Iranian Journal of Pediatrics
Volume:24 Issue: 4, Aug 2014

Nonalcoholic fatty liver disease (NAFLD) has become the most common cause of chronic liver disease in the pediatric population in the last few decades (1, 2).

The debate about the effectiveness of homeopathy hits the headlines from time to time. Reported evidences for the role of homeopathy in psychiatric illness relevant to people with intellectual disabilities are patchy and inconsistent. In this review we summarize the best available evidence for the use of homeopathy to treat the psychiatric disorders common in this population. Systematic literature review was conducted through February 2012 to July 2012 in AMED, CINHAL, BNI, EMBASE, MEDLINE, PSYCHINFO and GOOGLE SCHOLAR. In the next steps thirty eight homeopathic associations were contacted and a top-up literature search was done on Scopus and World of Science databases till March 2014. Twelve relevant clinical trials were identified and included in this study. The quality of each trial was assessed by the Oxford quality scoring system (Known as Jadad score) as well as subjective review by two reviewers independently (good versus poor). The largest body of evidence pertained to the use of homeopathy in the treatment of attention deficit hyperactivity disorder (ADHD). There is heterogeneity in the quality of trials and also the outcome of studies but overall our findings suggest some potential for using homeopathy in ADHD. Current evidences do not support the use of homeopathy for treatment of speech and language difficulties. There was only one trial concerning the use of homeopathy in Autistic Spectrum Disorder. This was of a poor quality and unable to provide any recommendation. Whilst acknowledging the risk of publication and language bias in our study, the currently available evidences are neither conclusive nor comprehensive enough to give us a clear picture for the use of homeopathy in patients with intellectual disabilities. There are large gaps in the body of evidence concerning the role of homeopathy in the treatment of common disorders in intellectual disability, such as autism, challenging behavior or developmental arrest in childhood.

Cerebral palsy is the most common cause of spasticity and physical disability in children and spasticity is one of the commonest problems in those with neurological disease. The management of spasticity in children with cerebral palsy requires a multidisciplinary effort and should be started as early as possible. There are a number of treatments available for the management of spasticity. This article reviews the variety of options available for the clinical management of spasticity.

To analyze the effects of hyaluronidase and hirudoid treatment on drug extravasation in neonates. The medical records of 13 neonates with drug extravasation treated with hyaluronidase and hirudoid between August 1st, 2010 and May 1st, 2012 were analyzed retrospectively. The treatment procedure for drug extravasation adhered to the protocol in neonatal department. The information including age, sex, weight, diagnosis, size of affected area, site of extravasation and treatment was collected. The extravasation injuries alleviated and the symptoms improved after treatment, no adverse drug effects were reported with use of hyaluronidase and hirudoid. The treatment appeared to be beneficial in the management of extravasations of various medications in neonates and may be useful in reducing the severity of cutaneous toxicosis. However, further studies with large samples are still needed to assess the effectiveness and safety of hyaluronidase and hirudoid.

Kawasaki disease (KD) clinically presents as a systemic vasculitis syndrome with significant cardiovascular involvement. With different incidence among different ethnic groups, the role of certain human leukocyte antigens and their products has been considered as a crucial predisposing factor in the immune responses in this disease. We determined the distribution of human leukocyte antigens type B for 90 Iranian patients with Kawasaki disease in order to evaluate a possible association between these antigens and this disease in our area. We used the polymerase chain reaction (PCR) sequence specific primers (PCR-SSP) technique for antigen typing. Distribution of these antigens for 89 healthy Iranians used as control. While 7 (3.9%) of our patients were positive for human leukocyte antigen type B 40*, there were 18 (10.1%) subjects from the control group who had this antigen with statistically significant difference between patients and control group (CI= 95%, RR=1.15 and P= 0.02). Data were analyzed by Pearson chi-square test and Fisher''s exact test. SPSS version 15 was used for statistical analysis and a P value less than 0.05 considered statistically significant The presence of higher frequency of allele type-B40* in the control group may represent a protective role for this antigen with resultant decreased susceptibility to KD in our area.

