Iranian Journal of Pediatrics
Volume:20 Issue: 3, 2010

Practicing medicine according to the best evidence is gaining popularity in the medical societies. Although this concept, which is usually called Evidence Based Medicine (EBM) has been explained in many resources, it has not been addressed enough in pediatrics. In this review, we briefly explained Evidence Based Medicine approach and its applications in pediatrics in order to help the pediatricians to efficiently integrate EBM into their daily practice.

There are some controversial studies on effects of anti-epileptic drugs (AEDs) on serum IgG subclasses; however, the role of these medications is still unclear. The aim of this study was evaluation the effects of anti-epileptic drugs on serum concentration of IgG and its subclasses. Serum IgG and IgG subclasses of 61 newly diagnosed epileptic patients were measured at the beginning of monotherapy with carbamazepine, sodium valproate, and phenobarbital, and 6 months later. Measurement of IgG and its subclasses was performed using nephlometry and ELISA techniques, respectively. Reduction of at least one IgG subclass was found in 6 patients 6 months after treatment with AEDs. Among 27 patients receiving carbamazepine, decrease in at least one serum IgG subclass level was found in 5 patients. Among 20 patients using sodium valproate, only one patient showed decrease in IgG2 subclass. None of the 14 patients using phenobarbital revealed significant decrease in IgG subclasses. No infection was seen in the patients with reduction of subclasses. Although in our study, children with selective IgG subclass deficiency were asymptomatic, assessment of serum immunoglobulin levels could be recommended at starting the administration of AEDs and in serial intervals afterward in epileptic patients.

Dilated cardiomyopathy is the end result of chronic iron overload in patients with beta thalassemia major. The objective of the present study was to evaluate the safety and efficacy of Carvedilol in patients with beta thalassemia major and dilated cardiomyopathy. During a six-month period, fourteen patients with beta-thalassemia major and heart failure without diabetes mellitus referred to pediatric cardiology clinic enrolled in this double blind, randomly assigned study. All patients were on anti failure therapy with Digoxin, Captopril and Furosemide. Carvedilol was started at a dosage of 3.12 mg bid and for patients who had a systolic blood pressure >100 mmHg, heart rate >60/min and no signs of low cardiac output the dosage was increased every two weeks to a maximum of 25 mg bid. Clinical signs and symptoms, systolic and diastolic echocardiographic indexes and Tissue Doppler Imaging (TDI) data were collected from each patient. Eight patients received Carvedilol (Group 1) and six received placebo (Group 2). The mean age of patients in Group1 and 2 were 16±0.7 years and 17±3 years respectively. Only one patent in Group 1 tolerated increasing Carvedilol dosage to more than 6.25 mg bid. Changes in New York Heart Association (NYHA) classification, Ejection fraction, End diastolic dimension changes, TDI systolic(S), early (Ea) and late (Aa) diastolic waves were not statistically significant in these two Groups (P>0.05). Pulse Doppler E/A wave ratio of mitral valve in Group1 and Group 2 changed from 1.1±0.37 m/s to 1.8±0.40 m/s and from 1.34±0.30 m/s to 2.6±0.23m/s respectively (P=0.04). Patients with thalassemia and dilated cardiomyopathy have poor tolerance to increasing Carvedilol dosage and develop decreased systolic blood pressure during advancement of the drug dosage. Carvedilol can be effective in prevention of progression of diastolic dysfunction in these patients.

