Iranian Journal of Pediatrics
Volume:22 Issue: 4, 2012

The importance of pediatric research especially in the ethically proven trials resulted in considerable legislative attempts in association with compiling ethical guidelines. Because of children’s vulnerability conducting pediatric research raises different ethical issues; the two most important of which are informed consent and risk-benefit assessment. Differences in ethical standards and socio-cultural issues limit application of ethical standards. At the aim of finding a solution we critically reviewed guidelines, and literatures as well as Islamic points in addition to comparing different viewpoints in application of ethical standards in pediatric research The literature review showed that pediatric research guidelines and authors viewpoints have the same basic ethical core, but there are some variations; depend on cultural, religious, and social differences. Furthermore, these standards have some limitations in defining informed consent according to child’s age and capacity upon application In this regard Islamic approach and definition about growth development and puberty shed light and clarifies a clearer and more rational address to the issue

Silver–Russell syndrome (SRS) is a clinically and genetically heterogeneous syndrome which is characterized by severe intrauterine and postnatal growth retardation, and typical characteristic facial dysmorphisms. It has been associated with maternal uniparental disomy (UPD) for chromosome 7 and hypomethylation of imprinting control region 1 (IGF2/H19) in 11p15. UPD refers to the situation in which both copies of a chromosome pair have originated from one parent. UPD can be presented both as partial heterodisomy and isodisomy. The aim of this study was to determine the maternal UPD7 (matUPD7) in 13 Turkish SRS patients. Genotyping for matUPD7 was performed with microsatellite markers by polymerase chain reaction. The maternal UPD7 including the entire chromosome was identified in 1/13 (7.6 %) of individuals within SRS patients. There were no significant differences between clinical features of matUPD7 case and other SRS cases except congenital heart defects. It is often difficult to establish diagnosis of a child with intrauterine growth retardation (IUGR), growth failure and dysmorphic features. Thus, screening for matUPD7 in IUGR children with growth failure and mild SRS features might be a valuable diagnostic tool.Key Words: Silver–Russell Syndrome; Maternal UPD7; Microsatellite Markers; Intrauterine Growth Retardation

This study was conducted to compare serum Cytosolic β-Glucosidase (CBG) levels of age-matched control patients with those of infants with neonatal necrotizing enterocolitis (NEC), to determine eventual association between Serum Cytosolic β-Glucosidase levels with intensity of the disease in NEC infants.

A variety of sign, symptoms and laboratory findings are more common in children with organic abdominal pains. to evaluate the prevalence of organic and functional abdominal pains and relation of red flags to organic pains in 100 children with recurrent abdominal pain (RAP). One hundred consecutive patients with RAP were enrolled in the study. A complete interview and physical examination was made for each patient, accompanied by a series of laboratory, clinical and para-clinical examinations. The data were recorded and analyzed. Logistic regression analysis was used to model and formulize correlations between sign, symptoms, and laboratory findings with organic and functional abdominal pain. Among 100 patients (52% male, 48% female, Age: 9.29±3.17) diagnostic works up revealed organic pain for 57 patients. The most common symptoms of the patients included constipation, diarrhea, chest pain, cough, headache, vomiting, hematuria, and dysuria. Fecal incontinence, delayed puberty, organomegaly, jaundice, and family history of inflammatory bowel disease were reported in none of the patients with RAP. Fever, pain not located in periumbilical area, nocturnal pain, elevated erythrocyte sedimentation rate, weight loss, growth disorder, and abdominal tenderness were among the red flags which revealed diagnosis of organic pain in this study. A series of red flags could increase likelihood of finding organic pain in children with RAP.

