Iranian Journal of Pediatrics
Volume:23 Issue: 1, Feb 2013

The paper aimed to study the prevalence of depression in children and adolescents with epilepsy by reviewing the existing literature, looking for any association between depression in these children and their demographic or seizure related factors to highlight the potential risk factors for depression in epileptic children. A search of literature was carried out to study original English language articles published during the last 15 years, focusing on only epileptic children and adolescents, studying of depression as comorbidity of epilepsy, and describing demographic and epilepsy-related factors associated with depression. The articles included in this study have reported data on 1095 epileptic children aged 4-19 years old and showed that the prevalence of depression has continued to be very common in epileptic children and adolescents, ranged from 5.2% to 39.6%. On the whole, the findings did not support the presence of an association between depression and demographic or seizure variables in children. Pediatricians and other physicians working with epileptic children should have a high index of suspicion for depression as a comorbid condition in children with epilepsy. Early diagnosis and more comprehensive packages of care for depression in children with epilepsy will enable them to have a better quality of life.

Delayed passage of stool is a result of both gestational immaturity and illness severity. Small for gestational age (SGA) preterm infants are at high risk of gastrointestinal (GI) complications. We aimed to analyse the effects of a strict nutrition and stool protocol on GI problems in SGA compared to appropriate for gestational age (AGA) preterm infants Retrospective cohort analysis including all preterm infants with delayed meconium passage hospitalized at the Neonatal Intensive Care Unit of the Medical University of Graz, Austria. Infants were identified by a local data system and by the use of a strict feeding and stool protocol between 2001 and 2010. Main outcome parameters included neonatal morbidity, surgical intervention and mortality. Twenty-six SGA (median GA 28.6 weeks, birth weight 825 grams, 46% male) were compared to 101 AGA (median GA 28.4 weeks, birth weight 1168 grams, 55% male) preterm infants. Clinical signs of delayed meconium passage did not differ significantly between groups. Differences regarding percentage of necrotizing enterocolitis, ileus, spontaneous intestinal perforation, and surgical intervention did not differ between groups. Mortality rate was significantly higher in SGA (11.5%) compared to AGA (2.9%) infants (p=0.034). Despite similar morbidity SGA infants exhibited higher lethal complication rates following delayed meconium passage compared to AGA infants.

In developing countries, Helicobacter pylori infection is mainly acquired during childhood and may be a predisposing factor for peptic ulcer or gastric cancer later in life. Non­invasive diagnostic tools are particularly useful in children for screening tests and epidemiological studies. We aimed to determine the prevalence of H. pylori infection among Kurdish children in Sanandaj, West Iran. We used a Helicobacter Pylori Stool Antigen (HpSA) test to detect H. pylori infection. A questionnaire was used to collect data about age, sex, duration of breastfeeding, and family size. A total of 458 children aged 4 months to 15 years were enrolled in this study. The mean age of enrolled children was 5.6±5.4 years. Stool samples were positive for H. pylori in 294 (64.2%) children. The prevalence of H. pylori infection increased with age (P<0.001). We found a significant increase in the infection rate as the family size grew (P=0.005). There was no correlation between a positive H. pylori status and gender (P=0.6) or the duration of breastfeeding (P=0.8). It seems that the prevalence of H. pylori infection is very high in children in Sanandaj. It begins at early infancy (before 4th month of age) and cumulatively increases with age.