Juvenile systemic lupus erythematosus (JSLE) is a common rheumatologic disorder that involves multi organ systems. Prognosis of lupus in children may be poorer than in adults. In this study, we determined mortality and morbidity rates in the pediatric SLE in Iran. In a cross sectional study, we evaluated 120 children with SLE who had registered in our pediatric rheumatology database within 2004-2010. Data including sex, age, remission, age at the time of diagnosis, age at the time of study, various types of organ involvement, mortality and remission were extracted from this database. From 120 cases, 77% (92 cases) were females and 23% (28 cases) males (F:M=3.3). Mean follow up period was 56±32 months and mean age at the time of manifesting disease 10.34±2.9 years. Mortality rate was 10% (12 cases) in our investigation. Musculoskeletal involvement showed significant difference between various age groups (P<0.01), that was more frequent in group of 7 years and older. Frequency of oral ulcer and ophthalmic involvement in boys was significantly higher than in girls (P<0.05). Frequency of cardiovascular involvement (P<0.01) and renal involvement (P<0.01) was significantly higher in the patients who had no remission. There was a significant association between mortality rate and cardiac (P<0.02, OR=4.9), pulmonary (P<0.01, OR=10.167) and liver (P<0.05, OR=1.19) involvement. In our investigation 1-year survival rate was 97% and 5-year survival rate 89%. Liver, cardiac and pulmonary involvements have an association with higher mortality in JSLE patients.

This study investigated the expression and prognostic significance of the CD95 death receptor and CD20, a B cell-lineage associated marker, along with CD34 and CD44 non-lineage associated molecules in Iranian children with acute lymphoblastic leukemia (ALL). We performed immunophenotyping for expressions of the molecules in blood samples from children diagnosed with ALL by using a panel of monoclonal antibodies for flow cytometry analysis. The expression of markers was evaluated in relation to clinical and paraclinical features as well as response to treatment in the patients. CD95 showed a higher expression in T-ALL compared to B-ALL (P<0.001). Analysis of the clinical and laboratory findings at diagnosis in the group of B-ALL patients revealed an association between CD95 expression with lower white blood cell (WBC) numbers and bone marrow blasts (P<0.05). We detected a positive correlation between the expressions of CD95 and CD44 (r=0.445, P<0.01) in B-ALL patients. There was an association between CD20 expression and several poor prognostic factors that included increased extramedullary involvement (EMI) and decreased platelet numbers (P<0.008). The mean expression of CD34 in B-ALL was higher than T-ALL (P=0.004). At follow-up, complete remission duration (CRD) and survival duration did not significantly differ between patients who were positive or negative for each marker. Association of the studied molecules with several prognostic factors implies the significance of CD95 molecule as favorable and CD20 as unfavorable prognostic markers for childhood ALL.

One of the primary factors in managing patients with retinoblastoma is early diagnosis. The main idea of this study was to recognize the consequences of delay in diagnosis on therapy of the disease. A retrospective review of all children with proven retinoblastoma, who had presented to MAHAK hospital in Tehran, from April 2007 to Dec 2011, was performed. Grouping of intraocular tumors was applied as A to E according to International Classification of Retinoblastoma. There were 157 (91 boys) children eligible for study. The mean age was 1.21±0.11 years with average delay in diagnosis of 3.4±0.53 months. Classification of D group in both unilateral (93 patients) and bilateral tumors was the largest category. A significant relation (P=0.05) between delayed diagnosis time and tumor grouping was evident. The most frequent symptoms were leukocoria and strabismus. Age was significantly lower in the subgroup of bilateral tumors than in unilateral retinoblastomas (0.6±0.12 year vs 1.6±0.15 years). The diagnosis was delayed in subgroup of extra ocular retinoblastoma more than in intraocular tumors (8.7±2.9 months vs 2.9±0.52 months). The authors recommend early referring of suspected cases to ophthalmologists and pediatric oncologists and to organize educational programs to publisize signs and symptoms of the disease such as leukocoria, strabismus and ocular inflammatory disorders through national media. In conclusion, early diagnosis of retinoblastoma can be the primary factor in managing the patients as the delay in diagnosis accounts for highly advanced disease and poor prognosis.

To study the clinical profile and complications of childhood scrub typhus. Prospective observational study of 66 children with scrub typhus, admitted to a tertiary hospital in north India, during the period between January 2011 and December 2012. The diagnosis was confirmed by serology. All children presented with fever. Other common symptoms were vomiting (56%), facial swelling (52%), cough (35%), abdominal pain (33%), breathlessness (29%) and decreased urine output (29%). High grade fever (>101 oF) was recorded in 91% of children. Other common signs were hepatomegaly, splenomegaly, edema, tender lymphadenopathy and hypotension, observed in 82%, 59%, 39%, 38% and 36% of cases, respectively. An eschar and a maculopapular rash each were observed in 20% of patients. Meningoencephalitis (30.3%), severe thrombocytopenia (27.2%), shock (25.8%), acute kidney injury (16.7%) and hepatitis (13.6%) were the most common complications observed in these children. Other common complications were acute respiratory distress syndrome, respiratory failure requiring ventilation, bronchopneumonia and myocarditis. Ninety percent of children became afebrile within 48 hours of initiating an appropriate antibiotic. Median time to defervescence was 22 hours. The overall mortality rate was 7.5%. Causes of death were refractory shock, meningoencephalitis, acute respiratory distress syndrome, bronchopneumonia, acute kidney injury and myocarditis. Pediatricians should keep a high index of suspicion for scrub typhus in any febrile child having a maculopapular rash, hepatosplenomegaly, tender lymphadenopathy, thrombocytopenia and features suggestive of capillary leak. Pending serological confirmation, empirical therapy with doxycycline or azithromycin should be started, as delay in treatment would result in life threatening complications.

