Objective To investigate the curative effect of inhaling heliox and air oxygen mixture in meconium aspiration syndrome under the support of synchronized intermittent mandatory ventilation (SIMV). Methods Sixty-four children with MAS in NICU of the DaPing Hospital, Third Military Medical University from March 1st, 2014 to May 30th, 2015 were taken as samples. The children, who were treated with SIMV as respiratory support mode, were divided into Helio group (n=33) and control group (n=31) in accordance with the random number table. Heliox group inhaled Heliox 70 (He 70% O-2 30%) for 6 h after replacement of inhaling air oxygen mixture (30% O-2 ); Control group inhaled air oxygen mixed gas. Main outcome measures: oxygenation index (OI) during treatment and extubation time; Secondary outcome measures: incidence of the complications of mechanical ventilation, hospital stays in NICU, bood gas analysis, inflammation index and myocardial injury markers, amplitude integrated electroencephalography (aEEG) results. Results OI in the heliox group was significantly higher than that in the control group; the difference was statistically significant (P=0.022), and the extubation time, incidence of the complications of mechanical ventilation and hospital stays in NICU of heliox group were significantly lower than those of control group; the differences were statistically significant (P=0.037, 0.013 and 0.010). Blood gas analysis index (pH, HCO-3-, BE, PaO-2 , PaCO-2 ) in two groups (2, 6, 12, 24, 48 h) presented some discrepancy; the differences were statistically significant (P<0.05). The inflammation index (IL-6, IL-8, TNF-a CRP) at 6 h and myocardial injury markers (CK, CK-MB) at 24 h of the two groups were decreased compared with those at 0 h, and heliox group decreased more significantly compared with the control group; the differences were statistically significant (P<0.05). The aEEG abnormal checking rate of children born after 28 and 100 days of heliox group was 9.1% and 3.0%, significantly lower than 25.8% and 9.6% of the control group (P< 0.05). Conclusion Inhaling heliox has better curative effect and high security than inhaling air oxygen mixture for MAS under the support of SIMV, and it is worthy of further study.
Objective To diagnose thyroglossal duct cysts (TDCs) in anterior cervical region in children based on imaging findings and clinical physical examnination. Methods Forty-nine patients with suspected congenital midline cervical anomalies in anterior cervical region and with radiologic images before surgery were categorized into TDCs group and non-TDCs group depending on their histopathologic diagnosis from September 2012 to October 2014. Comparative study was performed between the two groups with following 6 indicators: movement of cysts with deglutition and protrusion of the tongue ,the location of cysts in CT and MRI,close proximity to the hyoid bone,relationship to the midline, density and size.Then the sensitivity,specificity,false positive ratio,positive predictive value,negative predictive value and 95%CI of single or combined indicators with statistical differences were evaluated. Results Of the 49 pediatric patients,32 patients［(3.9±2.7) years old,19 boys］ had histopathologically confirmed as TDCs. Other 17 patients［(5.2±3.0) years old,7 boys］ had epidermoid cysts(n=9),dermoid cysts (n=4),teratoid cyst (n=1),ranula (n=1), and cystic lymphangioma (n=2). ①Single factor analysis showed there were statistically significant differences in movements with deglutition and protrusion of the tongue, the location and relationship to the hyoid bone of cysts between the two groups. There were no statistical differences in relationship to the midline and size of masses between the two groups. ②The sensitivities of each 3 indicators (cysts moving with deglutition and protrusion of tongue,cysts in close promixity to hyoid bone, cysts located in submandibular region) were 75.0%,65.6%,68.8% respectively, and specificities were 70.6%,94.1%,82.4% respectively for TDCs. The sensitivity and specificity of any one of these 3 indicators were 93.8%,29.4% respectively for TDCs.The sensitivity and specificity of all these 3 indicators were 0%,100% respectively for TDCs .The sensitivity and specificity of any one of these 2 indicators(cysts in close promixity to hyoid bone, cysts located in submandibular region) were 84.4%,82.4% respectively. Conclusion It is very useful to diagnose TDCs in anterior cervical region with clinical physical examination and radiologic imaging in chilidren.
