Objective To develop reference ranges for plethysmographic parameters for Chinese neonates and to provide the basis for diagnosis or treatment of respiratory diseases and parameters setting of ventilators.Methods There were 406 infants who met the inclusion and exclusion criteria, aged 1-28 days, received pulmonary function tests (PFTs) which were performed in the whole body plethysmography to access plethysmographic functional residual capacity (FRCP), effective air way resistance (Reff) and their tidal breathing. After PFTs, data of 195 infants were excluded from analysis as their condition might disturb the normal respiration or failed to meet quality control standard. Finally, data of 211 infants without any respiratory diseases were enrolled in the analysis. According to their age, infants were grouped into 1-7 d, -14 d, -21 d and -28 d groups for comparison. Results For neonates, height and weight increased significantly with age (P=0.004, P<0.000 1). Values of FRCP, VT, and TEF25/50 raised with age, while values of sReff and Reff/kg declined. Values of TPTEF/TE, VPTEF/VE, FRCP/kg and VT/kg remained relatively constant with growth. The flow rate increased in step with the lung volume while small airways developed faster than big ones. Lung function of newborns was significantly associated with body length, weight, BMI and age, results of logistic regression and smoothed centiles of FRCP, VT, TEF25 indicated that body weight was the strongest predictor of lung function. Conclusion Newborns' lung volume and efficiency of respiratory increased with growth, whereas airway resistance declined with age. The current reference ranges of plethysmographic parameters can provide basis for parameters setting of ventilators and reference for evaluating respiratory development, diagnosing or treating respiratory diseases.
Objective To describe the patterns of fine motor development of both the affected and the contralateral upper limbs in children with spastic hemiplegia using nonlinear mixed effect model (NONMEM), so as to further characterize the patterns of the fine motor development in children with spastic hemiplegia.Methods From 2000 to 2012, patients with congenital spastic hemiplegia were consecutively enrolled from 8 rehabilitation centers and 6 special education schools in Shanghai. Their fine motor functions were assessed with the fine motor function measure scale (FMFM) for children with cerebral palsy (CP). Both the affected side and the contralateral side were assessed with FMFM, while the scores were analyzed respectively in the nonlinear mixed effect model. Two major parameters obtained from the model were used as clinical interpretations. The first parameter was 90% limits of fine motor function. The second was the age at which children were expected to achieve 90% limit of FMFM.Results A total of 536 children (360 male 67.2%, 284 right hemiplegia 53.0%, 252 left hemiplegia 47.0%) were enrolled in the study. The youngest child was 5 months old at first evaluation, the eldest 17.8 years old, and with average (3.4±3.2) years old. Altogether 792 pairs of FMFM scores were studied, 1.48 pairs for each child. The average age for all 792 pairs of assessments was (4.1±3.8) years old. 73 children completed twice evaluations, while 54 completed three times or more. Stable limit model was used to fit the fine motor function model of the contralateral limb, while decline model was used to fit the model of the affected limb. Both models fit successfully. FMFM maximum score of the contralateral limb was 73.4, significantly higher than that of the affected limb, 64.0. Age-90 was 2.7 years for the contralateral side and 3.3 years for the affected limb. After reaching the maximum, FMFM score of the affected limb began to decline and dropped 12% from the peak of 64.0 to 56.5.Conclusions The fine motor development limit of the affected limb was lower than that of the contralateral side. It took more time for the affected limb to reach its developmental limit and the score tended to drop after that. The training of fine motor functions for children with hemiplegia should be based on the different developmental features of both limbs.
