World Journal of Pediatrics

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  Article: 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
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  ABSTRACT: BackgroundAlthough 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. MethodsFive 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. ResultsThe 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). ConclusionMorning 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 wordschildren-urinary copper/zinc ratio-Wilson’s disease-24-hour urinary copper excretion
  World Journal of Pediatrics 04/2012; 6(2):148-153.
• Article: Dendritic cells modifi cation during sublingual immunotherapy in children with allergic symptoms to house dust mites

  Federica Angelini, Valentina Pacciani, Stefania Corrente, Romina Silenzi, Alessandra Di Pede, Antonella Polito, Carla Riccardi, Silvia Di Cesare, Marie Laure Yammine, Paolo Rossi, Viviana Moschese, Loredana Chini
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  ABSTRACT: BackgroundThe importance of dendritic cells (DCs) in the initiation of the Th2-mediated inflammatory response to allergens is well known and more recently it has been proposed that DCs have a pivotal role in maintaining tolerance to allergens. The aim of this study was to investigate whether the success of sublingual immunotherapy (SLIT) in allergic asthma is mediated by the induction of changes of DCs functions. MethodsTen children with allergic asthma sensitive to house dust mite were studied before and after 12 months of SLIT. Immature DCs were derived from peripheral blood monocytes cultured for 6 days in presence of interleukin (IL)-4 and GM-CSF and stimulated with lipopolysaccharide for the last 24 hours to induce maturation. ResultsAfter 12 months of SLIT, mature DCs derived from SLIT-treated patients showed a statistically significant defect of CD86 up-regulation, an increase of IL-10, and a reduction of IL-12 production. ConclusionSLIT induces changes in DCs functions that might be responsible for an impairment of T cell activation or drive T cells towards a regulatory activity, thus restoring immune tolerance to allergens. Key wordsallergy–CD86–dendritic cells–IL-10–sublingual immunotherapy
  World Journal of Pediatrics 04/2012; 7(1):24-30.
• Article: Prevalence of childhood asthma and allergies in Serbia and Montenegro

  Zorica Živković, Zoran Vukašinović, Sofija Cerović, Suzana Radulović, Snežana Živanović, Eva Panić, Mila Hadnadjev, Omer Adžović
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  ABSTRACT: BackgroundThis is the first multi-center epidemiological study on the prevalence of childhood asthma in Serbia and Montenegro. The International Study of Asthma and Allergies in Childhood (ISAAC) phase 3 is a large epidemiological multinational and multicentric study on the prevalence of asthma, allergic rhinitis and eczema in children. MethodsThe 12-month period prevalence of asthma, allergic rhinitis and eczema was calculated using an ISAAC phase 3 questionnaire for two age groups: 6–7 years old and 13–14 years old. ResultsIn the 13 485 children from five study centers who responded to the questionnaire, the prevalence for childhood asthma ranged from 2.5% to 9.8%, for allergic rhinoconjunctivitis (hay fever) from 4.6% to 21%, and for eczema from 8.2% to 17.2%. The prevalence of current wheezing was high in both age groups (16.5% and 12.4% respectively). ConclusionsThe prevalence of asthma is higher in 6–7 years old school children in the urban and largest cities of Belgrade and Nis, and in 13–14 years old children in Podgorica. The prevalence of asthma, allergic rhinitis and eczema in the school children of Serbia and Montenegro seems similar to that of other countries in Central and South-Eastern Europe. Key wordsasthma–children–eczema–prevalence–rhinitis
  World Journal of Pediatrics 04/2012; 6(4):331-336.
• Article: Kaposi’s varicelliform eruption (eczema herpeticum) in an infant

  Sumit Mehndiratta
  World Journal of Pediatrics 04/2012; 6(3):282-282.
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  Article: Psychological effects of earthquakes in children: prospects for brief behavioral treatment

