Iranian Journal of Pediatrics
Year:2013 | Volume:23 | Issue:6|Papers Count:29

Objective: To systematically evaluate therapeutic success of the ketogenic diet (KD) as a treatment option for epilepsy.Methods: Using MEDLINE and Google Scholar search, we searched for studies investigating the therapeutic success of ketogenic diet for epilepsy. We estimated therapeutic success rate for ketogenic diet as a treatment option for epilepsy and its 95% CIs using generic inverse variance method.Findings: A total of 38 studies met the inclusion criteria. In retrospective studies, the weighted success rate of the patients who take the KD as a treatment option for epilepsy was 58.4% (95% confidence interval (95%CI)=48.7%-69.9%) at 3 months (n=336), 42.8% (95%CI=36.3%-50.3%) at 6 months (n=492), and 30.1% (95%CI=24.3%-37.2%) at 12 months (n=387), in prospective studies, weighted success rate was 53.9% (95%CI 45.5%-63.8%) at 3 months (n=474), 53.2% (95%CI=44.0%-64.2%) at 6 months (n=321), and 55.0% (95%CI=45.9%-65.9%) at 12 months (n=347).Conclusion: This meta-analysis provides formal statistical support for the efficacy of the ketogenic diet in the treatment of epileptic patients.

Obesity in children and adolescents is a hot issue throughout the world. Numerous complications are related to childhood obesity, such as cardiovascular disease, diabetes, insulin resistance and psychological problems. Therefore, identification and treatment of this problem have an important role in the health system. In this clinical approach, we have provided a general overview of the assessment and management of obesity in children and adolescents, including definitions, history-taking, physical examinations, and laboratory testing for general practitioners and pediatricians. Furthermore, conventional therapies (physical activity, eating habits and behavioral modification) and non-conventional treatments (drugs and surgery options) have been discussed.

Objective: A high incidence of snake bite envenomation has been reported from rural India. Due to inadequate epidemiological data, the incidence is underestimated. This study analyses the pattern of snake bite and their management in children in rural areas of Maharashtra, India. To determine the age, mode of presentation, seasonal variation, clinical profile and outcome of patients with snake bite less than 15 years of age.Methods: This study is a retrospective, descriptive study including 162 patients, who presented with history of snake bite. Clinical data about age, sex, clinical manifestations, complications and outcome were obtained from case records and were analyzed.Findings: Out of the 162 patients 98 (60.49%) were males. The bites were vasculotoxic in 147 (90.74%) and neuroparalytic in 15 (9.25%) patients. Mainly bites occurred from July to September with 84 (51.85%) bites.Bites were more common in males in age more than 5 years (89%) with bite marks mainly on lower limbs in 120 (74.04%) patients. Deaths were reported in patients who reported late to the hospital with a mortality rate of 1.85%.Conclusion: Snake bite is a life threatening emergency. The key to minimizing mortality and severe morbidity is aggressive management of the ABC's of resuscitation, and timely and judicious administration of adequate dose of anti-venom.

Objective: Shiga toxin-producing E. coli (STEC) is a pathogenic E. coli that may cause hemolytic uremic syndrome (HUS) after diarrheal disease through Shiga toxins. Management of the patients with STEC infection is different from that of other diarrheal diseases due to increase in frequency of HUS after antibiotic administration. Few studies were conducted in Iran and epidemiology of STEC remains obscure, this necessitates examination of stools especially in young children for this bacterium.Methods: We determined the frequency of STEC in 947 E. coli strains isolated from diarrheal stools of children less than 14 years in Tehran with conventional culture methods and multiplex-PCR via determining the STX1 and STX2 genes, between October 2008 and September 2009. We also evaluated the association between stool exam findings and presence of STEC.Findings: Twenty seven (2.8%) of E. coli isolates were positive for STX1 or STX2 genes, most of which occurred in spring (P<0.05). There was no significant association between STEC positivity and stool exam findings. Eighteen out of 27 (66.7%) Shiga toxin positive samples were isolated from males and the rest from females. The most common STX -positive diarrheal samples showed loose consistency (P<0.017).Conclusion: Although the low frequency of STEC in our population indicates that it is not a major problem in our population, STEC should be regarded as an important infection because of its severe consequences. Further studies with greater sample size are needed to confirm our results.