Objective Helium-oxygen mixture (heliox) was suggested to be beneficial in preterm infants in previous studies, but the evidence was limited. The aim of the study was to assess the effectiveness of nasal intermittent positive pressure ventilation (NIPPV) with heliox (70% helium and 30% oxygen) on length of ventilation (LoV) and cardiopulmonary protection in preterm infants with RDS.Methods Infants <37 weeks of gestational age diagnosed as RDS and required ventilation support with NIPPV in the first hour after birth were eligible for the study. Infants were randomly assigned to be supported with heliox or 30% air-oxygen mixture groups during the first 3 hours of enrollment, followed by air-oxygen mixture until NIPPV was no longer needed. The main outcome was the LoV to achieve clinical stability. Analysis followed intention to treat principle.Results 36 infants (19 in the heliox and 17 in the control groups) were allocated to the treatment from December 2012 to May 2013. Heliox administration significantly decreased LoV in test group compared with the control group (39.29 hours vs 57.80 hours, P=0.02). Carbon dioxide elimination was better in the heliox group than that in the control group (10.39 mmHg vs 6.0 mmHg, P=0.03). Analysis of lung inflammation cytokines and myocardial injury markers showed no statistical significance between the two groups (P>0.05). LoV was significantly and positively correlated with IL-6 level at baseline (r=0.474, P=0.006). No side effects of hypothermia were found in the whole study.Conclusions Heliox delivered with NIPPV was safe and effective in reducing LoV and increasing carbon dioxide elimination, but seemed to be ineffective in attenuating lung inflammation and myocardial damage in preterm neonates with RDS.
Objective To explore the reliability and validity of Chinese version Autism Diagnostic Observation Schedule (ADOS), and discuss the clinical value and applicability of ADOS in China.Methods After developing the inclusion and exclusion criteria, 60 children were selected as subjects who were clinically diagnosed as autism (AD, 24), pervasive developmental disorder - not otherwise specified (PDDNOS, 22), other developmental disorders(10) and typical developmental children (4), and administered ADOS- Module 1. Spearman rank correlation coefficient was used to measure the homogeneity reliability (Cronbach's α coefficient)and test-retest reliability, Kruskal-Wallis and Bonferroni correction test to examine empirical validity of the Chinese version ADOS-Module 1. In addition, the agreement between clinical diagnosis and ADOS classification was compared respectively according to DSM-Ⅳ-TR and DSM-Ⅴ.Results The Chinese version ADOS-Module 1 was composed of 4 domains - A. communication; B. reciprocal social interaction; C. play and D. repetitive behaviors and restricted interests. The results were as follows: ①Homogeneity reliability: the Cronbach's α coefficient of the whole schedule was 0.93, 0.79 for A domain, 0.89 for B domain, 0.93 for A + B domains. ②Test-retest reliability: test-retest reliability coefficients were 0.84 for A domain, 0.92 for B domain, 0.89 for A+B domain, 0.91 for C domain, and 0.88 for D domain (All P＜0.01). ③Empirical validity: all of the differences in scores of A domain, B domain and the sum of A and B domains between autism group and 3 other groups were statistically significant (all P≤0.001). The differences of scores between PDDNOS group and 3 other groups were statistically significant (all P＜0.01). The scores of non-ASD group did not significantly differ from the typical group (P>0.05). ④The consistency of the clinical diagnoses and ADOS classifications: according to the DSM-Ⅳ-TR criteria, the consistency rate of the clinical diagnoses and ADOS classifications was 71.4%. According to the DSM-Ⅴ criteria, the rate was 91.1%, the sensitivity and specificity of ADOS-Module 1 was 96.5% and 61.5%, respectively.Conclusion The reliability and validity of Chinese version ADOS Module-1 is good that it will be helpful in improving early diagnosis of children with suspected autism spectrum disorders, with high clinical value in China.
