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Vol 8, No 4
Vol 8, No 4 November 2012 ISSN 1708-8569
 
Review article
Meta-analysis
Original articles
Brief report
Clinical summary
Case reports
   
Review article:
An overview of risk factors for poor neurodevelopmental outcome associated with prematurity
  Tao Xiong, Fernando Gonzalez, De-Zhi Mu
  Author Affiliations: Department of Pediatrics, West China Second University Hospital, Sichuan University, Chengdu 610041, China (Xiong T, Mu DZ); Key Laboratory of Obstetric & Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, Sichuan University, Chengdu 610041, China (Xiong T, Mu DZ); Departments of Neurology and Pediatrics, Newborn Brain Research Institute, University of California, San Francisco, California, USA (Gonzalez F)

Corresponding Author: De-Zhi Mu, MD, PhD, Department of Pediatrics, West China Second University Hospital, Sichuan University, Chengdu, China (Tel: +86-28-85503226; Fax: +86-28-85559065; Email: dezhi.mu@ucsf.edu)

doi: 10.1007/s12519-012-0372-2

Background: Preterm birth is a major cause of neonatal mortality and morbidity. While advances in medical care have improved the survival of preterm infants, neurodevelopmental problems persist in this population. This article aims to review factors associated with their neurodevelopmental outcomes.

Data sources: English language studies of neurode-velopmental outcomes in preterm infants were retrieved from PubMed. A total of 100 related publications were included.

Results: Early gestational age and birth weight are the most significant predictors of poor long-term neurological outcome. Structural changes of the brain, infection, male gender and neonatal intensive care unit  course are also important factors affecting eventual outcome. Other complex biological and socio-economic factors, which extend from prenatal through postnatal periods, up through and including adulthood, also affect the trajectory of brain development in preterm infants.

Conclusions: Neurodevelopmental problems continue to affect the preterm population. There is a critical need for collaboration among geneticists, obstetricians, pediatricians, and neuroimaging and rehabilitation experts to determine early predictive factors and neuroprotective therapies to properly treat or prevent poor neurodevelopmental outcomes in these infants.

Key words: factor; infant; neurodevelopment; outcome; preterm

World J Pediatr 2012;8(4):293-300

  [Abstract] [Full Text] [PDF]  
Meta-analysis:
Is Helicobacter pylori infection associated with Henoch-Schonlein purpura in Chinese children? a meta-analysis
  Li-Jing Xiong, Yu Tong, Zhi-Ling Wang, Meng Mao
  Author Affiliations: Department of Pediatrics, West China Second University Hospital, Sichuan University, Chengdu, China (Xiong LJ, Tong Y, Wang ZL, Mao M); Laboratory of Early Developmental and Injuries, West China Institute of Woman and Children's Health, West China Second University Hospital, Sichuan University, Chengdu, China (Xiong LJ, Tong Y, Mao M); Key Laboratory of Obstetric & Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, Chengdu 610041, China (Xiong LJ, Tong Y, Mao M)

Corresponding Author: Meng Mao, MD, Department of Pediatrics, West China Second University Hospital, Sichuan University, No.20 Renmin South Road (SectionIII), Chengdu 610041, China (Email: dffmmao@126.com)

doi: 10.1007/s12519-012-0373-1

.Background: The prevalence of Helicobacter pylori (H. pylori) infection is high in China. It not only causes the damage of gastric epithelium, but also plays a potential pathogenic role in several extraintestinal diseases. Henoch-Schonlein purpura (HSP) is one of the most common vasculitis syndromes affecting children. Although its cause is unclear, HSP is often considered to be associated with infectious agents. This meta-analysis of previously published studies was conducted using a predefined protocol to evaluate the underlying association between H. pylori infection and HSP in Chinese children.

Methods: Predefined search strategy and inclusion criteria were set up to select studies reporting the prevalence of H. pylori infection among HSP children and control groups. Included studies were subjected to quality assessment and data extraction by two independent reviewers. The pooled odds ratio (OR) was calculated as the effect size via both traditional and cumulative meta-analysis. Heterogeneity was investigated by subgroup analysis, and the nonparametric "trim and fill" method was performed to adjust the overall estimate for the existence of publication bias.

