Author Affiliations: Department of Pediatrics, Shanghai First People's Hospital, Shanghai Jiaotong University, Shanghai 200080, China (Wang XF, Hong JG)

Corresponding Author: Jian-Guo Hong, MD, Department of Pediatrics, Shanghai First People's Hospital, Shanghai Jiaotong University, Shanghai 200080, China (Tel: +86-13501803655; Fax: +86-21-37798435; Email: hongjianguo@hotmail.com)

doi: 10.1007/s12519-011-0325-1

Background: Asthma is a common disease in children and acute severe asthma exacerbation can be life-threatening. This article aims to review recent advances in understanding of risk factors, pathophysiology, diagnosis and treatment of severe asthma exacerbation in children.

Data sources: Articles concerning severe asthma exacerbation in children were retrieved from PubMed. Literatures were searched with MeSH words "asthma", "children", "severe asthma exacerbation" and relevant cross references.

Results: Severe asthma exacerbation in children requires aggressive treatments with β2-agonists, anticholinergics, and corticosteroids. Early initiation of inhaled β-agonists and systemic use of steroids are recommended. Other agents such as magnesium and aminophylline have some therapeutic benefits. When intubation and mechanical ventilation are needed, low tidal volume, controlled hypoventilation with lower-than-traditional respiratory rates and permissive hypercapnia can be applied.

Conclusions: Researchers should continue to detect the risk factors, pathophysiology, diagnosis and treatment of severe asthma exacerbation in children. More studies especially randomized controlled trials are required to evaluate the efficacy and safety of standard and new therapies.

Key words: asthma; children; severe asthma exacerbation

                 World J Pediatr 2011;7(4):293-301

Author Affiliations: National Center for Birth Defects Monitoring (Dai L, Zhu J, Liang J, Wang YP), Department of Obstetric and Gynecology (Wang H), and Pediatric Department (Mao M), West China Second University Hospital, Sichuan University, Chengdu 610041, China

Corresponding Author: Meng Mao, Department of Pediatrics, West China Second University Hospital, Sichuan University, 20 Section 3 of Renmin Nanlu, Chengdu 610041, China (Tel: +86-28-85501363; Fax: +86-28-85501389; Email: dffmmao@126.com)

doi: 10.1007/s12519-011-0326-0

Background: Birth defects are a global public health problem because of their large contribution to infant mortalities and disabilities. It is estimated that 4%-6% of Chinese newborns are affected by birth defects every year. Surveillance is a basic approach to understanding the occurrence and associated factors of birth defects. The Ministry of Health of China initiated a national hospital-based birth defects monitoring system 20 years ago. Nearly every province in this country has established its own surveillance system in the past. The authors reviewed the result of the monitoring system at different administrative levels in China.

Data sources: Available publications on the surveillance of birth defects and data from national and provincial birth defects surveillance systems were reviewed to evaluate the effectiveness of the surveillance systems.

Results: According to the 2009 data, the national hospital-based birth defects surveillance system monitored over 1.3 million births, which accounted for more than 8% of births in China. In addition, 30 provincial hospital-based surveillance programs covered a birth population of more than 3.6 million (22% of births in China). Great achievements have been made in terms of case ascertainment, data quality control, and online reporting. But the surveillance systems in China still have some limitations. A short ascertainment period may miss some internal anomalies, inherited metabolic diseases, and malformed fetus aborted before the 28th gestational week. Discrepancies in antenatal or postnatal diagnosis of birth defects between surveillance institutes may affect the detection rate and introduce biases. Absence of baseline data and lack of integrated database systems limit the application of surveillance data to etiological studies and affect the process of decision-making.

Conclusions: The surveillance system for birth defects is prerequisite to propose, conduct and assess any interventions for the disease. To meet the need of study and prevention of birth defects, measures should be taken to improve the national and provincial birth defects surveillance systems in China.

