Iranian Journal of Pediatrics
Volume:20 Issue: 1, 2010

Febrile seizures (FS) are the most common form of convulsive phenomena in human being and affect 2% to 14% of children. It is the most common type of seizures that every pediatrician is dealing with. It is the most benign type of all seizures occurring in childhood. There are many debates on how to approach to febrile seizures in pediatric neurology and there are many possible malpractices in this field. Some of the most common frequent queries are- How could we differentiate FS from seizures and fever associated with serious infections involving the central nervous system?- When should we refer the affected child for further investigations such as lumbar puncture, EEG, neuroimaging, and routine biochemical studies?- How should we treat FS in its acute phase?- How could we assess the risk for further recurrences as well as other risks threatening the child''s health in future?- How could we select the patients for treatment or prophylaxis?- Which medication(s) should be selected for treatment or prophylaxis?Trying to answer the above-mentioned questions, this review article will present a four steps algorithmic clinical approach model to a child with febrile seizures based on the current medical literature.

Pediatric immunology came into sight in the second half of 20th century, when pediatricians and basic immunologists began to give attention to diagnosis and treatment of children with primary immunodeficiency diseases (PIDs). Understanding the genetic and mechanistic basis of PIDs provides unique insight into the functioning of the immune system. By progress in basic and clinical immunology, many infrastructural organizations and academic centers have been established in many countries worldwide to focus on training and research on the immune system and related disorders. Along with progress in basic and clinical immunology in the world, pediatric immunology had a good progress in Iran during the last 33-year period. Now, patients with PIDs can benefit from multidisciplinary comprehensive care, which is provided by clinical immunologists in collaboration with other specialists. Patients with history of recurrent and/or chronic infections suggestive of PIDs are evaluated by standard and research-based testing and receive appropriate treatment. The progress in PIDs can be described in three periods. Development of training program for clinical fellowship in allergy and immunology, multidisciplinary and international collaborative projects, primary immunodeficiency diseases textbooks, meetings on immunodeficiency disorders, improvement in diagnosis and treatment, and construction of Iranian primary immunodeficiency association, Students'' research group for immunodeficiencies, Iranian primary immunodeficiency registry, and the immunological societies and centers were the main activities on PIDs during these years. In this article, we review the growth of modern pediatric immunology and PIDs status in Iran.

Hypoxic ischemic encephalopathy (HIE) is a major cause of permanent neurological disabilities. Perinatal asphyxia may induce neonatal mortality after birth or neurological impairment among survivors. There are no reliable methods for identifying infants at risk for this disorder. We measured the ratio of lactate/creatinine (L/C) in urine by proton nuclear magnetic resonance spectroscopy within 6 and 24 hours after birth in 50 normal infants and 50 infants with asphyxia who developed hypoxic-ischemic encephalopathy. The study was performed from September 2006 to May 2007. For statistical analysis, the SPSS software was used. Group comparisons were performed with chi-square and t-test(1,5). L/C ratio was 3.3+ 2 among asphyxiated neonates in the first six hours after birth which was 11 folds greater than in normal neonates (0.3+ 0.08, P=0.0001). This ratio decreased to 1.5±0.55 for asphyxiated cases in the first 24 hours after birth, which was 5 folds greater than in control group (P=0.0001). Asphyxiated neonates were subdivided into Group A with mild asphyxia and L/C ratio 2.5±0.5; Group B with moderate asphyxia and L/C ratio 4.2±1.5; and Group C with severe asphyxia and L/C ratio 3.4±3.3. The severity of asphyxia correlated with the greater L/C ratio among our cases and was significant (P=0.0007). The sensitivity and specificity of L/C ratio in cut off point of 0.48, was 96.1% and 100% respectively. Measurement of the urinary L/C ratio soon after birth maybe a promising tool to identify asphyxiated neonates and also to predict the severity of asphyxia.

Late-onset sepsis is responsible for high morbidity and mortality in newborn infants in the world and in particular in developing countries. In this study, we evaluated whether clinical characteristics, laboratory parameters and measurements of serum interleukin-8 (IL-8) are able to discriminate between late neonatal sepsis and normal baby.