Control of residual pulmonary hypertension after closure of left to right shunts in children is still a challenging issue. The purpose of this study was to compare the effect of two phosphodiesterase inhibitors in pediatric cardiac surgical patients. A total of 48 postoperative children were enrolled in the study between 2008 and 2010. Patients were stratified based upon choice of pulmonary vasodilator into three equal groups (n=16); Milrinone group received intravenous milrinone (0.75 µ/kg/min), Sildenafil group received oral sildenafil (0.3 mg/ kg every 3 hours) and the Combination group received both medications. Demographic variables and types of congenital anomalies were not different among the 3 groups. Patients in the Combination group had higher preoperative pulmonary artery to aortic (PA/AO) pressure ratios compared to other 2 groups (p=0.001). Postoperatively, patients in Milrinone group incurred lower systolic PA and PA/AO pressures compared to sildenafil group (p=0.014, 0.003), but it was the same in sildenafil and combination group (p=0.206. 0.330 respectively). Pulmonary hypertensive crisis was noted in 6 patients in Sildenafil group, and 3 patients in Combination group (P=0.02). Significant rise in PA pressure was noticed after discontinuation of drug in Milrinone group (p=0.001), which was not observed in the Combination group (p= 0.654). No mortality was noticed in any of the groups. Intravenous milrinone is more effective than oral sildenafil in control of postoperative pulmonary hypertension and elimination of pulmonary hypertensive crisis. Combination of two drugs reduces the risk of rebound pulmonary hypertension after discontinuation of milrinone.

Electroencephalography (EEG) is useful in seizure diagnosis. The purpose of this study was to compare efficacy and safety of oral chloral hydrate (CH) and promethazine (PZ) in sedation induction for EEG of children. In a parallel single-blinded randomized clinical trial, sixty 1-10 year old children referred to EEG Unit of Shahid Sadoughi Hospital from January 2010 to February 2011 in Yazd- Iran, were evaluated They were randomized to receive orally 70 mg/kg chloral hydrate or promethazine 1 mg/kg. The primary outcomes were efficacy in adequate sedation and successful recording of EEG. Secondary outcomes included clinical side effects, time from administration of the drug to adequate sedation, caregiver’s satisfaction on a likert scale (1-5), and total stay time in EEG un. Twenty four girls (40%) and 36 boys (60%) with mean age of 2.9 ± 1.9 years were evaluated. Adequate sedation (Ramsay sedation score of four) was obtained in 43.3 % of PZ and 100% of CH group (p=0.00001). Also in 70% of PZ and 96.7% of CH group, EEG was successfully recorded (p=0.006). So, CH was a more effective drug. In CH group, EEG was performed in shorter time after taking the drug (mean± SD: 32.82± 9.6 minutes vs. 52.14 ± 22.88 minutes, P= 0.0001) and the parents waited less in the EEG unit (mean± SD: 1.29± 0.54 hours vs. 2.6 ± 0.59 hours, P= 0.0001). They were also more satisfied (mean ± SD: 4.6 ± 0.6 scores vs. 3.1 ± 1.4 scores, P= 0.001). Mild side effects such as vomiting in 20% of CH (N=6) and agitation in 6.6% of PZ group (N=2) were seen. No significant difference was seen from viewpoint of side effects frequency between the two drugs. The results of the present study, showed that chloral hydrate can be considered as a safe and more effective drug in sedation induction for sleep EEG in children.

Immediate, short and midterm outcome of balloon pulmonary valvuloplasty are well known, but there is limited information on long term results. We report long term results of 2–13. 5 years follow up of balloon pulmonary valvuloplasty in children. From June 1998 to January 2012 sixty consecutive patients (33 females, 27 males) with moderate to severe valvar pulmonary stenosis (right ventricular to pulmonary artery pressure gradient greater than 50 mmHg) were considered for balloon valvuloplasty. The gradient was measured pre and immediately post–valvuloplasty at catheterization, and then by echocardiography at follow up. Follow up studies were performed 2–13.5 years (mean±SD; 7.1±2.5 years, median: 5.5 years) after procedure, by Doppler echocardiography in all patients and catheterization and angiography in two patients. Balloon pulmonary valvuloplasty BPV was successful in 53 of 60 (88.3%) patients whereas surgical valvotomy was necessary in 6 to 60 (10%). There was one immediate death due to perforation of the right ventricular outflow tract. Pulmonary valve systolic pressure gradient decreased from 83.3±32.1 to 19.3±14.2 mmHg immediately after BPV and to 12.3±6.6 mmHg at late follow up (P<0.001). Pulmonary insufficiency was noted in 20 (38%) patient at short–term, but it was demonstrated in 17 (32%) at late follow up. A second valvuloplasty was performed in two (3.8%) patients presenting with re-stenosis. The short, intermediate and long-term outcomes of pulmonary balloon valvuloplasty in children are excellent. Therefore it can be considered as the treatment of choice for children with pulmonary valve stenosis.

