Iranian Journal of Pediatrics
Volume:19 Issue: 3, 2009

Juvenile myelomonocytic leukemia (JMML) is a rare myeloproliferative disorder of childhood. It was recently demonstrated that mononuclear cells from spleen, peripheral blood and bone marrow of the patients with JMML proliferate in vitro and population of the cells differentiate within 7 days to the CD1a positive dendritic cells. In the present study, the role of GM-CSF in the proliferation and differentiation of the JMML cells in vitro was investigated. MNC from patients with JMML was cultured for 7 days in RPMI/10%FCS and 1% penicillin-streptomycin. The phenotype of the cultured cells was determined at day 7 using antibodies against Ki-67, CD1a, CD14 and CD68. GM-CSF concentration was first measured at days 1, 3, 5 and 7 in the supernatants of the cultured mononuclear cells from two patients with JMML. To study the roll of GM-CSF in JMML cultures, GM-CSF activity was neutralized using monoclonal antibody (Ab) against human GM-CSF in different concentrations (0.05 µg/ml, 0.5 µg/ml, 5 µg/ml and 10 µg/ml). The effect of neutralizing antibody was determined at day 7 in treated and untreated cultures by immunostaining using antibodies against Ki-67, CD68, CD1a and CD14. The expression of GM-CSF, GM-CSFR, TNF-α, IFN-γ and SCF was also determined at day 7 in mononuclear cells treated and untreated cultures by RT-PCR. GM-CSF neutralization led to decrease of the total cell number. Additionally, neutralization of GM-CSF decreased in dose dependent manner the number of CD1a, CD14 and Ki-67 positive cells in JMML cultures, whereas the percentage of CD68 remained unchanged. The expression of GM-CSF, GM-CSFR, TNF-α and SCF could be demonstrated at the mRNA level in the antibody treated and untreated culture as well. GM-CSF plays an important role in the proliferation and differentiation of JMML cells into the CD1a and CD14 positive cells.

A paucity of data exists regarding the relation of mean right atrial pressure (RAP) to Doppler parameters of right atrial and ventricular filling in pediatric patients with congenital heart disease. Fifty patients (30 male and 20 female) with mean age of 4.96±4.05 who were admitted in the pediatric cardiology ward of Nemazee Hospital affiliated to Shiraz University of Medical Sciences, were included in this study. Patients were categorized into two groups according to their RAP measured by cardiac catheterization: Group 1 (40 patients) were those with mean RAP <8 mmHg and group 2 (10 patients) who had mean RAP ³8 mmHg. Data gathered from hepatic venous flow, tricuspid diastolic flow and pulse tissue Doppler of lateral tricuspid annulus of each patient were then compared with right atrial pressure obtained by cardiac catheterization. If change of peak S wave velocity of hepatic vein in respiration was more than 38%, sensitivity and specificity of a RAP more than 8 mmHg was 90% and 51.3% respectively with likelihood ratio (LR) equal to 1.85; a peak S wave velocity of less than 70 mm/sec also showed a RAP more than 8 mmHg with sensitivity and specificity of 70 and 82.1 respectively (LR=3.9). A peak expiratory D wave velocity of hepatic vein more than 63 mm/sec was indicator of RAP more than 8 mmHg with sensitivity and specificity of 60% and 92.3% respectively (LR=7.8). This study showed that hepatic venous flow can be valuable for estimation of mean RAP in pediatric patients with congenital heart disease.

Iron deficiency anemia (IDA) resulting from lack of sufficient iron for synthesis of hemoglobin is most common hematologic disease of infancy and childhood. There is little information about the renal function in iron deficiency anemia. The aim of the present study was to examine the renal function in children with iron deficiency anemia. This case-control study was performed in children with iron deficiency anemia (n=20) and healthy age matched subjects (n=20). Blood and urine samples were obtained for hematological and biochemical investigation. Statistical analysis was performed by unpaired sample t-test and Pearson''s correlation coefficient. Fractional excretion of sodium (FENa+) was significantly higher in children with iron deficiency anemia than control subjects (P=0.006). There was no significant difference in the level of urine specific gravity, calcium/creatinine ratio, β2-microglobulin and creatinine clearance between case and control groups (P>0.05). There was no correlation between FENa and Hb (r=0.13, P=0.57), FENa+ and Fe+2 (r=0.079, P=0.72), FENa+ and TIBC (r=-0.083, P=0.71), FENa+ and ferritin (r=0.039, P=0.86) as well as Hb and β2-microglobulin (r=0.02, P=0.37) in IDA patients. The results showed that FENa+ was higher in children with iron deficiency anemia than in normal subjects. The findings revealed evidence of tubular damage in IDA; this needs confirmation by more investigation.

