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Vol 10, No 3
Vol 10, No 3 August 2014 ISSN 1708-8569
 
Review articles
Original articles
Brief report
Case reports
   
Review articles:
Dosage individualization in children: integration of pharmacometrics in clinical practice
  Wei Zhao, St¨¦phanie Leroux, Evelyne Jacqz-Aigrain
 
Dosage individualization in children: integration of pharmacometrics in clinical practice
 
Wei Zhao, St¨¦phanie Leroux, Evelyne Jacqz-Aigrain
Paris, France
 
Author Affiliations: Department of Clinical Pharmacy, School of Pharmaceutical Sciences, Shandong University, Jinan 250012, China (Zhao W); Department of Pediatric Pharmacology and Pharmacogenetics, Hôpital Robert Debr¨¦, APHP, Paris, France (Zhao W, Leroux S, Jacqz-Aigrain E); Clinical Investigation Center CIC1426, INSERM, Paris, France (Zhao W, Jacqz-Aigrain E); EA7323, Universit¨¦ Paris Diderot-Universit¨¦ Paris Descartes, Paris, France (Zhao W, Leroux S, Jacqz-Aigrain E); Division of Neonatology, Department of Child and Adolescent Medicine, CHU de Rennes, Rennes, France (Leroux S)
 
Corresponding Author: Wei Zhao, Pharm D, PhD, Department of Clinical Pharmacy, School of Pharmaceutical Sciences, Shandong University, Jinan 250012, China; Department of Pediatric Pharmacology and Pharmacogenetics, Hôpital Robert Debr¨¦, 48 Boulevard S¨¦rurier, 75935 Paris Cedex 19, France (Tel: 0033 140033656; Fax: 0033 140035779; Email: wei.zhao@rdb.aphp.fr)
 
doi: 10.1007/s12519-014-0493-x
 
Background: Children are in a continuous and dynamically changing state of growth and development. A thorough understanding of developmental pharmacokinetics (PK) and pharmacodynamics (PD) is required to optimize drug therapy in children.
 
Data sources: Based on recent publications and the experience of our group, we present an outline on integrating pharmacometrics in pediatric clinical practice to develop evidence-based personalized pharmacotherapy.
 
Results: Antibiotics in septic neonates and immunosu-ppressants in pediatric transplant recipients are provided as proof-of-concept to demonstrate the utility of pharmacometrics in clinical practice. Dosage individualization based on developmental PK-PD model has potential benefits of improving the efficacy and safety of drug therapy in children.
 
Conclusion: The pharmacometric technique should be better developed and used in clinical practice to personalize drug therapy in children in order to decrease variability of drug exposure and associated risks of overdose or underdose.
 
World J Pediatr 2014;10(3):197-203
 
Key words: developmental pharmacokinetics;
                    developmental pharmacology;
                    pediatric pharmacology;
                    personalized therapy;
                    pharmacometrics;
                    quantitative pharmacology
  [Abstract] [Full Text] [PDF]  
The role of surfactant and non-invasive mechanical ventilation in early management of respiratory distress syndrome in premature infants
  Narayan Prabhu Iyer, Maroun Jean Mhanna
 
The role of surfactant and non-invasive mechanical ventilation in early management of respiratory distress syndrome in premature infants
 
Narayan Prabhu Iyer, Maroun Jean Mhanna
Cleveland, Ohio, USA
 
Author Affiliations: Department of Pediatrics, Division of Neonatology, Case Western Reserve University School of Medicine, MetroHealth Medical Center, Cleveland, Ohio 44109, USA (Iyer NP, Mhanna MJ)
 
Corresponding Author: Maroun Jean Mhanna, MD, MPH, Department of Pediatrics, Metro Health Medical Center, 2500 Metro Health Drive, Cleveland, OH 44109, USA (Tel: 216-778-1346; Fax: 216-778-4223; Email: mmhanna@metrohealth.org)
 
doi: 10.1007/s12519-014-0494-9
 
Background: Surfactant replacement therapy has been used for few decades for the treatment of respiratory distress syndrome (RDS) and has significantly improved morbidity and mortality in premature infants. Non-invasive respiratory support has recently emerged as a strategy in the early management of RDS. In this review, we discuss the different strategies of early management of RDS.
 
Data sources: A literature search of PubMed database was conducted to review the subject. The quality of evidence of key clinical studies was graded according to a modified grading system of the international GRADE group.
 
Results: Continuous positive airway pressure (CPAP) with selective surfactant is a safe alternative to routine intubation, surfactant and mechanical ventilation in preterm infants with spontaneous breathing, and such an approach has been associated with decreased risk of death and bronchopulmonary dysplasia. There is a risk of pneumothorax when using a high pressure of CPAP (¡Ý8 cm of H2O), a high partial pressure of carbon dioxide (PCO2 >75 mm of Hg), and a high fraction of inspired oxygen (FiO2 >0.6) as a threshold for intubation while on  CPAP.
Conclusion: Not all preterm infants need surfactant treatment, and non-invasive respiratory support is a safe and effective approach.
 