Results: Ten eligible studies covering 749 HSP children and 560 controls were included for meta-analysis. Observational epidemiology studies clearly aimed at detecting the potential association between H. pylori infection and HSP with retrospective data collection from the children enrolled consecutively. Overall, 49.27% (369/749) of HSP children had evidence of H. pylori infection compared with 23.39% (131/560) of children in the control group. The pooled OR of H. pylori infection in HSP children (10 studies with 749 HSP children) was 3.80 [95% confidence interval (CI): 2.54-5.68, P<0.001], and the overall estimate from the cumulative meta-analysis confirmed the association with more narrow confidence interval (OR=3.35, 95% CI: 2.95-3.81). In HSP children mainly with abdominal manifestations (8 studies with 337 HSP children), the pooled OR was 4.62 (95% CI: 2.66-8.01, P<0.001). The adjusted pooled OR was 2.04 (95% CI: 1.48-2.82, P<0.001), determined by the nonparametric "trim-and-fill" method for eliminating the effect of publication bias. H. pylori eradication therapy (4 studies with 266 HSP children) was capable of reducing the recurrence of HSP (RR=0.38, 95% CI: 0.25-0.58, P<0.001). Although the subgroup analysis for heterogeneity suggested that diagnostic methods and geographical diversity might be account for the heterogeneity, statistical analysis of differences revealed no differences between subgroups, indicating their limited impact on the overall estimates.

Conclusions: These results suggest the necessity of screening H. pylori infection in HSP children, particularly in those with gastrointestinal manifestations in China. Eradication therapy may reduce the recurrence of HSP in children with H. pylori infection. However, further mechanistic and more clinical studies in different populations and regions are needed to confirm this association and the effect of eradication of H. pylori in HSP children.

Key words: Helicobacter pylori; Henoch-Schonlein purpura; meta-analysis

World J Pediatr 2012;8(4):301-308
  [Abstract] [Full Text] [PDF]  
Original articles:
Psychomotor development of children born after preimplantation genetic diagnosis and parental stress evaluation
  Loretta Thomaidis, Sophia Kitsiou-Tzeli, Elena Critselis, Hera Drandakis, Vassiliki Touliatou, Stelios Mantoudis, Eleni Leze, Aspasia Destouni, Joanne Traeger-Synodinos, Dimitrios Kafetzis, Emmanouel Kanavakis
   

Author Affiliations: Developmental Assessment Unit, Second Department of Pediatrics, "P. & A. Kyriakou" Children's Hospital, National and Kapodistrian University of Athens, School of Medicine, Athens, Greece (Thomaidis L, Critselis E, Drandakis H, Mantoudis S, Kafetzis D); Department of Medical Genetics, National and Kapodistrian University of Athens School of Medicine, Athens, Greece (Kitsiou-Tzeli S, Touliatou V, Leze E, Destouni A, Traeger-Synodinos J, Kanavakis E)

Corresponding Author: Loretta Thomaidis, MD, PhD, Developmental Pediatrics, Developmental Assessment Unit, Second University Department of Pediatrics, "P. & A. Kyriakou", Children's Hospital, Livadias and Thivon Streets, Athens 11527, Greece (Tel: +302132009198; Fax: +302107774383; Email: dr_thomaidis@yahoo.gr)

doi: 10.1007/s12519-012-0374-0

Background: The increasing number of children conceived following preimplantation genetic diagnosis (PGD) necessitates the evaluation of their motor and cognitive development. The primary study objective was to evaluate the physical, developmental, and neurological outcome of children born after PGD in Greece. In addition, the secondary study objective was to compare the stress levels regarding parental roles between parents of PGD children and those of naturally conceived children.

Methods: A cross-sectional study design was applied. The study population consisted of 31 children (aged 2 months to 7.5 years) born after PGD analysis and their parents. The developmental evaluation of children included a detailed physical evaluation and cognitive assessment with the Bayley Scales of Infant Development. The parent stress index was applied to evaluate comparative parental stress levels between those parents of PGD children and those of naturally conceived healthy children.

Results: High rates of caesarean deliveries, increased incidence of prematurity, multiples and low-birth weight were observed among the 31 PGD children. Overall, 24 of the 31 PGD children had cognitive skills within normal range [general developmental quotient (GDQ): 86-115], while 6 children had lower levels of cognitive skills (GDQ<85). With regard to parental stress, PGD parents reported lower levels of parenting stress as compared to parents of naturally conceived children (P<0.01).

Conclusions: The enhanced frequency of poor cognitive and motor skills as well as low parental stress necessitates early detection and intervention for developmental delays among PGD children.