Objective To investigate the effect of infection control strategies in three phases on the occurrence of ventilator-associated pneumonia (VAP) in a Chinese NICU, providing effective strategies for clinical practice.Methods The hospital was relocated at a new site from June 2008. Neonates who had been subjected to tracheal intubation-mechanical ventilation for ≥48h and hospitalized by the newborn nursery for ≥5 d were included in this study. Three types of infection controlling strategies were corresponding to three time phases: controlling based on experience for phase 1, from Feb 1, 2006 to Jan 1, 2007; single infection controlling strategy for phase 2, from Aug 1 2008 to Jul 31 2009; and comprehensive infection controlling strategy for phase 3, from Jan 1, 2010 to Dec 31, 2010. Related data were collected for comparing VAP incidence and pathogen distribution during three phases after a bundle of infection preventive measures were gradually implemented step by step.Results Totally 491 neonates were included and the overall incidence of VAP was 27.3 per 1 000 ventilator-days. The incidence of VAP was 48.8, 25.7 and 18.5 per 1 000 ventilator-days for the phase 1, 2 and 3, respectively. Gram-negative bacilli (63 strains, 95.5%) were the primary VAP microorganisms in all phases, and the dominant organisms isolated for VAP during three periods were Acinetobacter baumannii（43 strians，65.2%）, followed by Klebsiella pneumoniae（10 strains，15.2%）, Escherichia coli and Pseudomonas aeruginosa(4 strains，6.1% in each).Conclusion The improvement of NICU environment together with the adoption of the evidence-based multi-faceted VAP control program could effectively reduce the occurrence of VAP with long-term effects, but the incidence was still high compared with the developed countries. Further studies are needed to improve the infection control strategies.
Objective The aim of this study was to develop a reference percentile curves of BP measured by automated device from a representative sample of preschool children in shanghai.Methods 4 619 healthy preschool children from 2 to 6 years of age were consecutive enrolled from March 2006 to November 2010 and all these children were born and received regular physical examination in Shanghai Minhang Maternal and Child Health Hospital. BP and heart rate were measured on the right arm at the level of the heart after each child had rested in the sitting position for 5 min, using automated BP recorder (Model 45NEO-E6, Welch Allyn,USA). All measurements were performed in the clinic room of the hospital with the temperature between 20℃ and 25℃. Children were kept natural posture when BP was measured by trained pediatricians. Centile curves for SBP and DBP were drawn by sex using LMS method, the 5th,50th,90th ,95th,99th percentiles of SBP and DBP were compared with those from Japanese preschool children with the same age.Results BP increased linearly with age for both boys and girls. Generally, the means of SBP and DBP for girls showed slightly lower than the boys (female: 5.55 mmHg vs 2.05 mmHg; male: 6.62 mmHg vs 4.62 mmHg), respectively. The percentiles of BP from the current study were significantly different from the Japanese references. The SBP 95th percentile of current study was significantly lower than Japanese references for boys and girls by 6-11 mmHg. The DBP 95th percentiles of boys aged 2 were slightly lower than Japanese reference, however, they remained the same levels from 3-6 years old. The percentiles of girls at 2-3 years old were lower than the Japanese reference, and exceeded at 3-4 years old and kept slightly higher to age of 6 years. Compared with the data from the Japanese, children in our study were found to have a lower height during the age range for both sexes（male:1.16 cm, 3.16 cm,, 6.06 cm,7.54cm and 6.3cm;female:1.15, 2.27, 6.68, 8.23, and 7.33 cm, respectively).Conclusions This study provides the median, 90th and 95th percentile reference values of SBP and DBP based on a single-center large sample of children aged 2-6 years. These reference percentiles may be useful to primary child care providers for the early screening and prevention of hypertension in Chinese preschool.
