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Vol 6, No 2
Vol 6, No 2 May 2010 ISSN 1708-8569
Review articles
Original articles
Case reports
Letter to the Editors
Congenital syphilis: not gone and all too forgotten
  Mary L. Kamb
  [Abstract] [Full Text] [PDF]  
Review articles:
Management of subependymal giant cell tumors in tuberous sclerosis complex: the neurosurgeon's perspective
  Moncef Berhouma
  Background: Tuberous sclerosis complex (TSC), an autosomal dominant genetic disorder, can lead to the development of hamartomas in various organs, including the heart, lungs, kidneys, skin and brain. The management of subependymal giant cell tumors (SGCTs) is still controversial, and peri- and/or intra-ventricular neoplasms may lead to life-threatening hydrocephalus. In the last years, many progresses have been made in research into the tumorigenesis and behaviors of SGCTs. This review aims to clarify the specific role of neurosurgeons in the multidisciplinary management of SGCTs in children with TSC.

Data sources: Based on the recent scientific literature and personal experience, we reviewed the up-to-date data and discussed the trends in the management of SGCTs in children with TSC. The data were collected after a bibliography made using PubMed/Medline with these terms: subependymal, subependymal giant cell astrocytoma, subependymal giant cell tumor, and tuberous sclerosis complex.

Results: SGCTs are shown to be generated from a glioneuronal lineage, but their filiation with subependymal nodules (SENs) is still under debate. While SENs may develop anywhere in the ventricular walls, SGCTs arise almost exclusively around the Monro foramina. In children with TSC, precise clinical and/or imaging criteria are mandatory to differentiate SENs that are always asymptomatic and riskless from SGCTs that have the potential to grow and therefore to obstruct cerebrospinal fluid pathways leading to hydrocephalus.

Conclusions: An earlier diagnosis of SGCT in neurologically asymptomatic children with TSC may allow a precocious surgical removal of the tumor before the installation of increased intracranial pressure signs, an attitude that is being progressively adopted to lessen the morbimortality rate.

Key words: cerebral ventricle; hydrocephalus; intraventricular tumor; microsurgery; subependymal giant cell astrocytoma; tuberous sclerosis

                     World J Pediatr 2010;6(2):103-110

  [Abstract] [Full Text] [PDF]  
Identifying patterns of immune-related disease: use in disease prevention and management
  Rodney R Dietert, Judith T Zelikoff

Background: Childhood susceptibility to diseases linked with immune dysfunction affects over a quarter of the pediatric population in some countries. While this alone is a significant health issue, the actual impact of immune-related diseases extends over a lifetime and involves additional secondary conditions. Some co-morbidities are well known (e.g., allergic rhinitis and asthma). However, no systematic approach has been used to identify life-long patterns of immune-based disease where the primary condition arises in childhood. Such information is useful for both disease prevention and treatment approaches.

Data sources: Recent primary research papers as well as review articles were obtained from PubMed, Chem Abstracts, Biosis and from the personal files of the authors. Search words used were: the diseases and conditions shown Figs. 1 and 2 in conjunction with comorbid, comorbidities, pediatric, childhood, adult, immune, immune dysfunction, allergy, autoimmune, inflammatory, infectious, health risks, environment, risk factors.

Results: Childhood diseases such as asthma, type-1 diabetes, inflammatory bowel disease, respiratory infections /rhinitis, recurrent otitis media, pediatric celiac, juvenile arthritis and Kawasaki disease are examples of significant childhood health problems where immune dysfunction plays a significant role. Each of these pediatric diseases is associated with increased risk of several secondary conditions, many of which appear only later in life. To illustrate, four prototypes of immune-related disease patterns (i.e., allergy, autoimmunity, inflammation and infectious disease) are shown as tools for: 1) enhanced disease prevention; 2) improved management of immune-based pediatric diseases; and 3) better recognition of underlying pediatric immune dysfunction.

