Iranian Journal of Pediatrics
Volume:21 Issue: 2, 2011

The Mashhad University of Medical Sciences and the Sheikh Hospital in Mashhad sponsored a Cleft Lip and Palate Workshop 30 April - 1 May 2009. During the Workshop, 6 surgical cases were performed and televised live to the audience attending the conference. Two of those cases were unilateral cleft lip repairs. The surgical technique used to repair these patients by the primary author (JGM) is a hybrid technique. It has evolved over the last decade as a result of prior surgical literature as well as first hand observation of various surgical colleagues. The following manuscript describes the surgical technique used at the Cleft Workshop in a step-wise or atlas-like fashion. The technique portion of the paper describes the repair of the unilateral cleft lip and nasal deformity in roughly the order the first author typically performs the procedure. More importantly, the final section of the paper details the principles that form the foundation for the techniques described.

Congenital adrenal hyperplasia (CAH) is characterized by impaired biosynthesis of cortisol. 21-hydroxylase deficiency is the most common cause of CAH affecting 1 in 10000-15000 live births over the world. The frequency of the disorder is very high in Iran due to frequent consanguineous marriages. Although biochemical tests are used to confirm the clinical diagnosis، molecular methods could help to define accurate diagnosis of the genetic defect. Recent molecular approaches such as polymerase chain reaction based methods could be used to detect carriers and identify different genotypes of the affected individuals in Iran which may cause variable degrees of clinical expression of the condition. Molecular tests are also applied for prenatal diagnosis، and genetic counseling of the affected families. Here، we are willing to delineate mechanisms underlying the disease، genetic causes of CAH، genetic approaches being used in the country and recommendations for health care improvement on the basis of the molecular and clinical genetics to control and diminish such a high prevalent disorder in Iran. Also، the previous studies on CAH in Iran are gathered and a diagnostic algorithm for the genetic causes is proposed.

Application of Continuous Positive Airway Pressure (CPAP) in neonate with respiratory distress is associated with reduction of respiratory failure، reduced complications and mortality. Bubble CPAP (B-CPAP) and ventilator-derived CPAP (V-CPAP) are two most popular CPAP modes. We aimed to determine whether B-CPAP and V-CPAP would have different survival rate and possible complications. This prospective clinical trial was performed on 50 preterm neonates weighing 1000-2000 gr who were admitted to the neonatal intensive care unit of Afzalipoor Hospital because of respiratory distress between June 2009 and May 2010. Patients were randomly allocated into treatment groups using minimization technique. Survival analysis was applied to estimate and compare survival rates. Duration of oxygen therapy، hospital stay as well as hospitalization costs were compared using independent sample t-test. Estimated survival rates at 24 hours in B-CPAP and V-CPAP groups were 100% and 77% respectively. Corresponding figures at 48 hours were 100% and 71%. In addition the hospitalization cost in V-CPAP group was significantly higher than in B-CPAP group. According to our results، B-CPAP was effective in the treatment of neonates who were suffering from respiratory distress and reduced the duration of hospital stay. In addition to mentioned benefits، its low cost may be the reason to use B-CPAP broadly compared with V-CPAP.

Beta-thalassemia major is an autosomal recessive disease causing severe and hemolytic anemia, which begins about 2-6 months after birth. Iron overload, which arises from recurrent transfusion and ineffective erythropoiesis, can enhance oxidative stress in thalassemic patients. The aim of this study was to evaluate the serum total antioxidant capacity of patients with ß-Thalassemia major. Sixty six Iranian patients with β-thalassemia major and 66 age-gender matched controls were evaluated for serum total antioxidant status (TAS), uric acid (UA), bilirubin and albumin. In addition, serum ferritin and transaminases were recorded in these subjects. Significant increases of TAS, UA, and bilirubin were observed in the patient group, compared with the control group (P<0.01). Mean TAS and bilirubin in male patients was higher than in females (P=0.005 and P=0.008, respectively). There was also direct correlation between TAS and albumin (P<0.001), bilirubin (P<0.001) and UA (P=0.002). Endogenous antioxidants such as ferritin, UA and bilirubin can result in increased level of TAS in the patients with Beta-thalassemia major. Compensatory excess of TAS to oxidative stress could also be the reason for difference between our findings and previous studies.