Prevalence of low birth weight deliveries may vary across different environments. The necessity of determination of regional data prompted this study. Information of all deliveries from January 2004 to December 2008 was obtained from delivery registry records retrospectively. Initial data including birth weight, vital status, sex, maternal age and mode of delivery were recorded using medical files. The frequency of low birth weight, very low birth weight, extremely low birth weight and stillbirth deliveries were determined. Among 19,533 total births, there were 450 (23.04 per 1000) stillbirths. Low birth weight rate was 10.61%. A significant increase in yearly distribution of low birth weight deliveries was observed (P<0.001). Very low birth weight and extremely low birth weight delivery rates were 3.14% and 1.58% respectively. Among 2073 low birth weight infants, 333 (16.06%) were stillbirths. The stillbirth delivery rate and the birth of a female infant among low birth weight deliveries were significantly higher than infants with birth weight ≥2500g (P<0.001, OR=28.37), (P<0.001) retrospectively. There was no statistical difference between low birth weight and maternal age. The rate of cesarean section among low birth weight infants was 49.4%. High low birth weight and stillbirth rates, as well as the increase in low birth weight deliveries over the past five years in this study are striking. For reduction of increased low birth weight rates, appropriate intervention methods should be initiated.

Functional constipation is a common and challenging problem in pediatrics. Fecal disimpaction prior to maintenance therapy is recommended to ensure successful treatment. The aim of this study was to compare the efficacy and patient's compliance of the two methods of paraffin oil administration (oral and rectal route) with the purpose of disimpaction in treatment of children with functional constipation. A total of 80 children (49 males and 31 females) aged 1-12 years, with functional constipation according to Rome III criteria, whose rectal examination confirmed fecal impaction were divided into two groups randomly. Group I received 3 ml/kg/day paraffin oil orally and group II received 3ml/kg/day paraffin oil rectally during 3 consequent days. Successful treatment was defined as no detectable fecal impaction in rectal examination after at most 72 hours. Patient compliance and family satisfaction also was evaluated using a scored questionnaire. Response to the treatment in both groups was with 92.5% and 82.5% in group I and II, respectively. So, there was no significant difference between the two methods of therapy. Family satisfying and compliance were obviously more achieved in group 1 (87.5% vs 57.5%) than in Group 2 (P<0.001). No parents in group I complained about type of treatment while 12.5% of parents in group II were unsatisfied with the mode of paraffin oil administration. The most common side effect of paraffin oil in both groups was anal oil seepage (27.5%). Nausea and abdominal pain were more common side effects in group 1 and 2 respectively. It seems that using paraffin oil per oral route in comparison with rectal route could be a preferred option for disimpaction in children causing less anxiety to the family.

There are some reports in which a condition of zinc deficiency and its associated outcomes with a change in concentration of serum copper among the thalassemic patients has been highlighted. The aim of this prospective study was to determine the serum zinc and copper levels in children with beta-thalassemia major. In this cross sectional study all children under 12 years affected by beta thalassemia major (40 patients) were evaluated for serum zinc and copper levels in Qazvin thalassemia center (Qazvin, Iran) in 2007. Serum measurements for zinc and copper were performed by atomic absorption spectrophotometer. The mean concentrations of serum zinc and copper levels were 67.35±20.38 and 152.42±24.17 μg/dl respectively. Twenty-six (65%) of thalassemic patients had zinc concentration under 70 µg/dl (hypozincemia). None of the thalassemic children had copper deficiency. No significant correlation between serum zinc level with age, weight, height, body mass index, duration of blood transfusion, desferrioxamine dose and ferritin level was observed in thalassemic patients (P=0.3). This study revealed that hypozincemia is common in thalassemic patients, but in contrast, there is no copper deficiency. Further evaluation in this regard is recommended.

