Iranian Journal of Pediatrics
Volume:19 Issue: 1, 2009

Nocturnal enuresis is a common diagnosis in patients referred to pediatric and pediatric nephrology clinics. Nocturnal polyuria is an important patho-physiologic factor in enuresis. Hypercalciuria, with altering concentrating capacity of the kidneys, can affect children''s response to desmopressin. This is a double blind clinical trial starting September 2007 to March 2008. One hundred and twenty four enuretic children, 76 (61.3%) males, 48 (38.7%) females, mean age 7.7 (±1.7), were evaluated by measuring random morning urinary calcium to creatinine ratio. Patients were divided into group 1 with a calcium to creatinine ratio equal to or more than 0.2 mg/mg, and group 2 with a ratio less than 0.2 (Hypercalciuric and non hypercalciuric respectively). All patients received 10 to 40 mcg of nasal desmopressin at bed time. The response was defined as reduction in wet nights, a "full response" (greater than 90 % reduction), "partial response" (50% to 90% reduction) and "no response" (less than 50% reduction). Chi-square method was used to compare the responses and P<0.05 was considered statistically significant. Nineteen patients in group 1 (Hypercalciuric) and 105 patients in group 2 (Non hypercalciuric) were studied. Response to desmopressin was "full" in 47.4% in group 1 and 64.8% in group 2. 42.1% and 26.7% had "Partial response" in group1 and 2 respectively (P<0.04). Hypercalciuria can affect negatively the responsiveness to desmopressin therapy

Determining the frequency of chromosome 22q11.2 microdeletion in children with congenital cardiac conotruncal abnormalities using Fluorescence in-situ Hybridization (FISH) technique and estimating relation between DiGeorge Syndrome and cardiac conotruncal abnormalities. One-hundred and eighty cases (106 Males, 74 Females) with selective congenital heart disease (conotruncal abnormalities) referred to the hospitals affiliated to Tehran University during 2004-2007 were evaluated by pediatric cardiologists. All patients were assessed for chromosome 22q11.2 microdeletion using FISH technique. Consequently, patients with 22q microdeletion were studied for T cell abnormalities. Median age of the patients at the time of study was 18 months (3d-16y). The microdeletion of chromosome 22q11.2 was detected in 17 (9.5%) patients with conotruncal abnormalities, including 5 (29.4%) Tetralogy of Fallot plus Supravalvular Pulmonary Stenosis, 4 (23%) Truncus Arteriosus, 5 (29.4%) Pulmonary Artesia with Ventricular Septal Defect, 2 (11.8%) CO AO+Intrrupted Aortic Arch and one case of Valvular Pulmonary Stenosis. Five of uncorrelated cases had crananiofacial dysmorphism. Chromosome 22q11.2 microdeletion FISH study should be considered in patients with cardiac lesions particularly conotruncal abnormality with or without syndromic problems (craniofacial dysmorphism and developmental delay) to provide an appropriate genetic counseling with more accurate estimation of recurrence risk and ultimately prenatal diagnosis in affected families.

Proper nutrition during pregnancy is essential for optimal fetal growth. Investigation of the relation between pregnancy weight gain and birth weight in rural regions of Rasht, center of Guilan Province in Iran, was the purpose of this study. In this cohort study, prenatal data of 918 women who attended local health centers with singleton term pregnancies were recorded. Maternal demographic characteristics, anthropometric measurements, total pregnancy weight gain and birth weight were recorded by health workers. The women were stratified based on their pre-pregnancy body mass index (BMI) into four groups: underweight women, women with normal weight, overweight women and obese women. The relation between weight gain and low birth weight (LBW, birth weight <2500 g) and macrosomia (birth weight >4000 g) was studied in these four groups. Data were analyzed using Chi-square test, independent t-test, Pearson correlation and logistic regression with 95% confidence intervals. More than 50% of underweight women and women with normal weight and almost 30% of overweight and obese women gained weight less than what is mentioned in the Institute of Medicine (IOM) recommendations. The incidence rate of LBW was 7.1% and that of macrosomia was 5%. Mean weight gain of women with LBW was significantly less than mean weight gain of women who had an infant with a birth weight more than 2500 g (P=0.002). Women who gained weight less than the recommended range had higher rate of LBW in their infants (P=0.01) and the incidence of macrosomia in women with a weight gain above the recommended weight was higher than that in others (P=0.012). Pregnancy weight gain less than what is mentioned in the IOM guideline was the only predictor for LBW (OR=2.79, CI=1.16-6.73, P=0.02). Pregnancy weight gains less than what is mentioned in the IOM recommendation was a significant predictor of LBW, regardless of pre-pregnancy BMI.