Key words: general developmental quotient; parental stress level; preimplantation genetic diagnosis; psychomotor development

World J Pediatr 2012;8(4):309-316

  [Abstract] [Full Text] [PDF]  
Sensitization to food allergens in Iranian children with mild to moderate persistent asthma
  Shirin Farjadian, Mozhgan Moghtaderi, Sara Kashef, Soheila Alyasin
   

Author Affiliations: Allergy Research Center (Farjadian S, Moghtaderi M, Kashef S), Department of Immunology (Farjadian S), and Department of Pediatric Immunology and Allergy (Kashef S, Alyasin S), Shiraz University of Medical Sciences, Shiraz, Iran

Corresponding Author: Mozhgan Moghtaderi, Allergy Research Center, Shiraz University of Medical Sciences, Zand St., Shiraz 71348-45794, Iran (Tel: +98 711 6474298; Fax: +98 711 6474298; Email: Moghtadery@sums.ac.ir)

doi: 10.1007/s12519-012-0375-z

Background: Asthma is the most common chronic respiratory disease in childhood. The clinical presentation of asthma may worsen after food allergen ingestion in sensitized patients. To avoid nonspecific dietary restrictions in children with asthma, laboratory-based advice about foods is potentially helpful. The purpose of this study was to determine food sensitization in children with asthma.

Methods: Seventy-nine children with mild to moderate persistent asthma were included in this study. Commercial food allergens including cow's milk, egg white, almond, potato, and soybean were used in skin prick tests. Specific IgE to 20 common food allergens was also measured in serum.

Results: Twelve (15.2%) of the patients had a positive skin prick test to at least one of the five food extracts. Sensitization was detected by skin prick tests to cow's milk and egg white (each 6.3%), almond (3.8%), potato (2.5%) and soybean (1.3%). Specific IgE levels ≥0.35 kAU/L were detected in the serum of 47% of the children with asthma. The most common food allergens were cow's milk (26.6%), hazelnuts (25.3%), wheat flour (15.2%) and egg white (12.6%). Patients with a history of at least one hospital admission due to asthma attack had a higher rate of sensitization to egg.

Conclusions: In our study, food sensitization was frequent in Iranian children with asthma. Although clinical food allergy could not be evaluated because food challenge tests were not used in our study, skin prick tests and serum-specific IgE to common food allergens might be helpful in identifying children with food sensitization.

Key words: childhood asthma; food sensitization; serum-specific IgE; skin prick test

World J Pediatr 2012;8(4):317-320

  [Abstract] [Full Text] [PDF]  
Clinical and epidemiological characteristics of 2009 pandemic influenza A in hospitalized pediatric patients of the Saurashtra region, India
  Rajesh K Chudasama, Umed V Patel, Pramod B Verma, Prerna Agarwal,
   

Author Affiliations: Department of Community Medicine, M P Shah Medical College, Jamnagar, Gujarat, India (Chudasama RK); Department of Community Medicine, P D U Medical College, Rajkot, Gujarat, India (Patel UV, Verma PB, Agarwal P, Bhalodiya S, Dholakiya D)

Corresponding Author: Rajesh K Chudasama, Vandana Embroidary, Mato Shree Complex, Sardar Nagar Main Road, Rajkot – 360 001, Gujarat, India (Tel: +91 94284 52080; Fax: +91 281 2455810; Email: dranakonda@yahoo.com; dranakonda@gmail.com)

doi: 10.1007/s12519-012-0376-y

Background: The first case of 2009 pandemic influenza A or H1N1 virus infection in India was reported in May 2009 and in the Saurashtra region in August 2009. We describe the two waves clinico-epidemiological characteristics of children who were hospitalized with 2009 influenza A infection in the Saurashtra region.

Methods: From September 2009 to February 2011, we treated 117 children infected with 2009 influenza A virus who were admitted in different hospitals in Rajkot city. Real-time reverse transcriptase polymerase chain reaction (RT-PCR) test was used to confirm infection, and the clinico-epidemiological features of the disease were closely monitored.

Results: In the 117 patients, with a median age of 2 years, 59.8% were male. The median time from onset of the disease to influenza A diagnosis was 5 days, and that from onset of the disease to hospitalization was 7 days. The admitted patients took oseltamivir, but only 11.1% of them took it within 2 days after onset of the disease. More than one fourth (29.1%) of the admitted patients died. The most common symptoms of the patients were cough (98.3%), fever (94.0%), sore throat and shortness of breathing. Pneumonia was detected by chest radiography in 80.2% of the patients.

