Author Affiliations: Department of Neonatology, Children's Hospital of Chongqing Medical University, Chongqing, China (Dong Y, Yu JL)

Corresponding Author: Jia-Lin Yu, MD, Department of Neonatology, Children's Hospital of Chongqing Medical University, No.136, 2nd Zhongshan Road, Yuzhong District, Chongqing 400014, China (Tel: 86-13896069217; Email: yujialin486@sohu.com)

doi:10.1007/s12519-011-0290-8

Background: Preterm birth rate continues to rise around the world mainly at the expense of late preterm newborns, recently defined as births between the gestational age of 34 weeks and 36-6/7 weeks. Late preterm infants are considered to have significantly more short-term and long-term adverse outcomes than term infants.

Data sources: Articles concerning morbidity, mortality and long-term outcomes of late preterm infants were retrieved from PubMed/MEDLINE published during the period of 2000-2010.

Results: Late preterm infants are the fastest growing subgroup of neonates, comprising the majority of all preterm births. Compared with term infants, they have significantly higher risk of morbidity, mortality and adverse long-term outcomes well beyond infancy into adulthood. However, epidemiology and etiology of late preterm births, the magnitude of their morbidity, the long-term life quality, and public health impact have not been well studied.

Conclusions: The growing number of late preterm neonates substantiates the importance to better understand and medically approach this special preterm subgroup. A long-term evaluation, monitoring and follow-up of late preterm infants are needed to optimize neonatal care and improve human health status.

Key words: late preterm birth; mortality; neonatal morbidity; outcome

                   World J Pediatr 2011;7(3):199-204

Author Affiliations: Division of Pediatric Surgery, Department of Surgery, University of Ilorin Teaching Hospital Ilorin, Nigeria (Nasir AA, Abdur-Rahman LO, Adeniran JO); Division of Pediatric Surgery, Department of Surgery, Ahmadu Bello University Teaching Hospital Zaria, Nigeria (Ameh EA); Department of Pediatric Surgery, Amrita Institute of Medical Sciences and Research centre, Kochi, India (Abraham MK)

Corresponding Author: A. A. Nasir, Division of Paediatric Surgery, Department of Surgery, University of Ilorin Teaching Hospital, PMB 1459, Ilorin, Nigeria (Tel: +234 803 3840 110; Email: draanasir@yahoo.com)

Background: Posterior urethral valve (PUV) is a significant cause of morbidity, mortality and ongoing renal damage in children. It accounts for end-stage renal disease in a proportion of children. This article aims at highlighting the current trend in the management of boys with posterior urethral valve.

Data sources: PubMed/Medline and bibliographic search for posterior urethral valve was done. Relevant literatures on presentation, pathology, evaluation, management and outcomes of PUV were reviewed.

Results: PUV which is increasingly diagnosed prenatally presents a spectrum of severity. The varied severity and degree of obstruction caused by this abnormality depend on the configuration of the obstructive membrane within the urethra. The decision to intervene prenatally is dependent on gestational age, amniotic volume, and renal function of fetal urine aspiration. Identification of the patients who may benefit from early intervention remains inconclusive. Endoscopic ablation of the valve is the gold standard of treatment but use of Mohan's valvotome and other modalities are invaluable in developing countries where endoscopic facilities are limited. Proximal urinary diversion may result in poor bladder compliance and should be reserved for patients with persisting or increasing upper urinary tract dilatation, increasing serum creatinine or inappropriate instruments. The behavior of the bladder and its subsequent management after valve ablation may influence the long-term renal outcome in PUV patients.

Conclusions: The care of children with PUV continues to improve as a result of earlier diagnosis by ultrasound, developments in surgical technique and meticulous attention to neonatal care. The ultimate goal of management should be to maximize renal function, maintain normal bladder function, minimize morbidity and prevent iatrogenic problems.