Objective To evaluate the clinical utility of mycoplasma pneumonia RNA detection （real-time isothermal transcription-mediated RNA amplification assay, MP-SAT） in the treatment of Mycoplasma pneumoniae pneumonia in children. Methods Children with community-acquired pneumonia in Beijing Pinggu District Maternal and Child Health Care Hospital, according to the mycoplasma pneumoniae diagnostic criteria，were divided into the Mycoplasma pneumoniae pneumonia and non-Mycoplasma pneumoniae pneumonia groups. ①When the results of serological tests were considered as the standard，the sensitivity and specificity of MP-SAT were evaluated. ②MP - SAT and serum MP -Ab positive cases were observed dynamically，the detection rate of MP-SAT and MP-Ab in different treatment course and the times of MP-SAT turning to negative and clinical recovery were compared. ③The differences of fever duration after macrolide administration，blood routine inspection （WBC, N, L）, hypersensitive c-reactive protein (hs-CRP), serum lactate dehydrogenase (LDH) and the imaging results admitted to hospital within 24 hours were compared. Results Mycoplasma pneumoniae pneumonia group included 73 cases, non-Mycoplasma pneumoniae pneumonia group included 150 cases. The sensitivity, specificity of MP-SAT were 83.6%（61/73） and 97.3%（146/150）,respectively. Children with both MP-SAT and MP-Ab positive results were followed to evaluate the treatment effect. After the treatment, the detection rate of SAT reduced significantly (P<0.05), while detection rate of serum MP-Ab increased significantly (P<0.05). The time of clinical recovery was (3.0±0.9) weeks, similar to that of MP-SAT turning to negative（3.3±0.8) weeks (P＞0.05). Those with longer time of turning to negative had a longer fever duration after macrolide administration，increasing neutrophil，decreasing lymphocyte， higher level CRP and LDH, and large field of consolidation were more frequently observed on X-ray film (P<0.05). Conclusion MP-SAT can be used as an index for evaluation of the treatment outcome of MPP.
Objective To investigate the main risk factors and long term outcome of preterm infants with bronchopulmonary dysplasia (BPD). Methods Children with BPD were collected from neonatal intensive care units (NICU) in Children′s Hospital Fudan University, from January 1, 2012 to December 31, 2013. All babies with gestational age ≤ 32 weeks, birth weight ≤1 500 g, and admission within 7 days after birth were included in this study. In the same period，according to the inclusion criteria, the same sample size of non BPD was collected as control group. Univariate and multiple logistic regression analyses were used to determine factors associated with the risk of BPD. At the same time ,the incidence of bronchitis,pneumonia,wheezing and rehospitalization were analyzed during the first two years of life. Results There were 156 cases in BPD group and matched 156 infants without BPD in control group. Mother′s information including age, the proportion of preeclampsia and vaginal delivery was significantly higher in BPD group than that in control group(P=0.046,0.025 and <0.001). Birth gestational age and birth weight of BPD group were significantly lower than those of control grouop ( P <0.001 ).Infant information including 1 min Apgar score, 5 min Apgar score, sepsis ≥72 h , patent ductus arteriosus ( PDA ), retinopathy of prematurity ( ROP ) , ventilator associated pneumonia ( VAP ) , the application of PS and mechanical ventilation ≥7 d was significantly higher in BPD group than that in control group. The factors increasing the risk of BPD determined by multivariate logistic analysis were: gestational age (OR=0.46，95%CI:0.37-0.58), mechanical ventilation ≥7 d(OR= 9.47，95%CI:3.70-24.27), PDA (OR= 2.21，95%CI:1.18-4.12 ) and preeclampsia( OR= 4.91，95%CI:1.26-19.15). In the first year of life, the incidence of bronchitis and wheezing in preterm-born children with a history of BPD was significantly higher than that in preterm-born children without BPD, but there was no significant difference in rehospitalization between the two groups. The incidence of pneumonia of BPD group was significantly decreased in the second year of life, while there was no significant difference in bronchitis, wheezing and rehospitalization compared to the first year of life. Conclusion Low gestational age, mechanical ventilation ≥7 d, patent ductus arteriosus, preeclampsia were highest risk factors for BPD. Among premature infants, BPD substantially increased the risk of lower respiratory tract infection during the first year of life.