Key words: birth defects; China; surveillance

World J Pediatr 2011;7(4):302-310

Tehran, Iran

Author Affiliations: Sina Trauma and Surgery Research Center, Tehran University of Medical Sciences, Tehran, Iran (Rasouli MR, Rahimi-Movaghar V, Maheronnaghsh R, Yousefian A); Research Centre for Neural Repair, University of Tehran, Tehran, Iran (Rahimi-Movaghar V); Department of Orthopaedics and Neurosurgery, Thomas Jefferson University and the Rothman Institute, Philadelphia, PA, USA (Rasouli MR, Vaccaro AR)

Corresponding Author: Vafa Rahimi-Movaghar, MD, Associate Professor of Neurosurgery, Sina Trauma and Surgery Research Center, Sina Hospital, Hassan-Abad Square, Imam Khomeini Ave, Tehran University of Medical Sciences, Tehran, 11365-3876, Iran (Tel: (+98) 915 342 2682, (+98) 216 671 8311; Fax: (+98) 216 670 5140; Email: v_rahimi@sina.tums.ac.ir; v_rahimi@yahoo.com)

doi: 10.1007/s12519-011-0327-z

Background: Spinal cord injury (SCI) is a devastating event that results in permanent disability for injured children. Among all etiologies of SCI, motor vehicle crashes (MVCs) are the leading cause and account for 29% of all traumatic SCIs in children. We tried to evaluate types and mechanisms of MVC-related spinal column and spinal cord injuries, risk factors, safety issues and legislation.

Data sources: A literature review was performed using PubMed from 1966 to 12th April 2010 with the following key words: children OR pediatric, spine, injury OR trauma, restraint, seat belt, motor vehicle, road OR traffic, collision OR crash, safety. Cross referencing of discovered articles was also performed.

Results: Risk factors for MVC-related SCI include single vehicle crashes, vehicle rollover, and ejection of the passenger from the vehicle. Any anatomic region of the spinal cord may be injured as a result of MVC and may vary according to the type of accident and restraint system usage. Increasing use of three-point seat belts, which are more protective than isolated lap seat belts, has decreased the incidence of MVC-related SCI. There is evidence that airbag use without seatbelt use is associated with an increased risk of cervical spine fractures with or without SCI. Vehicle designers need to give more attention to the prevention of vehicle rollover and to improve occupant protection when rollover occurs.

Conclusions: MVC is a common cause of SCI in children; therefore, paying attention to risk factors and modes of prevention is important. As MVC-related SCI can lead to permanent disability, prevention and education play an important role in decreasing childrens' morbidity and mortality. Making behavior, roads and vehicles safer can significantly reduce MVC-related SCI in children.

Key words: children; injury; motor vehicle crash; spinal cord

                  World J Pediatr 2011;7(4):311-317

Author Affiliations: Department of Pathology, Christian Medical College, Vellore, India (Manipadam MT, Nair S); Department of Hematology, Christian Medical College, Vellore, India (Viswabandya A, Srivastava A, Chandy M); Department of Child Health, Christian Medical College, Vellore, India (Mathew L)

Corresponding Author: Marie Therese Manipadam, Associate Professor, Department of Pathology, Christian Medical College, Vellore- 632004, Tamil Nadu, India (Tel: 91-416-2282005; Fax: 91-416-2232035; Email: mtm2005@cmcvellore.ac.in)

doi: 10.1007/s12519-011-0303-7

Background: There is a dearth of published literature on the frequency and distribution of pediatric and adolescent non-Hodgkin lymphoma (NHL) in India according to the 2001 WHO classification. The aim of this study was to record the distribution of the different subtypes, analyze the major subtypes, and compare it with the published data from other countries. A review of pediatric NHL statistics from population-based cancer registries was included in this study.

Methods: The study was conducted using information retrieved from the files of our institution. A total of 467 patients with lymphoma (excluding mycosis fungoides) were recorded in the under 20 years group over a period of 6 years, of which 252 patients suffered from NHL. The demographic characteristics, frequency and distribution of different subtypes were noted and compared with published reports from other parts of the world.