To determine the association between heart rate variability (HRV) and hypertension in Chinese children. The study was conducted in First Hospital of Jilin University, China. A total of 101 children were recruited in this study. They were divided into a high systolic blood pressure (SBP) group (HS group) and normal SBP group (NS group) according to the SBP levels. In the second set of experiments, the children were divided into a high diastolic blood pressure (DBP) group (HD group) and normal DBP group (ND group) according to the DBP levels. HRV measurements were performed, and the time domain and power spectrum values were calculated. The differences of low frequency (LF)/high frequency (HF) ratio, HF, and standard deviation of normal-to-normal RR intervals (SDNN) between daytime and nighttime were obviously abolished in HS and HD groups. The HS group displayed significantly lower values of HRV over a 24 h period compared to the NS group (SDNN, standard deviation of the averaged normal-to-normal RR intervals [SDANN], Triangle Index, root mean square successive difference [RMSSD], total power [TP], ultra-LF [ULF], and HF). Only the Triangle Index in the HD group was lower than that in ND group. We provide evidence that HRV is reduced and the circadian rhythm of HRV is weakened in hypertensive children, and hypothesize that a reduced HRV is a potential pathophysiological mechanism linking childhood hypertension and adulthood cardiovascular diseases.

Sleep problems are experienced by 25-30 percent of children and adolescents, regardless of age. The purpose of this study was to investigate if there is any relationship between gender or school entrance and sleep complaints. From June 2008 to May 2009 children aged 2 to 12 years were selected by clustered randomization of families. The Persian version of the BEARS questionnaire (Bedtime problems, Excessive sleepiness, Awakenings during the night, Regularity of sleep, Snoring) with five domains was filled out by general pediatricians. Prevalence of sleep complaints in each B-E-A-R-S category was calculated and compared for pre-school and school-age groups. BEARS questionnaire was completed for a total of 746 children (2-12 years old); 325 in pre-school-age group (2-6 years old) (142 females [43.7%] and 183 males [56.3%]) and 421 in primary school-age group (7-12 years old) with the average age of 3.93 (±0.16) years and 9.63 (±0.16) years respectively. The most common screening problem in both groups was excessive daytime sleepiness (64.9% and 62.9% respectively). Bedtime problems and also regularity and duration of sleep were significantly more prevalent in pre-school-age group (P<0.0002; odds ratio [OR] =1.98; 95% confidence interval [95%CI]: 1.98-4.20; and OR=2.00; 95%CI: 1.41- 2.84 respectively). The difference between mean sleep duration between pre-school age and school-age groups was statistically significant (P<0.0001). The current survey shows that different types of sleep problems are relatively high especially in the form of excessive daytime sleeping domain in preschool- and school-aged children. Bedtime problems and regularity problems were significantly more prevalent in pre-school-age group. School entrance seems to play a positive role for bedtime problems, and sleep-disordered breathing.