Key words: bladder management; posterior urethral valve; prenatal diagnosis; prognosis                  

World J Pediatr 2011;7(3):205-216

Jianghong Liu, Yue-Xian Ai, Alexandra Hanlon, Zumin Shi, Barbra Dickerman, Charlene Compher

Philadelphia, USA

Author Affiliations: University of Pennsylvania School of Nursing, 418 Curie Blvd., Philadelphia, Pennsylvania 19104, USA (Liu J, Hanlon A, Dickerman B, Compher C);  Jintan People's Hospital, Jintan, Changzhou, China (Ai YX); Discipline of Medicine, University of Adelaide, 122 Frome Street, Adelaide SA 5005, Australia (Shi Z)

Corresponding Author: Jianghong Liu, PhD, University of Pennsylvania School of Nursing, 418 Curie Blvd., Philadelphia, Pennsylvania 19104, USA (Tel: (215) 573-7492; Email: jhliu@nursing.upenn.edu)

Background: Although the prevalence of malnutrition has decreased, micronutrient deficiency still exists among children. While iron level has been studied, limited information on serum levels of zinc in Chinese children is available. This study aims to describe the status of micronutrients and its association with sociodemographic factors, and to assess associated risk factors.

Methods: A cross-sectional study was performed on 1375 Chinese preschool children. Venous blood samples were collected and analyzed for zinc and iron by atomic absorption spectrophotometry. Sociodemographic information was obtained from self-administered questionnaires given to the parents.

Results: The high prevalence of low serum zinc (38.2%) and iron (24.3%) was found. Children from rural preschools had the lowest zinc levels, whereas those from urban preschools had the lowest iron levels. Children living in small houses had the highest prevalence of low serum zinc (47.1%), and children from small families (≤3 persons) had a higher prevalence of low serum zinc than those from large families (41.8% vs. 34.1%).

Conclusions: Our findings in the recent socioeconomic development of China suggest the need for continuous monitoring of nutritional factors and highlight the importance of public health implication in preschool children even in developed regions.

Key words: iron; micronutrient deficiency; socio-demographic factors; zinc

                   World J Pediatr 2011;7(3):217-223

Author Affiliations: Departments of Pediatrics (El-Karaksy H, El-Raziky M, El-Koofy N, El-Sayed R, Mohsen N), Radio-diagnosis (El-Kiki H) and Pathology (El-Hennawy A), Cairo University, Cairo, Egypt; Department of Pediatrics, Research Institute of Ophthalmology, Cairo, Egypt (Fahmy M); Department of Genetics, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia (Rashed MS); Pharmagene Laboratory, Giza, Egypt (Rashed MS)

Corresponding Author: Hanaa Mostafa El-Karaksy, 44 Mohei El-Deen Abu El-Ezz Street, Dokki, Cairo, 12311, Egypt (Tel: 202 33375913; Fax: 202 25311616; Email: hanaakaraksy@yahoo.com)

doi: 10.1007/s12519-011-0287-3

Background: Hereditary tyrosinemia type 1 (HT1) is an increasingly recognized inborn error of metabolism among Egyptian children. This study was undertaken to define the presenting clinical, biochemical and imaging features and outcome of 2-(2-motrp-4-trifluoro-methylbenzoyl)-1, 3-cyclohexanedione (NTBC) therapy and liver transplantation in a cohort of Egyptian children diagnosed with HT1. 

Methods: The study was carried out at the Pediatric Hepatology Unit at Cairo University Children's Hospital. HT1 was diagnosed by quantification of succinylacetone (SA) in dry blood spots. 