Conclusions: In children with infection-related illness, the survival rate was about 71% after oseltamivir treatment. The median time for virus detection with real-time RT-PCR is 5 days. Early diagnosis and treatment may reduce the severity of the disease.

Key words: antiviral drug; clinical features; epidemiology; influenza A; intensive care

                    World J Pediatr 2012;8(4):321-327

  [Abstract] [Full Text] [PDF]  
+276 G/T single nucleotide polymorphism of the adiponectin gene is associated with the susceptibility to biliary atresia
  Wanvisa Udomsinprasert, Tewin Tencomnao, Sittisak Honsawek, Wilai Anomasiri, Paisarn Vejchapipat, Voranush Chongsrisawat, Yong Poovorawan
   

Author Affiliations: Program in Medical Sciences, Department of Biochemistry, Faculty of Medicine, Chulalongkorn University, Bangkok 10330, Thailand (Udomsinprasert W, Honsawek S, Anomasiri W); Center for Excellence in Omics-Nano Medical Technology Development Project, Department of Clinical Chemistry, Faculty of Allied Health Sciences, Chulalongkorn University, Bangkok 10330, Thailand (Tencomnao T); Department of Surgery, Faculty of Medicine, Chulalongkorn University, Bangkok 10330, Thailand (Vejchapipat P); Center of Excellence in Clinical Virology, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok 10330, Thailand (Chongsrisawat V, Poovorawan Y)

Corresponding Author: Yong Poovorawan, MD, Center of Excellence in Clinical Virology, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University and King Chulalongkorn Memorial Hospital, Rama IV road, Patumwan, Bangkok 10330, Thailand (Tel: +662-256-4929; Fax: +662-256-4909; Email: Yong.P@chula.ac.th)

doi: 10.1007/s12519-012-0377-x

Background: Biliary atresia (BA) is an intractable neonatal inflammatory and obliterative cholangiopathy, leading to progressive fibrosis and cirrhosis. Adiponectin, an anti-inflammatory adipokine, is known to play a possible role in liver diseases. The objective of our study was to determine the relationship between adiponectin gene polymorphisms and BA susceptibility.

Methods: A total of 106 BA patients and 107 healthy controls were included in this study. Two single nucleotide polymorphisms (SNPs) of the adiponectin gene, +45T/G (rs2241766) and +276G/T (rs1501299), were genotyped by polymerase chain reaction-restriction fragment length polymorphism analysis.

Results: Genotype distributions of +45 T/G and +276 G/T SNPs were seen in the Hardy-Weinberg equilibrium for both BA patients and controls. The frequency of the G/G genotype at +276G/T was significantly higher in BA patients than in the controls (P=0.009). Regarding +45T/G in BA patients, the frequency of the T/T genotype tended to be lower than in the controls, but the difference was not significant. Moreover, the G allele at +276G/T in BA patients was more common than in the controls (P=0.0043). In contrast, the frequency of the T allele at +45T/G was not significantly different between BA patients and the controls. None of the haplotypes studied was found to significantly influence the risk of BA.

Conclusions: +276G/T SNP is strongly associated with BA, particularly with the G allele. We postulate that the +276G/T adiponectin gene polymorphism confers increased susceptibility to BA.

Key words: adiponectin; biliary atresia; polymerase chain reaction-restrictionfragment length polymorphism; single nucleotide polymorphism

World J Pediatr 2012;8(4):328-334

  [Abstract] [Full Text] [PDF]  
Decrease of renal aquaporins 1-4 is associated with renal function impairment in pediatric congenital hydronephrosis
  Zhen-Zhen Li, Lu Xing, Zhan-Zheng Zhao, Jin-Sheng Li, Rui Xue, Avinash Chandra, Rikke Nørregaard, Jian-Guo Wen
   

Author Affiliations: The Institute of Clinical Medicine (Li ZZ, Xing L, Li JS, Xue R, Chandra A, Wen JG), Pediatric Urodynamic Center, Urology Department (Xing L, Li JS, Wen JG), Nephrology Department (Zhao ZZ), and Neurology Department (Chandra A), First Affiliated Hospital of Zhengzhou University, Zhengzhou, China; The Water and Salt Research Center, Institute of Clinical Medicine, Aarhus University, Aarhus, Denmark (Nørregaard R)

Corresponding Author: Jian-Guo Wen, MD, PhD, The Pediatric Urodynamic Center, Urology Department of First Affiliated Hospital of Zhengzhou University, The Institute of Clinical Medicine Universities, Zhengzhou 450052, China (Tel: +86-371-66295219; Fax: +86-371- 66989950; Email: jgwen@zzu.edu.cn)

doi: 10.1007/s12519-012-0378-9

Background: Renal aquaporins (AQP1-4) concentration is downregulated and is in proportion to the degree of hydronephrosis graded by ultrasound in pediatric congenital hydronephrosis (CH). However, the relationship between the expression of AQP1-4 with the changes of renal function impairment (RFI) evaluated by 99mTc-DTPA renal dynamic imaging is still unclear. This study aimed to investigate the relationship between AQP1-4 expression and degree of RFI in children with CH.

