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Vol 5, No 4
Vol 5, No 4 November 2009 ISSN 1708-8569
 
Review articles
Original articles
Brief report
Case reports
   
Review articles:
Nonalcoholic fatty liver disease in children living in the obeseogenic society
  H Hesham A-Kader
   

Background: 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.

Data sources: 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.

Results: NAFLD affects a substantial portion of the population including children.

Conclusions: The rising incidence of NAFLD, non-alcoholic 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.

Key words: fatty liver; nonalcoholic fatty liver disease; obesity; pediatric liver disease

                   World J Pediatr 2009;5(4):245-254

  [Abstract] [Full Text] [PDF]  
Internalizing correlates of dyslexia
  Daniele Mugnaini, Stefano Lassi, Giampaolo La Malfa, Giorgio Albertini
   

Background: 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.

Data sources: 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.

Results: 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.

Conclusion: 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.

Key words: anxiety; dyslexia; internalizing symptoms; reading disability

                  World J Pediatr 2009;5(4):255-264

  [Abstract] [Full Text] [PDF]  
Vitiligo in children
  Talia Kakourou
  Background: Vitiligo is an acquired depigmentary disorder affecting around 1% of the world's population. In 25% of cases it has its onset prior to the age of 14 years.

Data sources: Articles on vitiligo in children published after 1995 were retrieved from PubMed. The prevalence, etiology, clinical presentation, differential diagnosis, treatment and management of vitiligo in children were summarized.

Results: Vitiligo is characterized by acquired, sharply demarcated depigmented macules or patches on the skin, the mucous membranes and/or white hair and it is mainly differentiated from congenital achromic skin lesions. It is frequently associated with various autoimmune diseases. Hashimoto's thyroiditis is the most common association in children. Information on the nature, possible causes and course of the disease leads to acceptance of the disorder and higher compliance with the treatment. The choice of medical treatment depends on the type, location and duration of lesions as well as the eagerness of the child and his/her parents to pursue therapy.

Conclusion: The management of childhood vitiligo includes information and reassurance of young patients and their parents on the disease, thyroid investigation, avoidance of trigger factors, topical treatment and proper follow-up.

Key words: management; thyroiditis; treatment; vitiligo

         World J Pediatr 2009;5(4):265-268

  [Abstract] [Full Text] [PDF]  
Original articles:
Diet intervention on obese children with hypertension in China
  Yan-Ping Wan, Ren-Ying Xu, Ying-Jie Wu, Zhi-Qi Chen, Wei Cai
   

Author Affiliations: Department of Clinical Nutrition, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China (Wan YP, Xu RY, Wu YJ, Chen ZQ); Clinical Nutrition Center, Xin Hua Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China (Cai W)

Corresponding Author: Yan-Ping Wan, Department of Clinical Nutrition, 145 Shan Dong Middle Road, Shanghai 200001, China (Tel: +86-21-53882227; Fax: +86-21-68383318; Email: wanyp204@yahoo.com.cn)

doi:10.1007/s12519-009-0051-0

Background: Obesity has made obesity-related diseases a worldwide problem. This study was undertaken to evaluate the effects of diet-oriented intervention on obese children with hypertension in China and to determine the relationship between anthropometric indexes and hypertension.

Methods: A total of 469 obese children, aged 6 to 18 years, were evaluated between January 2001 and December 2005; 184 of them were diagnosed with hypertension. Hypertensive children were provided with individual diet-oriented intervention for more than 6 months. Physical exercises were recommended for obese children at least 30 minutes per day. Height, body weight, waist circumference, systolic blood pressure, and diastolic blood pressure were taken for each subject before and after intervention.

Results: Of the 184 children enrolled, 139 (75.5%; 86 boys) completed the study. Weight, body mass index (BMI), waist circumference, and hip circumference all decreased after a 6-month intervention, despite a 2.1 cm increase in height. Systolic and diastolic pressures decreased by 16.6 and 13.3 mmHg compared with baseline levels. Of the 139 children, 103 (74.1%) who had blood pressure in the normal range (<90th percentile for age and sex) were taken as a response group. The other 36 children who remained hypertensive showed no obvious differences in anthropometric measurements and were taken as a non-response group. Weight, BMI, BMI%, waist circumference, hip circumference and blood pressure in the 139 children showed significant differences after the intervention compared with baseline values. Weight, BMI, BMI%, waist circumference, and hip circumference were positively correlated with both systolic and diastolic blood pressures; they were correlated more strongly with systolic pressure than with diastolic pressure.

Conclusions: Diet-oriented intervention can decrease blood pressure in most obese children with hypertension. Weight, height, BMI, BMI%, waist circumference, and hip circumference are closely associated with blood pressure.

