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Vol 5, No 2
Vol 5, No 2 May 2009 ISSN 1708-8569
 
Commentary
Editorial
Review articles
Original articles
Case reports
   
Commentary:
Pediatric surgical oncology in China: present and future
  Jin-Zhe Zhang
 

Beijing, China

Author Affiliations: Department of Pediatric Surgery, Beijing Children's Hospital, Capital Medical University, Beijing 100045, China (Zhang JZ)

Corresponding Author: Jin-Zhe Zhang, MD, Honorary Fellow of the Royal College of Surgeons (HonFRCS), Member of Chinese Academy of Engineering (MCAE), Professor and Senior Consultant of Pediatric Surgery, Beijing Children's Hospital, Beijing 100045, China (Email: jinzhezhang@sina.com)

doi:10.1007/s12519-009-0018-1

  [Abstract] [Full Text] [PDF]  
Editorial:
Creating a global climate for pediatric cardiac care
  Jacques G Leblanc
   

Vancouver, Canada

Author Affiliations: British Columbia Children's Hospital, 4480 Oak Street, Suite AB307, Vancouver, BC V6H 3V4, Canada (Leblanc JG)

Corresponding Author: Jacques G Leblanc, MD, FRCSC, Clinical Professor, British Columbia Children's Hospital, 4480 Oak Street, Suite AB307, Vancouver, BC V6H 3V4, Canada (Tel: +1-604-875-3165; Fax: +1-604-875-3159; Email: jleblanc@cw.bc.ca)

doi:10.1007/s12519-009-0019-0

  [Abstract] [Full Text] [PDF]  
Review articles:
Disorders of sex development: update on the genetic background, terminology and risk for the development of germ cell tumors
  Martine Cools, Leendert HJ Looijenga, Katja P Wolffenbuttel, Sten LS Drop
 

Background: Considerable progress has been made  on genetic mechanisms involved in disorders of sex development and on tumor formation in dysgenetic gonads. Clinical and psychological outcome of patients are, as far as evaluated, unsatisfactory at present. Guidelines are emerging in order to optimize long-term outcome in the future.

Data sources: The information obtained in this review is based on recent original publications and on the experience of our multidisciplinary clinical and research group.

Results: This review offers an update on our knowledge concerning gene mutations involving in disorders of sex development, on the renewed nomenclature and classification system, and on the mechanisms of tumor development in patients.

Conclusions: The consensus meeting on disorders of sex development has renewed our interest in clinical studies and long-term outcome of patients. Psychological research emphasizes the importance to consider male gender identity wherever possible in cases of severe undervirilization. Patient advocacy groups demand a more conservative approach regarding gonadectomy. Medical doctors, scientists and governmental instances are increasingly interested in the set-up of international research collaborations. As a consequence, it is expected that new guidelines for the optimal care of patients will be proposed in the coming years.

  [Abstract] [Full Text] [PDF]  
Functional gastrointestinal disorders: past and present
  Rana Fayez Ammoury, Marian Del Rosario Pfefferkorn, Joseph Marino Croffie
 

Background: Chronic abdominal pain is a common complaint in childhood and adolescence. Despite decades of clinical observations and research, it still poses a challenge to pediatric health care professionals. The aim of this review is to highlight the epidemiology of pediatric chronic abdominal pain and to describe the pathogenesis of this disorder, its clinical manifestations, evaluation and therapeutic options.

Data sources: Articles on chronic abdominal pain in the recent years from PubMed, MEDLINE, and reference textbooks were reviewed.

Results: Chronic abdominal pain, a functional gastro-intestinal disorder (FGID), is a multifactorial condition that results from a complex interaction between psychosocial and physiologic factors via the brain-gut axis. A thorough history coupled with a complete physical examination and normal screening studies rule out an organic cause in 95% of the cases. It is highly important for the physician to establish a trusting relationship with the child and parents because successful treatment including modification of physical and psychological stress factors, dietary changes, and drug therapy depends greatly on education, reassurance and active psychological support.

Conclusions: FGIDs are a cause of great anxiety, distress and morbidity in children as well as adults. As our understanding of these conditions improves, our therapeutic interventions will progress not only to overcome them but also to intervene early in the disease course so as to limit long-term impact.

