Objective To assess reliability, validity and responsiveness of evaluation to comfort by using Echelle DouleurInconfort Nouveau-Ne, neonatal pain and discomfort scale (EDIN) in 0 to 5 year-old children with acute fever. Methods Pretesting-version of EDIN was introduced into China through translating and back translating, then each version was discussed and modified, and developed by the experts. Five pediatric clinical experts and guardians of 30 children with acute fever were recruited to administer the EDIN scale Chinese pre-testing version to form Chinese version of EDIN by surface validity test. One hundred guardians of children with acute fever completed Chinese version of EDIN to evaluate the validity, reliability and responsiveness with correlation coefficient and Cronbach coefficient. Results ①Five experts and 30 children guardians considered that the scale items were clear and understandable, completed filling in the scale within 5 minutes. ②97 cases filled in Chinese version EDIN to analyze reliability and validity. The split-half reliability was 0.887, the test-retest reliability was 0.734, the Cronbach coefficient scale factor was 0.892. ③The consistency rate of the Wong-Baker scale and EDIN scale was 0.885, the criterion-related validity was good. Component analysis showed facial activity defined the 78.4% of the full variant. ④There were significant differences in 5 dimensions and the total scores in reactivity of the first visit and the third visit after 72 hours, which indicated the reactivity was very good (P<0.001). Conclusion Evaluation of the Chinese version of EDIN shows that the criterion-related validity, reliability and reactivity are relatively good, the construct validity is subject to increase. It can be applied to evaluate the comfort in 0 to 5 year-old children with acute fever.
Objective To investigate the current status of critical ill newborns based on the data of critical neonatal collaboration network, to improve the level of diagnosis and treatment of critical ill newborns. Methods Data of small premature infants, neonatal sepsis, neonatal necrotizing enterocolitis (NEC) and invasive and non-invasive mechanical ventilation (treatment more than or equal to 4 h). Information of general information, perinatal information, patent ductus arteriosus (PDA), nervous system, retinopathy of prematurity (ROP), oxygen to support or ventilator in the treatment of neonatal respiratory distress syndrome (NRDS), infection and the inflammatory markers, anti infection treatment as well as NEC was abstracted from all recruited subjects. Unified training was conducted and responsible person was arranged for each collaborative hospitals for data collection, entry and verify. Results A total of 1 501 critical ill newborns from 5 hospitals from January 2013 to September 2014, including 435 cases of low birth weight premature, 814 cases of neonatal septicemia, 343 cases of NEC, 742 cases of invasive and non-invasive mechanical ventilation. The overall survival rate was 75.2% (1 129/1 501). According to birth weight, the survival rate was 50.0% (3/6), 56.5% (13/23), 65.4% (53/81), 74.9%(131/175) and 82.0(123/150) for the small preterm infants with birth weight < 750 g, -999 g, -1 249 g, -1 499 g, ≥1 500 g, respectively. According to the gestational age, the survival rate was 23.1% (3/13), 65.0% (13/20), 69.4% (26/36), 71.2% (52/73), 71.8% (61/85), 80.9%(89/110) and 81.6% (80/98) in the small preterm infants with gestational age < 27, 27-, 28-, 29-, 30-, 31- and ≥32 weeks. 315 cases (72.4%) received cerebral ultrasound examination, of them 45.3% (197 cases) was completed in 3 d after birth. 264 cases (60.7%) were treated with mechanical ventilation including 163 cases with invasive mechanical ventilation, 101 cases with only nCPAP. 217 cases (49.9%) were diagnosed as NRDS including 165 cases with PS treatment. 74/813 cases (9.1%) were treated with carbapenem antibiotic for more than 2 weeks. Survival rate did not significantly differed between the neonates treated with or without carbapenem antibiotics, 88.9% (16/18) vs 87.5%(42/48), χ2=0.024, P=1.000. Among 343 NEC neonates, 174 cases were diagnosed in 10 days of abdominal distension, only 44 cases had confirmed diagnostic significance among 313 cases taken abdominal X-ray examination. 742 cases (49.4%) received mechanical ventilation treatment, 180 cases with simple invasive ventilation and 562 cases with invasive ventilation. The overall complication rate significantly differed between the neonates treated with noninvasive mechanical ventilation and mechanical ventilation, 1.1%(2/180) vs 17.4%(98/562). Conclusion The critical neonatal collaboration network has positive significance for understanding and improving the level of diagnosis and treatment of critically ill newborns in China. The diagnosis and treatment of critical ill neonates and taking cerebral ultrasound examination in 3 days after birth, noninvasive ventilation and the practice of INSURE technology and other aspects need to be further strengthened.