Methods: The expression of AQP1-4 was evaluated in 45 children with unilateral ureteropelvic junction obstruction (28 boys and 17 girls, average age: 28±10 months) and 15 children undergoing nephrectomy for nephroblastoma (8 boys and 7 girls, average age: 26±8 months) by immunoblotting and immunohistochemistry. Renal function was graded into mild and severe RFI by 99mTc-DTPA renal dynamic imaging.

Results: One-way analysis of variance with Bonferonni's correction showed a significantly reduced protein expression of AQP1-4 in the severe RFI group compared with those in both mild RFI group and controls (AQP1: 0.52±0.09 vs. 0.91±0.06 vs. 1.23±0.033; AQP2: 0.68±0.12 vs. 1.09±0.06 vs. 1.52±0.08; AQP3: 0.59±0.16 vs. 0.94±0.08 vs. 1.31±0.07; AQP4: 0.64±0.06 vs. 1.14±0.07 vs. 1.61±0.07; P<0.001, respectively). In kidneys with severe RFI, there was a reduction in the protein concentration of all four AQP isoforms which was more pronounced compared with those seen in kidneys with mild RFI and in the controls.

Conclusion: AQP1-4 expression is reduced in proportion with the impairment degree of renal function graded by 99mTc-DTPA renal dynamic imaging in human CH.

Key words: aquaporins; congenital hydronephrosis; renal function; 99mTc-DTPA renal dynamic imaging

World J Pediatr 2012;8(4):335-341

  [Abstract] [Full Text] [PDF]  
Diffusion-weighted MRI for detection and differentiation of musculoskeletal tumorous and tumor-like lesions in pediatric patients
  Henning Neubauer, Laura Evangelista, Nicole Hassold, Beate Winkler, Paul Gerhardt Schlegel, Herbert Köstler, Dietbert Hahn, Meinrad Beer
   

Author Affiliations: Department of Pediatric Radiology, Institute of Radiology, University of Wuerzburg, Josef-Schneider-Straße 2, Wuerzburg 97080, Germany (Neubauer H, Evangelista L, Hassold N, Beer M); Department of Pediatrics, University of Wuerzburg, Josef-Schneider-Straße 2, Wuerzburg 97080, Germany (Winkler B, Schlegel PG); Institute of Radiology, University of Wuerzburg, Oberduerrbacher Str. 6, Wuerzburg 97080, Germany (Koestler H, Hahn D).

Corresponding Author: Henning Neubauer, MD, MBA, Department of Pediatric Radiology, Institute of Radiology, University Hospital Wuerzburg, Josef-Schneider-Straße 2, Wuerzburg 97080, Germany (Tel: 0049-931-201-34715; Fax: 0049-931-201-34857; Email: neubauer@roentgen.uni-wuerzburg.de).

doi: 10.1007/s12519-012-0379-8

Background: MRI is the diagnostic mainstay for detection and differentiation of musculoskeletal tumors. However, a projection regarding the biological dignity of lesions based on standard MRI sequences remains difficult and uncertain. This study was undertaken to analyse whether diffusion-weighted MRI (DWI) can distinguish between benign and malignant musculoskeletal tumorous and tumor-like lesions in pediatric patients.

Methods: MR examinations of 44 consecutive pediatric patients (26 girls, mean age 11±6 years) including standard sequences and DWI (b=50/800 s/mm2) at 1.5 or 3 Tesla were retrospectively evaluated. The study group contained 10 patients with non-treated malignant tumors and 34 patients with benign lesions. Size, relative signal intensity and apparent diffusion coefficient (ADC, unit ×103 mm2/s) were determined in one lesion per patient.