World J Pediatr 2014;10(3):204-210
 
Key words: non-invasive mechanical ventilation;
                    prematurity;
                    respiratory distress syndrome
  [Abstract] [Full Text] [PDF]  
Bilirubin nomograms for identification of neonatal hyperbilirubinemia in healthy term and late-preterm infants: a systematic review and meta-analysis
  Zhang-Bin Yu, Shu-Ping Han, Chao Chen
   
Bilirubin nomograms for identification of neonatal hyperbilirubinemia in healthy term and late-preterm infants: a systematic review and meta-analysis
 
Zhang-Bin Yu, Shu-Ping Han, Chao Chen
Nanjing, China
 
Author Affiliations: Department of Pediatrics, Nanjing Maternity and Child Health Care Hospital of Nanjing Medical University, No. 123 Tian Fei Xiang, Mo Chou Road, Nanjing 210004, China (Yu ZB, Han SP); Department of Neonatology, Children's Hospital of Fudan University, No. 399 Wanyuan Road, Minhang District, Shanghai 201102, China (Chen C)
 
Corresponding Author: Shu-Ping Han, Department of Pediatrics, Nanjing Maternity and Child Health Care Hospital of Nanjing Medical University, No. 123 Tian Fei Xiang, Mo Chou Road, Nanjing 210004, China (Tel: +86-025-52226561; Fax: +86-025-52226561; Email: shupinghan@njmu.edu.cn)
 
doi: 10.1007/s12519-014-0495-8
 
Background: Hyperbilirubinemia occurs in most healthy term and late-preterm infants, and must be monitored to identify those who might develop severe hyperbilirubinemia. Total serum bilirubin (TSB) or transcutaneous bilirubin (TcB) nomograms have been developed and validated to identify neonatal hyperbilirubinemia. This study aimed to review previously published studies and compare the TcB nomograms with the TSB nomogram, and to determine if the former has the same predictive value for significant hyperbilirubinemia as TSB nomogram does.
 
Methods: A predefined search strategy and inclusion criteria were set up. We selected studies assessing the predictive ability of TSB/TcB nomograms to identify significant hyperbilirubinemia in healthy term and late-preterm infants. Two independent reviewers assessed the quality and extracted the data from the included studies. Meta-Disc 1.4 analysis software was used to calculate the pooled sensitivity, specificity, and positive likelihood ratio of TcB/TSB nomograms. A pooled summary of the receiver operating characteristic of the TcB/TSB nomograms was created.
 
Results: After screening 187 publications from electronic database searches and reference lists of eligible articles, we included 14 studies in the systematic review and meta-analysis. Eleven studies were of medium methodological quality. The remaining three studies were of low methodological quality. Seven studies evaluated the TcB nomograms, and seven studies assessed TSB nomograms. There were no differences between the predictive abilities of the TSB and TcB nomograms (the pooled area under curve was 0.819 vs. 0.817).
 
Conclusions: This study showed that TcB nomograms had the same predictive value as TSB nomograms, both of which could be used to identify subsequent significant hyperbilirubinemia. But this result should be interpreted cautiously because some methodological limitations of these included studies were identified in this review.
 
World J Pediatr 2014;10(3):211-218
 
Key words: hyperbilirubinemia;
                    newborn;
                    systematic review;
                    transcutaneous bilirubin
  [Abstract] [Full Text] [PDF]  
Original articles:
QDPR gene mutation and clinical follow-up in Chinese patients with dihydropteridine reductase deficiency
  De-Yun Lu, Jun Ye, Lian-Shu Han, Wen-Juan Qiu, Hui-Wen Zhang, Jian-De Zhou, Pei-Zhong Bao, Ya-Fen Zhang, Xue-Fan Gu
 
QDPR gene mutation and clinical follow-up in Chinese patients with dihydropteridine reductase deficiency
 
De-Yun Lu, Jun Ye, Lian-Shu Han, Wen-Juan Qiu, Hui-Wen Zhang, Jian-De Zhou, Pei-Zhong Bao, Ya-Fen Zhang, Xue-Fan Gu
Shanghai, China
 
Author Affiliations: Department of Pediatric Endocrinology and Genetic Metabolism and Shanghai Institute for Pediatric Research, Xinhua Hospital, Shanghai Jiaotong University School of Medicine, Shanghai 200092, China (Lu DY, Ye J, Han LS, Qiu WJ, Zhang HW, Zhou JD, Bao PZ, Zhang YF, Gu XF)
 
Corresponding Author: Jun Ye, MD, Shanghai Institute for Pediatric Research, Xinhua Hospital, Shanghai Jiaotong University School of Medicine, No.1665 Kong Jiang Road, Shanghai 200092, China (Tel: 86-21-25076455; Fax: 86-21-65791316; Email: yejun2314@163.com)
 
doi: 10.1007/s12519-014-0496-7
 
Background: This study aimed to investigate the mutation spectrum of the QDPR gene, to determine the effect of mutations on dihydropteridine reductase (DHPR) structure/function, to discuss the potential genotype-phenotype correlation, and to evaluate the clinical outcome of Chinese patients after treatment.
 