Objective To investigated the risk factors for pediatric acute leukemia complicated with fungemia. Methods Pediatric acute leukemia patients with blood culture fungus positive from 01/2002 to 06/2014 were taken as fungemia group,from Oncology Center of Beijing Children′s Hospital affiliated to Capital Medical University;While the control group included the patients with same in hospital time, same sex, without fungal infection history and fungemia happened.The sample size was 1:2 for fungemia group to control group. The data of age, morbid state(remission or not),relapse situation,in hospital time,using hormonal chemotherapy regimens or not,neutropenia time and degree, central venous indwelling catheter, using broad-spectrum antibiotics and accompanied with septicemia were collected. Single factor and multiple-factor analyses were used to investigate the risk factors for pediatric acute leukemia complicated with fungemia. Results There were 48 cases in fungemia group,male patients 38, median time of using chemotherapeutics was 98（3-300）d, median age was 5.6（1.4-13.8）years.There were 96 cases in control group, male patients 64, median time of using chemotherapeutics was 201（4-1 080）d, median age was 6.7（1-15）years. ① All 48 cases had fever in fungemia group, median time of fever duration time was 8.6（1-42）d. Median time from admitting to hospital to fungemia was 17.6（1~115）d;② 5 cases were with fungal infection history（10.4%）;7 cases were complicated with organ function failur（14.6%）；10 cases with bacteria septicemia（20.8%）;11 cases died or giving up treatment（22.9%）.③ Cultivating result showed that in 17 cases both candida parapsilosis and tropicalis（35.4%） were positive, in 8 cases candida albicans（16.7%） positive, in 2 cases season and candida （4.2%） positive, and candida glabrata, penicilium, fusarium and trichosporon asahii positive in one case respectively（2.1%）.④ Single-factor analysis showed that morbid state(no remission), neutropenia time>7 days, neutropenia degree<0.5×109·L-1, using broad-spectrum antibiotics and complicating with other organs fungal infection were risk factors of pediatric acute leukemia complicated with fungemia.Multi-factor analysis showed that using broad-spectrum antibiotics was independent risk factors of pediatric acute leukemia with fungemia，OR=28.3(95%CI: 5.9-136.2). Conclusion The main pathogen agent of fungimia was candida,especially non-candida albicans. Using broad-spectrum antibiotics was an independent risk factor of pediatric acute leukemia with fungemia
Objective To evaluate the risk factors for NEC in gestational age (GA)<34 weeks neonates. Methods Risk factors associated with NEC were investigated using a retrospective case-control design. Patients with Bell′s stage NEC ≥Ⅱ were identified between 1st March 2010 and 1st March 2015. Each case was paired with two GA- and weight-matched controls. Data were collected from medical records,including ICP, asphyxia after birth, family allergy history, breast feeding, MAS, sepsis, CHD, probiotics and transfusion of PRBC, and univariate and logistic regression analyses employed to analyze the risk factors related to NEC. Results A total of 66 cases and 132 controls were enrolled.The demographic characteristics of the two groups were not statistically significant. The onset time of 66 cases was 2-27 d after birth and the average time was 15.5 d. Variation in months after NEC was diagnosed was noted, with a peak in January (9 cases) and a trough in August (2 cases). Intrahepatic cholestasis of pregnancy (ICP) （OR=2.38,95%CI:1.74-5.49）,transfusion with packed red blood cells (PRBCs) （OR=3.33,95%CI:1.76-5.78）,postnatal asphyxia (OR=2.49,95%CI:1.69-4.95) and sepsis （OR=4.05,95%CI:2.01-6.87） were significantly associated with NEC. Probiotic use was protective factor. Conclusion A seasonal variation in months in babies with NEC was observed. A range of conditions was found to be associated with an increased risk of NEC under 34 weeks′ gestation. IICP, asphyxia after birth, sepsis and transfusion of PRBC were found to be associated with an increased risk of NEC under 34 weeks′ gestation. Probiotics was a protective factor.