Results: Mean ADC was 0.78±0.45×103 mm2/s in patients with malignant tumors and 1.71±0.75 ×103 mm2/s in patients with benign lesions (P<0.001). Relative operating characteristics (ROC) analysis showed a sensitivity of 90% and a specificity of 91% for malignancy, based on an ADC cut-off value of ≤1.03. On logistic regression, mean ADC and lesion size accounted for 62% of variability in benign vs. malignant tumors. For malignant tumors, the signal intensity ratio was higher on DWI than on T1w post-contrast images (P<0.002). Two cases of local tumor recurrence were diagnosed by DWI only.

Conclusions: DWI shows promising results for determination of biological dignity in musculoskeletal tumors. Mean ADC ≤1.03×103 mm2/s is a strong indicator of malignancy at the first diagnosis. The use of DWI for early diagnosis of tumor recurrence in comparison with standard MRI sequences should be evaluated in prospective studies.

Key words: diffusion-weighted imaging; magnetic resonance imaging; musculoskeletal tumor; pediatric patient                 

World J Pediatr 2012;8(4):342-349

  [Abstract] [Full Text] [PDF]  
Mid-term differences in right ventricular function in patients with congenital diaphragmatic hernia compared with controls
  Matthew J Egan, Nazia Husain, Jack R Stines, Nasser Moiduddin, Melanie A Stein, Leif D Nelin, Clifford L Cua
   

Author Affiliations: The Heart Center (Egan MJ, Husain N, Stines JR, Moiduddin N, Cua CL) and Center for Perinatal Research (Stein MA, Nelin LD), Department of Pediatrics, Nationwide Children's Hospital, Columbus, OH, USA

Corresponding Author: Clifford L Cua, MD, Assistant Professor of Pediatrics, Nationwide Children's Hospital 700 Children's Hospital, Columbus, OH 43205, USA (Tel: 614-722-2530; Fax: 614-722-2549; Email: clcua@hotmail.com)

doi: 10.1007/s12519-012-0380-2

Background: Patients with congenital diaphragmatic hernia (CDH) may have abnormal lung development, which may cause detrimental effects on right ventricular (RV) function. This study aimed to determine if there are persistent echocardiographic differences in RV function in patients with CDH years after repair versus control patients.

Methods: Patients who underwent repair for CDH were recruited. RV function was evaluated by strain analysis and tissue Doppler imaging (TDI). Wilcoxon's rank-sum test was used for analysis.

Results: Seven CDH patients and 16 control patients were studied. There was no difference in age between the CDH and control groups (6.2±1.7 years vs. 5.7±1.7 years). TDI demonstrated significantly lower values in the RV early diastolic wave (12.8±1.5 cm/s vs. 16.1±3.1 cm/s) and RV systolic wave (10.2± 0.8cm/s vs. 13.4±1.3 cm/s) when comparing the CDH group and the control group. Interventricular apical septal strain was significantly lower in the CDH group than in the control group (-20.1±4.6% vs. -25.4±4.1%). There was a trend towards lower strain values in the RV mid-lateral segment in the CDH group (-30.8±9.9% versus -39.7±6.0%, P=0.06) and a lower global RV strain (-27.8±3.0% vs. -31.1±3.1%, P=0.06).

Conclusions: Patients who underwent CDH repair continue to have differences in RV function years after repair. Follow-up is needed to determine how these differences impact cardiac function in adult survivors of CDH.

Key words: cardiology; congenital diaphragmatic hernia; echocardiography; strain

World J Pediatr 2012;8(4):350-354
  [Abstract] [Full Text] [PDF]  
A comparison of criteria for diagnosis of atopic dermatitis in children
  Zbigniew Samochocki, Jowita Dejewska
  Author Affiliations: Department of Dermatology, Medical University of Warsaw, Koszykowa 82A, Warsaw 02-008 , Poland (Samochocki Z); Department of Dermatology, Central Clinical Hospital of Military Institute of Medicine, Warsaw, Szaserów 128, Warsaw 00-909 , Poland (Dejewska J)

Corresponding Author: J Dejewska, Department of Dermatology, Central Clinical Hospital of Military Institute of Medicine, Warsaw, Szaserów 128, Warsaw 00-909, Poland (Email: jdejewska@yahoo.pl)

doi: 10.1007/s12519-012-0381-1

Background: Atopic dermatitis (AD) diagnosis in children gives rise to many problems. Although the Hanifin and Rajka criteria are acknowledged and used universally in diagnosing AD, their evaluation is a major source of difficulty in pediatrician's daily practice. The simplified criteria, revised by Williams et al, seem to be more useful and represent the most common clinical manifestations of AD. The aim of the present study was to compare the efficacy of the two different criteria for diagnosing AD in children.