  Ebru Şalcıoğlu, Metin Başoğlu
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  ABSTRACT: BackgroundTreatment of child earthquake survivors is a relatively less investigated issue in disaster research. A review of the evidence on the mental health effects of earthquakes, risk factors, and findings from treatment studies may provide useful insights into effective treatment of traumatized children. Data sourcesStudies of child and adolescent earthquake survivors included the PILOTS database (electronic index for literature on psychological trauma) and relevant evidence from various studies of adult earthquake survivors. ResultsEvidence points to elevated rates of posttraumatic stress disorder (PTSD), depression, and earthquake-related fears in children and adolescents. Traumatic stress appears to be mediated by loss of control over fear induced by exposure to unpredictable and uncontrollable earthquakes. This implies that interventions enhancing sense of control over fear are likely to be most effective. Recent studies indeed show that a control focused behavioral treatment (CFBT) involving mainly encouragement for self-exposure to feared situations is highly effective in facilitating recovery from earthquake trauma. Evidence also suggests that CFBT can be delivered through booklets and similar media. ConclusionsPilot studies suggest that CFBT has promise in effective treatment of PTSD in children. Further research is needed to confirm these preliminary findings and to develop self-help tools for children.
  World Journal of Pediatrics 04/2012; 4(3):165-172.
• Article: Management of subependymal giant cell tumors in tuberous sclerosis complex: the neurosurgeon’s perspective

  Berhouma Moncef
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  ABSTRACT: BackgroundTuberous 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 intraventricular 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 sourcesBased 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. ResultsSGCTs 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. ConclusionsAn 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 wordscerebral ventricle-hydrocephalus-intraventricular tumor-microsurgery-subependymal giant cell astrocytoma-tuberous sclerosis
  World Journal of Pediatrics 04/2012; 6(2):103-110.
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  Article: Asthma School Program in children and their parents

  Zorica Živković, Snežana Radić, Sofija Cerović, Zoran Vukašinović
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  ABSTRACT: BackgroundThis study was undertaken to analyze the clinical efficiency of Asthma Education Intervention (AEI, Asthma School) in children and their parents, a program was designed to produce acceptable asthma knowledge and to improve the treatment. MethodsThis study assessed the effectiveness of an educational intervention within 12 months after attending Asthma School. The study was designed as a population based cohort study. The project endorsed by the European Respiratory Society (ERS) Educational Grant was launched in 2004 and finished in 2006, but the Asthma School continued working. Three hundred and two asthmatics recruited during hospital treatment of acute asthma exacerbation completed the study together with their parents. The intervention group of 231 asthmatics received full Asthma School program. The non-intervention group enrolled 71 asthmatics receiving usual instructions for asthma management. Clinical and educational outcomes were investigated immediately after completion of the program and 12 months later. ResultsSignificant achievements were found in the intervention group in asthma knowledge (baseline score 63%, 82% after 12 months, P<0.05), compliance (70% before, 90% after), and inhalation technique (20% before, 70% after). No change was found in the attitude and behavior regarding asthma prognosis in adolescent patients and parents. ConclusionThis study together with others clearly confirm the effectiveness of educational intervention for childhood asthma.
  World Journal of Pediatrics 04/2012; 4(4):267-273.
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  Article: Analysis of renal impairment in children with Wilson’s disease

  Xiao-Hui Zhuang, Ying Mo, Xiao-Yun Jiang, Shu-Mei Chen
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  ABSTRACT: BackgroundSince the diverse manifestations of renal impairment appear in different periods of Wilson’s disease, misdiagnosis or missed diagnosis is not rare. This study was undertaken to find the clinical features of renal impairment in children with Wilson’s disease or hepatolenticular degeneration (HLD). MethodsEighty-five children with HLD who had been treated at our department between January 1991 and June 2006 were retrospectively studied. The clinical data of 25 patients with renal impairment were analyzed. ResultsIn the 85 HLD patients, 34 had renal impairment. Nine of the 34 patients with D-penicillamine treatment were excluded. In the remaining 25 patients, 7 had initiated symptoms of renal impairment, 5 of them with edema, 1 with gross hematuria, and 1 with acute hemolysis and acute renal failure. Twelve of the 25 patients had proteinuria, 14 had hematuria, and 5 had both proteinuria and hematuria. Urine glucose was positive in 4 patients, urine N-acetyl-β-D-glucosaminidase (NAG) increased in 5, and urine β2-microglobulin increased in 6. Urine red blood cell (RBC) phase was detected in 7 patients, including glomerular hematuria in 5 patients and non-glomerular hematuria in 2. Blood urea nitrogen and creatinine increased in 1 patient. B-ultrasound revealed bilaterally enlarged kidneys in 3 patients. Kidney biopsy showed diffuse mesangial proliferation and IgA deposit in mesangial region in 1 patient. All of the 25 patients had cornea K-F ring and the level of ceruloplasmin decreased. Six patients had a family history of HLD. ConclusionsThe manifestations of renal impairment with HLD are varied. HLD should be excluded from patients with unexplained renal impairment, while those with HLD should take examinations of the kidney to identify renal impairment. We propose that renal function and urinalysis should be checked regularly in patients receiving treatment of D-penicillamine.
  World Journal of Pediatrics 04/2012; 4(2):102-105.
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  Article: Theophylline-associated status epilepticus in an infant: pharmacokinetics and the risk of suppository use.