Methods: Nine DHPR-deficient patients were enrolled in this study and seven of them underwent neonatal screening. QDPR gene mutations were analyzed and confirmed by routine methods. The potential pathogenicity of missense variants was analyzed using Clustal X, PolyPhen program and Swiss-PDB Viewer 4.04_OSX software, respectively. The clinical outcomes of the patients were evaluated after long-term treatment.
 
Results: In 10 mutations of the 9 patients, 4 were novel mutations (G20V, V86D, G130S and A175R), 4 were reported by us previously, and 2 known mutations were identified. R221X was a hotspot mutation (27.7%) in our patients. Eight missense mutations probably had damage to protein. Six patients in this series were treated with a good control of phenylalanine level. The height and weight of the patients were normal at the age of 4 months to 7.5 years. Four patients, who underwent a neonatal screening and were treated early, showed a normal mental development. In 2 patients diagnosed late, neurological symptoms were significantly improved.
 
Conclusions: The mutation spectrum of the QDPR gene is different in the Chinese population. Most mutations are related to severe phenotype. The determination of DHPR activity should be performed in patients with hyperphenylalaninemia. DHPR-deficient patients who were treated below the age of 2 months may have a near normal mental development.
 
World J Pediatr 2014;10(3):219-226
 
Key words: dihydropteridine reductase;
                    hyperphenylalaninaemia;
                    tetrahydrobiopterin
  [Abstract] [Full Text] [PDF]  
No relationship between mode of delivery and neonatal mortality and neurodevelopment in very low birth weight infants aged two years
  Jia-Jun Zhu, Ying-Ying Bao, Guo-Lian Zhang, Li-Xin Ma, Ming-Yuan Wu
 
No relationship between mode of delivery and neonatal mortality and neurodevelopment in very low birth weight infants aged two years
 
Jia-Jun Zhu, Ying-Ying Bao, Guo-Lian Zhang, Li-Xin Ma, Ming-Yuan Wu
Hangzhou, China
 
Author Affiliations: Department of Neonatology, Women's Hospital, Zhejiang University School of Medicine, Hangzhou 310006, China (Zhu JJ, Bao YY, Zhang GL, Ma LX, Wu MY)
 
Corresponding Author: Ming-Yuan Wu, Women's Hospital, Zhejiang University School of Medicine, 1 Xueshi Road, Hangzhou 310003, China (Tel: +86-571-89992350; Email: wumy@zju.edu.cn)
 
doi: 10.1007/s12519-014-0497-6
 
Background: To compare neonatal mortality and neurodevelopmental outcomes at two years of age in very low birth weight infants (¡Ü1500 g) born by cesarean with those by vaginal delivery.
 
Methods: In this retrospective, case-control study, we evaluated neonatal mortality, medical conditions and neurodevelopmental outcomes at two years of corrected age in 710 very low birth weight (VLBW) infants born between January 2005 and December 2010. Of the 710 infants, 351 were born by the cesarean and 359/710 by vaginal route.
 
Results: There were no significant differences in neonatal mortality between the cesarean delivery group and vaginal delivery group [56/351 (15.9%) vs. 71/359 (19.8%), P=0.20]. VLBW infants delivered by the cesarean procedure had a higher incidence of respiratory distress syndrome than those born by the vaginal route [221/351 (63.0%) vs. 178/359 (49.6%), P<0.001]. There were no differences in other neonatal morbidities, including intraventricular hemorrhage [126/351 (35.9%) vs. 134/359 (37.3%), P=0.69], bronchopulmonary dysplasia [39/351 (11%) vs. 31/359 (8.6%), P=0.38] and necrotising enterocolitis [40/351 (11.4%) vs. 32/359 (8.9%), P=0.32] between the two groups. The incidence of poor neurodevelopment after cesarean delivery was similar to that after vaginal delivery [105/351 (29.9) vs. 104/359 (29.0%), P=0.78].
 
Conclusions: In neither neurodevelopment nor neonatal mortality did cesarean birth offered significant advantages to VLBW infants. Moreover, the operation might be associated with an increased risk of respiratory distress syndrome for VLBW infants. The mode of delivery of VLBW infants should be largely based on obstetric indications and maternal considerations rather than perceived better outcomes for the neonate.
 
World J Pediatr 2014;10(3):227-231
 
Key words: cesarean delivery;
                    neonatal mortality;
                    neurodevelopment outcomes;
                    vaginal delivery;
                    very low birth weight
  [Abstract] [Full Text] [PDF]  
Effect of pravastatin on endothelial dysfunction in children with medium to giant coronary aneurysms due to Kawasaki disease
  Chao Duan, Zhong-Dong Du, Yu Wang, Li-Qun Jia
 
Effect of pravastatin on endothelial dysfunction in children with medium to giant coronary aneurysms due to Kawasaki disease
 
Chao Duan, Zhong-Dong Du, Yu Wang, Li-Qun Jia
Beijing, China
 
Author Affiliations: Department of Hematology/Oncology, Beijing Children's Hospital, Capital Medical University, Beijing 100045, China (Duan C); Department of Cardiology, Beijing Children's Hospital, Capital Medical University, Beijing 100045, China (Du ZD); Department of Ultrasonography, Beijing Children's Hospital, Capital Medical University, Beijing 100045, China (Wang Y, Jia LQ)
 
Corresponding Author: Zhong-Dong Du, MD, PhD, Department of cardiology, Beijing Children's Hospital, Nan Li Shi Road, Beijing 100045, China (Tel: 86-10-59616643; Fax: +86-10-59616643; Email: zhongdong_du2012@126.com)
 
doi: 10.1007/s12519-014-0498-5
 
Background: Ongoing low-grade inflammation and endothelial dysfunction persist in children with coronary lesions diagnosed with Kawasaki disease (KD). Statins, frequently used in the management of high cholesterol, have also shown to improve surrogate markers of inflammation and endothelial dysfunction. This study was undertaken to investigate the efficacy and safety of pravastatin in children with coronary artery aneurysms due to KD.
 