Leukemia is the most prevalent type of cancer in children. The aim of this study was to estimate the 5-year survival rates of Acute Lymphoblastic Leukemia (ALL) and Acute Myeloblastic Leukemia (AML) as well as factors influencing them. This is a nonrandomized retrospective study conducted on 280 patients with ALL and AML. They were all below 15 years old children admitted to Shahid Faghihi hospital, Shiraz, Iran from 2004 to 2008.The survival rates were estimated by applying the Kaplan-Meier method. In addition, the log rank test was used to estimate the statistical significance of differences in the survival probability. Cox regression model was applied to conduct multivariate analysis for adjusting confounding variable. All analyses were performed in SPSS statistical software (version 16). P-values less than 0.05 were considered as statistically significant. The mean (± standard deviation) of the observation period was 28.2±16.1 months. In this period, 60 (24.7%) patients (47 ALL and 15 AML) passed away. The cumulative rate of survival in this study was 53.3±0.1 percent. This probability was 56.6±0.1% and 44.2±0.1% for ALL and AML patients, respectively, which indicates no statistically significant difference between them (P=0.8). According to Cox model, there was a significant relationship among the variables of platelet count and relapse with the survival rate. Platelet count was identified as a positive prognostic factor of the survival rate in ALL patients. However, on the base of our results and other studies, incidence of relapse and the number of relapses are significant factors of survival rates of leukemia.

Nocturnal enuresis is a common psychosocial concern for both parents and children. In the present study we have determined the prevalence of nocturnal enuresis in Iranian children and associated personal and familial factors with this problem. A cross sectional epidemiological study for detection of nocturnal enuresis prevalence rate and evaluation of associated familial and personal factors in elementary school children [7-11 years old] from Urmia were investigated. The subjects were selected by cluster sampling method.chi square test and logistic regression were used in univariate and multivariate respectively. Of the 1600 questionnaires were distributed, 918 [57%] were completed and included in the final analysis and rest of them were excluded which weren’t filled by parents and also out of our study age period range. The gender of the subjects was equally distributed [48.6% males and 51.4% females] approximately. Prevalence of nocturnal enuresis was 18.7% [n=172] and prevalence of day time incontinence was 5.5% [n=51]. There was not significant gender difference between these two groups. Enuretics had crowded families, positive family history, low educational level of parents, not working of father, working of mother, single parent, fail school performance, positive history of urinary tract infection[UTI], not breast feeding, low birth weight to compare of non-enuretics. But unwilling pregnancy, neonatal icter, febrile convulsion, and mother smoking didn’t increase prevalence of this problem. 19.8% [n=31] of children with this problem had been seen by physician. Medication was the most preferred treatment option for enuresis [64.5%]. Our results with enuresis prevalence and associated factors were comparable to other epidemiological studies from various countries. We found out Iranian families do not pay sufficient attention to their enuretic children.

Paucity of interlobular bile ducts (PIBD), defined as absence or marked decrease in the number of interlobular bile ducts, is one of the causes of neonatal cholestasis. Treatment includes treating the intractable pruritus caused by persistent cholestasis. PIBD can be part of a familial syndrome of cholestasis named Alagille syndrome (AGS). We report clinical status of a case series of Iranian patients with PIBD. In this retrospective study, patients with cholestasis admitted to the pediatric gastroenterology ward in a referral hospital in Shiraz from January 2006 to January 2010 and underwent liver biopsy were evaluated. Clinical and paraclinical status of children with the pathologic diagnosis of PIBD was assessed. Disease was presented in all jaundiced patients with aged in average 3 days at presentation. Seven patients had the criteria of AGS. Despite medical treatment, cholestasis was not controlled in 6 (28.6%) patients. Liver transplantation led to the survival of 5 patients while the other patient who did not undergo liver transplantation died at 2 months of age. One patient underwent peritoneal dialysis due to renal insufficiency and died at 9 months of age. After 1-5 years of follow-up, the mortality rate was 9.5%. In patients with intractable cholestasis, only patients that underwent liver transplantation survived. Thus, the most important criterion for liver transplantation in neonatal PIBD is intractable cholestasis. This is the first report that shows AGS can result in neonatal-onset renal insufficiency.