Key words: abdominal pain; bowel syndrome; gastrointestinal disorders

                World J Pediatr 2009;5(2):103-112

  [Abstract] [Full Text] [PDF]  
Original articles:
Postoperative sepsis in infants below 6 months of age
  Ulf Kessler, Marc Ebneter, Zacharias Zachariou, Steffen Berger
   

Background: Sepsis is a threatening postoperative complication especially in small infants. Regarding the advances in perinatal medicine, its incidence is unknown to date. We aimed to investigate the incidence, risk factors, laboratory findings and outcome of postoperative sepsis in infants younger than 6 months old.

Methods: We examined postoperative sepsis in babies below 6 months of age during a 4-year period at a tertiary pediatric institution.

Results: The rate of postoperative sepsis was 6.9%. Laparotomy with enterotomy, thoracotomy and diaphragmatic hernia repair (P<0.05, respectively) as well as low postnatal age and long operation time (P<0.001, respectively) were correlated with the incidence of sepsis. Significant independent predictors for the development of sepsis were the presence of a central venous catheter and perioperative antibiotic treatment (P<0.001, respectively). Coagulase negative Staphylococci were the major infecting organism associated with postoperative sepsis, accounting for 53% of monomicrobial infections. Complete blood counts with differential were not different between infants with sepsis and controls, who had undergone the same surgical procedures. Outcome was favorable in all cases; however, the length of hospital stay was significantly longer in sepsis patients (P<0.05).

Conclusions: Postoperative sepsis syndrome is a frequent complication in infants below 6 months of age and causes significant prolongation of hospital stay. Adequate prevention and therapeutic strategies warrant further prospective investigations.

Key words: central venous catheter; infant; postoperative care; sepsis

                  World J Pediatr 2009;5(2):113-117

  [Abstract] [Full Text] [PDF]  
Ultrasonographic characteristics of urolithiasis in children exposed to melamine-tainted powdered formula
  Yu He, Guo-Ping Jiang, Lei Zhao, Jing-Jing Qian, Xiu-Zhen Yang, Xiao-Ying Li,
 

Background: Since the outbreak of renal diseases among children who had consumed melamine-tainted powdered formula in 2008, urinary system ultrasound examination was carried out for these children at our hospital. This study aimed to investigate the ultrasonographic characteristics of urolithiasis caused by melamine-tainted powdered formula.

Methods: From September 12 to October 9, 2008, urinary system ultrasound examination for children after consumption of melamine-tainted powdered formula  was carried out at the Children's Hospital of Zhejiang University, China. A total of 15 577 infants and children were subjected to the examination. A questionnaire with items including demographic characteristics, history of exposure and clinical symptoms were completed before examination.

Results: Among the 15 577 children, 7988 were boys and 7589 girls with age ranging from 1 month to 15 years (mean age, 22 months). Urolithiasis was found in 562 patients with an identification rate of 3.6%. Urolithiasis was identified mainly in the children aged 36 months or younger (88.6%). Unilateral kidney calculi were found in 431 children and bilateral kidney calculi in 131 children. Eleven of these children also had ureteric calculi, 1 had bladder calculi, 2 had urethral calculi, and 2 had gallbladder calculi. Fifteen children had urinary tract obstruction. Ultrasonographic characteristics of these melamine calculi were different from the calcium oxalate calculi, i.e., the lesions were less echogenic, had a more "sandy" appearance and were less dense. The posterior portion of the calculi could be observed and was accompanied with a feeble or absent acoustic shadow.

Conclusions: Melamine-induced calculi were ultra-sonographically less echogenic, sand-like and discrete; the posterior portion of the calculi was accompanied with a feeble or absent acoustic shadow. It might be a new type of urolithiasis in children.

Key words: formula; infants; melamine; ultrasonography; urolithiasis

                   World J Pediatr 2009;5(2):118-121

  [Abstract] [Full Text] [PDF]  
Pediatric reference intervals for soluble transferrin receptor and transferrin receptor-ferritin index
  Cara Lianne Ooi, Nathalie Lepage, Ed Nieuwenhuys, Ajay Parkash Sharma, Guido Filler
 

Background: Recent studies showing an improved diagnosis of iron deficiency (ID) with soluble transferrin receptor (sTfR) and sTfR-ferritin index did not take into account the age-dependency of sTfR and ferritin. Moreover, there is a paucity of data on pediatric reference intervals for sTfR and sTfR-ferritin index.

