Author Affiliations: Department of Psychiatry and Legal Medicine, Vall d'Hebron University Hospital, Universitat Autònoma de Barcelona, Spain (Braquehais MD, Casas M); Department of Child and Adolescent Psychiatry and Psychology, Sant Joan de Deu Hospital, Universitat de Barcelona, Spain (Picouto MD); Department of Psychiatry, James J. Peters Veterans' Administration Medical Center and Department of Psychiatry, Mount Sinai School of Medicine, New York, USA (Sher L)

 Corresponding Author: María Dolores Braquehais, MD, PhD, Department of Psychiatry and Legal Medicine, Vall d'Hebron University Hospital Universitat Autònoma de Barcelona, Pg. de la Vall d'Hebron, 119-129, 08035 Barcelona, Spain (Tel:  +34-93 489 42 94;  Fax: +34- 93 489 45 87; Email: mdbraquehais@vhebron.net)

 doi:10.1007/s12519-012-0358-0

Background: Biological markers of vulnerability for current or future risk of suicide in adolescents could be important adjuncts to the treatment and prevention of this phenomenon.

Data sources: We conducted a PubMed search of all English-language articles published between January 1990 and June 2011 using the following search terms:  ("hypothalamic-pituitary-adrenal" OR "HPA") AND ("adolescence" OR "adolescent" OR "teenager") AND ("depression" OR "major depressive disorder" OR "suicidal behavior" OR "suicidal ideation" OR "suicidal thoughts" OR "deliberate self-harm" OR "suicidal attempt" OR "suicide").

Results: HPA axis activity can be examined using different methods that do not have the same biological interpretation. An abnormal HPA axis functioning together with an anomalous interaction between HPA mechanisms and other systems such as the serotonergic system may be one of the neurobiological correlates of emotion dysregulation (ED). ED may play an important role in adolescent suicidal behavior. Some psychopathological conditions such as depression or childhood psychological trauma that increase suicidal risk in adolescents are also associated with HPA axis dysregulation. ED, a personality trait, can also be viewed as a predisposing factor that augments the vulnerability to suffer from psychiatric conditions.

Conclusions: Correlating HPA axis dysfunction with psychological factors such as ED could lead to a better understanding of the role of HPA abnormalities in adolescent suicide and may enhance preventive and treatment strategies.

Key words: adolescence; hypothalamic-pituitary-adrenal axis; suicide

World J Pediatr 2012;8(3):197-206

Author Affiliations: Queensland University of Technology, School of Exercise and Nutrition Sciences and Institute of Health and Biomedical Innovation, Victoria Park Rd, Kelvin Grove, Queensland, Australia 4059 (Rachele JN, Washington TL, Cuddihy TF); Centre for Functioning and Health Research, Queensland Health, 199 Ipswich Road, Woolloongabba, Queensland, Australia 4102 and Queensland University of Technology, School of Public Health and Social Work and Institute of Health and Biomedical Innovation, Victoria Park Rd, Kelvin Grove, Queensland, Australia 4059 (McPhail SM); Royal Melbourne Institute of Technology, 124 La Trobe Street, Melbourne, Victoria, Australia 3000 (Cuddihy TF)

Corresponding Author: Jerome N Rachele, Victoria Park Rd, Kelvin Grove, Queensland 4059, Australia (Email: jerome.rachele@connect.qut.edu.au)

Background: The accurate evaluation of physical activity levels amongst youth is critical for quantifying physical activity behaviors and evaluating the effect of physical activity interventions. The purpose of this review is to evaluate contemporary approaches to physical activity evaluation amongst youth.

Data sources: The literature from a range of sources was reviewed and synthesized to provide an overview of contemporary approaches for measuring youth physical activity.

Results: Five broad categories are described: self-report, instrumental movement detection, biological approaches, direct observation, and combined methods. Emerging technologies and priorities for future research are also identified.