Results: T-lymphoblastic lymphoma/leukemia constituted the majority (32.1%) of all NHLs in children and adolescents in our study. The other major subgroups were Burkitt's lymphoma, anaplastic large cell lymphoma, and diffuse large B cell lymphoma. Burkitt's lymphoma in this study had clinical presentations similar to those seen in western countries.

Conclusions: The distribution of different subtypes of lymphoma in pediatric and adolescent NHL in India differs considerably from that in western countries and other eastern countries.

Key words: adolescent; anaplastic large cell lymphoma; Burkitt's lymphoma; children; non-Hodgkin lymphoma

World J Pediatr 2011;7(4):318-325

Mansoura, Egypt

Author Affiliations: Department of Pediatrics, Faculty of Medicine, Mansoura University, Egypt (El-Morsy Z, Khashaba MT, Soliman OE, Yahia S, Abd El-Hady D)

Corresponding Author: Othman EL-Sayed Soliman, Mansoura University Children's Hospital El-Gomhoria St. Postal Code: 35516, Mansoura, Egypt (Tel: +20115691116; Fax: 002 050 2234092; Email: oesoliman65@yahoo.com)

Introduction: More than 200 mutations have been found in patients with Gaucher disease (GD) and some mutations usually have a high frequency in certain populations. Genotype/phenotype correlation in patients with GD has not been established. This study was designed to determine underlying mutations in Egyptian children with GD and to assess their relation to disease phenotypes.

Methods: This study comprised 17 patients with GD and 10 healthy controls. Thirteen patients were type 1 GD, 2 type 2, and 2 type 3. DNA was extracted from peripheral blood leukocytes. Exons 9 and 10 were amplified by polymerase chain reaction, and deoxyribonucleic acid sequencing was done with an ABI 310 genetic analyzer.

Results: Wild type allele was detected in 95% (19/20) and a normal variant in 5% (1/20) of controls. L444P allele was encountered in 50% (13/26) of the alleles in type 1 patients, H451P in 7.7% (2/26) and recombinant alleles (RecNcil, RecNcil + M450L, RecFs, RecFs + M450L) in 34.6% (9/26). L444P and Rec alleles each occurred in 50% (2/4) of type 2 and 3 patients. A new mutation was seen in this study {g.7336A>C, (M450L)} and 2 mutant alleles were not determined. Type 1 GD patients had L444P/L444P genotype (23.1%) and Rec alleles/L444P (53.8%), while type 2 and 3 GD patients had Rec alleles/L444P genotypes (100%) with a poor phenotype/genotype correlation.

Conclusions: L444P and Rec alleles are common in the studied patients. Novel mutations are continuously detected, adding to the expanding panel of GD mutations. No significant genotype-phenotype association was observed.

Key words: children; Gaucher disease; glucosidase acid beta mutation; phenotype

World J Pediatr 2011;7(4):326-330

Author Affiliations: The Neonatal Intensive Care Unit, Women's Hospital, Zhejiang University School of Medicine, Hangzhou, China (Sun G); Department of Urology, Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China (Xu ZM, Tang DX); Department of Information, Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China (Liang JF); Department of Women's Health Care, Women's Hospital, Zhejiang University School of Medicine, Hangzhou, China (Li L)

Corresponding Author: Ge Sun, The Neonatal Intensive Care Unit, Women's Hospital, Zhejiang University School of Medicine, Hangzhou 310006, China (Tel: +86 571 87061501 ext 2033; Fax: +86 571 87061878; Email: sunge051266@sina.com)

doi: 10.1007/s12519-011-0328-y

Background: One of the challenges that pediatricians face when examining birth defects is to understand the trends in its occurrence and provide clues to etiology. This study was undertaken to retrospectively assess the prevalence of 10 common neonatal congenital malformations by reviewing a database of all deliveries from 28 weeks up until 7 days of birth from January 1998 to December 2009 in Zhejiang Province, China.

Methods: Ten common neonatal congenital malformations were selected for analysis. The incidence  and the Cochran-Armitage Trend were assessed via SAS9.2. A P value ≤0.05 was considered statistically significant.