Results: Twenty-two patients were diagnosed with HT1 in a period of 3 years from August 2006 to July 2009. Infants with focal hepatic lesions and hepatomegaly (n=13) were younger at diagnosis than those with rickets (n=5) (median age: 3.25 vs. 10 months; P=0.05). Alpha fetoprotein was highly elevated in all children. Seven children died within a few weeks of diagnosis before therapy was initiated. Ten children were treated with NTBC. The response to NTBC treatment was apparent by a steep drop in serum alpha fetoprotein (AFP) and undetectable SA in urine within 2 months. Three children underwent living donor liver transplantation after treatment with NTBC for 10, 18 and 22 months respectively, despite adequate response to therapy because of financial issues. The explanted livers were all cirrhotic with no dysplasia or malignant transformation.

Conclusions: Focal hepatic lesions are the commonest presentation of HT1 patients and they present at an earlier age than rickets. NTBC is effective but very expensive. Liver transplantation is still considered in HT1 patients. 

Key words: children; Egypt; hereditary tyrosinemia type 1; liver transplantation; 2-(2-nitro-4-trifluoro-methylbenzoyl)-1,3- cyclohexanedione (NTBC)

                      World J Pediatr 2011;7(3):224-231

Author Affiliations: Department of Nephrology, Children's Hospital of Fudan University, Shanghai 201102, China (Gao J, Xu H, Kuang XY, Huang WY, Rao J, Qian QY, Cheng XY, Feng ZM, Xu J, Zhang X, Wang X); Department of Biostatistics and Social Medicines, School of Public Health, Fudan University, Shanghai 201102, China (Zhao NQ)

Corresponding Author: Hong Xu, MD, PhD, Department of Nephrology, Children's Hospital of Fudan University, Shanghai 201102, China (Tel: 86-21-64931006; Fax: 86-21-64931901; Email: hxu@shmu.edu.cn)

Background: Melamine-contaminated milk powder was the cause of the 2008 outbreak of urolithiasis in young children and infants in China, but the prognosis of these children remains unknown. We hypothesized that urolithiasis induced by melamine-contaminated milk powder may be associated with secondary renal injury.

Methods: A total of 8335 children (≤6 years old) with a history of consuming melamine-contaminated milk powder were screened. Urine analysis and urinary system ultrasonography were performed. For children with urolithiasis, the basic information and the results of examination were recorded, and effective therapy was given. They were followed up for 6 months after the original diagnosis, and urinary microprotein profiles were measured.

Results: Of the 8335 children, 105 (1.26%) were diagnosed with melamine-contaminated milk powder-associated urolithiasis. The size of the stone was correlated with the duration of exposure to melamine. Six months later, 69.8% (67) of the children with urolithiasis passed stones (follow-up rate: 91.4%). Of the 67 children, 28 passed stones within 2 months. The higher possibility of passing a stone was correlated with the smaller diameter of the stone (P<0.001). The detection rate of abnormal urinary microprotein excretion (microalbumin, immunoglobulin G, and N-acetyl-β-D-glucosidase) was 52.4% in children with persistent stones and 38.2% in those who passed their stones. The detection rate was lower in children who passed stones within 2 months (31.8%) than in those who passed stones in 2 to 6 months (50.0%). The levels of microalbumin/creatinine and immunoglobulin G/creatinine were significantly higher in children with persistent stones than in those who passed their stones.

Conclusions: Early passage of a stone may reduce the renal injury induced by melamine-contaminated milk powder-associated urolithiasis.

Key words: children; follow up; melamine; renal injury; urolithiasis     

World J Pediatr 2011;7(3):232-239

Author Affiliations: Department of Pulmonology, Children's Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China (Xu YC, Xu D, Tao XF, Li SX, Tang LF, Chen ZM); Hangzhou Meteorological Bureau, Hangzhou 310051, Zhejiang Province, China (Zhu LJ)

Corresponding Author: Zhi-Min Chen, Department of Pulmonology, Children's Hospital, Zhejiang University School of Medicine, 57 Zhugan Xiang, Hangzhou 310003, China (Tel: +86 571 87061007 ext 61711; Fax: +86 571 87033296; Email: drchenzm@163.com)

doi:10.1007/s12519-011-0318-0

Background: Mycoplasma pneumoniae (M. pneumoniae) is an important pathogen of pediatric respiratory infections and the relation of M. pneumoniae pneumonia (MPP) with meteorological factors remains obscure. This study aims to investigate the epidemiological characteristics of childhood MPP and observe if there is a relationship between epidemiological characteristics and meteorological factors in Hangzhou.