Objective: Ceftriaxone is a third-generation cephalosporin which is widely used for treatment of infection in children accompanied by complications like urinary tract lithiasis and gallbladder psudolithiasis or sludge. The aim of this study was to investigate the incidence and predisposing factors that contribute to these complications in children.Methods: This quasi-experimental and before- and after-study was conducted in 96 children who were hospitalized for treatment of different bacterial infections and received 50-100 mg/kg/day ceftriaxone divided into two equal doses intravenously under conditions of adequate hydration. Sonographic examinations of urinary tract and gallbladder were carried out before and after treatment for that purpose. Patients with positive sonographic findings after treatment were followed with serial sonographic examinations.Findings: Post-treatment sonography demonstrated nephrolithiasis in 6 (6.3%) and gallbladder stone in one (1%), all were asymptomatic. Comparison of the groups with and without nephrolithiasis demonstrated no significant differences with respect to age, body weight, diagnosis, season of hospitalization, dosage of drug and the duration of treatment. Nephrolithiasis had a significant relation with male gender (P=0.02).Conclusion: Our results showed that pediatric patients may develop small sized, asymptomatic renal stones during a 2-6 day course of normal or moderate dose of ceftriaxone therapy. Close monitoring of ceftriaxone treated patients especially on high dose long term therapy for nephrolithiasis and gallbladder psudolithiasis or sludge is recommended.

Objective: Temporary vesicostomy is a urinary diversion procedure for patients with upper urinary tract (UUT) dilatation, secondary to bladder outlet obstruction or dysfunction. The aim of this study was to evaluate our experience in children undergoing such diversion, analyzing its efficacy to prevent urinary tract infection (UTI), improve or resolve hydronephrosis, stabilize or improve kidney function and restore the health of UUT.Methods: In this retrospective study, patients who had vesicostomy by Blocksom technique due to bladder outlet obstruction or dysfunction were evaluated in Mofid Children's Hospital (in Tehran) from March 2007 to March 2012. The reason for applying this procedure was failure in clinical treatment. Data regarding gender, age, diagnosis, time of any surgical intervention, associated anomalies, primary/secondary complications and mortality were collected using a questionnaire, and evaluated by giving a grade that ranged from 0 (worst) to 10 (best) based on Lickert's scale.Findings: From a total number of 53 patients, (88.7% male and 11.3% female) with a mean age of 225 days, 66% had posterior urethral valve and 16 (30%) neurogenic bladder. UTI was present in all cases, hydronephrosis in 52 (98.1%), and vesico-ureteral reflux only in 45 (84.9%) patients. Valve ablation was performed in 17 cases, and clean intermittent catheterization in14 patients which were unsuccessful. We performed vesicostomy in all patients. Mortality rate was 7.5%. Vesicostomy was closed in 35 patients. Cure rate was 85% in UTI, 82.7% in hydronephrosis, 80% in VUR, and 86.5% in kidney function.Conclusion: Vesicostomy is a simple procedure that protects upper urinary tract, decreases hydronephrosis, and improves kidney function. The procedure is well tolerated and reversible, with less complication and should be considered in children in whom conservative and medical treatment has failed.