Results: Of 83 888 perinatals, 374 (4.46‰) suffered from congenital heart diseases (CHD), 77 (0.92‰) from congenital hydrocephalus, 32 (0.38‰) from intestinal atresia/stenosis, 36 (0.43‰) from anorectal malformations, 149 (1.78‰) from kidney malformations, 139 (3.31‰) from hypospadias (male), 178 (2.12‰) from orofacial clefts (OFC), 188 (2.24‰) from polydactyly, 62 (0.74‰) from syndactyly, and 269 (3.21‰) from accessory auricle anomaly. Their trend of prevalence varied as follows: CHD, P=0.0026; hydrocephalus, P=0.0042; intestinal atresia/stenosis, P=0.0103; anorectal malformations, P=0.4332; kidney malformations, P<0.0001; hypospadias, P=0.0021; OFC, P=0.005; polydactyly, P=0.0867; syndactyly, P=0.1941; and accessory auricle anomaly, P=0.0011. The upward trend was as follows: CHD (P=0.0026), intestinal atresia/stenosis (P=0.0103), kidney malformations (P<0.0001), hypospadias (P=0.0021), OFC (P=0.005), and accessory auricle anomaly (P=0.0011). The incidence of hydrocephalus (P=0.0042) showed a downward trend. No statistical significant trend was found in anorectal malformations (P=0.4332), polydactyly (P=0.0867) and syndactyly (P=0.1941).

Conclusions: The incidences of CHD, intestinal atresia/stenosis, kidney malformations, hypospadias, OFC, and accessory auricle anomaly have increased in the last 12 years, but the incidences of anorectal malformations, polydactyly and syndactyly remain stable. The incidence of hydrocephalus shows a downward trend.

Key words: congenital malformation; incidence; neonate

                                                                                                     World J Pediatr 2011;7(4):331-336

Corresponding Author: Won Soon Park, MD, Department of Pediatrics, Samsung Medical Center, 50 Irwon-dong, Kangnam-gu, Seoul 135-710, Korea (Tel: 82-2-3410-3523; Fax: 82-2-3410-0043; Email: wonspark@skku.edu)

doi: 10.1007/s12519-011-0281-9

Background: We detected swallowing dysfunction by the modified barium swallow (MBS) test and determined risk factors for swallowing dysfunction in very low birth weight (VLBW) infants with oral feeding desaturation near discharge.

Methods: We retrospectively reviewed 41 VLBW infants referred for MBS test because of significant oral feeding desaturation at ≥35 weeks of postmenstrual age. Infants who showed impaired airway protection, including inadequate epiglottic closure, laryngeal penetration and/or tracheal aspiration by MBS test, were compared to those without impaired airway protection.

Results: Eleven infants (26.8%) showed impaired airway protection by MBS test. They had a significantly lower gestational age at birth but a similar postmenstrual age compared to those without impaired airway protection. All infants with impaired airway protection were born at ≤28 weeks of gestation.

Conclusions: Swallowing dysfunction resulting in aspiration should be considered as a cause of significant oral feeding desaturation in infants born at ≤28 weeks of gestation regardless of postmenstrual age.

Key words: aspiration; modified barium swallow; penetration; prematurity

World J Pediatr 2011;7(4):337-343

Author Affiliations: Autonomous University of Tamaulipas (UAT), University Center Tampico-Madero, Tamaulipas, México (Vázquez EM, Barrientos MC, Córdova JA, Lin D, Beltrán FJ); Mexican Institute of Social Security (IMSS), Madero city, Tamaulipas, México (Vázquez F); Public Health Institute, Veracruzana University, México (Vázquez CF)

Corresponding Author: Eliza M. Vázquez, MD, Faculty of Medicine of Tampico. Autonomous University of Tamaulipas (UAT), 89339 University Center Tampico-Madero, Tamaulipas, Mexico (Tel: (52) (833) 216-5247; Email: dra.eliza.vazquez@gmail.com)

doi: 10.1007/s12519-011-0300-x

Background: Asthma has been associated with a great number of negative health outcomes. This study was undertaken to detect the association between asthma and dental caries in the primary dentition.