Objective To quantitatively estimate the association between particulate matter with asthma in children. Methods PubMed, EMBASE, Ovid, Cochrane Library, CBM, CNKI and Wanfang database were searched up to November 2014, and additional studies were manual screened. Observational studies assessing the association between inhalable particulate matter（PM25，PM10） and risk of childhood asthma were included. The quality of the literatures was evaluated by the Newcastle Ottawa Scale and AHRQ. The adjusted effect sizes and corresponding 95% CI for asthma attack corresponding to a 10 μg·m-3 increment in exposure to inhalable particulate matter were investigated and conducted to identify the acute and chronic effects. Furthermore, subgroup analysis was conducted by the sizes of inhalable particulate matter. RevMan 5.3 and Stata 12.0 software were used to perform heterogeneity analysis and the test of publication bias. The pooled effect was conducted on the basis of effect model. Results Thirty-one studies were identified, including 10 cohort studies, 12 cross-sectional studies, 8 case-crossover studies and 2 time-series studies. ①Twenty-two literatures reported the chronic effects of exposure to inhalable particles on childhood asthma, which exhibited heterogeneity (P<0.001, I2=72%). The pooled effect sizes of odds ratio based on random effect model were 110 (95%CI: 1.03-1.17), which indicated that the incidence of pediatric asthma increased 10% by a weighted average of adjusted OR for a 10 μg·m-3 increase in inhalable particles. In subgroup analysis, the combined odds ratios of PM2.5 and PM10 were 1.08 (95%CI: 1.02-1.15) and 1.0 (95%CI: 1.01-1.20) respectively. ② Nine literatures reported the acute effects of exposure to inhalable particles on childhood asthma. The pooled effect sizes were 1.05 (95%CI: 1.02-1.08), which indicated that the incidence of pediatric asthma increased 5% by a weighted average of for a 10 μg·m-3 increment of adjusted OR in inhalable particles. In subgroup analysis, the combined OR of PM2.5 and PM10 corresponded to 1.06 (95%CI: 1.02-1.10) and 1.05 (95%CI: 1.02-1.08) respectively. ③The test of publication bias using Egger's regression method showed the absence of publication bias in reports of acute effects, and the presence in reports of chronic effects. Conclusion There is significant association between the level of PM 2.5, PM 10 and the risks of acute and chronic childhood asthma.
Objective To investigate the prognosis and clinical features of congenital heart disease (CHD) with airway anomalies in children. Methods Children with CHD hospitalized from Jan. 2012 to Dec. 2012 were underwent chest multidetector helical CT airway reconstruction and/or fiberobronchoscopy. Patients were divided into airway abnormal group and airway normal group, the former group was divided into airway indication subgroup and heart indication subgroup. Clinical data including clinical features, ultrasonic cardiogram, chest multidetector helical CT airway reconstruction, and fiberobronchoscopy inspection results were retrospectively analyzed. The treatment, outcome and prognosis of CHD patients with airway anomalies were evaluated. Results A total of 460 CHD cases were recruited, of them 195（42.4%） cases of CHD accompanied with airway anomalies. The types of airway anomalies included airway stenosis, tracheal bronchus, symmetrical bronchus and airway malacia. Among them, airway stenosis was the most common(152/195, 77.9%），mainly resulting from extrinsic compression by vascular malformation or enlarged heart (80.9%,123/151).The incidences of airway anomalies differed among different CHD (vascular rings, 84.2%; outlet obstructive constipation, 51.5%; cyanotic CHD, 49.1%; left-to-right shunt, 33.8%; P＜0.05). The proportion of CHD differed from airway indication subgroup and heart indication subgroup（P＜0.05），the ratio of left-to-right shunt was higher in airway indication subgroup（62.4% vs 20.3%），the ratio of cyanotic CHD was higher in heart indication subgroup (12.0% vs 514%). Airway abnormal group was more likely to result in wheezing and pneumonia≥3 times than airway normal group, and airway indication subgroup was more likely to result in cough, wheezing and repeated pneumonia≥3 times than heart indication subgroup. The total mortality rate was 18.2% (22/121). None patients in airway abnormal group underwent surgery.Nineteen cases with airway stenosis (4 with mild stenosis and 15 with moderate stenosis) underwent multidetector helical CT airway reconstruction. Six of them recovered 1 year later after cardiac surgery and 6 of them improved within 1 year after operation. No change was found in 5 cases while 2 cases were progressed without correction of cardiovascular malformations. Among airway abnormal group, nine cases died in hospitalization and 13 cases died during follow-up. Conclusion The morbidity and mortality of children with CHD and airway anomalies was high. Airway stenosis was the most common type of airway anomalies. It should be noticed that vascular rings and/or CHD patients who had repeatedly respiratory infection, wheeze, long-term pulmonary atelectasis or pulmonary consolidation of post operation and failure of ventilator weaning might be accompanied with airway anomalies. Conservative management was the first choice for mild and moderate airway stenosis. More than 1 year after release of extrinsic compression, airway stenosis could recover gradually in part of patients.