  Zenichiro Kato, Atsushi Yamagishi, Mitsuhiro Nakamura, Naomi Kondo
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  ABSTRACT: Theophylline has been widely used to treat asthma, but recent studies have revealed that the possible risks for seizure may result in the revision of the therapeutic guidelines. An 8-month-old boy who had been treated with oral sustained-release theophylline and additional aminophylline suppository was hospitalized. A combination of diazepam, lidocaine and thiopental was required to stop his convulsion. The pharmacokinetic study indicated that the usage of a sustained-release formula should not usually be over 15 mg/ml, but the additional use of an aminophylline suppository elevated the concentration to over 20 mg/ml and resulted in the severe adverse effects. The parents of children and also physicians should be educated to ensure the proper use of the suppository formula.
  World Journal of Pediatrics 11/2009; 5(4):316-8.
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  Article: Nonalcoholic fatty liver disease in children living in the obeseogenic society.

  H Hesham A-Kader
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  ABSTRACT: The problem of obesity in children has grown considerably in recent years in the United States as well as the rest of the world. This has resulted in a marked increase in the prevalence of nonalcoholic liver disease in the pediatric age group. Nonalcoholic fatty liver disease (NAFLD) is currently the most common hepatic disorder seen in pediatric hepatology practice. We have reviewed the most recent literature regarding the prevalence, pathogenesis as well as the most recent advances in the diagnostic and therapeutic modalities of NAFLD in children. NAFLD affects a substantial portion of the population including children. The rising incidence of NAFLD, nonalcoholic steatohepatitis (NASH) and cirrhosis emphasizes the need for effective treatment options. The lack of complete understanding of the pathogenesis of NAFLD still limits our ability to develop novel therapeutic modalities that can target the metabolic derangements implicated in the development of the disorder.
  World Journal of Pediatrics 11/2009; 5(4):245-54.
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  Article: Ileal duplication with extensive gastric heterotopia in a girl.

  Hui-Min Jia, Ke-Ren Zhang, Ri-Bin Qu
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  ABSTRACT: Gastrointestinal duplications are rare congenital abnormalities known to occur at any level of the alimentary tract from the mouth to the anus. The cause of intestinal duplication has not been established. Several theories have been put forward to explain different types of duplications. Some of these duplications are large sized and giant, and only 4 cases have been reported. A 4-year-old girl was referred to our hospital with a history of abdominal pain, abdominal distension, and diarrhea mixed with black blood for 20 days. Technetium-99m scintigraphy identified heterotopic gastric mucosa at the middle and lower abdominal region. Enteric duplication was suspected. Operatively, duplication was found to be located at the ileum with abnormal hypertrophy in shape, 50 cm of the ileum was resected, and an ileoileal end-to-end anastomosis was made. Stomach-like mucosa and some ring structures were identified instead of the normal intestinal mucosa when opening this ileal duplication. Microscopically, most of mucosa showed gastric corpusfundic glands. This is an unusual case of enteric duplication. Ultrasonography, computed tomography and technetium-99m scintigraphy are helpful in the diagnosis of duplication.
  World Journal of Pediatrics 11/2009; 5(4):322-4.
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  Article: Internalizing correlates of dyslexia.