Magnesium (Mg) is thought to be an important element in the pathogenesis of acute asthma attacks. We hypothesized that erythrocytic Mg would be decreased during an acute asthma exacerbation. We aimed at investigating plasma and erythrocytic Mg in acute asthmatic children. This case-control study included 30 Egyptian outpatients with acute asthma. Thirty healthy matched children were included as controls. All candidates had measurements of plasma and erythrocytic Mg levels before and after treatment. No significant differences were detected in plasma Mg levels between cases and controls (1.53±0.33 mmol/L versus 1.67±0.50 mmol/L respectively, P =0.2). However, erythrocytic Mg levels were significantly reduced in cases when compared to controls (1.06±0.43 mmol/L versus 2.57±0.59 mmol/L respectively, P<0.001). Plasma Mg levels did not significantly change in acute asthmatics before and after their rescue treatment (1.53±0.33 mmol/L versus 1.68±0.31mmol/L respectively, P=0.07). In contrast, the study detected a significant increase in erythrocytic Mg levels in cases after their treatment from acute attacks (1.06±0.43 mmol/L versus 1.56±0.23 mmol/L respectively, P<0.001), with significant negative correlation with severity of attack (Spearman’s rho=-0.647, P<0.001). Erythrocytic Mg levels were significantly lower during the acute asthma, and were negatively correlated with severity of exacerbation, while plasma Mg did not significantly change. Only erythrocytic Mg levels were significantly elevated after receiving rescue treatment.

Cow's milk allergy has different presentations in children and can cause functional bowel symptoms such as chronic constipation. The aims of this study were to investigate the role of cow’s milk allergy as a cause of chronic constipation and effect of cow’s milk free diet (CMFD) on its treatment in children. We performed a randomized clinical study comparing CMFD with cow’s milk diet (CMD) in two groups each consisting of 70 patients (age range, 1-13 years) with chronic functional constipation (defined as Rome III criteria). All subjects had been referred to a pediatric gastroenterology clinic and had previously been treated with laxatives for at least 3 months without success; also all 140 patients performed skin prick test. The case group received CMFD for 4 weeks. After that they received CMD for 2 extra weeks. The control group received CMD for whole 6 weeks. A response was defined as decreased in signs and symptoms that not fulfilled Rome III criteria after 4 weeks of CMFD and came back to Rome III criteria after 2 weeks of CMD challenge. After 4 weeks 56 (80%) patients of the case group responded in comparison to 33 (47.1%) patients in the control group (P=0.0001). In the case group after 2 weeks challenge 24 out of 56 (42.8%) responders developed constipation according to Rome III criteria. With other words, the frequency of cow’s milk allergy among constipated patients was 80%. Only one patient had positive skin prick test. In children, chronic constipation can be a manifestation of cow’s milk allergy. At present, although several aspects must be further investigated, a therapeutic attempt with elimination diet is advisable in all children with constipation unresponsive to correct laxative treatment.

Intensified management of gestational diabetes mellitus can normalize birth weight. However, it is still unknown whether intrauterine exposure to maternal diabetes is a risk factor for changing hormone levels involved in the development of insulin resistance in these infants. We compared insulin and leptin levels in appropriate for gestational age (AGA) infants of diabetic and non diabetic mothers. We performed a cross-sectional study in the department of Neonatology of the Hospital of Gynecology-Pediatrics, in Leon, Mexico. We evaluated 182 full term AGA newborns (86 infants of diabetic and 96 of non-diabetic mothers). A venous blood sample was taken from cord blood immediately after the separation of the placenta and glucose, insulin and leptin levels were measured. In all diabetic mothers HbA1c was also evaluated immediately post-partum. Leptin, insulin and insulin resistance index were significantly higher in infants of diabetic mothers. Leptin levels were positive correlated with insulin, parents` body mass index and age in the entire group. In infants of diabetic mothers only insulin levels showed a significantly correlation, whereas in those of non-diabetic mothers only mothers` age was significantly correlated with leptin levels. AGA infants of diabetic mothers showed higher leptin, insulin levels and insulin resistance index than those of non-diabetic mothers.