Conclusions: There will always be a trade-off between accuracy and available resources when choosing the best approach for measuring physical activity amongst youth. Unfortunately, cost and logistical challenges may prohibit the use of "gold standard" physical activity measurement approaches such as doubly labelled water. Other objective methods such as heart rate monitoring, accelerometry, pedometry, indirect calorimetry, or a combination of measures have the potential to better capture the duration and intensity of physical activity, while self-reported measures are useful for capturing the type and context of activity.

Key words: accelerometer; instrument; measurement; physical activity; youth

World J Pediatr 2012;8(3):207-216

Author Affiliations: Department of Pediatrics, Faculty of Medicine, Department of King Abdulaziz University, Jeddah, Kingdom of Saudi Arabia (Kari JA)

Corresponding Author: Jameela Abdulaziz Kari, Consultant Pediatric Nephrologist, King Abdulaziz University, PO Box 80215, Jeddah 21589, Kingdom of Saudi Arabia (Tel: +996 55677904; Fax: +996 (2) 6684603; Email: jkari@ doctors.org.uk)

Background: Pediatric nephrology is a growing subspecialty in the Kingdom of Saudi Arabia (KSA). Pediatric nephrologists are challenged with a different spectrum of renal diseases. Moreover, there is a lack of epidemiological studies for most of these diseases. In this article, we discuss the spectrum of renal diseases in KSA and highlight the differences that exist between reports from KSA and those from other countries.

Data sources: PubMed and MEDLINE databases were searched for articles on pediatric renal diseases.

Results: Genetically mediated renal diseases are considerably high in KSA. Congenital and infantile nephrotic syndrome is higher in KSA than in other countries. Post-infectious glomerular pathology is rather common but is declining, while tropical infections such as schistosomiasis have been controlled. Neurogenic bladder caused by spinal lesion is an important cause of chronic kidney disease among pediatric patients. Renal stones are also more frequent in KSA than in other countries.

Conclusions: The spectrum of pediatric renal diseases in KSA is rather different from that reported from Western countries. More epidemiological studies are required to understand the actual incidence and nature of these diseases.

Key words: children; kidney anomalies; renal diseases

World J Pediatr 2012;8(3):217-221

Author Affiliations: Department of Pediatric Critical Care and Pharmacology, Rainbow Babies and Children's Hospital, University Hospitals, Case Medical Center, USA (Allareddy V); Nance College of Business Administration, Cleveland State University, Ohio, USA (Rampa S); Department of Developmental Biology, Harvard School of Dental Medicine, USA (Allareddy V)

Corresponding Author: Veerasathpurush Allareddy, Department of Developmental Biology, Harvard School of Dental Medicine, 188 Longwood Avenue, Boston, MA 02115, USA (Tel: 216-571-1009; Email: VA15@hsdm.harvard.edu)

doi: 10.1007/s12519-012-0361-5

Background: This study examines hospital charges and length of stay (LOS) associated with septicemia during hospitalization for leukemia treatment among children aged ≤18 years.

Methods: Nationwide Inpatient Sample (year 2008) was used. All hospitalizations (regardless of their discharge status following hospitalization) among children aged ≤18 years with a primary diagnosis of leukemia were selected. Leukemia was identified using ICD-9-CM codes in the primary diagnosis field. The presence of septicemia during hospitalization was identified using ICD-9-CM codes in the secondary diagnosis field. Multivariable linear regression analyses were made to examine the effect of septicemia on hospitalization charges and LOS.

Results: Totally 6220 hospitalizations were attributed to leukemia treatment. Among these, 787 had septicemia. The mean hospitalization charge for those with septicemia was $279 137 and for those without septicemia was $113 530. The average LOS for those with septicemia was 33.18 days while the LOS for those without septicemia was 13.79 days. Septicemia was associated with increased hospitalization charges and a prolonged duration of stay in hospital after adjustments for confounders (P<0.0001).

Conclusions: Septicemia is associated with adverse outcomes among children hospitalized for leukemia treatment.