Transcatheter closure of secundum atrial septal defect (ASD) with Amplatzer device is an alternative procedure to surgical repair، with some limitations. The aim of this study was to assess the initial and mid-term results of the treatment of ASD with Amplatzer septal occluder in children and adolescents. From May 2003 to January 2008 sixty three consecutive children and adolescent patients underwent transcatheter closure of ASD at a mean±SD age of 8. 5±4. 8 years (range 2. 2 to 18 years). All procedures were performed under local anesthesia and moderate sedation or general anesthesia with transthoracic echocardiography and fluoroscopic guidance. Stretch diameter of ASD was determined by balloon sizing catheter. Device selection was based on and matched to the standard diameter of the septal defect. Follow up at 24 hours، 1 month، 6 months، 12 months and yearly thereafter included physical examination، electrocardiography and transthoracic echocardiography. The mean ASD diameter، measured with transthoracic echocardiography and balloon catheter were 19. 5± 5. 5 mm and 20. 9± 6. 2 mm، respectively. The mean follow up period was 32. 4±18. 8 months. Deployment of the device was successful in 57 (90. 5%) and failed in 6 (9. 5%) patients. The major complication included dislodgement of device in 1 patient and device embolization to right ventricular inlet (surgically removed) in 1 patient. The minor complication included transient atrial tachycardia in 10 patients، paroxysmal supraventricular tachycardia in 2 patients during procedure، successfully terminated with medication. At 24 hour، 1 month، 6 month and 1 year follow up، total occlusion rates were 73. 6 %، 91%، 94. 7%، and 94. 7%، respectively. Transcatheter occlusion of ASD with Amplatzer device is an effective and safe procedure with minimal complication rate and short hospital stay، as well as excellent short and intermediate outcome in children and adolescents.

Laryngoscopy and tracheal intubation may cause significant cerebral and systemic hemodynamic responses. Many drugs have been shown to be effective in modifying these hemodynamic responses, including fentanyl, sufentanil, alfentanil and remifentanil. The purpose of the current study was to compare the efficacy of fentanyl, sufentanil, alfentanil and remifentanil on blunting cardiovascular changes during laryngoscopy and intubation in children. Eighty children, 1-6 years old, classified as American Society of Anesthesiologists physical status I and II who were scheduled for elective surgery with general anesthesia and orotracheal intubation, were enrolled in this randomized and double-blinded study. Patients were randomly assigned into four groups of 20 patients. Group F received fentanyl 1µg/kg-1, group S received sufentanil 0.1 µg/kg-1, group A received alfentanil 10 µg/kg-1 and group R received remifentanil 1 µg/kg-1 intravenously. After establishment of neuromuscular blockade confirmed with a nerve stimulator, laryngoscopy and orotracheal intubation were performed 3 min after induction. Hemodynamic variables including systolic and diastolic blood pressure (SAP, DAP) and heart rate (HR) were recorded at base line (before opioid administration), before laryngoscopy and one minute after orotracheal intubation. The patients'' characteristics and laryngoscopy grade were similar in all groups. There was no significant difference in the mean values of SAP, DAP and HR at each measured time between the four groups. There was significant difference in the mean values of SAP, DAP and HR measured over time in each group. The intravenous fentanyl attenuated laryngoscopy-induced SAP, DAP and HR increases better than sufentanil, alfentanil or remifentanil and hemodynamic stability is better preserved with fentanyl.

This study aimed to develop and test the validity of a risk score to be used as a simple tool to identify those children at high risk of sonographic non-alcoholic fatty liver disease (NAFLD). This cross-sectional study was conducted among 962 participants aged 6-18 years in Isfahan, Iran. They consisted of three groups of nearly equal number of normal-weight, overweight and obese individuals. Coefficients of the logistic regression models were used to assign a score value for each variable and the composite sonographic NAFLD risk score was calculated as the sum of those scores. Performance of model was assessed by receiver operating characteristic (ROC) curve procedure. Data of 931 participants was included in the analysis. The sonographic findings of 16.8% of participants were compatible with NAFLD. Age, sex, body mass index, waist circumference and serum triglycerides level were diagnosed as factors associated with NAFLD. The risk score was calculated as 50 for sonographic NAFLD. This study, to the best of our knowledge is the first of its kind in the pediatric age group, focuses on predicting sonographic NAFLD from easily-measured factors. It may suggest an association of hypertriglyceridemic-waist phenotype with NAFLD in the pediatric age group.