A study to validate and calibrate Pediatric Index of Mortality-2 (PIM2) in children admitted to our pediatric intensive care unit (PICU). This is a prospective cohort study performed in Bahrami Children’s Hospital affiliated to Tehran University of Medical Sciences. We studied the patients admitted to PICU from May 2007 to November 2008. Clinical measures were identified upon arrival in PICU. We used PIM2 score and logistic regression analysis to compare expected mortality risk with observed mortality rate. Receiver operating characteristics (ROC) curve analysis was done and standardized mortality ratio was calculated. PIM2 Index assessment was performed by use of Hosmer and Lemeshow goodness-of-fit test. 240 patients were included in this study. The model fit was achieved adequately (P value = 0.741). The area under the ROC curve was 0.795 (0.715-0.875 for 95% confidence interval) and standardized mortality ratio was 1.8 (1.28-2.465 for 95% confidence interval) High-risk group diagnosis with adjusted odds ratio (AOR)=14.75, pupil reaction to light (AOR=0.13) and duration of stay in PICU (AOR=1.03) had significant statistical association to pediatric mortality. PIM2 is a good index for prediction of mortality in our pediatric intensive care unit. This study revealed that there is significant statistical association between the children mortality and the length of hspita;ization, pupillary light reflex and the risk level category on admission.

Precocious puberty is of concern because of the underlying disorders, the short adult stature, and the psychosocial difficulties. This study was carried out in order to evaluate the characteristics of children referred to pediatric endocrinology clinic with diagnosis of precocious puberty. In a cross-sectional study between February 2007 and September 2009, all of the children referred to pediatric endocrinology clinic in North-West Iran with diagnosis of precocious puberty were recruited. Data of 106 girls (82.2%) and 23 boys (17.8%) were analyzed. Mean age of the patients at the time of referral was 6.6±2.8 years (ranging 0.3-14 yr), which was 7±3.9 (ranging 0.3-14 yr) for boys and 6.6±2.5 (ranging 0.8-12 yr) for girls (P=0.6). Out of 129 subjects, 56(43.4%) had precocious puberty, 71.4% (35 cases) of them were due to central precocious puberty and 28.6% (16 cases) were pseudo-precocious puberty. 73 out of 129 subjects (56.6%) were due to normal variants of puberty, normal puberty, and no puberty. 87.5% of subjects with central precocious puberty were idiopathic. Most of children referred with diagnosis of precocious puberty have benign normal variants. Most of cases with precocious puberty are affected with central precocious puberty, especially with idiopathic form of it.

The purpose of this study is to propose an innovative method of knowledge transfer that aims to improve health literacy about pediatric infectious diseases prevention in families. Children have an appreciable role in this scheme. This study is a before and after trial that has been conducted in Hamedan in 2009. After changing seven infectious disease topics into childish poems, we selected five kindergartens randomly and taught these poetries to the children. Teaching process held after a pretest containing 24 questions that examined103 of parents about mentioned topics. The same post-test was given after 4 months of teaching process. The mean of correct answers to the pretest was 59.22 % comparable with 81.00%. for post-test (P<0.00). Gender and knowledge degree could not change the results significantly. Assuming one’s correct answers to the questions as his/her Knowledge Mark, the mean of this variable increased 5.32 by this method. This cost-effective and joyful method had successful results in promoting health knowledge. Children are able to play an active role in family’s health situation. Learning within family atmosphere without any obligations makes our scheme a solution for paving the knowledge transferring way.

Recent data suggest that during mechanical ventilation, lateral patient position (in which the endotracheal tube is horizontal) decreases the incidence of bacterial colonization of ventilated neonates. The objective of this study was to evaluate the influence of lateral and supine position on bacterial colonization of endotracheal tube in neonates. We conducted a prospective, randomized, clinical trial with 31 intubated neonates (intubated within 48 hours after birth); sixteen neonates were positioned supine (supine group), and fifteen were maintained in the lateral position (lateral group).Tracheal aspirates were cultured in second and fifth days of mechanical ventilation. Data were analyzed with SPSS version 16. In the second day of ventilation, positive cultures were recognized in 6.2% of supine group and 6.7% of lateral group. After 5 days, tracheal cultures were positive in 25% (4 neonates) of supine group and 13.3% (2 neonates) of lateral group that wasnt statistically significant (P=0.9 in second day and P=0.9 in the fifth day). The most common organisms isolated from tracheal aspirates were Gram-negative rods (Klebsiella). Since respiratory contamination is very common among ventilated neonates and the effect of lateral position on bacterial colonization of endotracheal tubes of intubated neonates wasnt established in our study, further studies are required to suggest ways to decrease bacterial colonization of intubated neonates.