Key words: body mass index; children; diet intervention; hypertension; obesity

                   World J Pediatr 2009;5(4):269-274

  [Abstract] [Full Text] [PDF]  
Co-assessment of iron, vitamin A and growth status to investigate anemia in preschool children in suburb Chongqing, China
  Ke Chen, Xuan Zhang, Ting-Yu Li, Li Chen, Ping Qu, You-Xue Liu
   

Author Affiliations: Child Health Care, Children's Hospital, Chongqing Medical University, Chongqing, China (Chen K, Zhang X, Li TY, Chen L, Qu P, Liu YX); Chengdu Maternal and Child Health Care Hospital, Chengdu, China (Chen K)

Corresponding Author: Ting-Yu Li, Child Health Care, Children's Hospital, Chongqing Medical University, 136 Second Zhongshan Road, Chongqing 400014, China (Tel: +86 23 6362 3604; Fax: +86 23 6362 4479; Email: tyli@vip.sina.com)

doi:10.1007/s12519-009-0052-z

Background: Anemia is a widespread public health problem, which is due to many factors, nutritional or non-nutritional. Iron, vitamin A and growth status were assessed to investigate anemia of preschool children in suburb Chongqing, China.

Methods: A descriptive, cross-sectional survey was performed on 459 preschool children aged 2 to 7 years randomly chosen from the kindergartens in 6 suburban districts of Chongqing. Weight and height levels, hemoglobin, erythrocyte protoporphyrin, serum retinol, and ferritin concentrations were measured to evaluate the anthropometric and nutritional status.

Results: The rates of stunt, underweight, overweight, wasting, obesity, anemia, iron deficiency, vitamin A deficiency (VAD), and marginal VAD were 6.3%, 3.9%, 3.7%, 1.5%, 3.1%, 23.5%, 15.0%, 6.3% and 25.9%, respectively. Serum retinol concentration was significantly lower in children with anemia than in those without anemia (P=0.003), and the retinol concentration was associated with hemoglobin (Pearson's correlation coefficient, r=0.22, P<0.01). Children with VAD had a significantly increased risk for anemia (odds ratio, 2.56; 95% confident interval, 1.15-5.70). In all 108 children with anemia, only 42 were related to VAD and 12 related to iron deficiency, suggesting that almost half of the anemia children cannot be explained solely by iron deficiency or VAD.

Conclusions: Vitamin A and iron deficiency are still public health problems in some localities of China. Public health interventions in anemia control should be used to eliminate deficiencies of vitamin A, iron, and other micronutrients by deliberate supplementation. Attention must be paid to such deficiencies in high-risk groups, especially in preschool children.

Key words: anemia; iron; micronutrient deficiency; preschool children; vitamin A

                 World J Pediatr 2009;5(4):275-281

  [Abstract] [Full Text] [PDF]  
Celiac disease in 87 children with typical and atypical symptoms in Black Sea region of Turkey
  Gönül Dinler, Erdal Atalay, Ayhan Gazi Kalaycı
   

Author Affiliations: Unit of Pediatric Gastroenterology Hepatology and Nutrition (Dinler G, Kalaycı AG) and Department of Pediatrics (Atalay E), Faculty of Medicine, Ondokuz Mayıs University, Samsun, Turkey

Corresponding Author: Gönül Dinler, Pediatric Gastroenterology Hepatology and Nutrition Department, Ondokuz Mayıs University Faculty of Medicine, Samsun 55139, Turkey (Tel: +90 3623121919-3734; Fax: +90 3624576041; Email: gdinler@omu.edu.tr)

doi:10.1007/s12519-009-0053-y

Background: 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.

Methods: 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.

Results: 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.

Conclusions: 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.

Key words: atypical presentation; celiac disease; child; iron deficiency anemia; short stature

                   World J Pediatr 2009;5(4):282-286

  [Abstract] [Full Text] [PDF]  
Congenital rubella syndrome: pattern and presentation in a southern Nigerian tertiary hospital
  Innocent Ocheyana George, Angela Ine Frank-Briggs, Raphael Sonny Oruamabo
   

Author Affiliations: Department of Pediatrics and Child Health, University of Port Harcourt Teaching Hospital, Port Harcourt, Nigeria (George IO, Frank-Briggs AI); College of Health Sciences, Niger Delta University Wilberforce Island, Bayelsa State, Nigeria (Oruamabo RS)

Corresponding Author: I. O. George, Department of Pediatrics and Child Health, University of Port Harcourt Teaching Hospital, Port Harcourt, Nigeria (Email: geonosdemed@yahoo.com)

doi:10.1007/s12519-009-0054-x

Background: 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.