To determine the prevalence of congenital hypothyroidism (CH), permanent and transient CH. From November 2006 to September 2007, 63031 newborns were screened by measuring serum TSH obtained by heel prick. The neonates who had a TSH≥5mU/L were recalled for measurement of serum T4, thyroid stimulating hormone (TSH) and TSH receptor blocking antibodies (TRBAb) in venous samples. In 43 primarily diagnosed as cases of CH, treatment was discontinued at age 2-3 years for 4 weeks and T4 and TSH were measured again. Permanent or transient CH was determined from the results of these tests and radiologic evaluation. The incidence of congenital hypothyroidism was found to be 1:1465 with a female to male ratio of 1.19:1. The most common clinical findings were prolonged jaundice (73%), large anterior fontanel (56%) and wide posterior fontanel (55%). In 43 patients with CH, prevalence of permanent and transient form of the disorder was 53.6% and 46.4% respectively. Permanent CH was associated with higher initial TSH level than transient hypothyroidism (P<0.001). The most common etiology of permanent CH was dyshormonogenesis (57%). TRBAb was found in 6.8% of the total 43 cases. Congenital hypothyroidism in Iran may have different etiologies. Due to higher rate of transient CH than other similar researches, it is reasonable to follow these patients for a longer period to rule out the possibility of permanent hypothyroidism.

One of the most significant problems in pediatric dentistry is behavioral resistance of preschool children in the first visit. There is a debate on parental presence in operation room. The purpose of this study was to evaluate the Iranian 5-year-old children''s behavior including anxiety and cooperation relative to parental presence in the first and second dental appointments. The study was conducted on sixty seven 5-year-old children selected according to inclusion criteria and randomly divided into two subgroups. Children in group I were visited in parent''s presence and in group II in parent''s absence. Before the child''s first dental visit, parents were interviewed. Forty eight of the children receiving the initial examination were recalled for a second visit. The children''s responses during the Holst procedure of the first visit and restorative second visit were assessed using a combination of two measures including heart rate and clinical behavior. The dentist-patient interactions were regulated by standardized scripts and recorded on videotape. Then, the behavior of the child on the recording during each visit was quantified by two pediatric dentists independently according to Venham 6-point rating scale and Frankle 4-point rating scale. There were no significant differences between the heart rate measures of children in group I and II in the first and second visit (0.67, 0.8 respectively). There were also no significant differences between the clinical anxiety scores of children in the two groups in the first and second visit (0.98, 0.42 respectively). Moreover, there were no significant differences between the clinical cooperation scores of children in group I and group II in the first and second visit (0.88, 0.40 respectively), neither were there any significant differences between response measures of each child between two visits (P>0.05). In addition, there were no significant differences related to sex, parental education and dental experiences (P>0.05). Parental presence or absence doesn''t affect an Iranian 5-year-old child''s anxiety on the first and second dental visit, as well as an Iranian 5-year-old child''s cooperation on the first and second dental visit.

Disease and hospitalization can be the first crisis that a child encounters. The aim of this study is to reveal a clear picture of the meaning of hospitalization in children, to show the experience and behavior of hospitalized children and to discover the meaning and understanding of hospitalization in them. This study is a phenomenology study of qualitative research within the framework of Husserl Eidetic phenomenology through comprehensive interviewing. The objective group consisted of children 7-11 years old and their parents hospitalized in the children''s ward of 22 Bahman Hospital and the surgery ward of 15 Khordad Hospital during the study (2008). Method of selection of participants was as follows: having experience of hospitalization, having ability to answer the questions, and being volunteered. Sample size was detected by data saturation. In the method of sampling, an object group of 20 (12 children and 8 parents) were chosen and interviewed. The Seven Colaizzi Stages were used for analysis of data. The analysis of the interviews and the written narrations of the participants led to the extraction of 6 inner themes consisting of sickness, environment, reciprocal relationship, parents'' personal problems, mental and emotional matters and a spiritual dimension; all of which define a specific aspect of the experience of hospital in children and parents. The experience of hospitalization in children can be considered as a process of effort for returning to health and, on the whole, the regaining of the individual''s status in the world. Nurses can ease this process by showing the importance of experience and feelings of individuals at the time of hospitalization and help people to adapt themselves to their new surroundings. This matter can enable the nurses to utilize methods of helping in the adaptation of individuals and thus guide the unique powers present in every individual to ease and quicken recovery.