This was a prospective (case-control) study conducted between March 2007 and April 2008, at the neonatal intensive care unit, Ghaem Hospital, Mashhad, Iran. The study comprised 93 neonates ≥72 hours of life. The infants were categorized in two groups based on the clinical presentation, and biochemical markers including complete blood count, C-reactive protein (CRP) and blood culture: 1) Control group including 42 infants with routine screening and 2) Case group consisting of 38 infants with definitive infection (positive blood and/or cerebrospinal fluid culture) or clinical sepsis (clinical and laboratory signs of infection without positive blood or CSF culture). Receiver-operating characteristic curves were used for the determination of thresholds for the infection group versus healthy neonate group.

Eighty infants were enrolled in this study. IL-8 and CRP decreased in order of definitive infection, clinical sepsis and healthy subjects respectively (P<0.001). Sensitivity, specificity, positive predictive value, negative predictive value for serum levels were 0.95, 0.1, 0.97, 0.1 for IL-8 and 0.83, 0.86, 0.83, 0.69 for CRP respectively (cut-off point for IL-8 >60pg/ml and for CRP>6mg/dl).

IL-8 may be a valid and early predictive marker of neonatal infection. Also, IL-8 is associated with severity of infection.

this study was designed to determine the effect of clofibrate on neonatal uncomplicated jaundice treated with home phototherapy.

This clinical trial study was performed on 60 newborns with jaundice that received home phototherapy. Inclusion criteria were body weight between 2500 to 4000 gr, breastfed, total serum bilirubin (TSB) between 14 to 20 mg/dl, aged over 72 hours. The neonates were randomly divided into two groups. All received home phototherapy. Group i received a single dose of 50 mg/kg clofibrate and the other group served as control group. Total serum bilirubin level was measured every 24 hours.

Two groups were matched regarding weight, sex, age and first TSB. At 24 and 48 hours of treatment, the mean values of TSB in the clofibrate group were 13.72 (1.56), 9.5 (0.56) and in the control group 15.30 (1.44), 12.6 (1.44). The results show that TSB was significantly decreased after 24 and 48 hours in clofibrate group (P<0.001). The mean duration of phototherapy in group I was 72(0.0) hours and in the control group 76.80 (±9.76) hours. The duration of phototherapy was significantly shorter in clofibrate group (P<0.001).

Clofibrate is effective for outpatients with neonatal hyperbilirubinemia who are under home phototherapy. Of course, further studies are needed for approved routine use of this drug in the treatment of neonatal jaundice.

Available accurate data on the concentrations of copper (Cu) and zinc (Zn) in human milk throughout lactation and infant formulas is important both for formulating nutritional requirements for substances and to provide a base line for the understanding the physiology of their secretion. The objective of this study was to analyze the concentrations of zinc and copper in infant formulas and human milk during prolonged lactation. Levels of these metals were examined in relation to selected parameters such as age, weight, height, education and occupation of mothers.

Thirty mothers referred to the selected clinics in Tehran entered the study. Human milk samples were collected at 2 months postpartum. Zinc and copper concentrations were determined by atomic absorption

The mean values of Zn and Cu in human milk were 2.95±0.77mg/L and 0.36±0.11 mg/L. The mean values of Zn and Cu in infant formulas were 3.98±0.25 mg/L and 0.53±0.17mg/L.

No significant relationship was found between levels of trace elements in human milk and evaluated parameters such as age, weight, height, education and occupation of mothers. The concentrations of zinc and copper in breast milk were lower than those reported in the literature.

Reinfection rate of Helicobacter pylori after successful eradication is low in developed countries. This study was performed to determine the reinfection rate of H. pylori during a follow up period of 12 months in Iranian children.

In this prospective study, children with H. pylori infection were treated with triple omeprazole based regimen. Patients with negative 13C urea breath test (UBT) performed after 8 weeks of therapy, were followed up by the same test after 1 year.

Thirty seven patients, aged 5 to 17 years, were studied. Among them 25 (67.5 %) were boys. After eradication therapy of H. pylori, 34 patients had negative 13C UBT. Reinfection occurred in 5 (14.7 %) patients. Reappearance or continuing symptoms after treatment were associated with higher rate of recurrence (P=0.042).

Recurrence rate of H. pylori was high in our children. Successful eradication significantly decreased complaints and further symptoms. Follow up and reevaluation of patients is necessary especially when there are symptoms after eradication.