Methods: M. pneumoniae DNA in nasopharyngeal aspirates of hospitalized pneumonia children were detected by polymerase chain reaction from January 1, 2007 to December 31, 2009. The positive rates of MPP (MPP rates) in different years, seasons and ages were compared. The relationship between MPP rates and meteorological data, including mean air temperature (ºC), mean relative humidity (%), monthly precipitation (mm) and raining days were analyzed.

Results: In 14 799 pneumonia cases found from 2007 to 2009, the MPP rate was 18.5%. Altogether 1610 boys (16.9%) and 1134 girls (21.4%) suffered from MPP with a significant difference between both genders (χ²=45.68, P<0.001). In children younger than 1 year, 1-2 years, 3-6 years, and older than 7 years, the MPP rates were 9.8%, 21.1%, 44.4% and 61.6%, respectively. The MPP rates were significantly higher in older children than in younger ones (trends test χ²=46.72, P<0.001). In a descending order, the MPP rates in summer, autumn, spring, and winter were 27.8%, 23.9%, 18.0% and 11.6%, respectively (χ²=372.75, P<0.001). The MPP rates in 2007 to 2009 were 12.9%, 19.3% and 23.6%, respectively (trends test χ²=13.72, P<0.001). Of the four meteorological factors, only monthly mean air temperature was included in the multiple linear regression model (P<0.001).

Conclusions: This study showed that the MPP rate was higher in older children than in younger ones. Girls had a higher positive rate of MPP than boys. In Hangzhou, MPP was more prevalent in summer and autumn. Air temperature was the only meteorological factor affecting the prevalence of MPP.

Key words: meteorological factor; Mycoplasma pneumoniae; pneumonia; temperature

                   World J Pediatr 2011;7(3):240-244

Murat Tuncel, Pınar Özgen Kıratlı, Tamer Aksoy, Murat Fani Bozkurt

Ankara, Turkey

Author Affiliations: Department of Nuclear Medicine, Hacettepe University Faculty of Medicine, Sihhiye, Ankara, Turkey (Tuncel M, Kıratlı PÖ, Aksoy T, Bozkurt MF)

Corresponding Author: Murat Tuncel, MD, Department of Nuclear Medicine, Hacettepe University Faculty of Medicine, Sihhiye, Ankara 06110, Turkey (Tel: +90 536 213 03 41; Fax: +90 312 309 35 08; Email: muratmtx@yahoo.com)

Background: Scintigraphic imaging is a useful screening tool for patients with suspected gastro-esophageal reflux. New scintigraphic interpretation methods have recently been introduced. This study was undertaken to evaluate the efficiency of various scintigraphic interpretation methods in the detection of gastroesophageal reflux and to measure their influence on inter-reader agreement.

Methods: Scintigraphic images of 49 children with suspected gastroesophageal reflux were interpreted by three different methods: visual interpretation, time activity curves, and condensed images. The readings were performed by three specialists and a resident. The discordant results were resolved by a consensus reading done together by all interpreters based on the three different methods. The gastroesophageal refluxes were grouped according to their number, location and intensity.

Results: Gastroesophageal reflux scintigraphy revealed 22 patients with negative results and 27 with positive results. The sensitivity, positive predictive value and specificity for each of the three specialists vs. the resident were 96%, 96% and 81% vs. 96%; 93%, 90% and 96% vs. 81%; and 90%, 86%, and 95% vs. 73%, respectively. The mean inter-observer reproducibility (κ value) was 0.910 for visual interpretation, 0.652 for time activity curves and 0.789 for condensed images. Twenty-seven percent of the results were discordant and most of these refluxes were of low grade (92%), low intensity (77%) and localization in the distal esophagus (54%).