Objective: Approximately one-third of all children with epilepsy do not achieve complete seizure improvement. This study evaluated the efficacy of Vigabatrin in children with intractable epilepsy.Methods: From November 2011 to October 2012, 73 children with refractory epilepsy (failure of seizure control with the use of two or more anticonvulsant drugs) who were referred to the Children's Medical Center and Mofid Children's Hospital were included in the study. The patients were treated with Vigabatrin in addition to their previous medication, and followed-up after three to four weeks to determine the daily frequency, severity, and duration of seizures in addition to any reported side effects.Findings: Of the 67 children, 41 (61.2%) were males and 26 (38.8%) females, their age ranging from three months to 13 years with an average of 3.1 [standard deviation (SD), 2.6] years. The mean daily frequency of seizures at baseline was 6.61 (SD, 5.9) seizures per day. Vigabatrin reduced the seizure frequency ≤2.9 (SD, 5.2) (56% decline) and 3.0 (SD, 5.3) (54.5% decline) per day after three and six months of treatment, respectively. A significant difference was observed between seizure frequencies at three (P<0.001) and six months (P<0.001) after Vigabatrin initiation compared with the baseline. Somnolence [3 (4.5%)], horse laugh [1 (1.5%)], urinary stones [1 (1.5%)], increased appetite [1 (1.5%)], and abnormal electroretinographic pattern [3 (4.5%)] were the most common side effects in our patients.Conclusion: This study confirms the short-term efficacy and safety of Vigabatrin in children with refractory epilepsies.

Objective: Placental hormones such as resistin, adiponectin, ghrelin and leptin are known to have considerable role in fetal growth and there are some articles published in this area recently. Nevertheless there is a shortage of data showing association between resistin level and fetal growth, that was why we decided to conduct a study to evaluate this association.Methods: This study was approved by ethic committee of pediatric health research center and research vice chancellor of Tabriz University of Medical Sciences. In this case-control study we measured the insulin, glucose and resistin in the cord blood of neonates with gestational age of 37 weeks or more in Al Zahra tertiary hospital from March 2011 to March 2012. Thirty-nine appropriate for gestation age (AGA) neonates and 41 small for gestation age (SGA) neonates were studied.Findings: The umbilical cord blood resistin level was not found to have significant correlation with the type of delivery [normal vaginal delivery (NVD) or cesarean-section (C-S)], neonate's gender, maternal age or body mass index (BMI). There was no significant difference in the levels of Insulin and glucose between AGA and SGA groups. Resistin level of blood cord in AGA group was 613.76±180.10 (range: 132.6-983.80 ng/ml) and in SGA group it was 1303.47±537.07 (range: 800.9-3001 ng/ml) (P<0.001). Neonates’ weight in AGA group was 3162.82±407.92 g and in SGA group it was 2425.85±32.84 g (P<0.001).Conclusion: In this study resistin level had reverse correlation with fetal weight in term neonates. The SGA neonates with growth insufficiency have higher resistin levels in their cord blood than AGA neonates with same gestational age. It is recommended to conduct more studies in future with larger population of patients to clarify the resistin role in neonatal birth weight.

Objective: In this study we aimed to determine the prevalence of cardiac malformations in fetuses of Iranian diabetic mothers with pre-gestational and gestational diabetes mellitus (GDM) and to find the patterns of different cardiac malformations.Methods: One-hundred and seventy diabetic pregnant women (68 preGDM and 102 GDM) (mean age: 32.17±4.8 years) and 85 healthy controls (mean age: 31.35±4.55 years) were recruited from September 2008 to July 2012. Fetal echocardiography was performed to assess cardiac malformation. In order to study major factors that may affect the results, a complete history was obtained.Findings: Fetal echocardiography was performed at mean gestational age of 24.7±5.4 and 20.27±3.9 weeks in diabetic patients and control group, respectively. Fifteen (8.8%) fetuses of diabetic mothers were detected to have cardiac malformations compared with 1 (1.17%) fetus in control group (OR: 8.13, 95%CI: 1.1-62.61, P -value=0.02). Hypertrophic cardiomyopathy noted as the most common cardiac malformation occurred in 6 out of 15 (40%) fetuses, and was found significantly more common in pre-GDM compared to GDM group (7.4% vs 1%, P -value=0.04). Despite the higher incidence of cardiac malformation in pre-GDM compared to GDM group, the difference was not significant. Further, no significant association was observed between the variables including, parity, diabetic regimen, parents’ consanguinity, maternal history of hypertension or hypothyroidism and occurring cardiac malformations (P -value>0.05).Conclusion: In this study we detected cardiac malformations in 8.8% of our diabetic referrals. The result of the present study shows that screening diabetic mothers for fetal cardiac malformations could be beneficial.