Conclusions: Identification of immune-related disease patterns beginning in childhood provides the framework for examining the underlying immune dysfunctions that can contribute to additional diseases in later life. Many pediatric diseases associated with dysfunctional immune responses have been linked with an elevated risk of other diseases or conditions as the child ages. Diseases within a pattern may be interlinked based on underlying immune dysfunctions and/or current therapeutic approaches for managing the entryway diseases. It may be beneficial to consider treatment options for the earliest presenting diseases that will concomitantly reduce the risk of immune-linked secondary conditions. Additionally, improved disease prevention is possible with more relevant and age-specific immune safety testing.

Key words: disease management; disease prevention; environmental risk factors; patterns of disease; pediatric immune dysfunction

                                     World J Pediatr 2010;6(2):111-118

  [Abstract] [Full Text] [PDF]  
The contribution of intraoperative transinguinal laparoscopic examination of the contralateral side to the repair of inguinal hernias in children
  Baruch Klin, Yigal Efrati, Ibrahim Abu-Kishk, Sorin Stolero, Gad Lotan
  Background: Bilateral inguinal hernias are relatively common in children. This fact has led to a controversy of more than 50 years concerning the necessity of bilateral surgical exploration during the repair of unilateral inguinal hernias in children. The advent of transinguinal laparoscopic visualization of the contralateral side is a turning point and a major contribution to the subject, offering the opportunity to reassess the systematic bilateral exploration and the "wait and see" policies currently in use at most services of pediatric surgery.

Data sources: The current information concerning intraoperative transinguinal laparoscopic evaluation of inguinal hernias in children was summarized in a didactic way. A MEDLINE search (PubMed) from 1995 to the present days was conducted.

Results: A patent processus vaginalis (PPV) is not equal to a future symptomatic hernia. There is still no definitive evidence on which PPVs will become a hernia (5.8% to 11.6%) and which remain clinically insignificant. Diagnostic intraoperative transinguinal laparoscopic evaluation of the contralateral side is today the most simple and accurate way to reduce the incidence of negative explorations.

Conclusion: Diagnostic intraoperative transinguinal laparoscopic evaluation of the contralateral side during pediatric inguinal hernia repair is a simple, accurate, fast, and effective method to assess the contralateral processus vaginalis, improving decision-making, reducing the number of negative explorations, and sparing the surgeon the embarrassment associated with the appearance of a metachronous hernia at a later date. It is easily learned and should be part of every pediatric surgeon's practice.

Key words: bilateral exploration; inguinal hernia; laparoscopy; processus vaginalis

                    World J Pediatr 2010;6(2):119-124
  [Abstract] [Full Text] [PDF]  
Original articles:
Congenital syphilis: still a serious, under-diagnosed threat for children in resource-poor countries
  Carsten Krüger, Isaack Malleyeck

Background: With 700 000 to 1.5 million new cases annually, congenital syphilis remains a major infectious cause of morbidity and mortality in neonates, infants and children in resource-poor countries. We therefore analyzed the extent of congenital syphilis in the pediatric patient population at our rural hospital in Tanzania.

Methods: For this retrospective analysis, from January 1, 1998 to August 31, 2000, all cases of congenital syphilis were collected from the medical records of the neonatal and pediatric department at Haydom Lutheran Hospital in rural northern Tanzania. Age, sex, weight, clinical signs and symptoms, venereal disease research laboratory (VDRL) results of mother and/or child, hemoglobin concentration, treatment, and outcome were recorded and analyzed.

Results: Fourteen neonates and infants were included. The earlier the diagnosis, the more it rested on maternal data because the presentation of neonatal congenital syphilis resembled neonatal sepsis. Syphilitic skin lesions were only seen in the post-neonatal age group. VDRL results were positive in 11 of the 14 mothers, and in 4 of the infants. Anemia was common in older infants. No patient showed signs of central nervous system involvement. Two patients died, and the remaining were cured after standard treatment with procaine penicillin.

Conclusions: Highlighting the variable picture of congenital syphilis, this report demonstrates how difficult it is to make a correct diagnosis by solely history and clinical presentation in a resource-poor setting. Hence false-positive and false-negative diagnoses are common, and clinicians have to maintain a high index of suspicion in diagnosing congenital syphilis. Therefore, an important approach to control and finally eliminate congenital syphilis as a major public health problem will be universal on-site syphilis screening of all pregnant women at their first antenatal visit and immediate treatment for those who test positive.