To investigate age and sex variations in undernutrition using mid-upper arm circumference (MUAC) cut-off values among 2016 (930 boys and 1086 girls). 3-5 years old rural children of Bengalee ethnicity at 66 Integrated Child Development Services (ICDS) Centers of Nadia District, West Bengal, India.

In a cross sectional study in west Bengal of India, MUAC was measured using standard technique. A total of 2028 children (935 boys and 1093 girls) from 66 ICDS centers were enrolled in this study. The response rate was approximately 95%.

Mean MUAC among boys was higher than girls at all ages except 5 years. Significant sex differences were not observed over ages. The age-combined rates of overall (moderate and severe) undernutrition among boys (38.49%) was higher than among girls (32.22%).The age-combined rates of moderate undernutrition were 36.34% and 31.03% among boys and girls, respectively. The rates of severe undernutrition were 2.15% and 1.20% among boys and girls, respectively. There were sex differences in both moderate and severe undernutrition. In general, there was an increasing trend in the rates of overall undernutrition from 3 to 5 years in both sexes.

These children were experiencing severe nutritional stress.

Sickle cell disease (SCD) is a common hereditary disease in Iran. In developed countries, newborn screening programs have been established to ensure early diagnosis, but in most developing countries, screening is not performed and the diagnosis is often delayed. The aim of the present work was to investigate the clinical presentation of SCD in Iran and comparison of its hematologic indices with normal children.

The study included 44 pediatric patients (26 boys and 18 girls) with sickle cell anemia (SS), 27 sickle /β°-thalassemia (Sβ°), and 21 sickle /β+-thalassemia (Sβ+). Fifty seven healthy individuals matched with the patients were randomly selected as controls.

Mean age at diagnosis in SS group was 4.3 years. At the time of diagnosis all patients were anemic, 89% complained of painful crises. Hemoglobin(Hb) concentration, red blood cell (RBC) count and Hb×RBC product in SS group was significantly lower than in control group (P<0.001), mean corpuscular volume (MCV) and mean corpuscular hemoglobin (MCH) showed no significant differences. Hb×RBC product below 45 and MCH/RBC above 7 have the best sensitivity and specificity for differenting SS group and the control normal group (91 and 98% for Hb×RBC and 89 and 100% for MCH/RBC respectively). Mean age at diagnosis in Sβ+ group was higher than in SS and Sβ° groups (7.45 year vs 4.26 and 4.25 year) (P<0.001). In addition, Sβ° and Sβ+ groups had significantly lower MCV, MCH, and Hb×RBC indices compared with control group.

We suggest that in an anemic patient with history of pain crises, normochrome normocytic anemia, Hb×RBC <45 and MCH/RBC ≥7, SCD should be considered and the patient evaluated accordingly to confirm the diagnosis.

Hepatitis C virus (HCV) is an etiological agent responsible for occurrence of post-transfusion hepatitis in thalassemic patients. This study identified hepatitis C genotypes in pediatric and adolescent thalassemic patients and their correlation with age، blood transfusion، HCV RNA viral titer and liver function.

This study considers cross-sectional data from the Center for Thalassemia in Zahedan (Iran) carried out between August 2005 and September 2007. Twenty multitransfused patients suffering from β-thalassemia major and chronic HCV infection (13 males، 7 females) were included in the study. Patients were considered eligible for the study if they were seropositive for HCV RNA polymerase chain reaction (PCR) before initiation of evaluation. Blood sample was taken for HCV genotype and viral titer as well as biochemical markers. Type specific primer and real-time RT-PCR HCV were used for determination of viral genotype and HCV-RNA titer.

There was a significant positive correlation between serum HCV RNA titer and genotypes (P<0001). Serum HCV RNA levels were found higher in genotype 3a than in others. The most prevalent genotype in thalassemic patients was genotype 3a (40%) followed by 1b (25%)، unclassified (20%) and la (15%). There was no meaningful relationship between genotype، Alanine aminotranferease، ferritin and alkaline phosphatase. Age، serum HCV RNA titer and number of transfusions were the only significant factors associated with genotypes (P<015، P<0. 0001 and P<0. 001 respectively).

This study showed that HCV genotype and viral titer are related to the number of blood transfusions received by thalassemic patients. Screening donated blood in blood banks would prevent the occurrence of hepatitis C in this high-risk group.