Key words: health outcomes; leukemia; pediatric oncology; septicemia

World J Pediatr 2012;8(3):222-228

Author Affiliations: Institute of Radiology, Department of Pediatric Radiology, University Hospital Wuerzburg, Josef-Schneider-Straße 2, 97080 Wuerzburg, Germany (Neubauer H, Platzer I, Beer M); Department of Pediatrics, University Hospital Wuerzburg, Josef-Schneider-Straße 2, 97080 Wuerzburg, Germany (Mueller V, Liese J); Pediatric Surgery Unit, Department of Surgery, University Hospital Wuerzburg, Oberduerrbacher Str. 6, 97080 Wuerzburg, Germany (Meyer T); Institute of Radiology, University Hospital Wuerzburg, Oberduerrbacher Str. 6, 97080 Wuerzburg, Germany (Koestler H, Hahn D)

Corresponding Author: Henning Neubauer, MD, MBA, Institute of Radiology, Department of Pediatric Radiology, University Hospital Wuerzburg, Josef-Schneider-Straße 2, 97080 Wuerzburg, Germany (Tel: 0049-931-201-34715; Fax: 0049-931-201-34857; Email: neubauer@roentgen.uni-wuerzburg.de)

doi: 10.1007/s12519-012-0362-4

Background: Diffusion-weighted MRI (DWI) is helpful for detection of brain abscess and pelvic abscess in adults. In the present study, we evaluated the diagnostic performance of DWI in children and young adults with abdominal and soft tissue abscess formations.

Methods: Seventeen patients (11 females, aged 13 ± 6 years) with suspected abdominal or soft-tissue abscess underwent routine MRI including free-breathing DWI and contrast-enhanced T1w imaging. Seventeen random age-matched patients with non-purulent abdominal fluid collections served as controls. Mean apparent diffusion coefficent (ADC) was measured for abscess, muscle, liver, spleen and kidney tissue as well as for cerebrospinal fluid, urine and free abdominal fluid.

Results: All fluid collections were identified on diffusion-weighted images. Thirteen of 14 confirmed abscess formations showed an ADC < 1.0 × 10^-3 mm²/s with a mean value of 0.80 ± 0.38 mm²/s. One tuberculous soft-tissue abscess had a higher ADC of 1.85 × 10^-3 mm²/s. Ring enhancement on T1w imaging was seen in three non-purulent fluid collections. There were no false-positive findings in the control group.

Conclusions: Diffusion-weighted MRI is highly sensitive for abscess and may add specificity to contrast-enhanced T1w imaging of ring-enhancing fluid collections. DWI with free-breathing rapid image acquisition and without the need of intravenous contrast application constitutes a particularly useful choice in pediatric imaging.

Key words: abscess; contrast medium; diffusion-weighted imaging; magnetic resonance imaging; pediatric

World J Pediatr 2012;8(3):229-234

Author Affiliations: Department of Pathology (Ochicha O) and Department of Hematology (Gwarzo AK, Gwarzo D), Bayero University/Aminu Kano Teaching Hospital, Kano, Nigeria

Corresponding Author: Ochicha Ochicha, Pathology Department, Aminu Kano Teaching Hospital, PMB 3452 Kano, Nigeria (Email: ochicha@gmail.com)

doi: 10.1007/s12519-012-0363-3

Background: With effective immunization control of several devastating childhood infections in the developing world, non-infectious diseases such as malignancies have become increasingly important causes of pediatric morbidity and mortality. Therefore this 10-year retrospective study was undertaken to evaluate and document the pattern of childhood cancers in our locality.

Methods: We reviewed 438 childhood (≤15 years) malignancies diagnosed at the histopathology and hematology laboratories of our teaching hospital in a 10-year period (2001-2010).

Results: The 438 malignancies comprised 10.9% of all cancers. The maligancies frequently seen in early childhood (0-4 years) accounted for 46.1% and in late childhood (5-9 years) for 34.7%. Retinoblastoma (30.6%), Burkitt lymphoma (19.9%) and acute leukemia (16.9%) were the most common pediatric cancers. Unlike in most other parts of the world, acute myeloid leukemia was slightly more prevalent than acute lymphoblastic leukemia.