Retinopathy of prematurity (ROP) is a serious complication in preterm infants. To avoid this complication the risk factors leading to the disabling disease should be evaluated and prevented. This is a descriptive study. All preterm infants with birth weight under 1500 g and preterm infants with birth weight between 1500-2000 g who had unstable clinical condition and admitted in neonatal intensive care unit from February 2006-March 2007 at tertiary hospitals of Shiraz University, Iran, were introduced into the study. All infants are examined by indirect ophthalmoscopy. Risk factors analysis was performed in two groups. Group 1 consisted of infants with no ROP or ROP that regressed spontaneously, and Group 2 of those with severe ROP that needed laser therapy. Of 199 preterms, ROP that needed laser therapy was detected in 19 (9.5%); 65 (32.6%) had ROP that regressed spontaneously and 115(57.8%) had no ROP. Risk factor analysis showed significant P-values for gestational age, birth weight, Apgar score of first minute, mean duration of mechanical ventilation, mean duration of oxygen therapy, eclampsia-preeclampsia, hypoxia, hyperoxia, Pa CO2 >60 mmHg, pH>7.45 and frequent blood transfusions. Using stepwise logistic forward regression showed the three factors mean duration of oxygen therapy, birth weight and mechanical ventilation to be independently significant variables for increasing the rate of ROP. The main risk factors for development of threshold ROP are low birth weight, mechanical ventilation and duration of oxygen therapy. So it seems that prevention of premature delivery and judicious oxygen therapy is the main step for prophylaxis of ROP.

The thalassemias are among the most common genetic disorders worldwide, occurring more frequently in the Mediterranean region. The aim of this study was to determined frequency of sensory-neural hearing loss in major ß- thalassemias transfusion dependent patients in south of Iran. A cross sectional study on 308 cases of major beta-thalassemia patients referring to Thalassemia Center of Shiraz University of Medical Sciences between 2006-2007 years. The diagnosis of ß- thalassemia major was based on clinical history, complete blood count and hemoglobine electrophoresis. Clinical data such as serum ferritin level, deferoxamine (DFO) dose, mean daily doses of DFO (mg/kg) and audiometric variables was recorded. Out of 308 cases, 283 (96.5%) had normal hearing and 10 (3.5%) sensorineural hearing loss. There was no statically significant difference between two groups regarding mean age, weight, age at the first blood transfusion, age at the first DFO infusion. We found the lowest incidence of sensorineural hearing loss in a large population of patients suffered from major thalassemia who received DFO. We show that DFO is not ototoxic at a low dose. When considering all related literature, as a whole there has been much critical misrepresentation about DFO ototoxicity.

This research was designed to identify the validity and reliability of the Persian version of Denver Developmental Screening Test II (DDST-II) in Iranian children, in order to provide an appropriate developmental screening tool for Iranian child health workers. At first a precise translation of test was done by three specialists in English literature and then it was revised by three pediatricians familiar with developmental domains. Then, DDST-II was performed on 221 children ranging from 0 to 6 years, in four Child Health Clinics, in north, south, east and west regions of Tehran city. In order to determine the agreement coefficient, these children were also evaluated by Ages and Stages Questionnaires (ASQ) test. Because ASQ is designed to use for 4-60 month- old children, children who were out of this rang were evaluated by developmental pediatricians. Available sampling was used. Obtained data was analyzed by SPSS software. Developmental disorders were observed in 34% of children who were examined by DDST-II, and in 12% of children who were examined by ASQ test. The estimated consistency coefficient between DDST-II and ASQ was 0.21, which is weak, and between DDST-II and the physician's examination was 0.44. The content validity of DDST-II was verified by reviewing books and journals, and by specialist's opinions. All of the questions in DDST-II had appropriate content validity, and there was no need to change them. Test-retest and Inter-rater methods were used in order to determine reliability of the test, by Cronbach's α and Kauder-Richardson coefficients. Kauder-Richardson coefficient for different developmental domains was between 61% and 74%, which is good. Cronbach's α coefficient and Kappa measure of agreement for test-retest were 92% and 87% and for Inter-rater 90% and 76%, respectively. Persian version of DDST-II has a good validity and reliability, and can be used as a screening tool for developmental screening of children in Tehran city.

The role of initial serum uric acid on admission in critically ill patients is controversial; we presumed that uric acid level can predict the mortality of the admitted patients to intensive care unit as a simple test.