Methods: This study involved 250 children with AD. All of the patients underwent clinical examinations and the diagnostic procedures according to the criteria of Hanifin and Rajka as well as those of Williams.

Results: According to the Hanifin-Rajka criteria, AD was diagnosed in 173 children, of whom 153  were diagnosed positive by the criteria of Williams. Among the 77 children who were diagnosed without AD according to the Hanifin and Rajka criteria, 4 were detected with AD by the Williams criteria. Four children with scabies and seborrhoeic dermatitis were misdiagnosed as having AD according to the Williams criteria due to the atypical locations of their lesions and the histories of asthma or hay fever and xerosis.

Conclusions: Although the criteria of Hanifin and Rajka are the gold standard for diagnosing AD, the Williams criteria are also very useful in children older than 4 years. The most useful Williams criteria for AD diagnosis in children is pruritus with history of lesions in characteristic locations and history of generally dry skin. The significant increase in the detectability of AD is found by raising the borderline age for the first skin lesion development from 2 to 5 years of age.

Key words: atopic dermatitis; diagnosis; Williams criteria

World J Pediatr 2012;8(4):355-358
  [Abstract] [Full Text] [PDF]  
Brief report:
Beta-galactosidase deficiencies and novel GLB1 mutations in three Chinese patients with Morquio B disease or GM1 gangliosidosi
  Hong-Lin Lei, Jun Ye, Wen-Juan Qiu, Hui-Wen Zhang, Lian-Shu Han, Yu Wang, Xue-Fan Gu
  Author Affiliations: Department of Pediatric Endocrinology and Genetic Metabolism, Shanghai Institute for Pediatric Research, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200092, China (Lei HL, Ye J, Qiu WJ, Zhang HW, Han LS, Wang Y, Gu XF)

Corresponding Author: Jun Ye, MD, Shanghai Institute for Pediatric Research, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, 1665 Kong Jiang Road, Shanghai 200092, China (Tel: 86-21-25076455; Fax: 86-21-65791316; Email: yejun2314@yahoo.com.cn)

doi: 10.1007/s12519-012-0382-0

Background: This paper aims to report GLB1 activities and mutation analysis of three patients from the mainland of China, one with Morquio B disease and two with GM1 gangliosidosis.

Methods: GLB1 activity and GLB1 gene mutation were analyzed in the three patients who were clinically suspected of having Morquio B disease or GM1 gangliosidosis. Novel mutations were analyzed by aligning GLB1 homologs, 100 control chromosomes, and the PolyPhen-2 tool.

Results: The enzymatic activity of GLB1 was found to be 5.03, 4.20, and 4.50 nmol/h/mg in the three patients, respectively. Patient 1 was a compound heterozygote for p.[Arg148Cys] and p.[Tyr485Cys] mutations in the GLB1 gene. Patient 2 was a compound heterozygote for p.[Tyr270Phe] and p.[Leu337Pro] mutations. Patient 3 was a homozygote for p.[Asp448Val] mutation. Three mutations (p.[Tyr485Cys], p.[Tyr270Phe] and p.[Leu337Pro]) were novel variants and were predicted to damage GLB1 function.

Conclusions: The enzymatic activity and related gene analysis of β-galactosidase should be performed in clinically suspected individuals to confirm diagnosis. The three novel mutations, p.[Tyr485Cys], p.[Tyr270Phe], and p.[Leu337Pro], are thought to be disease-causing mutations.

Key words: β-galactosidase; enzyme assays; GM1 gangliosidosis; Morquio B disease; mutation analysis

                                                                                                                                                                      World J Pediatr 2012;8(4):359-362
  [Abstract] [Full Text] [PDF]  
Clinical summary:
Tick-borne encephalitis in children and adolescents in the Czech Republic between 1960 and 2007
  Petr Pazdiora, Věra Štruncová, Miroslava Švecová
 

Author Affiliations: Institute of Epidemiology of Medical Faculty of Charles University, Pilsen (Pazdiora P), Clinic of Infectious Diseases of the University Hospital in Pilsen (Štruncová V), Institute of Microbiology of the University Hospital in Pilsen (Švecová M), Regional Hygiene Station of Pilsen Region (Pazdiora P), Czech Republic

Corresponding Author: Petr Pazdiora, MD, PhD, Regional Hygiene Station of Pilsen Region, Skrétova 15, Plzeň 303 22, Czech Republic (Tel: +420377402926; Fax: +420377426083; Email: pazdiora@fnplzen.cz)

doi: 10.1007/s12519-012-0383-z

Background: The Czech Republic ranks among the countries with the highest prevalence of tick-borne encephalitis worldwide. The region of West Bohemia has the second highest morbidity within the Czech Republic.