Lung function tests have become an integral part of assessment of pulmonary disease. As few studies on pulmonary function tests have been carried out in young children in India, the study was carried out in normal urban and rural school children in Ludhiana district of North India to determine pulmonary functions in the age group 6-15 years and to find its correlation with regards to age, sex, height and weight.

The study group included 600 normal children between 6-15 years age from different urban and rural schools in the region. A preformed questionnaire was interviewed and detailed general physical and systemic examination was done. Pulmonary function tests were measured by using Micromedical Gold standard fully computerized portable auto spirometer (Superspiro Cat No. SU 6000).

The present study shows, all the three independent variables (age, weight and height) have linear positive correlation with lung function parameters, both for boys and girls. Lung function values in boys were significantly higher as compared to that of girls. Urban children had higher lung function parameters than rural children except IRV, FEF25%. Among all anthropometric parameters, height was the most independent variable with maximum coefficient of correlation.

Equations derived from the present study for estimation of the expected values of lung function will help to interpret the observed lung function values in children of North India.

Thyroid nodules are rare in children. Multiple diagnostic modalities are used to evaluate the thyroid mass. The aim of this study was to determine results of management of thyroid nodules in children with special attention to the role of fine needle aspiration biopsy (FNAB) in diagnosis.

Thirty-two children who underwent surgery for thyroid nodules in Mofid Children''s Hospital within 10 years (1996to 2005) were retrospectively studied. From clinical records we obtained data about demographic characteristics, clinical manifestations, ultrasonography (USG) findings, and FNAB results, pathological reports, surgical therapy and complications. Data was analyzed statistically for association with thyroid cancer.

Twenty-five patients (78.1%) were girls, and 7 (21.9%) boys. Mean age was 10.9 (range 8 to 14) years. 24 (75%) patients had benign and 8 (25%) malignant tumors. 18 (56.25%) nodules were located in the right lobe. Statistical analysis revealed sensitivity, specificity, accuracy, and positive and negative predictive values as follows: 80%, 65%, 63%, 25%, and 86% for USG; 35%, 41%, 40%, 18%, and 66% for RNS; 91%, 94%, 90%, 74%, and 96% for FNAB respectively.

Clinical judgment as determined by serial physical findings with USG continues to be the most important factor in the management of thyroid nodules in children. FNAB is the most accurate method of investigation and its accuracy is improved by USG guidance.

Urinary tract is one of the most common sources of infection in children under the age of two years. Many known and unknown risk factors predispose to this important disease in children. This study was conducted to determine whether using a specific type of diaper plays a role in urinary tract infection (UTI) in girls under the age of 2 years.

This case control study was performed in hospitalized children; girls with their first urinary tract infection were selected as cases, and those admitted for other reasons comprised the control group. Two groups were matched for age (±1 month), and other known risk factors for UTI. Type of diapers (superabsorbent, standard disposable and washable cotton), used for these children during six months, from October 2007 to March 2008, were compared in both groups.

Fifty-nine matched pair infant girls less than 2 years were selected. It was revealed that in cases with UTI superabsorbent diapers were used more frequently than in controls (Odds ratio =3.29, P-value=0.005) There were no significant differences in other factors like number of diapers used per day, the time between defecation and diaper change, mothers'' educational level, level of family income and mother''s occupation

The use of superabsorbent diapers could be a risk factor for urinary tract infection in infant girls.

Allgrove syndrome is a rare autosomal recessive condition characterized by adrenal insufficiency, achalasia, alacrima and occasionally autonomic disturbances. Mutations in the AAAS gene, on chromosome 12q13 have been implicated as a cause of this disorder.Case(s) Presentation: We present various manifestations of this syndrome in two related families each with two affected siblings in which several members had symptoms including reduced tear production, mild developmental delay, achalasia, neurological disturbances and also premature loss of permanent teeth in two of them,

The importance of this report is dental involvement (loss of permanent teeth) in Allgrove syndrome that has not been reported in literature.

Pearson syndrome (PS) is a rare multisystem mitochondrial disorder of hematopoietic system، characterized by refractory sideroblastic anemia، pancytopenia، exocrine pancreatic insufficiency، and variable neurologic، hepatic، renal، and endocrine failure.