Conclusion: Gastroesophageal scintigraphy is a useful tool for detecting patients with suspected reflux, and visual interpretation is better than the other two methods in terms of accuracy and inter-observer reproducibility.

Key words: gastroesophageal reflux; inter-observer variability; scintigraphy; time activity curve

World J Pediatr 2011;7(3):245-249

Author Affiliations: Department of Pediatric Neurology, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai, China (Li L, Bao WQ); Department of Neurology, Affiliated Children's Hospital of Zhejiang University School of Medicine, Hangzhou, China (Shui QX, Zhao ZY); PICU, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai, China (Zhu XD)

Corresponding Author: Ling Li, Pediatric Department, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, No.1665 Kongjiang Road, Shanghai 200092, China (Email: lingli12@live.cn)

Background: Although more and more new potent antibiotics have been used, the incidence of neurological sequelae of Streptococcus pneumoniae meningitis has not improved in children over the last decade. The expression of TrkB mRNA, a receptor of brain-derived neurotrophic factor, is associated with the incidence of neurological sequelae of Streptococcus pneumoniae meningitis.

Methods: Rats of 3 weeks old were used to construct a model of Streptococcus pneumoniae meningitis and served as normal controls. They were administered with antibiotics or antibiotics plus dexamethasone, respectively. The expression of the TrkB gene was detected in the brain by in situ hybridization.

Results: In the brains of Streptococcus pneumoniae inoculated rats, TrkB mRNA was significantly up-regulated after inoculation for 24 hours, and then down-regulated in a dose-dependent manner after treatment with antibiotics. This up-regulation was seen after treatment with antibiotics plus dexamethasone. TrkB mRNA expression was also observed in some infiltrating inflammatory cells.

Conclusions: The results of the study support the hypothesis that TrkB signal transduction pathways might play an important role in Streptococcus pneumoniae meningitis, probably by protecting the brain from damage. The role of TrkB might be weakened after the treatment with antibiotics. Our findings suggest that targeting TrkB receptors might be a rational strategy for prevention of neurological sequelae caused by Streptococcus pneumoniae meningitis.

Key words: meningitis; mRNA; Streptococcus pneumoniae; TrkB

                   World J Pediatr 2011;7(3):250-256

Author Affiliations: Department of Cardiothoracic Surgery, Children's Hospital, Zhejiang University School of Medicine, and Zhejiang Key Laboratory for Diagnosis and Treatment of Neonatal Diseases, Hangzhou 310003, China (Shu Q, Shi Z, Xu WZ, Li JH, Zhang ZW, Lin R, Zhu XK, Yu JG)

Corresponding Author: Jian-Gen Yu, Department of Cardiothoracic Surgery, Children's Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China (Tel: 0086-13989869539; Fax: 0086-571-87033296; Email: yujiangen@hotmail.com)

doi:10.1007/s12519-011-0319-z

Background: This study was to investigate the advantages of thoracoscopy-assisted minimally invasive Nuss operation for the treatment of pectus excavatum (PE) in children.

Methods: A total of 406 patients with PE (female: 93; male: 313) with an average age of 6.8 years (range: 3.5-17.5 years) were included in this study. Associated diseases included congenital heart disease in 9 patients and congenital pulmonary cyst in 2. The Haller index of the patients ranged from 3.35 to 7.23, with an average of 5.17±1.64. Minimally invasive Nuss operation was performed for all the patients.