Objective: Patients with cystic fibrosis (CF) usually have abnormal intestinal microbiota and dysregulated immune mediators due to massive exposure to antibiotics. Probiotics as immunomodulatory and antiinflammatory substances are considered to improve both clinical and biochemical intestinal and pulmonary function in CF patients. We decided to investigate the effects of probiotics on quality of life and pulmonary exacerbations in children with cystic fibrosis.Methods: In a prospective, randomized, controlled clinical trial, 37 CF patients (2-12 years old) were randomly divided into two groups.20 patients of probiotic group took probiotics (2×109CFU/d) for one month while 17 patients of control group took placebo capsules. Quality of life was determined using PedsQLTM4.0 questionnaire at the beginning, then three and six months after completing the treatment period. Rate of pulmonary exacerbation in probiotic group patients was also evaluated during three months after intervention and compared to the same three months of the previous year. Results were analyzed using SPSS (11.5). P<0.05 was considered statistically significant.Findings: Significant improvement was observed in the mean total score of parent reported quality of life among probiotic group patients in comparison with placebo group at 3rd month (P=0.01), but this was not significant at 6th month of probiotic treatment. Rate of pulmonary exacerbation was significantly reduced among probiotic group (P<0.01).Conclusion: Probiotics are considered as useful nutritional supplements on reducing number of pulmonary exacerbations and improving quality of life in patients with cystic fibrosis. Effects of probiotics seem to be temporary and probably continuous ingestion might have more stable improving effects on quality of life.

Objective: Timely identification and prompt resuscitation of newborns in the delivery room may cause a decline in neonatal morbidity and mortality. We try to identify risk factors in mother and fetus that result in birth of newborns needing resuscitation at birth.Methods: Case notes of all deliveries and neonates born from April 2010 to March 2011 in Mahdieh Medical Center (Tehran, Iran), a Level III Neonatal Intensive Care Unit, were reviewed, relevant maternal, fetal and perinatal data was extracted and analyzed.Findings: During the study period, 4692 neonates were delivered, 4522 (97.7%) did not require respiratory assistance. One-hundred seven (2.3%) newborns needed resuscitation with bag and mask ventilation in the delivery unit, of whom 77 (1.6%) babies responded to bag and mask ventilation while 30 (0.65%) neonates needed endotracheal intubation and 15 (0.3%) were given chest compressions. Epinephrine/volume expander was administered to 10 (0.2%) newborns. In 17 patients resuscitation was continued for>10 mins. There was a positive correlation between the need for resuscitation and following risk factors: low birth weight, preterm labor, chorioamnionitis, pre-eclampsia, prolonged rupture of membranes, abruptio placentae, prolonged labor, meconium staining of amniotic fluid, multiple pregnancy and fetal distress. On multiple regression, low birth weight, meconium stained liquor and chorioamnionitis revealed as independent risk factors that made endotracheal intubation necessary.Conclusion: Accurate identification of risk factors and anticipation at the birth of a high-risk neonate would result in adequate preparation and prompt resuscitation of neonates who need some level of intervention and thus, reducing neonatal morbidity and mortality.

Objective: ETV6/RUNX1 (also known as TEL/AML1) is the most frequent gene fusion in childhood acute lymphoblastic leukemia (ALL). Sixty-three patients were enrolled in this study to explore the distribution of this gene in Iranian population.Methods: This study used 63 peripheral blood and bone marrow (PB/BM) samples from children with ALL.Immunophenotyping of PB and BM samples were performed using flow cytometry to illustrate the lineage. Moreover, reverse transcriptase polymerase chain reaction (RT-PCR) technique was used to amplify transcripts of leukemia-specific chromosome fusion gene ETV6/RUNX1 and to monitor the expression levels of the ETV6/RUNX1 in patients according to Van Dongen et al protocol.Findings: On the basis of French-American-British (FAB) classification, 47 individuals had ALL-L1. The incidence of ETV6/RUNX1 fusion gene in this study was 34.9%. The laboratory and clinical features of twenty two ETV6/RUNX1 positive ALL cases were similar to those of other studies. The most positive cases of ETV6/RUNX1 fusion gene had the early pre B ALL and pre B ALL immunophenotypes.Conclusion: The ETV6/RUNX1 fusion gene is a common genetic anomaly in Iranian childhood ALL patients and the prevalence of the ETV6/RUNX1 fusion gene is similar to that of ALL patients in other countries. However early pre B cells were the most common type in studied patients.