Methods: The study enrolled 14 healthy children and 13 male children, aged 2-10 years, with medium-to-giant coronary aneurysms for at least 12 months after the onset of KD. Pravastatin was given orally to the KD group at a dose of 5 mg/day for children under 5 and 10 mg/day for children older than 5 years. To determine the effects of pravastatin on endothelial function, high-frequency ultrasound was performed before the start of the study and 6 months after pravastatin therapy. The parameters measured were brachial artery flow-mediated dilation (FMD), non-flow mediated dilation (NMD), and carotid artery stiffness index (SI). High sensitive C-reactive protein (hs-CRP) levels, the circulating endothelial progenitor cells (EPCs) number, and serum lipid profiles were also determined at baseline and after 6 months of pravastatin treatment.
 
Results: Before treatment, the KD group had significantly decreased FMD (P<0.05) and increased SI and hs-CRP levels (P<0.05) compared with controls. After 6 months of pravastatin therapy, FMD improved significantly compared to the baseline KD group (3.16¡À6.49 to 10.05¡À7.74, P<0.05), but remained significantly less than that in the control group with no significant changes in NMD and SI. There were significant decreases in markers of inflammation after treatment. The hs-CRP levels decreased significantly from 2.93¡À0.81 mmol/L to 2.14¡À0.82 mmol/L (P<0.05) and the serum apo-B and apo-B/apo-A1 ratio were also reduced (P<0.05) in the KD group. However, the circulating EPC number was not significantly different between baseline and that following pravastatin treatment in the KD group and the control group (P>0.05). No significant complications were noted with paravastatin therapy.
 
Conclusions: Pravastatin improves endothelial function and reduces low-grade chronic inflammation in patients with coronary aneurysms due to KD. Children with coronary aneurysms due to KD may benefit from statin therapy.
 
                                                                                                                                                          World J Pediatr 2014;10(3):232-237
 
Key words: coronary aneurysm;
                    endothelial function;
                    endothelial progenitor cells;
                    Kawasaki disease;
                    statins
  [Abstract] [Full Text] [PDF]  
Congenital duodenal obstruction in neonates: a decade's experience from one center
  Qing-Jiang Chen, Zhi-Gang Gao, Jin-Fa Tou, Yun-Zhong Qian, Min-Ju Li, Qi-Xing Xiong, Qiang Shu
 
Congenital duodenal obstruction in neonates: a decade's experience from one center
 
Qing-Jiang Chen, Zhi-Gang Gao, Jin-Fa Tou, Yun-Zhong Qian, Min-Ju Li, Qi-Xing Xiong, Qiang Shu
Hangzhou, China
 
Author Affiliations: Department of Pediatric General Surgery (Chen QJ, Gao ZG, Tou JF, Qian YZ, Li MJ, Xiong QX), and Department of Pediatric Thoracic Surgery, Children's Hospital, Zhejiang University Shool of Medicine, Hangzhou 310003, China (Shu Q)
 
Corresponding Author: Qiang Shu, Department of Pediatric Thoracic Surgery, Children's Hospital, Zhejiang University Shool of Medicine, 57 Zhugan Xiang, Hangzhou 310003, China (Tel: +86-571-87033296; Fax: +86-571-87033296; Email: shuqiang@zju.edu.cn)
 
doi: 10.1007/s12519-014-0499-4
 
Background: Congenital duodenal obstruction (CDO) is one of the most common anomalies in newborns, and accounting for nearly half of all cases of neonatal intestinal obstruction. This study aimed to review our single-center experience in managing congenital duodenal obstruction while evaluate the outcomes.
 
Methods: We conducted a retrospective analysis of the records of all neonates dianogsed with congenital duodenal obstruction admitted to our center between January 2003 and December 2012. We analyzed demographic criteria, clinical manifestations, associated anomalies, radiologic findings, surgical methods, postoperative complications, and final outcomes.
 
Results: The study comprised 287 newborns (193 boys and 94 girls). Birth weight ranged from 950 g to 4850 g. Fifty-three patients were born prematurely between 28 and 36 weeks' gestation. Malrotation was diagnosed in 174 patients, annular pancreas in 66, duodenal web in 55, duodenal atresia or stenosis in 9, preduodenal portal vein in 2, and congenital band compression in 1. Twenty patients had various combinations of these conditions. Presenting symptoms included bilious vomiting, dehydration, and weight loss. X-rays of the upper abdomen demonstrated the presence of a typical double-bubble sign or air-fluid levels in 68.64% of patients, and confirmatory upper and/or lower gastrointestinal contrast studies were obtained in 64.11%. Multiple associated abnormalities were observed in 50.52% of the patients. Various surgical approaches were used, including Ladd's procedure, duodenoplasty, duodenoduodenostomy, duodenojejunostomy, or a combination of these. Seventeen patients died postoperatively and 14 required re-operation.
 
