Iranian Journal of Pediatric Hematology and Oncology
Volume:6 Issue: 2, Spring 2016

Childhood leukemia, the most common type of cancer in children and teens, is a cancer of the white blood cells. As antioxidants can promote immune system against all types of cancer, the aim of this study was to explore the metabolic regulation and anti-oxidative effect of sesquiterpen-rich extract of Ferula Asafoetida (FA) on children with leukemia.
In this study, 75 leukemic children with a mean age of 5 to 12 years old were treated with doses of 50 and 100 mg of FA extract daily. Then metabolic profile and oxidative biomarkers were assessed. ROS (Reactive oxygen species) and vasculature enzymes SOD (superoxide dismutase)/ CAT(catalase) in the action of extract were also examined.
Receiving FA extract reduced glucose levels of plasma significantly from 105.42±45.22 to 81.40±41.74 mg/dL in 50 mg group and from 101.72±38.82 to 65.86±40.10 mg/dL in 100 mg group (p Treatment with Ferula Asafoetida ethanolic extract regulated the metabolic pathway. Sesquiterpens in the extract confers protection against oxidative stress-induced complications largely through interfering with ROS and PCO/MDA generation.

Childhood cancer as a life-threatening illness has negative consequences for both the patients and their family. The aim of this study was to examine the impact of child cancer on their mother’s quality of life and to assess the association of quality of life with resilience among mothers of children with cancer.
In this cross-sectional study, a total of 145 mothers of children with cancer participated. Data were collected by two questionnaires: (a) PedsQL™ Family Impact Module, (b) Connor-Davidson Resilience Scale. Datawere analyzed using SPSS version 13. Pearson correlation test was run for the analysis.
The result showed that total scale score, HRQOL Summary score, and the Family Functioning Summary Score were 41.68±20.62, 43.10±22.40, and 45.06±24.30, respectively. The mean score of resilience among mothers of children with cancer was 86.80±19.08. There was a positive association between resilience and all dimensions of life quality (p Measuring HRQOL and resilience in a preventive strategy would help to address vulnerable mothers and to present psychosocial support for them. Designing and implementing family-oriented and parent-focused intervention strategies in health care systems are recommended for improving the quality of life of families with cancer children.

Β-thalassemia is known as the most common inherited form of anemia worldwide and Iran. Considering high birth rate in the province, it seem necessary to study demographic factors and to assess common laboratory tests in order to find out whether patients receive adequate care or not.
A cross–sectional descriptive analytical study was conducted on 603 patients aged 1-46 years who had been referred to thalassemia clinic at Ali Asghar Hospital in Zahedan in 2014. Overall mean transfusion interval was 23.4 days with 22.8 days for males and 24.1 days for females. Demographic and laboratory data of the patients were gathered through interviews and the patients’ records.
Out of 603 patients, 323 (54.3%) were male and 280 (45.7%) were female. In total, 77.2% were Balouch, 18.3% Sistani, 3.1% Afghani, and the rest belonged to other ethnic groups. Consanguinity was seen in 57.8% (close relatives) and 19.7% (distant relatives) of cases. Mean number of their offspring was 4.7 and mean number of β-thalassemia children was 1.5 for each family. The mean hemoglobin level before the last three blood transfusions was 9.5 g/dl, and mean ferritin level according to the last three examinations was 3801.9ng/ml. The highest frequency of blood group belonged to “O” (38.4%). In the population studied, the mean volume of transfused blood was 468.5 mL per injection. Regarding used blood products, 93% of the patients received packed cells and 7% washed cells.
In spite of different training programs implementation, the quality of the evaluated indices in this province was lower compared to other regions that can be attributed to high rate of thalassemia incidence. Therefore, regular consultation programs in media seem necessary considering the local culture and language especially through influential local people.