Objective To illustrate the clinical features, efficiency of anti-arrhythmic medications and prognosis in pediatric tachycardia-induced cardiomyopathy (TIC) . Methods Retrospective review of medical records was carried out in children with TIC, and defined as LVFS <30% associated with various tachyarrhythmia in Children′s Hospital of Fudan University. Data with respect to the clinical findings, anti-arrhythmic therapy, electrocardiographic and echocardiographic evaluation and biomarkers were collected. Clinical follow-up was continued until both LVFS≥30% and Z-LVEDD>2.0 were achieved. Multivariate linear and logistic regression analysis was performed to evaluate possible predictors of time to myocardial recovery. Results A total of 56 pediatric cases with TIC were included in our study from May 2003 to January 2016. Forty patients （71.4%）were infants and 37 were boys (66.1%). Atrial tachycardia and paroxysmal supraventricular tachycardia were the most common types of responsible tachyarrhythmia, with the percentage of 53.6% and 37.5%, respectively. Anti-arrhythmic medications as part of management were used in all 56 cases and complete, partial and none effective response to medications occurred in 29 (51.8%), 23 (41.1%) and 4 (7.1%) cases, respectively. Amiodarone was the most commonly used medication, and its effectiveness was achieved in 92.3% (36/39) children, including both used alone and in combination with other medications. Except for 2 cases of death, left ventricular fractional shortening (LVFS) was significantly increased from (23.7±3.7)% to (32.8±4.6)% (P<0.001), and Z-LVEDD was decreased from (2.79±3.04) to (0.37±2.0) ( P<0.001) in the remaining 54 cases. Median time for recovery of LVFS and Z-LVEDD was 10 days and 42 days, respectively. Plasma (N-terminal) pro B-type natriuretic peptide was reduced significantly from (15 711±11 453) pg·mL-1 to (1 287±1 510) pg·mL-1 (P<0.001). Predictor of earlier left ventricular size normalization was smaller baseline Z-LVEDD (β=0.528，95%CI：0.127-0.506，P=0.002). Conclusion Myocardial dysfunction is wholly or partially reversible after control of the responsible tachyarrhythmia in pediatric TIC. Early recognition and treatment by clinicians are crucial for favorable recovery of left ventricular function.
Objective A difficult hurdle in whole exome sequencing application is rapid data interpretation. In this study, whole-exome sequencing and WuXi NextCODE software were used to rapidly identify pathogenic mutations in 5 undiagnosed cases. Methods The exome targets of the patient′s DNA were captured with the SureSelct Human All Exon kit followed by sequencing with the Illumina HiSeq2000 platform. The WuXi NextCODE software was used for the data analysis. The detected variant was confirmed with Sanger direct sequencing. Results 5 trio families with undiagnosed probands were recruited. A heterozygous missense mutation was identified in FGFR2 gene in proband 1, compound heterozygous missense mutations in GBE1 gene in proband 2, and a heterozygous missense mutation in TBX1 gene in proband 3 by whole-exome sequencing of trio samples. A homozygous missense mutation was identified in IL10RA gene in proband 4, and compound heterozygous missense mutations in IL10RA gene in proband 5 by whole-exome sequencing of proband only. Conclusion This study clearly showed the efficacy of whole-exome sequencing and was helpful to rapid genetic diagnosis for undiagnosed cases.