To analyze the clinical and radiological features of paragonimiasis in children and raise the awareness of this disease. A total of 58 paragonimiasis patients were reviewed. They were 42 boys and 16 girls aged 2.0 to 15.3 years. Among these patients, 20 were diagnosed in the recent 5 years, 46 with a history of raw water or food ingestion. Except 2 patients without any complaint, the most common features involved the systemic (41, 70.7%) and respiratory systems (43, 74.1%), followed by abdominal, cardiac and nervous systems, with rash and mass. Eosinophilia was noted in 46 (79.3%) patients, granulocytosis in 45 (77.6%), anemia in 14 (24.1%), and thrombocytopenia in 3. Imageology showed pneumonia in 26 (44.8%) patients, pleurisy in 28 (48.3%), hydropericardium in 17 (29.3%), ascites in 16 (27.6%), and celiac lymphadenitis in 13 (22.4%). Besides hepatomegaly and splenomegaly, calcification and multiple lamellar low echogenic areas in the liver were noted, each in one patient. Abnormal brain imaging was noted in 4 of 10 patients. Karyocyte hyperplasia with eosinophilia was noted in all the 19 patients who received bone marrow puncture. Paragonimiasis should be considered in the differential diagnosis of patients with multiple organs or system lesions, especially those with eosinophilia, serous cavity effusion, respiratory, cardiac, digestive system, nervous system abnormality, and/or mass. Healthy eating habit is helpful for paragonimiasis prevention.

0bjective: Juvenile idiopathic arthritis is the most common rheumatologic disorder of childhood. Cardiac involvement as pericarditis, myocarditis and valvular disease is common in Juvenile idiopathic arthritis. However, there are, few study concerning systolic and diastolic functions of the left ventricle in children with Juvenile idiopathic arthritis. P wave dispersion is a sign for the prediction of atrial fibrillation. A recent study found that rheumatoid arthritis patients had an abnormally high P wave duration and P wave dispersion, markers for supraventricular arrhythmogenicity. In this study, we aimed to evaluate P wave dispersion and its relation with diastolic dysfunction of the left ventricle in patients with Juvenile idiopathic arthritis. We performed electrocardiography and Doppler echocardiography on patients and controls. Maximum and minimum P wave duration were obtained from electrocardiographic measurements. P wave dispersion defined as the difference between maximum and minimum P wave duration was also calculated. No statistically significant differences were found between the patients and controls in minimum, maximum P wave duration and P wave dispersion. Among the diastolic parameters in patients group, increased late flow velocity, decreased early flow velocity and prolonged isovolumic relaxation time reflected diastolic dysfunction. During 12 months of follow-up, no supraventricular arrhythmias were documented in Juvenile idiopathic arthritis with diastolic dysfunction. Juvenile idiopathic arthritis with diastolic dysfunction has normal atrial conduction parameters and therefore seemingly do not have an increased risk of atrial fibrillation.

Asphyxia-induced cardiac insult is one of the major causes of mortality and morbidity in the course of perinatal asphyxia. Nowadays, a remarkable trend of interest is sensed introducing a plausible modality for early detection of cardiac insults at the beginning stages of asphyxia. In this study we aimed to evaluate diagnostic utility of transmitral Doppler–derived parameters as well as left atrial ejection force index as a marker of left atrial contractile function in these patients. In a prospective study selected cases of 26 asphyxiated newborns with preserved systolic function underwent conventional transmitral Doppler flow echocardiographic assessment. Left atrial ejection force index was further calculated for all patients. Data was compared with normal ranges of healthy newborns in order to clarify the diagnostic utility of these parameters for determining minor cardiac insults in this age group. We found that mildly asphyxiated newborns showed an increase in the values of left atrial ejection force index (5.44±2.12 kilodyne vs. 6.66±2.17 kilodyne, P= 0.02) and left atrial filling fraction (39%±10% vs. 45%±8%, P= 0.01). Furthermore, the acceleration and deceleration rate of early filling flow peak velocity were decreased in this group of asphyxiated newborns. Assessment of left atrial ejection force in mildly asphyxiated newborns reveals that newborns with even mild asphyxia, although could not be categorized in conventional grading system, suffer to some extent from a ventricular filling abnormality. This type of latent ventricular filling abnormality could simply be unmasked by calculation of atrial ejection force index.

