Iranian Journal of Pediatrics
Volume:21 Issue: 4, 2011

Neonatal sepsis (NS) is a common and life-threatening disorder in infants. Previous studies showed that interleukin-6 (IL-6) may be a valid non-invasive and rapid method for diagnosis of NS. We conducted this review to assess the validity of IL-6 for predicting NS. This was a systematic review with meta-analysis. Embase, Medline and Web of Science databases were searched between January 1990 and December 2009. The search terms used were "cytokine", "neonate", "sepsis" and "interleukin-6". We used standard methods recommended for meta analyses of diagnostic test evaluations. The analysis was based on a summary ROC (SROC) curve. Meta-regression analysis was used to assess the effects of some confounding factors on the results of meta-analysis. Potential presence of publication bias was tested using funnel plots and the Egger test. Meta-analysis was performed on 13 publications including 353 infants with sepsis and 691 control infants. The pooled sensitivity and specificity of IL-6 was 0.79 and 0.84, respectively. The maximum joint sensitivity and specificity (i.e., the Q value) in SROC curve was 0.82 and the area under curve (AUC) was 0.89 (95% CI: 0.84-0.94). Meta-regression analysis showed that the diagnostic accuracy of IL-6 was not affected by confounding variables. The evaluation of publication bias showed that the Egger test was not significant (P=0.07). IL-6 seems to be a valid marker for predicting NS. It may be considered for early diagnosis of sepsis in neonatal care units.

ventilator associated pneumonia (VAP) is defined as nosocomial pneumonia in mechanically ventilated patients. It is considered to be most important cause of infection-related death in intensive care unit. We studied the characteristics and risk factors of VAP in critically-ill neonates. Fifty six consecutive neonates with different diagnosis admitted from January to October 2010 to neonatal intensive care unit (NICU), Zagazig University Hospitals who needed mechanical ventilation were included in the study. There were 32 neonates, 18 males and 14 females with proven diagnosis of VAP, and 24 neonates, 11 males and 13 females without VAP served as control group. All studied neonates were subjected to history taking, clinical examination, routine investigations (Complete blood count, C-reactive protein, arterial blood gases, blood culture and liver and kidney function tests), and chest X-ray daily as well as non-bronchoscopic alveolar lavage culture for VAP group only. Of 56 neonates who needed mechanical ventilation, 57.1% developed VAP. Prematurity, low birth weight and prolonged duration of mechanical ventilation were risk factors for developing VAP. Increased total leucocytic count, CRP and hypoalbuminemia were significantly presented in VAP-group. There were significant differences between VAP and non-VAP groups regarding hypothermia, mucopurulent endotracheal tube secretion, PaCO2 and PaO2. Microorganisms associated with blood stream infection in VAP diagnosed group were Klebsiella (15.6%), S. aureus (12.5%), Pseudomonas (9.4%), E. coli (6.2%), Candida (3.1%); 53.1% of obtained blood cultures were sterile. Of non-bronchoscopic alveolar lavage cultures obtained from VAP patients, 68.6% showed gram negative infection, 21.8% showed gram positive organisms and 9.3% revealed Candida infection. The most important risk factors of VAP are prematurity, low birth weight, prolonged duration of mechanical ventilation, enteral nutrition and umbilical catheterization.

Preterm and low birth weight (LBW) infants are at greater risk of developing bilirubin-associated brain damage compared with term infants. Certainly, phototherapy, if used appropriately, is capable of controlling the bilirubin levels in LBW infants; but there is not a unique phototherapy treatment strategy in LBW infants. This study was designed to compare the prophylactic phototherapy and late treatment of jaundiced newborns weighing 1000-1500 grams. Sixty newborns with birth weight 1000-1500 g were studied. They were divided into two groups: the "Prophylactic" group, in which phototherapy started within six hours after birth and continued for at least 96 hours, and the "Treatment" group, which received phototherapy when indicated according to birth weight and suspended when bilirubin level fell below 50% of bilirubin level for blood exchange. Mean value of daily transcutaneous bilirubin (TCB), duration of phototherapy, the need for blood exchange, and the highest TCB value in both groups were analyzed. In the prophylactic group, the highest daily mean rate of TCB was 7.71±1.84 mg/dl, which happened on the third day. In the treatment group, it was 8.74±1.72 mg/dl on the fourth day after birth. The TCB values in prophylactic group were significantly less than those of the treatment group only on the fourth and fifth days after birth (P<0.001). Although the median duration of phototherapy in the treatment group was shorter than that of the prophylactic group (137.60±57.39 vs 168.71±88.01 hours, respectively), this difference was not statistically significant. Only one neonate needed blood exchange in the treatment group. The prophylactic phototherapy treatment for babies weighing 1000-1500 g significantly decreases bilirubin levels on the fourth and fifth days after birth but the clinical course of hyperbilirubinemia does not alter in LBW infant, as indicated by the non-significant change in the duration of phototherapy.