Rhabdomyosarcoma is the most common soft tissue sarcoma among children which has two major subtypes: embryonal rhabdomyosarcoma (ERMS) and alveolar rhabdomyosarcoma (ARMS). Distinction between these subtypes is mandatory to choose proper treatment and to determine prognosis. Histopathologic study is the main method, but nowadays molecular studies like PCR are also used. The aim of this study was to evaluate the frequency of PAX3 and PAX7 mutations in children with rhabdomyosarcoma.
In this cross- sectional survey, Paraffin blocks of 34 Rhabdomyosarcoma cases with mean age of 6.3 ± 2.9 years were studied in Mofid Children's Hospital's Pathology Department, Tehran, Iran, during a 10-year period. Tumoral lesions dissected and embedded in paraffin blocks for PCR study (Tissue dissection method). Pure RNA extraction, cDNA synthesis, and PCR process were performed according to iNtRON biotechnology company kits’ protocols. All of these cases were analyzed regardingPAX3 and PAX7 mutations.
Out of 34 cases, 32 were ERMS and two were ARMS. None of the ERMS samples was t (2; 13) or t (1; 13) positive. Moreover, two ARMS cases were negative for PAX3 and PAX7 mutations. No significant difference was seen for age below and above five years (P= 0.69) as well as for tumor location (trunk tumor and limbs/head tumor) (P= 0.11).
This study revealed lack of PAX3 and/or PAX7 mutations in both ERMS and ARMS. However, careful morphological evaluation cannot be replaced by the PCR-based t(2;13) and t(1;13) assay of childhood sarcomas, it can be used to make certain current histopathological diagnosis.

Bachgroun: Hematopoietic stem cell derived from umbilical cord blood (UCB) has been used for regenerative medicine in hematological abnormalities. MicroRNAs are important regulators of gene expression that control both physiological and pathological processes such as development of tissue and cancer. Some studies have shown that miR-33, has a critical role in control of self-renewal cells. Here to understand the role of adipose derived mesenchymal stem cells (ASCs) as a feeder layer on expansion of hematpoietic stem cells (HSCs), the expression of p53 and miR-33a were evaluated.
Material and Isolated human ASCs in passage 3 were cultured as a feeder layer. Ex vivo cultures ofcord bloodCD34 cells that expand in three culture conditions for 7 days were performed in following groups: fresh CD34 cells, in the present of cytokines, directly cultured on feeder layer,indirectly cultured on feeder layer(cultured on thincert plate with 0.4 µm pore size). Expression of genes p53 and Mir-33a were analyzed by real-time PCR.
The expression of p53 was significantly (p0.05) up regulated in HSCs directly cultured on feeder layer compare to without feeder layer.
Defining the role of ADSCs in controlling the HSC self-renewal through mir33 and p53may lead to the prevention and treatment of hematopoietic disorders.

Immune thrombocytopenic purpura (ITP) is a disease characterized by decrease of the peripheral blood platelet count. The disease presents in acute and chronic forms. Because of the importance and high prevalence of ITP, it was decided to study the therapeutic response of patients with ITP.
Material and A cross-sectional study was conducted at Ghaem hospital, Mashhad, Iran. The diagnosis was based on clinical symptoms and laboratory findings. All of the patients were treated by ITP conventional treatment. Then, the therapeutic response was evaluated.
The population of this study included 288 ITP patients, 159 were diagnosed with acute ITP (132 pediatric: 6 months to12 years and 27 adult: 13-58 years old) and 69 with chronic ITP (43 female and 26 male). Among pediatric patients, 75 were girls and 57 boys. The mean age in acute ITP was 11 years and in chronic ITP was 28 years old. According to T-test, significant relation was found between age in type of ITP (P=0.000). In men, %77 suffered from acute ITP and %23 from chronic form while in female acute ITP was %62 and chronic ITP was %38. According to Fisher-Test, no significant relation was found between sex in type of ITP (P=0.842). After the conventional treatment with prednisolone, 19 cases were considered as corticosteroid-refractory ITP. The second step in treatment of corticosteroid-refractory ITP was splenectomy. Responsiveness to splenectomy was 84% and 16% were unresponsive to splenectomy and immunosuppressive therapy, and they expired with signs of bleeding.
The acute type of ITP was common among patients at age range of 3 to 4 years. The chronic ITP was more common among adults and also more frequent in female. Splenectomy in patients with chronic ITP was associated with a good response in most cases, but the response to immunosuppressive agents in this group was poor.