Conclusions: Congenital duodenal obstruction is a complex entity with various etiologies and often includes multiple concomitant disorders. Timely diagnosis and aggressive surgery are key to improving prognosis. Care should be taken to address all of the causes of duodenal obstruction and/or associated alimentary tract anomalies during surgery.
 
World J Pediatr 2014;10(3):238-244
 
Key words: congenital;
                    duodenal obstruction;
                    neonate
  [Abstract] [Full Text] [PDF]  
Somatic growth of lean children: the potential role of sleep
  Yan-Rui Jiang, Karen Spruyt, Wen-Juan Chen, Xiao-Ming Shen, Fan Jiang
 
Somatic growth of lean children: the potential role of sleep
 
Yan-Rui Jiang, Karen Spruyt, Wen-Juan Chen, Xiao-Ming Shen, Fan Jiang
Shanghai, China
 
Author Affiliations: Department of Developmental and Behavioral Pediatrics, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China and MOE-Shanghai Key Laboratory of Children's Environmental Health (Jiang YR, Spruyt K, Chen WJ, Jiang F); Shanghai Institute for Pediatric Research, Xinhua Hospital, Shanghai Jiaotong University School of Medicine and MOE-Shanghai Key Laboratory of Children's Environmental Health, Shanghai, China (Shen XM)
 
Corresponding Author: Fan Jiang, Department of Developmental and Behavioral Pediatrics, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, 1678 Dongfang Rd. Shanghai 200127, China (Tel: 86-21-38626012; Fax: 86-21-58706129; Email: fanjiang@shsmu.edu.cn)
 
doi: 10.1007/s12519-014-0500-2
 
Background: Despite the current obesity pandemic, childhood malnutrition remains an urgent, public health concern. Similar to the obesity pandemic, childhood malnutrition is influenced by genetic and a number of social, environmental and biological factors. In this study, we investigated the association between sleep duration and somatic growth in lean children.
 
Methods: A stratied, randomly clustered sampling design was used to select fifth grade students from 10 primary schools in Shanghai. Based on a body mass index below the 15th percentile a subsample of 143 lean children aged 10-11 years old was defined. Sleep duration and other potential confounders were surveyed through parental or self-report questionnaires. Body measurements were collected and used to calculate the Z score of weight, height, body mass index as well as body fat percentage.
 
Results: Compared with children who slept <9 hours, those who slept for ¡Ý10 hours grew taller and gained more weight after adjusting for confounding factors. When children slept 9-10 hours, they had significantly higher Z score of weight and body mass index.
 
Conclusions: Prolonged sleep not only benefits weight gain but also improves height in lean children. Our findings might provide important public health advice such that prolonged sleep may be an effective modifier of nutritional problems in childhood.
 
World J Pediatr 2014;10(3):245-250
 
Key words: height;
                    lean children;
                    sleep;
                    somatic growth;
                    weight
  [Abstract] [Full Text] [PDF]  
Improved outcomes of transported neonates in Beijing: the impact of strategic changes in perinatal and regional neonatal transport network services
  Xiang-Yong Kong, Xiu-Xiang Liu, Xiao-Yang Hong, Jing Liu, Qiu-Ping Li, Zhi-Chun Feng
 
Improved outcomes of transported neonates in Beijing: the impact of strategic changes in perinatal and regional neonatal transport network services
 
Xiang-Yong Kong, Xiu-Xiang Liu, Xiao-Yang Hong, Jing Liu, Qiu-Ping Li, Zhi-Chun Feng
Beijing, China
 
Author Affiliations: Newborn Care Center, Bayi Children's Hospital, the Military General Hospital of Beijing, the People's Liberation Army, Beijing 100700, China (Kong XY, Hong XY, Liu J, Li QP, Feng ZC); Department of Pediatrics, Hospital of Binzhou Medical University, Binzhou, Shandong, China (Liu XX)
 
Corresponding Author: Zhi-Chun Feng, MD, PhD, Bayi Children's Hospital, the Military General Hospital of Beijing, the People's Liberation Army, 5 Nanmen Chang, Dongcheng District, Beijing 100700, China (Tel: +86-10-64075199; Fax: +86-10-66721786; Email: zhjfengzc@126.com)
 
doi: 10.1007/s12519-014-0501-1
 
Background: Infants born outside perinatal centers may have compromised outcomes due to the transfer speed and efficiency to an appropriate tertiary center. This study aimed to evaluate the impact of regional coordinated changes in perinatal supports and retrieval services on the outcome of transported neonates in Beijing, China.
 