Conclusions: Although there were notable differences, our findings were in broad agreement with those of most other sub-Saharan African series, but differed markedly from those in the Western world and other high income countries. Further studies are required to identify the environmental factors for the high prevalence of non-familial retinoblastoma and possibly acute myeloid leukemia.

Key words: acute leukemia; Nigeria; pediatric cancer; retinoblastoma

World J Pediatr 2012;8(3):235-239

Author Affiliations: Department of Pediatrics, Shengjing Hospital of China Medical University, No. 36 Sanhao Street, Heping District, Shenyang 110004, Liaoning, China (Xu W, Liu CF, Zhao Y, Li JJ, Wang LJ, Wen GF, Liu Z)

Corresponding Author: Chun-Feng Liu, MD, Department of Pediatrics, Shengjing Hospital of China Medical University, No. 36 Sanhao Street, Heping District, Shenyang 110004, Liaoning, China (Tel: +8602423692117; Fax: +8602483955509; Email: xuw@sj-hospital.org)

doi: 10.1007/s12519-012-0364-2

Background: This article reviews the chest radiography of children with severe infection caused by a novel influenza A (H1N1) virus of swine origin (S-OIV). We analyzed the role of their pulmonary images in predicting the severity and diagnosis of the disease.

Methods: Among 97 patients with confirmed novel H1N1 infection, 42 patients treated with mechanical ventilation formed group 1, and the remaining 55 patients constituted group 2. The initial and subsequent radiograhic findings in groups 1 and 2 were compared with respect to the pattern, distribution, and extent of the abnormality.

Results: In group 1, 24 patients presented with three or more lung zone diseases, whereas only 5 patients in group 2 demonstrated these findings (P<0.001). A pneumomediastinum or pneumothorax was observed in 24/42 patients in group 1 and in 18/55 patients in group 2 (P=0.019). Twelve patients in group 1 and 5 in group 2 developed a ground-glass opacity cyst with a honeycomb appearance (P=0.007).

Conclusions: The most common radiographic and computed tomography findings in children who were severely infected with S-OIV included unilateral or bilateral ground-glass opacities with or without associated focal or multifocal areas of consolidation. Children with bilateral involvement or with greater opacity on the chest radiographs were more likely to worsen and require the mechanical ventilation.

Key words: chest radiography; children; H1N1; infectious diseases; pneumonia

World J Pediatr 2012;8(3):240-246

Author Affiliations: Department of Pediatrics, GSVM Medical College, Kanpur, India (Rao YK, Kumar P, Tripathi VN); Department of Community Medicine, GSVM Medical College, Kanpur, India (Midha T); Department of Pulmonary Medicine, GSVM Medical College, Kanpur, India (Rai OP)

Corresponding Author: Yashwant Kumar Rao, L-20, GSVM Medical College, Kanpur-208002, India (Email: ykraoneo@yahoo.co.in)

doi: 10.1007/s12519-012-0365-1

Background: In developing countries, facilities for measuring arterial oxygen saturation are not available in most settings, which make it difficult for health providers to detect hypoxemia in children with acute respiratory tract infection (ARI). Most health providers rely on symptoms and signs to identify hypoxemia and start oxygen therapy. Therefore, this study was conducted to determine the clinical predictors of hypoxemia in children with ARI.

Methods: It was a cross-sectional study carried out at the Pediatric Emergency Department of GSVM Medical College, Kanpur, India in children in the age group between 2 months and 5 years, presenting with ARI. All children with ARI attending the pediatric emergency department from April 2007 to September 2008 were included in the study. Clinical signs and symptoms including fever, cough, nasal flaring, inability to feed/drink, cyanosis, chest wall retraction, wheezing, grunting, tachypnea and crepitations were noted and oxygen saturation (SpO₂) was measured. Hypoxemia was defined as SpO₂ <90%.