Methods: Data were obtained from a cohort of 1160 Mexican children aged 4-5 years. We used the questionnaire of the International Study of Asthma and Allergies Diseases in Childhood. Asthma was classified according to parents' reports. Caries indexes were measured as the number of decayed (d), missing (m), and filled (f), teeth (t) (dmft) or surfaces (dmfs). Decayed teeth included initial caries in this study. Adjusted odds ratios (adjusted ORs) were determined for asthma using logistic regression model. Gender, sugary products consumption, and oral hygiene habits were utilized as covariates.

Results: The prevalence of dental caries was 17.9% in the 1160 children. Approximately 226 (19.5%) children were identified with asthma. Among them, 166 (73.5%) presented with symptoms during the day and 60 (26.5%) during the day and night. The prevalence of caries in children with asthma was 19.9%; it was higher in children with nocturnal asthma symptoms than in those with asthma symptoms only during the day. Logistic regression model showed that asthma (adjusted OR=1.24; 95% confidence interval [95% CI]=0.84–1.81) was not associated with caries. A significant association was found between nocturnal asthma symptoms (adjusted OR=1.85; 95% CI=1.00–3.44) and dental caries.

Conclusions: Asthma is not associated with dental caries. Nocturnal asthma symptoms appear to be associated with dental caries in the primary dentition.

Key words: asthma; dental caries; tooth deciduous

World J Pediatr 2011;7(4):344-349

Author Affiliations: Department of Genetics and Metabolism (Chen XX, Zhou XL, Yang RL, Shi YH, Mao HQ, Qu YP, Wang X); Department of Preventive Medicine and Child Health Care, Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China (Qin YF, Zhao ZY)

Corresponding Author: Zheng-Yan Zhao, Department of Preventive Medicine and Child Health Care, Children's Hospital, Zhejiang University School of Medicine, 57 Zhu Gan Xiang, Hangzhou, 310003, China (Tel: +86-571-87061007-2435; Fax: +86-571-87033296; Email: zhaozy@zju.edu.cn)

doi: 10.1007/s12519-011-0314-4

Background: This study was undertaken to explore the clinical outcome and prognosis of subclinical hypo-thyroidism detected by newborn screening.

Methods: Newborn screening was conducted at 1156 health care institutions in Zhejiang Province from October 1999 to September 2006. Included were (1) infants who had thyroid-stimulating hormone (TSH) ≥20 mU/L, and normal or lower normal levels of triiodothyronine (T₃) and thyroxine (T₄) and (2) infants with TSH between 5.6 mU/L and 20 mU/L at a confirmatory examination and follow-up showing TSH levels ≥20 mU/L or delayed reduction in T₄ levels. These infants were considered as having subclinical hypothyroidism and levothyroxine (L-T₄) at an initial dose of 3-5 μg/kg per day was administered. The levels of TSH and T₄, developmental quotient (DQ), and index of growth were evaluated.

Results: A total of 204 infants met our criteria for subclinical hypothyroidism, with an incidence of 1/8809. After 2-4 weeks of standard therapy, serum TSH level dropped to normal and T₄ reached a higher normal level in all the 204 infants. Evaluations of 60 patients after 2 years of therapy showed that their average DQ was 101±14.61, and body weight and height were within the normal ranges. Bone age test for 54 patients revealed normal development in 44, slightly retarded development in 7, and advanced development in 3.

Conclusions: Newborns with high TSH levels should be given particular attention to ensure early diagnosis. A L-T₄ dose of 3-5 μg/kg per day was effective in the initial treatment of subclinical hypothyroidism.