Objective To evaluate whether there is mycobacterium tuberculosis specific effector T-cell spot counting difference in children with active TB and LTBI through using interferon-γ release assays (IGRAs) and whether it can be used to differentiate active tuberculosis（TB） from latent tuberculosis infection（LTBI） or not.Methods A total of 93 active TB cases (27 severe and 66 non-severe cases of tuberculosis) and 47 LTBI cases with positive T.SPOT.TB results but without anti-tuberculosis treatment were included, mycobacterium tuberculosis specific effector T-cell spot counts were analyzed in the two groups.Results The median of spot counts in active TB was significantly larger than that in LTBI, 84(6-710) vs 17(6-316)(P=0.000), significant difference was also seen between non-severe cases of TB and severe cases,99(6-710) vs 44(6-268) (P=0.011) , non-severe cases of TB and LTBI (P=0.000), while there was no significant difference between severe cases of TB and LTBI (P=0.084). There was a large range of overlapping of spot counts distribution between active TB and LTBI, non-severe cases of TB and LTBI, severe cases of TB and LTBI, non-severe and severe cases of TB. Receiver operator characteristic (ROC) curve analysis showed the best cut-off value was 43.5 for T.SPOT.TB in differentiating active TB from LTBI, the sensitivity and specificity were 69.9% and 70.2%, respectively. Conclusions Although mycobacterium tuberculosis specific effector T-cell spot counting in children with active TB especially in non-severe cases of TB is significantly larger than that in LTBI which reflects mycobacterium tuberculosis bacilli load to some extent, it cannot be used to differentiate active TB from LTBI in children.
Objective To investigate the feasibility of purification of human umbilical cord mesenchymal stem cell-derived cardiomyocytes by genetic modification, as a new source of stem cells for clinical myocardial cell transplantation.Methods First of all, the human cardiac myosin light chain (MLC-2v) gene promoter and puromycin resistance gene were fused by genetic modification, and then the human umbilical cord mesenchymal stem cells were transfected. Secondly, the MLC-2V-positive cells were purified by puromycin and then the cells were cultured. After that, the MLC-2v-positive cells were induced by 5-azacytidine and the expression of cardiac markers were detected.Results The expression of myocardial cell markers troponin I (troponin I), MLC-2v and desmin could be detected in the green fluorescent protein-expressing MLC-2v cells induced by 5-azacytidine. RT-PCR analysis showed that human umbilical cord mesenchymal stem cells expressed the mRNA of Nkx2.5 and desmin after transfection and induction, which were absence without transfection and induction.Conclusions Human umbilical cord mesenchymal stem cells can be induced to relatively purified cardiomyocyte-like cells by genetic modification, which will become the important source of cardiomyocytes for transplantation in patients.
Objective To discriminate the genotype of neonates with Prader-Willi syndrome (PWS) by using methylation specificity multiple connected probe amplification technology (MS - MLPA) and Affymetrix SNP chip, identify the correlation between genotype and phenotype in deletion subtypes.Methods 13 Prader-Willi syndrome neonates with confirmed diagnosis by molecular biological methods were included in the study. Among them 3 cases were tested by cytogenetic SNP chip for detection of pathogenic copy number variations(CNVs) and the rest were tested by multiplex ligation-dependent probe amplification (MLPA).Clinical features according to the diagnostic criteria were recorded.Results 5 individuals with type Ⅰ deletion(del Ⅰ), 8 with type Ⅱ deletion(del Ⅱ) were analyzed. Central hypotonia and poor suck were observed in all the patients. Characteristic facial feature was found in 4 neonates with del Ⅰ(80%) and in 4 neonates with del II (50%). Hypopigmentation was found in 2 with del Ⅰ (40%) and in 3 with del Ⅱ(37.5%). Hypogonadism was found in 2 with del Ⅰ (40%) and in 7 with del Ⅱ(87.5%).Conclusion Central hypotonia and poor suck were common in PWS neonates. Characteristic facial feature and premature birth may be more likely found in neonates with deletion Ⅰ, whereas high incidence of hypogonadism was found in deletion Ⅱ noenates. Type Ⅰ deletion may have various phenotypes.