Results: Of the 261 children included in the study, 62 (23.8%) had hypoxemia. Chest wall retraction (sensitivity=90%), crepitations (sensitivity=87%), nasal flaring (sensitivity=84%), tachypnea (sensitivity=81%) and inability to feed (sensitivity=81%) were observed to be the most sensitive indicators of hypoxemia while the best predictors were cyanosis [positive predictive value (PPV)=88%] and nasal flaring (PPV=53%).

Conclusions: Chest wall retraction was found to be the most sensitive indicator, and cyanosis was the most specific indicator for hypoxemia. Of all the clinical signs and symptoms of hypoxemia, none had all the attributes of being a good predictor. A new hypoxemia score has been designed using a combination of clinical signs and symptoms to predict the need for supplemental oxygen therapy.

Key words: acute respiratory tract infections; hypoxemia; hypoxemia score; pulse oximetry

World J Pediatr 2012;8(3):247-251

Author Affiliations: Department of Pediatric HIV, B.J. Wadia Hospital for Children, Mumbai, India (Shah I, Gupta S, Shah DM, Dhabe H, Lala M)

Corresponding Author: Ira Shah, 240 D. Walkeshwar Road, Malabar Hill, Mumbai 400006, India (Tel: 91-22-32905610; Email: irashah@pediatriconcall.com)

doi: 10.1007/s12519-012-0366-0                                                                                                                                            

Background: There are several studies on renal manifestations in human immunodeficiency virus (HIV) infected children from American and African regions, but similar studies from India are lacking. A cross-sectional study was carried out in 28 HIV infected antiretroviral therapy (ART) naïve children coming to the pediatric HIV clinic.

Methods: Demographic data of the children, clinical presentations including blood pressure, detailed laboratory investigations (serum creatinine, glomerular filtration rate), urine analysis (urine morphology, urine albumin, pus cells, and red blood cells), and CD4 counts were collected.

Results: Of the 28 children, 15 (53.6%) had renal manifestations with a male to female ratio of 1:1.5. The most common renal manifestation in our study was abnormal glomerular filtration rate (GFR) in 11 (44.0%) of 25 children. This was followed by pus cells in urine in 6 (21.4%) of the 28 children while 3 (10.7%) of them had proteinuria. The mean age of children with renal manifestations was 5.04±2.75 years as compared to those without renal manifestations who had a mean age of 7.38±2.95 years (P=0.0390). CDC class and sex were not associated with renal manifestations.

Conclusions: Our study suggests that reduced GFR is the common renal manifestation, particularly in younger children. Other renal manifestations are related to proteinuria. The lack of correlation of CDC classification with renal manifestations mandates screening of children with HIV for renal disease. A more detailed study of renal manifestations in HIV-infected children is needed.

Key words: children; human immunodeficiency virus; renal manifestations

World J Pediatr 2012;8(3):252-255

Author Affiliations: Children's Hospital, Zhejiang University School of Medicine (Wang JH, Zhang W, Tou JF, Huang SJ, Liu WG, Xiong QX, Zhao ZY)

Corresponding Author: Zheng-Yan Zhao, MD, Children's Hospital, Zhejiang University School of Medicine, 57 Zhugan Xiang, Hangzhou 310003, China (Tel: 86-571-87061007 ext 12435; Fax: 86-571-87078641; Email: Zhaozy@zju.edu.cn)

doi:10.1007/s12519-012-0367-z

Background: The management of the contralateral asymptomatic side when a child with initial unilateral inguinal hernia undergoes herniorrhaphy continues to be controversial. Age less than 6 months at initial herniorrhaphy is considered as a high risk factor of the occurrence of metachronous contralateral inguinal hernia (MCIH). We performed herniorraphy for patients ≥1 year with initial unilateral hernia at one-day-set outpatient-surgery department without any intervention of contralateral groin. In this study, we reviewed the characteristics of development of MCIH in this condition and discuss the management strategies of MCIH.