  Daniele Mugnaini, Stefano Lassi, Giampaolo La Malfa, Giorgio Albertini
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  ABSTRACT: Over the last ten years a considerable amount of literature has described the socio-emotional discomfort that is often associated with learning disorders at all ages, but a comprehensive review about internalizing symptoms in dyslexia is needed. Medical and psychological search engines (PubMed, PsychArticles and Academic Search Elite) were used to identify all those studies published in peer-reviewed journals, relative to the association of reading difficulties, dyslexia, or learning disorders/disabilities, and internalizing symptoms, anxiety, or depression. The present review of studies confirms dyslexia as a specific risk factor for an increased internalizing, anxious and depressive symptomatology. The severity of dyslexia, its comorbidity with attention deficit disorder/hyperactivity disorder, the level of perceived social support and female gender are some of the factors that mostly influence its psycho-social outcomes. Findings of this review confirm that suitable social, health and school policies aimed at identifying and treating dyslexia as a cause of discomfort are called for, and confirm the clinical need to assess and contrast additional risk factors that may increase the probability of this suffering in dyslexic students.
  World Journal of Pediatrics 11/2009; 5(4):255-64.
• Article: Foreskin development in 10 421 Chinese boys aged 0-18 years.

  Chao Yang, Xing Liu, Guang-Hui Wei
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  ABSTRACT: Few studies on foreskin development and the practice of circumcision have been done in Chinese boys. This study aimed to determine the natural development process of foreskin in children. A total of 10 421 boys aged 0 to 18 years were studied. The condition of foreskin was classified into type I (phimosis), type II (partial phimosis), type III (adhesion of prepuce), type IV (normal), and type V (circumcised). Other abnormalities of the genitalia were also determined. The incidence of a completely retractile foreskin increased from 0% at birth to 42.26% in adolescence; however, the phimosis rate decreased with age from 99.7% to 6.81%. Other abnormalities included web penis, concealed penis, cryptorchidism, hydrocele, micropenis, inguinal hernia, and hypospadias. Incomplete separation of foreskin is common in children. Since it is a natural phenomenon to approach the adult condition until puberty, circumcision should be performed with cautions in children.
  World Journal of Pediatrics 11/2009; 5(4):312-5.
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  Article: Effect of melatonin on proliferation of neonatal cord blood mononuclear cells.

  Wei Zhou, Ping Wang, Li Tao
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  ABSTRACT: Pineal melatonin (MLT) is a neuroendocrine hormone that possesses a wide variety of biological effects. MLT regulation of the immune system has been studied in recent years. But very little is known about MLT interaction with neonatal cord blood mononuclear cells (CBMCs) and the lymphocyte immune system in neonates. This study was designed to investigate the proliferative effects of MLT on CBMCs and peripheral blood mononuclear cells (PBMCs). Cord blood samples were collected from 10 normal full-term infants at the Guangzhou Maternal and Infant Hospital, China. Ten samples of adult peripheral blood were also collected from healthy volunteers. (3)H-thymidine ((3)H-TdR) incorporation was used to analyze the influence of MLT on proliferation of CBMCs. The effects of MLT on proliferation of CBMCs and PBMCs were compared. (3)H-TdR incorporation increased in a dose-dependent manner with varying MLT concentrations (50 pg/ml-50 ng/ml), but peaked at 5 ng/ml. After incubation with MLT (5 ng/ml), interleukin-2 (IL-2, 50 ng/ml), MLT+phytohemagglutinin (PHA, 5 microg/ml), and MLT+IL-2, respectively in CBMCs media, (3)H-TdR incorporation rates were 114 327+/-52 863, 16 087+/-9006, 118 360+/-59 207, and 17 682+/-7391. Compared to the control cell suspension (14 133+/-8688), (3)H-TdR incorporation rates of the MLT and MLT+PHA groups were significantly increased (t=5.9143, P<0.001; t=5.5078, P<0.001). (3)H-TdR incorporation was not different between the IL-2 and MLT+IL-2 groups (t=0.4983, P>0.05; t=0.9839, P>0.05). PHA treatment (110 397+/- 48 663) presented no difference in (3)H-TdR incorporation compared to the MLT or MLT+PHA groups (t=0.1730, P>0.05; t=0.3286, P>0.05). (3)H-TdR incorporation was significantly greater in CBMCs than in PBMCs cultures after addition of various stimulators to the culture media. MLT promoted proliferation of PBMCs and also enhanced proliferation of CBMCs. The proliferative effects of MLT were greater on CBMCs than on PBMCs.
  World Journal of Pediatrics 11/2009; 5(4):300-3.
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  Article: Celiac disease in 87 children with typical and atypical symptoms in Black Sea region of Turkey.