Results: The operations were performed successfully and no operative mortality occurred. The average blood loss during the operation was less than 10 mL and the operating time ranged from 30 to 85 minutes with an average of 45 minutes. The length of hospital stay ranged from 5 to 9 days with an average of 7 days. Struts were implanted in 12 (3.0%) of the 406 patients. Injury of the pericardium occurred in 1 patient during the operation. Early post-operative complications occurred in 9 patients with pneumothorax and 6 patients with pleural effusion, which were cured by puncture or drainage. Poor wound healing occurred in 4 patients (1.0%) and was cured by nutritional support. During a 3-month to 6-year follow-up, 2 patients had scoliosis and 3 patients had displacement of the strut, which was cured by a second Nuss operation. Allergy occurred in 2 patients: the symptoms were improved in 1 patient after conservative treatment, but the strut was removed in advance due to allergy in the other patient. Totally 154 patients (40.0%) underwent operation for strut removal. Excellent repair results were achieved in 387 (95.3%) patients, good repair results in 12 (3.0%), and fair results in 7 (1.7%).

Conclusions: Thoracoscopy-assisted Nuss operation has many advantages including small and masked incision, short operative time, minimal blood loss, fast recovery, less trauma, and satisfactory outcomes of repair. Nuss is a safe and reliable technique for repair of PE.

Key words: minimally invasive; Nuss operation; pectus excavatum; thoracoscopy

                  World J Pediatr 2011;7(3):257-261

Author Affiliations: Department of Pediatric Surgery (Gu S, Hong L) and Department of Pathology (Zhang ZD, Yin MZ), Shanghai Children's Medical Center, School of Medicine, Shanghai Jiaotong University, Shanghai 200127, China; Department of Pediatric Surgery, Xinhua Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai 200092, China (Wu YM)

Corresponding Author: Ye-Ming Wu, Department of Pediatric Surgery, Xinhua Hospital, Shanghai 200092, China (Tel: +86-21-65790000-3008, Fax: +86-21-65795173; Email: wuyeming57@hotmail.com)

Background: The present diagnosis of teratomas is limited to visual examination of their tissues. For the sake of treatment, teratomas are graded according to degrees of nerve tissue maturation. Mature fetal nerve tissue contains the astrocyte-specific intermediate filament protein, the glial fibrillary acidic protein (GFAP). This study aimed to investigate GFAP expressions in the nerve tissue of immature and mature teratomas, and to evaluate if GFAP is indicative of teratoma maturation in pediatric patients.

Methods: Nerve tissue specimens were collected from immature (10 children) and mature teratomas (45 children). Nerve tissue specimens as a control group were taken from 33 children with neuroblastoma. GFAP expression of the specimens was studied by immunohistochemical and semi-quantitative analyses.

Results: GFAP expression was low in the nerve tissue of immature teratomas and high in that of mature ones. A semi-quantitative analysis confirmed statistically significant difference between the GFAP expressions of immature and mature teratomas (P=0.0001).

Conclusion: GFAP is highly expressed in the nerve tissue of mature teratomas and low in that of immature ones, suggesting that the GFAP expression is a meaningful indicator of teratoma maturation. It is helpful for pathologists to diagnose and classify teratomas.

Key words: children; glial fibrillary acidic protein; nerve tissue; teratoma

World J Pediatr 2011;7(3):262-265

Author Affiliations: Department of Pediatrics, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong, China (Hon KL)

Corresponding Author: Kam-lun Ellis Hon, MBBS, MD, FAAP, FCCM, Department of Pediatrics, The Chinese University of Hong Kong, 6/F, Clinical Sciences Building, Prince of Wales Hospital, Shatin, Hong Kong, China (Tel: (852) 2632 2859; Fax: (852) 2636 0020; Email: ehon@cuhk.edu.hk)

Background: Filicide is the tragic crime of murdering one's own child. This report aims to summarize all such cases involving filicide with suicide and analyze possible risk factors pertinent to the city of Hong Kong.

Methods: All cases involving filicide with suicide between 1998 and 2010 were reviewed and possible risk factors pertinent to the city of Hong Kong were analyzed.