Key words: congenital hypothyroidism; diagnosis; L-thyroxine; newborn screening

World J Pediatr 2011;7(4):350-354

Author Affiliations: Department of Neonatology (Mutlu M, Aslan Y), Department of Pediatric Endocrinology (Karagüzel G, Ökten A), and Department of Radiology (Cansu A), Karadeniz Technical University, Faculty of Medicine, Trabzon, Turkey

Corresponding Author: Mehmet Mutlu, Department of Pediatrics, Karadeniz Technical University, Faculty of Medicine Trabzon, Turkey (Tel: 0462 377 5568; Email: drmehmetmutlu38@hotmail.com)

doi: 10.1007/s12519-011-0259-7

Background: Adrenal hemorrhage (AH) is a relatively uncommon condition in neonates. This study aimed to review the clinical, laboratory and ultrasonographic findings of AH in newborns.

Methods: The medical records of 13 newborns with AH who had been admitted to our neonatal intensive care unit were retrospectively reviewed.

Results: Of the 13 newborns with AH, 8 (62%) were term and 10 (77%) were male babies. Clinical presentations included neonatal jaundice (85%), paleness and/or flank mass (38%), discoloration of the scrotum (15%), and hypotonia/lethargy or hypotension (8%). Five newborns had anemia and four had adrenal insufficiency. Adrenal insufficiency was observed in 80% of the premature infants with AH. AH occurred on the right side in 9 patients (69%). The most predisposing cause of AH was disseminated intravascular coagulation secondary to sepsis or perinatal hypoxia in preterm infants, and large for gestational age in term infants. Ultrasonography (USG) revealed a hypoechoic mass in 7 newborns (54%), a mixed solid-liquid mass in 5 (38%), and an echogenic mass (8%) in 1. Hemorrhage disappeared within 8.6±4.5 (4-16) weeks.

Conclusions: AH occurs in the newborns with unexplained jaundice. Adrenal insufficiency is more frequent in preterm than in mature infants. Abdominal USG is required to determine AH in a newborn with swelling and bluish discoloration of the scrotum. Serial USG is the best modality for monitoring AH to prevent unnecessary surgery.

Key words: adrenal hemorrhage; newborn; ultrasonography

World J Pediatr 2011;7(4):355-357

Author Affiliations: Clinical Research Dept., Pasteur Institute of Iran, Tehran, Iran (Aghakhani A, Khadem-Sadegh A, Eslamifar A, Ramezani A); Iranian Society for Support of Patients with Infectious Diseases, Tehran, Iran (Banifazl M); Iranian Blood Transfusion Organization Research Center, Iran (Izadi N); University of California, San Francisco, USA (McFarland W); Arak University of Medical Sciences, Arak, Iran (Sofian M); Pediatric Dept., Shahid Beheshti University of Medical Sciences, Tehran, Iran (Pournasiri Z); Iranian Research Center for HIV/AIDS, Tehran, Iran (Foroughi M)

Corresponding Author: A Ramezani, Clinical Research Dept., Pasteur Institute of Iran No 69, Pasteur Ave., Tehran, 13164, Iran (Tel: +98 21 66968852; Fax: +98 21 66465147; Email: amitisramezani@hotmail.com; iiccom@iiccom.org)

doi: 10.1007/s12519-011-0286-4

Background: A potential problem of hepatitis B immunization is that vaccine-induced antibody to hepatitis B surface antigen (anti-HBs) declines to low levels with age. This study investigated the persistence of anti-HBs in vaccinated children in a low hepatitis B virus (HBV) endemic area.

Methods: Plasma samples of 938 children between ages of 8 months and 15 years were tested for the presence of anti-HBs.

Results: The seroprotection rate was 60%. Protective antibody level was detected in 65% of children one year after vaccination, and in 30%, 29% and 24% 5, 10 and 15 years after vaccination, respectively. The mean anti-HBs titer declined with post-vaccination time (to 66 mIU/mL in 1 year, 60 mIU/mL in 5 years, 40 mIU/mL in 10 years to 37 mIU/mL in 15 years after vaccination).

Conclusions: Children vaccinated against HBV during infancy may show low levels of antibody during adolescence. Our data suggest that a booster dose of vaccine may be required in low HBV endemic areas.