Under-nutrition continues to be a primary cause of ill-health and premature mortality among children in developing countries. Present study attempted to assess the overall prevalence of undernutrition using composite index of anthropometric failure (CIAF) among rural preschool children of Arambag, Hooghly District, West Bengal, India. A total of 1012 rural children (498 boys; 514 girls) aged 2-6 years were measured. Three commonly used undernutrition indicators, ie, stunting, wasting and underweight, as well as the Composite Index of Anthropometric Failure (CIAF) were used to evaluate the nutritional status of the subjects following internationally accepted cut-off points. For assessing CIAF, Svedberg''s model of six groups of children was used. Among the studied children, 26.6%, 50.0% and 63.3% were stunted, wasted and under weight, respectively. The CIAF showed a higher prevalence of undernutrition of 73.1% of the studied children suffering from anthropometric failure, in comparison to other three indicators (stunting, underweight and wasting). Out of six subgroups of CIAF with undernourished children, group C (containing children who are wasted and underweight) is the highest (32.0%), more-or less one-third of the studied children and children of group F (stunted only) is the lowest (2.7%). Group B (wasting only) and Y (underweight only) show the same prevalence ie 7.2% and 7.1% respectively. Children who simultaneously have wasting, stunting and underweight (ie group D) account for 10.7%. There was no significant age group difference between no failure and CIAF. We found that CIAF may be a better indicator of nutritional stress as compared to the traditional measures of stunting, underweight and wasting. The distinct advantage of the CIAF may be that it can highlight the seriousness and severity of overall undernutrition ie the actual load of undernutrition in a population.

Helicobacter pylori (H. Pylori) is now recognized as a major etiological factor in the pathogenesis of gastritis and peptic ulcer disease. There is concrete evidence that eradication of the bacterium reverses histological gastritis, and results in significant reduction of duodenal and gastric ulcer recurrence. Poor compliance and antibiotic resistance are the main causes for failure of anti H. pylori therapy. In this study we determined efficacy of omeprazole based triple therapy with b.i.d. dosing of furazolidone, amoxicillin for 2 weeks and omeprazole in Iranian children. This prospective study included 37 children, in whom H. Pylori infection was diagnosed endoscopically. H.Pylori positive children were treated with a two weeks course of furazoidone (6 mg/kg/day) and amoxicillin (50 mg/kg/day) plus omeprazole (1-2 mg/kg/day). Eradication was assessed by 13C UBT. Mean age of patients was 10.2 yr (5-15 yr), 25 (67.5%) patients were boys. H. Pylori was eradicated in 34 children (per patient 91.9%, per protocol 86%). Side effects occurred in 3 (8.1%) patients, but these were mild and it was not necessary to discontinue treatment. Three children (8.1%) remained H. pylori positive. Our study showed that the association of furazolidone plus amoxicillin with a proton-pump inhibitor could be a valuable alternative for eradication of H. Pylori infection in children. It is an effective, affordable treatment that allows good compliance and produces low adverse effect rates.

Zinc deficiency is common in developing countries and can affect growth of children. The objective of this study was to determine the prevalence of zinc deficiency and its effect on growth in elementary school children of South Khorasan province. This cross-sectional study was carried out on 908 9-11 year-old school children of South Khorasan province (East Iran) from October to December 2007. The subjects were chosen by multi-stage cluster random sampling method from urban and rural areas. Anthropometric measurements were made following standard techniques. Children were considered Stunting, underweight and wasting as height-for-age and weight-for-age and weight-for-height Z-score below or equal -2 standard deviation using the National Center for Health Statistics (NCHS) reference population. Serum zinc level was measured by atomic absorption spectrometry. Statistical analysis was performed by the SPSS statistical package using t-test, Chi-square and ANOVA. Totally, 474 subjects (52.2%) were girls and 697 subjects (76.8%) resided in city. The prevalence of stunting, underweight and wasting was 13.2%, 6.8% and 5.5%, respectively. The mean value of zinc plasma level was 87.7 (±32.7) μg/dl; it was significantly higher in girls and in urban areas. There were no significant differences in mean serum zinc in school children with normal growth and subjects that were stunted, underweight or wasted. The prevalence of zinc deficiency was 28.1% and it was significantly higher in rural areas. The prevalence of zinc-deficiency was significantly higher in stunted school children than non-stunted children. Zinc deficiency is common in elementary school children of South Khorasan province, so searching studies for underlying factors of zinc deficiency and supplementation of zinc, especially in stunted students is recommended.