Methods: The subjects of this study were children who were treated at our outpatient-surgery department from January 2006 to December 2006. A total of 2129 patients with initial unilateral hernia and aged ≥1 year underwent an ipsilateral herniorhhaphy only. Patients were followed up for the development of MCIH to 60 months. The Chi-square test was used for intergroup comparison, a level of P<0.05 was considered as statistically significant.

Results: Among these children 1341 (63.0%) were obtained 60 months follow-up data, 1146 (85.5%) were boys and 195 (14.5%) were girls. MCIH developed in 70 (5.2%) patients, 61 were boys and 9 were girls. In 570 patients aged 12-23 months, 43 developed MCIH (7.5%); in 564 patients aged 24-59 months, 21 developed MCIH (3.7%); and in 207 patients ≥60 months, 6 patients developed MCIH (2.9%), the difference between these groups was highly significant (P=0.004). In male patients, 30 right-sided MCIHs occurred after 423 initial left-sided herniorrhaphies (7.1%) and 31 left-sided MCIHs occurred after 723 initial right-sided herniorrhaphies (4.3%), difference between these two groups was significant (P=0.041). Seventy-seven percent of the MCIHs occurred within 1 year, 94% occurred within 2 years after initial herniorraphy.

Conclusions: As the overall incidence of MCIH in patients aged ≥1 year was 5.2%, routine contralateral groin exploration is not suggested. Transinguinal laparoscopy could be considered as an alternative of conventional "wait and see" policy, especially in patients less than 2 years or left-sided initial unilateral inguinal hernia. If "wait and see" policy is adopted, patients should be closely followed up for 2 years.

Key words: contralateral patent processus vaginalis; inguinal hernia; transinguinal laparoscopy

World J Pediatr 2012;8(3):256-259

Author Affiliations: Division of Neonatology (Salvador A, Schutzman DL), Division of Pediatric Infectious Diseases (Arbeter A), and Division of Pediatrics (Kerur B), Albert Einstein Medical Center, 5501 Old York Road, Philadelphia PA 19141, USA

Corresponding Author: David L Schutzman, MD, Albert Einstein Medical Center, 5501 Old York Rd., Philadelphia, PA 19141, USA (Tel: 215-456-6698; Fax: 215-456-6769; Email: schutzmand@einstein.edu)

doi: 10.1007/s12519-012-0368-y

Background: There are currently no standard recommendations regarding the optimal method to obtain a blood culture in neonates.

Methods: We performed an online survey of the membership of the Section on Perinatal Pediatrics of the American Academy of Pediatrics regarding their practices when drawing blood cultures. The survey included questions regarding the type of antisepsis used in preparing the site for sampling, the amount of blood drawn and preferred site for obtaining the culture.

Results: Overall 715 of 2955 (24%) members responded to the survey. There was wide variability in responses to all of the questions. However, virtually all providers washed their hands and wore gloves while performing the procedure, and virtually all providers obtained ≥0.5 mL of blood for the sample.

Conclusions: Given the wide variability of practices among the members of the Section, evidence-based standards are needed to guide clinical practice for this procedure.

Key words: blood culture; infection; neonate

World J Pediatr 2012;8(3):260-262

Author Affiliations: Department of Infection Control, Women's Hospital, Zhejiang University School of Medicine, Hangzhou 310006, China (Lin R, Wu B, Xu XF, Liu XC, Ye H, Ye GY)

Corresponding Author: Xin-Fen Xu, MD, Women's Hospital, Zhejiang University School of Medicine, Hangzhou 310006, China (Tel: 86-0571-89991038; Fax: 86-0571-87061878; Email: xuxinfen@zju.edu.cn)

doi:10.1007/s12519-012-0370-4

Background: A molecular epidemiological survey was conducted on an extended-spectrum beta-lactamase-producing Klebsiella pneumoniae (ESBLKp) infection in our neonatal intensive care unit (NICU) from February to June 2008.