Methods: The medical records of all cases of CRS presenting to the Pediatric Department of UPTH from January 2000 to December 2007 were reviewed.

Results: 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 (χ2=8.68, P=0.28). Unfortunately, long-term outcome of the 19 babies was not available.

Conclusion: 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.

Key words: congenital rubella syndrome; maternal infection; national program on immunization

                  World J Pediatr 2009;5(4):287-291

  [Abstract] [Full Text] [PDF]  
Nuss operation for pectus excavatum: a single-institution experience
  Yong-Zhong Mao, Shao-Tao Tang, Yong Wang, Qiang-Song Tong, Qing-Lan Ruan
   

Background: The Nuss procedure for repair of pectus excavatum (PE) has been accepted worldwide because of minimal invasiveness and excellent cosmetic results. We summarized our experience with the treatment of 115 patients aged 2.7-18 years.

Methods: All the 115 patients underwent the Nuss procedure successfully from July 2003 to February 2008. They were divided into two groups: children group (below 12 years) and adolescents group (aged 12-18 years).

Results: The rate of complications was 14.7% and 37.5% in the children and adolescents groups, respectively (P<0.05). There was significant difference in operation time, length of hospital stay, and analgesic time between the two groups (P<0.05). The initial results of Nuss procedure were excellent.

Conclusions: The Nuss procedure can be performed with excellent early results in children. We suggest that children with PE should accept the Nuss procedure as early as possible when they are over 5 years old.

Key words: adolescents; children; complication; Nuss procedure; pectus excavatum
  [Abstract] [Full Text] [PDF]  
Interventional occlusion of congenital vascular malformations
  Chun-Hong Xie, Cheng-Sen Xia, Fang-Qi Gong, Yin-Bao Zhou, Wei-Hua Zhu
   

Background: 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.

Methods: 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.

Results: 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.

Conclusions: 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.

Key words: embolization; patent ductus arteriosus; transcatheter; vascular malformations

                  

World J Pediatr 2009;5(4):296-299

  [Abstract] [Full Text] [PDF]  
Effect of melatonin on proliferation of neonatal cord blood mononuclear cells
  Wei Zhou, Ping Wang, Li Tao
   

Author Affiliations: Department of Neonatology, Guangzhou Children's Hospital, Guangzhou Medical College, Guangzhou 510120, China (Zhou W, Wang P, Tao L)

Corresponding Author: Wei Zhou, MD, Department of Neonatology, Guangzhou Children's Hospital, Guangzhou Medical College, Guangzhou 510120, China (Tel: +86-20-81330577; Fax: +86-20-81861650; Email: zhouwei_pu002@126.com)

doi:10.1007/s12519-009-0057-7

Background: 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). 

Methods: 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. 3H-thymidine (3H-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.

Results: 3H-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 μg/ml), and MLT+IL-2, respectively in CBMCs media, 3H-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), 3H-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). 3H-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 3H-TdR incorporation compared to the MLT or MLT+PHA groups (t=0.1730, P>0.05; t=0.3286, P>0.05). 3H-TdR incorporation was significantly greater in CBMCs than in PBMCs cultures after addition of various stimulators to the culture media.

Conclusions: MLT promoted proliferation of PBMCs and also enhanced proliferation of CBMCs. The proliferative effects of MLT were greater on CBMCs than on PBMCs.

Key words: cell proliferation; cord blood; melatonin; mononuclear cell; neonate  

                            World J Pediatr 2009;5(4):300-303

  [Abstract] [Full Text] [PDF]  
Risk factors and laser therapy for retinopathy of prematurity in neonatal intensive care unit
  Li Liu, Tian Tian, Chong-Xun Zheng, Vatavu Ileana, Anca Ioana, Ciomartan Tatiana, Racovitan Oana
   

Author Affiliations: Department of Neonatology, The First Affiliated Hospital of Medical College, Xi'an Jiaotong University, Xi'an 710061, China (Liu L, Tian T); Biomedical Engineering Institute of Xi'an Jiaotong University, Xi'an 710061, China (Zheng CX); Institute for Mother and Child Care (IOMC) of Bucharest Medical University, Sector 2, 72309, Romania (Ileana V, Ioana A, Tatiana C, Oana R)

Corresponding Author: Li Liu, MD, Department of Neonatology, The First Affiliated Hospital of Medical College, Xi'an Jiaotong University, Xi'an 710061, China (Tel: 86-29-85323829; Fax: 86-29-85263190; Email: nellie918@yahoo.com.cn)

doi:10.1007/s12519-009-0058-6

Background: With the increasing survival rates of preterm infants, the incidence of retinopathy of prematurity (ROP) is also increasing. This study aimed to investigate the risk factors for ROP in Neonatal Intensive Care Unit, and the effects of laser therapy.