A recent study reported association of high bilirubin concentrations with decrease in basal vesical tonicity and relaxation of pre-contracted ureteral and vesical smooth muscles in vitro, and authors discussed that recovery of antenatal hydronephrosis might partly be associated with decreased bladder resistance to the urine flow due to hyperbilirubinemia. We aimed to investigate whether any relationship between serum bilirubin levels and antero-posterior renal pelvic diameters or pelvicaliceal dilatations exist during newborn period. Neonates with hyperbilirubinemia (group 1) and healthy neonates (group 2) were randomly selected to the study. Capillary blood samples were used to measure micro-bilirubin. Urinary system ultrasound (US) was performed in both groups by an experienced radiologist. Group 1 (31 neonates, 16 males, 15 females) and group 2 (22 neonates, 11 males, 11 females) were identical by means of postnatal age, gender and weight (P>0.05). Mean serum bilirubin levels were 11.1±3.1 mg/dl and 1.4±0.2 mg/dl in group 1 and 2, respectively. Renal length and renal pelvis antero-posterior (AP) diameters were not different between study groups. Pelvis AP diameters of right kidney were 2.1±0.7 mm in group 1 and 1.9±0.7 mm in group 2, and of left kidney were 2.4±0.8 mm in group 1 and 2.3±0.6 mm in group 2. There was no correlation between bilirubin levels and renal length and renal pelvis AP diameters (P>0.05). In this study we were not able to demonstrate any relationship between serum bilirubin levels and renal pelvic diameters and pelvicaliceal dilatation in hyperbilirubinemic neonates. So, it is thought that hyperbilirubinemia might not have a direct effect on outcome of the pelvicaliceal dilatation.

It is propounded that febrile neonates with low risk criteria (LRC) can be carefully observed without parenteral antimicrobial therapy; but yet, reliability of LRC to exclude serious bacterial infection (SBI) is uncertain. The records of all febrile term neonates, seen in the emergency room and admitted in neonatal ward of 17 Shahrivar children's hospital of Rasht, Iran from January 2004 to January 2009 were reviewed. All of them underwent full sepsis workup. The prevalence of SBI in total population and LRC positive and negative neonates were calculated A total of 202 records of previously healthy febrile neonates were evaluated. SBI was shown in 38 (18.8%). The most common type of SBI was urinary tract infection (UTI). Sixty-two (31%) neonates had LRC, and only one (1.6%) had SBI (UTI with E. coli). SBI was significantly more common in neonates without LRC (26.6% versus 1.6%, P<0.001). The negative predictive value (NPV) of LRC to exclude SBI was 98.4% (95%confidence interval: 96.7% to 100%).Conclosion: These findings suggest that LRC may be relied upon to exclude SBI in febrile neonates. We propose that all febrile neonates be admitted, ill or LRC negative neonates should undergo a full sepsis work up and be administered systemic antibiotics immediately. LRC positive neonates should be under close observation.

Sepsis is an important cause of morbidity and mortality in neonates especially in developing countries where identification of the germs and treatment is often unsatisfactory. The aim of the study was to assess the clinical presentation, and bacteriological profile of neonatal infections, and the sensitivity of the causative germs to antibiotics. We carried out a prospective analytic study in the Yaounde Gynaeco-Obstetric and Pediatric Hospital in Cameroon over a 6 months period from 18th November 2008 to 18th May 2009. On the basis of history and/or clinical findings and paraclinical investigations, 218 neonates out of a total of 628 admissions were investigated and managed for neonatal infection. The most frequent symptoms were fever (44.95%), refusal to feed/irritability (32.11%), and respiratory distress/cough (28.90%). Premature birth and prolonged rupture of membranes were the most frequent risk factors. Klebsiella spp, Escherichia coli and Enterobacter spp were the most frequent germs identified in respectively 28.6%, 21.4% and 14.3% of the positive samples. Overall sensitivity of the cultures to ampicillin, netilmicin and gentamycin was poor at 29.4%, 31.4% and 18.9% respectively, whereas imipenem, ofloxacin, ciprofloxacin and ceftazidime had the best sensitivities in 91.7%, 90%, 85.3% and 69.4% of the cultures respectively. The mortality rate was 22%, and low birth weight, premature birth and septicemia were significant risk factors for death. Mortality from neonatal sepsis in this context is still high and there is an upsurge of multi-resistant germs to currently used antibiotics, calling for the need for rational use of antibiotics in the management of these infections.