Objective To explore causative genes of nephrotic syndrome(NS) in children and its mutant characteristics. Methods All NS children in Children's Hospital of Fudan University from Jan. 1st, 2011 to Dec. 31th, 2013 were enrolled. The clinical data including medical records and follow-up information, and peripheral blood were collected. Firstly, NS was classified into familial and sporadic NS according to family history. Then sporadic NS was divided into congenital NS (CNS), infantile NS, early-child NS and late-child NS in accordance with the age of onset. Early- and late-child NS were divided into steroid-sensitive NS (SSNS) and steroid-resistant NS (SRNS) on the basis of initial sensitivity to glucocorticoid, and then SRNS was divided into initial- and late-resistant SRNS. NPHS1, NPHS2, PLCE1, LAMB2, LMX1B, COQ2 and WT1 genes were tested in patients with CNS by direct sequencing, while NPHS1, NPHS2, PLCE1 and WT1 were tested in patients with infantile NS. To early- and late-child NS, NPHS2 and WT1 were tested by direct sequencing, 42 common gene mutations in 8 main genes, like NPHS1 was tested by SnapShot analysis. Results Causative mutations in NPHS1 was found in 8 cases (80%), none mutations in NPHS2, PLCE1, LAMB2, LMX1B, COQ2, or WT1 gene was found in 10 CNS cases. 6 out of 12 cases with infantile NS had mutations, 3 of which was in WT1 gene, 2 in NPHS2 gene, and 1 in NPHS1 gene. In 132 cases of early-onset NS children, 8 cases (6.1%) with initial SRNS had gene mutations. WT1 gene mutation was found in 3 cases (3/32, 9.4). NPHS2 mutations were detected in 2 patients (2/32, 6.3%). NPHS1 mutations were identified in 2 patients (2/32, 6.3%). INF2 mutation was found in one case. No mutations were found in early-child NS children with late SRNS or SSNS. There were no causative mutations of tested genes detected among 84 cases of late-child NS children. Conclusion Candidate gene screening should be performed in congenital and infantile NS as well as in early-child NS children with initial SRNS. High frequency of mutations in NPHS1, NPHS2 and WT1 was found in children with infantile NS and early-child NS children with initial SRNS. NPHS1 is a common causative gene in this study. Mutational analysis is suggested in children with congenital NS. Routine gene screening is not suggested in children with late SRNS and SSNS.
Objective To study the baseline level of fecal calprotectin (FC) and the factors that may affect the FC levels in preterm newborns within 2 weeks of life. Methods Preterm newborns were recruited from March 2011 to May 2011 from Yueqing Hospital Affiliated to Wenzhou Medical University and The first affiliated Hospital of Nanjing Medical University. Neonates who were suffered from severe gastrointestinal disease and the other severe didease were excluded. Feces were collected in 2, 3, 4, 5, 6, 7, 14 days of life. FC was detected by ELISA. Results A total of 42 patients including 25 males were recruited with the median gestational age (GA) of 34.5(28-36.4) weeks and median birth weight (BW) of 2 200(930-3 200) g. ①The mean FC level in meconium was (84.7±15.8) μg·g-1, the mean FC levels in 3, 4, 5, 6, 7, 14 days of life were (76.5±12.4), (86.0±17.6), (77.5±17.4), (82.2±15.5), (84.3±16.1) and (80.3±19.6) μg·g-1, respectively. There was no significant difference of FC level among different days of life in preterm newborns. ② There was significant difference of meconium FC level between caesarean section and natural labor newborns. There was no evidence that the meconium FC was influenced by gender, GA and BW and that FC levels in 3, 4, 5, 6, 7 and 14 days of life were not influenced by age, gender, GA, BW or delivery mode in preterm newborns. Conclusion The FC levels within 2 weeks of life in the preterm newborns did not vary with age. The delivery mode could influence fecal FC.