This study was conducted to determine the distribution and risk factors of retinopathy of prematurity (ROP) in premature infants referred to neonates intensive care unit (NICU) of central hospital of Kerman University of Medical Sciences, to obtain primary information on ROP in Kerman, Iran. In a cross sectional prospective study, data of premature infants screened for ROP including possible risk factors and eye examination results were recorded during 2006-2008 and analyzed by using logistic regression and chi-square tests. Out of 83 premature infants, 24 (29%) had different stages of ROP (CI 95%: 0.19-0.39). The infants'' mean gestational age (GA) and mean birth weight (BW) in ROP group were 30.17±1.8 weeks and 1247.92±237.1 grams (g), respectively. Logistic regression analysis showed a significant relation between GA and BW with ROP (P<0.001). Indication for treatment was set in 6 (25%) infants. The results of this study illustrate a relatively high prevalence of ROP in this series. GA and BW were independent ROP determinants.

Human pandemic influenza H1N1 virus as the cause of febrile respiratory infection ranging from self-limited to severe illness has spread globally during 2009. Signs and symptoms of upper and lower respiratory tract involvement, fever, sore throat, rhinitis, myalgia, malaise, headache, chills and fatigue are common. In this article we report the clinical presentation of Influenza A (H1N1) in our hospitalized children. Between September and October 2009, all children requiring hospitalization for suspected H1N1 infection were transferred to Pediatric Infectious Diseases ward. For all patients the throat swab was taken for PCR testing to confirm or exclude the diagnosis of H1N1 Influenza A. Case patients consisted of H1N1-positive patients. Age, sex, symptoms, signs, laboratory data, CXR changes, details of therapy, duration of admission and patient outcome were documented. Twenty patients were H1N1 positive. Mean age of the patients was 65.50±9.8 months. Fever and coughs were with 55% the most commonly reported symptoms. Other presentations included vomiting (55%), abdominal pain (25%), cyanosis and dyspnea (5%), body ache (40%), rhinorrhea (80%), sore throat (35%), head stiffness (5%) and loss of conciousness (5%). The median temperature of the patients was 38.5ºC. Chest X-Ray changes were noted in 13 out of 20 patients (65%). Mean leukocyte and platelet was 6475 and 169000 respectively. Seventeen (85%) patients were treated with Oseltamivir, 3 patients received adjuvant antibiotics. The mean duration of admission was 3 days. Three patients required intensive care support and all of them expired due to superinfection. Our data confirm that the presentation of influenza in children is variable and 2009 H1N1 influenza may cause leucopenia and thrombocytopenia.

QT dispersion (QTd) has been proposed as a marker of ventricular repolarization inhomogeneity and several investigations have proved the relationship between it and cardiac ischemia, ventricular arrhythmia and sudden cardiac death. The aim of this study was to assess the relation between coronary artery involvement and QTd, and QTc dispersion (QTcd) in the acute phase of Kawasaki disease (KD). We studied 65 patients with acute KD. Patients were divided into 3 groups. Group one consisted of 48 patients without coronary artery involvement. Group two comprised 13 patients with small to medium size aneurysm. In Group three there were 4 patients with giant aneurysm or multiple small to medium size aneurysms or thrombosis in coronary arteries. For each patient 12 lead ECG was obtained, and QT, QTc, QTd, QTcd, and RR interval were calculated. There were 40 males and 25 females with a mean age of 41.4±31.1 months. There was no significant difference in QT, QTc, RR measurements between 3 groups. QTd was greater in group 3 versus group 1 and 2, but the difference was not statistically significant (P=0.06). QTcd was significantly greater in group 3 than in groups 1 and 2 (75.02±11.53 ms versus 46.82±15.39 ms and 48.88±10. 55 ms respectively (P = 0.04). The sensitivity of QTcd ≥60 ms to detect the patients with severe coronary arteries involvement was 100%, the specificity was 93.4%, positive predictive value was 50%, negative predictive value was 100%, and accuracy was 93.8%. QTcd can be used as a predictive factor for diagnosis of severe coronary arteries involvement in the acute phase of KD.