Due to recent scientific progress in assisted reproductive techniques (ART), infertile couples can now become fertile. Thus, a number of infants in our country are the results of these costly interventions. This study has been undertaken to evaluate the physical growth process of different methods of ART infants by standard growth charts from birth until nine months of age. This was a descriptive cross-sectional study of 333 infants conceived through ART [intracytoplasmic sperm injection (ICSI) and in vitro fertilization (IVF)] at Royan Institute. A sequential, non-random sampling method in a period of 22 months was used. Their growth was assessed by measuring infant's weight, height and head circumference, and physical examination. The growth patterns were calculated by recording the values in standard growth charts. The final analysis was done with SPSS version 16 and by using Chi-square test. In comparison with growth charts, the weights of one-third of the infants were less than two standard deviations (SD) at birth and one-fourth had head circumference less than three SD at birth. Low birth weight (LBW) infants were six times more than infants of normal population. From birth to six months of age, growth abnormalities were seen in a substantial number of infants. However, at nine months of age, there was no significant difference observed between infants conceived by different methods of ART (IVF and ICSI). Multiple births are the most important confounding factor impacting the growth process of ART infants. Multiple pregnancies can lead to low birth weight, height and head circumference, and growth abnormalities up to six months of age. This abnormality improves by increasing age of the infants.

Cochlear implantations have become a routinely performed and successful surgical intervention in both adults and children. The current article reports the complications encountered in various age groups of consecutive children who underwent implantation in our center. We performed a prospective analysis of all profoundly deaf children who underwent cochlear implantation from March 2006 to July 2009 at Baqhiyatallah Cochlear Implantation Center. All patients were younger than 5 years old at the time of implantation. The minor complications occurred in 49 (18.7%) cases, The most common postoperative complications were temporary facial weakness detected in 15 cases (5.7%) all of which were reversible. Magnet wound was observed in 14 (5.3%) patients, keloid formation in 10 (3.8%), wound infection in 2 (0.8%), otitis media in 5 (2%), and electrode movement, meningitis, vertigo, Laryngospasm each in 1 (0.4%) case was detected among our patients. Cochlear implantation in children continues to be reliable and safe in experienced hands, with a low percentage of severe complications as long as the patient is monitored closely.

The purpose of this study was to examine effects of performing preoperative preparation program on children's anxiety. This study was performed in Amirkola Pediatrics Hospital, Mazandaran. A randomized controlled trail was performed on 122 children (7-12 years of age) admitted for elective surgery during 15 months. The researcher randomly assigned eligible participants in to the experimental and control groups, after pre-test baseline measurement had been taken. Analyzing was performed through independent t-test and χ2 test. P<0.005 was considered statistically significant. The experimental group received therapeutic play and the control group received routine preoperative information preparation. The mean and standard deviation of the state anxiety scores of children in experimental and control groups before intervention were 35.52±6.99 and 34.98±6.78, after intervention 31.44±5.87 and 38.31±7.44 respectively. The state anxiety score was lower significantly in the experimental group prior to preoperative surgery than in the control group (P=0.000). Performing preoperative program with using therapeutic play intervention is effective for preparing children before surgery and decreases their anxiety.