This study was performed to provide the curves of weight and height of 0-2 year-old children in Jahrom, Southeastern Iran. In a prospective study, 597 children born in Jahrom entered our study from April 2001 to December 2002. The height and weight of these children were recorded 18 times from one month to two years of age. Healy-Rasbash-Yang (HRY) method was used to estimate age related smoothed centiles. This method was implemented for the World Health Organisation as GROSTAT computer package. The mean weight of male newborns, except for one-month olds, was more than that of females. Before two months of age and among 2-year-olds, there was no significant difference between the weight of males and females. The mean height of male newborns, except for one month of age, was more than that of females. No more than cubic polynomials were needed to smoothly fit height for age and weight for age of children. The height of male and female children showed an increase by age up to two years. The weight of male and female children also increased by age but the speed of increase to six months was more than that of six months to two years of age. Our findings show that height and weight of children under two years of age in Jahrom were lower than those of National Center for Health Statistics (NCHS). It is therefore deemed necessary to provide and establish a corresponding standard curve for this region. According to the NCHS measurements, some children may be considered thinner or shorter for no obvious reason.

Short stature is a common problem encountered by pediatricians and is the most common cause for referral to pediatric endocrinologists. Although most children referred with short stature are normal and classified as normal variants of stature (constitutional growth delay and familial short stature), it may sometimes be the only obvious manifestation of an endocrine or systemic disease. The objective of this study was to assess the characteristics of patients referred to pediatric endocrinology clinic because of short stature and determination of the etiology. Three hundred-seventy nine children and adolescents were studied which referred with short stature to pediatric endocrinology clinic. After complete clinical and paraclinical evaluation and appropriate treatment (if needed), patients were followed for at least six months. From 379 studied patients with a mean age of 9.7±3.7 years, 192 (50.7%) were girls and 187 (49.3%) boys (P=0.066); short stature in 132 (34.8%) of patients was not approved. Normal variants of Short stature (familial and constitutional) constituted 53.3% of etiology in short patients. In 11.5% of short patients, no obvious etiology was found, and 9.8% were born with intra uterine growth retardation. Other causes were growth hormone deficiency, hypothyroidism, skeletal dysphasia, Turner syndrome, and malnutrition. A great number of children and adolescents referred with short stature to pediatric endocrinology clinics are not really short. Greater than half of short patients are normal variants of Short stature.

An immune response to heat shock proteins appears to be involved in atherogenesis. To date, there has been no report on the impact of dairy or calcium consumption on serum antibody titers to heat shock protein 27 (anti-HSP27). We have investigated whether an increase in dairy food consumption is capable of affecting serum antibody titers to heat shock protein 27 (anti-HSP27) level in children. Overweight and obese children (n=99, age: 12-18 y, body mass index: 27-40 kg/m2) were randomized to receive a calorie restricted diet providing a 500 kcal/d deficit from total energy expenditure and two (n=38), three (n=26) or four (n=35) servings of dairy products/day. Serum anti-HSP27 level in addition to the serum hs-CRP and lipid profile were measured at baseline and after 12 weeks. Serum anti-HSP27 concentrations did not change significantly in any of the mentioned groups. Serum hs-CRP and lipid profile did not change significantly either, apart from a significant increase in HDL-cholesterol in the low-dairy group. An increased intake of dairy products does not lead to a significant change in serum anti-HSP27 level in overweight and obese children.

Exercise-induced bronchospasm (EIB) is a common condition in elite athletes. The purpose of this study was to evaluate the diagnostic value of self-reported EIB symptoms in children. In a cross sectional study in 2005, all soccer player boys of 3 football schools of Shahr Rey a town in the south of Tehran, enrolled in this study. All subjects were asked for the presence of four cardinal symptoms of EIB (cough, wheeze, shortness of breath, chest pain/discomfort) during and after exercise. Self reported symptom-based EIB was defined as having at least two out of four symptoms. Findings were compared to spirometric criteria as a gold standard test. A total of 371 boys were enrolled in this study. The mean age of children was 11.67 ± 1.53 years (range 7-16 years). According to spirometric findings, 74 (19.9 %) subjects had EIB. The sensitivity and specificity of self-reported symptoms for EIB diagnosis were 13.0 % and 89.9 %, respectively. Self-reported symptoms of EIB in children can be useful for epidemiological study. Our results in Iran are comparable with studies in other countries and point to a relatively high prevalence of EIB among athlete children.

The aim of this study was to evaluate the electrophysiologic findings of Guillain Barre Syndrome (GBS) in children and their relation with clinical progress of the disease. Twenty-three children with GBS were evaluated between 2005 and 2007. Electrophysiologic evaluations were performed at admission and one month later. Five patients needed respirator, 15 were bedridden, 1 developed recurrence 6 months later, and 2 experienced chronic GBS. The most common findings included: decreased amplitude of muscle action potential (CMAP) (96%), increased distal latency (74%), increased F wave latency (69%), and decreased nerve conduction velocity (NCV) (61%). Sensory nerve conduction (evaluating sural nerve) was normal in 78% of the cases. These measures did not significantly change after 1 month. Electrodiagnostic evaluations are helpful at the primary stages of GBS for diagnosis. Fibrillation potentials and positive sharp waves showing denervation and axonal injury are presentative of longer duration of the disease and a worse prognosis.