Key words: clinical diagnosis; congenital syphilis; prevention; resource-poor countries; universal screening

                    World J Pediatr 2010;6(2):125-131

  [Abstract] [Full Text] [PDF]  
Mycobacterium bovis bacillus Calmette-Guerin treated human cord blood monocyte-derived dendritic cells polarize naïve T cells into a tolerogenic phenotype in newborns
  En-Mei Liu, Helen KW Law, Yu Lung Lau

Background: As one of the first infectious challenges of life, the impact of neonatal Mycobacterium bovis bacillus Calmette-Guerin (BCG) vaccination on the polarization of neonatal T helper subset has not been well defined.

Methods: We investigated the effect of BCG-treated cord blood (CB) dendritic cells (DCs) on naïve CD4+ T cells polarization compared with that of adult blood DCs.

Results: BCG-treated CB DCs had significantly lower expression of CD83 and a higher ratio of CD47/Fas than BCG-treated adult blood DCs. BCG induced significantly lower IL-12 but relatively higher IL-10 production from CB DCs than adult blood DCs. Moreover, in comparison with BCG-treated adult blood DCs, BCG-treated CB DCs induced higher IL-10 production and cytotoxic T-lymphocyte antigen 4 (CTLA-4) expression, and lower interferon-gamma (IFN-γ) production from naïve CD4+ T cells. On the other hand, lipopolysaccharide-treated CB DCs had similar capacity as prime naïve CD4+ T cells did to produce higher IFN-γ, lower IL-10 production, and CTLA-4 expression compared with their adult counterparts.

Conclusion: These results suggested that BCG-treated CB DCs might be semi-mature DCs which polarize naïve T cells into a tolerogenic T cell phenotype in newborns.

Key words: bacillus Calmette-Guerin; cord blood; dendritic cells; newborns; T cells; tolerance

                 World J Pediatr 2010;6(2):132-140

  [Abstract] [Full Text] [PDF]  
Diagnostic accuracy, reliability and validity of Childhood Autism Rating Scale in India
  Paul SS Russell, Anna Daniel, Sushila Russell, Priya Mammen, Julie S Abel, Lydia E Raj, Satya Raj Shankar, Naveen Thomas

Background: Since there is no established measure for autism in India, we evaluated the diagnostic accuracy, reliability and validity of Childhood Autism Rating Scale (CARS).

Methods: Children and adolescents suspected of having autism were identified from the unit's database. Scale and item level scores of CARS were collected and analyzed. Sensitivity, specificity, likelihood ratios and predictive values for various CARS cut-off scores were calculated. Test-retest reliability and inter-rater reliability of CARS were examined. The dichotomized CARS score was correlated with the ICD-10 clinical diagnosis of autism to establish the criterion validity of CARS as a measure of autism. Convergent and divergent validity was calculated. The factor structure of CARS was demonstrated by principal components analysis.

Results: A CARS score of ≥33 (sensitivity = 81.4%, specificity = 78.6%; area under the curve = 81%) was suggested for diagnostic use in Indian populations. The inter-rater reliability (ICC=0.74) and test-retest reliability (ICC=0.81) for CARS were good. Besides the adequate face and content validity, CARS demonstrated good internal consistency (Cronbach's α=0.79) and item-total correlation. There was moderate convergent validity with Binet-Kamat Test of Intelligence or Gessell's Developmental Schedule (r=0.42; P=0.01), divergent validity (r=-0.18; P=0.4) with ADD-H Comprehensive Teacher Rating Scale, and high concordance rate with the reference standard, ICD-10 diagnosis (82.52%; Cohen's κ=0.40, P=0.001) in classifying autism. A 5-factor structure explained 65.34% of variance.

Conclusion: The CARS has strong psychometric properties and is now available for clinical and research work in India.