Objective To investigate the clinical, genetic and pathologic features of Duchenne muscular dystrophy(DMD) infants.Methods The DMD infants were collected from June 2009 to March 2013 and diagnosed by DMD genetic testing and (or) muscle biopsy. Clinical, genetic and pathological data were retrospectively reviewed and followed up 3 to 41 months.Results In total, 43 cases of DMD infants were enrolled into the study, aged 1 to 34 (18.2±11.3) months at diagnosis. Among them, 21/43 cases (48.8%) and 13/43 cases (30.2%) showed elevated serum CK and transaminase levels respectively. 31/36 cases (86.1%) of DMD infants showed movement development delay. ①The serum CK, AST and ALT levels were significantly elevated. ②DMD gene frameshift deletion mutation was found in 29 cases (67.4%), non-frameshift deletion mutation in 2 cases (4.6%), simply duplications mutation in four cases (11.6%), duplications with coexisted deletion mutation in 1 case (2.3%), point mutations in 7 cases (16.3%). ③ 8/12 patients showed prompted myogenic EMG. 12 patients showed normal nerve conduction velocity. ④ Skeletal muscle biopsy showed variations in fiber size, a large number of opaque, necrotic and regenerated fibers and connective tissue hyperplasia. The muscle fiber membrane showed complete absence of dystrophin expression. Conclusions DMD infants have a lot of muscle fibers degeneration, necrosis. Early diagnosis and intervention are needed.
Objective To explore the clinical features and prognosis of children with Castleman's disease（CD）.Methods The clinical manifestations，laboratory results，pathological data，treatment and outcome of nine children with CD diagnosed in Peking Union Medical College Hospital from January 1990 to December 2011 were retrospectively analyzed.Results Of nine children，the age of patients at disease onset varied from 6 to 17.7 years. Time for diagnosis ranged from 3 weeks to 12 years.5 children were unicentric Castleman's disease（UCD）and manifested in the form of localized tumour that was generally asymptomatic, 4 children with UCD with detailed pathological data were all of hyaline-vascular type. One of the 5 cases was associated with paraneoplastic pemphigus（PNP）.Each of the 4 children without complications underwent a curative surgical resection with excellent prognosis. The other 4 children with multicentric Castleman's disease（MCD）presented obvious systemic symptoms（fever, splenomegaly，anemia, and hyperglobulinemia）, growth retardation in 2 cases. One child with MCD achieved remission after chemotherapy.The other 3 just underwent the surgical resection and remained clinical remission from 16-36 months.Conclusion CD rarely occurs in children, it has complicated clinical manifestations which are similar to that in adult patients. Pathological examination is essential for diagnosis and management.UCD without complications has a good prognosis，while UCD in association with PNP has a poor prognosis.The prognosis of MCD varies and seems to have a more favourable course in children than in adults.
Objective To investigate the relationship of inflammatory cytokines、coagulation function and pediatric critical illness score in children with severe pneumonia complicated with sepsis.Methods From January 2010 to November 2012, the children who stayed in PICU of Fujian Provincial Maternal and Children Hospital for more than 24 h and had been diagnosed as severe pneumonia complicated with sepsis were identified. According to the pediatric critical illness score (PCIS) criteria, three groups including extremely critical group (＜70 scores），critical group (70-80 scores） and non-critical group (＞80 scores）were recruited, and their inflammatory cytokines and coagulation function were measured at the same time.Results A total of 101 children comprising 47 males and 54 females were enrolled, 53 children were in non-critical group, 42 children in critical group and 6 children in extremely critical group. ① With the children's critical condition getting worse, interleukin-6, soluble P-selectin and D-dimer levels increased, platelet count level decreased, with significant differences among three groups (P<0.05). The C-reactive protein level increased with the decrease of PCIS, but with no significant difference between the extremely critical group and the critical group, but the levels were significantly lower than that in the non-critical group (18.3 vs 37.8 vs 46.9, P= 0.003). The white cell counts did not significantly differ from each other among three groups (P＞0.05). ② In this research, there was positive correlation between interleukin-6, soluble P-selectin, D-dimer and PCIS, negative correlation between platelet count and PCIS and no correlation between white blood cell leve