The aim of this study was to determine the prevalence of attention-deficit/hyperactivity disorder (ADHD) symptoms in Iranian preschool children based on evaluations by parents and teachers because a thorough understanding of epidemiologic features of ADHD symptoms in preschool children is important for prevention and management. Children between the ages of three and six attending kindergarten participated in this study. For the survey, 37 kindergartens were selected by multistage (stratified cluster random) sampling, consisting of 2213 children with a design effect equal to 1.5. A 19-item observer-rating questionnaire was generated to assess ADHD symptoms in children within the last 6 months. This questionnaire was used by both teachers and parents to assess ADHD behavior in participating children. Of 1403 children aged 3-6 years, 362 were classified as having ADHD symptoms according to their parent evaluation [25.8% (23.6-28.1%)] and 239 according to their teachers evaluation [17% (14.1-20.4%)]. Child rank among siblings, mother's education level, and interest in aggressive television programs were all independent explanatory variables according to parent's evaluation. Gender, parent education, child rank, single parent and interest in aggressive television programs were all independent explanatory variables according to teacher's evaluation. Our findings reveal a large discrepancy in the prevalence of ADHD symptoms in preschool children based on evaluation by parents and teachers. Thus, it seems that the ADHD screening should be performed in multiple settings in order to identify children who need further investigations.

Atrial septal defect (ASD) device closure is routinely done under the guide of transesophageal or intracardiac echocardiography which are expensive techniques and not easily affordable in developing countries. Using metallic devices, we attempted 32 ASD device closures under transthoracic echocardiography. Of those, 30 procedures were successful (94 %). In two patients with relatively large ASD we encountered difficulty in positioning the device. These patients were referred for surgical closure. ASD device closure can be carried out successfully in most patients under transthoracic echocardiography in situations where transesophageal or intravenous echocardiographies are not available or affordable.

Acute Poisoning in children is still an important public health problem and represents a frequent cause of admission in emergency units. The epidemiological surveillance specific for each country is necessary to determine the extent and characteristics of the problem, according to which related preventive measures can be taken. The present retrospective study describes the epidemiology of accidental and suicidal poisonings in a pediatric population admitted to the Pediatric Emergency Department of Eskisehir Osmangazi University Hospital during the year 2009. Two hundred eighteen children were reffered to the emergency department due to acute poisoning. 48.4% of patients were boys and 51.6% were girls. The majority of cases were due to accidental poisoning (73.3% of all patients). Drugs were the most common agent causing the poisoning (48.3%), followed by ingestion of corrosive substance (23.1%) and carbon monoxide (CO) intoxication (12.5%). Tricyclic antidepressant was the most common drug (11.7%). Methylphenidate poisoning, the second common drug. 262 patients were discharged from hospital within 48 hours. Preventable accidental poisonings are still a significant cause of morbidity among children in developing countries. Drugs and corrosive agents are the most frequent agents causing poisoning.

Acute appendicitis is the most common cause of abdominal surgery in children. Similarity between signs and symptoms of appendicitis and other common pediatric illnesses, atypical manifestations of appendicitis in young children, and children's inability to give precise explanation for their symptoms contribute to considerable delay in proper diagnosis and increased rate of perforation. Current study reports the surgical and pathological findings of appendectomies in the largest Children's Hospital in Iran. It also evaluates whether common protocol for pathologic evaluation following appendectomy is beneficial. Pathologic reports of 947 appendectomies, performed with the presumptive diagnosis of acute appendicitis, were gathered. Correlation between surgical and pathologic findings was assessed. Demographic characteristics of patients between surgical and pathological subgroups were also compared. The mean age of participants was 6.9±3.5 years. Eighty seven (25.5%) children had abnormal pathological findings and normal surgical report. None of miscellaneous findings including appendicular carcinoid tumor 3 (0.3%), oxyuriasis 2 (0.2%), and mycobacterial infection 4 (0.5%) were recognizable during the surgery. Of all pathologically confirmed cases with perforated appendicitis, 9.7% were not detected during the surgery. In current study, acute appendicitis was the most common pathological diagnosis, however, high normal appendectomy rate along with noticeable proportion of surgically missed perforated appendicitis and unusual histopathologies strongly supported routine histological examination.