Objective: Breath-holding spells are known as benign attacks, frequencies of which decrease by the development of the autonomic nervous system. The present study aims to compare the electrocardiographic repolarization in children with breath-holding spells.Methods: In this study, QT dispersion, QTc dispersion, T peak to T end dispersion, and P wave dispersion of the twelve-lead surface electrocardiography of fifty children who had breath-holding spells were measured and compared with normal children from April 2011 to August 2012.Findings: Forty-four (88%) patients had cyanotic spells, while 6 (12%) had pallid spells. QTc dispersion was increased in the patients with breath-holding spells (148.2±33.1) compared to the healthy children (132±27.3) and the difference was statically significant (P=0.01). Meanwhile, no statistically significant differences were observed between the patients and the control subjects regarding the other parameters (P>0.05).Conclusion: QTc dispersion was significantly increased in the patients with breath-holding spells compared to normal children and this is a sign of cardiac repolarization abnormality as well as the increased risk of cardiac arrhythmia in patients with breath-holding spells.

Objective: To determine the risk factors associated with lower respiratory tract infections (LRTI) related hospitalizations in preterm infants receiving palivizumab throughout the high season for respiratory syncytial virus (RSV) infection.Methods: Premature infants who were commenced on palivizumab prophylaxis during the RSV season were included in the study following parental consent. Information on demographic, social, prenatal and postnatal clinical characteristics was recorded and risk factors associated with hospitalization were evaluated for each patient.Findings: While 234 participants (Group 1, 92.8%) did not require hospitalization during the study period, 18 patients (Group 2, 7.2%) were hospitalized at least once for LRTI during the RSV season. The rate of moderate-severe bronchopulmonary dysplasia (BPD) was significantly higher in group 2 compared to group 1 (38.9% vs 16.2%, P=0.016). Of the 18 infants who were hospitalized, 6 (33.3%) tested positive for RSV while the remaining 12 patients (66.7%) were negative for RSV. Odds ratio (OR) analysis of several risk factors revealed the presence of BPD (OR: 3.28, 95%CI: 1.19-9), being from a family with low socioeconomic status (OR: 3.64, 95%CI 1.08-12.3) to be associated with a higher likelihood of LRTI-related hospitalization.Conclusion: Our data demonstrated that RSV is an important LRTI agent and cause of hospitalization especially in preterm infants with additional risks such as BPD, gestational age of<28 weeks and low socioeconomic status. We suggest that improving care conditions and decreased BPD with prematurity would help in prevention of LRTI hospitalization.

Objective: This study was conducted to determine the prevalence of low birth weight (LBW) and its related risk factors in an appropriate sample of neonates in Shahroud, northeast Iran.Methods: At this study, a random sample of 1000 neonates were selected of which 72 neonates were LBWs. We used univariate and multivariate logistic regression methods to evaluate the LBW risk factors in LBWs compared to normal weight infants.Findings: 7.2% of neonates were LBWs and 6.1% born before 37 weeks of gestation. Prematurity, high-risk pregnancy and maternal age have significant statistical association with LBW. Odds Ratio (OR) for prematurity was 42.82 (95%CI, 21.93-83.57), for high risk pregnancy 2.76 (95%CI, 1.47-5.19) and for maternal age group more than 35 years in comparison to 19-35 years age group 0.2 (95%CI, 0.05-0.71).Conclusion: Based on this study, prematurity and high risk pregnancy were the most important risk factors for LBW. There was also a reverse association between maternal age and LBW.