Chronic constipation is a common elimination dysfunction in children. It can be treated with several drugs of different efficacy in different age groups. This study assessed the efficacy of Polyethylene glycol (PEG) and oral liquid paraffin in treatment of childhood chronic functional constipation. A total of 126 functional constipated children aged 1 to 15 years were divided into two therapeutic groups using systematic and random sampling technique. In addition, children were divided into three age groups of 12 to 23 months, 24 to 59 months and over 60 months old. They were scored based on five main criteria as follow: stool frequency per week, painful defecation, blood-stained stools, stool consistency and number of encopresis incidents per month. At the end of one therapeutic month, the scores obtained by drugs were compared with each other. Data were analyzed by using SPSS software, McNamara test, sign test, independent t test, and paired t-test. The scoring in PEG group was increased from 13.13 (±2.18) to 17.20 (±2.07) while in paraffin group, it increased from 13.48 (±1.90) to 16.78 (±2.51). Comparison of the mean scores showed a significant difference of the two groups after one month (P=0.02). Our results have shown that PEG is more effective than oral liquid paraffin for treatment of childhood functional constipation. Therapeutic response to the drugs varied among the different age groups.

It is reported that some inflammatory markers are predictive factors for atherosclerosis in childhood and adolescence and cardiovascular disease in adulthood. We investigated whether markers of inflammation including: cytokine [Interleukin-6 (IL-6)]، acute-phase reactant [C-reactive protein (CRP)]، white blood cell (WBC) count and its subgroups are associated with maximal oxygen consumption (VO2max) in overweight and normal children. Subjects were 26 boys aged 11-14 years included in two groups of overweight (n=10) and normal weight (n=16) children. VO2max was measured employing an incremental graded exercise test. IL-6 and CRP levels as well as WBC count were measured. Multivariable regression was employed to evaluate whether inflammatory markers were associated with VO2max. Mean VO2max for all subjects (n=26) was 36. 35±10. 42 ml/kg/min. This rate was lesser for overweight subjects (25. 77±5. 04) than in normal weight children (41. 54±5. 96). Log IL-6، log CRP، and WBC count were correlated with VO2max. Also subgroups of WBC including Leukocytes، Lymphocyte، Neutrophils، Monocytes and Eosinophils associated with VO2max. IL-6، CRP and WBCs were inversely associated with aerobic or cardiorespiratory fitness levels measured by VO2max in children. This was independent of BMI of the subjects.

Studies demonstrated that 5-10% of preschool children have visual impairment. By age seven، up to 13% of children will have some defect in visual acuity. Both prematurity and low birth weight have been associated with an increased incidence of ophthalmic disorders. In this study we determined prevalence of visual impairment in low birth weight and normal birth weight school age children in Mashhad. This is a cross sectional study. The target population consisted of all children referred to educational organizations for screening before entering school in Mashhad، Iran. 2400 children enrolled in the study and were evaluated for amblyopia، refractive errors، color vision disturbance and optic nerve problems. Data were analyzed by SPSS. Prevalence of ophthalmic problems in all children was 5. 43% and in low birth weight and normal birth weight 8. 29% and 5. 74% respectively. Incidence of ophthalmic problems was significantly (P=0. 029) higher in low birth weight children than in normal birth weight children. The most common ophthalmic disease in both low birth weight and normal birth weight children was refractive errors 81. 5% vs. 68. 8 % (P<0. 05). Prevalence of myopia، amblyopia and color vision disturbance was also higher in low birth weight than in normal birth weight children. Low birth weight children are at greater risk of the visual impairment that may occur at an early age and result in long term morbidity. Visual outcome of low birth weight neonates should be evaluated routinely.