Key words: autism; diagnostic accuracy; India; reliability; validation

                   World J Pediatr 2010;6(2):141-147

  [Abstract] [Full Text] [PDF]  
Urinary copper/zinc ratio: a promising parameter for replacement of 24-hour urinary copper excretion for diagnosis of Wilson's disease in children
  Jian-She Wang, Yi Lu, Xiao-Hong Wang, Qi-Rong Zhu

Background: Although 24-hour urinary copper excretion is valuable for diagnosis of Wilson's disease, accurate, timed collection entails practical difficulties. This study aimed to investigate the feasibility of morning urinary copper/creatinine or copper/zinc ratio as replacement parameter for diagnosing Wilson's disease.

Methods: Five random urinary samples collected during 24 hours from two inpatients were used to estimate the consistency of urinary copper/creatinine and copper/zinc ratios. The correlation of the ratios with 24-hour urinary copper excretion was studied in 15 patients with liver diseases. The diagnostic value of morning urinary copper/zinc ratio was further studied in 9 children with Wilson's disease and 22 children with other liver diseases.

Results: The coefficients of variation of urinary copper/creatinine and copper/zinc ratios during 24 hours were 12.5% and 9.3% respectively. The morning urinary copper/creatinine ratio, copper/zinc ratio, and 24-hour urinary copper excretion were correlated well. The area under receiver-operating characteristic curve was comparable between the morning urinary copper/zinc ratio and 24-hour urinary copper excretion (0.983 vs. 0.977).

Conclusion: Morning urinary copper/zinc ratio seems to be a promising parameter in replacement of 24-hour urinary copper excretion for diagnosis of Wilson's disease.

Key words: children; urinary copper/zinc ratio; Wilson's disease; 24-hour urinary copper excretion

                   World J Pediatr 2010;6(2):148-153

  [Abstract] [Full Text] [PDF]  
Peripherally inserted central catheters and the incidence of candidal sepsis in VLBW and ELBW infants: is sepsis increased?
  Bin Xia, Jun Tang, Ying Xiong, Xi-Hong Li, De-Zhi Mu
  Background: Peripherally inserted central catheters (PICCs) have been widely used in neonatal clinics. However, the complications such as infection after PICC treatment are also confronting neonatologists especially in developing countries. This study was undertaken to investigate whether PICCs is a safe treatment for very low birth weight (VLBW) infants and extremely low birth weight (ELBW) infants.

Methods: Fifty-nine VLBW and ELBW infants receiving PICCs and 89 VLBW and ELBW infants receiving peripheral intravenous catheters (PIVCs) were included in this study. The incidence of sepsis and mortality were compared retrospectively between the two groups.

Results: There was no difference in the total sepsis incidence and mortality between the PICCs and PIVCs groups (P=0.11 and P=0.61 respectively). However, the candidal sepsis incidence was higher in the PICCs group than in the PIVCs group [6/59 (10.2%) vs 2/89 (2.2%); P=0.044 (Exat Sig. 1-sided),  OR=4.93, 95% CI 0.96-25.3].

Conclusion: Placement and indwelling of PICCs are a potential risk factor for candidal sepsis among VLBW and ELBW infants.

Key words: candidal sepsis; extremely low birth weight; peripherally inserted central catheters; premature infants; very low birth weight

                   World J Pediatr 2010;6(2):154-157

  [Abstract] [Full Text] [PDF]  
Continuous renal replacement therapy for patients with acute kidney injury caused by melamine-related urolithiasis
  Zi-Hao Yang, Chen-Mei Zhang, Tao Liu, Xiao-Fang Lou, Zhen-Jie Chen, Sheng Ye

Background: In 2008 there was an epidemic of renal disease affecting infants after consumption of melamine-tainted milk products. Most of the infected children were asymptomatic or with mild symptoms, and a few suffered from acute obstructive kidney injury secondary to melamine-contained renal stones (8 of 15 577 children screened at our hospital for urolithiasis). This study was intended to retrospectively review the management of acute kidney injury using continuous renal replacement therapy (CRRT) in the 8 children with acute kidney injury.

Methods: The 8 infants with acute kidney injury caused by melamine-related urolithiasis were referred to the pediatric intensive care unit at the hospital in late 2008. CRRT was given to treat their kidney injuries. Medical records of the infants were reviewed for demographic features, diagnosis, CRRT treatment, and outcomes.