Celiac disease is an intestinal disorder identified by mucus inflammation, villous atrophy and crypt hyperplasia. This disorder can be controlled by elimination of gluten from daily diet. Patients with celiac disease are at greater risk of gastrointestinal malignancy and non-Hodgkin lymphoma than are the general population. This study tries to present the value of gluten patch test for diagnosis of celiac disease. In this investigation, the study population was divided into case and control groups. The case group consisted of patients with celiac disease. The control group were patients involved in celiac disease but suffering from other gastrointestinal disorders. Both gluten patch and placebo patch were attached to the skin between the scapulas. The results were read twice: 48 hours and 96 hours after the patch was applied. Patients who showed irritation reactions were withdrawn from this study. The results were analysed by SPSS software, Spearman's test, chi square, and Mann-Whitney tests. The value obtained from the gluten patch test after 96 hours are as follows: specification at 95%, sensitivity at 8%, positive prediction value at 67%, and negative prediction value at 43%. It can be concluded that the gluten patch test is not an efficient test for screening of celiac disease, however, it can be useful for diagnosis of celiac disease if employed and studied with clinical symptoms and serologic and biopsy tests. Furthermore, we should doubt our judgment if the result of gluten patch test for the patient with celiac disease is positive.

The aim of this study was to determine prevalence and risk factors of hypospadias in newborn infants of a private hospital in Mashhad city located in northeast Iran. All live birth deliveries in maternity hospital were enrolled from Oct 2006 to Sep 2008. All hypospadias cases were compared with the next male live births for possible risk factors. This study included only solitary hypospadias cases, those associated with other anomalies were excluded. Both parents were asked to fill out the same written questionnaires seperately. Data was analyzed by using SPSS. During two years 6149 babies were born in our hospital; 25 cases (0.4%, 4 in1000 live births) of hypospadias were identified. Hypospadias occurred in 0.76% of male deliveries. Most cases of hypospadias were born in summer and winter was the season which least number. Positive family history (P=0.04) was regarded as a potential risk factor that was present in 44% of cases in hypospadias group. Iron supplement consumption in first trimester of pregnancy in control group was significantly more than in hypospadias group (P=0.001) and also usage of folic acid in control group before and in first trimester of pregnancy was taken more by mother in control group than in hypospadias group (P=0.049 and P=0.001 respectively). Prevalence in this population was intermediate (4 in 1000 live births). Summer was the most epidemiology factor for occurring of hypospadias probably due to conception in cold season. Iron and folic acid supplementation may have preventive effect in hypospadias.

Studies in different populations have shown great variation in the prevalence of thyroid diseases in patients with type 1 diabetes mellitus (T1DM). Our aim was to study the prevalence of thyroid disorders such as autoimmunity of thyroid (AIT), thyroid dysfunction, and goiter in children and adolescents with T1DM, compared with age- and sex-matched healthy controls in Isfahan. One hundred patients with T1DM who were referred to Isfahan Endocrine and Metabolism Research Center and 184 healthy schoolchildren matched for age and sex were included. They were examined for goiter by two endocrinologists. Thyroid function test and serum thyroid antibodies (anti-TPO Ab and anti-Tg Ab) were measured. The prevalence of subclinical hypothyroidism was high in both groups (18%). T1DM patients had lower frequency of goiter (21% vs. 38%, P=0.001), and higher prevalence of positive AIT (22% vs. 8%, P=0.001), anti-TPO Ab positivity (19.3% vs. 5.3%, P=0.000), and anti-Tg Ab (11.1% vs. 6.4%, P=0.1) in comparison with the control group. Being positive for AIT in diabetic patients meant an odds ratio of 5 (CI 95 %: 1.5-15.6) for thyroid dysfunction. There was no association between age, sex, duration of diabetes and HbA1C with serum anti-TPO Ab and anti-Tg Ab concentrations in this group. Our results demonstrated the high prevalence of AIT and thyroid dysfunction in patients with T1DM. We suggest regular thyroid function and antibody testing in these patients.

Beta thalassemia major is a prevalent hereditary disease in Mediterranean region especially Iran. Early blood transfusion is necessary for most of the patients and frequent transfusion can cause various medical problems for the patients. The aim of this study was to find major causes of hospital admission in beta thalassemia major patients to reach the accurate preventive and therapeutic plans for these patients. Four hundred twenty six patients were admitted to the Nemazee Hospital (the main University referral Hospital Center affiliated to Shiraz University of Medical Sciences in Fars Province, southern Iran) during 3 years period (January 2007 to January 2010). A questionnaire was filled containing age, gender, hemoglobin level, frequency of blood transfusions, deferoxamine injection, cause of hospital admission and hospital course. The mean age of patients was 11.28 years. The mean serum ferritin level was 1820±749 µg/lit. Two hundred fifty five (59.75%) patients were male and 171 (40.25%) patients were female. The top five most prevalent causes of hospital admission were splenectomy (21.8%), infections (19.9%), congestive heart failure (19.0%), diabetes mellitus (13.4%), and Liver biopsy (11.5%). (P=0.0002) Results of this study revealed that infections and complications due to iron overload are major causes of hospital admission in beta thalassemia major patients.