Investigation of toxicity of silver nanoparticle, especxially blood toxicity, is necessary because this nanoparticle is used a lot in diffrerent parts of life and invironment. The aime of this is evaluation the effects of silver nanoparticles with different concentrations on blood cells pregnant Wistar rats.
In this case control study, 30 pregnant rat were divided into 5 experimental groups (three treatment groups, one control group, and one injection control group). Treatment and Control groups received different concentration of silver nanoparticle (250, 500, and 1000 ppm) and normal food and water conditions during the experiment, respectivelly for 18 days. After this rime, rats were investigated in terms of blood cells.
The results showed that being treated with silver nanoparticles led to significant reduction of RBCs at the concentrations of 500 and 1000 ppm (p Based on results, silver nanoparticles revealed toxic effects on blood cells in high concentration (1000 ppm). So, these nanoparticles must be used with more caution.

Acute myeloid leukemia (AML) is a malignant disorder involving blood cells, characterized by obstructed or distorted differentiation of hematopoietic stem cells. In cytogenetically normal AML (CN AML), molecular abnormalities in NPM, FLT3, CEBPA, and BAALC genes are observed. Initially, high BAALC (Brain and Acute Leukemia Cytoplasmic gene) expression was introduced in a study on AML cases with trisomy 8. The present systematic review aimed to determine the prognostic value of BAALC expression in CN-AML patients within the age range of 1 month to 15 years.
In this systematic review, scientific databases including PubMed, Scopus, Embase, ISI, and Cochrane Library were searched. All the evaluated retrospective studies focused on the prognostic value of BAALC expression.
Overall, BAALC expression was reported in 30-60% of AML patients (mean=45%). High expression of BAALC gene was reported in M0, M1, and M2 subtypes of pediatric AML. In only one study, in addition to these subtypes, M4 was also reported (12.5% of cases).
This systematic review resulted in a significant association between high BAALC expression and poor response to chemotherapy in pediatric AML. However, OS (Overall Survival) and EFS (Event - Free Survival) findings were contradictory, relapse and death increase in the presence of high BAALC expression. Therefore, BAALC expression could be considered as a clinical prognostic marker for the treatment of AML patients. Further efficient prognostic indicators should be identified in order to gather information about therapeutic strategies for AML and risk analysis in these patients.

Adrenocortical tumors (ACTs) are extremely rare in infants. Pediatric ACTs are therapeutic and diagnostic challenge because histological criteria for distinguishing benign from malignant tumors seen in adults are not always reliable in children .The aim of this report was to present clinical features, hormonal profile, and histopathological characteristics of an infant with Adrenal tumors.
A 10-month-old boy with adrenocortical tumors (ACT) presented with restlessness and pseudoprecocious puberty. Plasma testosterone, dehydroepiandrosterone, and 17-OH progesterone were increased. An abdominal spiral computed tomography (CT) scan with intravenous contrast identified a well-defined left-sided suprarenal mass with areas of necrosis. Left adrenalectomy was performed. Immunohistochemical staining showed that tumor cells were strongly positive with inhibin and focal positive with vimentin. The diagnosis of adrenocortical carcinoma was made on the basis of immune histochemistry. The postoperative course was uneventful.
This case emphasized the importance of early consideration of an adrenocortical tumors causing pseudoprecocious puberty in infant. The final pathologic diagnosis should be based on imunohistochemical staining patterns.