions.Methods From April 2011 to May 2012,36 patients (28 men,8 women,age range 60 - 84y) with 40 arteriosclerotic lesions of femoro-popliteal arteries (de novo lesions in 25 arteries,in-stent restenosis in 15 arteries; Rutherford score of 3 - 5 ) were included in the treatment by SilverHawk directional atherectomy device.All the patients were diagnosed via low-extremity artery CTA and arteriography.The mean ankle brachial index (ABI) of treated limbs was 0.53 ±0.12.Results The overall technical success rate was 100％ (40/40).The procedural success rate was 87.5％ (35/40).There was no perioperative mortality with three cases developing complications,vascular injury occurred in two cases.One was treated by stent-graft while the other was managed conservatively; Procedure-related arterial embolization occurred in one case and was treated by suction through catheter.Symptoms were relieved in all patients,ABI increased to 0.72 ± 0.18.All the patients were followed up for an average of (6.4 ± 1.2) months.Postoperative restenosis developed in one case and treated with balloon angioplasty and stent placement after six month.Conclusions SilverHawk directional atherectomy device is effective and safe in treament of arterial stenoses/occlusions of the femoropopliteal lesions,with satisfactory early results.

Objective: To explore the genetic basis for a fetus with Dandy-Walker malformation. Methods: G-banding chromosomal karotyping, single nucleotide polymorphism microarray (SNP array) and fluorescence in situ hybridization (FISH) were carried out for the fetus. Chromosomal karyotyping and FISH assay were also carried out for both parents. Results: SNP array has detected a 4266 kb microdeletion at 6p25.3p25.1 in the fetus, which was confirmed by FISH. FISH analysis of the parents demonstrated that the father has carried a cryptic t(6; 14)(p25.1; p13) translocation, while the fetus has a der(6)t(6; 14)(p25.1; p13) derived the paternal translocation. Conclusion The der(6)t(6; 14)(p25.1; p13) probably underlies the Dandy-Walker malformation in the fetus. The 6p25.3p25.1 microdeletion is due to unbalanced gametes produced by the father’s cryptic balanced translocation.

Objective:To analyze the molecular characteristics of Echovirus 11 (Echo11) strains isolated in Xiangyang, Hubei Province from 2016 to 2017 based on the sequences of VP1 gene.Methods:Rectal and throat swab specimens were collected from children with hand, foot and mouth disease (HFMD) in Xiangyang from 2016 to 2017. Echo11 strains were detected by real-time reverse transcriptase PCR (RT-PCR) and isolated after cultured in human rhabdosarcoma (RD) cells. The VP1 regions of Echo11 strains isolated from RD cells and the whole genomes of three representative Echo11 strains were amplified by conventional RT-PCR and the sequences were analyzed. DNAStar7.0 (MegAlign) and MEGA6.0 (Data) were used to analyze the homology and mutation sites in nucleotide and amino acid sequences. Neighbor-joining method was used to construct phylogenetic trees. Recombination analysis was performed with SimPlot software (BootScanning).Results:A total of 11 Echo11 strains were isolated from 3 494 HFMD cases, accounting for 0.31%. They were highly homologous in the VP1 gene. These strains shared 98.4%-100.0% homology in nucleotide sequences and 98.3%-100.0% homology in amino acid sequences. The homology between the 11 Echo11 strains and the prototype strain (Echo11/Gregory, X80059) was 73.9%-74.8% in nucleotide sequences and 87.7%-88.7% in amino acid sequences. All of the Echo11 strains circulating in Xiangyang were classified into lineage D, having a similarity to the strains circulating in some regions of mainland China since 2013. In multiple regions of the genome, the Echo11 strains isolated in Xiangyang were highly similar to the Henan Echo1 strains in 2010 and the Hubei Echo6 strains in 2015, suggesting there was recombination within the genome of Echo11 strains in Xiangyang.Conclusions:The Echo11 strains circulating in Xiangyang from 2016 to 2017 belonged to lineage D and were recombinant strains.

Objective To analyze the clinical efficacy of AngioJet mechanical thrombus aspiration system for patients with acute pulmonary embolism (PE).Methods Clinical data of 28 cases of acute pulmonary embolism (PE) patients was retrospectively analyzed,8 cases (AngioJet group) were treated with AngioJet + CDT,20 cases were treated by pigtail catheter thrombolysis(CDT group)alone,the total amount of urokinase,thrombolytic time,related detection index and the occurrence of complications were compared between the two groups.Results The dosage of urokinase in the two groups was (72.5 ± 44.4) × 104U and (169.0 ± 59.3) × 104 U respectively,P ＜ 0.05.The catheter indwelling time was (1.0 ± 0.89) days and (2.65 ± 0.86) days respectively (P ＜ 0.05).There were no statistically significant differences in SBP,PaO2,SpO2 and D-dimer between the two groups before and after operation (P ＞ 0.05).Conclusion Both AngioJet and CDT are effective methods for the treatment of acute PE.The combination of the two methods can accelerate the improvement of clinical symptoms,reducing the dosage of thrombolytic drugs and the occurrence of surgery-related complications.