Methods: A study cohort of 436 apparently healthy children was analyzed to establish reference intervals for ferritin, transferrin, sTfR and sTfR-ferritin index. To account for age-dependency, standard deviation scores (Z-scores) for these markers were calculated. The association between these parameters and C-reactive protein (CRP) was analyzed.

Results: The Z-scores of ferritin, transferrin and sTfR had a significant association with CRP, whereas the Z-score of sTfR-ferritin did not correlate with CRP. The reference intervals of these parameters were reported.

Conclusion: Among the different markers of ID, the Z-scores of sTfR, transferrin and ferritin, but not sTfR-ferritin index, associate with the inflammatory status.

Key words: C-reactive protein; ferritin; reference interval; soluble transferrin receptor; soluble transferrin receptor-ferritin index; transferrin

                   World J Pediatr 2009;5(2):122-126

  [Abstract] [Full Text] [PDF]  
Serum immunoglobulin G, M and IgG:IgM ratio as predictors for outcome of childhood nephrotic syndrome
  Ranjit Ranjan Roy, Eliza Roy, Mohammed Habibur Rahman,
 

Background: Nephrotic syndrome is an immune mediated disorder of the kidney associated with T cell dysfunction and secondary disturbance of B cell with changes in levels of immunoglobulin and IgG:IgM ratio. These changes in immunoglobulin levels can be used as a proxy marker to understand the clinical variety and prognosis of nephrotic syndrome.

Methods: We studied 43 children with nephrotic syndrome during January 2003 to January 2005 in the Pediatric Nephrology Unit, Department of Pediatrics, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh. Blood samples were collected from the 43 patients, and serum levels of IgG, IgM and IgG:IgM were measured by liquid phase immunoprecipitation assay. Another 20 healthy children attending the laboratory for blood grouping and hepatitis B screening test were enrolled as controls.

Results: In the 43 children with nephrotic syndrome, 24 had steroid sensitive nephrotic syndrome (SSNS) and 19 steroid resistant nephrotic syndrome (SRNS). Compared with healthy children, the IgG level was low, IgM level was high, and IgG:IgM ratio was low (P<0.05). The serum IgG level and IgG:IgM ratio were significantly lower in children with SRNS and in children with frequent relapse (FRNS) combined with steroid dependent nephrotic syndrome (SDNS) than in those with infrequent relapse nephrotic syndrome (IFRNS) (P<0.05, respectively).

Conclusions: Management of different nephrotic syndromes is based on the levels of immunoglobulins along with clinical and biochemical parameters. The decrease of IgG level as a predictive marker for unfavorable prognosis of nephrotic syndrome in children needs further evaluation in larger scale studies.

Key words: immunoglobulins; nephrotic syndrome; prognosis

  World J Pediatr 2009;5(2):127-131

  [Abstract] [Full Text] [PDF]  
Age of puberty in a representative sample of Iranian girls
  Hessam Hassanzadeh Kashani, Morvarid Sadat Kavosh, Ammar Hassanzadeh Keshteli, Mehdi Montazer, Nooshin Rostampour, Roya Kelishadi, Keivan Shariatnejad, Pooneh Memar-Ardestani, Saeyed Mohsen Hosseini, Zahra Abdeyazdan, Mahin Hashemipour
  Isfahan, Iran

Author Affiliations: Medical Students Research Center, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran (Kashani HH, Kavosh MS, Keshteli AH); School of Medicine, Iran University of Medical Sciences, Tehran, Iran (Montazer M); Isfahan Endocrine & Metabolism Research Center, Isfahan University of Medical Sciences, Isfahan, Iran (Rostampour N, Memar-Ardestani P, Abdeyazdan Z, Hashemipour M); Isfahan Cardiovascular Research Center, Isfahan University of Medical Sciences, Isfahan, Iran (Kelishadi R); Department of Biostatistics, Faculty of Health, Isfahan University of Medical Sciences, Isfahan, Iran (Shariatnejad K, Hosseini SM)

Corresponding Author: Mahin Hashemipour, MD, Professor of Pediatric Endocrinology, Isfahan Endocrine & Metabolism Research Center, Isfahan University of Medical Sciences, Sedigheh Tahereh Research Center, Khorram Street, Jomhouri Square, Isfahan, 81876-98191, Iran (Tel: +98 (311) 3359933; Fax: +98 (311) 3373733; Email: hashemipour@med.mui.ac.ir)

doi:10.1007/s12519-009-0026-1

Background: To obtain normal references for growth and pubertal development in a given population, assessment of pubertal stages is of great importance. This study aimed to determine the age of appearance of secondary sexual characteristics in a representative sample of Iranian girls.