Streptococcus pneumoniae is a common worldwide potential pathogen causing pneumonia among children and the detection of pneumococcal infections by conventional culturing techniques is cumbersome. The present study describes a comparative analysis of sensitive nested-PCR and bacterial culture in pediatric patients with clinical and radiological indication of S. pneumoniae infection. PCR was performed using outer primers to amplify a 348-bp region and inner primers a 208-bp region of the pneumolysin gene. For pneumolysin PCR assay, DNA from peripheral blood and middle ear fluid (MEF) samples was extracted by salting out method. The sensitivity of the assay was evaluated with about 0.06 pg of purified S. pneumoniae genomic DNA. Among 90 MEF culture negative samples from acute otitis media pediatric patients, 8.8 % pneumolysin-PCR positivity was detected, demonstrating the sensitivity and reliability of PCR for rapid pneumonia evaluation. Binomial test of proportionality performed on (SPSS 17) gives P< 0.05 indicating that PCR technique is statistically significant and sensitive in the diagnosis of S. pneumoniae infection. The research work evaluated the effectiveness and efficacy of nested-PCR for detecting S. pneumoniae in pediatric patients with clinical and radiological confirmation of bacterial infection. This simplified method permitted quick selection of the patients and played a significant role in preliminary management of pneumococcal infections.

Hypophosphatemic rickets is an uncommon metabolic bone disorder which affects all ages and both sex. It is characterized by low concentration of serum phosphate levels, impairement of mineralization of bone matrix and teeth with variable etiology. Dental problems in this disorder have not been described well in previous study. All hypophosphatemic rickets patients who came to a referral clinic during 2008-2010 enrolled in this study. All patients had low phosphorous and high ALP, normal PTH and 25 hydroxy vitamin D and normal of low level of serum calcium. After diagnosis all patients were examined by a dentist for enamel hypoplasia, taurdontism, dental abscess, gingivitis, dental caries, and dentition delay. Nineteen patients enrolled in this study. The average age of the patients was 10 years (±4.23) (range 3-17). 79% percent of patients had regular follow-up after diagnosis of background disease. Dental caries and delay in the dentition are most prevalent (each one 47.7%) that was followed by enamel hypoplasia in 42.1% patients. Other problems were taurdontism in 15.8% patients, dental abscess and gingivitis in 10.9%. Hypophosphatemic rickets is a disease with different clinical features; one of them is dental problem that dental caries is the most common problem.

The aim of this study is assessment of effects of different assisted reproductive techniques (ART) like in vitro fertilization (IVF) and intra cytoplasmic sperm injection (ICSI) on prelinguistic behavior of infants conceived by these techniques. In this descriptive, cross sectional study, prelinguistic behavior of 151 full term ART infants of Royan Institute have been assessed in Children's Health and Development Research Center of Tehran from August 2007 until August 2009. Questionnaires were completed by parents at 9 months old. The questionnaire was standard according to Early Language Milestone Scale-2 (ELM-2). Data were analyzed by SPSS version 16 and using chi-square test. Twenty-two (14.5%) of infants were conceived by IVF and 129 (85.4%) by ICSI. Number of infants with delay in reduplicated babbling in ICSI method was more than in IVF. There was only a significant difference in echolalia delay in the two sexes. Echolalia was delayed more in boys. Delay of reduplicated babbling was more in infants of younger mothers. There was no relation between speech and language defect of parents and infants. This study showed that prelingustic behavior of ART infants are affected by kind of ART method, infant sex, and mother’s age at the time of pregnancy.

Brain abscess in young infants is extremely rare and usually associated with a previous history of bacterial meningitis or septicemia. Here we report a cerebellar abscess mimicking brain tumor with atypical clinical and paraclinical presentations. A two-month old previously well-baby boy was referred to us with persistent vomiting, strabismus and developmental regression. The brain imaging showed a right cerebellar mass with multiple small cysts inside the lesion. Elevated serum alfa-fetoprotein associated with cystic and solid posterior fossa mass proposed the preoperative diagnosis of teratoma but tumor cells were not found inside the pathology specimen. The culture of the sample was positive for staphylococcus aureus. The interest of this case lies in the atypical features of clinical and radiological evaluations in a young infant associated with an abnormal alfa-fetoprotein level of serum.