Results: During the study period, there were 14 cases of parents committing suicide and filicide in Hong Kong. Sixteen victims (9 boys and 7 girls, ages range from 2 months to 33 years) and a three-month fetus were involved; only two survived. The parents (12 mothers and 3 fathers, ages range from 25 years to 64 years) all died. The majority of perpetrators (n=7) had alleged history of psychiatric illness or post-puerperal depression. The majority jumped from heights (n=11 off tall buildings, n=1 off a bridge), one father used town-gas, and one couple burned charcoal (carbon monoxide poisoning).

Conclusions: These rare but tragic cases involved children of both sexes and all ages. Although psychosocial risk factors may be clues for interventions, the rarity of these incidents and the impulsiveness of the act make preventive measures virtually impossible in a city full of high-rise buildings.

Key words: filicide; parents; suicide

                   World J Pediatr 2011;7(3):266-268

Author Affiliations: Department of Pediatric Surgery, Children's Hospital, Zhejiang University School of Medicine, 57 Zhugan Xiang, Hangzhou 310003, China (Gao ZG, Shao M, Xiong QX, Tou JF, Liu WG)

Corresponding Author: Wei-Guang Liu, Children's Hospital, Zhejiang University School of Medicine, 57 Zhugan Xiang, Hangzhou 310003, China (Tel: 86-571-87061007; Fax: +86-571-87033296; Email: ebwk@zju.edu.cn)

Background: We aim to describe our experience of laparoscopic cholecystostomy and bile duct lavage in the treatment of inspissated bile syndrome.

Methods: Between January 2005 and December 2009, 16 infants with inspissated bile syndrome underwent laparoscopic cholecystostomy and bile duct lavage in our department. They were 7 males and 9 females, aged 40 days to 3 months, with an average of 65±23.4 days. A laparoscopic aided cholecystostomy was done. Cholangiography and bile duct lavage were performed during the operation. One week after the operation, bile duct lavage with saline was repeated every 2 to 3 days. According to bilirubin levels and liver function, the tube was kept for 2-4 weeks.

Results: The level of bilirubin decreased and liver function was greatly improved after bile duct lavage. Direct bilirubin level, aspartate aminotransferase and γ-GT were significantly decreased 1-2 months after the operation compared with those before surgery (P<0.05).

Conclusions: The treatment strategy for inspissated bile syndrome with laparoscopic cholecystostomy and biliary duct lavage is feasible and effective.

Key words: bile duct lavage; inspissated bile syndrome; laparoscopic cholecystostomy

                   World J Pediatr 2011;7(3):269-271

Author Affiliations: Department of Radiology, East Hospital, Tongji University School of Medicine, Shanghai 200120, China (Zhu JQ, Hao NX); Department of Pediatrics (Bao WQ) and Department of Pathology (Wu XR), Xin Hua Hospital, Shanghai Jiaotong University School of Medicine, Shanghai 200092, China

Corresponding Author: Wei-Qun Bao, MD, Department of Pediatrics, Xin Hua Hospital, Shanghai Jiaotong University School of Medicine, Shanghai 200092, China  (Tel: 86-21-65790000 ext 8235; Email: melvine0305@sina.com)

Background: Multiple calcified primary central nervous system lymphoma (PCNSL) is extremely rare in childhood.

Methods: We report a 4-year-old boy suffering from multiple calcified B-cell lymphoma in the brain with immunodeficiency.

Results: The boy had a history of walking weakness and seizure for 4 months. The serum levels of immuno-globulin G, A and M were decreased. Brain MRI showed multiple lesions which had ring enhancement. CT showed calcification in all of the lesions. The boy was firstly misdiagnosed with multiple chronic brain abscesses. Pathological analysis of biopsy confirmed the diagnosis of anaplastic diffuse large B-cell lymphoma.

Conclusion: PCNSL should be included in the differential diagnosis of intracranial mass with calcification.