This study aimed at assessing the putative association between the fingertip patterns of right and left digits II and intellectual functioning. The study involves the evaluation of dermatoglyphic patterns on right and left digits II in 342 adolescents (144 talented ones، 102 normal individuals and 96 subjects with learning disabilities) from the Shahrekord city in Iran. Comparisons between the frequencies of fingerprint patterns of each digit were made on the basis of two groups at a time employing Chi-square test. The most frequent dermatoglyphic pattern was whorl on both fingers in the 3 groups. An observation of right digit II revealed that the normal adolescents in comparison to the talented ones had a greater number of the whorl patterns (P=0. 02)، while the latter had more ulnar loops than the former (P=0. 09). Group comprising those with learning disabilities had more ulnar loops than the group composed of the normal adolescents (P=0. 09)، and there was a predominance of radial loops among the talented subjects as opposed to those among the individuals with learning disabilities (P=0. 002). There was no significant association in the relative frequencies of different finger patterns on left digit II between the groups (P>0. 05). Our results support an association between some dermatoglyphic patterns observed on right digit II with IQ level in adolescents. Further researches، needless to say، especially employing various quantitative dermatoglyphic indices and larger-sized samples are recommended.

Nutrition of the early childhood is of paramount importance because the foundation for life time strength and intellectual vitality is laid during this period. The present study aims to evaluate the effect of weaning biscuits supplementation of the nutritional parameters and cognitive performance of the selected children. Three Balwadies situated in Salem District، Tamilnadu، India were selected. A total number of 40 school children in Grade II malnutrition، 15 from Balwadi I، 14 from Balwadi II and 11 from Balwadi III comprised the study sample. All the 40 were selected for the experimental study. Home diet without any supplementation was followed by Group I (n=10، control group)، potato flour biscuit was supplemented to Group II (n=10)، Maize biscuits were given to Group III (n=10) and Green gram biscuits were given to Group IV (n=10) for the period of 3 months. Parameters like anthropometric measurements، hemoglobin content and clinical picture were analyzed before and after supplementation، cognitive performance of the supplemented children was assessed at the end of the study period. There was significant difference in height، weight، blood hemoglobin and clinical picture after three months on their home diet in group I. In groups II، III and IV significant increase in all the above parameters was noticed. More increase was found in group II children supplemented with potato flour biscuits for a period of 3 months. About cognitive performance better results were obtained in Group II followed by group IV (supplemented with green gram biscuits) and group III (supplemented with maize biscuits). Least was obtained by control group children who were in their home diet. All these observations evidence that if such weaning biscuits made with potato flour، maize and green gram can form a daily ingredient in their diets، it will bring out better all round development of the children.

Methicillin resistant staphylococcus aureus (MRSA) is a frequent cause of infections in children. The purpose of this study was to determine the frequency of nasal colonization of S. aureus in children and detection of inducible clindamycin resistance (ICR) by disk approximation test (D-test). This was a cross-sectional study conducted in Hamedan from 2007 to 2008. 520 nasal swabs were obtained from children under 12 years of age at the time of admission and 287 swabs at the time of discharge. Antibiogram was performed by method of disk diffusion for oxacillin، erythromycin، clindamycin، cefazolin and vancomycin as well as D-test. Chi-square test was applied for statistical analysis. Out of 520 patients، 118 (22. 3%) were colonized with S. aureus as community-acquired (CA-S. aureus). Of 287 patients، 64 (22. 3%) were colonized with isolates of S. aureus at discharge time. Of these 64 patients، 32 cases were colonized with hospital acquired (HA-S. aureus) isolates after admission. Only one CA-MRSA isolate was resistant to clindamycin، 5% of 118 CA-S. aureus isolates and 6. 3% of HA-S. aureus isolates had inducible clindamycin resistance (D-test). Also 37. 5% of CA-MRSA isolates at the time of admission and 22. 2% of HA-MRSA isolates at discharge had positive D-test. We emphasize that D-test should be used routinely and clindamycin should not be used in patients with infections caused by inducible resistant S. aureus.