Malnutrition, overweight and obesity are major health concerns in modern societies and especially among children. Overweight and obesity affect children's current and future health. It is known that the prevalence of overweight differs by race, sex, and geographic location. In a cross-sectional study 30092 Iranian children aged 7-18 years in six ethnic groups were selected by a cluster sampling. Prevalence of obesity and overweight and distribution of body mass index (BMI) by gender, age, and ethnicity were measured. Cutoff points of BMI for defining obesity and overweight were based on the Iranian, and IOTF standard values. This study showed a significant ethnic difference in BMI. Prevalence of overweight and obesity among Iranian children was 9.27% and 3.22% respectively comparing international standards. The frequency of overweight and obesity was higher in boys. This study showed a significant difference in BMI among different ethnic groups.

Acute mixed-lineage leukemia (AMLL) is characterized as the acute leukemia involved with acute myeloid cells and lymphoid cells at the same time. The AMLL is easily misdiagnosed because of a dual character involved with lymphoid and myeloid cells. At present, researches of AMLL in adults are more common. Only some are reported for children. Therefore, our aim was to study clinical characteristics of the childhood AMLL. From January 2000 to July 2009, 14 cases of AMLL children were selected by morphological and immunophenotyping methods from 185 cases of childhood acute leukemia admitted to the Department of Pediatrics, Tongji Hospital, Tongji Medical College of Science and Technology of Huazhong University. Medical records of all AMLL cases were reviewed for clinical characteristics. Fourteen cases of AMLL were screened from 185 cases of acute leukemia by morphology, immunology, cytogenetics and molecular (MICM). The rate of childhood AMLL accounted for 7.57% of pediatric acute leukemia (AL) diagnosed in the research period; white blood cell count in most of the patients was normal, the average value being 31.0×109/L in the first visit. In the 14 cases of AMLL, 8 cases were B-Ly+/My+, 2 cases were T-Ly+/My+B, and 4 were T+B-Ly+/My+. Among them, nine cases received treatment. Consequently, 6 cases reached complete remission (CR); 1 case had not complete remission; 2 cases did not complete the treatment. The diagnosis of AMLL should depend on the comprehensive evaluation of MICM. As there are still many problems concerning AMLL, it is very necessary that the research units collaborate with each other to improve the prognosis of childhood AMLL. The limitations and applications of the results are that they are only based on the patients of one hospital.

Although ventricular septal defect (VSD) is the most common congenital heart disease, it is usually diagnosed late. The presentation of the disease is variable; sometimes it is so quiet and silent that might even improve and heal spontaneously, and in some certain cases if the appropriate, on time and early treatment is not done, this would lead to irreparable complications and mortality even in the early life period. This study reviews the diagnostic process, treatment and follow-up of the patients. It is hoped that the results of the present study be used to improve the patient's condition. This was a cross-sectional study done on 145 patients with VSD during 54 months in Isfahan. The disease was identified through color Doppler echocardiogram, cardiac catheterization and angiography if necessary. The required data were collected at the time of definite diagnosis. Mean age at initial and definite diagnosis of the disease was 17 months and 44 months, respectively. Heart murmur led to initial diagnosis in 85% of the cases. In 27.5% VSD was associated with other cardiac anomalies. Pulmonary artery hypertension existed in 16.5% of the cases. Fifty nine surgeries were performed on 40 patients. In routine physical examination of the infants, the probability of heart disease should be considered; conducting echocardiogram in suspected cases would lead to early diagnosis and eventually timely treatment. Appropriate follow-up of the patients will provide optimal care and treatment at proper time.

Churg-Strauss syndrome (CSS), a systemic vasculitis accompanied by asthma and eosinophilia, almost invariably affects the lung and is frequently associated with cutaneous involvement. It rarely has cardiac involvement. We report an unusual case of CSS with myocardial involvement and stroke. A 16-year old female suffered of allergic asthma for 4 years. She was under treatment with oral prednisolone and seretide inhalation. After CSS diagnosis, she developed paroxysmal atrial tachycardia. Serum levels of Troponin I and Troponin T were increased indicating massive myocardial damage probably due to myocarditis. After 5 months she developed acute hemiparesis without any evidence of ischemic or hemorrhagic event. She was treated with IVIg, intravenous pulses of methylprednisone and cyclophosphamide for each complication. Myocarditis and stroke may also complicate CSS which should be taken in consideration for better management.