Key words: calcification; immunodeficiency; primary central nervous system lymphoma

                  World J Pediatr 2011;7(3):277-279

Author Affiliations: Department of Radiology, Xinhua Hospital, Medical College of Jiaotong University, Shanghai 200092, China (Tu BW, Li YH); Department of Urological Surgery, Renji Hospital, Medical College of Jiaotong University, Shanghai, China (Ye WJ)

Corresponding Author: Wei-Jing Ye, Department of Urological Surgery, Renji Hospital, Medical College of Jiaotong University, Shanghai 200001, China (Email: yeweijingsh@126.com)

Background: Botryoid Wilms' tumor is a rare kind of Wilms' tumor. We report two cases of this tumor.

Methods: Case 1, a 2-year-old boy, was admitted with macrohematuria for 5 months. Case 2, a 19-month-old boy, was referred for a palpable abdominal mass. The two cases were checked by 64-row multi-slice spiral CT (MSCT) and scanned with the dynamic contrast enhancement. The masses were excised and pathologically confirmed.

Results: In case 1, the mass occurred in the renal pelvis and calyx bilaterally, with heterogeneous density and prominent calcification. By contrast enhanced CT scan, the mass was mildly enhanced. In case 2, the left renal pelvis and ureter were filled with the tumor. Unenhanced scan revealed that the mass was homogeneous and non-calcified. In contrast, the mass was slightly and heterogeneously enhanced. Macroscopically, the mass filled in the pelvicalyceal system and had a botryoid appearance. Microscopically, the typical features of Wilms' tumor with blastemal, epithelial, and stromal components were evident.

Conclusion: Botryoid Wilms' tumor should be included in the differential diagnosis of tumors in the pelvicalyceal system no matter it is unilateral or bilateral.

Key words: botryoid Wilms' tumor; computed tomography; enhancement

World J Pediatr 2011;7(3):274-276

Author Affiliations: University Children's Hospital, Medical School Skopje, Macedonia (Lozanovski VJ); Department of Pediatric Nephrology, University Children's Hospital, Skopje, Medical School Skopje, Macedonia (Ristoska-Bojkovska N, Tasic V); Department of Biochemistry, Medical School, Skopje, Macedonia (Korneti P); Department of Pediatric Endocrinology and Genetics, University Children's Hospital, Skopje, Medical School Skopje, Macedonia (Gucev Z)

Corresponding Author: Vladimir J. Lozanovski, Universitätsklinik für Allgemein-, Viszeral- und Transplantationschirurgie, Universität Heidelberg, Im Neuenheimer Feld 132 E09, 69120 Heidelberg, Deutschland (Tel: +49 15 77 83 38 38 1; Email: v.lozanovski@yahoo.com)

Background: Oculocerebrorenal (Lowe) syndrome is an X-linked multisystem disease characterized by renal proximal tubulopathy, mental retardation, and congenital cataracts. We present a 19-year-old boy who was found to have low molecular weight proteinuria, hypercalciuria, mild generalized hyperaminoaciduria and intermittent microscopic hematuria at the age of 3.

Methods: Standard clinical and biochemical examinations and mutational analysis of the CLNC5 and OCRL1 gene were performed for the patient.

Results: The patient fulfilled diagnostic criteria for Dent disease, but lacked mutation in CLCN5. Sequencing of candidate genes revealed a mutation in his OCRL1 gene, which encodes for enzyme PIP2 5-phosphatase. The enzyme was not detected by western blot analysis, and decreased activity of the enzyme PIP2 5-phosphatase was observed in cultured skin fibroblasts. The boy had only mild mental retardation, mildly elevated muscle enzymes, but no neurological deficit or congenital cataracts, which are typical for Lowe syndrome.

Conclusions: Children with Dent phenotype who lack CLCN5 mutation should be tested for OCRL1 mutation. OCRL1 mutations may present with mild clinical features and are not necessarily associated with congenital cataracts.

Key words: cataracts; Dent disease; Lowe syndrome; OCRL1; CLCN5              

World J Pediatr 2011;7(3):280-283