Neonatal mortality is a major health problem in sub-Saharan Africa and the risk factors are not well established. The objective of this study was to determine the risk factors for neonatal mortality at the Yaounde Gynaeco-Obstetric and Pediatric Hospital. We conducted a retrospective and analytic case-control study from the medical records of newborns admitted at the neonatal unit of this hospital between 1st March 2003 to 31st December 2012. 850 subjects were enrolled; that is 425 cases and 425 controls. The intra-hospital neonatal mortality rate was 9.83%. The main causes of neonatal mortality were in descending order: neonatal sepsis (60.2%), complications from prematurity (42.6%), birth asphyxia (37.4%), and congenital malformations (11.8%).The most prominent risk factors for neonatal mortality after multivariate analysis with logistic regression were: prolonged membrane rupture (OR: 3.8719, 95% CI: 2.3619-6.3471; P=0.0000), low birth weight (OR: 1.6240, 95% CI: 1.0108-2.6091; P=0.0450), Apgar score less than 7 at the 5th minute (OR: 6.8979, 95% CI: 4.0709-11.6883; P=0.0000), and congenital malformations (OR: 4.3307, 95% CI: 1.6120-11.6347; P=0.0037). Delivery by cesarean section (OR: 0.2644, 95% CI: 0.1478-0.4732; P=0.0000) and being born in this hospital (OR: 0.4409;95% CI: 0.2566-0.7576; P=0.0030) were protective. Neonatal mortality was influenced by both maternal and neonatal factors. This could be reduced through sensitization of pregnant women on the need of good quality antenatal visits, and capacitating the health personnel on the adequate management of high risk neonates.

This study aimed to evaluate serum selenium levels and mean platelet volume in children who experience simple febrile convulsion. The study comprised 42 patients diagnosed with simple febrile convulsions and a control group of 30 healthy children. Blood samples were taken following a febrile convulsion. Selenium levels in the serum of both the patients and control subjects were measured with the hydride formation method on an atomic absorption spectrometry device and mean platelet volume was evaluated. When the mean values of the febrile convulsion patients were compared with those of the control group, the mean selenium levels and thrombocyte count were found to be statistically significantly low (P=0.002, P=0.01 respectively) and the mean platelet volume values were statistically significantly high (P=0.002). While low serum selenium levels cause the onset of a febrile seizure in patients with simple febrile convulsion, it is thought that the increased mean platelet volume shows infection activity causing febrile convulsion.

Hypospadias is a common birth defect of the penis. Besides the abnormal position of the urethral opening, there is usually a ventral preputial defect with preputial redundancy in dorsal shaft. There are many flap procedures for correcting this defect. Here, we present our experience of skin coverage procedure with better cosmetic results. It is a prospective study on patients with mid-shaft to glandular hypospadias operated from June 2008 to December 2012. The operations were performed by one surgeon in two hospitals and the cosmetic results were evaluated by the surgeon, parents, and another pediatric surgeon by a satisfaction questionnaire. In this procedure, inner prepuce was incised curvilinearly, remaining 5 mm in medial and 8 mm in lateral aspects of the inner prepuce. For skin repair, dorsal flaps were approximated in midline along median raphe. Sixty-three patients with mean age of 25.75±8.46 (7-93) months were followed up for 7.06±3.34 (2-15(months. There were 4 complications. The overall satisfaction with penile skin coverage was 93.7% for parents and 98.4% for surgeons. Patients’ age and primary site of meatus had a significant correlation with cosmetic results (P<0.05), while urethroplasty techniques and post-operative complications were not significant. Reapproximation of dorsal flaps in midline is a simple method and can be used in most cases of uncomplicated primary hypospadias. By this technique a more normal appearance can be achieved.