Objective To analyze the etiology of community-acquired pneumonia in hospitalized children. Methods A retrospective descriptive study was undertaken among pediatric patients with community-acquired pneumonia admitted to Beijing Children's Hospital from December 2012 to November 2013.Clinical data including gender, age, hospitalization time, etiology and other data were collected. The etiology characteristics of these patients in different ages and seasons were analyzed. Results ①1 853 hospitalized children aged from 28 days to 18 years with community-acquired pneumonia were included,and 1 447(78.1%)patients were positive for at least one pathogen. The bacteria positive rate was 27.0%, and the most frequent detected bacteria was streptococcus pneumonia, followed by haemophilus influenza and klebsiella pneumonia. The virus positive rate was 22.5%, the most common detected virus was respiratory syncytial virus,followed by adenovirus. Mycoplasma pneumonia positive rate was 48.7%. Mixed infection rate was 23.0%. ②With the growth of the age, the proportion of cases with single bacterial or viral infection decreased sharply, the proportion of cases with mycoplasma pneumoniae infection increased significantly, and the proportion of cases with multiple pathogens increased. Streptococcus pneumoniae infection often occurred in cases under 3 years of age (75.9%). Haemophilus influenzae infection (75.0%) and klebsiella pneumonia infection (68.4%) were found commonly in cases under 1 year of age. Respiratory syncytial virus infection was much more common in infants under 1 year of age (76.2%), adenovirus infection was more common in children under 3 years of age (82.3%). ③Single bacterial infection occurred the most commonly in spring while single virus infection occurred predominantly in winter, and single mycoplasma pneumoniae infection occurred the most commonly in autumn. Mixed pathogen infection occurred the most commonly in winter and spring; non-confirmed pathogens infection occurred the most commonly in spring. Streptococcus pneumoniae infection was more common in winter and spring, and haemophilus influenza infection was more common in spring. Klebsiella pneumoiae infection often occured in winter and spring. Respiratory syncytial virus infection was much more common in winter, and adenovirus infection was more common in winter and spring. Conclusion Mycoplasma pneumoniae most commonly infects children older than 5 years. Instead, bacterial and virus often infect children under 1 year old. Bacterial and viral infections occurs commonly in winter，while mycoplasma pneumoniae infections often occurs in summer and autumn.
Objective To assess the relationship between angiotensin converting enzyme (ACE) gene polymorphism and pediatric idiopathic nephrotic syndrome (PNS). Methods Case-control studies searched from the database of PubMed, EMBASE, Wiley Online Library, Cochrane Library, Science Citation Index, Google Scholar, China National Knowledge Infrastucture, Wanfang Data, China Biology Medicine, China science and technology journal were recruited to summarize the association between ACE gene polymorphisms with PNS from January 2000 to May 2014. Meta-analysis of the frequency of D, I alleles and DD, DI, II genotypes between PNS group and the control group were performed by Stata 12.0 software. Results Fourteen literatures including 2 849 subjects (1 264 in PNS group and 1 585 in the control group) were recruited. ① The frequency of D allele in PNS group was higher than that in the control group （OR=1.277，95%CI 1.080-1.509，P=0.004）and the frequency of I allele in PNS group was lower than that in the control group (OR=0.811，95%CI 0.738-0.892，P＜0.001). ② The frequency of DD genotype in PNS group was higher than that in the control group (OR=0.746，95%CI 0.634-0.877，P＜0.001), the frequency of II genotype in PNS group was lower than that in the control group (OR=0.746，95%CI 0.634-0.877，P＜0.001) and there was no significant difference of the frequency of DI genotype between PNS and the control groups. ③ Six literatures did not meet Hardy-Weinberg equilibrium, but it did not bias the study by sensitive analysis. ④ The articles reported D allele and DD genotype showed significant heterogeneity. The control group and race could explain partial heterogeneity, but Hardy-Weinberg equilibrium was not the cause of heterogeneity. Conclusion The relation about DD gene and D allele with PNS was not indubitable. The strength of the association about ACE gene and PNS gene was possibly lower.