To assess the national inequality of school readiness and autism among 6-year-old Iranian children before school entry using a national health assessment survey. In a cross-sectional nationwide survey, all Iranian children entering public and private elementary schools were asked to participate in a mandatory national screening program in Iran in 2009 in two levels of screening and diagnostic levels. The study population consisted of 955388 children (48.5% girls and 76.1% urban). Of the whole children, 3.1% of the 6-year-old children had impaired vision. In addition, 1.2, 1.8, 1.4, 7.6, 0.08, 10, 10.9, 56.7, 0.7, 0.8 and 0.6 percent had color blindness, hearing impaired, speech disorder, school readiness, autism, height to age retardation, body mass index extremes, decayed teeth, having disease with special needs, spinal disorders, and hypertension, respectively. The distribution of these disorders was unequally distributed across provinces. Our results confirmed that there is an inequality in distribution of school readiness and autism in 6-year-old children across Iranian provinces. The observed burden of these distributions among young children needs a comprehensive national policy with evidence-based province programs to identify the reason for different inequality among provinces.

Helicobacter pylori is recognized as a major etiological factor in the pathogenesis of gastritis and peptic ulcer disease. H. Pylori eradication has a failure rate of more than 30% in pediatric patients, particularly because of poor compliance, antibiotic resistance and occurrence of side-effects. This study was aimed to determine whether adding the probiotics to a standard anti-H. pylori regimen could minimize the gastrointestinal side-effect prevalence and improve the eradication rate. Double-blind randomized placebo controlled study conducted at Children’s Medical Center in Tehran, Iran. Sixty six H. pylori positive children were treated with a triple drug treatment protocol (omeprazole+amoxycillin+furazolidon) and randomly allocated to receive either probiotic or placebo. All patients underwent esophagogastroduodendoscopy. H. pylori infection was diagnosed by either rapid urease test (RUT) or histology. H. pylori status was assessed after 4-8 weeks of the completion of treatment with stool H. pylori antigen test. The side effects of the treatment were determined in each group. Mean age of patients was 9.09 (range 3‐14) years, 44 (65.7%) patients were boys (sex ratio 2:1). All 66 patients completed the course of treatment and follow-up. The rate of H. pylori eradication was significantly higher in probiotic group (P=0.04). In probiotic supplemented children there was a lower rate of nausea/vomiting (P=0.02) and diarrhea (P=0.039) during treatment. This study showed that probiotics have positive effect on the eradication of H. pylori infection. Adjuvant therapy with probiotic is recommended in order to reduce the frequency of antibiotic induced side-effects during treatment with antibiotics.

Most neonatal encephalopathic disorders appear to be caused by perinatal events. Persistent myocardial ischemia leads to cellular necrosis and release of troponin from cardiac muscles. Fetal distress during labor may be detected by monitoring the fetal heart rate. However little is known about the relationship, if any, that exists between fetal heart rate abnormalities and the fetal cardiac musculature and its function. The aim of this study was to investigate the relationship, if any, of umbilical cord serum levels of cardiac troponin T with fetal bradycardia or late deceleration. In this cross sectional study, troponin T level in umbilical cord blood of 80 neonates are measured. There were 23 versus 57 fetuses with and without late deceleration or bradycardia. Level of cardiac troponin T in umbilical blood of neonates with fetal bradycardia or late deceleration was elevated in comparison to neonates without bradycardia or late deceleration. There was no relation between umbilical troponin T level and mode of delivery. Infants with fetal bradycardia or late deceleration during labor had significantly higher cord cardiac troponin T levels. If troponin level is normal, the probability of hypoxia will be very low.