Retinopathy of prematurity (ROP) is a disease of the eye caused by disorganized growth of retinal blood vessels resulting in scarring and retinal detachment. All preterm babies are at high risk for ROP, and very low birth weight is an additional risk factor. An increased incidence of ROP is expected in Iran because of improved survival of low birth weight and premature babies, and it is obvious that pediatricians and ophthalmologists are concerned about prevention and timely treatment of ROP in these children. To asses the real situation of ROP in our NICU we studied its prevalence and risk factors. This was a retrospective analysis of premature infants with birth weight of ≤1500 grams or gestational age of ≤32 weeks, admitted April 1, 2005 to March 28, 2006, to the Neonatal Intensive Care Unit of Qaem Hospital, Mashhad, Iran. The collected data of 47 cases in this cross-sectional study are analyzed by SPSS (Mann_Whitney, t-Student). Forty five infants were included in the study. Out of these, 4 (8.5%) developed ROP (inclusive all stages). Our analysis revealed that low gestational age, sepsis and respiratory distress syndrome were independent predictors for the development of ROP. The frequency of ROP in our hospital was lower than the range reported in developed countries, and our risk factors were a little different.

Febrile convulsion is the most common disorder in childhood with good prognosis. There are different hypotheses about neurotransmitters and trace element (such as zinc) changes in cerebrospinal fluid and serum, which can have a role in pathogenesis of febrile convulsion. The aim of the present prospective analytical case-control study was to determine whether there was any changes in serum zinc level in children with febrile convulsion during seizure. Ninety-two children aged 6 months to 5 years were divided into three groups: group A, 34 children with febrile convulsion, group B, 40 children having fever without convulsion, and group C, and 18 children with non-febrile convulsion. Serum zinc levels for the three groups were estimated by atomic absorption spectrophotometry (AAS). Data were analyzed to compare zinc level among the three groups using appropriate statistical tools employing SPSS 13. Serum zinc levels of groups A, B, and C had a mean value of 76.82±24.36mg/1, 90.12±14.63 mg/1 and 94.53±17.39 mg/l, respectively. Serum zinc level of group A was lower than those of the other two groups (P<0.006). It was also lower in group B than in group C (P<0.006). These findings revealed that serum zinc level decreases during infection; this decrease was more significant in patients with febrile convulsion.

Reversible posterior leukoencephalopathy syndrome (RPLE) is an increasingly recognized disorder, most commonly associated with immunosuppressive and cytotoxic agent, malignant hypertension and toxemia of pregnancy. Neurological symptoms are headache, mental and visual disturbance, and seizures. Brain MRI findings are symmetrical bilateral hyper intense areas of occipital and parietal lobes in T2–weighted magnetic resonance (MR) images. This finding is reversible with prompt successful treatment which may take days to weeks for full reversal. We observed a case of status epilepticus which occurred 32 days after liver transplantation in a patient on regimen consisting of Tacrolimus (FK 506). He had no history of neurologic disease or metabolic abnormalities. Typical findings of RPLE were present on brain MRI. The recurrence of seizures terminated after a few hours with adding Na-valproate to Phenytoin, Phenobarbital and Midazolam. The radiological finding of brain MRI gradually diminished after several weeks. A physician should be alert of seizures during early post transplant period of liver transplantation. He should bear in mind the diagnosis of RPLE and consider discontinuation of immunosuppressive drugs.

Copper sulfate is a blue and odorless salt with various industrial, chemical, agricultural and medicinal applications. Copper sulfate poisoning is rare in children. A 23-month old boy accidentally ingested a solution of copper sulfate, used as a disinfectant agent in animal husbandry. He was referred to Children’s Hospital of Tabriz because of frequent vomiting and lethargy. The major systemic complications were intravascular hemolysis, anemia and acute renal failure. The patient was successfully treated with multiple packed cell transfusions, dimercaprol, penicillamine and peritoneal dialysis. Plasma copper level 15 days after ingestion was 216 μg/dl. Copper sulfate is a highly toxic agent that, when ingested, can cause local and systemic damage including coma, shock, severe intravascular hemolysis, hepatotoxicity and acute renal failure with high mortality.

Ours is a developing country so infectious diseases contribute maximum to the morbidity and mortality. Among these, water borne diseases like diarrhea, typhoid, infectious hepatitis etc. are on rise. Sometimes more than one type of infection coexists which makes the diagnosis and management a challenging task. We report a case of Coinfection of Salmonella typhi with Hepatitis A and E. A 5 year old male child came to us with complaints of fever and jaundice for last 9 days. Blood culture of patient was positive for Salmonella typhi. Viral markers turned out to be positive for Hepatitis A and E. To the best of our knowledge coinfection of Hepatitis A and E with Salmonella has rarely been reported earlier. In view of the restricted finances in our country vaccines against typhoid and Hepatitis A can not be incorporated in the national immunization schedule at present but these vaccines can be offered on an individual basis.