Methods: This cross-sectional study was conducted during 2005-2006 in 3192 girl students, aged 6-17 years, in Isfahan, Iran. Participants were selected by multistage random cluster sampling from school students. Secondary sexual characteristics were evaluated by inspection and palpation, and were recorded according to Tanner staging. The self-reported date of menarche (if any) was recorded as well. Data were analyzed with Probit analysis based on the status quo method.

Results: The median ages (10th-90th percentile) of Tanner stage 2 breast development (B2) and Tanner stage 2 pubic hair growth (PH2) were 10.14 years (8.33-11.95 years) and 10.78 years (9.09-12.48 years), respectively. The ages of the 3rd percentile for B2 and PH2 were 7.48 and 8.29 years, respectively. The median age of menarche among the 3192 girls who had experienced menarche was 12.65 years (11.18-14.11 years).

Conclusions: The median age of puberty onset is 10.14 years, and the onset of puberty before 7.5 years is considered as precocious puberty in a representative sample of Iranian girls. The values obtained from the present study can provide baseline data for analysis of time trends, as well as for international comparisons.

Key words: menarche; precocious; pubarche; puberty; secondary sexual characteristics; telarche

                  World J Pediatr 2009;5(2):132-135

  [Abstract] [Full Text] [PDF]  
Neonatal screening for congenital hypothyroidism and phenylketonuria in China
  Jian-Ying Zhan, Yu-Feng Qin, Zheng-Yan Zhao
 

Background: Neonatal screening is helpful to prevent serious disabitily and sufferings caused by congenital or inherited disease. This study was to review the status of neonatal screening for congenital hypothyroidism (CH) and phenylketonuria (PKU) in China.

Methods: We analyzed data of neonatal screening for CH and PKU in the past two decades which were obtained from the national network of neonatal screening centers collected by the National Center for Clinical Laboratory.

Results: Of 18.8 million newborns screened from 1985 to 2007, 9198 were identified with CH, giving a prevalence of 1/2047. In 19.0 million newborns screened in the same period, 1638 had PKU, with a prevalence of 1/11 572. An increasing number of neonates have been subjected to neonatal screening in China annually during this period. Data from Zhejiang Neonatal Screening Center showed that the recall rate of neonates suspected with CH and PKU was 95.52% in 2007. Confirmatory tests were performed and treatments were initiated in most of the neonates with CH and PKU within a month after birth.

Conclusions: More governmental support at different levels is needed to make neonatal screening more efficient. The screening should be improved with a satisfactory control system including shorter time of report and a higher recall rate.

Key words: congenital hypothyroidism; neonatal screening; phenylketonuria

                   World J Pediatr 2009;5(2):136-139

  [Abstract] [Full Text] [PDF]  
Signal pathways in ouabain-induced proliferation of leukemia cells
  Jia-Wei Xu, Run-Ming Jin, En-Qin Li, Yan-Rong Wang, Yan Bai
 

Background: Cardiotonic steroids (CTSs) can bind to Na+/K+-ATPase and activate protein kinase cascades, resulting in changes in cell proliferation, differentiation or apoptosis in a cell-specific manner. We explored the participation of ouabain-activated signaling pathways in growth regulation of leukemia cells.

Methods: Lymphocytic leukemia Jhhan cells and megakaryocytic leukemia M07e cells were incubated at different concentrations of ouabain (0, 1 and 10 nmol) for 24 hours. Cell proliferation was measured by methyl thiazolyl tetrazolium (MTT) assay. To probe the role of ouabain-induced signaling in control of cell growth, we employed Src kinase inhibitor PP2 and the MEK inhibitor PD98059, respectively. The expression of Na+/K+-ATPase ¦Á1 subunit of leukemia cells was evaluated by RT-PCR and Western blotting.

Results: One nmol and 10 nmol ouabain promoted proliferation of both Jhhan and M07e cells. Ouabain also up-regulated the expression of Na+/K+-ATPase ¦Á1 subunit. Addition of either PP2 or PD98059 blocked the effects of ouabain on cell proliferation.