Key words: children; hepatitis B surface antibody; hepatitis B virus; Iran

World J Pediatr 2011;7(4):358-360

Author Affiliations: Pediatric Surgery Unit, Department of Surgery, Faculty of Medicine, Prince of Songkla University, Hat Yai, Songkhla 90110, Thailand (Laohawiriyakamol S, Sangkhathat S, Chiengkriwate P, Patrapinyokul S)

Corresponding Author: Surasak Sangkhathat, MD, PhD, Pediatric Surgery Unit, Department of Surgery, Faculty of Medicine, Prince of Songkla University, Hat Yai, Songkhla 90110, Thailand (Email: surasak.sa@psu.ac.th)

Background: Snakebite is common in children especially in the developing countries. This study was undertaken to determine the role of surgery in the treatment of venomous snake bite in pediatric patients.

Methods: The clinical data of 58 pediatric patients aged 0-16 years who had been treated for venomous snakebite from January 1999 to December 2008 were analyzed.

Results: Of the 58 patients, 43 (74.6%) were male. Peak age incidence was around 2-3 years (28.8%). The majority of envenomations occurred in the summer and rainy seasons, especially in the latter, during flooding. The bites occurred during 6 pm to 12 pm in 27 patients (49.0%). The main bite site was the lower extremities in 49 patients (83.9%). The main species of the snake were Malayan pit viper (Calloselasma rhodostoma) in 28 patients (47.5%) and cobra (Ophiophagus hunnah or Naja spp.) in 21 patients (35.6%). Soft tissue necrosis occurred more in cobra bites (47.6%) than viper bites (3.6%). The most common organism identified in necrotic tissue was Morganella morgagnii. Four patients with cobra bite had respiratory failure that required ventilatory support. Compartment syndrome was suspected in 2 patients. Surgical intervention was necessary in 13 patients. Most procedures involved serial wound debridement, followed by skin grafting. One case needed a toe amputation because of necrosis. The average length of hospital stay in patients who needed surgical management was 18.8 days (range: 12.1-25.5 days). There were no mortalities.

Conclusions: Surgery plays an important role in the management of snakebite patients, especially for those with cobra bite with tissue necrosis.

Key words: children; snakebite; surgery

                   World J Pediatr 2011;7(4):361-364

Ankara, Turkey

Author Affiliations: Department of Pediatric Nephrology and Rheumatology (Gökçe İ, Gök F), Department of Pediatric Gastroenterology (Gökçe S), Department of Pediatric Cardiology (Kılıç A), Department of Radiology (Bozlar U, Kocaoğlu M), and Department of Pathology (Öngürü Ö), Gülhane Military Academy of Medicine, School of Medicine, 06018, Etlik, Ankara, Turkey

Corresponding Author: İbrahim Gökçe, Department of Pediatric Nephrology, Gülhane Military Medical Academy, School of Medicine, 06018, Etlik, Ankara, Turkey (Tel:  0 (506) 7035001; Fax: 09 0312 304 43 81; Email: gokcemd@yahoo.com)

doi: 10.1007/s12519-011-0255-y

Background: Constrictive pericarditis (CP) represents a rare cause of protein-losing enteropathy (PLE) resulting from intestinal lymphangiectasia (IL). In this report, we describe an 8-year-old Turkish boy with IL and PLE secondary to CP.

Methods: The boy was introduced to our clinic due to bilateral pretibial edema and swelling of the eyelids caused by hypoproteinemia. Physical examination revealed a distended right jugular vein. Laboratory investigation revealed PLE with fecal concentration of alpha-1 antitripsin of 4.87 mg/g. Histopathologic examination of random biopsies obtained from the duodenum revealed markedly dilated lymphatics compatible with IL. Constrictive pericarditis was diagnosed by tagged cine cardiac magnetic resonance imaging.