Objective To understand whether there are al differences of MECP2 between male and female brains, which may contribute to the gender differences in MECP2 associated diseases, e.g. autism. Methods Genomic DNA of brain tissues from four normal aborted fetuses was obtained by phenol-chloroform extraction. By using MethPrimer online software, the CpG island of MECP2 gene was enriched between -1 000 bp to + 1 200 bp. To detect the level of methylation, bisulfite sequencing method was used (by using the EZ DNA Methylation-goldTM Kit ). For ultrasound fractured genomic DNA, ChIP assay was performed with genomic hydroxymethyl kit (diagenode, hMeDIP Kit). The levels of MECP2 in brain tissues were detected with cDNA that was obtained by FastQuant RT Kit (With gDNase) and were quantitated using SuperReal PreMix Plus (SYBR Green) kits. Results Sample 1, male,weight 106 g, length 17.4 cm;sample 2, female, weight 100 g,length 19.1 cm; sample 3, male, weight 500 g, length 28.3 cm; sample 4, female, weight 510 g, length 31.5 cm. The level of MECP2 in brain of male (sample1=0.0367, sample3=0.0155) was higher than that in female (sample2=0.0177, sample 4=0.0088) during early embryonic stage. The methylation level of the core promoter region of MECP2 in male was significantly lower than that in female per X chromosome, especially in the core promoter region -309 bp to -179 bp. Almost no methylation appeared on MECP2 in brain of male while the hydroxymethylation level was the opposite. Conclusion These results indicated that the gene modifications on MECP2 in male may contribute to its . This may in turn increase the susceptibility of male to MECP2 mutations in disease such as autism and result in sex ratio changes in patient population.
Objective To improve the recognition of eosinophilic gastroenteritis（EG） in children. Methods The data of clinical manifestation, imageological examination, diagnosis and management from a 14-year-old boy with eosinophilic gastroenteritis presenting as acute pancreatitis were retrospectively analyzed. Clinical features of similar cases from published literatures were summarized.And related literatures were also reviewed systematically. Results A 14-year-old boy was admitted to our hospital due to abdominal pain and diarrhea for 1 week.He was early diagnosed as acute pancreatitis.Test results showed significant increase of eosinophils proportion (62%)，serum IgE (393 U·mL-1) and proportion of eosinophils in bone marrow cytology. Esophagogastroduodenoscopy indicated eosinophilic infiltration in duodenum. Furtherly，he was confirmed eosinophilic gastroenteritis by duodenal mucosa biopsy which showed eosinophilic infiltration. After the therapy of prednisone and anti-allergic drugs,he had complete remission 1 week later.And the peripheral eosinophils proportion was decreased to normal. And the boy didn’t have a relapse more than two years. The Wanfang digital knowledge service platform，CNKI, Elsevier, and PubMed were searched for relevant articles. 11 studies were included and 14 cases were summarized. All patients were misdiagnosed due to the initial performance of acute pancreatitis. All patients had nonspecific clinical manifestations such as abdominal pain, nausea and vomiting,but with peripheral blood EOS significantly increased.The proportion of EOS in ascites had important significance to the diagnosis of EG complicated with acute pancreatitis.All patients had different degrees of abnormal changes of stomach and duodenum mucosa.13 patients receiving glucocorticoid treatment after diagnosis had good prognosis. One case replased after surgical removal of the pancreas, and treating with glucocorticoid after diagnosis had good prognosis. Conclusion Children with EG complicated with acute pancreatitis are rare. Acute pancreatitis with unknown causes and accompanied with eosinophilia or bad clinical outcome after conventional treatment of enzyme or acid inhibition may indicate the possibility of eosinophilic gastroenteritis. Endoscope pathology biopsy should be taken as early as possible to confirm the diagnosis.
【Abstract】 Objective:The purpose of this paper is to investigate,summarize the clinical characteristics,and provide evidence for the diagnosis and treatment of infants with autoimmune hepatitis (AIH).Methods:Based on 2 cases of AIH in our hospital we analyzed patient's symptoms,physical signs,results of their auxiliary examination,the corresponding theurapeutic schemes results,and the literature data.Results:Thrombocytopenia and abnormal liver function were the original symptoms.Hepatosplenomegaly were the main clinical manifestations in those 2 patients.Patient's liver function test,immunoglobulin,serum antibodies,liver biopsy results,and good response to corticosteroid alone or in combination with azathiprine (AZA) all supported the diagnosis of AIH.Conclusions:The clinical symptoms of AIH disease may not specific and AIH is considered rare in the infant population.Clinicians should always consider AIH in children presenting with chronic or acute liver disease.Liver biopsy is required as soon as possible to establish the diagnosis,it can provide evidence for the treatment,drug withdrawal,and prognosis.Once the diagnosis is clear, treatment should be instituted as soon as possible to avoid progression of the disease and it consist of corticosteroids alone or in combination with azathioprine (AZA).