Results: Before CRRT, hypertension was found in 6 of the 8 children. Varying degrees of oliguria, anuria, elevated levels of blood urea nitrogen (BUN) (13.11-35.6 mmol/L) and creatinine (Cr) (238.8-773.7 μmol/L) were observed in these patients. After CRRT, the levels of BUN, Cr and electrolytes decreased. Urine output and edema were improved clinically.

Conclusion: CRRT can rapidly improve renal function, avoiding such surgical interventions as lithotripsy, percutaneous nephrolithotomy, and ureteroscopy. It is an efficient modality to treat acute kidney injury caused by melamine-related urolithiasis.

Key words: acute kidney injury; continuous renal replacement therapy; infant formula; melamine; urolithiasis

  [Abstract] [Full Text] [PDF]  
Flash visual evoked potentials at 2-year-old infants with different birth weights
  Jing-Jing Feng, Ting-Xue Wang, Chen-Hao Yang, Wei-Ping Wang, Xiu Xu

Background: Increased prevalence of visual impairments has been reported in preterm populations. However, it remains unclear about the long-term visual electrophysiological outcomes and their association with visual cognitive functions in premature infants.We investigated visual electrophysiological outcome of 2-year-old infants of different birth weights by flash visual evoked potentials (FVEPs) in order to explore the correlation between visual cognitive functions and FVEPs and to assess the application of FVEPs in evaluating the visual capability of an infant.

Methods: The FVEPs of 77 infants, including 25 very low birth weight (VLBW) premature infants, 16 low birth weight (LBW) premature infants and 36 full-term infants, were tested with a visual electrophysiological testing device. Neuromotor development was assessed with the Bayley Scales of Infant Development, Second Edition (BSID-II). The visual cognitive functions were evaluated by scoring the proportion passed of 12 items chosen from the BSID-II for infants at 23 to 25 months of age.

Results: The second prominent positive wave (P2) was the major component presented in all three groups. The mean latency of P2 in the VLBW, LBW and full-term groups was 149.65±23.79 ms, 129.39±8.70 ms, and 126.14±7.73 ms respectively. There was no significant difference in mean latency of P2 wave between the LBW and full-term groups; the mean latency of the P2 wave in the VLBW group was delayed more significantly than those of the other two groups. The difference in amplitude of the P2 peak to the preceding N2 peak (N2P2) between the three groups was not statistically significant. The latency of the P2 main wave was negatively correlated with mental developmental index (MDI) (r'MDI = -0.35) and visual cognitive capability (r'visual capability = -0.21).

Conclusions: The latency of the P2 main wave on FVEPs was delayed more significantly in premature infants than in full-term infants at 2 years of corrected age. The visual functional development was delayed in premature infants, especially in VLBW infants (gestational age <32 weeks). The FVEPs were reported low but there were statistically significant correlations between measures of visual cognition and P2 peak latency. As a noninvasive and convenient method, FVEPs are useful in assessing certain aspects of an infant's visual development and visual function.

Key words: flash visual evoked potentials; preterm infants; visual development

                    World J Pediatr 2010;6(2):163-168

  [Abstract] [Full Text] [PDF]  
SP600125, an inhibitor of c-Jun NH2-terminal kinase, blocks expression of angiotensin II-induced monocyte chemoattractant protein-1 in human mesangial cells
  Gui-Xia Ding, Ai-Hua Zhang, Song-Ming Huang, Xiao-Qin Pan, Rong-Hua Chen

Background: We investigated the role of c-Jun NH2- terminal kinase (JNK), a member of the mitogen-activated protein kinase family, in the expression of angiotensin II (Ang II)-induced monocyte chemoattractant protein-1 (MCP-1) and transforming growth factor-1 (TGF-1), and in the production of fibronectin (FN), by human mesangial cells (HMCs).