Hyper Immunoglobulin M (hyper IgM) is a rare immunodeficiency disorder first reported in 1961 [1], characterized by recurrent infections with low levels of serum IgA, IgG and IgE and normal to high levels of serum IgM [1]. Onset of manifestation of X-linked HIGM occurs in the 1st or 2nd year of life with recurrent pyogenic infections especially of upper and lower respiratory tract and diarrhea in 40% of the patients [1]. Cryptosporidium infections are a common cause of diarrhea in these patients complicated with hepatobiliary disease, which is severe and often fatal [1]. Gallbladder hydrops occurs in acutely severe ill children with inflammatory or non inflammatory conditions [2].

Uncovering of harmless primary vesicoureteral reflux (VUR) results in follow up of just a VUR image causing unnecessary stress for the parents, patients and physicians. Furthermore, voiding cystourethrography, gold standard for VUR diagnosis, is an invasive method having radiation hazard.Following questions will remain unanswered until non-invasive diagnostic methods for VUR are discovered: Is VUR a physiological phenomenon that disappears during growth in children? Which VUR is benign or harmful for the kidney [1] ? Is the degree of VUR a sufficient parameter to state that it is harmless.It is necessary to look over the results of some studies for making comments on the questions above. The prevalence of VUR in healthy children was reported as 0.4-1.8% based on statistical calculations. However, this ratio increased up to 17.2% based on the assessments in healthy renal units [2]. Spontaneous resolution rate of VUR is decreased as its grade increases. Today, we need a non-invasive gold standard method for diagnosis of VUR in both healthy and ill children [3, 4].The last recommendations of American Academy of Pediatrics was based on a formal meta-analysis of recent studies that did not detect a statistically significant benefit of prophylaxis in preventing recurrence of febrile UTI in infants without reflux or those with grades I to IV VUR [5].A previous study showed that antimicrobial prophylaxis did not reduce the risk of recurrent UTI, but rather led to infections with resistant microorganisms [6]. In conclusion, unrevealing of harmless primary VUR in children should also be considered as success.

Tinea capitis (TC) is the dermatophytosis widespread in the world [1, 2]. Not only specter of causative species in a specific geographical area is changing in time, but also the distribution of each one worldwide. No data exists on TC for recent 25 years in our country. Following goals were set for the study: to determine the specter of TC causatives and importance of age and sex in disease development, to identify source of the disease.We have conducted a prospective study. During the last three years 13901 dermatological patients were examined. Among them 118 patients with TC were identified and studied. Skin scrapings and hair samples were examined by microscope. Fungal cultures were grown on Sabouraud's Dexstrose Agar with chloramphenicol. Exposure to the light of Wood's lamp was used in all patients.The data analyses were evaluated by SPSS system. Likelihood Ratio Chi square test and Fisher’s exact test were used. P<0.01 was considered statistically significant.From 118 patients 92 (78%) were males and 26 (22%) females. Age of the patients varied from 12 months to 14 years, distributed into 3 age groups: 28 (23, 7%) patients were in 12 months to 4 years age group, 58 (49, 2%) patients were from 5 to 9 years old, and 32 (27, 1%) from 10 to 14 years.Microscopic examination revealed ectotrix-type causative agent in 107 (90, 7%) cases, and endotrix type in 11 (9, 3%) cases. The results of culturing were positive in 81 (68, 6%) cases. Zoophilic causative agent was in 69 (85, 2%) cases, and anthropophilic causative agent in 12 (14, 8%) patients. Among those Trichophyton violaceum was diagnosed in 7 (8, 6%) cases, Trichophyton mentagrophytesin 33 (40, 7%), Trichophyton verrucosumin 5 (6, 2%), Microsporum canis in 31 (38, 3%), and Microsporum ferrugineum in 5 (6, 2%). Trichophyton mentagrophytes and Microsporum canis were significantly more frequent than all the other causatives. Green light under Wood's lamp was observed in 36 cases. In all cases of Trichophyton mentagrophytes and Trichophyton verrucosum the disease was caused by cattle. In cases caused by Microsporum canis the source was a cat. In case of infections caused by Microsporum ferrugineum and Trichophyton violaceum the source was impossible to identify.Statistical processing of the resultant data of our study disclosed the association of age with spread of the disease. It turned out, that the highest prevalence of the disease is observed in children from 5 to 9, also the disease was prevalent primarily in males (P<0.01). We also discovered changes in causatives’ species. Trichophyton violaceum was predominant causative agent among Trichophytons in the 80ies, less prevalent wasTrichophyton mentagrophytes. According to the results of our study, Trichophyton mentagrophytes turned out to be the predominant causative. The rise of Microsporum canis frequency in Georgia confirms its causative importance worldwide [3-5]. Dominant in the 80ies, Trichophyton violaceum and Microsporum ferrugineum were found in single cases only. One of the important results of the study was identification of Trichophyton verrucosum among causative agents - never being observed before in the previous studies in our country. Eventually changing of TC causative species was reflected in dramatic decrease of anthropophilic species and significant increase of zoophilic species.finally, in our study we have identified signs characteristic for TC: excess of zoophilic forms compared to anthropophilic ones, dominance of Trichophyton mentagrophytes and Microsporum canis in the specter of causatives, spread of the disease primarily in children, with highest prevalence in the 5-9 age group and male gender.