Conclusion: Ouabain activates Src and ERK1/2 pathways and regulates the proliferation of leukemia cells.

Key words: cell proliferation; leukemia; Na+/K+-ATPase; ouabain; signal transduction

                  World J Pediatr 2009;5(2):140-145

  [Abstract] [Full Text] [PDF]  
Case reports:
Duodenal heterotopic pancreas in a child
  Li-Duan Zheng, Qiang-Song Tong, Shao-Tao Tang, Zhi-Yong Du, Qing-Lan Ruan
 

Methods: An 8-year-old boy presented with upper abdominal pain and intermittent vomiting, without a history of melena, hematochezia, hematemesis, clay-colored stools, jaundice, hepatitis or dyscrasia. No specific medication or change in position relieved the pain. Based on the elevated serum amylase levels, and the findings of CT, barium meal X-ray examination, magnetic resonance imaging, and upper gastrointestinal endoscopy, a duodenal mass was diagnosed initiatively. Intraoperative frozen section analysis was performed for the diagnosis. The mass was dissected.

Results: Intraoperative frozen section analysis and routine pathological examination confirmed the diagnosis of duodenal heterotopic pancreas. The patient had an uneventful recovery and remained asymptomatic postoperatively during a follow-up period of 16 months.

Conclusions: Heterotopic pancreas should be considered in children with a duodenal mass and abdominal pain. Intraoperative frozen section analysis is helpful in the diagnosis of the disease. Surgical treatment of the lesion should be performed to prevent bleeding, ulceration, outlet obstruction or malignant degeneration.

Key words: child; duodenum; heterotopic pancreas

                 World J Pediatr 2009;5(2):146-148

  [Abstract] [Full Text] [PDF]  
Massive pan-gastrointestinal bleeding following cocaine use
  Troy Emanuel Gibbons, Kadria Sayed, George Joseph Fuchs
 

Background: An 18-year-old adolescent with cystic fibrosis developed massive gastrointestinal bleeding.

Methods: History, physical examination, upper and lower endoscopy and wireless capsule endoscopy were performed.

Results: Upper and lower endoscopy did not reveal cause of persistent bleeding. Wireless capsule endoscopy revealed pan-gastrointestinal ischemic injury. Further discussion with the patient revealed recent cocaine ingestion.

Conclusion: Most reported cases of gut injury following cocaine abuse describe juxtapyloric and colonic injury; this case demonstrates that ischemic gut injury after cocaine use can be extensive and may be the reason for the associated high mortality.

Key words: cocaine abuse; gastrointestinal bleeding; wireless capsule endoscopy

World J Pediatr 2009;5(2):149-151

  [Abstract] [Full Text] [PDF]  
Esophageal candidiasis in an immunocompetent girl
  Mohammed Yahya Hasosah, Mahmmod Showail, Ashraf Al-sahafi, Mohammed Satti, Kevan Jacobson
 

Background: Infections of the esophagus are rare and most commonly seen in children with immune suppression resulting from malignancy, chronic metabolic or infectious disease, or immunosuppressive drug therapy.

Methods: An 18-month-old girl on inhaled corticosteroid for bronchial asthma presented with coffee-ground emesis and melena. Upper endoscopy revealed yellow-white plaques scattered over the mucosa of the distal esophagus. Biopsy results showed chronic esophagitis with features of reflux disease. Gram staining of esophageal brushing showed pseudohyphae, and the culture was positive for candida species.

Results: The patient was treated with omeprazole 2 mg/kg per day and fluconazole 6 mg/kg per day for three weeks. Immunological workup was normal and she was negative for human immunodeficiency virus. Post treatment endoscopy showed normal appearance of esophageal mucosa and normal histology. At 12 months after treatment, the child remained asymptomatic and continued to thrive.

Conclusions: We believe that this child developed esophageal candidiasis secondary to long-term use of inhaled corticosteroid associated with immune suppression on a background of reflux esophagitis. Anti-acid and antifungal therapies are effective in the treatment.

Key words: esophageal candidiasis; reflux esophagitis; upper gastrointestinal bleeding

                                                                                                           World J Pediatr 2009;5(2):152-154
  [Abstract] [Full Text] [PDF]  
   
 
 
 
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