Methods: Between 1960 and 2007, laboratories confirmed 410 cases of tick-borne encephalitis in children and adolescents of West Bohemia. Available epidemiological data were analyzed.

Results: The highest incidence (per 100 000 population) was found in the group of 15-19 years for both genders (males: 6.2; females: 4.3). Data on the consumption of non-pasteurized milk were found in 5.4% of patients. The preschool age group showed its highest incidence in June and September, and the risk of infection for older children was in July and August.

Conclusions: The current low coverage of vaccination leads to an insignificant improvement to the overall frequency of this disease.

Key words: children; tick-borne encephalitis; transmission; vaccines                            

World J Pediatr 2012;8(4):363-366

  [Abstract] [Full Text] [PDF]  
Case reports:
Peritoneal metastasis of third ventricular atypical teratoid/rhabdoid tumor after VP shunt implantation for unexplained hydrocephalus
  Yi-Peng Han, Yang Zhao, Xiao-Guang He, Jie Ma
   

Author Affiliations: Department of Pediatric Neurosurgery, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai 200092, China (Han YP, Zhao Y, He XG, Ma J)

Corresponding Author: Jie Ma, Department of Pediatric Neurosurgery,  Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai 200092, China (Tel: +86-021-25078421; Email: majie365@hotmail.com)

doi: 10.1007/s12519-012-0384-y

Background: Atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system (CNS) is a highly malignant neoplasm seen frequently in infancy and early childhood. This report presents a 9-year-old girl of primary third ventricular AT/RT with peritoneal metastasis after ventriculoperitoneal (VP) shunt catheter implantation for hydrocephalus before the identification of the CNS tumor.

Methods: The data of clinical course, laboratory and imaging studies were obtained and carefully reviewed. Serial imaging studies including enhanced CT and MRI were performed at the first admission, during which the patient was diagnosed with a non-malignant communicating hydrocephalus. Secondary radiological studies were carried out 5 months after VP shunt, during which the patient demonstrated worsening clinical signs of intracranial hypertension. An imaging study identified a tumor in the third ventricle.

Results: The patient was treated by a surgical resection, showing the specimen was pathologically consistent with AT/RT 5 months after VP shunt. Systematic chemotherapy and radiotherapy were prescribed for the patient. After 6 months, PET/CT revealed peritoneal metastasis but negative findings in the CNS. The parents of the patient refused further intervention, and she died one month later.

Conclusion: VP shunt in a patient with AT/RT may cause distant seeding of the tumor in unrelated areas of the body, even after intensive multimodality treatment. Further studies on shunt related metastases are needed.

Key words: atypical teratoid/rhabdoid tumor; central nervous system; shunt metastasis; ventriculoperitoneal shunt

World J Pediatr 2012;8(4):367-370

  [Abstract] [Full Text] [PDF]  
Successful surgical repair of d-transposition of the great arteries in a separated conjoined twin
  Gang Chen, Bing Jia, Wen-Bo Zhang
   

Author Affiliations: Children's Heart Center, Children's Hospital of Fudan University, Shanghai 201102, China (Chen G, Jia B, Zhang WB)

Corresponding Author: Bing Jia, 399 Wanyuan Road, Shanghai 201102, China (Tel: +86-021-64931532; Fax: +86-021-64931517; Email: jiabing2012@hotmail.com)

doi: 10.1007/s12519-012-0385-x

Background: Conjoined twins are very rare examples of congenital malformations. In 75% of thoraco-omphalopagus conjoined twins, the intracardiac anatomy determines outcome and long-term survival.

Methods: We successfully separated one case of thoraco-omphalopagus conjoined twins, with one having d-transposition of the great arteries. After control of sepsis, the twin underwent an arterial switch and complete repair of her cardiac defect.

Results: She made a full recovery and was discharged from the hospital 20 days after surgery.

Conclusion: Careful planning, skillful surgical separation and cardiac surgery by a combined medical and surgical team is the key to save the twins in such a rare case.

Key words: conjoined twins; sugery; transposition of great vessels

 World J Pediatr 2012;8(4):371-373

  [Abstract] [Full Text] [PDF]  
   
 
 
 
World Journal of Pediatric Surgery
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