Methods: JNK activation in cultured human mesangial cells was determined by Western blotting with an antibody against the phosphorylated Ser63 residue of c-Jun. Binding of the activator protein (AP-1) to the MCP-1 AP-1 motif was detected via the electrophoretic mobility shift assay (EMSA). The transient luciferase reporter was used to examine MCP-1 promoter activity; an RNase protection assay and ELISA were used respectively to detect the expression of MCP-1 mRNA and production of MCP-1, TGF-β and FN.

Results: Anthra (1,9-cd) pyrazol-6(2H)-one (SP600125), a pharmacological inhibitor of JNK, almost completely abolished Ang II-induced Ser63 phosphorylation of c-Jun at concentrations of 5-20 μmol/L: JNK activity was reduced by 75% with 10 μmol/L SP600125, and by 90% with 20 μmol/L. Ang II increased AP-1 binding to the MCP-1 AP-1 motif in a time-dependent manner, as detected by EMSA, while SP600125 effectively blocked this increased AP-1 binding in a concentration-dependent manner. Treatment with 100 nmol/L Ang II led to a steady increase in MCP-1 mRNA expression, and to an enhanced production of MCP-1, TGF-β and FN. These effects were blocked by SP60025 in a dose-dependent manner. SP600125 also reduced MCP-1 mRNA stability: the half-life of MCP-1 mRNA was approximately 5 hours in cells treated with Ang II only, but was reduced to 2 hours when treated with a combination of Ang II and SP600125.

Conclusions: These results show that the JNK/AP-1 pathway is involved in the expression of MCP-1 and TGF-β, and in extracellular matrix production. JNK is an important therapeutic target for glomerulonephritis and glomerulosclerosis.

                                  World J Pediatr 2010;6(2):169-176

  [Abstract] [Full Text] [PDF]  
Case reports:
Familial crossed polysyndactyly in four generations
  Pooja Dewan, Nitin Agarwal, Preeti Dewan, Vineet Batta

Background: Polydactyly is the most common malfor-mation of the limbs. "Crossed" polydactyly of hands and feet, i.e., preaxial in one and postaxial in the other, is extremely rare. It has not been included in the standard classification of hand and feet anomalies.

Methods: We report an Indian family with 7 affected members across 4 generations who had "crossed polysyndactyly". All but one affected member had involvement of all four limbs. There were no other congenital anomalies in any of the family members.

Results: Familial crossed polysyndactyly appeared to follow an autosomal dominant transmission. This is probably the first case of familial crossed polysyndactyly without any associated anomalies.

Conclusion: Familial crossed polysyndactyly is a rare malformation and all family members should be screened for other congenital malformations.

Key words: familial; polysyndactyly

World J Pediatr 2010;6(2):177-180

  [Abstract] [Full Text] [PDF]  
Agrobacterium radiobacter bacteremia in a child with acute lymphoblastic leukemia
  Elpis Mantadakis, Anna Kondi, Athanassia Christidou, Maria Kalmanti

Background: Agrobacteria are Gram-negative tumorigenic plant pathogens that rarely cause infections in humans.

Methods: The authors describe a 7-year-old boy with acute lymphoblastic leukemia who carried a central venous catheter and developed bacteremia due to Agrobacterium radiobacter (A. radiobacter).

Results: Microbiological cure was achieved after administration of systemic ceftriaxone along with gentamicin lock therapy to the central venous catheter for 10 days. Catheter removal was not required, and the patient has not relapsed with bacteremia due to the same pathogen for more than 6 months.

Conclusions: A. radiobacter is an emerging pathogen affecting immunocompromised children, particularly those with leukemia who carry central venous catheters. Although it has a low virulence, erratic susceptibility patterns, and high frequency of resistance to many antibiotics, ceftriaxone appears to be successful in treatment of most cases. Catheter removal for the clearance of bloodstream infections due to A. radiobacter may not be required in selected patients like the present case.

Key words: Agrobacterium radiobacter; bacteremia; central venous catheters; leukemia

                  World J Pediatr 2010;6(2):181-184

  [Abstract] [Full Text] [PDF]  
Letter to the Editors:
Vitamin D supplementation could reduce risk of sepsis in infants
  William B. Grant
  [Abstract] [Full Text] [PDF]  
World Journal of Pediatric Surgery
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