OBJECTIVE: To diagnose a fetus with Phelan-McDermid syndrome (PMS) using various techniques. METHODS: Single nucleotide polymorphism array (SNP Array), multiplex ligation-dependent probe amplification (MLPA), fluorescence in situ hybridization (FISH) were applied in conjunction for the prenatal diagnosis of the fetus. RESULTS: SNP Array detected a 4.03 Mb microdeletion at 22q13.31q13.33 in the fetus, which was confirmed by FISH and MLPA. FISH analysis of the parents suggested that the 22q13.31q13.33 deletion has a de novo origin. CONCLUSION Combined use of various techniques can enable accurate prenatal diagnosis and genetic counseling.
Chromosome Deletion ; Chromosome Disorders ; diagnosis ; Chromosomes, Human, Pair 22 ; Female ; Fetus ; Humans ; In Situ Hybridization, Fluorescence ; Pregnancy ; Prenatal Diagnosis

Objective:To analyze the prognosis and influencing factors of acute necrotizing encephalopathy (ANE) in children.Methods:Clinical data of ANE patients admitted to Pediatric Intensive Care Unit, Beijing Children′s Hospital, Capital Medical University from March 2012 to February 2019 were retrospectively analyzed.Survivors were followed up by telephone or outpatient department, and the quality of life was evaluated by pediatric overall performance category scale.The t-test or rank sum test was used for comparison between groups, and the COX risk regression was used to analyze the influencing factors of prognosis. Results:A total of 38 patients were enrolled in this study with the male-to-female ratio of 1.24∶1.00, and median age of 29.5(10.0-130.0) months.They were followed up for the median of 27(15-96) months.The overall survival rate at 7 days, 14 days and 2 months after disease onset were 57.9%, 42.1%, and 34.2%, respectively.The mortality rate at discharge was 34.2%(13/38 cases), and the cumulative mortality rate at the 1 st, 3 rd and 12 th months after discharge was all 68.4%(26/38 cases). The complete reco-very rate was 10.5%(4/38 cases) after one-year follow-up.The univariate analysis indicated that cardiopulmonary resuscitation before admission, Glasgow coma score < 5 at admission, complication with shock/cerebral hernia/multiple organ dysfunction syndrome, creatine kinase isoenzyme> 100 U/L, lactate dehydrogenase>1 000 U/L, hypoalbuminemia, hyperglycemia, hyperurea, prolonged prothrombin time and elevated international standardized ratio were risk factors for the prognosis of ANE in children ( β=3.519, 6.967, 6.803, 3.000, 6.389, 3.471, 2.252, 1.616, 2.377, 3.092, 2.713, and 4.510, respectively, all P<0.05). Meanwhile, high-dose Methylprednisolone[20-30 mg/(kg·d)] and immunoglobulin (2 g/kg, divided into 2-5 days intravenous drip) treatment were protective factors ( β=0.625, 0.405, respectively, all P<0.05). The COX multivariate analysis showed that high-dose Methylprednisolone treatment [20-30 mg/(kg·d)] was an independent protective factor for the prognosis of children with ANE [95% CI: 0.449(0.213-0.944), P=0.035]. Conclusions:Early application of high-dose Methylprednisolone and immunoglobulin may contribute to the good clinical outcome.Children with neurological sequelae should be actively treated with rehabilitation, and the quality of life may be gradually improved.

Acute necrotizing encephalopathy (ANE) is a rare but distinctive acute encephalopathy with global distribution.It usually begins with a virus-associated febrile illness, which deteriorates rapidly, and leading to convulsion, unconsciousness and even coma.The neuroradiologic features of ANE are multi-focal and symmetrical brain lesions, involving bilateral thalamus.ANE has no specific treatment method at present.The majority of ANE patients are accompanied with varying degrees of neurological sequelae, and even death.In this review, we summarized the epidemiology, diagnosis and prognosis of this rare but fatal disease in children.

Objective:To compare the mid-term clinical effects of AngioJet rheolytic thrombectomy assisted catheter-directed thrombolysis (ART+CDT) with catheter-directed thrombolysis (CDT) in the treatment of acute deep venous thrombosis of lower extremities.Methods:Ninety-one patients admitted to the Department from Jan 2016 to Dec 2017 were placed with inferior vena cava filters and divided into ART+CDT group (30 cases)and CDT group (61 cases). Total urokinase dosge, thrombolytic time, operative cost, length of hospital stay, detumescence rate, thrombus clearance rate, cumulative patency rate of lower limb veins, Villalta score at 2 years and 5 years, thrombosis recurrence rate and chronic venous insufficiency quality of life questionnaire were compared between the two groups.Results:The success rate of surgery was 100% in both groups, there was no mortality. There were significant differences in the short-term postoperative outcomes between the two groups in terms of total dosage of urokinase, thrombolysis time, total cost of surgery, length of hospital stay, detumescence rate, venous patency scores before and after treatment, and venous patency rate (all P<0.05). For the mid- and long-term postoperative outcomes of 2 and 5 years, there were no significant differences in the incidence of PTS, recurrence rate of thrombus, chronic venous function scale, and cumulative patency rate at 2 years (all P>0.05). Conclusions:ART+CDT has a significant advantage over CDT alone in terms of early efficacy and early reopening of blood flow in patients. Both ART+CDT and CDT have a low incidence of PTS and a low recurrence rate of thrombus in the mid-term follow-up, and both have satisfactory performance in the mid- and long-term efficacy of interventional treatment of deep venous thrombosis of lower limbs.