The name ichthyosis is derived from the Greek ikhthus meaning "fish" and refers to the similarity in appearance of the skin to fish scale. The ichthyoses are a heterogeneous group of disorders. There are few studies about the oral manifestations of these disorders. But early reports of ichthyosis in the Indian and Chinese literature date back to several hundred years. Oral manifestations of the 14-year-old female patient with ichthyosis are presented. Physical examination revealed thick, brownish scales covering the entire body surface including all larger body flexures and corneae. She had short and dry hair. There were no nail abnormalities and hearing loss. We consider that this patient represents a new manifestation of lamellar ichthyosis disease, because congenitally teeth missing and cephalometric analysis measurements have not been reported before.

Jejuno-ileal atresia (JIA) is a congenital anomaly characterized clinically by bilious vomiting and abdominal distension. The incidence of JIA is between 1: 330 to 1: 3000 live births in different parts of the world. It has been associated with various congenital anomalies but the association of JIA with biliary atresia is extremely rare (0-3.2 %). We herein present a case of jejunal atresia with meconium peritonitis associated with biliary atresia. The patient was a boy who was born at 39 weeks of gestation with polyhydramnious detected on Prenatal Ultra Sonography done at 8th month of gestation. It is important to explore gallbladder in cases of JIA especially when associated with meconium peritonitis.

Takayasu arteritis (TA) or giant cell vasculitis is the third common vasculitis after Henoch-Schoenline purpura and Kawasaki disease in children. This vasculitis usually affects large vessels and is more common in females during the childhood. Aim of this report is to present three Iranian children (two boys, one girl) with TA. Mean age of our cases on admission was 10 years. Patients most commonly presented with episodes of systemic symptoms including, fever, headache, increased arterial blood pressure in one limb and convulsion. Other related symptoms were myalgia, limb pain, chest pain, and abdominal pain. Supra sternal, and abdominal bruit was noted in all patients. Angiography was performed in all cases. This revealed stenosis of the left subclavian artery and common carotid artery in one patient, generalized aortitis in one case and stenotic right renal artery in one patient. One boy was diagnosed as having systemic onset of juvenile idiopathic arthritis. One case was referred with hand pain and headache, and one case for control of hypertension. Follow-up ranged from 2 to 7 years since diagnosis. All patients were treated with prednisone, azathioprine, aspirin, and antihypertensive drugs. Each patient received either methotrexate, or hydroxychloroquine or mycofenolate mofetil. Although TA is uncommon before 10th year of life, it should be considered in patients presenting with hypertension and systemic symptoms such as fever, limb pain and pulseless limb.

Peutz-Jeghers syndrome is a rare hereditary disorder characterized by hamartomatous polyps in the gastrointestinal tract and typical pigment lesions. It is a rare cause of multiple intussusceptions. Previous studies on Peutz-Jeghers syndrome reported only one case of multiple intussusceptions. We describe a case of appendiceal and multiple small intestine intussusceptions presenting as peritonitis in a patient with Peutz-Jeghers syndrome. A 17-year-old girl presented with an 8 day history of a sharp, non-radiating periumbilical pain. She underwent surgery with the diagnosis of peritonitis. Intraoperative findings included appendiceal and multiple small intestine intussusceptions. The final pathological evaluation of the specimen confirmed the diagnosis of Peutz-Jeghers syndrome. Multiple intussusceptions may occur as the primary manifestation of Peutz-Jeghers syndrome. Because of its complications, in view of the presence of multiple polyps, early intervention is strongly recommended.

Giant coronary artery aneurysms caused by Kawasaki disease are rare; however, they are one of the most serious complications and can be lethal. We report a 3.5-month-old boy referred to us because of high fever for fifteen days, generalized maculopapular rash, irritability and cough. Transthoracic echocardiography showed dilatation of right coronary (RCA) and left main coronary (LCA) arteries. Serial echocardiography revealed rapidly progressive dilatation of coronary artery aneurysms of RCA and LCA. We performed invasive cardiac catheterization with selective coronary angiography when the boy was 16 months old. Selective right and left coronary arteriography showed a super giant fusiform aneurysm of RCA and a diffuse giant aneurysm of the proximal LCA. Regression of coronary artery aneurysms was not observed during 6 years of follow up. Pediatricians should be alert for possibility of incomplete Kawasaki disease in young infants with atypical presentation. They are at higher risk of coronary aneurysm formation. The diagnosis often was late with higher complication rate of coronary aneurysm. Echocardiography is an important tool for diagnosis of incomplete Kawasaki disease. Selective coronary angiography is the gold standard for diagnosis, and estimation of shape and size of aneurysms.