Methods: Cultures of clinical samples from neonates in the NICU, the hands of healthcare workers and the environment of the NICU were subjected to ESBLKp isolation. Pulsed-field gel electrophoresis was performed to determine Klebsiella pneumoniae strains (type A-D).

Results: In 1439 neonates, 38 (2.6%) had infections and 65 (4.5%) had colonizations with ESBLKp. Microbiological sampling of the NICU environment yielded 33 (14.9%) ESBLKp isolates from 222 samples. Clone A was found in 88.2% of the infected neonates, 66.7% of the colonized neonates, 69.7% of the environmental samples, and the hands of a healthcare worker.

Conclusions: The detection rate of ESBLKp is high in environmental samples, especially those from frequently touched surfaces. Since ESBLKp was identified on the hands of a healthcare worker in the present study, hand and environmental hygiene is mandatory for infection control in neonatal intensive care units.

Key words: infection; Klebsiella Pneumoniae; molecular epidemiology; neonatal intensive care unit

World J Pediatr 2012;8(3):268-271

Author Affiliations: Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany (Reismann M, Glueer S, Ure BM, Metzelder ML); Institute of Radiology, Northern City Hospital Hannover, Hannover, Germany (Gossner J); Department of Pediatric Pulmonology and Neonatology, Hannover Medical School, Hannover, Germany (Schwerk N)

Corresponding Author: Martin L. Metzelder, Hannover Medical School - Pediatric Surgery, Carl-Neuberg-Str. 1, Hannover 30625, Germany (Email: mmetzelder@yahoo.com)

doi: 10.1007/s12519-011-0283-7

Background: The size of congenital pulmonary malformation (CPM) in infants might interfere with the feasibility of thoracoscopic resection. This study was undertaken to evaluate the impact of the size of CPM on the applicability of video-assisted thoracic surgery (VATS) in infants.

Methods: Twenty-two infants were operated on for CPM from November 2000 to June 2009. The intra- and postoperative course was analyzed retrospectively from patient charts. Preoperative scans were evaluated blindly by a radiologist to calculate the relation between the maximum size of the lesion and the thoracic diameter in VATS and open procedures.

Results: VATS was performed in 14 (64%) of the 22 patients and thoracotomy in 8. VATS was successfully performed in 11 (79%) of the 14 patients, whereas VATS was converted to thoracotomy due to lack of overview in 3 (21%). The mean relative size of CPM at preoperative imaging was 0.34 ± 0.05 (range: 0.3-0.4) in patients who received successful VATS, 0.57 ± 0.06 (range: 0.5-0.6) in converted cases, and 0.68 ± 0.10 (range: 0.5-0.8) in infants who underwent thoracotomy. The relative CPM size was significantly lower in successful VATS than in cases of conversion (P<0.01) and thoracotomy (P<0.01).

Conclusions: The relative size of CPM at preoperative imaging might be useful information for a decision-making on the use of VATS in infants. A relative CPM size below 0.5, which is less than half of the thoracic diameter, indicates a good feasibility for thoracoscopic resection of CPM. A larger size may indicate that VATS might be technically difficult.

Key words: children; congenital pulmonary malformation; feasibility; video-assisted thoracic surgery

World J Pediatr 2012;8(3):272-274

Author Affiliations: Division of Pediatric Cardiology (Zhou KY, Hua YM) and Division of Ultrasound Cardiography (Zhu Q), West China Second University Hospital, West China Medical School, Sichuan University, Chengdu, China.

Corresponding Author: Qi Zhu, MD, Division of Ultrasound Cardiography, West China Second University Hospital, West China Medical School, Sichuan University, Chengdu 610041, China (Tel: +86 28 85501651; Fax: +86 28 85503744; Email: zhuqidq101@163.com)

doi: 10.1007/s12519-011-0306-4

Background: Without timely treatment, fetal atrial flutter (AF) could result in congestive heart failure, hydrops fetalis and even fetal demise.

Methods: Prenatal echocardiography was used to confirm AF and assess fetal cardiac function with cardiovascular profile score. Transplacental digoxin therapy was adopted, and the patient was followed up for 10 months.