Barter’s syndrome is a heterogeneous disorder characterized by deficient renal reabsorption of sodium and chloride, and hypokalaemic metabolic alkalosis with hyper-reninaemia and hyperaldosteronaemia. Bartter syndrome type III (BS type III), due to mutations in the CLCNKB gene, is highly variable. The aim of our study was to describe the clinical presentation in a Chinese girl with BS type III and to explore mutations or SNPs of CLCNKB gene in her family. The clinic data of the patient was collected. Mutations or SNPs was invesitaged by sequencing of the exon of CLCNKB gene. The clinic analysis confirmed the diagnosis of BS type III. The coexistence of 13 reported SNPs and 11 novel SNPs of CLCNKB gen were found in the patient and her parent. a novel heterozygous C to G transition at nucleotide 2471 in exon 20 of CLCNKB gene harbored uniquely by the patient were revealed. a novel heterozygous C to G mutation at nucleotide 2471 of CLCNKB gene and some new SNPs were identified in a Chinese girl with BS type III having persistent hypokalemia. The novel mutation and SNPs make the genetic background of the patient more complicated.

Pulmonary alveolar proteinosis (PAP) is a rare disease in children, characterized by intra-alveolar accumulation of large amounts of surfactant proteins, which severely reduce gas exchange. Whole lung lavage (WLL) is the preferred technique for the treatment of severe PAP. This report presents nine pediatric cases with advanced PAP who underwent WLL under general anesthesia during a 9 year period. One patient was treated with multiple unilateral WLL without employing cardiopulmonary bypass (CPB) and eight cases were treated by simultaneous lavage of both lungs using partial CPB. Our experience suggested that partial CPB was useful to support oxygenation during WLL in small children with severe PAP in whom lung separation and selective lavaging of each lung were impracticable.

Nijmegen Breakage Syndrome(NBS) is a rare autosomal recessive disorder with specific clinical features, characteristic chromosomal breakage and combined imunodeficiency. Patients with this condition also associate growth retardation with microcephaly, predisposition to malignancy and specific skin manifestations Here we report 3 year old girl known with NBS associated with cutaneous sarcoid-like lesions. She presented with one year history of squamous lesions on the face and the upper and lower limbs. The lesions were biopsied and the histopatological examination revealed nonnecrotizing epitheloid granulomas and raised the suspicion of a sarcoid-like entity. The interest of this case will serve to better understand clinical mainifestations in a rare genetic entity. Close follow-up is advised as cutaneous granulomas may be the first manifestation of systemic granulomas.

Rosai Dorfman disease (RDD) is a rare disease of histiocytic cells that is a cause of benign cervical lymphadenopathy (LAP) and massive generalized lymph node enlargement in children and adults. There are also some reports of cases with other human body organs involvement with or without LAP. A 7-month-old infant with a chief complaint of generalized massive LAP was referred to our center. RDD was diagnosed for him by cervical lymph node biopsy and according to his clinical manifestations, histopathologic and immunoreactivity study on the obtained sample. However, axillary and inguinal nodes are usually smaller than those in the neck areas, we present a case with enlarged axillary and inguinal lymph nodes in size about 4-6 cm. RDD is not a malignant illness and despite lymph node enlargement, most often decreases in its size happen without special treatment.

Thromboembolic events are a known complication of Inflammatory Bowel Disease (IBD) especially during disease relapse, more commonly in deep veins of extremities and lung, and rarely as Cerebral Sino venous Thrombosis (CSVT). We describe an 11 year-old male patient with 3 months history of Ulcerative Colitis (UC) who presented as pseudotumor cerebri due to superior sagittal sinus thrombosis during an acute exacerbation of his colitis, that successfully treated with heparin and then warfarin. In any known cases of UC that presenting as acute severe headache, consider CSVT and request brain MRI and MRV to facilitate the diagnosis and early treatment.

Neonatal Marfan syndrome is a rare and severe phenotype of this disease. A poor prognosis is anticipated due to the high probability of congestive heart failure, and mitral and tricuspid regurgitations with suboptimal response to medical therapy and difficulties in surgical management at an early age. We presented two consecutive patients with this disease who are the first reported cases from Iran to the best of our knowledge. Unfortunately both of them died shortly after the diagnosis. Neonatal Marfan syndrome was reported from Iran and has a poor prognosis like the patient reported from elsewhere.