Methods: Information about transported newborns between phase 1 (July 1, 2004 to June 30, 2006) and phase 2 (July 1, 2007 to June 30, 2009) was collected. The strategic changes during phase 2 included standardized neonatal transport procedures, skilled attendants, a perinatal consulting service, and preferential admission of transported neonates to the intensive care unit of the tertiary care center. Data from phase 2 (after- strategic changes) were compared with those of phase 1 (the period of pre-strategic changes) after a 12-month washout period, especially regarding the reduction in mortality and selected morbidity.
 
Results: There was a large increase in the number of transported infants in phase 2 compared with phase 1 (2797 vs. 567 patients). The average monthly rate of increase of transported infants was 383.3% (from 24 infants per month to 116 infants per month). The mortality rate of transported neonates reduced significantly from phase 1 to phase 2 (5.11% vs. 2.82%; P=0.005), particularly for preterm infants (8.47% vs. 4.34%; P=0.006). In addition, transported neonates during phase 2 had significantly decreased morbidities.
 
Conclusions: Regional coordinated strategies optimizing the perinatal services and transport of outborn sick and preterm infants to tertiary care centers improved survival outcomes considerably. These findings have vital implications for health outcomes and resource planning.
 
World J Pediatr 2014;10(3):251-255
 
Key words: morbidity;
                    mortality;
                    neonatal transport network;
                    outcome
  [Abstract] [Full Text] [PDF]  
Declining age of puberty of school girls in southern Thailand
  Somchit Jaruratanasirikul, Atchariya Chanpong, Nuttaporn Tassanakijpanich, Hutcha Sriplung
 
Declining age of puberty of school girls in southern Thailand
 
Somchit Jaruratanasirikul, Atchariya Chanpong, Nuttaporn Tassanakijpanich, Hutcha Sriplung
Songkhla, Thailand
 
Author Affiliations: Department of Pediatrics (Jaruratanasirikul S, Chanpong A, Tassanakijpanich N); Epidemiology Unit, Faculty of Medicine, Prince of Songkla University, Hat-Yai, Songkhla 90110, Thailand (Sriplung H)
 
Corresponding Author: Somchit Jaruratanasirikul, MD, Department of Pediatrics, Faculty of Medicine, Prince of Songkla University, Hat-Yai, Songkhla 90110, Thailand (Tel: 66-074-429618; Fax: 66-074-429618; Email: somchit.j@psu.ac.th)
 
doi: 10.1007/s12519-014-0472-2
 
 
Background: Declines in the onset age of secondary sexual development have been reported in a number of recent studies in western and Asian countries. A study of Hat-Yai school girls in 1994 in Thailand showed that the ages at thelarche and menarche were 9.9 and 12.4 years, respectively. This study was to determine whether there was a decline in the average age of puberty in Hat-Yai school girls from 1994 to 2012.
 
Methods: A cross-sectional study was conducted in 2140 healthy Hat-Yai school girls, aged 6-18 years, from June 2011 to March 2012. Breast and pubic hair development was assessed by the Tanner staging method. Probit analysis was used to calculate the median ages of different stages of breast and pubic hair development, and the age of menarche.
 
Results: The median ages of girls having thelarche and menarche were 9.6 (95% CI, 9.4-9.8) and 12.2 (95% CI, 11.9-12.4) years, which declined from 9.9 (95% CI, 9.7-10.1) and 12.4 years (95% CI, 12.2-12.6) in the 1994 study respectively, however, without statistical significance. The youngest ages of girls who had breast stage 2 and menarche were 7.2 and 9.2 years, respectively. The final height at age 14 years in this cohort (157.3 cm) was 2.3 cm greater than that of girls in 1994 (155.0 cm).
 
Conclusions: From 1994 to 2012, the age at onset of thelarche and menarche of girls in Hat-Yai municipality declined by 0.2 and 0.3 years, respectively. The earlier age at onset of puberty had no effect on final adult height.
 
World J Pediatr 2014;10(3):256-261
 
Key words: growth;
                    menarche;
                    puberty;
                    secular trend;
                    thelarche
  [Abstract] [Full Text] [PDF]  
Placental growth factor expression is reversed by anti-vascular endothelial growth factor therapy under hypoxic conditions
  Ai-Yi Zhou, Yu-Jing Bai, Min Zhao, Wen-Zhen Yu, Lv-Zhen Huang, Xiao-Xin Li
 
Placental growth factor expression is reversed by anti-vascular endothelial growth factor therapy under hypoxic conditions
 
Ai-Yi Zhou, Yu-Jing Bai, Min Zhao, Wen-Zhen Yu, Lv-Zhen Huang, Xiao-Xin Li
Beijing, China
 
Author Affiliations: Department of Ophthalmology, the Second Affiliated Hospital of Medical College of Xi'an Jiaotong University, Xi'an 710004, Shaanxi Province, China (Zhou AY); Key Laboratory of Vision Loss and Restoration, Ministry of Education, Department of Ophthalmology, People's Hospital, Peking University, Beijing, China (Zhou AY, Bai YJ, Zhao M, Yu WZ, Huang LZ, Li XX)
 
Corresponding Author: Xiao-Xin Li, MD, Key Laboratory of Vision Loss and Restoration, Ministry of Education, Department of Ophthalmology, People's Hospital, Peking University, Xizhimen South Street 11, Xi Cheng District, Beijing 100044, China (Tel: 010-88325413; Fax: 010-88325413; Email: drlixiaoxin@163.com)
 
doi: 10.1007/s12519-014-0502-0
 
Background: Clinical trials have revealed that the anti-vascular endothelial growth factor (VEGF) therapies are effective in retinopathy of prematurity (ROP). But the low level of VEGF was necessary as a survival signal in healthy conditions, and endogenous placental growth factor (PIGF) is redundant for development. The purpose of this study was to elucidate the PIGF expression under hypoxia as well as the influence of anti-VEGF therapy on PIGF.
 