Obesity increases cardiac diseases by increasing tendency to atherosclerosis. Our aim was to define epicardial adipose tissue thickness, and its related factors in obese children. Total of 94 patients were divided into obesity with metabolic syndrome (MS) (n=30), obesity without MS (n=33), and control (n=31) groups. Auxological values with fasting glucose, fasting insulin, alanine transaminase, serum lipid levels, and high sensitive C-reactive protein levels were evaluated. Epicardial adipose tissue thickness, interventricular septum thickness and left ventricular mass were measured by echocardiography. Weight, body mass index, waist circumference, insulin, alanine transaminase, and high sensitive C-reactive protein values were markedly higher in obesity group when compared with controls (P<0.001). Epicardial adipose tissue thickness was 0.64±0.23 cm in obesity with MS; 0.60±0.20 cm in obesity without MS, and 0.27±0.12 cm in control group (P<0.001). Interventricular septum thickness and left ventricular mass values were markedly high in obesity without MS group (P<0.001 and P=0.002). Our study has indicated that obesity has unfavorable effects on heart starting in the adolescence.

Recently, new predictors of vesicoureteral reflux (VUR) in children with a first febrile UTI such as Procalcitonin (PCT) were introduced as selective approaches for cystography. This study wants to show the capability of PCT in predicting presence of VUR at the first febrile UTI in children. Patients between 1 month and 15 years of age with febrile UTI were included in this prospective study. PCT values were measured through a semi-quantitative method in four grades comprising values less than 0.5, 0.5-2.0, 2.0-10.0 and above 10.0 ng/ml. The independence of PCT levels in predicting VUR were assessed after adjustment for all potential confounders using a logistic-regression model. A total of 68 patients, 54 (79.4%) girls and 14 (20.6%) boys were evaluated. PCT level demonstrated a significant difference between patients with positive VUR and those with negative VUR (P=0.012). To calculate the independent factors that may predict the presence of VUR, all included variables were adjusted for age and sex. Results of logistic regression showed that a PCT level between 2.0 and 10.0 ng/mL could independently predict presence of VUR (Odds ratio=6.11, CI 95%= 1.22-30.77, P=0.03). Our finding in this study showed that readily available semi-quantitative measures for PCT are feasible for detecting patients with VUR. We suggest that in semi-quantitative measurements of PCT, levels between 2.0 and 10.0 ng/ml could be an independent predictor of positive VUR.

High risk pregnancies increase the risk of neonatal mortality and morbidity. In order to identify the influence of pregnancy complications on the period of neonatal stay in Neonatal Intensive Care Units (NICUs), an analysis has been carried out in our center. In a cross-sectional-descriptive analytical study, the data including NICU length of stay was gathered from 526 medical records of neonates. We also assessed their maternal complications such as premature rapture of membranes (PROM), urinary tract infection (UTI), preeclampsia, oligohydramnios, and twin/triplet pregnancy. Finally we analyzed the relation between variables by SPSS statistics software version 19. The level of significance was considered P<0.05. 37 of 526 neonatal medical records were excluded. Of the 489 babies hospitalized in NICU for 1 to 54 days; 28.42% born were preterm, 308 with birth weight <2500 gram and 170 with birth weight between 2500 and 4000 gram. There was a significant relation between length of neonatal NICU stay and maternal PROM (P=0.001), preeclampsia (P=0.01), UTI (P=0.02), multiple gestation (P=0.03), and oligohydramnios (P=0.003). We found a positive correlation between numbers of gestation and length of NICU stay (P=0.03). A positive correlation existed between neonatal complication and length of NICU stay (P<0.001). By increasing maternal health level and prenatal care services, neonatal outcome can be improved and length of stay in NICUs decreased.

We aimed to assess the effect and duration of gluten-free diet on bone health in children with celiac disease in our study. Sixty three patients with celiac disease (CD) formed the study group. They were divided into two subgroups according to their dietary compliance. Bone mineral density (BMD) values of the patients at two and five years of gluten-free diet (GFD) were determined. The relationship between BMD and compliance to GFD was found to be statistically significant (P<0.01). BMD z-scores were increased (0.12±0.15 and 0.10±0.14 units respectively) (P<0.01). The patients in group 1 and 2 had mean -1.18±0.83 and -2.06±0.73 z-scores in the first DXA. In the second DXA, these values were -1.10±0.73 and-1.94±0.93 respectively. Dietary compliance is important for bone health, and the time needed to normalize the BMD is not known. Patients with positive anti-endomysium antibody (EMA), poor dietary history and history of bone pain should be evaluated with DXA during follow-up.