Results: The healthy male baby was delivered with normal postnatal electrocardiogram and echocardiogram. Neither arrhythmia nor neurodevelopmental impairment was found during the follow-up.

Conclusion: Timely transplacental digoxin therapy can successfully treat fetal AF and allow the fetus to recover from AF associated fetal heart failure and hydrops fetalis prior to delivery.

Key words: fetal atrial flutter; heart failure; hydrops fetalis; transplacental digoxin therapy

World J Pediatr 2012;8(3):275-277

Author Affiliations: Department of Pediatrics (Hui J, Chung C, Yuen P), Joint Metabolic Clinic (Hui J, Law E, Chung C, Tang N), and Department of Chemical Pathology (Law E, Fung S, Tang N), Prince of Wales Hospital, The Chinese University of Hong Kong, China

Corresponding Author: Joannie Hui, Department of Pediatrics, The Chinese University of Hong Kong, China (Email: joanniehui@cuhk.edu.hk)

doi: 10.1007/s12519-011-0301-9

Background: Holocarboxylase synthetase deficiency is an inborn error of biotin metabolism leading to multiple carboxylase deficiency which is often biotin responsive. This disease is believed to be relatively common among the Asian population.

Methods: A 6-year-old Vietnamese boy presented with recurrent episodes of severe metabolic acidosis precipitated by intercurrent illnesses. An extensive skin rash was present since the onset of his illness. Multiple carboxylase deficiency was considered a likely diagnosis based on the history and the characteristic skin rash.

Results: This diagnosis was later confirmed by urine organic acid and molecular genetic studies. Urine organic acid showed characteristic excretion of glycine conjugates. Serum biotinidase activity was normal. Sequencing of the holocarboxylase synthetase gene revealed the patient being homozygous for a common mutation R508W. The patient showed a dramatic response to biotin within days of its administration.

Conclusion: This case illustrates a potential highly treatable inborn error of metabolism that can be recognized on clinical grounds and its favorable response to biotin treatment.

Key words:  biotin; biotinidase deficiency; HLCS gene; holocarboxylase synthetase deficiency; inborn error of metabolism; multiple carboxylase deficiency; rash

World J Pediatr 2012;8(3):278-280

Author Affiliations: Department of Pediatrics, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853, China (Li XY, Shi XY, Ju J, Yang XF, Zou LP); Department of Pediatrics, First Affiliated Hospital of Chinese PLA General Hospital, 51 Fucheng Street, Beijing 100037, China (Hu XH)

Corresponding Author: Li-Ping Zou, Department of Pediatrics, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853, China (Tel: +86 10 55499016; Fax: +86 10 55499016; Email: zouliping21@hotmail.com)

doi: 10.1007/s12519-012-0357-1

Background: Mucopolysaccharidosis type II (MPS II; also known as Hunter syndrome) is an X-linked multisystem disorder resulting from the defective activity of the enzyme iduronate-2-sulfatase (IDS). Genetic testing is crucial in clarifying and diagnosing different types of MPS diseases. In this paper we report a novel IDS nonsense mutation resulting in MPS II in several patients from a Chinese family.

Methods: IDS enzyme activity, polymerase chain reaction, and DNA sequencing were performed to confirm the diagnosis of MPS II.

Results: Three patients had no detectable IDS activity. Two genetic tests revealed a novel IDS nonsense mutation (c.1030G>T, p.E344X) inherited from their mothers. The nonsense mutation shortened the peptide chain from 550 to 344 amino acids, which is believed to be a disease-causing mutation.

Conclusions: MPS II is inherited in an X-linked manner. The risk to sibs depends on the carrier status of the mother. Genetic testing is necessary to identify disease-causing mutation. With this information, carrier testing for at-risk female relatives and prenatal testing for pregnancies at increased risk become possible.

Key words: iduronate 2-sulfatase gene; mucopolysaccharidosis II; mutation

World J Pediatr 2012;8(3):281-283