Methods: CoCl2-induced hypoxic human umbilical vein endothelial cells (HUVECs) were used for an in vitro study, and oxygen-induced retinopathy (OIR) mice models were used for an in vivo study. The expression patterns of PIGF under hypoxic conditions and the influence of anti-VEGF therapy on PIGF were evaluated by quantitative reverse transcription-polymerase chain reaction (RT-PCR). The retinal avascular areas and neovascularization (NV) areas of anti-VEGF, anti-PIGF and combination treatments were calculated. Retina PIGF concentration was evaluated by ELISA after treatment. The vasoactive effects of exogenous PIGF on HUVECs were investigated by proliferation and migration studies.
 
Results: PIGF mRNA expression was reduced by hypoxia in OIR mice, in HUVECs under hypoxia and anti-VEGF treatment. However, PIGF expression was reversed by anti-VEGF therapy in the OIR model and in HUVECs under hypoxia. Exogenous PIGF significantly inhibited HUVECs proliferation and migration under normal conditions, but it stimulated cell proliferation and migration under hypoxia. Anti-PIGF treatment was effective for neovascular tufts in OIR mice (P<0.05).
 
Conclusion: The finding that PIGF expression is iatrogenically up-regulated by anti-VEGF therapy provides a consideration to combine it with anti-PIGF therapy.
 
World J Pediatr 2014;10(3):262-270
 
Key words: human umbilical vein endothelial cells;
                    oxygen-induced retinopathy;
                    placental growth factor;
                    vascular endothelial growth factor
  [Abstract] [Full Text] [PDF]  
Brief report:
Clinical characteristics and mutation analysis of three Chinese children with autosomal recessive polycystic kidney disease
  Shu-Ping Liu, Jie Ding, Fang Wang, Yan-Qin Zhang, Jin-Tang Ye
 
Clinical characteristics and mutation analysis of three Chinese children with autosomal recessive polycystic kidney disease
 
Shu-Ping Liu, Jie Ding, Fang Wang, Yan-Qin Zhang, Jin-Tang Ye
Beijing, China
 
Author Affiliations: Department of Pediatrics, Peking University First Hospital, Beijing, 100034, China (Liu SP, Ding J, Wang F, Zhang YQ); Department of Radiology, Peking University First Hospital, Beijing, 100034, China (Ye JT)
 
Corresponding Author: Jie Ding, Department of Pediatrics, Peking University First Hospital, No.1, Xi'anmen Street, Beijing, China (Tel: 86-10-83572168; Fax: 86-10-66530532; Email: djnc_5855@126.com)
 
doi: 10.1007/s12519-014-0503-z
 
Background: There are few studies on the genotypes and phenotypes of autosomal recessive polycystic kidney disease in Chinese patients.
 
Methods: PKHD1 mutations in three children were detected with PCR and direct sequencing, and their clinical data were retrospectively reviewed.
 
Results: All of the children had bilateral enlarged polycystic kidneys, congenital hepatic fibrosis and intrahepatic bile duct dilatation. One of three children had classical multiple small cysts throughout the kidneys, and the other two children had bilateral multiple renal cysts of various sizes. Two children had abnormally shaped livers, portal hypertension and splenomegaly. Two heterozygous mutations (p.T36M, and p.P137S) were detected in Patient 1 and two were detected in Patient 2 (p.L2658X and p.V836A). One heterozygous mutation (p.L1425R) was detected in Patient 3.
 
Conclusions: The study shows that renal and liver phenotypes of the Chinese children varied. Five mutations were identified in the three children, three of which were novel mutations.
 
World J Pediatr 2014;10(3):271-274
 
Key words: cirrhosis;
                    mutation;
                    polycystic kidney
  [Abstract] [Full Text] [PDF]  
Case reports:
A novel CLCN5 mutation in a Chinese boy with Dent's disease
  Li-Na Ji, Chao-Ying Chen, Jing-Jing Wang, Li Cao
 
 
Li-Na Ji, Chao-Ying Chen, Jing-Jing Wang, Li Cao
Beijing, China
 
Author Affiliations: Nephrology Department, Children's Hospital Affiliated to Capital Institute of Pediatrics, No 2 Yabao Road, Chaoyang District, Beijing 100020, China (Ji LN, Chen CY, Wang JJ, Cao L)
 
Corresponding Author: Li-Na Ji, Nephrology Department, Children's Hospital Affiliated to Capital Institute of Pediatrics, No 2 Yabao Road, Chaoyang District, Beijing 100020, China (Tel: 86-10-85695649; Email: lnji008@sina.com)
 
doi: 10.1007/s12519-014-0504-y
 
Background: Dent's disease is a rare X-linked recessive hereditary disease caused by mutations in either the CLCN5 or OCRL1 genes. This disease is characterized by manifestations of proximal renal tubule dysfunction associated with low molecular weight proteinuria (LMWP), hypercalciuria, nephrocalcinosis, nephrolithiasis, and progressive renal failure.
 