Nephropathic cystinosis is an autosomal recessively inherited metabolic disorder presenting with metabolic acidosis, Fanconi syndrome and renal failure. We present a 6-year-old girl with severe growth failure, hyponatremia and hypokalemia. Her parents were 4th degree relatives. Two relatives were diagnosed as end stage renal failure. She also had persistant hypokalemic hypochloremic metabolic alkalosis. Her renal function was normal at presentation. She was thought to have Bartter syndrome with supporting findings of elevated levels of renin and aldosterone with normal blood pressure, and hyperplasia of juxtaglomerular apparatus. Her metabolic alkalosis did not resolve despite supportive treatment. At 6th month of follow-up proteinuria, glucosuria and deterioration of renal function developed. Diagnosis of cystinosis was made with slit lamp examination and leukocyte cystine levels. At 12th month of follow-up her metabolic alkalosis has converted to metabolic acidosis. In children presenting with persistant metabolic alkalosis, with family history of renal failure, and parental consanguinity, cystinosis should always be kept in mind as this disease is an important cause of end stage renal failure which may have features mimmicking Bartter syndrome.

Congenital midline cervical cleft (CMCC) is a rare developmental anomaly of the anterior neck, mostly found in Caucasian females. Most authors consider it within the spectrum of branchial arch developmental abnormalities. There have been about 100 reported cases in the literature. However, there is no agreement on the age of surgery in this anomaly. In this report we present our experience of managing this anomaly in the early neonatal period in two neonates. One of them had concomitantly congenital heart disease. It is important to recognize this rare congenital anomaly in neonate. Although the exact time of surgery for CMCC is not mentioned, based on our results the earliest time of intervention is recommended since then the operating result will be excellent.

Induction of general anesthesia in a patient with compromised airway has always caused dilemma to anesthesiologist, especially for toddler this is paramounted. An 18 month old boy had a huge sized lingual mass which not only filled entire oral cavity but protruded outside the mouth up to about 5 cm. Having in mind the diagnosis of hemangioma, prior to induction it was attempted to reduce the mass but could be reduced only minimally by manual decompression. While the tongue, still outside the mouth, with full preparedness for anesthesia was induced maintaining the spontaneous ventilation. During laryngoscopy and intubation the tongue was pulled out of the oral cavity to ease the passage of the endotracheal tube. The mass was excised successfully and patient had an uneventful postoperative course. Maintenance of spontaneous ventilation is the crucial safety element during induction.

transcatheter balloon valvuloplasty has been the accepted first line treatment for congenital pulmonic stenosis (PS) in children. Transcatheter closure of perimembranous ventricular septal defect (VSD) with Amplatzer VSD occluder is an alternative to surgical repair. A 12 year old boy presented with history of exertional dyspnea and atypical chest pain. Physical finding were suggestive of severe pulmonic stenosis. Transthoracic echocardiography, right and left ventricular angiography showed medium to large sized perimembranous VSD and severe valvular pulmonary stenosis. Transcatheter closure of VSD was done first so as to avoid large left to right shunt across VSD after Balloon pulmonary valvuplasty. we demonstrated the feasibility and success in treating combined ventricular septal defect and severe pulmonary valve stenosis with transcatheter interventional procedure in the same session.

Congenital insensitivity to pain with anhidrosis is an extremely rare disorder in which injuries can often be missed by patient, parents and even by orthopaedic surgeon. Pain and tenderness, on which a trauma team so much depends to make a clinical diagnosis and to decide whether to go for radiological evaluation can be misleading in this rare syndrome. So complete clinical examination still forms the corner stone to avoid misdiagnosis and pick up the rare disorders. We present a 5 year old girl child, who was brought to us as a case of one and a half month old neglected trauma left leg and was diagnosed to be suffering from Congenital insensitivity to pain with anhidrosis (HSAN Type IV). Congenital insensitivity to pain with anhidrosis is extremely rare entity, in which patients are subjected to repeated injuries which are often neglected. There is no specific treatment but patient training and parent education are key to avoid further neglect and damage.