Atopic dermatitis (AD) is the most common chronic relapsing skin disease seen in infancy and childhood. The intestinal microbiota play an important role in immune development and may play a role in the development of allergic disorders. Manipulation of the intestinal microbiota by synbiotics may therefore offer an approach to the prevention or treatment of AD and allergic diseases. We studied the clinical and immunologic effects of a new symbiotic (a mixture of seven probiotic strains of bacteria and Fructooligosaccharide) in infants and children with AD. In a randomized, double-blind, placebo-controlled study, 40 infants and children aged 3 months to 6 years with AD received either a synbiotic or placebo for 8 weeks. The Severity Scoring of Atopic Dermatitis (SCORAD) index was recorded at baseline and also at 4 and 8 weeks of treatment. There was no significant difference between the probiotic and placebo group in baseline characteristics including sex, age, family history, corticosteroid usage and prick testing. Mean age was 23 months. The synbiotic group showed a significantly greater reduction in SCORAD than did the placebo group (P=0.001). No specific effect was demonstrated of the probiotics employed on cytokine profile (P=0.4, P=0.6). Egg white was the most common (45%) allergen followed by peanut and cow''s milk. This study provides evidence that a mixture of seven strains of probiotics and Fructooligosaccharide can clinically improve the severity of AD in young children. Further studies are needed to investigate the effects on underlying immune responses and the potential long term benefits for patients with AD.

Evaluating the effect of zinc sulfate in improving the clinical manifestations of acute bronchiolitis in children younger than 2 years. This was a double blind pilot trial on 50 patients aged 2 to 23 months at Ghaem and Dr. Sheikh Hospitals in Mashhad from January 2008 to March 2009. Patients were randomly divided into two groups: a case group received oral zinc sulfate and to the control group was given placebo. Mean age of case group was 168.0±108.6 days and control group 169.2±90.4 days (P=0.98) with male predominance in both groups. At first there was no statistically significant difference between the two groups in reducing the symptoms. But 24 hours after treating, improvement of some important manifestations including tachypnea, subcostal and intercostal retraction, wheezing and cyanosis revealed statistically significant difference in control group in comparison with case group (P=0.04). Zinc sulfate has no benefit in improving clinical manifestations of acute bronchiolitis.

Ferrous sulfate drops are routinely used in Iran in infants older than 6 months of age. Effect of ferrous sulfate drops in preventive or therapeutic doses on gastrointestinal mucosa of infants is not studied as yet. Upper gastrointestinal complications due to acute iron poisoning are well known in this age group. In this article, we reviewed published articles on iron deposition in upper gastro-intestinal tract and also introduce clinical, endoscopic and histological findings in three cases with iron deposition in duodenal mucosa.Cases Presentation: We encountered three cases of iron deposition in duodenal mucosa among about 8000 biopsies during a 10 year period which is a very low incidence despite routine use of iron supplement in children above 6 months of age in this country. One of our cases suffered from steatorrhea and another from failure to thrive, which raises concern about effects of iron deposition in small intestine. The clinical significance and effects of iron deposition in pediatric age group is yet to be elucidated. Iron deposition as a solitary finding is not reported in duodenal biopsies of infants as yet. Since iron supplement is widely used in this age group, it is justified to consider its deposition and possible effects on absorption.