It has been found that asphyxia influences proliferation and differentiation of brain neural stem cells in newborn animal models, and that peripheral blood stem cells play an important role in repairing brain damage. But it has not been reported yet whether asphyxia influences peripheral blood stem cells differentiating into neural cells, and whether with the progress of the disease there is a change of peripheral blood stem cells differentiating into neural cells in newborns with hypoxic ischemic encephalopathy (HIE). Fifty term HIE infants were enrolled in research from March, 2007 to March, 2010. There were 10 cases of the severe HIE patients with good improvement, the severe HIE patients with poor improvement, the moderate HIE patients, the mild HIE patients and the controls, respectively. The peripheral mononuclear cells collected within 24 hours and on 7th day after birth were cultured in vitro for 10 days to differentiate into neural cells. The induced nestin positive cells were identified with Immunohistochemistry and counted. Within 24 hours after birth, there were no difference of induced nestin positive cells among the severe HIE patients with good improvement (68.99±7.85), the severe HIE patients with poor improvement (71.43±6.88),the moderate HIE patients (73.34±6.46), the mild HIE patients (70.46±6.66) and the controls (71.13±7.19, F=0.51, P=0.7). In the severe HIE patients with obvious improvement, the induced nestin positive cells from 7th day peripheral blood mononuclear cells (94.50±15.57) increased markedly compared with that within 24 hours (68.99±7.85, t=4.66, P<0.001), and were higher than the induced nestin positive cells from 7th day peripheral blood mononuclear cells in the severe HIE patients with no obvious improvement (94.50±15.57 vs 69.48±5.32, t=4.62, P<0.001). The ability of peripheral mononuclear cells differentiating into neural cells in term infants with good improvement suffering from severe HIE was enhanced, which may suggest possible relationship between the brain repair and the peripheral stem cells.

The aim of this study was to evaluate the outcome of germ cell tumors in patients admitted to our center during a ten year period. In a retrospective descriptive study, patients with the pathological diagnosis of germ cell tumor (GCT) were included. All records were evaluated and patients followed by personal visit in clinic or phone call. Data regarding age, sex, tumor site, bio-chemical assay, pathology, treatment and outcomes were gathered. For qualitative variables we computed frequency and percentage and for quantitative variables, mean and standard deviation. Survival analysis was performed using Kaplan-Meier. All statistical analyses were performed by SPSS version16.0. Forty four patients consisted of 32 girls (72.7%) and 12 boys (27.3%). Their median age was 23 months. The most common pathological tumor types were 18 (40.9%) mature teratomas and 14 (31.8%) yolk sac tumors. Extra gonadal tumors were more prevalent (32 cases) and consisted of 21 (47.7%) sacrcoccygeal, 7 (15.9%) retroperitoneal, 2 (4.4%) mediastinal and 2 (4.4%) cervical tumors. In gonadal tumors 9 patients had ovarian and 3 patients testicular involvement. Staging at the time of diagnosis revealed stage one in 23 (52.3%) cases. All patients were treated surgically and the most common procedure was total resection in 41 (93.2%) patients. Fifteen (34.1%) patients received chemotherapy. In follow-up 31 (77.5%) patients were in complete remission, 9 (22.5%) had died, and 4 cases did not appear to follow-up visits. The median survival was 16 months (IQR 4-49 months). The highest mortality rate was found in patients with yolk sac tumors (8 of 13 cases). The patients with extra-gonadal GCT and a high AFP level have the worst prognosis and lower survival rate. Combination of surgery and chemotherapy can lead to a better prognosis.

Neonatal lupus erythematosus (NLE) is a transient autoimmune disease of developing fetus and neonate in mothers with systemic lupus erythematosus (SLE). In this report we introduce an infant with NLE whose mother had rheumatoid arthritis. Our case was a 40 day old male infant with discoid-like and annular skin lesions over forehead and neck, irritability and low grade fever. There was a history of prematurity due to preeclampsia. There was no cytopenia or cardiac involvement but liver enzymes were more than 5-fold increased. FANA, Anti Ro and La were negative. The mother had a history of un-controlled rheumatoid arthritis for 12 years with deformity in metacarpal and PIP and ulnar deviation in hands. FANA=1/640 and anti-SSB/La was positive in the mother but there was no other clinical and paraclinical sign of SLE. Without any treatment and during months, the skin and mucosal lesions gradually disappeared without any scar and liver enzymes reached the normal level. After 6 months follow up, he was symptom free with normal growth and development. We recommend to check anti SSA/Ro and anti SSB/La antibodies in all pregnant women with connective tissue diseases to prevent life-threatening involvement of the infant.