Rubinstein–Taybi syndrome (RTS) was first described by Michail et al [1] and subsequently by Rubinstein and Taybi [2]. We present a typical sixmonth-old girl with RTS. Her mother had ovarian cancer and polyhydramnios during the pregnancy. Parents are closely related. There were frequent respiratory infections resulting in two hospital admissions. Physical examination revealed typical facial changes including downward-sloping palpebral fissures, prominent forehead, hypertelorism, limited mouth opening, large beaked nose, and high arched palate (Fig.1). A history of increased tearing was compatible with nasolacrimal duct obstruction. Other features include general hypotonia with delayed developmental milestones, short and broad thumbs and toes (Fig.1). Chest x-ray showed cardiomegaly (Fig.2). She had normal karyotype.

Hyperbilirubinemia is a common and in most cases benign clinical condition in neonates. Phototherapy is used for management of neonatal hyperbilirubinemia [1]. Phototherapy may lead to complications including skin rash, diarrhea, hyperthermia, chills, dehydration, DNA damage to lymphocytes, retinal degeneration, bronze baby syndrome especially in cholestatic jaundice and PDA opening in LBWs and Hypocalcemia [2].

Hypothyroidism is one of the most common endocrine disorders, and many levothyroxine prescriptions are written to replace the hormone deficit. Herewith we report a thyroid hormone overdose in a 6 year old boy.6 year old Beckwith-Wiedeman syndrome boy with developmental delay presented with accidental ingestion of 2.5 mg of levothyroxine 6 hours prior to admission. He has been receiving levothyroxine for hypothyroidism since neonatal period and currently (weight=17 kgs) he was on 100 mg of levothyroxine once a day. There were no tremors, irritability, convulsions or diarrhea. On examination his temperature was 98.6o, pulse rate 100/min, RR 20/min and BP was 100/60 mm Hg. Other systems examination was unremarkable. His Thyroid profile is shown in Table 1. Gastric lavage and gastrointestinal decontamination was done. His complete blood count, blood sugar, blood urea, serum creatinine, Aspartate transaminase (SGOT), lactate dehydrogenase (LDH), creatine kinase MB (CK-MB) and electrocardiography were within normal limits. He was monitored for overdose features. After 24 hours he was tachycardic (PR 120/min), febrile (99.60F) with blood pressure 112/80mm Hg (>95th centile) along with sweating of palms and soles. In view of tachycardia, sweating and hypertension, Propranolol and Dexamethasone was started. After 76 hours features of thyroid toxicity subsided and drugs were tapered. Child was restarted on thyroxine and discharged on 8thday. Child was followed up at 3 and 6 months, there was no feature of hypothyroidism.Levothyroxine overdose in children typically follows a benign course [1]. Children may be asymptomatic or have clinical features like fever, tachycardia, hypertension, tremor, insomnia, irritability and convulsions [1-3].