  Gönül Dinler, Erdal Atalay, Ayhan Gazi Kalayci
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  ABSTRACT: Celiac disease presents with a spectrum of clinical disorders. The variety of clinical presentations largely depends on age and extraintestinal findings. This study aimed to determine typical and atypical cases according to presenting symptoms and to evaluate their biochemical and pathological parameters. Eighty-seven patients with celiac disease in our unit between 2000 and 2007 were reviewed. Their diagnosis was made by serological and histological examination. The patients were divided into two groups according to their typical or atypical symptoms. The mean age of the patients at diagnosis was 8.2 years (range, 1-18 years), but patients presenting with typical symptoms were younger than those presenting with atypical symptoms. The patients in the two groups did not differ significantly in sex, weight and height Z scores except age. Diarrhea (96.3%), abdominal distention (65.4%) and failure to thrive (60%) were the most common clinical presentations in the typical group, and short stature (62.5%) and anemia (31.2%) were the most common in the atypical group. Total/subtotal villous atrophy was significantly higher in the typical group than in the atypical group. Many children with celiac disease show an atypical form. The understanding of presentations of celiac disease may prevent delayed diagnosis. Celiac disease should be specially investigated in patients with recurrent iron deficiency anemia, short stature and autoimmune disorders.
  World Journal of Pediatrics 11/2009; 5(4):282-6.
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  Article: Interventional occlusion of congenital vascular malformations.

  Chun-Hong Xie, Cheng-Sen Xia, Fang-Qi Gong, Yin-Bao Zhou, Wei-Hua Zhu
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  ABSTRACT: New materials and devices have been used in the management of cardiac malformations. In this paper, we present our experience with interventional occlusion of congenital vascular malformations. Between January 1997 and December 2005, 139 patients with congenital vascular malformations who had undergone interventional occlusion in the Children's Hospital, Zhejiang University School of Medicine were studied. The clinical data of the patients were retrospectively reviewed including pre-operative evaluation, surgical procedures, immediate complete closure rate, short-term complications, and short-term outcome. Of the 139 patients, 126 had patent ductus arteriosus, and successful deployment was achieved in 121 of the 126 patients (96%, 121/126). Six patients had coronary artery fistula and 14 different coils were used for embolization; the immediate complete closure rate was 83.3%, and the complete closure rate after one month was 100%. The abnormal vessels of 3 patients with pulmonary sequestration were completely occluded using four 0.038-inch Gianturco coils. In 3 patients with aortopulmonary collaterals, 14 abnormal vessel branches were occluded with sixteen 0.038-inch Gianturco coils, reaching a closure rate of 100%. One patient with pulmonary arteriovenous fistula was occluded successfully with two 0.038-inch Gianturco coils. Transcatheter closure using coils is a safe and effective alternative to surgical ligation in the management of congenital vascular malformations in children. Selection of appropriate coils is important to achieve a better outcome.
  World Journal of Pediatrics 11/2009; 5(4):296-9.
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  Article: Congenital rubella syndrome: pattern and presentation in a southern Nigerian tertiary hospital.

  Innocent Ocheyana George, Angela Ine Frank-Briggs, Raphael Sonny Oruamabo
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  ABSTRACT: Congenital rubella syndrome (CRS) resulting from maternal rubella infection can result in miscarriages, still birth and rubella infection of the infant. The aim of this study was to evaluate the pattern and presentation of CRS over an 8-year period as seen in the University of Port-Harcourt Teaching Hospital (UPTH), Nigeria. The medical records of all cases of CRS presenting to the Pediatric Department of UPTH from January 2000 to December 2007 were reviewed. There were 16 394 babies delivered in UPTH from January 2000 to December 2007. Of these babies, 19 were clinically diagnosed as having CRS, but none had laboratory confirmation. They had a mean age of 5.1 +/-3.2 months (range: 1-11 months). Seventy-five percent of their mothers had presumed rubella infection during the first trimester of pregnancy. Cataract was noticed to be the most prominent systemic manifestation in 18 of the 19 babies. Other common manifestations included hearing impairment (n=14), microcephaly (n=13), patent ductus arteriosus (n=11), and low birth weight (n=10). A surge was observed in the number of babies with CRS from 2004 to 2007; however, this was not statistically significant (chi (2)=8.68, P=0.28). Unfortunately, long-term outcome of the 19 babies was not available. CRS is commonly diagnosed at our center. Thus, mounting effective surveillance for CRS and considering the inclusion of rubella vaccine in Nigeria are of extreme importance.
  World Journal of Pediatrics 11/2009; 5(4):287-91.