Acute disseminated encephalomyelitis (ADEM) is a rare, monophasic, demyelinating disease of the CNS which sometimes could be refractory to traditional treatment. We present a case of fulminant ADEM which is treated with combination of corticosteroid, intravenous immunoglobulin and cyclosporine. Immunosuppressive agents such as cyclosporine may be effective especially in fulminant form of the disease.

Lipoid congenital adrenal hyperplasia, is the rarest and usually the most severe form of adrenal steroidogenic defect,which may presents as infantile cholestasis. Here we present a 45 days old infant who came to our attention with cholestasis and severe intractable vomiting and electrolyte disturbances. Evaluation resulted in diagnosis of congenital adrenal hyperplasia. Hydrocortisone and flodrocortisone improved the symptoms including jaundice and vomiting. Hyponatremia and hyperkalemia also resolved with above mentioned treatment. Congenital adrenal hyperplasia as one of the causes of neonatal cholestasis should be kept in mind, whenever there are also electrolytes abnormalities.

The Abdominal Inflammatory Myofibroblastic Tumor (AIMT) is a rare tumor with unknown etiology which usually occurs in children and adolescents. It is composed of myofibroblastic spindle cells intermixed with inflammatory cells. We present four cases of AIMT.Cases Presentation: We herein present four cases of AIMT in different ages (range: 3.5 to 13 years) and in different organs (stomach, periduodenal, mesenteric, and colon). There were two females and two males. The main symptoms were abdominal pain/mass/obstruction, vomiting, and weight loss. In all four patients, diagnosis was made by laparatomy and pathologic examination of excised mass lesion. Three patients underwent complete excision and no residual disease was present, one patient received chemotherapy due to tumor recurrences. The patients were followed up in average for four years. As the imaging and laboratory tests are non-specific, the diagnosis of AIMT is rarely made before surgery. AIMT should, therefore, be considered when a mass arises in an unusual location in the pediatric age group. Complete surgical resection should be performed whenever possible and the child should be kept on long-term follow-up.

Acute lymphoblastic leukemia (ALL) is the most common malignancy in children. Bone pain is an important symptom that can be severe. Eosinophilia without any other abnormal laboratory findings is rare in ALL. Strongyloides stercoralis in ALL causes disseminated fatal disease. This 9-year-old girl presented with bone pain in lumbar region. Bone pain was the only symptom. The patient didnt have organomegaly. The BM samples were studied by flow cytometry, which showed pre-B cell ALL. Larva of Strongyloides stercoralis was found in fecal examination. Plain chest x ray showed bilateral para-cardiac infiltration. Strongyloidiasis was treated before starting chemotherapy. After two days treatment with Mebendazol the patient developed cough, dyspnea, respiratory distress and fever. The treatment changed to Ivermectin for 2 days. Chemotherapy started five days after diagnosis of leukemia. The patient complained merely of bone pain in lumbar region without any other signs and symptoms. Peripheral blood smear showed eosinophilia without any other abnormality. Stool examination showed Strongyloides stercoralis larvae. We suggest that all patients diagnosed as ALL in tropical and subtropical regions should be evaluated for parasitic infection especially with Strongyloides stercoralis.

The fracture of clavicle is the most frequently observed bone fracture as birth trauma and it is usually unilateral. It is seen following shoulder dystocia deliveries or breech presentation of macrosomic newborns. We report two macrosomic newborns with bilateral clavicle fracture and brachial plexus palsy due to birth trauma. Chest X-rays confirmed bilateral fracture of clavicles. Both patients were recovered without any sequel. Bilateral clavicular fracture should be considered in any neonate with bilateral absent Moro reflexes.

Pleuritic pain is not an unusual problem in children. Other concomitant symptoms should be considered for diagnostic approach in a child with pleuritic chest pain. In this report we discuss chest pain in a 6-year-old child with regard to other signs and symptoms. Finally, we found a rare life-threatening complication of juvenile systemic lupus erythematosus (JSLE) in our patient.