Neonatal congenital diaphragmatic hernia (CDH) is a complex anomaly, the clinical course of which and the amount of viscera herniated. Acute gastrointestinal complications of neonatal CDH are mainly caused by the incarceration of the stomach and intestines [1], which are significantly reduced by prenatal diagnosis and appropriate neonatal treatment. Neonatal gastric perforation associated with CDH is an unusual surgical emergency, the symptoms of which may be quite nonspecific [1], and the radiographic findings may show pleural effusion and massive hydroperitoneum [2, 3]. Rapid diagnosis and appropriate treatment are essential. We present a 4-day-old neonate with gastric perforation and CDH, who was treated successfully.

I read with interest Rabbani et al's paper entitled “Dental problems in hypophosphatemic rickets, a cross sectional study” in the fourth issue of Iran J Pediatr 2012[1]. Clinical oral manifestations of hypophosphatemic rickets often include premature tooth exfoliation, hypoplastic enamel and dental infections. Apical rarefaction, rickety bone trabeculation and absent or abnormal lamina dura are frequent radiographic findings [2].

Sandifer's Syndrome (SS) is a rare pediatric manifestation of gastro-esophageal reflux (GER) disease characterized by abnormal and dystonic movements of the head, neck, eyes and trunk. Although Sandifer initially observed the association, Kinsbourne and Oxon first reported it based on the observations of Sandifer [1]. The syndrome is most certainly underrecognized, and delays in diagnosis are due to atypical presentations or cases in which the diagnosis is not part of the differential [2, 3].

Although the premature tooth exfoliation (PTE) can be associated with some systemic diseases such as changes in immune system or connective tissue disorders, we didn’t find any report on this disorder and hypophosphatemic rickets. In bone metabolic disorders, there are a few reports on hypophosphatasia and PTE [1-3]. Although, some factors such as severe oral infection can predispose PTE in HR patients, we recommend a study on prevalence of PTE and its related factors in HR patients in future.The inflammation of the gingiva was evaluated by Gingival Index of Loe and Silness index [4]. It was missed to mention in our article. As we mentioned, dental examination was performed by the same dentist in all patients [5].We agree with Ramezani about the prevalence of taurodontism as a common dental problem with wide range of prevalence in normal population (up to 8% [6]), but as we reported in our paper the prevalence of this disorder was 15% that is very more common than in normal population [5]. It was reported in 0.3% of normal children [7].Finally, for diagnosis of taurodontism, as mentioned in our report, orthopantomogram was performed in all taurodontism suspicious patients [5].

Dear Editor: We read with interest the Iranikhah et al's article entitled “Stool Antigen Tests for the Detection of Helicobacter Pylori in Children” in the second issue of 2013 ofIran J Pediatr [1].The authors have well reported an interesting investigation about the detection of bacterial antigens in stool for diagnosis of Helicobacter pylori in children. Also they appropriately used endoscopy and biopsy as a gold standard test for validation of the H. pylori infection and statistical indices are calculated accordingly. In the study, sensitivity, specificity, positive and negative likelihood ratios for H. pylori stool antigen was reported to be 85%, 93%, 89.7%, and 90% respectively, while 89.7%, and 90% are not likelihood ratios, they are positive and negative predictive values.

Extremely low birth weight (ELBW) is defined as a birth weight less than 1000g. Acute kidney failure (AKF) is registered in 6%-24% of critically ill neonates treated at the intensive care units (ICU). The primary causes of AKF in these neonates are prerenal mechanisms (around 85%), which include hypovolemia, hypotension and hypoxemia. A medication therapy is applied first and in case it does not yield the desired results, a renal replacement therapy is applied [1, 2].

The prevalence of obesity and overweight in adolescents have been growing in recent decades worldwide [1]. Obesity in children and adolescents has been proposed as a major health problem in developed countries and in developing countries [2, 3].

Myelomeningocele (MMC) is a malformation commonly associated with diverse cerebral abnormalities including Chiari II, hydrocephalus, corpus callosum agenesis, and absence of septum pellucidum [1]. Gross choroid plexus anomalies like cyst and bifida have been reported before [2-4] but to our best knowledge, interchoroid plexus adhesion has never been reported so far.