We report five term neonates born at an altitude of 1890 meters with transient early neonatal cyanosis due to right-to-left shunting at atrial level through patent foramen ovale. The five neonates with no clinical sign or symptom other than marked cyanosis were examined in two neonatal units of Erzurum city. Hematologic and radiologic examinations were normal. Partial oxygen pressure (PO2) in the arterial blood samples was lower than 45 mmHg in all of the patients, and did not increase more than 15 mmHg in any of the patients after inhalation of 100% oxygen. Echocardiography revealed normal intracardiac structure. The right-to-left interatrial shunt at diastole was detected through a patent foramen ovale in all of these infants. By only observation with no treatment, diastolic right-to-left shunt disappeared in 40.15±9.52 hours. Oxygen saturation was increased from 69.80±9.55 percent to 90.40±8.80 percent. The patients were discharged from the hospital at 5.6±0.4 days of life. Follow up for 6 months revealed no clinical problem in any of the cases. Transient cyanosis can be seen in the very early neonatal period because of interatrial right-to-left shunting in some healthy term neonates born at an altitude of 1890 meters. Decreased right atrial compliance due to relative hypoxia at that altitude can be speculated to be the causative mechanism.

Henoch-Schönlein purpura (HSP) is the most common vasculitis of childhood. HSP can affect multiple organs presenting with a characteristic rash in most of the patients. Familial Mediterranean Fever (FMF) is an inherited inflammatory disease common in mediterranean populations. HSP is the most common vasculitis seen in children with FMF. A 16 year old boy was referred with history of abdominal pain lasting for 20 days. He was hospitalized and had appendectomy. Due to the persistence of his abdominal pain after surgery he was admitted to our hospital. His physical examination showed palpable purpuric rashes symmetrically distributed on lower extremities. Abdominal examination revealed periumbilical tenderness. Laboratory tests showed elevated erythrocyte sedimentation rate, C-reactive protein and fibrinogen. Urinalysis revealed microscopic hematuria and severe proteinuria. The fecal occult blood testing was positive. Based on these clinic findings, the patient was diagnosed as HSP with renal, gastrointestinal tract and skin involvement. We performed DNA analysis in our patient because he had diagnosis of vasculitis with severe symptoms and found that he was carrying heterozygote P369S mutation. Our case is noteworthy as it indicates that it may be important not to overlook presence of FMF mutations in patients with a diagnosis of severe vasculitis.

Congenital germ cell tumors are uncommon. The most common site of teratoma is in the sacrococcygeal region. Teratoma arising from the head and neck comprises less than 10% of reported cases and of these, nasopharyngeal lesions are rare. Teratomas are generally benign, and have a well recognized clinical and histopathological entity. We present a case of nasopharyngeal teratoma (NPT) associated with a wide cleft palate. A 20 day old female neonate with a teratoma of the nasopharyngeal area, and wide cleft palate was referred to our center. The protruded mass which measured 6×4×3cm, was of soft consistency, blocked the airway, and prevented oral feeding. Preoperative evaluation and imaging was performed and mass was excised 2 days after admission. Pathology revealed a well-differentiated mature solid teratoma (hairy polyp). The patient had no complication in the post-operative period. Cleft palate was surgically repaired when 2 years old. She is now a six year old girl with normal development. Congenital nasopharyngeal teratomas are usually benign. Surgery is the treatment of choice, and should be undertaken on an urgent basis, especially in a patient who presents with signs and symptoms of airway obstruction.

Pneumomediastinum is commonly caused by iatrogenic injury such as surgery on the cervical planes and chest or by tracheostomy. It is also well known that emphysema may occur after dental treatments using an air turbine drill, but there have been few cases of emphysema which extended to the mediastinum. A 16-year-old boy is presented with subcutaneous emphysema and pneumomediastinum which developed 24 hours after surgery for extraction of an inferior second molar. We first describe history, clinical presentation and radiologic appearance of our patient and then review the literature about oral surgery causing pneumomediastinum. Surgical procedures in oral cavity can lead to the development of emphysema and pneumomediastinum when air turbine dental drills are used. To avoid these complications, air turbine high speed drills should be used only in necessary cases.

Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder with a birth incidence of 1:6.000. It can virtually affect any organ system and all racial and ethnic groups. Clinical manifestations of TSC have variable penetrance. Many affected patients come to medical attention because of seizures or dermatological manifestations. Since no single feature of TSC is pathognomonic, an evaluation of all clinical features is necessary.. ....