Objective To detect serum NSE and VILIP-1 levels in rats to provide the serum markers for early diagnosis of hypoxic-ischemic encephalopathy(HIE). Methods Rat HIE model was made and classified in three groups: Sham-operation, control and treatment group, with 48 rats in each group. Serum NSE and VILIP-1 levels were tested at 6 time points（1,6,12,24,48, and 72 h） by killing 4 rats for all groups (sham/sevo/drug groups) with ELISA. Levels of NSE and VILIP-1 at different time points were compared among groups by univariate ANOVA. Diagnostic value of serum NSE and VILIP-1 at 24 h to HIE (gold standard) were tested by ROC method.Results ①NSE levels elevated in sevo groups, the statistical differences were found at 12,24,48 h time points, and dropped in drug groups, statistical differences were found at 12,24,48,72 h time points. ②VILIP-1 increased in sevo groups, statistical differences were found in 12,24,48,72 h groups, and decreased in drug groups, statistical differences were found in 6,12,24 h groups. ③For NSE, the area under ROC curve was 0.649 (95％CI：0.511-0.898) and the sensitivity and specificity were 72.4% and 63.7% respectively when the diagnostic criteria was 1 630 mg·L-1. For VILIP-1, the area undery ROC curve was 0.840 (95％CI：0.622-0.931) and the sensitivity and specificity were 75.9% and 99.9% respectively when the diagnostic cretiria was 590 mg·L-1. With the combination of NSE and VILIP-1, the diagnosis of HIE would be more accurate, since the area under ROC curve was 0.862 （95％CI：0.641-0.944）and the positive prognosis value was 0.901 3. ④Vitamin E could decrease the serum NSE, VILIP-1 levels in rat with HIE, howerver no improvement of neurobehavior was observed. The expression of both NSE and VILIP-1 was reduced in drug groups than in sevo groups. Conclusions NSE and VILIP-1 increased in HIE. To diagnose HIE, both sensitivity and specificity were higher with VILIP-1 than NSE, and it would be more accurate to combine NSE with VILIP-1.
Objective: In order to find more effective treatment for a child with immunoglobulin resistant Kawasaki disease, we performed an evidence-based clinical practice. Method: We searched UpToDate（~2012.10）、Cochrane library (Issue 10, 2012)、pubmed(1978~2012.10)、China National Knowledge Infrastructure (CNKI, 1978~2012.10) for system reviews, randomized controlled trials concerning the effect of intravenous immunoglobulin (IVIG), glucocorticoid, or their combination treating immunoglobulin resistant Kawasaki disease and evaluated the clinical evidence quality with GRADEpro software. Rerults: There are evidence of high quality showing that low dose of glucocorticoid combined with IVIG is superior to IVIG alone for patients with immunoglobulin resistant Kawasaki disease in bringing down fevers and reducing coronary artery dialation. Based on the above evidence, clinical experience and the consent of the patient's parents, intravenous Methylprednisolone (IVMP, 2mg/kg) plus a third course of IVIG (1g/kg) was administered to the patient. Methylprednisolone was gradually withdrawed while Asprin and Dipyridamole were sustained. Follow-up showed the patient's temperature and laboratory test returned to normal gradually; Cardiac ultrasonography showed no progressing coronary artery injury in the following 6 months. Conclusion: Low dose of IVMP plus IVIG could be effective treating immunoglobulin resistant Kawasaki disease. Evaluation of its long term effect is requied.
The Appraisal of Guidelines for Research & Evaluation (AGREE) instrument has been the important standard for Clinical Practice Guideline appraisal, since released in 2003. The second version, AGREEⅡ was released in 2009. Some domestic researchers has translated and used the instruments. We want to explain each items of AGREE Ⅱ detailedly through appraising Chinese Clinical Practice Guidelines, hoping to provide reference for the domestic researchers understanding and using AGREEⅡ appropriately.