Objective To analyze the clinical manifestations and prognosis of cyclic vomiting syndrome plus (CVS+). Methods CVS+ children in Neurology Department and Digestive Department at Beijing Children's Hospital, Capital Medical College from Oct. 2011 to Feb.2014 were retrospectively analyzed. The clinical manifestations, genetics and prognosis were summarized. Results Five cases (4 females and 1 male) were enrolled, with the age of seeing doctors ranging from 5.8 to 10.4 years and the age of onset ranging from 2 to 7 years. Course of disease ranged from 2.4 to 6.7 years. ①Weight of 2 cases and height of 1 case was below the 3rd percentile. ②All 5 cases had recurrent vomiting as the first and main symptom, vomiting lasted for 2-8 days, interval ranged from 1 week to 6 months. All cases had additional neurology diseases or symptoms after 1-15 months of the first onset. One child had automotive nerve disorder, 1 child had anxiety and depression, 3 children had epilepsy and myopathy, 2 children had ataxia. All cases had maternal family history, including 4 cases for migraine, 3 for nystagmus, 2 for deaf and 3 for exercise intolerance. ③Two cases had metabolic acidosis during vomiting period, and recovered after vomiting remission. Two cases had elevated lactic and metabolic acidosis in vomiting period and interphase. Four cases had hypokalemia and hyponatremia during vomiting episode, and get well after remission. All cases had brain atrophy on brain MRI. One case had short T1 signal on bilateral basal ganglia. Another 1 case had long T2 signal on bilateral basal ganglia. And 1 case had long T2 signal on left occipital lobe. Four cases had epileptic discharge on inter-ictal phase. ④Four cases received mitochon-drial gene sequencing, 2 of them had 3243A>G mutation, mutation ratio was 65.6% and 34.0%, diagnosed with mitochondria encephalopathy with lactic acidosis and stroke like seizures. Two cases carried polymorphism 16519T>C. ⑤All cases received symp-tomatic treatment during vomiting phase. Three cases took antiepilepsy medicine orally. Four cases were followed up for 12-21 months, 2 of them still had episode vomiting, the other 2 cases had remission for 6 months. Three of them had mental retardation. Conclusion It is important to consider CVS+ if patients characterized by severe discrete episodes of intractable vomiting, and additional neurology disease. Severe discrete episodes of intractable vomiting maybe a special symptom of motochondrial disease.
Androgen insensitivity syndrome(AIS) is one of the most common diseases in 46,XY disorders of sex development. But its diagnostic criteria is not clear because of similar phenotype and changing with age, which makes it difficult to diagnose and distinguish with other diseases like 5α- reductase deficiency and Swyer syndrome. It‘s quite important for patients to consider the sex selection,?selection?and opportunity of surgery, tumor incidence and psychosexual health in foundation of accurate diagnosis and classification. So this article summarizes the history, clinical characteristics and classification, diagnosis and different diagnosis, identification of sex selection and gonad treatment in order to make reference for better diagnosis and identification of AIS.
Abstract Objective To study the clinical features in nephrotic syndrome with Duchenne muscular dystrophy, To explore the possible relationship between nephrotic syndrome and Duchenne muscular dystrophy. Methods The clinical features, renal pathology, gene diagnosis and the response to steroid of one NS patient combined with DMD were analyzed and smnmarized,who was hospitalized in Hunan Children’s Hospital in May 2014 . Results ① Clinical characteristics: The patient was a 10-year-old boy. There was no family history of similar diseases; his brother was 20 years old, healthy. The patient with nephrotic syndrome frequency relapse, accompanied by aspartate aminotransferase, alanine aminotransferase, creatine kinase, lactate dehydrogenase increased sharply; the renal pathology was mild mesangial proliferative glomerulonephritis. ②Genetic analysis: Duchenne -type Muscular Dystrophy with exon 52 of dystrophin gene deletion, Chromosome karyotype analysis was normal. Conclusion Nephrotic syndrome with Duchenne muscular dystrophy in this patient with refractory nephropathy, exon 52 of dystrophy gene deletion and the pathogenesis of NS has no direct contact, but it may be related to the frequent relapse of NS, which needs further study.