Methods: We report a Chinese boy with Dent's disease, clinically diagnosed by LMWP and hypercalciuria. Genetic analysis was made of the CLCN5 and OCRL1 genes. Related studies were also reviewed.
 
Results: A splice site mutation IVS6, +2T>C of the CLCN5 gene was revealed in this case, and it was not reported previously.
 
Conclusions: Clinical and genetic analysis is valuable for the diagnosis of Dent's disease. A novel mutation in the CLCN5 gene was identified in our patient.
 
                                                                                                                                                          World J Pediatr 2014;10(3):275-277
 
Key words: CLCN5;
                    Dent's disease;
                    gene mutation;
                    hypercalciuria;
                    proteinuria
  [Abstract] [Full Text] [PDF]  
GATA3 mutation in a family with hypoparathyroidism, deafness and renal dysplasia syndrome
  Zi-Yang Zhu, Qiao-Li Zhou, Shi-Ning Ni, Wei Gu
 
GATA3 mutation in a family with hypoparathyroidism, deafness and renal dysplasia syndrome
 
Zi-Yang Zhu, Qiao-Li Zhou, Shi-Ning Ni, Wei Gu
Nanjing, China
 
Author Affiliations: Department of Pediatric Endocrinology, Nanjing Children's Hospital Affiliated to Nanjing Medical University, 72 Guangzhou Road, Nanjing 210008, China (Zhu ZY, Zhou QL, Ni SN, Gu W)
 
Corresponding Author: Wei Gu, MM, Department of Pediatric Endocrinology, Nanjing Children's Hospital Affiliated to Nanjing Medical University, 72 Guangzhou Road, Nanjing 210008, China (Email: guwei154@163.com)
 
doi: 10.1007/s12519-014-0505-x
 
Background: The hypoparathyroidism, deafness and renal dysplasia (HDR) syndrome is an autosomal dominant disorder primarily caused by GATA3 gene mutation. We report here a case that both of a Chinese boy and his father had HDR syndrome which caused by a novel mutation of GATA3.
 
Methods: Polymerase chain reaction and DNA sequencing was performed to detect the exons of the GATA3 gene for mutation analysis.
 
Results: Sequence analysis of GATA3 revealed a heterozygous nonsense mutation in this family: a mutation of GATA3 at exon 2 (c.515C >A) that resulted in a premature stop at codon 172 (p.S172X) with a loss of two zinc finger domains.
 
Conclusion: We identified a novel nonsense mutation which will expand the spectrum of HDR-associated GATA3 mutations.
 
World J Pediatr 2014;10(3):278-280
 
Key words: HDR syndrome;
                    mutation
  [Abstract] [Full Text] [PDF]  
Lemierre's syndrome with double heterozygote status in the methylenetetrahydrofolate reductase gene
  Mostafa Behpour-Oskooee, Abdollah Karimi, Shirin Sayyahfar
 
Lemierre's syndrome with double heterozygote status in the methylenetetrahydrofolate reductase gene
 
Mostafa Behpour-Oskooee, Abdollah Karimi, Shirin Sayyahfar
Tehran, Iran
 
Author Affiliations: Pediatric Infectious Diseases, Department of Pediatrics, Najmieh Hospital, Baghyatollah University of Medical Sciences, Tehran, Iran (Behpour-Oskooee M); Pediatric Infectious Diseases, Pediatric Infectious Research Center, Mofid Children Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran (Karimi A); Pediatric Infectious Diseases, Department of Pediatrics, Ali Asghar Children Hospital, Iran University of Medical Sciences, Tehran, Iran (Sayyahfar S)
 
Corresponding Author: Shirin Sayyahfar, MD, Department of Pediatrics, Ali Asghar Children Hospital, Iran University of Medical Sciences, Vahid Dastgerdi Street, Shariati Street, Tehran, Iran (Tel: +98 21 22255218; Fax: +98 21 220063; Email: sayyahfar.sh@iums.ac.ir)
 
doi: 10.1007/s12519-014-0475-z
 
Background: There are some risk factors being more vulnerable to Lemierre's syndrome such as a hypercoagulable state.
 
Methods: We report a rare case of Lemierre's syndrome with ethmoid and maxillary sinusitis, bilateral mastoiditis, and sigmoid sinus thrombosis.
 
Results: Genetic study revealed a double heterozygote status in the methylenetetrahydrofolate reductase gene including C677T and A1298C.
 
Conclusion:  It is suggested to screen patients with Lemierre's syndrome for a hypercoagulable state to consider anticoagulant therapy.
 
World J Pediatr 2014;10(3):281-283
 
 
Key words: Lemierre's syndrome;
                    mastoiditis;
                    methylenetetrahydrofolate reductase;
                    sigmoid sinus thrombosis
  [Abstract] [Full Text] [PDF]  
   
 
 
 
World Journal of Pediatric Surgery
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