This study aims to evaluate the role of breastfeeding in the acquisition of Helicobacter pylori (H. pylori) infection in Iran and to compare the histopathologic changes occurring in children feeding on breast milk with those in infants feeding on formula. In a case-control study parents of children with and without H. pylori infection who had undergone endoscopic survey and gastric biopsy in the Children's Medical Center, Tehran, were asked about their feeding practices during the first 6 months after birth, the duration of breastfeeding period, the symptoms, and the duration of symptoms and concomitant diseases. A total of 154 children were included in this study. From this sample, 77 children formed the case group and 77 children formed the control group. A significant difference was found between H. pylori infection and feeding with formula (P=0.045). In case group, a significant difference was found between breastfeeding and age of the infected child (P=0.034), shorter duration of symptoms (P=0.016), and finally degree of H. pylori colonization (P=0.021). It appears that breastfeeding in the first 6 months after birth can decrease the degree of H. pylori colonization, postpone infection until older age, shorten the duration of symptoms, and be concomitant with milder gastritis.

This paper presents the incidence rates of childhood cancers using the data obtained from Golestan population based cancer registry (GPCR) between 2004 and 2006. GPCR registers only primary cancers based on standard protocols of the international association of cancer registries (IACR). We collect data on newly diagnosed (incident) cancer cases from all public and private diagnostic and therapeutic centers of the whole province. CanReg-4 software was used for data entry and analysis. Totally 5076 cancer cases (all ages) were diagnosed in GPCR between 2004 and 2006. Of these, 139 (2.74 %) were children (aged 0-14 years) with mean (±SD) age of 8.06 (±4.48) years. The age standardized incidence rates for childhood cancer were 119.8 and 78.3 per 1000000 person-years in male and female children, respectively. Leukemia was the most common childhood cancer in Golestan province of Iran. Lymphomas and central nervous system tumors were the second and third ones, respectively. The incidence rates of childhood cancers were relatively high in Golestan province of Iran. So, controlling of childhood cancers should be mentioned as an important issue in health policy making in this area.

Cryptosporidiosis is an important enteric parasitic infection among infants and children in developing countries with significant morbidity and mortality especially among immuno-suppressed individuals. The aim of the present study was to estimate the prevalence of enteric cryptosporidiosis in children presenting with diarrhea in Isfahan. This cross-sectional study was conducted from August 2007 to June 2008 in three university hospitals in Isfahan. Children aged 1 month to 10 years presenting with acute or persistent diarrhea were selected consecutively. The oocyst of C. parvum was investigated in stool specimens using a modified acid-fast staining method. During the study period, 606 children (mean age 42.4±30.0 months, 58.1% female) were recruited. Acute and persistent diarrhea was present in 422 (69.6%) and 184 (30.4%) of the children, respectively. Twenty eight (4.6%) specimens were oocyst positive. The prevalence of Cryptosporidium infection was significantly higher in children with persistent diarrhea compared to children with acute diarrhea (12.5% vs 1.2%; P<0.001). Most of the infected children were under 5 years of age (89.2%), however, the age difference between infected and non-infected children was not statistically significant. Also, there was no significant difference between infected and non-infected children in gender. The prevalence of Cryptosporidiosis in children presenting with persistent diarrhea is considerable and we suggest routine stool examination for Cryptosporidium in this group of children.

Spina bifida occulta (SBO) has been largely considered a benign entity without clinical significance; however, there has been dispute among various authorities, and some believe that the lesion may be linked with various neurologic conditions like urologic dysfunctions. Vesicoureteral reflux (VUR) and lower urinary tract dysfunction are closely related. We examined whether the existence of SBO is related to the prevalence and severity of VUR in children. We investigated 359 children, 2-14 years old, referred to radiology department for obtaining voiding cystourethrogram after the first attack of febrile urinary tract infection. After treatment of infection, with written order of responsible physicians all underwent a standard voiding cystourethrogram to detect VUR and other lower urinary tract anomalies. The patients were divided into two groups: group1 patients who had not SBO and group 2 patients with SBO in postvoiding or KUB films. In each group the presence and severity of VUR was determined in relation to the location of SBO. Out of 359 children 228 (63.5%) had normal spine and 131 (36.5%) had SBO. 54 (23.7%) out of 228 children with normal spine had VUR and 40 (30.5%) out of 131 children with SBO had VUR. The prevalence of VUR in children without SBO and children with SBO was not statistically different. Also we compared the severity of VUR between the two groups and there was no significant difference or trend between presence of SBO and severity of reflux (Chi2 for trend). VUR was detected in 16.7% children with SBO in L5, 38.3% in L5-S1 and 26.6% in S1. There was no significant relation between location of SBO and prevalence of VUR. Location of SBO and prevalence of VUR are not related.