Methods: Thirty-five premature infants with various other diseases were studied. Data were collected including gender, gestational age, birth weight, maternal gravidity, Apgar score, antenatal and postnatal hypoxia, anemia, jaundice, infection, pregnancy complications, and head ultrasound manifestations. All patients were subjected to fundus examinations at a postmenstrual age of 34 weeks using an indirect ophthalmoscope. The infants were divided into ROP group and non-ROP group. Infants with ROP received laser treatment. The data of the infants were analyzed using univariate analysis and the logistic regression model.

Results: Twenty-seven (77.1%) of the 35 infants had ROP and 21 of them also had plus diseases. The recovery rates were 74.1% and 92.6% at 2 weeks and 3 months after laser therapy, respectively. The birth weight and gestational age in the ROP group were significantly lower than those in the non-ROP group (P<0.01). The lower the gestational age and birth weight, the higher the incidence of ROP. There was a significant difference in the incidence of ROP with postnatal hypoxia (P=0.013), anemia (P=0.012) and gravidity two (P<0.05), analyzed with the Fisher's exact probability test (P<0.05). The gestational age was significantly lower in the plus disease group than in the non-plus disease group (P<0.05).

Conclusions: The incidence of ROP is high in premature infants with various other diseases and is correlated with birth weight, gestational age, postnatal hypoxia, anemia and maternal gravidity. Laser therapy shows good outcomes.

Key words: laser therapy; premature infant; retinopathy of prematurity; risk factor

                   World J Pediatr 2009;5(4):304-307

  [Abstract] [Full Text] [PDF]  
Drugs controlling proteinuria of patients with Alport syndrome
  Jian-Guo Li, Jie Ding, Fang Wang, Hong-Wen Zhang
   

Author Affiliations: Department of Pediatrics, Peking University First Hospital, Beijing 100034, China (Li JG, Ding J, Wang F, Zhang HW)

Corresponding Author: Jie Ding, MD, PhD, Department of Pediatrics, Peking University First Hospital, No. 1, Xianmen Dajie, Beijing 100034, China (Tel: 8610 66551122 ext 3236; Fax: 8610 66134261; Email: djnc_5855@126.com).

doi:10.1007/s12519-009-0059-5

Background: Proteinuria is one of the risk factors for the progression of renal diseases including Alport syndrome (AS), a hereditary glomerular renal disease. This study aimed to evaluate the efficacy of angiotensin converting enzyme inhibitors (ACEIs) and/or tripterygium, a Chinese herbal medicine widely used in Chinese patients with hematuria and proteinuria, on proteinuria in patients with AS.

Methods: Twenty-nine children were enrolled into this retrospective study. Patients were divided into 3 therapy groups: ACEI group, tripterygium group, and ACEI plus tripterygium group.

Results: In the 29 children, 23 were male and 6 female. In the ACEI group and the tripterygium group, the effective rate was 87.5% and 25.0%, respectively and in the ACEI plus tripterygium group was 42.9%.

Conclusions: ACEI is effective in controlling proteinuria of AS patients. Tripterygium should be carefully administered in controlling proteinuria of AS patients.

Key words: Alport syndrome; angiotensin converting enzyme inhibitor; herbal medicine; tripterygium

                World J Pediatr 2009;5(4):308-311

  [Abstract] [Full Text] [PDF]  
Brief report:
Foreskin development in 10 421 Chinese boys aged 0-18 years
  Chao Yang, Xing Liu, Guang-Hui Wei
   

Author Affiliations: Department of Pediatric Oncology (Yang C) and Department of Pediatric Urology (Liu X, Wei GH), Children's Hospital,  Chongqing Medical University, Chongqing, China 

Corresponding Author: Guang-Hui Wei, 136 Zhongshan 2nd Road, Yuzhong District, Chongqing 400014, China (Tel: +86-23-63631928; Fax: +86-23-63622754; Email: ghwei@mail.chcmu.com)

doi:10.1007/s12519-009-0060-z

Background: 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.

Methods: A total of 10 421 boys aged 0 to 18 years were studied. The condition of foreskin was classied 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.

Results: 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.

Conclusions: 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.