Results: Pericardiectomy was performed for the patient. Genetic analysis was done and heterozygous mutation E148Q was detected as a disease-causing Mediterranean fever (MEFV) mutation. Colchicine was started after the operation. Six months after the initiation of regular colchicine therapy, echocardiography revealed disappearance of CP.

Conclusion: This is the first reported case of PLE with a distended right jugular vein due to CP secondary to familial Mediterranean fever associated with E148Q heterozygosity in the MEFV gene.

Key words: constrictive pericarditis; familial Mediterranean fever; intestinal lymphangiectasia; protein-losing enteropathy

World J Pediatr 2011;7(4):365-367

Delhi, India

Author Affiliations: Department of Pediatrics, LN Hospital, Delhi, India (Mehndiratta S); Department of Pediatrics, SDN Hospital, Delhi, India (Tyagi A); Department of Pediatrics, Hindu Rao Hospital Hospital, Delhi, India (Devgan V)

Corresponding Author: Sumit Mehndiratta, MBBS, DCH, DNB (Pediatrics), MNAMS, B-246 Yojna Vihar, Delhi, India (Email: drsmehndiratta@gmail.com)

doi: 10.1007/s12519-011-0256-x

Background: Ellis-van Creveld syndrome (EVC syndrome, MIM 225500) or chondroectodermal dysplasia is a rare, autosomal recessive disorder. This syndrome is characterized by a tetrad of chondrodystrophy, post axial polydactyly, and hidrotic ectodermal dysplasia, mostly involving teeth and nails and a high frequency of congenital cardiac anomalies, most frequently a common atrium. The genetic basis of this disorder has been identified as mutations in the Evc and Evc2 genes. We present a report of two affected siblings with features consistent with those of the syndrome.

Methods: A 2-month-old child with features of lower respiratory tract infection was admitted to the pediatric emergency department. Detailed examination revealed skeletal anomalies such as limb shortening and polydactyly in both hands. On cardiac evaluation, ventricular septal defect was found. There were no neonatal teeth. A diagnosis of EVC syndrome was made based on the findings.

Results: Screening of family members revealed that the elder sibling had features consistent with those of EVC syndrome. He was 4 years old, yet undiagnosed with short bones, polydactyly, partial anodontia and ventricular septal defect. The third child and the parents were unaffected. The treatment of this disorder is primarily supportive particularly for associated cardio-respiratory problems. The parents were extensively counseled for regular follow-up.

Conclusions: The diagnosis of this syndrome is based on clinical grounds supported by radiological evaluation. Prenatal diagnosis is possible by ultrasonography and genetic testing. Genetic counseling is required to make the parents aware of the risk of recurrences.

Key words: chondrodystrophy; chondroectodermal dysplasia; ectodermal dysplasia; polydactyly

World J Pediatr 2011;7(4):368-370

Author Affiliations: Division of Pediatric Endocrinology, Department of Pediatrics, B. J. Wadia Hospital for Children Parel, Mumbai 400012, India (Joshi R, Phatarpekar A)

Corresponding Author: Rajesh Joshi, D/3, Om Parshvanath Apartments, Saibaba Nagar, Borivali (West), Mumbai-400 092, India (Tel: +91-022-66916732; Email: rrj23@rediffmail.com)

doi: 10.1007/s12519-011-0254-z

Background: Neonatal diabetes mellitus (NDM) due to KCNJ11 gene mutation presents with diabetes in the first 3 months of life and sometimes with neurological features like developmental delay, muscle weakness and epilepsy.

Methods: A 5-week-old boy presented with diabetic ketoacidosis. Molecular genetic analysis of the patient revealed heterozygous missense mutation, L233F in the KCNJ11 gene, while his mother was mosaic for the same mutation.

Results: The treatment strategy was changed from insulin injections to oral glibenclamide and with a better glycemic control.

Conclusion: The patient with NDM due to mutation L233F (not reported till date) in the KCNJ11 gene can be successfully treated with oral glibenclamide therapy.

Key words: insulin; KCNJ11; neonatal diabetes mellitus   

World J Pediatr 2011;7(4):371-372