Diphallus is an extremely rare anomaly. Numerous associated genitourinary, gastrointestinal and other anomalies have been described with diphallus. These patients need several investigations, and finally surgical intervention.Cases Presentation: In this report we discuss six patients with diphallus which evaluated retrospectively. Five patients had complete diphallia, and one had bifid diphallus. Meatus was normal in 3, hypospadiac in 2, and epispadiac in one patient. The most common associated anomaly was bifid scrotum (5 cases), and other common anomalies consisted of bladder duplication (3 cases), imperforate anus (2 cases), and hypospadias (2 cases). Phalloplasty was performed for all but one. All the patients with urethral duplication have to be evaluated carefully because of the high incidence of other systemic anomalies.

The Ehlers-Danlos syndrome type VI (EDSVI) is an autosomal recessive connective tissue disease which is characterized by severe hypotonia at birth, progressive kyphoscoliosis, skin hyperelasticity and fragility, joint hypermobility and (sub-)luxations, microcornea, rupture of arteries and the eye globe, and osteopenia. The enzyme collagen lysyl hydroxylase (LH1) is deficient in these patients due to mutations in the PLOD1 gene. We report a 17-year-old boy, born to related parents, with severe kyphoscoliosis, scar formation, joint hypermobility and multiple dislocations, muscular weakness, rupture of an ocular globe, and a history of severe infantile hypotonia. EDS VI was suspected clinically and confirmed by an elevated ratio of urinary total lysyl pyridinoline to hydroxylysyl pyridinoline, abnormal electrophoretic mobility of the α-collagen chains, and mutation analysis. Because of the high rate of consanguineous marriages in Iran and, as a consequence thereof, an increased rate of autosomal recessive disorders, we urge physicians to consider EDS VI in the differential diagnosis of severe infantile hypotonia and muscular weakness, a disorder which can easily be confirmed by the analysis of urinary pyridinolines that is highly specific, sensitive, robust, fast, non-invasive, and inexpensive.

Familial Mediterranean Fever (FMF) is a genetic disease characterized by recurrent febrile attacks and inflammation of serous membranes. Amyloidosis is frequent in untreated FMF patients and is also the most important complication of FMF. It is generally seen with renal, hepatic, gastrointestinal, spleen, testicular and thyroidal involvement. Herein, we report a case with acute scrotum and hydrocele amyloidosis as a presenting finding in a child with FMF. Although the acute scrotum and scrotal swelling are not characteristic clinical features of FMF, this genetic disease should not be forgotten in the differential diagnosis of acute scrotum in patients of Mediterranean origins.

Left ventricle non-compaction (LVNC) or persistence of spongy myocardium is a rare form of congenital cardiomyopathy which presents with cardiac failure, thromboembolic events, arrhythmia and sudden death. We report a family with two deceased children and two alive offspring diagnosed with LV non-compaction. A 9-year-old boy and a 16-year-old girl of our reported family suddenly died following exercise and emotional provocation. Both had a history of convulsion and syncope, with the latter taking anticonvulsant medication. Following their demise, the other members of the family were evaluated by echocardiography. The remaining two offsprings, one boy and one girl were diagnosed as having isolated LVNC. Syncope and convulsion can be first manifestations of LVNC and heralding signs for sudden death in patients with LVNC. Echocardiography can be helpful for early diagnosis.