Key words: abnormalities; circumcision; external genitalia; foreskin

                 World J Pediatr 2009;5(4):312-315

  [Abstract] [Full Text] [PDF]  
Case reports:
Theophylline-associated status epilepticus in an infant:pharmacokinetics and the risk of suppository use
  Zenichiro Kato, Atsushi Yamagishi, Mitsuhiro Nakamura, Naomi Kondo
   

Author Affiliations: Department of Pediatrics, Graduate School of Medicine, Gifu University, Yanagido 1-1, Gifu 501-1194, Japan (Kato Z, Yamagishi A, Kondo N); Department of Pharmacy, Gifu University Hospital, Yanagido 1-1, Gifu 501-1194, Japan (Nakamura M)

Corresponding Author: Zenichiro Kato, MD, PhD, Department of Pediatrics, Graduate School of Medicine, Gifu University, Yanagido 1-1, Gifu 501-1194, Japan (Tel: +81 (58) 230 6386; Fax: +81 (58) 230 6387; Email: zen-k@gifu-u.ac.jp)

doi:10.1007/s12519-009-0061-y

Background: 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.

Methods: 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.

Results: 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.

Conclusion: The parents of children and also physicians should be educated to ensure the proper use of the suppository formula.

Key words: convulsion; pharmacokinetics; suppository; theophylline

                  World J Pediatr 2009;5(4):316-318

  [Abstract] [Full Text] [PDF]  
Progression of organ manifestations upon enzyme replacement therapy in a patient with mucopolysaccharidosis type I/Hurler
  Saadet Mercimek-Mahmutoglu, Christopher Reilly, Derek Human, Paula J. Waters, Sylvia Stoeckler-Ipsiroglu
 

Author Affiliations: Division of Biochemical Diseases, Department of Pediatrics, British Columbia Children's Hospital, Vancouver, Canada (Mercimek-Mahmutoglu S, Stoeckler-Ipsiroglu S); Department of Orthopedics, British Columbia Children's Hospital, Vancouver, Canada (Reilly C); Division of Cardiology, Department of Pediatrics, British Columbia (Human D); Biochemical Genetics Laboratory, Department of Pathology and Laboratory Medicine, British Columbia Children's Hospital, Vancouver, Canada (Waters PJ)

Corresponding Author: Saadet Mercimek-Mahmutoglu, MD, FCCMG, British Columbia Children's Hospital, Division of Biochemical Genetic Diseases, Room K3-208, ACB, 4480 Oak Street, Vancouver, B.C., Canada V6H 3V4 (Tel: 604-875-2628; Fax: 604-875-2349; Email: smahmutoglu@cw.bc.ca)

doi:10.1007/s12519-009-0062-x

Background: Enzyme replacement therapy (ERT) has been increasingly used as an interim treatment in severe mucopolysaccharidosis type I (MPSI)/Hurler patients prior to hematopoietic stem cell transplantation (HSCT).

Methods: We present the outcome of a patient with MPSI/Hurler after 14 months of ERT prior to HSCT.

Results: Urinary glucosaminoglycan excretion decreased by 70% after one month of ERT. Liver volume decreased by 14% of baseline after 12 months of ERT. Pre-existing thoracolumbar kyphosis progressed to thoracolumbar dislocation with complete displacement of facets after 12 months of ERT. New development of mitral valve thickening was found by echocardiography and mild hearing loss progressed to severe sensorineural hearing loss after 13 months of ERT.

Conclusions: ERT over a period of 14 months did not prevent progression of organ manifestations in our patient. Patients should be monitored every 6 months for cardiac, skeletal and audiological involvement on ERT.

Key words: Hurler disease; laronidase;  mucopolysaccharidosis type I

World J Pediatr 2009;5(4):319-321

  [Abstract] [Full Text] [PDF]  
Ileal duplication with extensive gastric heterotopia in a girl
  Hui-Min Jia, Ke-Ren Zhang, Ri-Bin Qu
   

Author Affiliations: Department of Pediatric Surgery, Shengjing Hospital Affiliated to China Medical University, Shenyang 110004, China (Jia HM, Zhang KR, Qu RB)

Corresponding Author: Ke-Ren Zhang, Department of Pediatric Surgery, Shengjing Hospital Affiliated to China Medical University, Shenyang 110004, China (Tel: 86-24-83955606; Email: zkr425@yahoo.com)

doi:10.1007/s12519-009-0063-9

Background: 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.

Methods: 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.

Results: 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 corpus-fundic glands.

Conclusions: This is an unusual case of enteric duplication. Ultrasonography, computed tomography  and technetium-99m scintigraphy are helpful in the diagnosis of duplication.

Key words: children; enteric duplications; gastric heterotopia

                  World J Pediatr 2009;5(4):322-324

  [Abstract] [Full Text] [PDF]  
   
 
 
 
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