We describe a six-month-old female infant with Pearson marrow syndrome who presented with neurological manifestations. She had several episodes of seizures. Hematopoietic abnormalities were macrocytic anemia and neutropenia. Bone marrow aspiration revealed a cellular marrow with marked vacuolization of erythroid and myeloid precursors. Analysis of mtDNA in peripheral blood showed 8. 5 kb deletion that was compatible with the diagnosis of PS.

PS should be considered in infants with neurologic diseases، in patients with cytopenias، and also in patients with acidosis or refractory anemia.

Children with overgrowth syndromes including isolated hemihyperplasia have an increased risk for developing embryonal tumors, particularly Wilms'' tumor and hepatoblastoma. Screening with sonography has been suggested as a method of identifying these tumors while they are still at an early stage.

We describe an infant diagnosed with Wilms tumor in whom isolated hemihyperplasia had been overlooked by several physicians prior to the tumor diagnosis.

We recommend tumor surveillance for all patients with Beckwith-Wiedemann syndrome and isolated hemihyperplasia at least for the first six years of life since full molecular characterization of every patient is not readily available.

Only few reported cases of tectocerebellar dysraphia with occipital encephalocele have been reported in the literature.

Three month baby boy, the first child of healthy, consanguineous parents presented with a small swelling over the occipital region since birth. The child also used to have apneic spells without cyanosis and spontaneous recovery. CT scan showed absence of the cerebellar vermis, absence of tectum and the 4th ventricle communicating with the occipital menigocoele sac and an occipital bone defect. The excision of the encephalocoele sac was performed, however the child continued to have apneic spells and did not do well.

In our child irregular respiration probably was the manifestation of the tecto-cerebellar dysraphia syndrome complex and associated shunt malfunction followed by seizures decompensated the physiology of the child leading to fatal outcome.

HDR syndrome (hypoparathyroidism, sensorineural deafness and renal disease) is an autosomal dominant condition, defined by the triad hypoparathyroidism, renal dysplasia and hearing loss. Hirschsprung (HSCR) disease is a variable congenital absence of ganglion cells of the enteric nervous system resulting in degrees of functional bowel obstruction. Rarer chromosomal anomalies are reported in combination with Hirschsprung disease like DiGeorge syndrome, mosaic trisomy 8, XXY chromosomal constitution, partial duplication of chromosome 2q, tetrasomy 9p, and 20p deletion.

Here, we describe an 8 year-old girl with HDR syndrome accompanied by Hirschsprung disease. Although the association of Hirschsprung disease with chromosomal anomalies has been reported, according to our knowledge, this is the first report of associated HSCR with HDR syndrome.

Lithium is used mainly for the treatment of Bipolar Disorder (BD). Case reports and several retrospective studies have demonstrated possible teratogenicity, but the data in different studies is inconclusive. The risk for cardiovascular malformations, particularly Ebstein''s anomaly and other congenital abnormalities have been reported. A 25-year-old gravida 1, para 1 woman at 38 weeks of gestation was admitted for an elective caesarean section. She had a history of BP for which she was treated with lithium 600mg q12h in the first trimester of pregnancy. There was no familial history of birth defects, any antenatal infection or exposure to any other medications, alcohol, smoking, or X-rays. A baby boy (3500g) was born. After 2 to 3 hours respiratory distress clinical picture and chest radiograph suggested diagnosis of congenital diaphragmatic hernia. Repair of his diaphragm was preformed and patient discharged after 12 days. Lithium probably produces a defect in normal development of the diaphragm and may pose specific risk for an anomaly known as congenital diaphragmatic hernia (CDH).

Goldenhar syndrome (oculoauriculovertebral dysplasia) is a rare congenital anomaly with unknown etiology and consists of non accidental association of hemifacial microsomia, auricular anomalies, epibulbar dermoid and vertebral anomalies. Although some malformations are more frequent in infants of diabetic mothers, developmental defects of first and second branchial arch is not a common finding in these patients. We report a female case of Goldenhar syndrome in a newborn infant of a diabetic mother (IDM). Follow up of this patient after 6 months showed normal neurodevelopment and no evidence of hearing loss. She had developed epibulbar dermoid tumor in her right eye. It is necessary to evaluate IDM for presence of anomalies implying oculoauriculo-vertebral dysplasia.